Sankara Nethralaya

Atlas of
Imaging in Ophthalmology
 Sankara Nethralaya
Atlas of
Imaging in Ophthalmology
Ambika Selvakumar
Director
Department of Neuro-ophthalmology
Sankara Nethralaya
(A Unit of Medical Research Foundation)
Chennai, Tamil Nadu, India

Veena Noronha
Consultant and Radiologist
Department of Radiology
Sankara Nethralaya, VRR Scan
Chennai, Tamil Nadu, India

Padmaja Minakshi Sundaram

Ex-consultant
Department of Neuro-ophthalmology
Sankara Nethralaya
Chennai, Tamil Nadu, India

Foreword
Neil R Miller

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Sankara Nethralaya Atlas of Imaging in Ophthalmology

First Edition: 2014

ISBN: 978-81-8448-900-2
Printed at
FOREWORD
Optimum patient care is, for the most part, dependent on pattern recognition. In the past,
this meant recognizing the cause of a combination of symptoms and signs. However, the
increasing availability of accurate diagnostic tests, particularly with respect to imaging, means
that physicians who deal with such patients also must be up-to-date with respect to
recognizing the imaging characteristics produced by a wide variety of disorders, including
congenital lesions, traumatic damage, tumors, infections, inflammations, and vascular lesions.
Atlas of Imaging in Ophthalmology provides the typical (and atypical) manifestations and
imaging findings produced by both common and uncommon neuro-ophthalmologic
disorders. The physician who understands and remembers these patterns will be well-
equipped to deal with the complex disorders that affect the eye, the orbit, and the afferent
and efferent visual pathways.

Neil R Miller MD FACS

Wilmer Eye Institute
Johns Hopkins Hospital
Baltimore, USA
PREFACE
In this last two decades, there have been intense revolutionary changes in the field of imaging and their applications
in various aspects of ophthalmology and particularly in neuro-ophthalmology. This book aims to cover the important
aspects of computed tomography (CT) and magnetic resonance imaging (MRI) in the diagnosis of various ophthalmic
diseases. Since there is a relative lack of a specific ophthalmology textbook or atlas that deals with CT and MRI
aspects in ophthalmology, and we have enormous data and collection of images of various common and relatively
rare clinical conditions, we decided to put everything together to guide all practicing ophthalmologists, neurologists
and radiologists in their day-to-day practice.
The atlas consists of extensive collection of CT and MRI images of various ocular, orbital and neuro-ophthalmic
disorders. It also deals with most of the neurological diseases with ophthalmic manifestations with vivid portrayal
of their imaging characteristics. All the clinical images used in this atlas belong to patients seen only in our organization
(Sankara Nethralaya: A Unit of Medical Research Foundation), which is a tertiary referral ophthalmic institution.
It can be used as a reference book for undergraduates, ophthalmology, radiology and neurology postgraduates.
The atlas also covers the recent advances in imaging, particularly in the techniques and hardware of magnetic
resonance imaging. Imaging studies are constantly undergoing refinement in technology (both software and
hardware), thereby enhancing the imaging quality and reporting. It is, therefore, of paramount importance that
clinicians and radiologists keep themselves updated on all the latest developments in these fields. It has been our
constant effort in the atlas to include, wherever, possible the latest updates in the field of imaging pertaining to
ophthalmology.

Ambika Selvakumar
Veena Noronha
Padmaja Minakshi Sundaram
ACKNOWLEDGMENTS
We are very grateful to our Chairman Emeritus, Dr SS Badrinath, for his constant encouragement in bringing this
extensive Atlas of Imaging in Ophthalmology. We have been greatly influenced by Dr Lingam Gopal, an explicit teacher
and guide, who motivated us to do this work and also gave constructive criticism. Our sincere thanks to all our
colleagues, Dr Vijayananth, Dr Shikha Bassi, Dr Smita Menon, Dr Muna Bende, who helped us to collect these
plethora of images and also helped us in writing the atlas.
Special thanks to our medical photographic department who took careful photographs of all our patients. Our
sincere thanks to Mr Mohan, Mr Deena of the multimedia department for scanning and archiving all the patients’
CT and MRI films. We appreciate the hardwork put in by our secretaries Mr Shreekanth and Mrs Usha in collecting
the patients’ clinical data and pictures.
We profusely thank all the patients who enriched our learning experience and consented us to use their imaging
and clinical pictures.
We are extremely grateful to Dr Deepak Arjundas, Dr G Arjundas, Dr Suresh Bapu, Dr Balamurugan, Dr Siddarth
Ghosh for their constant referral of patients to us and also helping us to manage these patients. Our families have
given immense support in completing the work. The atlas represents Sankara Nethralaya’s experience in the field of
imaging and its implications in ophthalmology. Last but not least, we sincerely acknowledge the endless support
and cooperation of M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India.
CONTENTS
SECTION 1: PHYSICAL PRINCIPLES OF COMPUTED TOMOGRAPHY AND MAGNETIC RESONANCE IMAGING

1. Physical Principles of Computed Tomography and Magnetic Resonance Imaging 3

SECTION 2: THE EYE

2. Imaging Anatomy of the Eye 21

3. Congenital Anomalies of the Globe 36
4. Inflammatory Lesions of the Eye 46
5. Intraocular Neoplasms 63
6. Leukocoria: Lesions Simulating Retinoblastoma 96
7. Detachment of Retina, Choroid and Vitreous 109
8. Intraocular Calcification 113
9. Ocular Trauma 119
10. Postoperative Evaluation of Eye and Orbit 131

SECTION 3: DISORDERS OF ORBIT

11. Imaging Anatomy of the Orbit 141

12. Congenital Lesions of the Orbit 158
13. Infective Lesions 170
14. Inflammatory Lesions 183
15. Vascular Lesions of the Orbit 197
16. Pediatric Malignant Orbital Tumors 217
17. Lymphoproliferative Disorders 226
18. Histiocytic Lesions 242
19. Lacrimal Gland Lesions 248
20. Neurogenic Tumors 271
21. Fibrous Lesions 276
22. Secondary Orbital Tumors and Paraorbital Lesions 282
23. Thyroid Eye Disease 302
24. Orbital Metastases 307
25. Orbital Trauma 310
xii Sankara Nethralaya Atlas of Imaging in Ophthalmology

SECTION 4: VISUAL PATHWAY LESIONS: OPTIC NERVE DISORDERS

26. Anatomy and Clinical Features 323

27. Congenital Optic Nerve Anomalies 326
28. Papilledema 339
29. Optic Neuritis 345
30. Infiltrative Optic Neuropathy 368
31. Traumatic Optic Neuropathy 389
32. Radiation Optic Neuropathy 396

SECTION 5: DISORDERS OF CHIASM

33. Anatomy and Clinical Features 403

34. Chiasmal Disorders in Pediatric: Young Adult 408
35. Chiasmal Disorders in Middle Age: Elderly 429
36. Chiasmal Disorders: No Age Predilection 452

SECTION 6: RETROCHIASMAL VISUAL PATHWAY LESIONS

37. Anatomy and Clinical Features of the Retrochiasmal Visual Pathway 467
38. Congenital Lesions 475
39. Infections 486
40. Hydrocephalus 504
41. Vascular Lesions 508
42. Intracranial Neoplasms 545

SECTION 7: EFFERENT PATHWAY LESIONS

43. Ocular Motility Disorders 603

44. Chronic Progressive External Ophthalmoplegia 628
45. Internuclear Ophthalmoplegia 630
46. Supranuclear: Gaze Palsy 634
47. Olivopontocerebellar Atrophy 638
48. Nystagmus 640

SECTION 8: DEMYELINATION

49. Primary Demyelination 659

50. Secondary Demyelination 672
 Contents xiii

SECTION 9: PHACOMATOSIS

51. Neurofibromatosis 683

52. Sturge-Weber Syndrome 690
53. Tuberous Sclerosis 694
54. Von Hippel-Lindau Disease 701
55. Wyburn-Mason Syndrome 706

SECTION 10: APPLICATIONS OF INTERVENTIONAL RADIOLOGY IN OPHTHALMOLOGY

56. Neurovascular Diseases in Ophthalmology: Diagnosis and Management 713

Index 739
SECTION 1
Physical Principles of
Computed Tomography and
Magnetic Resonance Imaging
SECTION OUTLINE
1. Physical Principles of Computed Tomography
and Magnetic Resonance Imaging
Chapter 1
Physical Principles of
Computed Tomography
and Magnetic Resonance Imaging
BASICS OF COMPUTED TOMOGRAPHY (CT)
The first CT scanner was developed by Sir Godfrey
Hounsfield in 1972; since then this modality has become
an important tool in diagnostic radiology. From the first
scanner to the present day multislice helical scanner, CT
technology has revolutionized the world of imaging and
enhanced patient management.
BASIC PHYSICS
Computed tomography uses X-rays to obtain cross-
sectional, two-dimensional images of the body. The cross-
sectional image is produced by 360° rotation of the X-ray
tube around the patient. The transmitted radiation is
measured by the detectors located inside the gantry like
a ring around the patient. The final image is generated
from these measurements. The gantry of the CT machine
houses the X-ray tube and the detectors (Fig. 1.1).
TYPES OF SCANNING TECHNIQUES
Axial (Sequential) Scanning
In sequential scanning, single slice is obtained with single
360° rotation of the tube (Fig. 1.2A). The disadvantage is
that the time taken for an individual study is long, hence
prone to motion artifacts and quality of reformations is
suboptimal.
Helical (Spiral) Scanning
With the advent of slip ring technology, the continuous
rotation of the X-ray tube around the patient is made
possible during continuous patient table movement. This
led to the development of helical scanning (Fig. 1.2B).
The transmitted radiation thus takes the form of a helix
or spiral around the patient acquiring volume of data.
This allows larger anatomical coverage of the body part
to be imaged, thereby reducing the possibility of artifacts
caused by patient movement especially pediatric and
critically ill patients. Faster scanning also increases patient
throughput.
Multislice or multidetector machines utilize the
principles of the helical scanner but incorporate multiple
rows of detector rings. They can therefore, acquire
multiple slices per tube rotation, thereby increasing the
anatomical coverage in a shorter time (Fig. 1.3).
CT TERMINOLOGIES
Pixel and Voxel
Every CT image is made of a square of matrix called the
picture element (pixel) and an object volume called voxel
(Fig. 1.4). The obtained CT image is subdivided into a
matrix of up to 512 × 512 or 1024 × 1024 elements. The
pixel width is determined by the field of view (FOV) and
matrix size, i.e. FOV/matrix. The voxel volume = pixel
area × slice thickness.
Higher the pixel, better is the image resolution.
HOUNSFIELD UNIT OR CT NUMBER
Each voxel is traversed during the scan by numerous
X-ray photons and the intensity of the transmitted
radiation is measured by the detectors. From these
intensity readings, the density or attenuation value, viz
Hounsfield unit or CT number is calculated and assigned
to every tissue.
4 Section 1 Physical Principles of CT and MRI

Fig. 1.1: Cross-sectional view of the gantry showing the orienta-

tion of the X-ray tube and detectors in a fourth generation CT
scanner

Fig. 1.3: Multislice imaging—generation of six slices per rotation of

the tube in a multidetector scanner

Fig. 1.2A: Sequential scan—single cross-sectional slice of the patient Fig. 1.4: Pixel represents the matrix and voxel represents the slice
in a single rotation thickness

Fig. 1.2B: Helical scan—rotation of the tube around the patient with Fig. 1.5: Scale representing the range of Hounsfield numbers of the
continuous table movement tissues seen in the body

Each pixel is assigned a numerical value (CT number),
based on the attenuation of X-rays by the tissue. This
number is compared to the attenuation value of water
and displayed on a scale of arbitrary units named
Hounsfield units (HU) after Sir Godfrey Hounsfield.
This scale assigns water as an attenuation value (HU)
of zero. Each number represents a shade of gray with
+1000 (white) and –1000 (black) at either end of the
spectrum (Fig. 1.5).
CT number of various tissues in the body is as follows:
• Brain gray matter o 35 – 40 HU
• Brain white matter o 30 – 35 HU
• Blood o Flowing blood – 40 HU
Acute hematoma o 70 to 90 HU (density depends on
the Hb concentration and coagulation profile)
• Calcification o + 80 and above
• Fat o – 10 to – 100
• CSF o 0 to 10 HU
• Bone o + 800 to 1000 (depends on the type of bone).
Window Level (WL) and Window Width (WW)
The term ‘window level’ represents the central
Hounsfield unit of all the numbers within the window
width. The window width covers the HU of all the tissues
of interest and these are displayed as various shades of
gray. Tissues with CT numbers outside this range are
displayed as either black or white. Both the WL and WW
can be set independently on the computer console and
their respective settings affect the final displayed image
(Figs 1.6A and B).
Slice Thickness
It is the collimation of the X-ray beam as it emerges from
the X-ray tube. The slice thickness can be varied
depending on the anatomical region to be covered by
varying the beam collimation. For example, orbit
scanning is done using 2 to 3 mm slice thickness, posterior
fossa using 4 to 5 mm slice thickness and supratentorial
brain parenchyma using 10 mm slice thickness.
Pitch
Pitch is the terminology used in helical scanning and
denotes the distance traveled by the table (in millimeters)
during one complete rotation of the X-ray tube, divided
by the slice thickness (millimeters). Increasing the pitch
by increasing the table speed, reduces dose and scanning
time, but at the cost of decreased image resolution (Figs
1.7A and B).
Table distance per 360q rotation (mm)
Pitch =
Slice thickness (mm)
Chapter 1 Physical Principles of CT and MRI 5
IMAGE POST-PROCESSING
Post-processing the acquired volumetric data during
spiral CT is done in ways appropriate to the clinical
situation such as:
• Multiplanar reformatting (MPR): After obtaining
the serial axial volumetric data, the computer
can reconstruct the data in sagittal and coronal planes.
With the current multislice CT scanner, it is possible
to obtain isotropic sagittal and coronal reconstructions
thereby reducing patient radiation dose. These are
useful in pediatric patients and trauma patients who
cannot be positioned for direct coronal (Fig. 1.8).
• Three-dimensional imaging: The acquired data can
also be projected as a three-dimensional model to
display spatial information or surface characteristics
(volume and surface rendering). This is useful in
pediatric craniofacial anomalies and post-trauma for
maxillofacial injuries to guide the surgeon in treatment
planning (Fig. 1.9).
• CT angiography (CTA): This involves injection of 100
to 120 ml of contrast media rapidly using a pressure
injector at a predetermined rate of injection. Serial
axial images are obtained. These images are then used
for reconstruction of the data using maximum
intensity projection to get a display of the vascular
tree. By altering the time of image acquisition and
contrast injection, we can obtain only the arterial or
venous phases (Figs 1.10A and B).
CT Contrast Media
These are iodine containing compounds. Iodine absorbs
X-rays within the CT range (120 KVp) since iodine has
an atomic number of 53 and atomic weight of 127.
There are two types of contrast agents used:
1. Ionic contrast: These are sodium or methylglucamine
combined with tri-iodinated benzene ring to form
soluble salts. These are hyperosmolar and hence are
likely to cause severe contrast reactions. These are
contraindicated intrathecally.
2. Nonionic contrast: These are near iso-osmolar and
hence tend to produce fewer side effects and
considered relatively safe for patients.
Absolute contraindication for contrast:
1. Previous contrast sensitivity
2. Abnormal renal parameters
Patients with diabetes and multiple myeloma are
more likely to develop altered renal function post-IV
contrast injection.
6 Section 1 Physical Principles of CT and MRI

A B

Figs 1.6A and B: (A) Soft tissue window settings of an axial CT scan of the brain (WW = 100, WL = 30) and (B) Bone window
setting of an axial CT scan of the brain (WW = 2000, WL = 220)

Figs 1.7A and B: (A) A low pitch—tight helic and (B) A pitch of more Fig. 1.8: Coronal reformation of the face showing fractures involving
than one—loose helic—shorter scan time at the cost of image the lateral wall of the left maxillary sinus, zygoma, lateral wall of the left
resolution orbit and frontal bone
 Chapter 1 Physical Principles of CT and MRI 7

Fig. 1.9: Three-dimensional volume rendered CT image of the skull

A B

Figs 1.10A and B: CT angiogram of the brain: (A) Showing axial MIP image and (B) Volume rendering showing the intracranial circulation
8 Section 1 Physical Principles of CT and MRI
Patients with myasthenia gravis, sickle cell anemia
and pheochromocytoma are at risk of developing
contrast-induced symptoms.
ADVANTAGES AND CLINICAL USE
OF COMPUTED TOMOGRAPHY
• CT is readily available in most hospitals and cost-
effective
• It is a rapid imaging modality with excellent image
resolution hence useful in trauma, pediatric and un-
cooperative patients
• Patients in whom MRI is contraindicated.
DISADVANTAGES
• Radiation: The effective doses from diagnostic CT
procedures are typically estimated to be in the range
of 2 to 5 mSv.
BIBLIOGRAPHY
1. Bushberg JT, Seibert JA, Leidholdt EM, Boone JM. The
essential physics of medical imaging, 2nd edn.
Philadelphia, Pa: Lippincott Williams and Wilkins, 2001.
2. Barrie Grossman C. Magnetic resonance imaging and
computed tomography of the head and spine. Williams
and Wilkins, 1996.
3. Huda W, Chamberlain CC, Rosenbaum AE, Garrisi W.
Radiation doses to infants and adults undergoing head
CT examinations. Med Phys 2001;28:393-9.
4. Mahadevappa Mahesh. Search for Isotropic Resolution in
CT from Conventional through Multiple-Row Detector.
Radiographics 2002;22:949-62.
5. Seeram E. Computed tomography: Physical principles,
clinical applications, and quality control. Philadelphia, Pa:
Saunders, 2001.
6. Zatz L. General overview of computed tomography
instrumentation. In Potts D (Ed). Radiology of the skull
and brain: Technical aspects of computed tomography. St
Louis, Mo: Mosby 1981;pp.4025-57.

BASICS OF MAGNETIC RESONANCE IMAGING
Magnetic resonance imaging (MRI) is based on the
principles of nuclear magnetic resonance (NMR).
PHYSICAL PRINCIPLES OF MR IMAGING
Magnetic resonance imaging is based on the absorption
and emission of energy in the radiofrequency range of
the electromagnetic spectrum. The human body is
primarily made of fat and water which has many
hydrogen atoms (almost 63%). The hydrogen atom (1H)
consists of a single positively charged proton which spins
around its axis. These charged particles create an
electromagnetic field, similar to that of a bar magnet
(Fig. 1.11).
The proton possesses a property called spin which
has a small magnetic field. These spinning particles have
a net magnetic moment which has both magnitude and
direction. In the absence of an external magnetic field,
these protons are randomly oriented.
When placed in a magnetic field of strength B, the
protons align themselves parallel or antiparallel to the
external magnetic field. There is a low energy state where
the poles are aligned N-S-N-S and a high energy state
N-N-S-S. These particles can undergo a transition
between the two energy states by the absorption of a
photon. A particle in the lower energy state absorbs a
photon and ends up in the upper energy state. The energy
of this photon must exactly match the energy difference
between the two states.
Application of a radiofrequency (RF) pulse of appro-
priate duration and amplitude excites these protons from
the lower energy state to the higher energy state.
The MRI signal results from the energy difference of
the spins emitted during transition from the higher
energy state to the lower energy state. The signal is thus
proportional to the population difference between the
states (Figs 1.12A and B).
When the RF pulse is applied, the protons are tipped
into the horizontal or X-Y plane by an angle termed as
the flip angle depending on the type of RF pulse. The
rate at which the protons precess is termed as frequency
and the angular position of the precessing spin is called
the phase of the spin.
The frequency of precession ( f ) is called the Larmor
frequency and is characteristic of the specific nucleus
and strength of the external magnetic field and is
expressed as:
f = JB
Chapter 1 Physical Principles of CT and MRI 9
Where f = mHz/sec, B is expressed in Tesla and J is the
gyromagnetic ratio of the specific nucleus and expressed as mHz/T.
Hydrogen has the highest gyromagnetic ratio and is the most
abundant body element, hence is the natural choice for H signal.
Radiofrequency Field
Every nucleus in the body precesses at its own Larmor
frequency and will produce an MR signal only when the
RF energy is delivered at the correct frequency. The
excitation RF pulses are delivered by coils that produce
an RF field perpendicular to the external magnetic field.
The RF is absorbed by the nuclei and the magnetic
moment is tipped away from the Z axis, i.e. axis of the
external magnetic field depending on the duration and
amplitude of the RF pulse.
Free-induction Decay
When the RF pulse is switched off, then magnetic
momentum of the nuclei begins to return to its original
position, thereby transferring the absorbed energy and
inducing alternating current signal in the receiver coil. This
is termed as free-induction decay (FID). As this occurs
immediately after the RF pulse, this signal is not used for
image data. The magnetization is manipulated to generate
a useful signal termed as echo, which produces the image.
T1 and T2 Relaxation
When the RF pulse is switched off, two processes take
place simultaneously:
a. Recovery of the net magnetic moment in the Z axis—
termed as longitudinal or T1 relaxation. T1 is the time
required for the build-up of 63 percent of the original
magnetization along the Z axis (Figs 1.13A to C).
b. Loss of phase coherence in the X-Y plane or transverse
plane, termed as T2 relaxation.
The nuclei while returning to the ground state
dissipate their excess energy to their surroundings, which
is called the lattice. This process is named as spin-lattice
relaxation (Fig. 1.14). Smaller molecules reorient more
rapidly than larger molecules. The medium-size
molecules, such as lipids relax faster as their frequency
of rotation is closer to the Larmor frequency than that
associated with pure water or larger molecules, such as
proteins. Thus, T1 relaxation times depend on magnetic
field strength because the latter affects the Larmor
frequency. Thus, water has long T1s.
10 Section 1 Physical Principles of CT and MRI

Fig. 1.11: Every spinning particle possesses a magnetic moment

(z) and creates a magnetic field similar to a bar magnet

Figs 1.12A and B: (A) Showing protons outside a magnetic field

and (B) Showing exited protons in a magnetic field moving from
a lower energy level to a higher energy level with two distinct
energy levels. The population difference is directly proportional
to the magnetic field strength

The transverse magnetization occurs due to magnetic
field generated by the surrounding electrons allow the
precessing nuclei to experience different field strength.
Loss of transverse magnetization (phase coherence)
occurs as the magnetic moments get out of phase as a
result of their mutual interaction. Anything that changes
the magnetic field strength also changes the precessional
frequency and causes a loss of phase coherence
(dephasing) and shrinking of the transverse
magnetization. This is called T2 relaxation or spin-spin
relaxation (Fig. 1.15). It denotes the loss of phase
coherence caused by interactions between neighboring
magnetic moments. T2 is the time required to reduce the
transverse magnetization to 37 percent of its original
value.
In biological tissues, the main contribution to T2
relaxation is from the relatively static magnetic field from
neighboring protons. Large molecules, which tend to
reorient more slowly than small molecules, promote T2
relaxation and have shorter T2 times. Free water has a
longer T2 than water associated with macromolecules.
The T2 is relatively independent on the field strength.
Repetition Time
The time between two RF excitation pulses is called the
repetition time (TR). The TR can be chosen from a certain
minimum value, depending on the imaging technique
and the MR system, to very long times.
Longer values of TR allow more T1 relaxation to occur,
and this property can be exploited to manipulate the
contrast between tissues with different T1s or the signal-
to-noise ratio in an image.
Echo Time
The time from the center of the RF excitation pulse to the
center of the echo is the echo time (TE). The amplitude of
the transverse magnetization at the echo peak depends
on TE and T2 of the tissue. As TE is prolonged, the
transverse magnetization becomes weaker. Adjusting TE
influences the contrast between tissues that have
different T2s.
Slice Orientation
The orientation of a slice, i.e. axial, coronal or sagittal
depends on which of the three magnetic field gradients
is activated during the RF pulse. An RF pulse in the
presence of the z-gradient creates a transverse slice. The
x- and y-gradients select slices in the sagittal and coronal
orientations, respectively. Oblique slices are created by
activating two or more gradients during an RF pulse.
Chapter 1 Physical Principles of CT and MRI 11
Slice Position
Slices are located where the Larmor frequency matches
the frequency of the RF pulse. The slice-selection gradient
lowers the Larmor frequency on one side of the center of
the magnet and raises it on the other side. Slice position
is controlled by changing the frequency of the RF pulse
because changing the amplitude of the slice-selection
gradient would inadvertently alter the thickness of the
slice.
INSTRUMENTATION
The key components of an MR system are the magnet,
the gradient, the radiofrequency subsystem, and the
computer.
The Magnet
The magnet is the main component of the MR system.
There are three types of magnets in common use for
MRI—permanent magnets, resistive electromagnets, and
superconducting electromagnets. The higher the field
strength better is the signal-to-noise ratio. The strength
of the magnetic field is measured in Gauss (G) or Tesla
(T) units (10,000 G = 1 T). Diagnostic MR systems
usually employ magnets with operating field strengths
ranging from 0.02 to 3T. Research systems operate
above 3T.
Superconducting Magnets
These are most commonly used magnets and operate at
field strength above 0.5T. Some metals (e.g. Hg) and alloys
(e.g. niobium/titanium, Nb/Ti; niobium/tin, Nb3Sn; and
vanadium/gallium, V3Ga) lose their electrical resistance
at very low temperatures and become superconductors.
The superconductor most widely used in the construction
of clinical magnets is Nb/Ti. This alloy becomes
superconducting at 10° Kelvin (K) in the absence of an
external magnetic field. This temperature is provided by
a bath of liquid helium (4° K) (Fig. 1.16).
Resistive Magnets
A resistive magnet is an electromagnet in which the
magnetic field is generated by the passage of electrical
current through a wire. The disadvantage is their high
power consumption limiting field strength.
Permanent Magnets
It uses a horse-shoe magnet. An advantage of these low-
field permanent magnet systems is that their C-shaped
12 Section 1 Physical Principles of CT and MRI
Figs 1.13A to C: (A) Alignment of the proton along the direction
of the external magnetic field (B0) in the z-axis, (B) After applying
the RF pulse of an appropriate frequency, the magnetization (M0)/
protons are tipped away from its equilibrium in the x-y plane and
(C) If a longer pulse lasting twice as long the magnetization is
inverted
Fig. 1.14: Spin-lattice relaxation time
Fig. 1.15: Spin-spin relaxation—T2 relaxation—loss of magne-
tization in the x'-y' plane is faster than the loss of magnetization in
the z-direction due to loss of phase coherence of the microscopic
components
Fig. 1.16: Schematic representation of the superconducting MR
systems. The bore is surrounded by the coils of the wire through
which electric current is passed and cooled by liquid helium to achieve
magnetization and desired field strength. The current is disconnected
once magnetized

design is patient friendly and therefore useful in
claustrophobic patients. Their field strength is limited to
0.5T (Fig. 1.17).
Magnetic Field Gradients
Magnetic field gradients are activated as pulses for a short
duration at timed intervals. It is a magnetic field that
increases in strength along a particular direction, e.g. x-,
y-, and z-gradients, according to the direction of change
of the magnetic field strength. The strength of a gradient
refers to the rate at which its magnetic field changes with
distance.
RF System
The excitation of the nuclei is done with a short duration
RF pulse close to or at the Larmor frequency of the nuclei.
The desired frequency is produced by a frequency
synthesizer.
The receiver detects signals in the high- and very-high-
frequency (HF and VHF) range. The magnetic resonance
signals are typically a few zV in amplitude.
Transmitter and Receiver Coils
The body part to be examined is placed inside a coil.
Separate coils can be used for transmitting and receiving
or a single coil can be used for both excitation and
detection (transceiver coil).
A coil is a winding of low-resistance wire, usually
made of copper. Volume coils are used for large body
parts. Surface coils are coils used to study small region
such as orbit. The advantage of surface coils is that their
signal-to-noise ratio is better as the part is close to the
coil. Surface coils can receive a good signal from the
tissues within the depth of half of its diameter.
COMMONLY USED PULSE SEQUENCES
Spin-Echo Pulse Sequence
In a spin-echo pulse sequence two RF pulses, i.e. 90° and
180o are applied spaced by a time interval of TE/2. After
the nuclei are excited by a 90° pulse, the spins dephase in
the x’-y’ plane, this is followed by a refocusing 180° pulse.
The faster spins lie behind the slower ones, but at time
TE/2 they make up, thus producing an echo. The 180°
pulse results in reversal of the phase of each spin. The
position of the spins has not changed, so they will
continue to rotate in the same direction. However, the
180° pulse causes the spins to return towards their starting
point (alignment), rather than rotating further away from
it. This 90°-180° pulse sequence is called spin echo (SE)
sequence (Fig. 1.18).
Chapter 1 Physical Principles of CT and MRI 13
By altering the echo delay time (TE), and the sequence
repetition time (TR), the SE sequence can be used to obtain
T1, T2, or proton density images.
The spin echo sequence has been largely replaced by
faster sequences such as fast spin echo and fast GRE
(Gradient recalled echo).
GRADIENT-ECHO IMAGING
Gradient-echo imaging is an imaging technique by which
images can be acquired in much shorter times than
conventional pulse sequences. The basic difference
between spin-echo and gradient-echo imaging is that,
gradient-echo uses gradient reversals to get an echo and
spin-echo uses 180-degree rephrasing pulse and gradient-
echo uses flip angle < 90 degrees (Fig. 1.19).
INVERSION RECOVERY IMAGING
The inversion recovery sequence uses a 180-degree
inverting pulse, a 90-degree pulse, and a rephrasing
180-degree pulse. The inversion time (TI) is determined
by the TR and T1 of the tissue needed to be suppressed
(Fig. 1.20). Commonly used inversion recovery pulse
sequences are:
• Fluid attenuated inversion recovery (FLAIR),
whereby the CSF bright signal is suppressed. It is now
a routinely used sequence in brain imaging and
especially to image periventricular plaques in multiple
sclerosis
• Short-tau inversion recovery (STIR) sequence, mainly
used in imaging the optic nerves. It suppresses the
orbital fat and highlights the lesions within the optic
nerve mainly in optic neuritis.
MAGNETIC RESONANCE ANGIOGRAPHY (MRA)
Advantages of MRA vs catheter angiogram:
• Noninvasive or minimally invasive
• Three-dimensional information can be obtained
• Can give surrounding soft tissue details.
Disadvantage: Flow dynamic information is lacking.
Techniques of Magnetic Resonance Angiography
The commonly used techniques in clinical practice are:
• Time-of-flight (TOF) MR angiogram
• Phase contrast (PC) MR angiogram
• Contrast-enhanced MR angiogram (CE-MRA).
Time-of-flight Angiogram
This is the most widely used MR angiography technique
for imaging the intracranial circulation. It gives reliable
14 Section 1 Physical Principles of CT and MRI

Fig. 1.17: Schematic diagram of a permanent MR system showing

the generation of the magnetic field in a vertical direction by
magnetized ceramic blocks

Fig. 1.19: Formation of a gradient echo. Instead of the 180° pulse,

a gradient pulse (–G) is used followed by a second gradient pulse
of opposite polarity (+G). In gradient-echo sequence, the signal
decay is determined by T2*, which is always less than T2

Fig. 1.18: Diagram of a spin-echo pulse sequence—spin-echo

pulse sequence. The spin system is excited by a 90° pulse.
After a time delay (W), one or several 180° pulses follow. This
leads to the formation of an echo. The time between the 90°
pulse and the peak of the echo is called echo time (TE). TR is
the repetition time between two complete pulse sequences
Fig. 1.20: Pulse sequence diagram of an inversion recovery pulse
sequence. The 180° inverting pulse is followed by a 90° pulse
and 180° rephasing pulse

vascular information without the need for intravenous
contrast.
Basic principle involves suppression of the static
background tissue and retaining the signal from the
flowing blood. The saturation of the stationary tissue is
done by using very short TR so that the stationary spins
do not have enough time to regain their longitudinal
magnetization. The flowing (unsaturated) spins which
enter the slice are unaffected by the slice selective RF
pulse, will be fully magnetized producing a bright signal
(Fig. 1.21). The signal produced is directly proportional
to the velocity of the flowing blood.
The flow saturation will occur when the spins in the
imaging volume are not entirely replenished after each
pulse.
The time-of-flight angiogram can be obtained using
2D or 3D sequences. In a 2D sequence, sequential thin
sections are obtained whereas in 3D a slab of tissue is
excited. Each of them has their advantages and
disadvantages.
2D angiograms are used to evaluate slow flowing
blood, but susceptible to turbulent flow. It has less spatial
resolution.
3D angiograms have high spatial resolutions and less
susceptible to turbulent flow.
Phase Contrast MR Angiogram
Moving spins undergo a phase shift in the presence of
paired opposing gradients. This phenomenon is utilized
in phase contrast MR angiography. The amount of phase
shift increases with increasing flow velocity. When the
flowing blood (moving spins) moves along the direction
of the gradient field, it precesses faster as the field increase
and undergoes a phase change. Thus, the motion is phase
encoded giving it both direction and magnitude
(Fig. 1.22).
The amount of phase shift is directly proportional to
the flow velocity, gradient strength and time interval
between the gradient applications. By choosing an
appropriate velocity encoding value (VENC), fast or slow
flowing blood can be imaged.
Phase contrast MR angiography can be acquired as
both 2D and 3D sequences.
Advantages of phase contrast MRA: It gives:
• Flow quantification
• Flow direction
• Excellent background suppression
• Can be used for imaging areas of slow flow.
Disadvantage: Long scan time.
Chapter 1 Physical Principles of CT and MRI 15
Contrast-enhanced MR Angiography (CE-MRA)
The limitations of TOF and PC angiograms such as flow
saturation, flow-related artifacts, breathing and pulsation
artifacts made depiction of blood vessels in the body
especially the abdomen difficult.
By using intravenous contrast and rapid gradient
imaging, it is now possible to obtain MR angiography
images almost at par with conventional angiogram.
The technique involves capturing of high magnetiza-
tion strength during the first pass of the vascular contrast,
i.e. gadolinium by appropriate timing using 3D
acquisition (Fig. 1.23).
Advantages:
• Insensitive to saturation effects of the RF pulse as
against TOF angiogram. Therefore, can cover vessels
over larger field of view
• Useful in large aneurysms where flow is complex.
MR CONTRAST
Most of the contrast agents in clinical use enhance tissue
relaxation. Gadolinium is a rare earth element and toxic
by itself, hence it is chelated with multidentate ligands
for safety such as diethylenetriamine pentetate (DTPA)—
tetra-azacyclododecane tetra-acetic acid (DOTA). It is a
paramagnetic substance and shortens the T1 relaxation
and hence makes the tissues with contrast appear bright.
Safety
• These contrast agents are considered safe with rate of
adverse reaction such as nausea and vomiting (1 to
2%) and hives (1%). Severe anaphylactoid reactions
have been reported with an estimate rate at 1 in
200,000 and 1 in 400,000
• These contrast agents can be safely used in children
above 2 years
• They should not be used in patients with com-
promised renal function. There have been cases
reported of nephrogenic systemic fibrosis in patients
with compromised renal function
• Should not be used in pregnancy as its bioeffect on
the fetus has not been established.
Newer Advanced MR Imaging Techniques
Diffusion Weighted Imaging
It is based on the principle of Brownian motion, which is
dispersion or random translation of a molecule in a liquid
due to thermal agitation.
Motion of molecules in biological tissues is complex.
Neuronal tissue consists of tightly and coherently packed
16 Section 1 Physical Principles of CT and MRI

Fig. 1.23: Contrast-enhanced TRICKS (Time Resolved Imaging

Fig. 1.21: Schematic representation of time-of-flight angiogram of Contrast Kinetics) angiogram image of the brain

A – Spins in the stationary tissue at time 0

B – Spins dephasing after exposed to gradients
C – Spins rephasing after switching off gradient Fig. 1.24: Axial diffusion weighted image showing restricted
D – Stationary spins rephased while moving spins are out of phase diffusion in the right occipital lobe suggestive of acute right posterior
Fig. 1.22: Schematic diagram of a phase contrast MR angiogram cerebral artery territory infarct

axons surrounded by glial cells. The movement of water
molecules is hindered in a direction perpendicular to the
orientation of the axonal fibers. Thus, motion of molecules
in biological tissues is anisotropic. The cell membranes
are thought to be responsible for anisotropic diffusion
rather than myelin. The restricted diffusion appears as a
bright signal on diffusion weighted images (Fig. 1.24).
Application:
• Stroke
• Multiple sclerosis
• Tumors
• Trauma
• Abscess.
Lesions bright on diffusion images:
• Acute infarct
• Bacterial abscess
• Acute demyelination
• Epidermoid cyst
• Tissues with high cellularity
• Subacute hemorrhage.
Functional Imaging
It is the demonstration of brain activation to a specific
stimulus based on the functional anatomy of the brain, for
example, the primary visual cortex is activated using a
flicker display or alternating checkerboard pattern as a
visual stimulus. Once the brain is activated using a
stimulus, there is change in the blood flow to the particular
region to supply the increased demand for oxygen and
glucose. This increase in the oxygen, i.e. deoxyhemoglobin
concentration causes local susceptibility effects which is
used to receive the signals using appropriate pulse
sequences. This is termed blood oxygen level dependent
(BOLD) contrast imaging (Fig. 1.25).
MAGNETIC RESONANCE SPECTROSCOPY (MRS)
MR spectroscopy utilizes the differences in the resonance
frequency of nuclei due to their different chemical bond.
This is also termed as chemical shift imaging. The
frequency difference varies with the magnetic field and
is directly proportional to the external magnetic field. It
is expressed in parts per million (ppm). The advantages
of a higher field strength while performing spectroscopy
is that it provides better signal-to-noise ratio and better
separation of metabolite peaks.
1H (proton) spectroscopy is used for brain imaging
as it is easy to perform and gives a better signal-to-noise
ratio as compared to 23Na and 31P. Of all the atomic nuclei,
1 H has the strongest response and found in all
biochemicals. MR spectroscopy thus provides details of
the brain chemistry (Figs 1.26A and B). The spectrum is
Chapter 1 Physical Principles of CT and MRI 17
read from right to left and the metabolites detected on
brain spectroscopy are:
• Lipid 0.9 -1.4 ppm
• Lactate 1.3 ppm
• N-acetyl asparatate (NAA) at 2 ppm
• Creatine (Cr) 3.0 ppm
• Choline (Cho) 3.2 ppm
• Myo-inositol 3.5 ppm.
The TE affects the metabolites detected, thus short
TE ~ 30 ms shows metabolites with short and long T2
relaxation times and with long TE ~ 270 ms only
metabolites with long T2 relaxations times are detected,
therefore the spectrum primarily consist of NAA, Cr and
Cho. Another advantage of long TE ~ 144 ms is that the
lactate peak at 1.3 ppm gets inverted.
Rather than absolute concentrations, one should rely
on the various ratios to give a clinical diagnosis.
Ratio Normal Abnormal
NAA/Cr 2.0 < 1.6
NAA/Cho 1.6 < 1.2
Cho/Cr 1.2 > 1.5
Indications:
• Tumors
• Radiation necrosis vs recurrence
• Infections
• Neurodegenerative disorders
• Metabolic brain disorders
• Stroke.
MR spectroscopy should be carefully interpreted and
correlated with MR images to make a final diagnosis.
BIBLIOGRAPHY
1. Barrie Grossman C. Magnetic resonance imaging and
computed tomography of the head and spine. Williams
and Wilkins, 1990.
2. David D Stark, William G Jr Bradley. Magnetic resonance
imaging, 3rd edn. CV Mosby.
3. Edelman R, Hesselink R, John, Zlatkin B Michael, Crues
V John. Clinical magnetic resonance imaging, 3rd edn,
Philadelphia: Saunders-Elsevier, 2006.
4. Peter A Rink. Magnetic Resonance in Medicine. The Basic
textbook of the European Magnetic Resonance Forum, 5th
revised edn, 2003.
5. Patric Hagmann, Lisa Jonasson, Philippe Maeder, Jean-
Philippe Thiran, Van J Wedeen, Reto Meuli.
Understanding Diffusion MR Imaging Techniques: From
Scalar Diffusion-weighted Imaging to Diffusion Tensor
Imaging and Beyond R. Radiographics 2001;21:767-79.
6. Ross BD, Colletti P, Lin A. MR spectroscopy of the brain:
Neurospectroscopy in Edelman. Hesselink, Zlatkin
and Crues (Eds): Clinical Magnetic Resonance Imaging,
3rd edn, Philadelphia: Saunders-Elsevier 2006;
pp.1840-910.
18 Section 1 Physical Principles of CT and MRI

Fig. 1.25: fMRI showing activation of the motor cortex after right
finger-tapping

A B

Figs 1.26A and B: Multivoxel MR spectroscopy: (A) Showing the voxel placed in the normal parietal white matter with NAA color
map and (B) Showing normal spectrum
SECTION 2
The Eye
SECTION OUTLINE
2. Imaging Anatomy of the Eye
3. Congenital Anomalies of the Globe
4. Inflammatory Lesions of the Eye
5. Intraocular Neoplasms
6. Leukocoria: Lesions Simulating Retinoblastoma
7. Detachment of Retina, Choroid and Vitreous
8. Intraocular Calcification
9. Ocular Trauma
10. Postoperative Evaluation of Eye and Orbit
Chapter 2
MR TECHNIQUE
High resolution MR imaging of the eye has taken a giant
leap in evaluating intraocular lesions. Though ultrasound
is still the first line investigation while imaging the eye,
MR plays a very vital role in characterizing intraocular
tumors, their orbital extension and any associated
intracranial disease.
MR imaging of the eye at our institution is performed
on a 1.5 T scanner (HDx GE, Milwaukee) using 3-inch
surface coil and the following protocol:
Axial and coronal T1-weighted images are obtained
using 3D fast spoiled gradient recalled (FSPGR) sequence
using the following parameters:
• TR = 20 ms and TE = 4.8 ms
• FA (flip angle – 15°)
• Slice thickness is 1 to 3 mm depending on the clinical
findings
• FOV = 14-16 mm
• Matrix = 256 × 256
• NEX = 2
• Axial planes are taken parallel to the optic nerve using
a 3 plane localizer
• Coronal planes are obtained perpendicular to the axial
planes.
Axial and coronal T2-weighted images are obtained
using 3D Fast spin echo sequence using the following
parameters:
• TR =2000 ms, TE = 85 ms
• Matrix = 256 × 256
• FOV = 14 mm
• NEX = 1
• Sagittal T1 or T2-weighted images are obtained
parallel to the optic nerve (oblique sagittal plane).
Postcontrast fat suppressed axial, coronal and sagittal
views are obtained preferably in the same plane as the
precontrast study using the following parameters:
Imaging Anatomy
of the Eye
THREE-DIMENSIONAL FSPGR
• TR = 20 ms and TE = 4.8 ms
• FA (flip angle – 15°)
• Slice thickness is 1 to 3 mm depending on the size of
the lesion
• FOV = 14-16 mm
• Matrix = 256 × 256
• NEX = 2
Brain imaging is done both pre- and postcontrast
using head coil:
• Precontrast screening is done using axial FLAIR
sequence
• Postcontrast is done in axial plane using T1 fast spin
echo sequence
Initial screening of both orbits is done using head coil
and axial T1 spin echo and T2 fast spin echo sequences.
Advantages of Surface coil Technique
• High signal to noise ratio allows high resolution
imaging
• Anterior structures, viz globe and the anterior optic
nerve best imaged using this technique.
Disadvantages
• Signal drop towards the apex
• Susceptible to motion artifacts—can be reduced by
applying an eye pad and closing the lid (routinely done
at our institution) or open and fix the eyes at a point
(practically difficult). Reflex blinking can be reduced by
applying topical anesthetic drops or artificial teardrops.
MR IMAGING ANATOMY OF THE EYE
MRI is far superior to CT scan for imaging the globe,
especially for intraocular lesions. On MR, the various
structures in the globe are identified based on their
different T1 and T2 values (Figs 2.1A and B).
22 Section 2 The Eye

Fig. 2.1A: Cross-section of eye anatomy

Fig. 2.1B: Axial T1-weighted image at the level of the optic nerve

• The cornea and sclera show medium-to-low signal
intensity on T1-weighted images and T2-weighted
images
• The ciliary body is hyperintense on T1-weighted
images and hypointense in T2-weighted images
• The iris is 0.3 to 0.6 mm in thickness and hence not
seen on clinical MR images
• The chorioretinal layer in which the normal retina is
0.2 to 0.3 mm cannot be differentiated from the highly
vascularized choroids on routine scans of 2 to 3 mm
slice thickness
• The aqueous and vitreous humor are hypointense on
T1-weighted images and hyperintense signal on T2-
weighted images
• Normal crystalline lens is composed of 65 percent
water and 35 percent protein and shows intermediate
signal on T1-weighted images and hypointense
signal on T2-weighted images due to short T2
relaxation time.
EXTRAOCULAR MUSCLES
The extraocular muscles show medium signal in T1- and
T2-weighted images.
CONNECTIVE TISSUE SYSTEM
• The tarsal plate is T1-hyperintense due to its lipid
content
• Fibrous orbital septum is T1-hypointense
• The intermuscular septum has similar signal as the
extraocular muscles.
ARTERIES
• Ophthalmic artery originating from the internal
carotid artery is visible on oblique sagittal images
• The intraorbital ophthalmic artery appears at the
lateral side of the optic nerve where it gives off central
retinal artery
• Distal to the bend or “knee” it crosses over the optic
nerve and runs forward first medial to superior
oblique and then between the superior oblique and
medial rectus muscle
• As the central retinal artery crosses over the optic
nerve it gives off the ciliary arteries on either side of
the optic nerve
• The central retinal artery enters the dural sheath
approximately 1 cm behind the globe
• Inferior to the trochlea the ophthalmic artery ends as
the dorsal nasal artery
• The lacrimal artery is seen close to the lacrimal gland.
• The supraorbital artery is seen between the orbital roof
and levator palpebrae superioris muscle.
Chapter 2 Imaging Anatomy of the Eye 23
VEINS
• The superior ophthalmic vein starts inferior to the
trochlea as its anastomosis with the angular vein as
continuation of the nasofrontal vein. It continues
posteriorly initially anteromedial to optic nerve and
then posterolateral over the ophthalmic artery to enter
the superior orbital fissure
• Inferior ophthalmic vein branches are seen in the
inferomedial orbit and the main trunk is visualized
lateral to the inferior rectus muscle.
NERVES
• The motor nerves, viz the superior and inferior branch
of the oculomotor nerve, abducens nerve and thin
trochlear nerve are identified in the coronal images
• The sensory nerves, viz; branches of the ophthalmic
division of the trigeminal nerve (frontal, lacrimal and
nasociliary) are identified on MR images
• The frontal nerve and its branches are identified on
axial and coronal images superior to the levator
palpebrae superioris
• The lacrimal nerve is best seen in coronal sections
• The infraorbital nerve is seen in the infraorbital canal.
The Optic Nerve
The optic nerve can be divided into four sections:
1. Intraocular optic nerve: It is approximately 1 mm in
length.
2. The intraorbital optic nerve has an S-shaped course
from the optic canal to the globe. The normal mean
pial diameter of the intraorbital segment of the optic
nerve measures between 3.2 mm (anterior) and 2.6
mm (posterior), the mean dural diameter measures
5.2 mm (anterior) and 3.9 mm (posterior).
3. The intracanalicular optic nerve is 5 mm long. The
ophthalmic artery is below the optic nerve within the
canal. The ethmoid and sphenoid sinus form the
medial wall, the lesser wing of the sphenoid the roof,
anterior clinoid process lateral wall and optic strut
caudal wall.
4. The intracranial optic nerve is surrounded by CSF.
The subarachnoid space between the pial and dural
sheath of the optic nerve is normally 0.5 to 0.6 mm
wide.
LACRIMAL SYSTEM
The lacrimal gland is superolateral to the globe. It shows
isointense signal in T1-weighted images and hypointense
signal in T2-weighted images. It is divided into the orbital
and palpebral lobes by the LPS aponeurosis.
The lacrimal sac and nasolacrimal duct are best seen
on T2-weighted images when fluid filled. The lacrimal
canaliculi are not visualized on routine MR imaging.
24 Section 2 The Eye

AXIAL MR ANATOMY OF THE EYE

T1WI 2 mm Thick Sections taken in Axial Plane from Inferior to Superior

Fig. 2.2: Axial T1-weighted image of the eye at the level of the inferior oblique tendon

Fig. 2.3: Axial T1-weighted image at the level of the inferior oblique insertion
 Chapter 2 Imaging Anatomy of the Eye 25

Fig. 2.4: Axial T1-weighted image at the level of the inferior rectus muscle

Fig. 2.5: Axial T1-weighted image just inferior to the optic nerve
26 Section 2 The Eye

Fig. 2.6: Axial T1-weighted image at the level of the optic nerve

Fig. 2.7: Axial T1-weighted image at the level just above of the optic nerve
 Chapter 2 Imaging Anatomy of the Eye 27

Fig. 2.8: Axial T1-weighted image at the level of the trochlea

Fig. 2.9: Axial T1-weighted image at the level of the superior rectus
28 Section 2 The Eye

Fig. 2.10: Axial T1-weighted image above the superior rectus muscle
 Chapter 2 Imaging Anatomy of the Eye 29

CORONAL MR ANATOMY OF THE EYE

2 mm Thick Coronal T2-weighted Images from Anterior to Posterior

Fig. 2.11: Coronal T2-weighted image at the level of the orbital septum

Fig. 2.12: Coronal T2-weighted image at the level of the inferior oblique muscle
30 Section 2 The Eye

Fig. 2.13: Coronal T2-weighted image of the eye at the level of inferior oblique insertion

Fig. 2.14: Coronal T2-weighted image at the level of the trochlea

 Chapter 2 Imaging Anatomy of the Eye 31

Fig. 2.15: Coronal T2-weighted image just beyond the equator

Fig. 2.16: Coronal T2-weighted image at the level of the optic nerve head
32 Section 2 The Eye

OBLIQUE SAGITTAL MR ANATOMY OF THE EYE

2 mm Thick T2 Section from Lateral to Medial

Fig. 2.17: Sagittal T2-weighted image of the eye at the level of the lacrimal gland

Fig. 2.18: Sagittal T2-weighted image at the level of the lateral orbit
 Chapter 2 Imaging Anatomy of the Eye 33

Fig. 2.19: Sagittal T2-weighted at the inferior rectus muscle

Fig. 2.20: Sagittal T2-weighted image at the level of inferior rectus muscle
34 Section 2 The Eye

Fig. 2.21: Sagittal T2-weighted image at the level of the optic nerve

Fig. 2.22: Sagittal T2-weighted image at the level of the medial rectus
 Chapter 2 Imaging Anatomy of the Eye 35

Fig. 2.23: Sagittal T2-weighted image at the medial orbit

Chapter 3
Congenital Anomalies
of the Globe
CONGENITAL CYSTIC EYE
• Congenital cystic eye results due to failure of the optic
vesicle to invaginate during the 4th week of embryo-
genesis
• It can be associated with other nonocular abnormali-
ties such as facial clefts, choanal atresia, malformation
of the sphenoid bone, agenesis of corpus callosum and
midbrain deformities.
IMAGING
Imaging is done to confirm the diagnosis and evaluate
any associated intracranial anomalies.
CT Orbit
• The normal globe is not visualized
• Cystic structure of CSF density not resembling the
normal globe is seen in the orbit. If large can cause
remodeling of the bony orbit
• A rudimentary connection to a thinned optic nerve
may be seen
• The cyst may have an attached stalk. If the stalk is
patent, then size of the cyst remains small due to
communication of the cyst with the cranial cavity
• The ipsilateral superior orbital fissure may be widened
• Extraocular muscles and optic nerve are usually
absent.
MRI Orbit
• On MR, the signal intensity of the cyst fluid may not
be equal to that of normal vitreous because the cyst is
normally filled with serum
• A nodular focus may be seen adjacent to the cyst wall.
DIFFERENTIAL DIAGNOSIS
Microphthalmia with colobomatous cyst.
BIBLIOGRAPHY
1. Baghdassarian SA, Tabbara KF, Matta CS. Congenital
cystic eye. Am J Ophthalmol 1973;76:269-75.
2. Guthoff R, Klein R, Lieb WE. Congenital cystic eye. Graefes
Arch Clin Exp Ophthalmol 2004;242(3):268-71.
3. Hayashi N, Repka MX, Ueno H, Iliff NNT, Green WR.
Congenital cystic eye; Report of two cases and review of
literature. Surv Ophthalmol 1999;44:173-9.
4. Kaufman LM, Villablanca JP, Mafee MF. Diagnostic
imaging of cystic lesions in the child’s orbit. Radiol Clin
North Am 1998;36(6):1149-63.
5. Mansour AM, Li HK. Congenital cystic eye. Ophthal Plast
Reconstr Surg 1996;12(2):104-7.
6. Pasquale LR, Romayananda N, Kubacki J, et al. Congenital
cystic eye with multiple ocular and intracranial anomalies.
Arch Ophthal 1991;109:985-7.
7. Shields JA, Shields CL. Orbital cysts of childhood:
classification, clinical features, and management. Surv
Ophthalmol 2004;49(3):281-99.
 Chapter 3 Congenital Anomalies of the Globe 37

A B

Figs 3.1A to C: (A) Axial CT scan of the orbit showing a large

right intraorbital cyst with nonvisualization of normal globe and
extraocular muscles, (B) Axial CT scan at higher level showing
a large cyst attached to the rudimentary optic nerve (arrow)
and (C) Axial CT scan of the orbit at higher level showing
C intraorbital cyst with an adjacent rudimentary tissue (arrow)
38 Section 2 The Eye
ANOPHTHALMIA
• Anophthalmia is complete absence of an eye by birth
due to a developmental defect
• Structures not derived from the neuroectoderm such
as extraocular muscles, eyelids, conjunctiva, lacrimal
apparatus and bony orbit are relatively preserved
• Bilateral anophthalmia is rare.
CT/MRI FINDINGS
• Small rudimentary tissue present
• Dystrophic calcification may be present within the
rudimentary tissue
• Bony orbit is smaller in size
• The optic nerve and extraocular muscles are
hypoplastic
• In true anophthalmia, optic nerve and optic chiasm
may be absent. Optic tracts may be rudimentary and
lateral geniculate body may be gliotic.
RECOMMENDED IMAGING
CT or MRI orbit with brain to look for intracranial
abnormalities especially midline defects, hydrocephalus
and intracerebral cysts.
BIBLIOGRAPHY
1. Char DH, Unsold R. Computed tomography: Ocular and
orbital pathology. In: Newton TH, Bilanuik LT (Eds).
Modern Neuroradiology, Volume 4. Radiology of the eye
and orbit. New York: Raven Press 1990;pp. 9.1-9.64.
2. Handler LF, Heher KL, Katowitz JA. Congenital and
acquired anophthalmia. Curr Opin Ophthalmol
1994;5(5):84-90.
3. O’Keefe M, Webb M, Pashby RC, et al. Clinical
anophthalmos. Br J Ophthalmol 1987;71:635-822.
4. Yanoff N, Duker JS. Ophthalmology. Philadelphia:
CV Mosby, 1999.
Chapter 3 Congenital Anomalies of the Globe 39

Fig. 3.2: Axial CT scan of the orbit showing absent left globe
with hypoplastic extraocular muscles (long arrow) and small
bony orbit. Also noted is a nasal dermoid (white arrow)

Fig. 3.3: Three-dimensional CT of the skull of the same patient

showing an extremely small left bony orbit
40 Section 2 The Eye
MICROPHTHALMIA
• Microphthalmia is defined as an eye that has an axial
length of less than 21 mm in an adult or less than
19 mm in a one-year-old child
• It can occur as an isolated disorder or with other ocular
and craniofacial anomalies such as Hallermann-Streiff
syndrome or systemic abnormalities such as micro-
phthalmos, dermal aplasia and sclerocornea (MIDS)
• Other causes of microphthalmia include congenital
rubella syndrome, persistent hyperplastic primary
vitreous and retinopathy of prematurity.
• Microphthalmia is of two types:
1. Small anatomically correct eye—simple
2. Small malformed eye complex.
It may be unilateral or bilateral. Bilateral microphthalmia
can be seen in Lowe’s syndrome (oculocerebral renal disease).
It may or may not be associated with colobomatous
cysts. Those with colobomatous cysts result from failure
of fusion of the fetal optic fissure.
CT/MR FINDINGS
• A small globe with small bony orbit may be seen
• Extraocular muscles and optic nerve are hypoplastic
• Colobomatous cyst may vary from small to large
almost occupying the orbit
• Colobomatous cyst may cause a conical deformity on
the posterior globe
• Cysts can be very large and at times larger than the
microphthalmic eye resulting in bony remodeling
• Often intraocular calcification may be seen which may
represent calcified lens.
BIBLIOGRAPHY
1. Char DH, Unsold R. Computed tomography: Ocular and
orbital pathology. In: Newton TH, Bilanuik LT (Eds).
Modern Neuroradiology, Volume 4. Radiology of the Eye
and Orbit. New York: Raven Press 1990;pp.9.1-9.64.
2. Foxman S, Cameron JD. The clinical implications of
bilateral microphthalmos with cyst. Am J Ophthalmol
1984;97:632-8.
3. Harris GJ, Dolman PJ, Simons KB. Microphthalmos with
cyst. Ophthalmic Surg 1992;23:432-3.
4. Lieb W, Rochels R, Gronemeyer U. Microphthalmos with
colobomatous orbital cyst: clinical, histological, immuno-
histological, and electron-microscopic findings. Br J
Ophthalmol 1990;74(1):59-62.
5. McLean CJ, Ragge NK, Jones RB, Collin JR. The
management of orbital cysts associated with congenital
microphthalmos and anophthalmos. Br J Ophthalmol
2003;87(7):860-3.
6. Tucker S, Jones RB, Collin JRQ. Systemic anomalies in
77 patients with congenital anophthalmos or micro-
phthalmos. Eye 1996;10:310-4.
7. Wright DC, Yuh WTC, Thompson HS, et al. Bilateral
microphthalmos with orbital cysts: MR findings. J Comput
Assist Tomogr 1987;11:727-9.
8. Waring GO 3rd, Roth AM, Rodrigues MM. Clinico-
pathologic correlation of microphthalmos with cyst. Am J
Ophthalmol 1976;82(5):714-21.
9. Yanoff N, Duker JS. Ophthalmology. Philadelphia: CV
Mosby, 1999.
 Chapter 3 Congenital Anomalies of the Globe 41

A B

Figs 3.4A and B: (A) Clinical photograph of a child showing microphthalmic right eye and (B) Axial CT scan of the orbit of the
same patient showing a small right globe (arrow) with multiple retro-ocular cysts (block arrow) resulting in expansion of the bony
orbit

A B

Figs 3.5A and B: (A) Clinical photograph showing a microphthalmic right eye, which is displaced upwards and forwards. Fullness
seen in right lower lid suggestive of inferior periocular cyst and (B) Axial CT orbit showing a small right globe with a large solitary
retro-ocular cyst causing posterior conical defect of the globe (arrow). The left eye shows a large disc coloboma (curved arrow)

Fig. 3.6: Coronal CT orbit showing small left globe (arrow) with
calcified lens and an inferior orbital colobomatous cyst (block
arrow) displacing the globe superiorly
42 Section 2 The Eye
CRYPTOPHTHALMIA
• Cryptophthalmia consists of partial or complete
failure of development of the eyelids, eyebrow,
palpebral fissure, eyelashes and conjunctiva
• Patients will have hidden eyes because the skin of the
eyelids is partially or fully sealed
• It is classified into three types:
1. Complete—eyelid is completely fused over
existing eye
2. Incomplete—eyelid is partially fused over existing
eye
3. Abortive—eyelid is completely fused and
underlying eye does not form.
• It may be unilateral or bilateral
• It may be associated with skin like cornea, an
incompletely developed anterior segment or a
rudimentary cyst-like globe
• Cryptophthalmos may be associated with systemic
anomalies
• Fraser’s syndrome consists of cryptophthalmos
associated with other malformations such as
abnormalities of genitals, kidneys, larynx, and ear and
abnormal or fused digits (syndactyly)
• There may be abnormal or absent punctums/
canaliculi and associated hypertelorism.
IMAGING (CT/MRI)
Incompletely developed anterior segment, or a
rudimentary cyst-like globe.
BIBLIOGRAPHY
1. Dollfus H, Verloes A. Dysmorphology and the orbital
region: A practical clinical approach. Surv Ophthalmol
2004;49(6):547-61.
2. Kabra M, Gulati S, Ghosh M, Menon PS. Fraser-crypto-
phthalmos syndrome. Indian J Pediatr 2000;67(10):
775-8.
3. Kanhere S, Phadke V, Mathew A, Irani SF. Cryptoph-
thalmos. Indian J Pediatr 1999;66(5):805-8.
4. Pe’er J, BenEzra D, Sela M, Hemo I. Cryptophthalmos
syndrome: Clinical and histopathological findings.
Ophthalmic Paediatr Genet 1987;8(3):177-82.
5. Slavotinek AM, Tifft CJ. Fraser syndrome and
cryptophthalmos: Review of the diagnostic criteria and
evidence for phenotypic modules in complex malformation
syndromes. J Med Genet 2002;39(9):623-33.
 Chapter 3 Congenital Anomalies of the Globe 43

A B C

Figs 3.7A to C: Axial CT scan of the orbit in a case of cryptophthalmos: (A and B) Showing a malformed left globe (thin arrow)
with malformed anterior chamber, absent lens and an anterior cystic component (thick arrow) and (C) Showing intraocular
calcification (arrowhead). The posterior half of the globe and optic nerve are well preserved
44 Section 2 The Eye
STAPHYLOMA
• Staphyloma is defined as areas of scleral ectasia that
are lined by uveal tissue. It can be anteior or posterior
• High myopia causes posterior staphyloma. Less
prominently, one can see equatorial staphylomata at
sites of scleral thinning
• Staphyloma involving the ciliary body is called ciliary
or intercalary staphyloma depending on its location.
It is caused by glaucoma and scleritis
• Anterior staphyloma develops secondary to infection,
trauma or radiotherapy. It consists of ectatic pseudo-
cornea (formed by fibrous tissue) lined by uveal tissue.
CT/MRI FINDINGS
• It is seen as a focal bulge of the ocular coats
• Posterior staphyloma is seen as a focal bulge in the
ocular coats temporal to the optic disc
• In anterior staphyloma, as the name suggest the bulge
is on the anterior ocular surface.
BIBLIOGRAPHY
1. Brodsky MC. Congenital optic disk anomalies. Surv
Ophthalmol 1994;39:89-112.
2. Curtin BJ. The posterior staphyloma of pathologic myopia.
Trans Am Ophthalmol Soc 1977;75:67-86.
3. Pruett RC. Complications associated with posterior
staphyloma. Curr Opin Ophthalmol 1998;9(3):16-22.
4. Steidl SM, Pruett RC. Macular complications associated
with posterior staphyloma. Am J Ophthalmol 1997;
123(2):181-7.
Chapter 3 Congenital Anomalies of the Globe 45

Fig. 3.8: Axial CT orbit showing a focal bulge of the anterior

ocular coats (anterior staphyloma) (arrow) with eccentric
calcification (block arrow)

Fig. 3.9: Axial CT orbit of a myopic patient showing a focal

bulge temporal to optic disc with scleral thinning—posterior
staphyloma (arrow)
Chapter 4
Inflammatory Lesions
ENDOPHTHALMITIS/PANOPHTHALMITIS
• Endophthalmitis is an inflammation of the eyeball
without involvement of the sclera. If the sclera is also
involved, then it is called panophthalmitis, usually
caused by an infection
• Sterile or noninfectious endophthalmitis is caused by
retained lens material and toxic agents
• Endophthalmitis is of two types: Endogenous
(i.e. metastatic) and exogenous
1. Endogenous endophthalmitis results from the
hematogenous spread of organisms from a distant
source of infection (e.g. endocarditis)
2. Exogenous endophthalmitis results from direct
inoculation as a complication of ocular surgery,
foreign bodies, or penetrating ocular trauma.
Organisms permeate the blood-ocular barrier
either by direct invasion (i.e. septic emboli) or by
changes in vascular endothelium caused by substrates
released during infection.
• Destruction of intraocular tissues may be due to direct
invasion by the organism and/or from inflammatory
mediators of the immune response
• The inflammation could be localized predominantly
to the anterior segment involving the iris, ciliary body
and the area of the intraocular lens with some spill
over vitritis, or could involve the entire globe with
grossly purulent exudates filling the vitreous cavity
• The inflammation can also spread to involve the
orbital soft tissue
• Bacterial endophthalmitis usually presents acutely
with pain, redness, lid swelling, and decreased visual
acuity. However, some bacteria (e.g. Propionibacterium
of the Eye
acnes) may cause chronic inflammation with mild
symptoms
• Fungal endophthalmitis may have an indolent course
over days to weeks. It is usually seen in penetrating
trauma with plant substance or soil-contaminated
foreign bodies.
IMAGING
• CT scan is usually performed to look for intraocular
foreign bodies in patients with history of trauma and
patients with suspected orbital cellulitis
• Thickening of the sclera and uveal tissues with
increased density in the vitreous and periocular soft
tissue structures may be seen
• MR imaging is rarely performed. It is reserved for
patients with orbital infection and cavernous sinus
involvement
• In MR imaging, the vitreous shows hyperintense
signal better appreciated in FLAIR sequence. The
orbital fat may show streaky soft tissue (stranding)
when infection spreads to the orbit. Thickening of the
uvea is better appreciated on T1 weighted images.
A two-dimensional echocardiogram and chest X-ray
should be done if an endogenous cause is suspected
• Ultrasound typically shows choroidal thickening and
echoes in the anterior and posterior vitreous which
support the diagnosis
• Retained lens material may also be seen in post-
cataract surgery inflammation and could be
contributing to the inflammation in addition to the
infection itself
 Chapter 4 Inflammatory Lesions of the Eye 47

A B

Figs 4.1A and B: A 54-year-old diabetic patient with history of cataract surgery developed signs of intraocular inflammation:
(A) Axial T1-weighted image and (B) Axial FLAIR images of the orbit showing mild proptosis of the left eye with thickening of the
ocular coats and increased signal in the vitreous cavity. The retinal detachment is appreciated in FLAIR image. Streaky soft
tissues are noted in the periocular and retrobulbar fat with mild thickening of the optic nerve suggestive of orbital spread of
infection

A B

Figs 4.2A and B: A 13-year-old female presented with clinical diagnosis of panophthalmitis: (A) Axial T2-weighted image and
(B) Coronal T2-weighted image of the orbit showing proptosis of the left eye with thickened ocular coats, retinal detachment and
periocular soft tissue thickening. A well-defined T2 hyperintense lesion with hypointense rim is seen in the inferotemporal episcleral
region—probably an abscess (dotted arrow)—A case of panophthalmitis with periocular abscess
48 Section 2 The Eye

Fig. 4.3: Axial CT scan of the orbit showing proptosis of the

right eye with increased density of the vitreous, absent lens,
thickened ocular coats with periocular and retrobulbar fat
stranding in a case of panophthalmitis

• Ultrasonography can detect retinal detachment that
may be important in the planning of the management.
BIBLIOGRAPHY
1. Aaberg TM Jr, Flynn HW Jr, Schiffman J, Newton J.
Nosocomial acute-onset postoperative endophthalmitis
survey: A 10-year review of incidence and outcomes.
Ophthalmol 1998;105(6):1004-10.
2. Lalitha P, Rajagopalan J, Prakash K, Ramasamy K, Prajna
NV, Srinivasan M. Postcataract endophthalmitis in South
India incidence and outcome. Ophthalmol 2005;112
(11):1884-9. Epub 2005 Sep 12.
3. Leibovitch I, Lai T, Raymond G, Zadeh R, Nathan F, Selva
D. Endogenous endophthalmitis: A 13-year review at a
tertiary hospital in South Australia. Scand J Infect Dis
2005;37(3):184-9.
4. Miller JJ, Scott IU, Flynn HW Jr, Smiddy WE, Newton J,
Miller D. Acute-onset endophthalmitis after cataract
surgery (2000-2004): Incidence, clinical settings and visual
acuity outcomes after treatment. Am J Ophthalmol
2005;139(6):983-7.
5. Norregaard JC, Thoning H, Bernth-Petersen P, Andersen
TF, Javitt JC, Anderson GF. Risk of endophthalmitis after
cataract extraction: Results from the International Cataract
Surgery Outcomes Study. Br J Ophthalmol 1997;81(2):
102-6.
6. Rumboldt Z, Moses C, Wieczerzynski U, Saini R.
Diffusion-weighted imaging, apparent diffusion
coefficients, and fluid-attenuated inversion recovery MR
imaging in endophthalmitis. AJNR 2005;26(7):1869-72.
7. Soriano ES, Nishi M. Endophthalmitis: Incidence and
prevention. Curr Opin Ophthalmol 2005;16(1):65-
70.
8. Taban M, Behrens A, Newcomb RL, Nobe MY, McDonnell
PJ. Incidence of acute endophthalmitis following
penetrating keratoplasty: A systematic review. Arch
Ophthalmol 2005;123(5):605-9.

SCLERITIS
• Scleritis is a chronic, painful, and potentially blinding
inflammatory disease that is characterized by edema
and cellular infiltration of the sclera and episcleral
tissues
• Scleritis is most common in the 4th to 6th decades of
life (peak incidence in the 5th decade) and is more
common in women
• The spectrum of ocular disease may extend from
trivial self-limiting episodes of inflammation to a
necrotizing process that can be associated with vision
threatening complications such as uveitis, glaucoma,
cataract, keratitis, retinal edema and optic
neuropathy.
CLASSIFICATION
Based on anatomical site of the inflammation, it is classified
as:
• Anterior scleritis
– Non-necrotizing—diffuse or nodular
– Necrotizing—with or without inflammation
(scleromalacia perforans)
• Posterior scleritis
– Nodular
– Diffuse.
Systemic Associations
• Scleritis is commonly associated with systemic
autoimmune disorders, including rheumatoid
arthritis, systemic lupus erythematosus, relapsing
polychondritis, spondyloarthropathies, Wegener
granulomatosis, polyarteritis nodosa, and giant cell
arteritis
• Scleritis may be the initial or only presenting clinical
manifestation of these potentially lethal disorders
• 45 percent of patients with scleritis particularly the
necrotizing type and 30 percent with posterior scleritis
have associated systemic disease.
Signs and Symptoms
• Pain is usually severe, constant, deep, boring, or
pulsating and is the most common symptom for which
patients seek medical assistance. Pain results from
both direct stimulation and stretching of the nerve
endings by the inflammation
Chapter 4 Inflammatory Lesions of the Eye 49
• Tearing or photophobia without mucopurulent
discharge, which is usually mild or moderate, may
occur in about 25 percent of patients with scleritis
• Diffuse tenderness upon palpation with possible
radiation to other parts of the head
• Decreased visual acuity may be caused by extension
of scleritis to the adjacent structures leading to
keratitis, uveitis, glaucoma, cataract, and fundus
abnormalities
• The primary sign is redness.
POSTERIOR SCLERITIS
• It is defined as scleral inflammation primarily arising
posterior to equator and is characterized by flattening
of the posterior aspect of the globe, thickening of the
posterior coats of the eye (choroid and sclera), and
retrobulbar edema
• Eighty-five percent can develop permanent visual
impairment from maculopathy, optic neuropathy or
exudative retinal detachment
• Eyelid edema, proptosis and ophthalmoplegia are
seen in posterior scleritis
• Nodular posterior scleritis is focal or zonal necro-
tizing granulomatous inflammation surrounding a
discrete sequestrum of scleral collagen. This type can
mimic uveal melanoma
• Diffuse posterior scleritis appears as the diffuse
thickening of the uveoscleral coat
• Perforation of the sclera may be seen in necrotizing
scleromalacia.
Causes: Scleritis may be isolated or in association with
several types of disorders, including autoimmune,
infectious, dermatologic, metabolic, or traumatic.
IMAGING
Ultrasonography (A and B scan)—posterior scleritis.
• It is the most helpful test to aid in diagnosing posterior
scleritis, which is characterized by flattening of the
posterior aspect of the globe, thickening of the
posterior coats of the eye (choroid and sclera), and
retrobulbar edema. Thickening of posterior sclera with
fluid in Tenon’s space.
50 Section 2 The Eye

A B

C D

Figs 4.4A to D: A middle-aged woman presented with painful loss of vision in the left eye: (A) Axial T1-weighted image, (B) Axial
T2-weighted image and (C and D) Coronal T2-weighted image; showing thickening of the ocular coats posterior to the equator
with ill-defined T2-hypointense soft tissue in the Tenon’s space and surrounding the optic nerve head. Thickening of the lateral
rectus muscle is also noted. Diffuse posterior scleritis with perineuritis
 Chapter 4 Inflammatory Lesions of the Eye 51

A B

Figs 4.5A and B: (A) Axial T2-weighted image at the level of the optic nerve showing thickening of the posterior sclera and uvea
temporal to the right optic nerve. The right optic nerve is also thickened and (B) Postcontrast T1-fat suppressed image showing
bilateral thickened and enhancing posterior sclera in the same patient at a different level—posterior scleritis

Fig. 4.6: Axial postcontrast T1-fat suppressed image of the orbit Fig. 4.7: Axial CT scan of the orbit showing bilateral thickening
showing thickened posterior sclera and enhancing soft tissue of posterior ocular coats with soft tissue in the Tenon’s space
in the Tenon’s space temporal to the right optic nerve and thickening of the left optic nerve sheath complex with
perineural inflammation
52 Section 2 The Eye
Computed Tomography
• CT scan shows thickening of the uveoscleral coat
which enhances on postcontrast scan
• Ill-defined soft tissue thickening may be seen in the
Tenon’s space
• It is a cost-effective diagnostic tool to aid in detecting
and differentiating posterior scleritis from orbital
inflammatory diseases and orbital neoplasm.
Magnetic Resonance Imaging
• Diffuse posterior scleritis presents as diffuse
thickening of the sclera, mainly located at the posterior
pole with an isointense to slightly hyperintense signal
with respect to vitreous on T1-weighted images and
hypointense on T2-weighted images
• Moderate-to-marked contrast enhancement related to
the degree of inflammation noted
• Associated orbital features such as perineuritis and
orbital inflammation are better appreciated on MRI
• Nodular posterior scleritis presents as an elevated
mass lesion slightly hyperintense on T1-weighted
images and hypointense on T2-weighted images with
respect to vitreous
• After administration of Gd-DTPA, nodular posterior
scleritis shows minimal enhancement. This feature
helps to differentiate it from uveal melanoma which
demonstrates moderate-to-marked enhancement
• MRI will help differentiate posterior scleritis from orbital
tumors, presenting with choroidal folds and retinal striae
which may also be seen in posterior scleritis.
BIBLIOGRAPHY
1. Benson WE. Posterior scleritis. Surv Ophthalmol
1988;32(5):297-316.
2. Biswas J, Mittal S, Ganesh SK, Shetty NS, Gopal L.
Posterior scleritis: Clinical profile and imaging
characteristics. Indian J Ophthalmol 1998;46(4):195-202.
3. Chaques VJ, Lam S, Tessler HH, Mafee MF. Computed
tomography and magnetic resonance imaging in the
diagnosis of posterior scleritis. Ann Ophthalmol
1993;25(3):89-94.
4. Osman Saatci A, Saatci I, Kocak N, Durak I. Magnetic
resonance imaging characteristics of posterior scleritis
mimicking choroidal mass. Eur J Radiol 2001;39(2):88-91.

OCULAR AND ORBITAL CYSTICERCOSIS
• Cysticercosis is a disease caused by the infestation of
the larval form of the parasitic tapeworm Taenia solium
• The eye, like nervous system and muscle tissue, is a
prime location for parasitic development because of
its rich vascularization
• Intraorbital cysticercosis accounts for 75 to 85 percent.
Depending on their size and location, orbital
cysticercosis may be associated with chemosis, ocular
pain, proptosis, periorbital swelling, ptosis, double
vision, and ophthalmoplegia
• Intravitreal involvement is the most common followed
by subretinal involvement. It can be seen as floating
cysticercus in the vitreous even on fundus
examination or by ultrasound
• Involvement of the palpebral conjunctiva, and
lacrimal glands is observed in rare cases. Diagnosis
of which can be facilitated by ultrasonography and
CT scan.
ULTRASOUND
The diagnosis of ocular cysticercosis is usually made on
ultrasound. It is seen as a ring like lesion with a central/
marginal echogenic nodule representing the scolex.
CT Scan
• It may be difficult to identify ocular cysticercosis even
on contrast-enhanced CT scan. At times, a dense
nodular intraocular lesion may be seen. Contrast
CT scan of the brain should be done to look for
neurocysticercosis
• The cyst wall may not be seen due to very low
attenuation difference between the fluid in the cyst,
vitreous and wall of cysticercosis
• Orbital cysticercosis is seen as ring enhancing lesion
with pin-head area of increased attenuation
representing the scolex
• In the orbit, focal thickening of the involved extra-
ocular muscle is seen due to associated inflammatory
reaction
Chapter 4 Inflammatory Lesions of the Eye 53
• Pathognomonic appearance of the thickened muscle,
the cyst and the scolex inside should lead to the
diagnosis of cysticercosis
• At times, multiple cysts may be seen within the muscle
with irregular margins and scolex may be identified.
MRI Imaging
• High resolution surface coil MR may be useful for
ocular cysticercosis in demonstrating the cyst wall
• Cysts located at the apex presenting as optic neuritis
or orbital apex syndrome are better evaluated by MRI
• Brain imaging should be done to look for concurrent
neurocysticercosis.
BIBLIOGRAPHY
1. Atul K, Kumar TH, Mallika G, Sandip M. Sociodemo-
graphic trends in ocular cysticercosis. Acta Ophthalmol
Scand 1995;73(5):438-41.
2. Chung GW, Lai WW, Thulborn KR, Menner C, Blair NP,
Pulido JS. Magnetic resonance imaging in the diagnosis of
subretinal cysticercosis. Am J Ophthalmol 2002;134(6):
931-2.
3. Mohan K, Saroha V, Sharma A, Pandav S, Singh U.
Extraocular muscle cysticercosis: Clinical presentations
and outcome of treatment. J Pediatr Ophthalmol
Strabismus 2005;42(1):28-33.
4. Pushker N, Bajaj MS, Betharia SM. Orbital and adnexal
cysticercosis. Clin Experiment Ophthalmol 2002;30(5):322-
33. Review.
5. Sekhar GC, Honavar SG. Myocysticercosis: Experience with
imaging and therapy. Ophthalmol 1999;106(12):2336-40.
6. Sharma T, Sinha S, Shah N, Gopal L, Shanmugam MP,
Bhende P, Bhende M, Shetty NS, Agrawal R, Deshpande
D, Biswas J, Sukumar B. Intraocular cysticercosis: Clinical
characteristics and visual outcome after vitreoretinal
surgery. Ophthalmol 2003;110(5):996-1004.
7. Sundaram PM, Jayakumar N, Noronha V. Extraocular
muscle cysticercosis: A clinical challenge to the ophthalmo-
logists. Orbit 2004;23(4):255-62.
8. Ursekar MA, Dastur DK, Manghani DK, Ursekar AT. Isolated
cysticercal infestation of extraocular muscles: CT and MR
findings. Am J Neuroradiol 1998;19(1):109-13.
54 Section 2 The Eye

A B C

Figs 4.8A to C: (A) Axial T1-weighted images precontrast, (B) Postcontrast and (C) Axial T2-weighted image using surface coil
technique showing a small nodule posterior to the ciliary body displaying intermediate signal in T1-weighted images and hypointense
in T2-weighted images with homogeneous contrast enhancement (arrow)—clinically proven cysticercosis

A B

Figs 4.9A and B: (A) Axial and (B) Coronal postcontrast CT scan of the orbit showing multiple nonenhancing cysts within the
superior oblique muscle (dotted arrow). Scolex is identified within one of the cyst (black arrow). Note: The grossly thickened
superior oblique muscle
 Chapter 4 Inflammatory Lesions of the Eye 55

A B

Figs 4.10A and B: A 32-year-old male with history of left lateral rectus palsy and blurring of vision. (A) Axial and (B) Sagittal
postcontrast fat-suppressed T1-weighted image of the orbit showing a well-defined cyst and scolex at the left apex compressing
the optic nerve (arrow)

A B

Figs 4.11A and B: (A) Axial and (B) Coronal postcontrast CT scan of the orbit showing a well-defined cyst with scolex in the left
superior oblique muscle (arrow)
56 Section 2 The Eye

A B

C D

Figs 4.12A to D: A 32-year-old female presented with recurrent painful loss of vision in the left eye: (A) Axial T1-weighted image,
(B) Coronal T2-weighted image, respectively are pretreatment images showing a well-defined cyst at the left orbital apex
compressing the optic nerve. No scolex is seen. Post-treatment scan: (C) Axial T2 and (D) Coronal T2 taken a month later
showing complete resolution of the cyst and residual thickening of the lateral rectus
 Chapter 4 Inflammatory Lesions of the Eye 57

A B C

Figs 4.13A to C: Cysticercosis involving the extraocular muscles. Coronal CT scans of the orbit showing well-defined single cyst
with scolex within: (A) Right inferior rectus, (B) Left superior rectus and (C) Left medial rectus

Fig. 4.14: Axial CT scan orbit showing cyst with scolex in the
nasal conjunctiva of the right eye and thickening of adjacent
medial rectus
58 Section 2 The Eye
VOGT-KOYANAGI-HARADA SYNDROME
• Vogt-Koyanagi-Harada (VKH) syndrome is a rare
systemic disease involving various melanocyte-
containing organs
• Bilateral uveitis associated with cutaneous,
neurologic, and auditory abnormalities characterize
this syndrome
• The entity encompasses the syndrome of iridocyclitis
associated with poliosis, vitiligo and dysacusis.
Initially, described by Vogt in 1906 and by Koyanagi
in 1929 a syndrome of posterior uveitis, papillitis and
serous retinal detachment and cerebrospinal fluid
pleocytosis described by Harada in 1926.
ETIOLOGY
• Clinical and experimental data support an immuno-
logic etiology
• An autoimmune reaction seems to be directed against
an antigenic component shared by uveal, dermal, and
meningeal melanocytes, possibly tyrosinase or a
tyrosinase-related protein
• It occurs more frequently in darkly pigmented races
• Affects both sexes and is more common in women
• Age of onset varies from 10 to 52 years, with mean
age at presentation 30 years
• It can affect children also.
CLINICAL PRESENTATION
Three stages have been described:
• Stage 1 (Prodromal stage): Flu-like symptoms, headache,
meningism and dysacusis
• Stage 2 (Ophthalmic stage): Granulomatous iridocyclitis,
vitritis, optic disc hyperemia, serous retinal
detachment (RD). Patients present with pain,
photophobia and decreased vision
• Stage 3 (Convalescent stage): Uveitis subsides. Derma-
tological signs may appear at this stage—alopecia,
vitiligo, poliosis. The ocular fundus also shows
depigmentation leading to a typical ‘sunset’
appearance.
IMAGING
Ultrasound Findings
• Low-to-medium reflective choroidal thickening that
is most marked in the peripapillary area and tapers
towards periphery.
• Serous retinal detachment
• Scleral thickening
• Mild-to-moderate vitreous opacities.
MR Imaging
• Unilateral or bilateral diffuse thickening of the
posterior uveal tract
• Exudative retinal detachment
• Thickened and inflamed posterior choroid shows a
uniform hyperintense signal with respect to the
vitreous and the overlying exudative retinal
detachment on T1-weighted images
• The thickened choroid is hypointense with respect to
the vitreous and retinal detachment on T2-weighted
images
• The signal of the subretinal fluid depends on its
proteinaceous content
• On postcontrast scans, the thickened posterior choroid
will enhance with no enhancement of the subretinal
fluid.
BIBLIOGRAPHY
1. Beniz J, Forster DJ, Lean JS, et al. Variations in clinical
features of the Vogt-Koyanagi-Harada syndrome. Retina
1991;11(3):275-80.
2. Damico FM, Kiss S, Young LH. Vogt-Koyanagi-Harada
disease. Semin Ophthalmol 2005;20(3):183-90. Review.
3. Mondkar SV, Biswas J, Ganesh SK. Analysis of 87 cases
with Vogt-Koyanagi-Harada disease. Jpn J Ophthalmol
2000;44(3):296-301.
4. Moorthy RS, Inomata H, Rao NA. Vogt-Koyanagi-Harada
syndrome. Surv Ophthalmol 1995;39(4):265-92. Review.
5. Rajendram R, Evans M, Rao NA. Vogt-Koyanagi-Harada
disease. Int Ophthalmol Clin 2005;45(2):115-34.
6. Read RW, Rao NA, Cunningham ET. Vogt-Koyanagi-
Harada disease. Curr Opin Ophthalmol 2000;11(6):437-
42.
7. Read RW. Vogt-Koyanagi-Harada disease. Ophthalmol
Clin North Am 2002;15(3):333-41.
8. Snyder DA, Tessler HH. Vogt-Koyanagi-Harada
syndrome. Am J Ophthalmol 1980;90(1):69-75.
9. Vaphiades MS, Read RW. Magnetic resonance imaging
of choroidal inflammation in Vogt-Koyanagi-Harada
disease. J Neuro-ophthalmol 2004;24(4):295-6.
 Chapter 4 Inflammatory Lesions of the Eye 59

A B

C D

Figs 4.15A to D: (A) Axial T1-weighted image, (B) Axial T2-weighted image and (C and D) Axial FLAIR images showing bilateral
thickened posterior uvea (small arrow) and hyperintense subretinal fluid with respect to the vitreous (thick arrow)
60 Section 2 The Eye
GRANULOMA
• A granuloma is a nodule, usually of less than 2 mm
in diameter, composed of macrophages, epithelioid
cells, lymphocytes and sometimes multinucleated
giant cells
• Granulomatous inflammation is usually seen in
sarcoidosis and tuberculosis
• In tuberculosis, solitary or multiple choroidal
tubercles may be seen.
IMAGING
• MRI with surface coil is the modality of choice
• Lesion larger than 2 mm can be imaged on the high
resolution surface coil MR imaging
• It is seen as a small or medium-sized elevated lesion,
which is minimally hyperintense to the vitreous in
T1- weighted image and hypointense in T2-weighted
image with thickening of adjacent ocular coats and
soft tissue in the Tenon’s space may be noted.
BIBLIOGRAPHY
1. Benchekroun S, el Mansouri Y, Rachid R, el Belhadji M,
Laouissi N, Zaghloul K, Amraoui A. Pseudotumorous
choroid granuloma in miliary tuberculosis. J Fr Ophthalmol
1999;22(7):771-5.
2. Chin PK, Jacobs MB, Hing SJ. Orbital tuberculoma
masquerading as an orbital malignancy. Aust NZJ
Ophthalmol 1997;25(1):67-9.
3. Demirci H, Shields CL, Shields JA, Eagle RC Jr. Ocular
tuberculosis masquerading as ocular tumors. Surv
Ophthalmol 2004;49(1):78-89. Review.
4. Helm CJ, Holland GN. Ocular tuberculosis. Surv
Ophthalmol 1993;38(3):229-56.
 Chapter 4 Inflammatory Lesions of the Eye 61

A B

C D

Figs 4.16A to D: A 12-year-old female with history of pain and redness in the left eye. Surface coil images of the left eye: (A) Sagittal
T2-weighted imaging, (B) Sagittal T1-weighted image, (C) Axial and (D) Sagittal postcontrast fat-suppressed T1-weighted images
showing a dome-shaped chorioretinal lesion. The lesion is slightly heterogeneous in T1-weighted image and hypointense in T2-
weighted image. There is homogeneous contrast enhancement. The ocular coats are thickened and the lesion extends into the
Tenon’s space. Granuloma was confirmed on histopathology
62 Section 2 The Eye

A B

Figs 4.17A to C: A 25-year-old female with history of fever, weight loss and positive chest X-ray for tuberculosis developed
blurring of vision in the right eye: (A) Axial T2-weighted image, (B) Axial T1-weighted image and (C) Axial T1 postcontrast fat-
suppressed surface coil image of the right eye showing a small elevated T2-hypointense lesion at and nasal to the optic disc with
homogeneous contrast enhancement—choriodal tuberculoma
Chapter 5
Intraocular Neoplasms
PEDIATRIC NEOPLASMS
RETINOBLASTOMA
INTRODUCTION
This is the most common intraocular malignant tumor in
children. Retinoblastoma is a type of primitive neuro-
ectodermal tumor arising from immature or primitive
neuroectodermal cells that are destined to become retinal
photoreceptors.
• The incidence of retinoblastoma is estimated to range
from 1 in 15000 to 30000 livebirths
• Bilateral lesions are seen in 25 to 35 percent of cases
• There is no gender or race predilection
• 50 percent cases of childhood leukocoria are caused
by retinoblastoma
• Retinoblastoma may be sporadic or inherited
(autosomal dominant)
• Parent with bilateral retinoblastoma has 45 percent
chance of passing it onto his/her progeny
• In patients with familial retinoblastoma, there is at least
one abnormal retinoblastoma gene, and the patients
are also prone to develop other nonocular malig-
nancies—especially after irradiation. These tumors
include intracranial primitive neuroectodermal tumors
(additional primary retinoblastoma) in the region
of the pineal body or suprasellar cistern and a variety
of sarcomas
• Osteogenic sarcoma is the most common nonocular
neoplasm associated with familial retinoblastoma
• Other nonocular tumors associated with familial
retinoblastoma are carcinomas and soft tissue
sarcomas. The most common sites are head, bones,
skin and brain
• Radiation to the orbit (to treat retinoblastoma) in these
patients has been implicated in activating the
transformation of the tumor cells to sarcomas
• Trilateral retinoblastoma is characterized by
occurrence of ectopic retinoblastoma in the pineal
body or parasellar region. It represents a focus of
multicentric malignancy rather than a second primary
or metastatic disease.
CLINICAL FEATURES
• The most common clinical manifestation of
retinoblastoma includes leukocoria and strabismus
• Pseudouveitis may be the presenting feature in diffuse
infiltrating retinoblastoma
• Signs of orbital inflammation may be seen but does
not always imply extraocular extension
• 90 to 95 percent cases present before 5 years
• Hereditary retinoblastoma present at an earlier age
• Mean age at presentation in bilateral retinoblastoma
is 7 to 16 months
• The average age of presentation for unilateral
retinoblastoma is 24 to 29 months.
64 Section 2 The Eye
A B
C D
A B
Figs 5.1A to D: Axial CT scan images
of different patients demonstrating
different types of calcifications seen
in retinoblastoma: (A) Shows a solitary
calcified lesion temporal to the left
optic disc (arrow), (B) Shows clumps
of calcification within a hyperdense
intraocular mass, (C) Shows bilateral
hyperdense intraocular masses with
multifocal speckled calcification and
(D) Shows hyperdense intraocular
mass filling the vitreous with multiple
calcifications
Figs 5.2A and B: Axial CT scan
of the orbits: (A) Showing bilateral
hyperdense intraocular masses
with calcification and gross thicke-
ning of the left optic nerve up to the
apex—bilateral retinoblastoma with
left optic nerve invasion and
(B) Showing calcified intraocular
mass in the right eye with extra-
ocular extension resulting in
proptosis—retinoblastoma with
extraocular extension

PATHOPHYSIOLOGY
• The most widely accepted concept is that retino-
blastoma arise from multipotential precursor cell
(mutation in the long arm of chromosome 13 band
13q14) that could develop in any inner and outer
retinal cell.
• Intraocular growth patterns are classified as follows:
i. Endophytic Growth
– Endophytic growth occurs when the tumor
breaks through the internal limiting membrane
and appears as a white to cream mass with
either no surface vessels or small irregular
tumor vessels on ophthalmoscopy
– This growth pattern is typically associated with
vitreous seeding. When vitreous seeding is
extensive, the tumor cells may be visible as
spheroid masses floating in the vitreous and
anterior chamber, simulating endophthalmitis
or iridocyclitis, and obscuring the primary
mass
– Secondary deposits or seeding of tumor cells
into other areas of the retina may be confused
with multicentric tumors.
ii. Exophytic Growth
– Exophytic growth occurs in the subretinal
space. This growth pattern often is associated
with subretinal fluid accumulation and retinal
detachment
– The tumor cells may infiltrate through the
Bruch’s membrane into the choroid and then
invade either blood vessels or ciliary nerves or
vessels. Retinal vessels are noted to increase in
caliber and tortuosity as they overlie the mass.
iii. Diffuse Infiltrating Growth
– This is a rare subtype comprising 1.5 percent
of all retinoblastoma
– It is characterized by a relatively flat infiltration
of the retina by tumor cells but without a
discrete tumor mass resulting in thickened
retinal leaflets
– The obvious white mass seen in typical
retinoblastoma rarely occurs
– Calcification is usually absent in these lesions
– It grows slowly as compared to typical
retinoblastoma.
iv. Both combined exophytic and endophytic: These
lesions are more common than pure endo- or
exophytic type.
v. Regressed type: Some may undergo spontaneous
regression with end-stage shrunken phthisical
nonfunctioning globe.
Chapter 5 Intraocular Neoplasms 65
Retinoblastoma has been classified into five groups for
preservation of the eye by International Intraocular
Retinoblastoma Classification (IIRC)
Group 1: Very favorable for maintenance of sight
A. Solitary tumor, smaller than 4 disc diameter
(DD), at or behind the equator
B. Multiple tumors, none larger than 4 DD, all
at or behind the equator
Group 2: Favorable for maintenance of sight
A. Solitary tumor, 4 to 10 DD at or behind the
equator
B. Multiple tumors, 4 to 10 DD behind the
equator
Group 3: Possible for maintenance of sight
A. Any lesion anterior to the equator
B. Solitary tumor, larger than 10 DD behind
the equator
Group 4: Unfavorable for maintenance of sight
A. Multiple tumors, some larger than 10 DD.
B. Any lesion extending anteriorly to the ora
serrata
Group 5: Very unfavorable for maintenance of sight
A. Massive tumors involving more than one
half of the retina
B. Vitreous seeding.
Seeds may be both subretinal and vitreous seeds: 3 mm
diffused seeds.
IMAGING
• The evaluation of a child with leukocoria should be
done systematically. The primary task is to rule in or
to exclude retinoblastoma
• To ensure appropriate therapy, retinoblastoma must
be differentiated from a host of benign simulating
lesions
• The diagnosis must be established early for maximum
ocular salvage
• Though the diagnosis by an ophthalmoscope is often
reliable, imaging is recommended to detect
extraocular extension, optic nerve infiltration,
choroidal infiltration and intracranial metastases or
second tumor. It also helps to differentiate it from
other simulating lesions.
ULTRASONOGRAPHY
• Ultrasonography is useful in distinguishing retino-
blastomas from non-neoplastic conditions
• It is also useful in detecting calcifications with
sensitivity of up to 80 percent
• The tumor appears as a hyperechoic mass within
the globe. Acoustic shadowing due to calcification
66 Section 2 The Eye

A B

Figs 5.3A and B: Status postenucleation of the left eye with ball implant. Axial CT showing the implant displaced medially by a
posterior and lateral peri-implant recurrence (arrows)

Fig. 5.4: Axial CT scan of the orbit showing enucleation status
right eye and a small-elevated calcified lesion at the left posterior
pole
Fig. 5.5: A 5-year-old child underwent enucleation for retino-
blastoma followed by radiotherapy. Had spontaneous extrusion
of the prosthesis, scleral necrosis with mass felt on deep
palpation. Axial CT scan of the orbit showing postenucleation
status right eye with an ill-defined soft tissue in the anterior
orbit and calcification. HPE—radiation necrosis. No tumor cells
seen

is common. Secondary retinal detachment may also
be demonstrable
• Limitations—retrobulbar structures such as optic
nerve invasion is poorly visualized.
CT SCAN
• High-resolution thin section (1-2 mm) CT scan of the
orbit provides a sensitive method for diagnosis and
detecting intraocular calcification
• The calcification may be small and single, large and
single, multiple and punctate or few fine-speckled foci
• Calcification is rarely present in the extraocular com-
ponent
• It is a valuable tool in the detection of extraocular
spread and optic nerve invasion as well as any
intracranial tumor
• The most common site of such intracranial tumors is
the suprasellar and pineal region
• CT scan is not sensitive in noncalcified retinoblastoma.
MAGNETIC RESONANCE IMAGING
• MRI is now routinely done at our institution for
retinoblastoma
Though it is not a sensitive tool to detect intraocular
calcification, MRI is sensitive in detecting an
intraocular tumor greater than 3 mm
• It is useful in noncalcified intraocular mass lesion not
detected on CT scan
• MR examinations should include dedicated orbit and
brain imaging. In cases with subarachnoid deposits,
imaging of the spinal canal should also be done
• Dedicated orbit sequences should include
gadolinium-enhanced imaging with fat suppression
to increase the conspicuity of enhancing tumor. The
use of high-field-strength magnets and surface coils
may improve detection of small lesions, optic nerve
invasion and tumor spread
• On T1-weighted images, the tumors usually have a
low- to-intermediate intensity and are usually difficult
to distinguish from surrounding vitreous
• On T2-weighted images, they demonstrate very low
intensity compared to vitreous. Calcification is more
pronounced on T2 sequences as it is hypointense
against hyperintense vitreous
• Role of diffusion weighted imaging—restricted
diffusion is seen in the tumor depend on viability and
tumor necrosis
• MRI is also useful in identifying any associated
hemorrhagic or exudative retinal detachment, which is
seen as subretinal area of higher signal intensity
compared to vitreous on both T1 and T2-weighted
sequences
Chapter 5 Intraocular Neoplasms 67
• After administration of Gd-DTPA, the tumor shows
moderate to marked heterogeneous enhancement,
depending on the extent of tumor necrosis.
Calcification remains markedly hypointense on Gd-
DTPA enhanced T1W images
• The diffuse infiltrating form poses a diagnostic
challenge as there is no discrete mass lesion, these lack
calcification and appear as diffuse retinal thickening.
The retina may be detached. Enhancement with
intravenous contrast material is typically uniform.
Tiny micronodules may be visualized at US or MR
imaging. These plaque like tumors rarely extend
through the choroid or into the optic nerve
• Although MRI is less sensitive than CT for detection
of calcification, it is extremely useful in differentiation
from the simulating lesions
• MR imaging is useful in non-calcified retinoblastomas,
to detect optic nerve and intracranial spread of the
disease.
BIBLIOGRAPHY
1. Apushkin MA, Shapiro MJ, Mafee MF. Retinoblastoma
and simulating lesions: Role of imaging. Neuroimaging
Clin N Am 2005;15(1):49-67.
2. Arrigg PG, Hedges TR 3rd, Char DH. Computed
tomography in the diagnosis of retinoblastoma. Br J
Ophthalmol 1983;67(9):588-91.
3. Beets-Tan RG, Hendriks MJ, Ramos LM, Tan KE.
Retinoblastoma: CT and MRI. Neuroradiol 1994;36(1):
59-62.
4. Brisse HJ, Lumbroso L, Freneaux PC, Validire P, Doz FP,
Quintana EJ, Berges O, Desjardins LC, Neuenschwander
SG. Sonographic, CT and MR imaging findings in diffuse
infiltrative retinoblastoma: Report of two cases with
histologic comparison. AJNR 2001;22(3):499-504.
5. Char DH, Hedges TR 3rd, Norman D. Retinoblastoma:
CT diagnosis. Ophthalmol 1984;91(11):1347-50.
6. DeGraaf P, Barkhof F, Moll AC, Imhof SM, Knol DL, van
der Valk P, Castelijns JA. Retinoblastoma: MR imaging
parameters in detection of tumor extent. Radiol
2005;235(1):197-207.Epub 2005 Feb. 4.
7. Duncan JL, Scott IU, Murray TG, Gombos DS, van Quill
K, O’Brien JM. Routine neuroimaging in retinoblastoma
for the detection of intracranial tumors. Arch Ophthalmol
2001;119(3):450-2.
8. Jacquemin C, Karcioglu ZA. Detection of optic nerve
involvement in retinoblastoma with enhanced computed
tomography. Eye 1998;12(Pt 2):179-83.
9. Kaufman LM, Mafee MF, Song CD. Retinoblastoma and
simulating lesions: Role of CT, MR imaging and use of
Gd-DTPA contrast enhancement. Radiol Clin North Am
1998;36(6):1101-17.
68 Section 2 The Eye

A B

Figs 5.6A and B: (A) Axial postcontrast T1-weighted showing an enhancing dome-shaped elevated chorioretinal lesion in the left
eye and (B) Coronal T2-weighted image showing the lesion to be hypointense in T2-weighted image

A B

Figs 5.7A and B: (A) Axial T1-weighted of the orbit and (B) Axial T2-weighted image showing an intraocular mass lesion slightly
hyperintense with respect to vitreous in T1-weighted image and axial T2-weighted image showing the lesion to be hypointense
calcification is seen as low signal intensity with the lesion (arrows)
 Chapter 5 Intraocular Neoplasms 69

A B

Figs 5.8A and B: (A) Axial CT scan of the orbit showing a hyperdense intraocular mass in the temporal quadrant with multifocal
calcification and shallow retinal detachment (arrow). (B) Corresponding axial T2-weighted image of the orbit showing a T2 hypointense
mass in the temporal quadrant (arrow) corresponding to the calcified mass on CT. Note: The associated retinal detachment

A B

Figs 5.9A and B: (A) Postcontrast axial T1-weighted image of the brain at the level of the suprasellar cistern in a retinoblastoma
patient showing enhancement in the sulcal spaces (arrows)—suggestive of subarachnoid depositis and (B) Plain axial CT scan of
the same patient done prior to the MRI showing effacement of the right sylvian fissure and basifrontal sulcal spaces (arrow)
70 Section 2 The Eye

Fig. 5.10: Axial T2-weighted image showing bilateral retino-

blastoma with extraocular extension and optic nerve invasion

A B

Figs 5.11A and B: (A) Axial T2-weighted image and (B) Coronal T2-weighted image showing bilateral retinoblastoma (L) left
extraocular extension and right optic nerve invasion (arrowhead). Note the large left extraorbital lesion (long arrow) and intracranial
disease (block arrow in Fig. 5.11A)

10. Mullaney PB, Karcioglu ZA, al-Mesfer S, Abboud EB.
Presentation of retinoblastoma as phthisis bulbi. Eye
1997;11(Pt 3):403-8.
11. Potter PD, Shields CL, Shields JA, Flanders AE. The role
of magnetic resonance imaging in children with
intraocular tumors and simulating lesions. Ophthalmol
1996;103(11):1774-83.
12. Provenzale JM, Gururangan S, Klintworth G. Trilateral
retinoblastoma: Clinical and radiologic progression. AJR
2004;183(2):505-11.
13. Schueler AO, Hosten N, Bechrakis NE, Lemke AJ, Foerster
P, Felix R, Foerster MH, Bornfeld N. High resolution
Chapter 5 Intraocular Neoplasms 71
magnetic resonance imaging of retinoblastoma. Br J
Ophthalmol. 2003;87(3):330-5.
14. Schulman JA, Peyman GA, Mafee MF, Lawrence L,
Bauman AE, Goldman A, Kurwa B. The use of magnetic
resonance imaging in the evaluation of retinoblastoma.
J Pediatr Ophthalmol Strabismus 1986;23(3):144-7.
15. Shields CL, Shields JA, Wagner R. Retinoblastoma.
J Pediatr Ophthalmol Strabismus 2003;40(1):6-9.
16. Tateishi U, Hasegawa T, Miyakawa K, Sumi M, Moriyama
N. CT and MRI features of recurrent tumors and second
primary neoplasms in pediatric patients with
retinoblastoma. AJR 2003;181(3):879-84.
72 Section 2 The Eye
MEDULLOEPITHELIOMA
It is a tumor of the nonpigmented ciliary epithelium
derived from neuroectoderm and appears to be second
most common intraocular tumor in the children after
retinoblastoma.
CLINICAL FEATURES
• Average age of presentation is usually 2 to 4 years
• It produces a peculiar notch in the lens
• When large enough it can present as a mass and can
produce cataract, iris neovascularization or glaucoma.
Medulloepithelioma may be classified as:
1. Non-teratoid: Which is a pure proliferation of cells of
the medullary epithelium.
2. Teratoid: Teratoid type will also show presence of
heteroplastic elements, such as cartilage, skeletal
muscle and brain tissue.
Both types can present with benign and malignant
features. Distant metastasis is uncommon.
RADIOLOGICAL FEATURES
• MRI orbit with surface coil is the preferred investigation
as the lesions are located in the ciliary body
• On precontrast scans, medulloepithelioma of
the ciliary body shows isointense to hyperintense
signal on T1W images and an isointense or
hypointense signal on T2W images with respect to
the vitreous
• Cystic areas may be also noted
• After Gd-DTPA administration, the tumor shows
moderate-to-marked enhancement.
BIBLIOGRAPHY
1. Al-Torbak A, Abboud EB, al-Sharif A, el-Okda MO.
Medulloepithelioma of the ciliary body. Indian J
Ophthalmol 2002;50(2):138-40.
2. Font RL, Rishi K. Diffuse retinal involvement in
malignant nonteratoid medulloepithelioma of ciliary
body in an adult. Arch Ophthalmol 2005;123(8):
1136-8.
3. Gopal L, Babu EK, Gupta S, Krishnakumar S, Biswas J,
Rao NA. Pigmented malignant medulloepithelioma of the
ciliary body. J Pediatr Ophthalmol Strabismus 2004;
41(6):364-6.
 Chapter 5 Intraocular Neoplasms 73

Figs 5.12A and B: Axial non-

contrast CT scan of the orbit in a
2-year-old child showing an ill-
defined hyperdense mass lesion
superolateral to the lens (dotted
arrow) extending into the anterior
chamber and vitreous cavity—
A B medulloepithelioma

A B

Figs 5.13A to D: A 3-year-old child

presented with redness, watering
and white lesion in the eye: (A)
Sagittal and (B) Coronal T2-
weighted surface coil images,
showing ill-defined ciliary body
lesion superior to the lens; display-
ing mixed hypo- and hyperintense
signal. Contrast-enhanced: (C) Axial
and (D) Coronal T1-weighted
FSPGR fat suppressed images
showing moderate enhancement in
the lesion (arrows)—medullo-
C D epithelioma
74 Section 2 The Eye
ADULT NEOPLASMS
MELANOMA
Malignant melanoma of the ciliary body and choroid
(posterior uvea) is the most common primary intraocular
malignant tumor in adults.
CLINICAL FEATURES
• It is unusual in blacks. White: Black ratio is about 15:1
• Most malignant tumors are detected in middle aged
or elderly patients
• It metastasizes hematogenously, the most common
site being liver
• On gross examination, uveal melanomas can be
mushroom shaped, oval, placoid or diffuse dark
brown lesions.
RADIOLOGICAL FEATURES
Uveal Melanomas
A-scan Ultrasound
• It is a very useful tool in tumors more than 2-3 mm
thickness
• Choroidal melanoma characteristically shows an
initial prominent spike, followed by low-to-medium
internal reflectivity with diminishing amplitude
• Vascular pulsations can be seen as fine oscillations of
the internal spiking pattern within the tumor
• Standardized ultrasonography has a diagnostic
accuracy of more than 95 percent. Performing
sequential A-scans, with accurate dimension
measurements, in cases of diagnostic uncertainty is
important
• It is very helpful in distinguishing ciliary body
melanomas from ciliary body cysts.
B-scan Ultrasound
• It is a routine test used in the evaluation of any
posterior segment mass
• It is especially useful in patients with media opacity
• For choroidal melanoma, B-scan is used to establish
the diagnosis, to evaluate possible extraocular
extension, and to estimate tumor size for periodic
observation and plan therapeutic intervention
• The ciliary body is a difficult ocular region to evaluate
with B-scan. Immersion technique and comparison
with the contralateral normal eye often are needed to
detect a small mass in the ciliary body
• The newer innovation of ultrasound biomicroscopy
is useful in evaluation anterior lesions including
ciliary body area
Intraocular melanomas have several distinctive features
that include the following:
• Initial high spike followed by low to medium
reflectivity of the lesion. Due to rapid attenuation of
the ultrasound, there is a rapid reduction in the lesion
spikes that leads to acute angle kappa (.)
• Choroidal excavation
• Shadowing of subjacent soft tissues
• Internal vascularity
• An acoustic quiet zone at the base of the tumor called
acoustic hollowing.
Ultrasound Biomicroscopy
It can differentiate very anterior choroidal melanomas
from those of ciliary body origin and helps define the
anterior border of the tumor.
Fluorescein angiography and indocyanine green
angiography do not show pathognomonic signs of
choroidal melanoma but can contribute to the diagnosis:
• Small choroidal melanomas may show fluorescein
angiographic changes similar to some choroidal
nevi—ranging from normal angiography to hypo-
fluorescence secondary to blockage of background
fluorescence
• Larger melanomas may show a patchy pattern of early
hypofluorescence and hyperfluorescence followed by
late intense staining
• Some choroidal melanomas demonstrate intrinsic
vascularization, visible throughout the angiogram
• The angiographic sign called “double circulation” pat-
tern refers to simultaneous fluorescence of retinal and
choroidal circulation within the tumor. When it occurs,
then it is fairly distinctive of choroidal melanomas.
CT scan of the eye is not as sensitive as ultrasound for
intraocular tumors:
a. It is useful to see extraocular extension
b. Uveal melanomas are hyperdense, sharply margi-
nated and show moderate contrast enhancement
 Chapter 5 Intraocular Neoplasms 75

A B

Figs 5.14A and B: (A) Noncontrast axial T1-weighted surface coil image of the left eye showing bilobed hyperintense choroidal
lesion (B) Axial T2-weighted image using head coil shows the hypointense signal in the melanoma

A B C

Figs 5.15A to C: Ciliochoroidal melanoma. Surface coil noncontrast: (A) Axial T1-weighted, (B) Coronal T2-weighted image and
(C) Sagittal T1-weighted images showing a well-defined dome-shaped T1 hyperintense and T2 hypointense lesion extending
from the ciliary body to the equator

Fig. 5.16: Melanoma with extraocular extension: Axial post-

contrast CT of the orbit showing a well-defined hyperdense right
intraocular mass with extraocular extension
76 Section 2 The Eye
c. Contrast-enhanced CT may help to differentiate
between choroidal or retinal detachment and a solid
tumor. Choroidal melanoma shows enhancement
with contrast, whereas exudation does not.
Magnetic Resonance Imaging
• MRI using surface coil imaging and gadolinium is
considered very sensitive in detecting uveal
melanomas that are greater than 3 mm in size
• The MR signal characteristics are thought to be related
to the paramagnetic properties of melanin. They have
a short T1 and T2 values, which is attributed to the
paramagnetic proton relaxation by stable radicals in
melanin
• Uveal melanomas, therefore, are rather unique in their
MR signal characteristics. They appear as moderately
high signal intensity lesions on T1-weighted images
and moderately low signal on T2-weighted images.
At times, they can be hyperintense on T2-weighted
images
• Though the paramagnetic property of melanin plays
an important role in MR signal characteristics of
melanomas, the histologic features of the tumor
undoubtedly contribute to their MR imaging features.
Necrosis and hemorrhage may be seen within large
tumors
• On contrast-enhanced MR, they demonstrate
moderate enhancement. Gadolinum-enhanced MRI is
useful in evaluating extraocular extension and optic
nerve invasion by the tumor and intracranial
metastases.
Differential Diagnosis
• Choroidal hematoma (signal changes in different
stages). Subacute blood also appears hyperintense on
T1-weighted images
• Organized subretinal exudates
• Choroidal detachment
• Choroidal metastases-mucin producing tumors
• Choroidal lymphoma.
IRIS MELANOMA
• Ultrasound biomicroscopy can be extremely helpful
in differentiating solid iris masses from iris cysts
• Ultrasound biomicroscopy is also useful to monitor
the characteristics of these lesions after brachy-
therapy.
BIBLIOGRAPHY
1. Alsbirk KE, Halaburt H. Computerized tomography of
malignant melanomas of the choroid. Acta Ophthalmol
(Copenh) 1983;61(6):1087-98.
2. Heller M, Guthoff R, Hagemann J, Jend HH. CT of
malignant choroidal melanoma: Morphology and
perfusion characteristics. Neuroradiol 1982;23(1):23-30.
3. Mafee MF, Peyman GA, Peace JH, Cohen SB, Mitchell MW.
Magnetic resonance imaging in the evaluation and
differentiation of uveal melanoma. Ophthalmol
1987;94(4):341-8.
4. Peyster RG, Augsburger JJ, Shields JA, Hershey BL, Eagle
R Jr, Haskin ME. Intraocular tumors: Evaluation with MR
imaging. Radiol 1988;168(3):773-9.
5. Peyster RG, Augsburger JJ, Shields JA, Satchell TV,
Markoe AM, Clarke K, Haskin ME. Choroidal melanoma:
Comparison of CT, fundoscopy, and US. Radiol
1985;156(3):675-80.
6. Sallet G, Serop S, Verbraeken H, Hanssens M. The value of
medical imaging techniques in the diagnosis of extraocular
extension of malignant melanoma of the choroid: A case
report. Bull Soc Belge Ophthalmol 1993;248:53-8.
7. Scott IU, Murray TG, Hughes JR. Evaluation of imaging
techniques for detection of extraocular extension of
choroidal melanoma. Arch Ophthalmol 1998;116(7):897-9.
 Chapter 5 Intraocular Neoplasms 77

Figs 5.17A and B: (A) Axial T1-

weighted image and (B) Axial T2-
weighted image of the right eye
showing a dome-shaped choroidal
lesion in the temporal quadrant
displaying hyperintense signal in
T1-weighted image (black arrow)
and hypointense signal in T2-
weighted image. Note the
A B choroidal excavation (white arrow)

A B

Figs 5.18A to C: (A) Axial T1-weighted, (B) Axial T2-

weighted image and (C) Coronal T1-weighted surface
coil images of the left eye showing small irregular
elevated choroidal lesion in the temporal quadrant
extending from the ciliary body upto the optic disc.
There is a large anterior conjunctival and superior
episcleral lesion of similar signal. Small extraocular
lesion is also seen temporal to the optic disc with
scleral discontinuity (arrow). The lesion is
hyperintense in T1-weighted image and hypointense
in T2-weighted image. HPE—Choroidal melanoma
C with extraocular extension
78 Section 2 The Eye

A B C

Figs 5.19A to C: Axial surface coil images: (A) Noncontrast T1-weighted image, (B) Contrast-enhanced fat suppressed T1-
weighted image and (C) Axial T2-weighted showing a dome-shaped choroidal lesion in the nasal quadrant displaying hyperintense
signal in T1 and hypointense signal in T2-weighted with moderate enhancement on postcontrast T1-weighted image (small
dotted arrow). Note the retinal detachment is hyperintense on both T1- and T2-weighted image and does not enhance with
contrast (long dotted arrow)

A B

Figs 5.20A and B: An 11-year-old boy presented with decrease in vision in the right eye following blunt ocular injury. Noncontrast
Axial CT scan of the orbits: (A) Showing hyperdense intraocular lesion in the right globe and (B) With V-shaped anterior margin
suggestive of retinal detachment
 Chapter 5 Intraocular Neoplasms 79

A B

C D

Figs 5.21A to D: MRI images of the same patient. (A) Axial noncontract T1-weighted image showing a large hypointense mass
in the nasal quadrant of the right eye (black arrow) not appreciated on CT scan and it can be seen separately from the hyperintense
retinal detachment, (B) Axial postcontrast T1-weighted image, showing enhancement within the mass against the non-enhancing
retinal detachment, (C) Coronal T2-weighted image and (D) Axial T2-weighted image showing the mass (white arrow) to be
relatively hypointense against the hyperintense retinal detachment and vitreous. HPE—melanoma
80 Section 2 The Eye
MELANOCYTOMA
• It is a deeply pigmented benign tumor that can occur
in the uvea and in the substance of the optic nerve
• Approximately, 50 percent of melanocytomas occur
in black race whereas the incidence of uveal malignant
melanoma is less than 1 percent in black race
• Melanocytoma of the optic disc appears as jet black,
elevated mass with fibrillated margins and is located
eccentrically on the optic disc.
MR IMAGING
• MRI is not helpful in differentiating choroidal
melanoma from melanocytoma
• Because of the melanin content melanocytoma is seen
as a hyperintense signal on T1W images and
hypointense signal on T2W images with respect to
vitreous
• There is minimal enhancement seen after Gd-DTPA
administration.
BIBLIOGRAPHY
1. LoRusso FJ, Boniuk M, Font RL. Melanocytoma
(magnocellular nevus) of the ciliary body: Report of ten
cases and review of the literature. Ophthalmol
2000;107(4):795-800.
2. Reidy JJ, Apple DJ, Steinmetz RL, Craythorn JM, Loftfield
K, Gieser SC, Brady SE. Melanocytoma: Nomenclature,
pathogenesis, natural history and treatment. Surv
Ophthalmol 1985;29(5):319-27.
 Chapter 5 Intraocular Neoplasms 81

A B

Figs 5.22A and B: (A) Axial T1-weighted image and (B) Axial T2-weighted image of the eye using surface coil showing a well-
defined hypointense lesion in the ciliary body in T2-weighted image and hyperintense in T1-weighted image similar to a ciliary
body melanoma. Histopathologically, it proved to be a case of melanocytoma

A B C

Figs 5.23A to C: (A) Axial T1-weighted image, (B) Axial T2-weighted image obtained using head coil showing no demonstrable
abnormality and (C) Axial T2-weighted surface coil image of the left eye using 1.2 mm slice thickness show a small nodular
hypointense lesion at the optic disc—melanocytoma (arrow)
82 Section 2 The Eye

LEIOMYOMA

Uveal leiomyoma is a rare benign tumor of the smooth MR FEATURES

muscle origin which can arise in the iris, ciliary body, or
choroid.
CLINICAL FEATURES
• They tend to occur in younger patients with female
predilection
• On ophthalmoscopy, it appears as yellowish elevated
highly vascularized mass
• Clinically, the tumor may simulate amelanotic
choroidal melanoma on the basis of clinical
appearance.
• Leiomyoma appears as a hyperintense mass with respect
to the vitreous on the nonenhanced T1W images and
hypointense on T2W images (isointense to brain paren-
chyma in T1- and T2-weighted images). The tumor is not
as hyperintense as a melanoma on T1-weighted images
• On Gd-DTPA enhanced T1W images the tumor
showed marked enhancement.
BIBLIOGRAPHY
1. Jellie HG, Gonder JR, Willis NR, Green L, Tokarewicz AC.
Leiomyoma of the iris. Can J Ophthalmol 1989;24(4):169-71.
 Chapter 5 Intraocular Neoplasms 83

A B

C D

Figs 5.24A to D: A 19-year-old female presented with a ciliary body lesion: (A) Axial T1-weighted image showing a well-
circumscribed intermediate signal intensity mass arising from the ciliary body and extending posteriorly up to the equator, (B)
Axial T2-weighted imaging showing the lesion to be predominantly hyperintense, (C) Postcontrast coronal T1-weighted image
showing moderate contrast enhancement and (D) Axial STIR image showing the hyperintense lesion more clearly against the
hypointense vitreous. HPE—leiomyoma
84 Section 2 The Eye
UVEAL METASTASIS
• It is currently believed that uveal metastasis is the
most common intraocular malignant disease
• The malignant cells get deposited in the eye by means
of short posterior ciliary arteries. This explains why
majority of the lesions are in the posterior half of the
eye.
CLINICAL FEATURES
• Clinically, choroidal metastasis is seen as yellow
creamy placoid or dome-shaped lesion associated in
75 percent of cases with secondary shifting retinal
detachment
• With the exception of choroidal metastasis from skin
melanoma, choroidal metastases are amelanotic or
minimally pigmented
• These are usually bilateral and tend to be multifocal
• The common sites to metastasize to the eye are breast,
bronchus, kidney and colon
• These tumors can metastasize to the retina and
choroid.
IMAGING
B-scan Ultrasonography
• Choroidal metastasis exhibits an ovoid or dome-
shaped choroidal mass with bumpy contour and
medium to high acoustic solidity and no appreciable
choroidal excavation
• A shifting retinal detachment may be demonstrated.
CT Scan
It lacks sensitivity and specificity.
MR FEATURES
• Metastatic lesions are slightly elevated, bumpy
lesions as against melanomas which are exuberant
in growth. Metastatic lesions are mottled and
diffuse in outline
• Lesions demonstrate variable signal intensities.
Metastatic lesions are relatively hyperintense with
respect to vitreous on T1W images and relatively
hypointense on T2W images. Thus, the metastatic
tumors share the same MR features as other
pigmented or amelanotic intraocular lesions
• Metastases from colloid carcinoma or mucin-
producing tumors (adenocarcinoma) may simulate
a uveal melanoma as proteinaceous fluid tends to
decrease the T1 and T2 relaxation times of the lesion
• Gd-DTPA enhanced T1W images with fat suppression
techniques are most helpful in detecting small
metastatic choroidal lesions (< 1.8 mm) by expanding
the dynamic range of the image. Gd-DTPA enhanced
MRI evaluation is particularly indicated in order to
image the brain and detect intracranial metastasis.
Postcontrast study with fat suppression for the eye
followed by brain screening should be done in all cases
with intraocular tumors.
BIBLIOGRAPHY
1. Demirci H, Shields CL, Chao AN, Shields JA. Uveal
metastasis from breast cancer in 264 patients. Am J
Ophthalmol 2003;136(2):264-71.
2. Sobottka B, Kreissig I. Ultrasonography of metastases and
melanomas of the choroid. Curr Opin Ophthalmol
1999;10(3):164-7.
3. Sobottka B, Schlote T, Krumpaszky HG, Kreissig I.
Choroidal metastases and choroidal melanomas:
Comparison of ultrasonographic findings. Br J Ophthalmol
1998;82(2):159-61.
4. Lemke AJ, Hosten N, Wiegel T, Prinz RD, Richter M,
Bechrakis NE, Foerster PI, Felix R. Intraocular metastases:
Differential diagnosis from uveal melanomas with high-
resolution MRI using a surface coil. Eur Radiol 2001;11(12):
2593-601.
 Chapter 5 Intraocular Neoplasms 85

A B C

Figs 5.25A to C: (A) Axial precontrast T1-weighted MRI of the eye using surface coil technique showing a slightly hyperintense
bumpy choroidal lesion (arrow). It demonstrates: (B) Moderate enhancement on postgadolinium scan and (C) Is hypointense in
T2-weighted image

Fig. 5.26: A 50-year-old male patient with small cell carcinoma

presented with pain and chemosis of the right eye. Sagittal T1-
weighted image showing an ill-defined intraocular lesion with
extraocular extension extending up to the optic nerve head
(arrow)
86 Section 2 The Eye

A B

Figs 5.27A and B: (A) Axial T2-weighted image and (B) Axial T1-weighted image showing a well-defined dome-shaped lesion in
the temporal quadrant of the right eye. It displays central T1 and T2 hyperintense signal suggestive of hemorrhage within surrounded
by a iso- to hypointense rim. HPE—metastases from adenocarcinoma

A B

Figs 5.28A to D: (A) Axial T1-weighted

image of the right orbit using surface coil
shows a fairly well-defined small elevated
choroidal lesion superior to the optic disc
(white arrow). It demonstrates (B) Moderate
enhancement on postgadolinium scan.
(C) Axial T2-WI and (D) Postcontrast T1-
weighted image using head coil showing a
nodular enhancing lesion in the vermis with
perilesional edema (black arrow). Patient
also had multiple supratentorial lesions.
Intraocular and intracranial metastases (No
C D known primary)
 Chapter 5 Intraocular Neoplasms 87

A B

C D

Figs 5.29A to D: (A) Axial T1-weighted image, (B) Coronal T1-weighted image, (C) Axial T2-weighted image and (D) Coronal T2-
weighted image using surface coil showing a small nodular elevated choroidal lesion in the inferior quadrant demonstrating
T1-hyperintense and T2-hyperintense signal similar to choroidal melanoma. HPE—metastases from adenocarcinoma
88 Section 2 The Eye

A B

C D

Figs 5.30A to D: A known case of medullary Ca of thyroid: (A) Precontrast axial T1-weighted image, (B) Axial T1 postcontrast fat
suppressed image, (C) Coronal T2-weighted image, showing small elevated bumpy enhancing lesion temporal to the left optic
disc (arrow) with associated retinal detachment (block arrow) and (D) Axial postcontrast image of the brain showing multiple
contrast enhancing nodular lesions in the posterior fossa suggestive of intracranial metastases

RETINAL ASTROCYTOMA
• Retinal astrocytoma is a benign yellow-white rare
retinal tumor that occurs in association with tuberous
sclerosis, neurofibromatosis or in isolation
• Early retinal astrocytoma looks exactly like an early
retinoblastoma and may present before any neurologic
or dermatologic manifestations of tuberous sclerosis
appear.
OPHTHALMOSCOPIC FINDINGS
• These tumors may appear in the retina or in the optic
nerve
• They appear as single or multiple nodules elevated
1 or 2 mm above the surface of the retina.
IMAGING
• Tumors are elevated more than 3 mm, can be
demonstrated on CT or MR imaging
• They appear as well-defined elevated hyperdense
lesions at the optic disc and may appear similar to
retinoblastoma
Chapter 5 Intraocular Neoplasms 89
• CT brain may show typical features of tuberous
sclerosis (subependymal nodules, subependymal
giant cell astrocytoma and cortical tubers)
• If the typical features of tuberous sclerosis are absent,
then differentiating from other ocular lesions such as
retinoblastoma may be difficult on imaging.
BIBLIOGRAPHY
1. Arnold AC, Hepler RS, Yee RW, Maggiano J, Eng LF, Foos
RY. Solitary retinal astrocytoma. Surv Ophthalmol
1985;30(3):173-81.
2. Bhende P, Babu K, Kumari P, Krishnakamar S, Biswas J.
Solitary retinal astrocytoma in an infant. J Pediatr
Ophthalmol Strabismus 2004;41(5):305-7.
3. Margo CE, Barletta JP, Staman JA. Giant cell astrocytoma
of the retina in tuberous sclerosis. Retina 1993;13(2):155-9.
4. Ramsay RC, Kinyoun JL, Hill CW, Aturaliya UP, Knobloch
WH. Retinal astrocytoma. Am J Ophthalmol 1979;88(1):32-6.
5. Redinova M, Barakova D, Sach J, Kuchynka P. Intraocular
astrocytoma without phacomatosis. Eur J Ophthalmol
2004;14(4):350-4.
6. Sharma A, Ram J, Gupta A. Solitary retinal astrocytoma.
Acta Ophthalmol (Copenh) 1991;69(1):113-6.
90 Section 2 The Eye

A B

Figs 5.31A and B: Axial CT scan of the orbits showing a hyperdense calcified elevated lesion at the left posterior pole—retinal
astrocytoma

CHOROIDAL HEMANGIOMA
Choroidal hemangioma is a benign congenital vascular
tumor of the choroid. Choroidal hemangiomas are seen
usually in association with Sturge-Weber syndrome, but
can occur as isolated lesions.
Two types of choroidal hemangiomas are seen, viz:
1. A circumscribed or solitary lesion not associated with
other abnormalities
2. Diffuse form associated with Sturge-Weber syndrome
and other facial nevus.
CLINICAL FEATURES
• The solitary form of these tumors can be seen as fairly
well defined, lentiform amelanotic, red-orange mass
mainly located posterior to the equator mainly juxta-
papillary or macular region
• The diffuse form seen in cases of Sturge-Weber
syndrome may involve the choroid, ciliary body, iris
and occasionally other nonuveal tissues
• Diffuse choroidal hemangioma can be unilateral or
bilateral.
IMAGING
B-scan Ultrasonography
The lesion appears as a dome-shaped choroidal mass with
internal acoustic solidity. Characteristically, the initial
spike is high and intralesional spikes are high.
CT Scan Findings
• Hemangioma appears as an well-defined hyperdense
mass that enhances markedly with contrast infusion
and on dynamic contrast-enhanced CT
• Contrast-enhanced CT of the brain should be done to
look for intracranial vascular malformations.
Chapter 5 Intraocular Neoplasms 91
MR Findings
• On nonenhanced T1W images, it appears as low signal
intensity lesion that is isointense or slightly hyper-
intense with respect to the vitreous
• On T2W images, choroidal hemangioma shows an
increased signal, which is isointense to hyperintense
to the vitreous and hence may not be visible on T2-
weighted images against the hyperintense vitreous
• STIR or FLAIR images help to delineate the lesion
from the vitreous
• On Gd-DTPA enhanced T1W images, with fat
suppression the circumscribed choroidal hemangioma
shows marked enhancement. This contrast enhance-
ment improves detection of the tumors that cannot
be seen on nonenhanced T1W and T2W images.
BIBLIOGRAPHY
1. De Potter P, Flanders AE, Shields JA, Shields CL, Gonzales
CF, Rao VM. The role of fat-suppression technique and
gadopentetate dimeglumine in magnetic resonance
imaging evaluation of intraocular tumors and simulating
lesions. Arch Ophthalmol 1994;112(3):340-8.
2. Gulani AC, Morparia H, Bhatti SS, Jehangir RP. Color
Doppler sonography: A new investigative modality for
intraocular space-occupying lesions. Eye 1994;8(Pt 3):307-
10.
3. Mafee MF. Uveal melanoma, choroidal hemangioma, and
simulating lesions: Role of MR imaging. Radiol Clin North
Am 1998;36(6):1083-99.
4. Peyster RG, Augsburger JJ, Shields JA, Hershey BL, Eagle
R Jr, Haskin ME. Intraocular tumors: Evaluation with MR
imaging. Radiol 1988;168(3):773-9.
5. Stroszczynski C, Hosten N, Bornfeld N, Wiegel T, Schueler
A, Foerster P, Lemke AJ, Hoffmann KT, Felix R. Choroidal
hemangioma: MR findings and differentiation from uveal
melanoma. AJNR 1998;19(8):1441-7.
92 Section 2 The Eye

A B C

Figs 5.32A to C: (A) Axial precontrast CT scan of the orbits, (B) Postcontrast axial and (C) Coronal CT images showing an elevated
diffuse hyperdense brilliantly enhancing choroidal lesion in the inferior and temporal quadrant of the right eye (black arrows)—
diffuse choroidal hemangioma

A B C

Figs 5.33A to C: (A) Axial STIR. (B) Axial T1-weighted and (C) Axial T2-weighted image using surface coil showing a small
elevated lesion nasal to the left optic disc (black arrow). It is slightly hyperintense in T1-weighted image with respect to vitreous;
isointense in T2-weighted image and STIR (black arrows)—choroidal hemangioma
 Chapter 5 Intraocular Neoplasms 93

A B

C D

Figs 5.34A to D: (A) Axial T1 precontrast, (B) Postcontrast T1-weighted, (C) Axial STIR and (D) Axial T2-weighted images
showing a small elevated lesion hypointense in T1 (A) (white arrow) and isointense to vitreous on T2-weighted image (D). Marked
enhancement noted on postgadolinium scans (B) (black arrow)
94 Section 2 The Eye

A B

C D

Figs 5.35A to D: (A) Axial T1-weighted image, (B) Coronal precontrast T1-weighted image, (C) Axial T2-weighted image and
(D) Axial postcontrast T1-weighted image showing a well-defined elevated mass lesion slightly hyperintense in T1-weighted
images and isointense to the vitreous on T2-weighted image, showing marked contrast enhancement (D)

CHOROIDAL OSTEOMA
• Choroidal osteomas are benign, juxtapapillary, ossi-
fying tumors, first described by Gass and Williams in
1978. They are choroidal tumors that mandate no
treatment
• They occur predominantly in young white (median
age, 20 years) females (90%)
• They have no predilection for either eye
• Choroidal osteoma may be bilateral in some cases
• The tumor ranges in size from 1-3 mm mostly located
near the optic disc. The tumor has the density of the
bone and is usually round to oval in shape
• Its etiology is unknown. It may be caused by osseous
metaplasia of the retinal pigment epithelium, or it may
represent a kind of choristoma
• The most important complications of the tumor are:
Subretinal neovascularization, subretinal and
intraretinal hemorrhages, serous and hemorrhagic
retinal detachments. Neovascularization can be
successfully treated by laser photocoagulation and
photodynamic therapy.
IMAGING
• While ophthalmoscopy and fluorescein angiography
are usually diagnostic, computed tomography (CT)
is important in establishing the correct diagnosis
• Ultrasonography and computed tomography
demonstrate calcified plaque like peripapillary lesion.
They are usually well-defined hyperdense lesions and
Chapter 5 Intraocular Neoplasms 95
can be incidental findings in CT obtained for other
ocular or orbital problems
• Choroidal osteoma must be differentiated mainly
from amelanotic choroidal melanoma, choroidal
nevus, choroidal hemangioma, choroidal metastasis,
granuloma, organized subretinal hemorrhage,
sclerochoroidal calcification and age-related macular
degeneration
• The exclusion of the malignant melanoma is most
important since radiation and enucleation are
therapeutic possibilities for melanoma and not
indicated in choroidal osteoma.
BIBLIOGRAPHY
1. Avila MP, El-Markabi H, Azzolini C, Jalkh AE, Burns D,
Weiter JJ. Bilateral choroidal osteoma with subretinal
neovascularization. Ann Ophthalmol 1984;16(4):381-5.
2. Bloom PA, Ferris JD, Laidlaw A, Goddard PR.
Appearances of choroidal osteomas with diagnostic
imaging. Br J Radiol 1992;65(778):845-8.
3. Bryan RN, Lewis RA, Miller SL. Choroidal osteoma. AJNR
1983;4(3):491-4.
4. De Potter P, Shields JA, Shields CL, Rao VM. Magnetic
resonance imaging in choroidal osteoma. Retina
1991;11(2):221-3.
5. Kelinske M, Weinstein GW. Bilateral choroidal osteomas.
Am J Ophthalmol 1981;92(5):676-80.
6. Shields CL, Shields JA, Augsburger JJ. Choroidal osteoma.
Surv Ophthalmol 1988;33(1):17-27.
Chapter 6
Leukocoria: Lesions
Simulating Retinoblastoma
LEUKOCORIA

Leukocoria is a white, pink-white or yellow-white pupillary reflex (cat’s eye). Leukocoria is caused by various
intraocular lesions such as:
• Persistent hyperplastic primary vitreous
• Retinopathy of prematurity
• Retinoblastoma
• Coats’ disease
• Retinal detachment
• Toxocariasis
• Retinal dysplasia
• Endophthalmitis
• Tumors other than retinoblastoma
• Congenital retinal folds
 Chapter 6 Leukocoria: Lesions Simulating Retinoblastoma
PERSISTENT HYPERPLASTIC PRIMARY
VITREOUS
• Persistent hyperplastic primary vitreous (PHPV) is a
congenital condition with persistent hyaloid
vasculature and mesenchymal tissue from the
embryonic primitive vitreous in a microphthalmic eye
• It is unilateral in 90 percent and bilateral in 10 percent
odd cases
• It is caused by the arrest of normal transformation
and normal regression of the embryonic vascular
connective tissue within the globe
• This disorder is complicated by anterior and posterior
segment anomalies and is clinically characterized by
leukocoria, shallow anterior chamber, cataracts, angle-
closure glaucoma, elongated ciliary processes,
retrolental fibrosis, microphthalmos and retinal
detachment in various combinations
• Imaging is helpful to exclude retinoblastoma in cases
with difficult or no visualization of the posterior
segment
• PHPV is usually detected at birth or in the neonatal
period
• Bilateral PHPV is associated with other ocular
abnormalities and genetic lesions such as trisomy 13,
Warburg disease and Norrie disease.
RADIOLOGICAL FINDINGS
Computed Tomography
• Unilateral microphthalmia
• Generalized increased attenuation of the vitreous
chamber
• Postcontrast CT shows enhancement of the retrolental
membrane
• CT obtained in decubitus view will show layering of
subretinal/subhyaloid fluid
• Intraocular calcification is absent.
Magnetic Resonance Imaging
MR imaging is superior to CT in the diagnosis of PHPV,
because it is valuable in revealing details of the
intraocular fluid and gravitational effects of the
intravitreal fluid.
MRI Findings
• Anterior type of PHPV includes a shallow or
collapsed anterior chamber, an anterior segment
97
anomaly and a retrolental vascular membrane, which
enhances after contrast enhancement.
• Posterior type consists of microphthalmos; the
hyaloid vessel seen as a tubular structure extending
from the retrolental surface to the detached retina; a
funnel-shaped retinal detachment, with the subretinal
fluid, a retrolental mass and vitreous hemorrhage. The
signal of the subretinal fluid is variable depending
on the blood products but it is usually hyperintense
on all sequences due to the presence of blood
degradation products or proteinaceous fluid.
BIBLIOGRAPHY
1. Castillo M, Wallace DK, Mukherji SK. Persistent
hyperplastic primary vitreous involving the anterior eye.
AJNR 1997;18(8):1526-8.
2. Edward DP, Mafee MF, Garcia-Valenzuela E, Weiss RA.
Coats' disease and persistent hyperplastic primary
vitreous: Role of MR imaging and CT. Radiol Clin North
Am 1998;36(6):1119-31, x. Review.
3. Goldberg MF, Mafee M. Computed tomography for
diagnosis of persistent hyperplastic primary vitreous
(PHPV). Ophthalmol 1983;90(5):442-51.
4. Haddad R, Font RL, Reeser F. Persistent hyperplastic primary
vitreous. A clinicopathologic study of 62 cases and review
of the literature. Surv Ophthalmol 1978;23(2):123-34.
5. Kaste SC, Jenkins JJ 3rd, Meyer D, Fontanesi J, Pratt CB.
Persistent hyperplastic primary vitreous of the eye:
Imaging findings with pathologic correlation. Am J
Roentgenol 1994;162(2):437-40.
6. Kuker W, Ramaekers V. Persistent hyperplastic primary
vitreous: MRI. Neuroradiol 1999;41(7):520-2.
7. Mackeen L, Nischol KK, Lam WC, Levin AV. High-
frequency ultrasonography findings in persistent
hyperplastic primary vitreous. JAAPOS 2000;4(4):217-24.
8. Mafee MF, Goldberg MF, Valvassori GE, Capek V.
Computed tomography in the evaluation of patients with
persistent hyperplastic primary vitreous (PHPV). Radiol
1982;145(3):713-7.
9. Mafee MF, Goldberg MF. Persistent hyperplastic primary
vitreous (PHPV): Role of computed tomography and
magnetic resonance. Radiol Clin North Am 1987;25(4):683-
92.
10. Smirniotopoulos JG, Bargallo N, Mafee MF. Differential
diagnosis of leukocoria: Radiologic-pathologic correlation.
Radiographics 1994;14(5):1059-79; quiz 1081-2.
11. Sun MH, Kao LY, Kuo YH. Persistent hyperplastic primary
vitreous: Magnetic resonance imaging and clinical
findings. Chang Gung Med J 2003;26(4):269-76.
98 Section 2 The Eye

Fig. 6.1: Axial CT scan of the orbit showing bilateral small

globes with hyperdense vitreous in a case of PHPV

Fig. 6.2: Axial CT scan of the orbit showing small right

globe with globular lens shallow anterior chamber and
hyperdense vitreous. A thin membrane seen extending
from the retrolental surface to the detached retina (arrow)
suggestive of PHPV
 Chapter 6 Leukocoria: Lesions Simulating Retinoblastoma 99

Figs 6.3A and B: (A) Axial FIESTA sequence and (B) Axial T1
spin-echo sequence showing a small right globe, retrolental
membrane (dotted arrow) and subretinal layering of fluid (black
B arrow)—persistent hyperplastic primary vitreous
100 Section 2 The Eye
RETINOPATHY OF PREMATURITY
• Retinopathy of prematurity (ROP) is a disease that affects
immature vasculature in the eyes of premature babies.
As smaller and younger babies are surviving, the
incidence of ROP has increased. With good screening
protocols, the disease can be detected at an early stage
• It is important to differentiate stage 5 ROP from other
causes of leukocoria
• Sex: Equal distribution
• Age: ROP is a disease of premature infants. All babies
less than 1500 gm birth weight or those born earlier
than 32 weeks’ gestational age at birth, are at risk of
developing ROP. Other possible risk factors include
respiratory distress syndrome, hyperoxic fluctuations
in oxygen saturation, intraventricular hemorrhage,
hypoglycemia, etc.
• Although it mostly starts bilaterally, the regression
can be different between the two eyes leading to
asymmetry.
STAGES
• Stage of immature retina without ROP: Only avascular
retina is made out but there is no clear demarcation
between vascular and avascular retina
• Stage 1: A clear demarcation line develops between
the vascular and avascular region
• Stage 2: The line acquires three dimensionality. A
broad, thick ridge clearly separates the vascular
from the avascular retina
• Stage 3: The extraretinal fibrovascular proliferation
(neovascularization) may be present on the ridge, on
the posterior surface of the ridge or anteriorly toward
the vitreous cavity. The neovascularization gives the
ridge a velvety appearance, a ragged border
• Stage 4: A subtotal retinal detachment beginning at
the ridge is seen. The retina is pulled anteriorly into
the vitreous by the fibrovascular ridge
– Stage 4A does not involve the fovea
– Stage 4B involves the fovea.
• Stage 5: A total retinal detachment is seen. The shape
of the funnel could be different based on whether
it is open or closed anteriorly and posteriorly
• Posterior plus disease is defined as dilation and
tortuosity of the peripheral retinal vessels, while
anterior plus disease is characterized by iris vascular
engorgement and pupillary rigidity. The presence of
plus disease very often dictates the need for treatment
• At the stage of retrolental fibroplasia (stage 5), the
differential diagnosis includes other causes of
leukocoria in a child such as retinoblastoma, persistent
hyperplastic primary vitreous (PHPV), endoph-
thalmitis, Coats’ disease, etc.
IMAGING
• Differentiating ROP from retinoblastoma, PHPV,
endophthalmitis and other conditions wherein retinal
detachment is the common feature, may sometimes
be difficult on imaging.
CT findings suggestive of end stage ROP are:
• Bilateral involvement
• Microphthalmic eyes
• Increased density in the vitreous
• Absent calcification (very rarely calcification can occur
in advanced stages and in blind eyes of long duration)
• Anterior chamber may be shallow
• There may be a persistent hyaloid system seen in ROP
and the recognition of this on clinical examination and
imaging is of prognostic importance. This feature is
better defined on ultrasonography rather than on CT
imaging
• The primary importance of CT scanning is to exclude
gross calcification and to be able to detect tumor
masses behind the white pupillary reflex.
BIBLIOGRAPHY
1. Haik BG, Saint Louis L, Smith ME, Ellsworth RM,
Abramson DH, Cahill P, Deck M, Coleman DJ. Magnetic
resonance imaging in the evaluation of leukocoria.
Ophthalmol 1985;92(8):1143-52.
2. Katz NN, Margo CE, Dorwart RH. Computed tomography
with histopathologic correlation in children with
leukocoria. J Pediatr Ophthalmol Strabismus 1984;21(2):
50-6.
3. Potter PD, Shields CL, Shields JA, Flanders AE. The role
of magnetic resonance imaging in children with
intraocular tumors and simulating lesions. Ophthalmol
1996;103(11):1774-83.
4. Smirniotopoulos JG, Bargallo N, Mafee MF. Differential
diagnosis of leukocoria: Radiologic-pathologic correlation.
Radiographics 1994;14(5):1059-79.
 Chapter 6 Leukocoria: Lesions Simulating Retinoblastoma 101

Fig. 6.4: Bilateral small globes with hyperdense vitreous and shallow anterior chambers in a case of ROP
102 Section 2 The Eye
COATS’ DISEASE
• Coats’ disease is an idiopathic primary vascular
anomaly of the retina that accounts for 16 percent of
the cases of leukocoria
• It is a developmental retinal vascular anomaly
consisting of leaking telangiectatic and aneurysmal
retinal vessels with associated lipid exudation that
most frequently occurs in one eye of otherwise healthy
juvenile boys
• It is mostly unilateral (up to 80% or more).
CLINICAL PRESENTATION
• It is a congenital condition that is present at birth;
however, the symptoms and signs often develop after
6 to 8 years of age
• The patients present with visual loss, strabismus or a
white pupillary reflex.
SIGNS
• Early cases show large areas of intraretinal and
subretinal yellowish exudation with dilated and
tortuous telangiectatic blood vessels in the retina
• Few cases regress spontaneously but many progress
to massive subretinal exudation
• The submacular exudates can be replaced by dense
scar
• In severe cases, the patient may develop massive
exudative retinal detachment and neovascular
glaucoma
• They can also develop cataract, glaucoma, rubeosis,
uveitis and sometimes phthisis bulbi.
IMAGING
• The ophthalmoscopic and biomicroscopic features of
advanced Coats’ disease may closely resemble an
exophytic retinoblastoma
• It is important to distinguish retinoblastoma from
Coats’ disease
• Coats’ disease is almost always unilateral and seen in
slightly older children than those who have retino-
blastoma.
The CT and MRI findings in Coats’ disease vary
with the stage of disease.
CT Findings
• CT is useful in the later stages of the disease because
it can demonstrate retinal detachment as increased
attenuation in the globe
• It is difficult to differentiate between Coats’ disease
and unilateral noncalcifying retinoblastoma on CT
scan
• Calcification is not a feature of Coats’ disease;
however, in upto 1/5th of all cases of advanced Coats’
disease, a submacular calcified nodule may be noted.
These represent exuberant proliferation and
metaplasia of the retinal pigment epithelium.
MR Findings
• MR imaging is superior to CT in differentiating Coats’
disease from retinoblastoma and other leukocoric eyes
• The subretinal exudates are hyperintense on T1 and
T2WI images. In retinoblastoma, MR charac-
teristically shows a mass that is hypointense on T2WI
and thus, it can be easily differentiated from subretinal
exudates
• The subretinal signal in Coats’ disease may vary since
it contains cholesterol, organized hemorrhage and
fibrosis. Its signal is presumed to be inhomogeneous
in character
• The detached retina may show enhancement following
intravenous injection of gadolinium. This enhancement
is due to intraretinal telangiectasia and microaneurysms
• The subretinal exudates do not enhance and therefore
helps in differentiating Coats’ disease from retino-
blastoma
• MR spectroscopy of the eye shows a tall lipid peak.
BIBLIOGRAPHY
1. Edward DP, Mafee MF, Garcia-Valenzuela E, Weiss RA.
Coats' disease and persistent hyperplastic primary
vitreous. Role of MR imaging and CT. Radiol Clin North
Am 1998;36(6):1119-31.
 Chapter 6 Leukocoria: Lesions Simulating Retinoblastoma 103

Fig. 6.5: Axial CT of the orbits in a 5-year-old boy

with leukocoria showing normal sized left globe with
homogeneous intraocular hyperdensity—no
calcification seen—a case of Coats’ disease

A B

Figs 6.6A to C: (A) Axial T2 FRFSE image showing normal

sized left globe and total retinal detachment with subretinal
layering of fluid, (B) Axial postcontrast T1-weighted image
showing no enhancing mass lesion and diffuse increased signal
in the globe and (C) Single voxel PROBE MR spectroscopy
C showing a prominent lipid peak at 1.35 ppm
104 Section 2 The Eye
2. Eisenberg L, Castillo M, Kwock L, Mukherji SK, Wallace
DK. Proton MR spectroscopy in Coats’ disease. Am J
Neuroradiol 1997;18(4):727-9.
3. Haik BG. Advanced Coats' disease. Trans Am Ophthalmol
Soc 1991;89:371-476.
4. Jones JH, Kroll AJ, Lou PL, Ryan EA. Coats’ disease. Int
Ophthalmol Clin 2001 Fall;41(4):189-98. Review.
5. Lai WW, Edward DP, Weiss RA, Mafee MF, Tso MO.
Magnetic resonance imaging findings in a case of advanced
Coats' disease. Ophthalmic Surg Lasers 1996;27(3):234-8.
6. Shields JA, Shields CL, Honavar SG, Demirci H, Cater J.
Classification and management of Coats' disease:
The 2000 Proctor Lecture. Am J Ophthalmol 2001;
131(5):572-83.
7. Shields JA, Shields CL, Honavar SG, Demirci H. Clinical
variations and complications of Coats' disease in 150 cases:
The 2000 Sanford Gifford Memorial Lecture. Am J
Ophthalmol 2001;131(5):561-71.
8. Shields JA, Shields CL. Differentiation of Coats' disease
and retinoblastoma. J Pediatr Ophthalmol Strabismus
2001;38(5):262-6; quiz 302-3. Review.
9. Smithen LM, Brown GC, Brucker AJ, Yannuzzi LA, Klais
CM, Spaide RF. Coats' disease diagnosed in adulthood.
Ophthalmol 2005;112(6):1072-8.
 Chapter 6 Leukocoria: Lesions Simulating Retinoblastoma
WALKER-WARBURG SYNDROME
• Walker-Warburg syndrome is a rare disorder that is
inherited as an autosomal recessive genetic trait.
Walker-Warburg syndrome is also known as HARD
+/-E syndrome, which is an acronym for Hydro-
cephalus, agyria, retinal dysplasia and in some
cases, encephalocele
• The most consistent features of this disorder are the
lack of normal folds of the brain (lissencephaly),
malformations of the brain (cerebellum), abnormali-
ties of the retina and progressive degeneration and
weakness of the voluntary muscles (congenital
muscular dystrophy)
• Typical ocular features include retinal dysplasia and
nonattachment, persistent hyperplastic primary
vitreous, optic atrophy, microphthalmia, corneal
opacities, congenital cataract, and congenital glaucoma
• The serum creatinine kinase level is elevated
• Imaging: MR is the imaging modality of choice. The
intracranial malformations, viz cerebellar hypo-
plasia, lissencephaly and migrational anomalies are
well seen.
BIBLIOGRAPHY
1. Arahata K, Ishii H, Hayashi YK. Congenital muscular
dystrophies. Curr Opin Neurol 1995;8(5):385-90.
105
2. Barkovich AJ. Neuroimaging manifestations and
classification of congenital muscular dystrophies. Am J
Neuroradiol 1998;19(8):1389-96.
3. Kurlemann G, Schuierer G, Kuchelmeister K, Kleine M,
Weglage J, Palm DG. Lissencephaly syndromes: Clinical
aspects. Childs Nerv Syst 1993;9(7):380.
4. Miller G, Ladda RL, Towfighi J. Cerebro-ocular dysplasia-
muscular dystrophy (Walker-Warburg) syndrome:
Findings in 20-week-old fetus. Acta Neuropathol (Berl)
1991;82(3):234-8.
5. Rhodes RE, Hatten HP Jr, Ellington KS. Walker-Warburg
syndrome. Am J Neuroradiol 1992;13(1):123-6.
6. Sasaki M, Yoshioka K, Yanagisawa T, Nemoto A, Takasago
Y, Nagano T. Lissencephaly with congenital muscular
dystrophy and ocular abnormalities: Cerebro-
oculomuscular syndrome. Childs Nerv Syst 1989;5(1):35-
7.
7. Schuierer G, Kurlemann G, von Lengerke HJ. Neuroi-
imaging in lissencephalies. Childs Nerv Syst 1993;9(7):391-
3.
8. Sener RN. Walker-Warburg syndrome: Diffusion MR
imaging. J Neuroradiol 2005;32(3):213-5.
9. Van der Knaap MS, Smit LM, Barth PG, Catsman-
Berrevoets CE, Brouwer OF, Begeer JH, de Coo IF, Valk J.
Magnetic resonance imaging in classification of congenital
muscular dystrophies with brain abnormalities. Ann
Neurol 1997;42(1):50-9.
106 Section 2 The Eye

A B

Figs 6.7A and B: (A) Axial CT scan of the brain showing nodular heterotopia (dotted arrow) and (B) Axial CT scan of the orbit
showing bilateral detachment and hypoplastic right cerebellar hemisphere (white arrow)
 Chapter 6 Leukocoria: Lesions Simulating Retinoblastoma
AICARDI SYNDROME
It is a very rare X-linked dominant disorder. It is named
after the French neurologist, Dr Jean Dennis Aicardi
who described 8 children with infantile spasms, agenesis
of corpus callosum and ocular abnormalities.
AGE AT PRESENTATION
The average age of presentation is from birth to the early
childhood but is most commonly identified between
3 and 5 months of age.
CLINICAL FEATURES
Ocular Features
• Chorioretinal lacunae (holes): The level of vision depends
upon the extent of lacunae, macular involvement and
the degree of mental impairment
• Congenital disc anomalies include coloboma,
hypoplasia and pigmentation
• Other ocular features include microphthalmia,
persistent pupillary membranes and iris coloboma.
Systemic Features
• They usually present with infantile spasms
• Mental retardation
• Microcephaly
• Abnormal facies, cleft lip and cleft palate
• Vertebral abnormalities.
107
Radiological Features
• MRI is preferred because its resolution is superior to
CT scanning
• MRI will show the partial or complete agenesis of the
corpus callosum
• Plain radiographs for skeletal malformations can help
confirm the diagnosis. The most notable findings
include costovertebral abnormalities, commonly
affecting the thoracic vertebrae.
BIBLIOGRAPHY
1. Aicardi J. Aicardi syndrome. Brain Dev 2005;27(3):164-71.
2. Baierl P, Markl A, Thelen M, Laub MC. MR imaging in
Aicardi syndrome. Am J Neuroradiol 1988;9(4):805-6.
3. Hall-Craggs MA, Harbord MG, Finn JP, Brett E, Kendall
BE. Aicardi syndrome: MR assessment of brain structure
and myelination. Am J Neuroradiol 1990;11(3):532-6.
4. Nielsen KB, Anvret M, Flodmark O, Furuskog P, Bohman-
Valis K. Aicardi syndrome: Early neuroradiological
manifestations and results of DNA studies in one patient.
Am J Med Genet 1991;38(1):65-8.
5. Rosser T. Aicardi syndrome. Arch Neurol
2003;60(10):1471-3.
6. Sutton VR, Hopkins BJ, Eble TN, Gambhir N, Lewis RA,
Van den Veyver IB. Facial and physical features of Aicardi
syndrome: Infants to teenagers. Am J Med Genet A 2005;
15;138(3):254-8.
108 Section 2 The Eye

A B

C D

Figs 6.8A to D: (A) Axial FLAIR, (B) Axial T1-weighted image, (C) Axial FLAIR and (D) Sagittal T1-weighted image showing a
small right eye with retinal detachment (small dotted arrow) with disc coloboma (long dotted arrow) in Figures A and B, respectively.
Axial FLAIR image of the brain and (C) Sagittal T1-weighted image (D) showing complete agenesis of the corpus (block arrow)
callosum with interhemispheric cyst (white arrow), shallow sulci and pachygyria (block white arrow)
Chapter 7
Detachment of Retina,
Choroid and Vitreous
RETINAL AND CHOROIDAL DETACHMENTS
RETINAL DETACHMENT
• Retinal detachment (RD) is the separation of the neuro-
sensory retina from the retinal pigment epithelium
(RPE)
• Depending upon the presence or absence of retinal break,
retinal detachments can be divided into two varieties:
1. Rhegmatogenous RD: This is caused by a retinal
break that permits liquefied vitreous to enter the
subretinal space causing retinal detachment.
2. Nonrhegmatogenous RD is characterized by
absence of a retinal break. There are two subtypes:
• Tractional: The sensory retina is pulled away
from RPE by contracting vitreoretinal
membranes, typically this variety is seen with
complications of diabetic retinopathy and other
vascular disorders such as Eales’ disease, vein
occlusions, etc. The configuration of the
detachment is typically concave towards the
vitreous unless a secondary retinal break forms
converting it into a combined tractional-
rhegmatogenous retinal detachment.
• Exudative (serous): The retinal detachment is
caused by exudation of fluid from inflamed
choroid or retinal pigment epithelium or
secondary to the tumors of the eye. Typically,
the detachment has a convex configuration and
the fluid shifts very quickly with position of
the head due to gravity.
Imaging
• The normal retina is thin and therefore beyond the
limits of resolution of CT and MR scanners
• However, the retina is visible when there is significant
contrast difference between the subretinal fluid and
the vitreous cavity
• Fluid in the subretinal space can be detected on CT
and MR imaging
• The appearance of the retinal detachment varies with
the amount of exudation and organization of the
subretinal exudates
• MR is far superior to CT in detecting subretinal fluid
and the underlying cause of retinal detachment
• On axial imaging, RD has a characteristic V-shaped
configuration with apex at the optic disc
• On coronal MR images, RD is seen as floating
membrane
• The exudative subretinal fluid rich in protein has
higher CT density and MR signal intensity than a
transudate. Hence, the subretinal fluid (transudate)
in cases of rhegmatogenous retinal detachment
produced by ingress of liquefied vitreous into the
subretinal space may be difficult to visualize on CT
or MR imaging
• In patients with exudative retinal detachment, MRI
is useful to detect intraocular tumors as the causative
factor.
110 Section 2 The Eye

A B

C D

Figs 7.1A to D: Retinal detachment: (A) Axial T1-weighted, (B) Axial T2-weighted, (C) Axial FLAIR and (D) Coronal T1-weighted
images of the orbit showing T1 and T2 hyperintense subretinal fluid with V-shaped configuration of the detached retina. Figure (D)
shows the folding of the retina (arrow)
 Chapter 7 Detachment of Retina, Choroid and Vitreous 111

Table 7.1: Change in the signal intensity with the age of the hematoma on T1- and T2-weighted images

Duration of hemorrhage T1-weighted image T2-weighted image

Less than 48 hours Isointense or slightly hypointense to Markedly hypointense to

normal vitreous normal vitreous
Early subacute Hyperintense to normal vitreous Hypointense to normal vitreous
Late subacute Hyperintense to normal vitreous Isointense to normal vitreous

Differential diagnosis: Posterior choroidal detachment—
the leaves of the detached membrane do not usually go
up to the optic disc.
CHOROIDAL DETACHMENT
• The choroid is the vascular layer of the eye and is
loosely adherent to the sclera
• Serous choroidal detachment is caused by collection
of fluid in the suprachoroidal space. Serous choroidal
detachment can occur after intraocular surgery such
as cataract surgery, glaucoma surgery and surgery
for retinal detachment
• Blood in the suprachoroidal space is termed hemorr-
hagic choroidal detachment and is seen typically after
blunt and penetrating trauma to the eye. Hemorrhagic
choroidal detachment is also seen as a rare complication
of intraocular surgery in cases with high myopia, etc.
Imaging
CT scan: Choroidal detachment appears as a dome-
shaped, semilunar or ring like with density depending on
its contents. Hemorrhagic choroidal detachment appears
hyperdense. Its density depends on the stage of
hemorrhage, the density decreases as the hemorrhage ages.
MR imaging: On MR imaging, the choroidal hemato-
mas appear as focal, well-defined lenticular lesions. The
signal intensity of which will depend on the age of blood
(Table 7.1).
In the early subacute stage, choroidal hemorrhage can
be confused with choroidal melanoma:
• A choroidal effusion appears as a ring-like or crescent-
shaped lesion. Effusions resulting from inflammation
display high signal intensity on T1 and T2-weighted
images due to high protein content.
BIBLIOGRAPHY
1. Mafee MF, Peyman GA. Choroidal detachment and ocular
hypotony: CT evaluation. Radiol 1984;153(3):697-703.
2. Mafee MF, Peyman GA. Retinal and choroidal
detachments: Role of magnetic resonance imaging and
computed tomography. Radiol Clin North Am
1987;25(3):487-507.
3. Mafee MF, Linder B, Peyman GA, Langer BG, Choi KH,
Capek V. Choroidal hematoma and effusion: Evaluation
with MR imaging. Radiol 1988;168(3):781-6.
4. Peyman GA, Mafee M, Schulman J. Computed tomo-
graphy in choroidal detachment. Ophthalmol 1984;91(2):
156-62.
112 Section 2 The Eye

A B

Figs 7.2A and B: In an 8-year-old child with bleeding disorder, (A) Axial CT of the orbit and (B) Showing a spontaneous hyperdense
choroidal detachment due to collection of blood in the suprachoroidal space

Fig. 7.3: Axial CT of the orbit showing a dome-shaped hyper- Fig. 7.4: Posterior hyaloid detachment. Axial CT of the orbit
dense lesion in the right globe following trauma suggestive of showing a hyperdense intraocular soft tissue with a sharp
choroidal hematoma anterior margin not converging at the optic disc (arrow)
Chapter 8
Intraocular Calcification
The pathophysiology of calcium deposition in ocular
tissues depends on the specific site.
Intraocular calcifications may be:
• Metastatic
• Dystrophic seen in hypercalcemic states
• Associated with degenerative ocular conditions
(neoplasia, inflammation, congenital dysplasias, and
aging or traumatic degeneration)
Calcifications in neoplasms may be a result of
tumor necrosis. Calcification is also seen in astrocytic
hamartomas and vascular lesions like Sturge-Weber
syndrome and von Hippel-Lindau disease
• Chronic post-traumatic degeneration is probably the
most common cause of dystrophic intraocular
calcification
• Other common causes include choroidal osteoma,
episcleral choristoma, and optic disc drusen
• Scleral calcification may be seen in elderly people at
or anterior to the sites of insertion of the horizontal
extraocular muscles
• The major causes of intraocular calcification are listed
here:
1. Cornea: Calcification of the cornea seen in band-
shaped keratopathy. It indicates the distribution
of calcium in the stroma of cornea anterior to the
basement membrane in the interpalpebral area.
The following conditions can cause band-shaped
keratopathy:
a. Chronic iridocyclitis, especially associated with
juvenile rheumatoid arthritis
b. Absolute glaucoma and phthisis bulbi
c. Chronic corneal disease such as interstitial
keratitis, exposure to toxins and drugs such as
mercury, phosphate containing corticosteroids,
silicone oil in anterior chamber
d. Keratoconjunctivitis sicca and climatic
exposure to desert conditions
e. Abnormal calcium metabolism as in
hyperparathyroidism, chronic renal failure and
vitamin D toxicity
f. Primary familial band keratopathy.
2. Sclera:
a. Idiopathic
b. Senile scleral plaque—well-circumscribed
areas of sclera degeneration located anterior
to insertion of horizontal muscles. The calcium
content in the plaques can be mistaken for
intraocular foreign bodies on radiological
examination
c. Hypercalcemia
d. Rheumatoid arthritis
e. Metastatic calcification
f. Linear sebaceous nevus syndrome
g. Microphthalmia with cyst.
3. Lens: Hypermature cataract
4. Choroid:
a. Trauma: Calcification can result from trauma
if it leads to phthisis bulbi
b. Uveitis: Chronic uveitis
c. Sturge-Weber syndrome
d. Choroidal osteoma.
114 Section 2 The Eye

5. Retina: g. CMV retinitis: Following viral retinal infections

a. Retinoblastoma
b. Drusen
c. Phthisis
d. Retinopathy of prematurity.
e. Coats’ disease: In general, presence of calcifi-
cation in a child with leukocoria would lead
to suspicion of retinoblastoma. Very rarely
Coats’ disease has been shown to have
calcification and could confuse the diag-
nosis
f. Astrocytoma
such as acute retinal necrosis or CMV retinitis,
calcification of the involved area can occur over
a period of time
h. Tuberous sclerosis: It is characterized by
occurrence of astrocytoma in the retina
i. von Hippel-Lindau disease
j. Medulloepithelioma: Arises from nonpigmented
ciliary epithelium.
6. Optic nerve:
a. Drusen
b. Astrocytoma.

EPIBULBAR OSSEOUS CHORISTOMA
A choristoma is a tumor-like growth that contains
displaced epithelial and other dermis like elements not
normally indigenous to the site in which they are found.
Three types are generally recognized as the prototypic
choristomas, namely dermoids, dermolipomas and
complex choristomas.
Epibulbar osseous choristomas are solitary nodules
that resemble conjunctival dermoids. They tend to be
more discrete and have sharper edges. The lesions are
usually located 5 to 10 mm behind the limbus, which is a
feature that distinguishes these lesions from other
conjunctival lesions. The cornea is usually spared but
adherence to bulbar conjunctiva may occur. The size and
shape may vary. They are usually composed of mature
compact bone surrounded by other choristomatous
elements. The lesions are usually stationary.
IMAGING
CT scan orbit is the imaging modality of choice, as the
osseous component will be better appreciated. It will
show an epibulbar hyperdense calcified lesion.
Chapter 8 Intraocular Calcification 115
BIBLIOGRAPHY
1. Cunha RP, Cunha MC, Shields JA. Epibulbar tumors in
children: A survey of 282 biopsies. J Pediatr Ophthalmol
Strabismus 1987;24(5):249-54.
2. Dreizen NG, Schachat AP, Shields JA, Augsburger JJ.
Epibulbar osseous choristoma. J Pediatr Ophthalmol
Strabismus 1983;20(6):247-9.
3. Gayre GS, Proia AD, Dutton JJ. Epibulbar osseous
choristoma: Case report and review of the literature.
Ophthalmic Surg Lasers 2002;33(5):410-5.
4. Gonnering RS, Fuerste FH, Lemke BN, Sonneland PR.
Epibulbar osseous choristomas with scleral involvement.
Ophthal Plast Reconstr Surg 1988;4(1):63-6.
5. Melki TS, Zimmerman LE, Chavis RM, Ellsworth R,
O’Neill JF. A unique epibulbar osseous choristoma.
J Pediatr Ophthalmol Strabismus 1990;27(5):252-4.
116 Section 2 The Eye

Fig. 8.1: Axial CT scan of the orbit showing bilateral sclera Fig. 8.2: A 5-year-old child with poor vision since childhood.
calcification. The left globe is dense due intraocular hemorrhage Axial CT scan of the orbit showing tiny left calcification in the
ocular coats. The brain sections taken showed intracranial
calcification due to congenital TORCH infection

A B

Figs 8.3A and B: Epibulbar osseous choristoma: (A) Axial CT scan of the orbits and (B) Showing extensive epibulbar ossification
bilaterally. Incidentally bilateral temporal arachnoid cysts noted (dotted arrow) in Figure A

PHTHISIS BULBI
• Phthisis bulbi is an end-stage ocular response to severe
ocular insult
• Clinically, the globe will be soft and hypotonus with
minimal or no vision and loss of much of the normal
architecture of the eye
• The globe may attain a quadrilateral shape due to
pressure of the muscles on a soft eye. Histo-
pathologically, the globe is small and shrunken with
marked thickening of the sclera, as well as
disorganization and atrophy of much of the
intraocular contents
• The intraocular contents are markedly disrupted and
difficult to recognize. Commonly, the retinal pigment
epithelium may undergo metaplasia leading to
intraocular ossification or bone formation in the end-
stage.
IMAGING
• Ultrasound: In the early stages of phthisis, the B scan
ultrasonogram typically depicts the shortening of the
ocular axial length and thickening of choroid.
Secondary changes such as retinal detachment,
Chapter 8 Intraocular Calcification 117
choroidal detachment and vitreous traction bands are
discernible
• After severe disorganization has occurred, detection
of individual abnormalities including tumor
differentiation becomes difficult. Vitreous becomes
filled with dense tissue. Secondary calcification occurs
attenuating the sound beam
• CT imaging: A small shrunken globe with or without
intraocular calcification or ossification is seen.
Calcification in phthisis is usually along the coats of
the eye unlike tumor calcification which is in the center
of the eyeball.
BIBLIOGRAPHY
1. Hadjistilianou T, De Francesco S, Marconcini S,
Mastrangelo D, Galluzzi P, Toti P. Phthisis bulbi and
buphthalmos as presenting signs of retinoblastoma: A
report of two cases and literature review. Eur J Ophthalmol
2006;16(3):465-9.
2. Potter PD, Shields CL, Shields JA, Flanders AE. The role
of magnetic resonance imaging in children with
intraocular tumors and simulating lesions. Ophthalmol
1996;103(11):1774-83.
118 Section 2 The Eye

Fig. 8.4: Axial CT scan of the orbits

showing small and shrunken right
globe with hyperdense vitreous and
subtle calcification in the posterior
ocular coats (arrow). Note: the
normal left globe—phthisis bulbi
Chapter 9
EYEBALL INJURIES
Injuries to the eyeball can be due to the following
mechanisms:
• Blunt injury
• Penetrating trauma
• Chemical injury.
These can result in foreign body retention, lens
dislocation, retinal or choroidal hemorrhage, and eyeball
rupture.
Imaging plays an important role as clinical
examination and ultrasound may not be possible in severe
and open globe injuries. Ultrasonography and
computerized tomography have greatly improved the
preoperative assessment of ophthalmic trauma.
The eyeball injuries can result in:
• Globe rupture
• Intraocular hemorrhage
• Injury to the lens.
GLOBE RUPTURE
• Globe rupture represents a major ophthalmologic
emergency and always requires surgical intervention
• The involvement of the posterior segment of the eye
is associated with permanent visual loss
• Globe rupture may occur when a blunt object impacts
the orbit, causing anterior compression of the globe and
raising intraocular pressure to a point that the sclera tears
• Ruptures from blunt trauma usually occur at the sites
where the sclera is thinnest, at the insertions of the
Ocular Trauma
extraocular muscles, at the limbus, and around the
optic nerve
• Sharp objects or those traveling at high velocity may
perforate the globe directly. Small foreign bodies may
penetrate the eye and remain within the globe.
Computed Tomography (CT)
• Ultrasound is contraindicated in open globe injuries.
Hence, CT scaning is the most preferred and sensitive
imaging study to detect occult rupture, associated
optic nerve injury, and small foreign bodies, as well
as to visualize the anatomy of the globe and orbit
• 1 mm thick sections should be obtained in axial plane.
The coronal sections can be direct or reformatted from
the axial sections
• Patients with head injury and those who cannot be
mobilized for direct coronal sections, coronal
reformations should be obtained.
1. Penetrating or severe blunt trauma may cause
laceration of the sclera with escape of the vitreous.
When this occurs there is decrease in the
intraocular pressure, resulting in flattening of
the normal spherical contour of the globe. This
has been referred as pear or flat tire appearance
on CT.
2. In the chronic phase, the post-traumatic eyeball
may appear small, deformed, calcified, and
shrunken (Phthisis bulbi).
120 Section 2 The Eye

A B

Figs 9.1A and B: (A) Axial CT scan of the orbit showing small and regular contour of the left globe following road traffic accident
(RTA) and (B) Coronal CT scan of the orbit showing irregular left globe with intraocular hemorrhage following blunt trauma—
ruptured globe. Note: Fractures of the medial and lateral walls of the maxillary sinus and orbit

A B

Figs 9.2A and B: (A) Axial CT scan of the orbit showing subretinal hyperdense fluid (blood) in the right eye following blunt trauma
to the globe (black arrow) and (B) Axial CT scan of the orbit showing heterogeneous hyperdense soft tissue in the left vitreous
cavity (white arrow) suggestive of vitreous hemorrhage following blunt trauma to the globe. Note: Shallow anterior chamber with
air within suggestive of corneal tear

Magnetic Resonance Imaging (MRI)
• MRI should be performed only after excluding a
ferromagnetic intraorbital foreign body in patients in
whom soft tissue of the orbit namely optic nerve,
extraocular muscles and globe need to be evaluated
and in whom CT is inconclusive
• MRI is excellent in identifying injuries of the soft
tissues of the globe and orbit
• MRI can be particularly helpful in localizing a
nonmetallic foreign body, such as wood, that appears
similar to soft tissue or air on CT scan
• MRI is contraindicated if metallic foreign body is
suspected.
Ultrasonography
• It is relatively contraindicated in open globe injuries
• Ultrasonography is becoming an increasingly
important diagnostic tool in ophthalmology, and it
may be needed to localize the site of rupture and to
rule out intraocular or intraorbital foreign.
INTRAOCULAR HEMORRHAGE
CT Scan
• Blood in the eyeball is seen as hyperdense soft tissue
with HU–40–80 depending of the age of blood
• Vitreous hemorrhage appears as homogeneous or
heterogeneous hyperdense soft tissue in the vitreous
cavity
• Retinal detachment appears as a V-shaped hyper-
density converging at the optic disc
• Choroidal detachment has a biconvex appearance,
terminating distant from the optic nerve insertion. It
is rounded or globular.
TRAUMA TO LENS
• Traumatic dislocation of the lens is seen as abnormal
location of the lens and it may be seen in the vitreous
• If there is doubt, decubitus CT may be helpful in
demonstrating lens mobility
Chapter 9 Ocular Trauma 121
• Post-traumatic cataract is seen as decreased density
of the lens with indistinct margins and at times the
lens can appear globular.
BIBLIOGRAPHY
1. Assaf AA. Traumatic retinal detachment. J Trauma
1985;25(11):1085-9.
2. Benjamin L, Wormald R. CT diagnosis of scleral rupture.
Eye 1987;1(Pt 6):757-61.
3. Bhimani S, Virapongse C, Sarwar M, Twist JF. Computed
tomography in penetrating injury to the eye. Am J
Ophthalmol 1984;97(5):583-6.
4. Doi M, Osawa S, Sasoh M, Uji Y. Retinal pigment epithelial
tear and extensive exudative retinal detachment following
blunt trauma. Graefes Arch Clin Exp Ophthalmol
2000;238(7):621-4.
5. Dolan BJ. Traumatic retinal detachment. Optom Clin
1993;3(2):67-80. Review.
6. Joseph DP, Pieramici DJ, Beauchamp NJ Jr. Computed
tomography in the diagnosis and prognosis of open-globe
injuries. Ophthalmol 2000;107(10):1899-906.
7. Kylstra JA, Lamkin JC, Runyan DK. Clinical predictors of
scleral rupture after blunt ocular trauma. Am J Ophthalmol
1993;115(4):530-5.
8. Olsen TW, Chang TS, Sternberg P Jr. Retinal detachments
associated with blunt trauma. Semin Ophthalmol
1995;10(1):17-27. Review.
9. Seigel RS, Sell J, Magnus DE. CT appearance of traumatic
dislocated lens. Am J Neuroradiol 1988;9(2):390.
10. Sevel D, Krausz H, Ponder T, Centeno R. Value of
computed tomography for the diagnosis of a ruptured eye.
J Comput Assist Tomogr 1983;7(5):870-5.
11. Wang HE, Ger DS, Gould SW. Diagnosis of traumatic lens
dislocations. J Emerg Med 2000;19(1):73-4.
12. Weissman JL, Beatty RL, Hirsch WL, Curtin HD. Enlarged
anterior chamber: CT finding of a ruptured globe. Am J
Neuroradiol 1995;16(4 Suppl):936-8.
13. Werner MS, Dana MR, Viana MA, Shapiro M. Predictors
of occult scleral rupture. Ophthalmol 1994;101(12):
1941-4.
14. Williams DF, Mieler WF, Williams GA. Posterior segment
manifestations of ocular trauma. Retina 1990;10(Suppl 1):
S35-44. Review.
122 Section 2 The Eye

A B

Figs 9.3A and B: (A) Sagittal T1-weighted image of the globe using a surface coil and axial T2-weighted image of the orbit and
(B) Showing dislocated lens (white arrow) in the left globe following blunt trauma to the eye

Fig. 9.4: Axial CT scan of the orbit showing dislocated lens in

the left eye following blunt trauma to the globe

FOREIGN BODIES OF THE EYE AND ORBIT
• Radiological evaluation plays a vital role in the
diagnosis and management of patients with orbital
and intraocular foreign bodies
• The form and composition of the foreign body is
extremely important in determining the best choice
of modality for diagnosis, treatment planning and
management.
Foreign bodies are classified as organic and inorganic:
• Inorganic foreign bodies are subdivided into metallic
and nonmetallic compounds. Metallic foreign bodies
are well tolerated; however, some components such
as copper have been reported to cause purulent
inflammation and iron carries the risk of causing
ocular siderosis.
• Inorganic nonmetallic foreign bodies include glass,
plastic, stone or other minerals. They are usually well
tolerated, unless they are sharp or large or are
contaminated leading to infection.
• Organic foreign bodies include a wide variety of
compounds such as wood, dirt or other vegetable matter.
They are associated with higher incidence of
complications such as anaerobic bacterial or fungal
infection that can develop into suppurative infection,
osteomyelitis, or chronic draining fistula. They can also
lead to intracranial infection—meningitis and brain
abscess. Formation of foreign body granuloma can occur.
IMAGING
• Radiological assessment of patients with suspected
foreign body include plain films, CT, MRI and
ultrasound. These studies confirm the presence and
absence of a foreign body and provide information
with regard to its composition and location.
a. Plain films: Water’s view and lateral view of the
orbit which are sufficient to exclude a metallic
fragment, but are unable to precisely localize a
metallic foreign body relative to the soft tissue of
the orbit.
b. CT scan: Thin 1 to 2 mm helical overlapping
sections are obtained for foreign body
identification and localization. It is able to localize
foreign bodies as small as 0.5 mm.
• The Hounsfield unit (HU) helps to identify the type
of foreign body
• Metallic foreign bodies have an Hounsfield unit
greater than +1000 and produce dense metallic
artifacts
Chapter 9 Ocular Trauma 123
• Glass foreign bodies have a HU ranging from 400 to
800 depending on the type of glass. The average
radiologic attenuation of the different types of glass
was found to be as follows: Green beer bottle, 550 H;
brown beer bottle, 539 H; 40-W fluorescent bulb, 285
H; 100-W incandescent bulb, 260 H; windshield glass,
175 H; window plateglass, 140 H; and spectacle glass,
80 H. The detection of glass foreign bodies depends
on the size, type and location of the glass foreign body.
• CT may also demonstrate associated soft tissue
injuries and intracranial foreign bodies
• A wooden foreign body may be mistaken for air
because air and wood may have similar CT density.
Wood may have a varied appearance on CT, ranging
from hypodense to hyperdense
• Therefore, air that has a linear configuration and does
not rise to the least dependent portion of the orbit
should raise the suspicion of retained wooden foreign
body
• Wood painted with metallic paints appears
hyperdense. When not initially diagnosed, wooden
foreign bodies create a granulomatous inflammatory
foreign body reaction
• CT demonstrates the foreign body as a linear dense
structure surrounded by a soft tissue mass with
density similar to that of muscle, corresponding to
the foreign body reaction.
a. Ultrasound is useful in identification of foreign
bodies in the anterior orbit and within the eyeball.
b. MR imaging:
• MRI may be done in nonmetallic foreign bodies
• MR is useful in identifying wood and plastic
foreign bodies
• MR is more useful in identifying eyeball and
optic nerve injuries.
BIBLIOGRAPHY
1. Boncoeur-Martel MP, Adenis JP, Rulfi JY, Robert PY,
Dupuy JP, Maubon A. CT appearances of chronically
retained wooden intraorbital foreign bodies. Neuroradiol
2001;43(2):165-8.
2. Chacko JG, Figueroa RE, Johnson MH, Marcus DM, Brooks
SE. Detection and localization of steel intraocular foreign
bodies using computed tomography: A comparison of
helical and conventional axial scanning. Ophthalmol
1997;104(2):319-23.
3. Dass AB, Ferrone PJ, Chu YR, Esposito M, Gray L.
Sensitivity of spiral computed tomography scanning for
124 Section 2 The Eye

A B

Figs 9.5A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing a large hyperdense metallic foreign body with
dense metallic artifacts at the superolateral rim of the left orbit partly embedded in the bone

A B

Figs 9.6A and B: Axial CT scan of the orbit of two different patients: (A) Axial CT scan of the orbit showing a hyperdense foreign
body (glass) in the lateral periocular region resulting in focal choroidal hematoma and globe indentation and (B) Axial CT scan of
the orbit showing a hyperdense right intravitreal glass foreign body with increased density in the vitreous suggestive of associated
vitreous hemorrhage

detecting intraocular foreign bodies. Ophthalmol
2001;108(12):2326-8.
4. Fulcher TP, McNab AA, Sullivan TJ. Clinical features and
management of intraorbital foreign bodies. Ophthalmol
2002;109(3):494-500.
5. Glatt HJ, Custer PL, Barrett L, Sartor K. Magnetic
resonance imaging and computed tomography in a model
of wooden foreign bodies in the orbit. Ophthal Plast
Reconstr Surg 1990;6(2):108-14.
6. Gor DM, Kirsch CF, Leen J, Turbin R, Von Hagen S.
Radiologic differentiation of intraocular glass: Evaluation
of imaging techniques, glass types, size, and effect of
intraocular hemorrhage. Am J Roentgenol 2001;
177(5):1199-203.
7. Green BF, Kraft SP, Carter KD, Buncic JR, Nerad JA,
Armstrong D. Intraorbital wood. Detection by magnetic
resonance imaging. Ophthalmol 1990;97(5):608-11.
8. Gunenc U, Maden A, Kaynak S, Pirnar T. Magnetic
resonance imaging and computed tomography in the
detection and localization of intraocular foreign bodies.
Doc Ophthalmol 1992;81(4):369-78.
9. Kollarits CR, Di Chiro G, Christiansen J, Herdt JB,
Whitmore P, Vermess M, Michels RG. Detection of orbital
and intraocular foreign bodies by computed tomography.
Ophthalmic Surg 1977;8(1):45-53.
10. Lagalla R, Manfre L, Caronia A, Bencivinni F, Duranti C,
Ponte F. Plain film, CT and MRI sensibility in the evaluation
of intraorbital foreign bodies in an in vitro model of the
orbit and in pig eyes. Eur Radiol 2000;10(8):1338-41.
11. Lagouros PA, Langer BG, Peyman GA, Mafee MF, Spigos
DG, Grisolano J. Magnetic resonance imaging and
intraocular foreign bodies. Arch Ophthalmol 1987;105(4):
551-3.
12. Lakits A, Prokesch R, Scholda C, Bankier A, Weninger F,
Imhof H. Multiplanar imaging in the preoperative
assessment of metallic intraocular foreign bodies. Helical
Chapter 9 Ocular Trauma 125
computed tomography versus conventional computed
tomography. Ophthalmol 1998;105(9):1679-85.
13. Lakits A, Prokesch R, Scholda C, Nowotny R, Kaider A,
Bankier A. Helical and conventional CT in the imaging of
metallic foreign bodies in the orbit. Acta Ophthalmol
Scand 2000;78(1):79-83.
14. Lakits A, Steiner E, Scholda C, Kontrus M. Evaluation of
intraocular foreign bodies by spiral computed tomography
and multiplanar reconstruction. Ophthalmol
1998;105(2):307-12.
15. LoBue TD, Deutsch TA, Lobick J, Turner DA. Detection
and localization of nonmetallic intraocular foreign bodies
by magnetic resonance imaging. Arch Ophthalmol
1988;106(2):260-1.
16. Papadopoulos A, Fotinos A, Maniatis V, Kavadias S,
Michaelides A, Avouri M, Kalamara C, Stringaris K.
Assessment of intraocular foreign bodies by helical-CT
multiplanar imaging. Eur Radiol 2001;11(8):1502-5.
17. Prokesch R, Lakits A, Scholda C, Bankier A, Ba-Ssalamah
A, Imhof H. Spiral CT and conventional CT in the
preoperative imaging of intraocular metal foreign bodies.
Radiologe 1998;38(8):667-73.
18. Specht CS, Varga JH, Jalali MM, Edelstein JP. Orbitocranial
wooden foreign body diagnosed by magnetic resonance
imaging. Dry wood can be isodense with air and orbital
fat by computed tomography. Surv Ophthalmol
1992;36(5):341-4.
19. Taylor WL Jr. MRI for metallic foreign bodies? Ophthalmol
2000;107(3):410-1.
20. Williams S, Char DH, Dillon WP, Lincoff N, Moseley M.
Ferrous intraocular foreign bodies and magnetic resonance
imaging. Am J Ophthalmol 1988;105(4):398-401.
21. Williamson TH, Smith FW, Forrester JV. Magnetic
resonance imaging of intraocular foreign bodies. Br J
Ophthalmol 1989;73(7):555-8.
126 Section 2 The Eye

A B

Figs 9.7A to C: Axial CT scans of orbits of different patients

showing foreign bodies in different locations in the eye: (A)
Showing a hyperdense foreign body (glass foreign body) in
the temporal ocular coat posterior to the limbus and (B and C)
Showing metallic foreign bodies in the posterior ocular coats
and in the right vitreous cavity respectively. Note: The
C artifacts from the metallic foreign bodies
 Chapter 9 Ocular Trauma 127

Fig. 9.8: Axial CT scan of the orbit showing

hyperdense foreign body embedded in the right
inferior ocular coats. Foreign bodies located in the
superior and inferior quadrant need coronal imaging
for precise localization

A B

Figs 9.9A and B: A 30-year-old patient with history of injury with luminescent bulb: (A) Axial CT scan and (B) Coronal CT scan of
the orbit showing curvilinear hyperdense foreign body in the right vitreous
128 Section 2 The Eye

A B

Figs 9.10A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing curvilinear (staple) right intravitreal
foreign body

Fig. 9.11: Road traffic accident. Axial CT scan of the

orbit showing a ruptured left globe with multiple foreign
bodies (wind-shield glass pieces) in the left orbit and
globe and a linear foreign body in the right ocular
coats with extensive intraocular hemorrhage
 Chapter 9 Ocular Trauma 129

Fig. 9.12: Coronal CT scan of the orbit showing

multiple intraorbital metallic foreign bodies in the right
orbit (abutting the optic nerve and in the lateral wall)

A B

Figs 9.13A to C: A 19-year-old boy with

history of injury with the toothbrush and
diplopia: (A) Coronal CT scan of the orbit
showing a linear well-defined hypodensity
extending from the nasal cavity into the left
inferior orbit displacing the inferior rectus,
(B) Coronal T1-weighted image and
(C) Coronal T2-weighted image showing
linear T1 and T2 hypointensity better
delineated than CT. Endoscopy confirmed the
C plastic foreign body (toothbrush)
130 Section 2 The Eye

A B

Figs 9.14A to C: Axial CT scan of the

orbits at different levels in a patient
with history of injury with wooden stick,
and discharging lateral sinus. CT scan
shows the hyperdense wooden
foreign body in the lateral rectus
muscle with surrounding foreign body
C reaction

A B

Figs 9.15A and B: A 4-year-old child presented with proptosis and history of injury with branch of tree: (A) Axial CT scan of the
orbit and (B) Coronal CT scan showing an ill-defined soft tissue in the right inferior orbit. Histopathology—fungal infection with
multiple wooden pieces were seen within, not appreciated on CT
Chapter 10
Postoperative Evaluation
of Eye and Orbit
IMAGING: POSTOPERATIVE EYE
Imaging of the orbit following surgery is done when any
postoperative complication is suspected.
APHAKIA
The absence of the crystalline lens is defined as aphakia.
The most common cause of aphakia is the surgical
removal of the lens.
Imaging
• The normal lens is seen as a dense lenticular opacity
on CT scan with HU ranging from 90 to 110. In
aphakia, this opacity will be absent
• In pseudophakia, the implanted artificial lens (IOL)
will be seen as a faint linear density in the normal
position of the lens. The width of the IOL will be much
smaller than the natural lens
• Imaging is not required generally for these conditions.
Ophthalmic ultrasound is superior for the lens-related
pathology.
SCLERAL BUCKLE
• Scleral buckling surgery is done for the management
of rhegmatogenous retinal detachment. Buckling
materials are usually made of solid silicone tires or
silicone sponges
• Silicone tires are usually placed circumferentially and
can vary in width as well as their circumferential
extent
• Sponges may be placed either radially or circum-
ferentially.
• Encircling band is a thinner silicone strip that is placed
circumferentially—360°. It may be placed along with
the silicone tires or alone
• When the buckle material is placed on the surface of
the full thickness sclera by sutures, then it is known as
explant
• If sclera is dissected and the buckle is buried within
the layers of sclera, then it is known as an implant.
Imaging
• Scleral buckle can be easily identified on CT scan. It is
seen as a dense ring encircling the globe especially
on coronal sections
• On axial sections, it is seen as two rounded densities
on either side of the globe
• Scleral buckle migration and extrusion can be
evaluated on orbital imaging.
SLIPPED AND LOST MUSCLES
• Slipped and lost muscles are complications of
strabismus surgery and scleral buckling surgery
• Slipped muscle is the condition where the muscle
retracts within the sheath at the site of new insertion
to the globe but lost muscle is the condition where
the entire muscle complex including the sheath
(capsule) is not attached to the globe
• Both the conditions can occur due to intraoperative
damage to the muscle or postoperative spontaneous
detachment of muscle from the globe. Rarely, they
can occur post-trauma
132 Section 2 The Eye

A B

C
Figs 10.1A to C: Aphakia: (A) Axial CT scan, (B) Coronal CT scan of the orbit showing bilateral absence of lens and (C) Axial
T2-weighted MR of the orbit shows surgical aphakia in the right globe with normal lens in the left globe
 Chapter 10 Postoperative Evaluation of Eye and Orbit
• These patients present in the immediate postoperative
period with a large angle deviation.
Need for Imaging
• Orbital MRI with surface coil is the preferred imaging
modality
• Imaging is mandatory to locate the position of these
missing muscles as they may not be able to assess
clinically
• High resolution images can be obtained by using
surface coils
• Multiplanar high-resolution MRI provides valuable
information such as location and contractile potential
of disinserted EOMs
• Dynamic imaging can show if there is still contractile
function in a damaged or severed extraocular muscle.
This technique is also useful in differentiating slipped
muscle or that with elongated scars from lost muscle as
the former two cause a fusiform enlargement posterior
to the globe when the missing muscle contract. This
differentiation is critical in the management.
ORBITAL IMPLANTS
There are two types of orbital implants:
1. Spherical orbital implants used after enucleation or
evisceration and include porous hydroxyapatite,
porous polyethylene (medpore) and nonporous
(acrylic) implants.
2. Reconstructive orbital implants are used to repair the
orbit and includes plates, sheets and screws.
Indications for Imaging
• Implant migration: Intraconal position of orbital
implants is important to be able to fit a good overlying
prosthesis. Implant migration can be picked up on
imaging
• The position of extraocular muscles can be assessed
in cases where a secondary orbital implant is planned
• It is necessary to exclude recurrent or residual tumor
within the orbit and also to rule out any intracranial
extension
• The development of orbital and facial bones can be
assessed. This is especially important in children with
anophthalmic sockets. Repeat scans may be needed
to monitor orbital growth
• It helps plan-staged reconstruction in cases of trauma
• Incomplete reduction of orbital fractures can be
assessed up
• Infection due to the implant, can be assessed.
133
CT SCAN
• It is superior to MRI in cases following trauma or
where implants have been used
• It gives accurate bony details
• Volumetric imaging of the orbit can be done, do look
for socket expansion postimplants or expanders.
MR Imaging
• Gadolinium-enhanced MR imaging helps to
determine the fibrovascular ingrowth of hydro-
xyapatite implants prior to pegging, in order to
integrate the implant with an overlying prosthesis
• Prior to socket reconstruction, it can help assess the
orbital vascularity in patients who have undergone
radiotherapy
• Gadolinium-enhanced MR imaging is preferred to
assess soft tissue tumor infiltration
• MR imaging is contraindicated in patients with
ferromagnetic foreign bodies.
SPHERICAL IMPLANTS
These are placed in the orbit following enucleation or
evisceration of the eye. They are of three types:
1. Silicone
2. Acrylic
3. Hydroxyapatite.
Silicone and acrylic implants are solid and do not
permit any ingrowth of vessels or fibrous tissue into the
substance of the same. Hydroxyapatite is a naturally
occurring substance found in coral. It is porous and has
500 zm diameter pores similar to bone. After placement,
it allows fibrovascular ingrowth into the implant and gets
vascularized. These implants are usually wrapped in
autogenous fascia, or preserved fascia or sclera. The recti
muscles are then sutured to the fascia or sclera in normal
anatomic locations to facilitate some amount of
movement of the implant.
Disadvantages of silicone and acrylic implants:
• Implant migration
• High rate of extrusion
• Poor prosthetic motility.
Disadvantages of hydroxyapatite implant:
• Increased rate of inflammation and infection
• Tissue breakdown
• Expensive.
134 Section 2 The Eye

A B

Figs 10.2A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing anterior migration of the scleral buckle (arrow)

A B

Figs 10.3A and B: Axial CT scan of the orbit: (A) Showing scleral buckle (white arrow) in the right eye and (B) With retinal detachment
(black arrow)
 Chapter 10 Postoperative Evaluation of Eye and Orbit
BIBLIOGRAPHY
1. Custer PL, Kennedy RH, Woog JJ, Kaltreider SA, Meyer
DR. Orbital implants in enucleation surgery: A report by
the American Academy of Ophthalmology. Ophthalmology
2003;110(10):2054-61.
2. De Potter P, Duprez T, Cosnard G. Postcontrast magnetic
resonance imaging assessment of porous polyethylene
orbital implant (Medpor). Ophthalmology 2000;107(9):
1656-60.
3. De Potter P, Shields CL, Shields JA, Flanders AE, Rao VM.
Role of magnetic resonance imaging in the evaluation of
the hydroxyapatite orbital implant. Ophthalmology
1992;99(5):824-30.
4. Flanders AE, De Potter P, Rao VM, Tom BM, Shields CL,
Shields JA. MRI of orbital hydroxyapatite implants.
Neuroradiology 1996;38(3):273-7.
5. Hamilton HE, Christianson MD, Williams JP, Thomas RA.
Evaluation of vascularization of coralline hydroxyapatite
ocular implants by magnetic resonance imaging. Clin
Imaging 1992;16(4):243-6.
6. Jordan DR, Gilberg S, Bawazeer A. Coralline
hydroxyapatite orbital implant (bio-eye): Experience with
158 patients. Ophthal Plast Reconstr Surg 2004;20(1):
69-74.
7. Klapper SR, Jordan DR, Ells A, Grahovac S. Hydro-
xyapatite orbital implant vascularization assessed by
magnetic resonance imaging. Ophthal Plast Reconstr Surg
2003;19(1):46-52.
135
8. Kuo MD, Hayman LA, Lee AG, Mayo GL, Diaz-Marchan
PJ. In vivo CT and MR appearance of prosthetic intraocular
lens. Am J Neuroradiol 1998;19(4):749-53.
9. Lane JI, Randall JG, Campeau NG, Overland PK,
McCannel CA, Matsko TA. Imaging of hydrogel episcleral
buckle fragmentation as a late complication after retinal
reattachment surgery. AJNR 2001;22(6):1199-202.
10. Park SW, Seol HY, Hong SJ, Kim KA, Choi JC, Cha IH.
Magnetic resonance evaluation of fibrovascular ingrowth
into porous polyethylene orbital implant. Clin Imaging
2003;27(6):377-81.
11. Sarvananthan N, Liddicoat AJ, Fahy GT. Synthetic
hydroxyapatite orbital implants: A clinical and MRI
evaluation. Eye 1999;13(Pt 2):205-8.
12. Shields CL, Shields JA, De Potter P, Singh AD. Problems
with the hydroxyapatite orbital implant: Experience with
250 consecutive cases. Br J Ophthalmol 1994;78(9):702-6.
13. Spirnak J. Magnetic resonance imaging versus bone scan
for assessment of vascularization of the hydroxyapatite
orbital implant. Ophthal Plast Reconstr Surg 1996;12(2):
127-30.
14. Spirnak JP, Nieves N, Hollsten DA, White WC, Betz TA.
Gadolinium-enhanced magnetic resonance imaging
assessment of hydroxyapatite orbital implants. Am J
Ophthalmol 1995;119(4):431-40.
15. Wu TE, Rosenbaum AL, Demer JL. Severe strabismus after
scleral buckling: Multiple mechanisms revealed by high-
resolution magnetic resonance imaging. Ophthalmology
2005;112(2):327-36.
136 Section 2 The Eye

Fig. 10.4: Status post-evisceration with ball implant (box arrow)

and silastic sheet for orbital floor repair (arrow)

A B C

Figs 10.5A to C: A 9-year-old female child with anophthamia had ball implant done at age 4 years: (A) Axial soft tissue window,
(B) Coronal CT scan of the orbit in bone window and (C) 3D reconstruction of the face showing superotemporal migration of the
ball implant. Note: The small bony right orbit compared to the left

Fig. 10.6: Coronal CT scan of the orbit showing inferior migration of the silastic
sheets placed for left floor repair (arrow)
 Chapter 10 Postoperative Evaluation of Eye and Orbit 137

A B

Figs 10.7A to C: (A) Axial CT scan; (B and C) Coronal CT scan of the orbits showing fatty density soft tissue in the right orbit
representing the dermal graft (black arrow) with an associated cyst adjacent to it
SECTION 3
Disorders of Orbit
SECTION OUTLINE
11. Imaging Anatomy of the Orbit
12. Congenital Lesions of the Orbit
13. Infective Lesions
14. Inflammatory Lesions
15. Vascular Lesions of the Orbit
16. Pediatric Malignant Orbital Tumors
17. Lymphoproliferative Disorders
18. Histiocytic Lesions
19. Lacrimal Gland Lesions
20. Neurogenic Tumors
21. Fibrous Lesions
22. Secondary Orbital Tumors and Paraorbital Lesions
23. Thyroid Eye Disease
24. Orbital Metastases
25. Orbital Trauma
Chapter 11
BONY ANATOMY
The orbit is a conical cavity which has a base, an apex
and four walls. The base opens in the face and has four
borders. The following bones constitute the formation of
the orbit:
1. Superior margin—frontal bone
2. Inferior margin—maxilla and zygoma
3. Medial margin—frontal, lacrimal and maxilla
4. Lateral margin—zygoma and frontal
• The apex lies near the medial end of superior
orbital fissure and contains the optic canal which
opens into the middle cranial fossa
• The roof (superior wall) is formed by the orbital
plate frontal bone and the lesser wing of sphenoid
• The orbital surface has trochlear fovea medially
and lacrimal fossa laterally
• The floor (inferior wall) is formed by the orbital
plate of maxilla, zygoma and palatine bones
Imaging Anatomy
of the Orbit
• The groove for nasolacrimal duct is located
medially near the orbital margin. The infraorbital
foramen which is located close to middle of the
floor, leads to the inferior orbital fissure and
connects the orbit to pterygopalatine and
infratemporal fossa through the infraorbital
foramen. It separates the floor from the lateral wall
• The medial wall is formed by the frontal process
of maxilla, lacrimal bone, orbital plate of ethmoid
and part of the body of the sphenoid
• The lateral wall is formed by the orbital process of
zygomatic bone and the orbital plate of greater
wing of sphenoid. The bones meet at the
zygomaticosphenoid suture
• The lateral wall is the thickest wall of the orbit
• Soft tissue anatomy has been discussed in the eye
section.
142 Section 3 Disorders of Orbit

CT ANATOMY OF THE ORBIT

Axial Sections taken at 3 mm Intervals from Inferior to Superior

Fig. 11.1: Axial CT scan at the level of the maxillary sinus

Fig. 11.2: Axial section at the level of inferior orbit

 Chapter 11 Imaging Anatomy of the Orbit 143

Fig. 11.3: Axial CT scan at mid-orbit level

Fig. 11.4: Axial CT scan of the orbit taken at the level of the optic nerves
144 Section 3 Disorders of Orbit

Fig. 11.5: Axial CT of the orbit—sections taken above the optic nerve

Fig. 11.6: Axial CT of the orbit—at the level of the superior ophthalmic vein
 Chapter 11 Imaging Anatomy of the Orbit 145

Fig. 11.7: Axial CT of the orbit at the level of the lacrimal glands
146 Section 3 Disorders of Orbit

Coronal Anatomy of the Orbit at 3 mm Intervals Perpendicular to the Axial Plane (Posterior to Anterior)

Fig. 11.8: Coronal CT of the orbit just proximal to the optic canal

Fig. 11.9: Coronal CT of the orbit at the apex

 Chapter 11 Imaging Anatomy of the Orbit 147

Fig. 11.10: Coronal CT orbit just proximal to the apex

Fig. 11.11: Coronal CT orbit just behind the globe

148 Section 3 Disorders of Orbit

Fig. 11.12

Fig. 11.13

Fig. 11.14

Figs 11.12 to 11.14: Coronal sections through the globe at 3 mm intervals

 Chapter 11 Imaging Anatomy of the Orbit 149

Axial Bone Window Sections taken at 3 mm Intervals

Fig. 11.15

Fig. 11.16
150 Section 3 Disorders of Orbit

Fig. 11.17

Fig. 11.18
 Chapter 11 Imaging Anatomy of the Orbit 151

Fig. 11.19
152 Section 3 Disorders of Orbit

Coronal Bone Window Section taken at 3 mm Interval: Posterior to Anterior

Fig. 11.20

Fig. 11.21
 Chapter 11 Imaging Anatomy of the Orbit 153

Fig. 11.22

Fig. 11.23
154 Section 3 Disorders of Orbit

Fig. 11.24

Fig. 11.25
 Chapter 11 Imaging Anatomy of the Orbit 155

Fig. 11.26

Fig. 11.27
156 Section 3 Disorders of Orbit

Fig. 11.28

Fig. 11.29

ORBIT PROTOCOLS
CT PROTOCOL
• The scanning plane should be parallel to the hard
palate or infraorbitomeatal line. The slice thickness
can vary from 2 to 3 mm depending on the clinical
indication
• Coronal imaging can be either reformatted images or
direct coronal sections
• The currently available multislice CT scanners, it is
possible to obtain isotropic coronal and sagittal
reformations, thereby reducing the radiation dose to
the patient
• Intravenous contrast should be given in conditions
such as, orbital tumors, vascular lesions and
infections. Contrast should be given in orbital trauma
Chapter 11 Imaging Anatomy of the Orbit 157
when vascular injury and carotid cavernous fistula is
suspected.
MR PROTOCOL
3 mm slice thickness in both axial and coronal planes.
• Axial T1 spin echo and T2 fast spin echo parallel to
the optic nerve
• Coronal T1 spin echo and T2 fast spin echo (with and
without fat suppression)
• Oblique sagittal T2 fast spin echo is done parallel to
the optic nerve
• The imaging parameters should be as per the
equipment used
• Axial T2 FLAIR screening of the brain should be
performed whenever orbit imaging is done.
Chapter 12
CHORISTOMA
Choristomas are defined as mature tissue elements not
normally found at their site of occurrence. Examples
include limbal dermoid, dermolipoma, ectopic lacrimal
gland, and episcleral osseous choristoma.
• Dermolipoma: A dermolipoma is a solid mass of
mature cells with few or no epithelial structures. It is
usually located subconjunctivally at the outer canthus
as a pinkish elevation (Fig. 12.1).
• It usually extends from the epibulbar surface
posteriorly into the orbit. The surface may have
keratinization or hair follicles. It is a known ocular
feature in cases of Goldenhar’s syndrome.
Congenital Lesions
of the Orbit
IMAGING STUDIES
On CT Imaging
• Dermolipoma shows density similar to that of fat
(Fig. 12.2).
• The entire orbital extent of the dermolipoma can be seen.
BIBLIOGRAPHY
1. Eijpe AA, Koornneef L, Bras J, Verbeeten B Jr, Peeters FL,
Zonneveld FW. Dermolipoma: Characteristic CT
appearance. Doc Ophthalmol 1990;74(4):321-8.
2. A Mortada. Orbital dermolipoma with Goldenhar’s
syndrome and exo-phthalmos. Brit J Ophthal 1969;53:786.
 Chapter 12 Congenital Lesions of the Orbit 159

Fig. 12.1: Yellowish-pink temporal subconjunctival mass suggestive of conjunctival lipoder-

moid (arrow)

Fig. 12.2: Axial CT scan of the orbits showing well-marginated conjunctival fat density soft
tissue bilaterally indicative of conjunctival lipodermoids (arrows)
160 Section 3 Disorders of Orbit
DERMOID AND EPIDERMOID CYST
• These are developmental choristomas that arise from
ectodermal cell rests pinched off at suture lines. While
both dermoid and epidermoid cysts are composed of
keratinized squamous epithelium, the dermoid cyst
also contains dermal appendages like hair and
sebaceous glands (Fig. 12.3).
• The most common site of presentation is the
superotemporal quadrant of the orbit, though any site
may be involved.
• Clinically, these cysts are of two types—superficial
(exophytic) and deep (endophytic). In some cases,
spontaneous or traumatic rupture of the cyst can occur
with consequent granulomatous inflammation and
rarely fistula formation.
IMAGING STUDIES
On CT Imaging
• Both dermoid and epidermoid cysts appear as a well-
defined mass with a central hypodensity suggestive
of its lipid content.
• Dermoids may show fat density within, which
appears as a low density area with a HU varying from
–15 to –100 (Fig. 12.4).
• Fat-fluid levels (Fig. 12.5), calcification and tooth (Figs
12.6A and B) may be seen within dermoid cysts but is
not a feature of epidermoids.
• The cysts can remodel the adjacent bone, and in some
cases can erode the bone to produce dumb-bell-
shaped dermoids (Figs 12.7A and B).
• Ruptured dermoid cyst shows surrounding
inflammatory changes.
On MRI Scan
• Dermoid and epidermoid cysts are hypointense on
T1 and hyperintense on T2-weighted images.
• If the cyst contains fat, hyperintense signal on T1-
weighted images is seen.
• Marginal enhancement of the cyst wall may be seen.
Recommendation
CT scan is the preferred imaging for the diagnosis.
BIBLIOGRAPHY
1. Abou-Rayyah Y, Rose GE, Konrad H, Chawla SJ, Moseley
IF. Clinical, radiological and pathological examinations
of periocular dermoid cysts: Evidence of inflammation
from an early age. Eye 2002;16(5):507-12.
2. Nugent RA, Lapointe JS, Rootman J, Robertson WD, Graeb
DA. Orbital dermoids: Features on CT. Radiology 1987;
165(2):475-8.
3. Shields JA, Kaden IH, Eagle RC Jr, Shields CL. Orbital
dermoid cysts: Clinicopathologic correlations, classification,
and management. The 1997 Josephine E. Schueler Lecture.
Ophthal Plast Reconstr Surg 1997;13(4):265-76.
 Chapter 12 Congenital Lesions of the Orbit 161

Fig. 12.3: Gross specimen photograph showing fat and hair

within the dermoid cyst

Fig. 12.4: Axial CT scan of nasal dermoid showing a well-defined

rounded fat density lesion at the nasal bridge (arrow)

Fig. 12.5: Axial CT scan showing a well-defined rounded medial

orbital mass containing fat. Remodeling of the medial orbital
wall is seen—deep dermoid (arrow)
162 Section 3 Disorders of Orbit

Figs 12.6A and B: (A) Axial CT scan of the orbit, bone window and (B) Soft
tissue showing a fairly well-marginated mixed solid and cystic intraconal mass
B containing a well-formed tooth and fat (arrow)—dermoid

Figs 12.7A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbits
showing a lobulated fat density extraconal soft tissue lesion in the
superotemporal quadrant of the left orbit with focal defect in the adjacent
B bone and extension into the adjacent temporal fossa—dumb-bell dermoid

ORBITAL TERATOMA
• This is a rare developmental tumor made of cells
derived from the three germ layers. They present
congenitally or in infancy. The tumor grows very
rapidly in size causing proptosis. Orbital teratoma can
rarely be malignant. The tumor can be cystic or solid
depending on the contents.
IMAGING TECHNIQUES
On CT Scan
• Orbital teratoma appears as a heterogeneous,
(Figs 12.8A and B) usually multilocular cystic mass.
• Multiple fat-fluid levels and calcification can be seen
within the mass.
Chapter 12 Congenital Lesions of the Orbit 163
• The mass can cause orbital enlargement without bony
erosion.
On MRI Scan
• The mass appears well-defined with heterogeneous
signal based on the contents of the teratoma.
• Calcification may be seen as low signal intensity areas.
BIBLIOGRAPHY
1. Mahesh L, Krishnakumar S, Subramanian N, Babu K,
Biswas J. Malignant teratoma of the orbit: A clinicopatho-
logical study of a case. Orbit 2003;22(4):305-9.
2. Walton DS. Congenital orbital teratoma. J Pediatr
Ophthalmol Strabismus 2005;42(1):64.
164 Section 3 Disorders of Orbit

A B

Figs 12.8A and B: Axial CT scan of the orbit showing an irregular heterogeneous retrobulbar mass lesion in the right orbit with
bony remodeling—histopathologically proven teratoma (arrows)

CONGENITAL BONY ABNORMALITIES OF THE ORBIT:
CRANIOSYNOSTOSIS
• Craniosynostosis is premature fusion of the cranial
sutures. Expansion of skull is greatly limited by the
synostosed sutures.
• Defects in the fibrous tissue that separate the bones
have been proposed as possible etiological factors.
Virchow (1851) observed that premature closure of a
cranial suture caused inhibition of growth per-
pendicular to the affected suture with compensatory
growth in the patent sutures.
• Compensation not only occurs in the area of
uninvolved sutures but also in the weaker areas of
the cranial vault and anterior base leading to
ethmoidal prolapse, reduction in depth of orbit and
anterior displacement of greater wing of sphenoid.
• The deformed skull can take different shapes
depending on the extent and number of sutures
closed.
• Closure of coronal or lambdoid suture prevents
growth in the anteroposterior axis leading to wide
skull—brachycephaly
• Closure of coronal suture along with involvement of
other sutures leads to high skull—oxycephaly or
acrocephaly.
• Closure of sagittal suture alone leads to a long narrow
skull—scaphocephaly
• Asymmetric involvement of sutures (unilateral
synostosis of coronal or lamboid suture and rarely
temporosquamous) leads to plagiocephaly.
• A trilobed skull may also be seen rarely in premature
closure of multiple cranial sutures.
• Signs of raised intracranial tension rarely occur unless
more than one suture has closed. It is important to
differentiate craniosynostosis from true microcephaly
where there is failure of the brain to enlarge.
CROUZON’S SYNDROME
(CRANIOFACIAL DYSOSTOSIS)
Variety of sutures may be affected and signs do not
become apparent till first few years of life. It is usually
transmitted as an autosomal dominant trait and rarely
occurs sporadically.
Clinical Features
• Maxillary hypoplasia with relative prognathism
• Shallow orbits
• Parrot beak nose and high-arched palate characterize
this syndrome.
Chapter 12 Congenital Lesions of the Orbit 165
Ophthalmic Signs and Symptoms
• Optic atrophy with or without papilledema
• Exophthalmos often with corneal complications,
rarely spontaneous luxation of globe
• V-exotropia, hypertelorism, nystagmus
• Rarely iris and corneal malformations, cataract and
pupillary abnormalities.
Neurological Features
• Increased intracranial pressure results in neurological
deficits
• Mental retardation.
APERT’S SYNDROME
(ACROCEPHALOSYNDACTYLY TYPE 1)
Apert described this syndrome in 1906 where
craniosynostosis occurs together with syndactyly of
hands and feet. Usually, the cases are sporadic but can
be autosomal dominant.
Systemic Features
Brachycephalic skull, prominent forehead, prominent
grooves over eyebrows, hypoplastic maxilla, high-arched
palate, hypertelorism, low set ears, hearing deficit,
syndactyly of hands and feet and mental retardation are
some of the characteristic features.
Ophthalmic Features
They include antimongoloid obliquity of palpebral
fissures, ptosis, proptosis, V-exotropia, optic atrophy with
or without papilledema, corneal and lens abnormalities,
congenital glaucoma, nystagmus and pigmentary
changes of the retina.
Imaging Features
• CT scan is the imaging modality of choice. The sections
should be studied in soft tissue and bone window
settings. A three-dimensional CT scan of the skull is
useful in guiding the surgeon for surgical
decompression.
• There is no regular pattern of calvarial deformity,
oxycephaly, brachycephaly, scaphocephaly and
trigonocephaly may be present.
166 Section 3 Disorders of Orbit

A B

C D

Figs 12.9A to D: Crouzon’s syndrome: (A) Lateral topogram of the skull showing tower skull with fusion of the coronal sutures
and lambdoid sutures and ridging of the skull (lacunar skull), (B) Axial CT scan of the brain in bone window setting showing fusion
of the lambdoid and coronal sutures with shallow orbits and (C and D) Three-dimensional reconstruction showing fusion of the
coronal, sagittal and lambdoid suture resulting in towering of skull. Bilateral maxillary hypoplasia is seen resulting in relative
prognathism

• Apert’s syndrome is characterized by irregular
craniostenosis with acrobrachycephalic skull.
• A striking Harlequin appearance of orbit is seen as a
result of elevation of roof and lateral wall of orbit.
The supraorbital rim is recessed and infraorbital rim
is hypoplastic. The orbital depth is markedly reduced
as a result of upward tilt of lesser wing of sphenoid
and orbital plate of the frontal bone. Frontalization of
greater wing of sphenoid is noted.
• Ballooning of ethmoid contributes to exotropia.
• Hypoplasia of maxilla results in exophthalmos and
relative prognathism.
• Optic canal is narrow resulting in optic atrophy. The
distance between the intracranial openings of optic
canal is usually normal.
• These patients may have marked extraocular muscle
anomalies ranging from apparent absence of muscle
to abnormal insertion.
PLAGIOCEPHALY
Refers to the abnormal skull or flat-head resulting from
fusion of unilateral coronal sutures. These children may
present with unilateral proptosis and facial asymmetry.
Imaging
• In patients with hemicoronal premature synostosis,
there is ipsilateral elevation of lesser wing of sphenoid
associated with upward extension of superolateral
wall of orbit.
• There is a flattening of the ipsilateral frontal bone.
• The greater wing of sphenoid is expanded, displaced
forward and downward resultings in a large cranial
fossa.
Chapter 12 Congenital Lesions of the Orbit 167
• On the side of fusion, there is a decrease in the volume
of the anterior cranial fossa, elevation of the ethmoidal
plate and tilt of the nasal septum, crista galli and
ethmoidal complex towards the side of fusion.
BIBLIOGRAPHY
1. Adam RU, Lee SH, Truex RC Jr. Computed tomography
in primary craniosynostosis. J Comput Tomogr
1980;4(2):125-31.
2. Aviv RI, Rodger E, Hall CM. Craniosynostosis. Clin Radiol
2002;57(2):93-102.
3. Benson ML, Oliverio PJ, Yue NC, Zinreich SJ. Primary
craniosynostosis: Imaging features. Am J Roentgenol
1996;166(3):697-703.
4. Carmel PW, Luken MG 3rd, Ascherl GF Jr.
Craniosynostosis: Computed tomographic evaluation of
skull base and calvarial deformities and associated
intracranial changes. Neurosurgery 1981;9(4):366-72.
5. Fernbach SK, Feinstein KA. Radiologic evaluation of the
child with craniosynostosis. Neurosurg Clin N Am
1991;2(3):569-85. Review.
6. Gellad FE, Haney PJ, Sun JC, Robinson WL, Rao KC,
Johnston GS. Imaging modalities of craniosynostosis with
surgical and pathological correlation. Pediatr Radiol
1985;15(5):285-90.
7. Leboucq N, Montoya P, Martinez Y, Castan P, Bourbotte
G. Lambdoid craniosynostosis: A 3D-computerized
tomographic approach. J Neuroradiol 1993;20(1):24-33.
8. Lupescu I, Hermier M, Georgescu SA, Froment JC. Spiral
CT evaluation of the craniosynostoses. J Neuroradiol
2000;27(2):128-39. Review.
9. Parisi M, Mehdizadeh HM, Hunter JC, Finch IJ. Evaluation
of craniosynostosis with three-dimensional CT imaging.
J Comput Assist Tomogr 1989;13(6):1006-12.
168 Section 3 Disorders of Orbit

A B

C D

Figs 12.10A to D: (A and B) Axial CT scan of the orbit showing bilateral proptosis with prominent perioptic CSF spaces due to
raised intracranial pressure and (C and D) Three-dimensional CT scan images of the same patient showing fusion of lambdoid
and coronal sutures

A B C

Figs 12.11A to C: Apert’s syndrome: (A) Clinical photograph of a 6-year-old boy with Apert’s syndrome showing bilateral
exophthalmos, hypertelorism, maxillary hypoplasia and relative prognathism and (B and C) Postoperative photographs of hands
and feet following surgery for syndactyly showing the abnormal digits
 Chapter 12 Congenital Lesions of the Orbit 169

A B C

Figs 12.12A to C: (A) Axial CT in bone window of the orbit of an 11-month-old female child and (B and C) Showing partial closure
of the left coronal suture with anterior bowing of the greater wing of sphenoid (arrows) resulting in left proptosis—plagiocephaly

A B C

Figs 12.13A to C: (A) Axial T2-weighted MRI of the brain showing an asymmetric calvarium, (B) Lateral topogram of the skull showing
tower skull with fusion of the coronal suture and (C) Three-dimensional CT of the skull showing fusion of the sagittal suture and left
coronal
Chapter 13
ORBITAL INFECTIVE LESIONS
• Orbital infections account for 60 percent of the orbital
disease processes. They can be acute, subacute or
chronic. Orbital cellulitis is a common cause of
proptosis in children.
• Orbital infections if not treated adequately may be
both vision and life-threatening, hence, it is essential
to have an early diagnosis and adequate treatment.
With the advent of better antibiotics and
refinement in imaging technology, the morbidity and
mortality associated with sino-orbital infections has
dramatically reduced.
• Preseptal infection rarely affects orbital functions,
while decreased vision and reduced ocular motility
are features of a postseptal orbital infection. Up to
seven percent of cases of orbital cellulitis result in
visual loss.
• Orbital infections can occur by three mechanisms:
1. Extension of an infection from the periorbital
structures, most commonly from the paranasal
sinuses, but also from the face, the globe, and the
lacrimal sac.
2. Direct inoculation of the orbit from trauma or
surgery.
3. Hematogenous spread from bacteremia.
• Symptoms in patients with orbital cellulitis include
fever, headache, malaise and a history of recent
sinusitis or upper respiratory tract infection. Proptosis
and ophthalmoplegia are the cardinal signs and
symptoms of orbital cellulitis.
Infective Lesions
• Other clinical signs include conjunctival chemosis,
decreased vision, elevated intraocular pressure and
pain on eye movements.
• Ethmoid sinusitis is the most common cause of orbital
cellulitis in all age groups and aerobic nonspore
forming bacteria are the organisms most frequently
responsible.
• Bacterial causes of orbital cellulitis are commonly
Streptococcus species, S. aureus, and Haemophilus
influenzae type B, Pseudomonas, Klebsiella, Eikenella, and
Enterococcus infections of the orbit are less common.
• Infection from the paranasal sinuses can spread to the
orbit via to the interconnecting valveless veins and
can then involve the cavernous sinus.
STAGING OF ORBITAL CELLULITIS
1. Preseptal cellulitis: The infection is limited to the
preseptal space.
2. Postseptal cellulitis and inflammatory edema: The
infection extends behind the orbital septum to involve
structures within the bony orbit.
3. Subperiosteal phlegmon and abscess: The most
common location of a subperiorbital abscess is along
the medial orbital wall, because it is thin and
perforated and is situated in close proximity with the
paranasal sinuses medially. Inflammatory tissue and
edema collect beneath the periosteum to form a
subperiosteal phlegmon which can subsequently
 Chapter 13 Infective Lesions 171

Fig. 13.1: Axial CT scan of the orbit showing extensive left

preseptal and periorbital soft tissue edema—preseptal cellulitis

A B

Figs 13.2A to C: (A) Coronal T1-

weighted image, (B) Coronal T2-
weighted image and (C) Coronal post-
contrast T1 fat-suppressed image. They
show left frontal, maxillary and ethmoid
sinusitis with superior subperiosteal
enhancing soft tissue (phlegmon),
surrounding fat stranding (arrows) and
thickening of the medial rectus, superior
oblique and superior rectus-levator
C complex—orbital cellulitis with phlegmon
172 Section 3 Disorders of Orbit

Figs 13.3A to C: A 14-year-old boy with history of painful proptosis

of the right eye with decreased vision since ten days. (A) Clinical
photograph showing gross proptosis of the right eye with conjunctival
chemosis and purulent discharge, (B) Postcontrast axial and
(C) Coronal CT scan of orbits showing pre- and postseptal
hypodense lesions with peripheral enhancement consistent with
abscess. Note: The distortion of the globe, proptosis and left temporal
C arachnoid cyst

develop into a subperiosteal abscess. The periorbita
is adherent relatively loosely to the bone of the medial
orbital wall, which allows abscess material to spread
easily within the subperiorbital space. Progression of
the disease may lead to superior ophthalmic vein
thrombosis.
4. Orbital abscess: A discrete pus collection is seen within
the orbit.
5. Cavernous sinus thrombosis: This arises from an
infection in an area having venous drainage to the
cavernous sinus. It can have unilateral or bilateral
presentation.
Diagnostic Techniques
• Imaging plays a vital role in identifying early post-
septal and intracranial infection.
• CT scan with contrast can be done in patients with
suspected postseptal cellulitis. However, if cavernous
sinus thrombosis is suspected MRI with contrast is
the modality of choice.
• CT scan should include thin sections of the orbit and
paranasal sinuses in axial and coronal planes.
INFLAMMATORY EDEMA AND
PRESEPTAL CELLULITIS
• CT and MRI scans demonstrate eyelid edema. No
orbital involvement is seen.
• Concurrent paranasal sinus infection may be evident.
POSTSEPTAL CELLULITIS
(INFLAMMATORY EDEMA)
• CT and MR imaging findings include soft-tissue
thickening of the eyelids. Usually, the process is
diffuse. The other findings include retrobulbar fat
stranding, extraocular muscles and optic nerve
thickening.
• In postseptal cellulitis, orbital imaging with contrast
is necessary to differentiate inflammatory edema,
orbital phlegmon and orbital abscess.
• Orbital phlegmon: Inflammatory edema may proceed
to phlegmon. CT/MRI show soft tissue along the
orbital wall in the subperiosteal space. Postcontrast
study shows diffuse enhancement of the soft tissue.
SUBPERIOSTEAL ABSCESS
• Elevation of the periosteum is usually contiguous to
the infected sinus. It appears as a necrotic center with
an enhancing peripheral rim on CT and MR imaging.
• A contrast-enhanced CT/MRI is essential to identify
an orbital or subperiosteal abscess. The ethmoid sinuses
Chapter 13 Infective Lesions 173
are commonly involved, hence, the subperiosteal
abscess are commonly located inferomedially or
superomedially.
• A diffusion weighted sequence will help in identifying
the abscess.
ORBITAL ABSCESS
Orbital abscess are usually seen after penetrating injury/
postsurgery or in systemic infection rather than following
sinusitis:
• Contrast-enhanced CT/MRI show well-defined
lesions with central necrosis and peripheral
enhancement. There may be associated fat stranding
and thickening of the extraocular muscles.
• MRI is superior to CT in demonstrating the abscess—
it appears as a central T1 hypointense and T2
hyperintense lesion with thick irregular rim.
Restricted diffusion is seen in bacterial abscess.
SUPERIOR OPHTHALMIC VEIN AND
CAVERNOUS SINUS THROMBOSIS
• Infections from the midface, paranasal sinuses and
orbit may spread to the cavernous sinus.
• A contrast-enhanced CT or MRI is essential for the
diagnosis.
• On CT: Cavernous sinus thrombosis is seen as
enlarged cavernous sinus with nonenhancing areas
and associated features of dilated superior ophthalmic
vein and thickened extraocular muscles on the same
side.
• On MRI: The superior ophthalmic vein will be dilated.
The normal flow void signal will be replaced by
hyperintense signal within on T1 and T2-weighted
images. The cavernous sinus will show abnormal soft
tissue and hyperintense signal within the thrombosed
vascular channels.
BIBLIOGRAPHY
1. Eufinger H, Machtens E. Purulent pansinusitis, orbital
cellulitis and rhinogenic intracranial complications.
J Craniomaxillofac Surg 2001;29(2):111-7.
2. Eustis HS, Mafee MF, Walton C, Mondonca J. MR imaging
and CT of orbital infections and complications in acute
rhinosinusitis. Radiol Clin North Am 1998;36(6):1165-83,
xi. Review.
3. Shovlin JP. Orbital infections and inflammations. Curr
Opin Ophthalmol 1998;9(5):41-8. Review.
4. Tovilla-Canales JL, Nava A, Tovilla Y, Pomar JL. Orbital
and periorbital infections. Curr Opin Ophthalmol
2001;12(5):335-41. Review.
174 Section 3 Disorders of Orbit

Fig. 13.4: Axial T2-weighted MRI of the orbit showing multiple

intraconal hyperintense lesions with hypointense rim suggestive
of abscess in a 4-year-old child presenting with proptosis
Staphylococcus aureus was grown on culture

A B

Figs 13.5A and B: (A) Axial contrast-enhanced CT scan and (B) Coronal contrast-enhanced CT scan of the orbits showing
opacification of the right maxillary sinus (dotted arrow) with fluid level within. There is a subperiosteal hypodensity with peripheral
enhancement (abscess) in the right inferior orbit (black arrow) with perinasal abscess and extension into the right nasal cavity
(white block arrow)
 Chapter 13 Infective Lesions 175

A B

Figs 13.6A to C: A 19-year-old male presented with rapid onset of proptosis of right eye with congestion and chemosis:
(A) Noncontrast-enhanced axial CT scan, (B) Contrast-enhanced axial CT scan of the orbit showing a well-defined soft tissue
lesion along the lateral wall of right orbit with central necrosis and thick irregular peripheral enhancement and (C) Coronal
diffusion-weighted image showing restricted diffusion within the abscess
176 Section 3 Disorders of Orbit
FUNGAL ORBITAL INFECTIONS
Fungal orbital infections occur less frequently as
compared to bacterial infections. They are usually seen
in immunocompromised individuals or those with
uncontrolled diabetes mellitus. Involvement of the nasal
cavity and paranasal sinuses can cause contiguous orbital
infection.
Mucormycosis and aspergillus are the most common
organisms responsible for fungal orbital infections.
Fungal infections are associated with high mortality,
hence early clinical and radiological diagnosis may
improve the overall outcome of the patients.
DIAGNOSTIC TECHNIQUES
CT and MRI help assess the location and extent of the
disease.
• Unilateral or bilateral sinusitis with diffuse soft tissue
infiltration and destruction of bony orbital walls is
usually seen.
• Fungal infections can produce vascular thrombosis.
• These infections can resemble aggressive orbital
tumors on imaging.
• Mycotic infection appears hypointense on T2-
weighted MRI scans, possibly due to the presence of
ferromagnetic elements such as iron, zinc, magnesium
and manganese in high concentration.
• MRI with contrast is the modality of choice in patients
with suspected fungal orbital infections as it
demonstrates the extent of the disease especially
intracranial spread.
BIBLIOGRAPHY
1. DelGaudio JM, Swain RE Jr, Kingdom TT, Muller S,
Hudgins PA. Computed tomographic findings in patients
with invasive fungal sinusitis. Arch Otolaryngol Head
Neck Surg 2003;129(2):236-40.
2. Levin LA, Avery R, Shore JW, Woog JJ, Baker AS. The
spectrum of orbital aspergillosis: A clinicopathological
review. Surv Ophthalmol 1996;41(2):142-54.
3. Roithmann R, Shankar L, Hawke M, Chapnik J, Kassel E,
Noyek A. Diagnostic imaging of fungal sinusitis: Eleven
new cases and literature review. Rhinology 1995;33(2):
104-10. Review.
4. Silverman CS, Mancuso AA. Periantral soft-tissue
infiltration and its relevance to the early detection of
invasive fungal sinusitis: CT and MR findings. Am J
Neuroradiol 1998;19(2):321-5.
5. Som PM. Imaging of paranasal sinus fungal disease.
Otolaryngol Clin North Am 1993;26(6):983-94. Review.
6. Zinreich SJ, Kennedy DW, Malat J, Curtin HD, Epstein JI,
Huff LC, Kumar AJ, Johns ME, Rosenbaum AE. Fungal
sinusitis: Diagnosis with CT and MR imaging. Radiology
1988;169(2):439-44.
 Chapter 13 Infective Lesions 177

A B

Figs 13.7A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbits showing homogeneous hyperdense mass at right
orbital apex (black arrow), extending into inferior orbital fissure and infratemporal fossa. HPE—confirmed fungal infection.
Note: The right lateral orbitotomy defect

A B

Figs 13.8A and B: (A) Coronal CT scan and (B) Axial CT scan of the orbits showing ill-defined heterogeneous extraconal inferior
and medial orbital mass with necrosis within. HPE—confirmed fungal lesion
178 Section 3 Disorders of Orbit

A B

Figs 13.9A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbits showing diffuse nonenhancing hypodense intraconal
and extraconal mass with calcification extending till the orbital apex encasing the optic nerve with bony remodeling and erosion of
the floor of the orbit

A B

Figs 13.10A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbits showing opacified ethmoid sinuses and hyperdense
soft tissue in paranasal sinuses with extraconal extension into the left medial and inferior orbit and thickening of the medial and
inferior rectus muscles—fungal sinusitis with orbital involvement
 Chapter 13 Infective Lesions 179

Fig. 13.11: A 30-year-old female presented with preseptal cellulitis

and progressed to cavernous sinus thrombosis in 24 hours.
T1-weighted postcontrast image of the orbits showing proptosis
of the right globe with enhancing soft tissue in the ethmoid sinus,
superior orbital fissure and cavernous sinus. Note: The distortion
of the globe. Biopsy form the nasal cavity showed aspergillosis

A B C

Figs 13.12A to C: (A) Coronal CT scan and (B and C) Axial CT scan of the orbits showing well-defined heterogeneous mass in the
left lacrimal sac area with areas of necrosis within. The adjacent paranasal sinuses are clear—fungal infection of the lacrimal sac

A B

Figs 13.13A and B: Axial CT scan of the orbits showing opacification of the paranasal sinuses with a large ill-defined lesion in the
left medial and inferior orbit resulting in proptosis and lateral displacement of the globe
180 Section 3 Disorders of Orbit

A B

Figs 13.14A to C: A 39-year-old male presented with proptosis of the right eye of few months duration. He gave history of
previous sinus surgery (histopathology was unavailable): (A) Coronal T2-weighted image, (B) Axial T2-weighted image and
(C) Coronal T1-weighted image showing postoperative changes in the nasal cavity, maxillary ostium and ethmoid sinuses on both
sides. T2-hypointense soft tissue lesion is seen in the right ethmoid sinus, maxillary ostium and a mass like lesion in the medial
and inferior right with the ethmoid sinus. Note: Mucosal thickening in the right maxillary sinus—fungal infection was confirmed on
histopathology

ORBITAL TUBERCULOSIS
Tuberculosis involving the orbit is rare. Spread may be
from an adjacent lesion or by hematogenous route.
Following presentations were observed in a series of 79
patients in a review article:
• Classical periostitis
• Orbital soft tissue tuberculoma or cold abscess with
no bony involvement
• Orbital abscess with bony involvement
• Spread from the paranasal sinuses
• Tuberculous dacryoadenitis
The ocular adnexa can also be involved.
DIAGNOSTIC TECHNIQUES
Imaging
1. Periostitis—CT scan will show areas of lysis with
periosteal reaction. On MRI, altered signal may be
noted in the affected bone seen as replacement of the
normal fatty marrow signal.
2. Orbital abscess with no bony involvement—thick-
walled rim enhancing lesion will be seen. The signal
from the abscess cavity will vary depending on the
degree of liquefaction.
Tuberculomas will show hypointensity or isoin-
tensity on T2-weighted images. This corresponds to
Chapter 13 Infective Lesions 181
restricted mobile protons within high protein contents
in organized caseation, cellular and collagenous
layers.
3. Abscess with bony involvement—imaging will show
a lytic bony lesion with surrounding thick-walled
abscess.
4. Tuberculous dacryoadenitis—imaging will show an
enlarged lacrimal gland with a necrotic center.
Periglandular fat stranding will be seen due to
surrounding inflammatory reaction. In the absence
of central necrosis, it may be difficult to differentiate
it from other causes of lacrimal gland enlargement.
BIBLIOGRAPHY
1. Aggarwal D, Suri A, Mahapatra AK. Orbital tuberculosis
with abscess. J Neuroophthalmol 2002;22(3):208-10.
2. Khalil M, Lindley S, Matouk E. Tuberculosis of the orbit.
Ophthalmology 1985;92(11):1624-7.
3. Kaur A, Kant S, Bhasker SK. Periorbital tuberculosis. Orbit
2007;26(1):39-42.
4. Madge SN, Prabhakaran VC, Shome D, Kim U, Honavar
S, Selva D. Orbital tuberculosis: A review of the literature.
Orbit 2008;27(4):267-77.
5. Roberts BN, Lane CM. Orbital tuberculosis. Eye 1997;
11(Pt 1):138-9.
182 Section 3 Disorders of Orbit

A B

Figs 13.15A and B: (A) Clinical photograph showing a mass in the left lateral orbit displacing the globe medially and (B) Axial CT
scan of the orbits showing a fairly well-defined mass in the lateral canthal region indenting the globe, with areas of necrosis
within—histopathology confirmed tuberculosis

A B

Figs 13.16A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing erosion of the lateral wall of the left orbit
(dotted arrow) and an adjacent lateral extraconal and temporal epidural abscess (black arrow)—tuberculous osteitis with abscess
Chapter 14
Inflammatory Lesions
ORBITAL PSEUDOTUMOR
• Orbital pseudotumor is a nonspecific idiopathic
inflammatory condition for which no identifiable local
or systemic cause can be found. Hence, this is a
diagnosis of exclusion
• It can involve any or all soft tissue components of the
orbit
• It almost always causes unilateral involvement of the
orbit, though in children bilateral involvement occurs
in up to 30 percent of cases
• Rapid onset unilateral painful proptosis, conjunctival
congestion, ptosis and decreased vision are the usual
presenting features
• Orbital pseudotumor can be classified as:
1. Acute and subacute idiopathic anterior orbital
inflammation
2. Acute and subacute idiopathic diffuse orbital
inflammation
3. Acute and subacute idiopathic myositic orbital
inflammation
4. Acute and subacute idiopathic apical orbital
inflammation
5. Idiopathic dacryoadenitis
• Open neuropathy occurs in apical involvement
• Anterior and diffuse involvement can mimic
episcleritis
• When the inflammation from the orbital apex extends
into the cavernous sinus, it is referred to as the Tolosa-
Hunt syndrome. Ocular motor nerve palsies along
with sensory loss along the ophthalmic and maxillary
divisions of the trigeminal nerve may be seen in
cavernous sinus involvement
• Orbital biopsy is done to confirm the diagnosis and
to exclude other causes, especially neoplasm
• Histopathology reveals nonspecific nongranulomatous
inflammation which is replaced by fibrosis in later
stages
• Good response to corticosteroid therapy is suggestive
of orbital pseudotumor. Recurrences can occur,
eventually leading to severe fibrosis and frozen orbit
• Differential diagnosis includes bacterial orbital
infection, thyroid ophthalmopathy, Wegener's
granulomatosis, sarcoidosis, orbital tuberculosis,
malignant orbital tumors.
DIAGNOSTIC TECHNIQUES
CT and MR findings are based on the location of the lesion
and include the following:
• Proptosis
• Extraocular muscle enlargement—a single or multiple
muscles may be involved simultaneously. The
involvement of the muscle tendon and belly gives the
muscle a tubular or fusiform shape with perimuscular
fat stranding
• Orbital fat infiltration—this is seen as diffuse streaky
densities or mass like infiltration of the retrobulbar fat
• Diffuse increase in the density of orbital fat with
obliteration of the optic nerve, muscles and circum-
ferential involvement of the globe is seen in sclerosing
pseudotumor due to subacute or chronic inflammation
184 Section 3 Disorders of Orbit

Fig. 14.1: Myositis—coronal CT scan of the orbit

showing thickening of the left superior muscle
complex with perimuscular fat stranding (arrow)

Fig. 14.2: Tenon’s fasciitis, scleritis and perineuritis.

Axial CT scan of the orbits showing thickening of the
ocular coats with an ill-defined soft tissue in the left
Tenon’s space, thickening of the optic nerve sheath
complex and perioptic haziness
 Chapter 14 Inflammatory Lesions 185

• Lacrimal gland enlargement—this is usually diffuse RECOMMENDATIONS

with preservation of the shape of the gland and
• CT scan is the preferred modality as orbital fat offers
thickening of the adjacent rectus muscles
excellent contrast
• Perineuritis—thickening of the optic nerve sheath
• When Tolosa-Hunt syndrome is suspected an MRI
complex with perioptic enhancement is seen
with contrast should be the imaging modality of
• Scleritis—thickening of the ocular coats with peri-
choice.
ocular fat stranding is seen
• Bone destruction—this rarely occurs. Bone
BIBLIOGRAPHY
remodeling may be seen in chronic sclerosing
pseudotumor 1. Curtin HD. Pseudotumor. Radiol Clin North Am
• In patients with painful III, IV and VI nerve palsies 1987;25(3):583-99.
one should look at the apex, superior orbital fissure 2. Flanders AE, Mafee MF, Rao VM, Choi KH. CT
and cavernous sinus. A contrast MR imaging is a characteristics of orbital pseudotumors and other orbital
preferred modality. inflammatory processes. J Comput Assist Tomogr
1989;13(1):40-7.
ADVANTAGES OF MR IMAGING 3. Gordon LK. Orbital inflammatory disease: A diagnostic
and therapeutic challenge. Eye 2006;20(10):1196-206.
• Extraorbital spread, orbital fat infiltration and
4. Rootman J, McCarthy M, White V, Harris G, Kennerdell J.
perineuritis are better delineated
Idiopathic sclerosing inflammation of the orbit: A distinct
• Chronic pseudotumor displays low signal intensity on clinicopathologic entity. Ophthalmology 1994;101(3):570-
T1 and T2-weighted images, the signal being darker 84. Review.
with increasing degree of fibrosis and variable contrast 5. Yousem DM, Atlas SW, Grossman RI, Sergott RC, Savino
enhancement. Hence, one can differentiate acute versus PJ, Bosley TM. MR imaging of Tolosa-Hunt syndrome.
chronic lesions and can predict the degree of fibrosis. Am J Roentgenol 1990;154(1):167-70.
186 Section 3 Disorders of Orbit

A B

Figs 14.3A and B: Lacrimal gland pseudotumor: (A) Clinical photograph showing S-shaped left upper eyelid deformity due to
lacrimal gland enlargement with edema and erythema characteristic of an acute inflammation and (B) Axial CT scan of the orbits
showing diffuse enlargement of the palpebral and orbital lobe of the left lacrimal gland with preservation of the shape of the gland

A B C

Figs 14.4A to C: Sclerosing pseudotumor. A 12-year-old girl presented with proptosis and frozen globe: (A and B) Axial CT scan
and (C) Coronal CT scan of the orbits showing diffuse soft tissue mass lesion infiltrating the retrobulbar fat and extraocular
muscles and completely surrounding the globe with extension into the infratemporal fossa and remodeling the bony orbit
 Chapter 14 Inflammatory Lesions 187

A B

C D

Figs 14.5A to D: A 47-year-old male presented with right optic neuritis: (A) Coronal T1-weighted image, (B) Coronal T2-weighted
image, (C) Axial T1-weighted image and (D) Coronal T1-postcontrast fat suppressed image. They show an ill-defined enhancing
soft tissue at the right orbital apex encasing the optic nerve (arrows)
188 Section 3 Disorders of Orbit
WEGENER’S GRANULOMATOSIS
• Wegener’s granulomatosis is a multifocal necrotizing
vasculitis which classically involves the respiratory
tract and kidneys
• Orbit involvement is common in the classic and
limited forms of the disease. In the limited form of
the disease, lung involvement does not occur and the
prognosis is better
• The most common mechanism of orbital involvement
is extension of the disease through the paranasal
sinuses or nasopharynx. Isolated orbital involvement
can also occur
• Proptosis, pain, decreased vision, limitation of ocular
movements and periocular swelling are the clinical
features. The presentation can be bilateral. The
lacrimal gland and lacrimal drainage system may also
be involved
• Apart from orbit imaging, a complete systemic work-
up is essential in the diagnosis of Wegener’s
granulomatosis which includes chest radiography and
serum antineutrophil cytoplasmic autoantibody
(ANCA) estimation where cytoplasmic ANCA is
known to be specific for Wegener’s granulomatosis
• Histopathological appearance of vasculitis,
granulomatous inflammation and tissue necrosis help
confirm the diagnosis
• Trimethoprim-sulfamethoxazole or methotrexate may
be valuable in selected patients with limited forms of
Wegener’s granulomatosis.
IMAGING CHARACTERISTICS
• CT and MR findings are nonspecific and show ill-
defined orbital infiltrations with or without lacrimal
gland involvement
• The orbits and paranasal sinuses can be concurrently
involved
• Orbital mass can be intraconal or extraconal. The
orbital apex can also be involved
• Extraocular muscle thickening can be seen
• Contrast enhancement is variable
• Hypointense signal is seen on T2-weighted images
depending on the degree of fibrosis.
BIBLIOGRAPHY
1. Bhatia A, Yadava U, Goyal JL, Chaturvedi KU. Limited
Wegener’s granulomatosis of the orbit: A case study and
review of literature. Eye 2005;19(1):102-4. Review.
2. Courcoutsakis NA, Langford CA, Sneller MC, Cupps TR,
Gorman K, Patronas NJ. Orbital involvement in Wegener
granulomatosis: MR findings in 12 patients. J Comput
Assist Tomogr 1997;21(3):452-8.
3. Duffy M. Advances in diagnosis, treatment, and manage-
ment of orbital and periocular Wegener’s granulomatosis.
Curr Opin Ophthalmol 1999;10(5):352-7.
4. Gordon LK. Orbital inflammatory disease: A diagnostic
and therapeutic challenge. Eye 2006;20(10):1196-206.
5. Muhle C, Reinhold-Keller E, Richter C, Duncker G, Beigel
A, Brinkmann G, Gross WL, Heller M. MRI of the nasal
cavity, the paranasal sinuses and orbits in Wegener’s
granulomatosis. Eur Radiol 1997;7(4):566-70.
6. Provenzale JM, Mukherji S, Allen NB, Castillo M, Weber
AW. Orbital involvement by Wegener’s granulomatosis:
Imaging findings. Am J Roentgenol 1996;166(4):
929-34.
 Chapter 14 Inflammatory Lesions 189

Fig. 14.6: A 30-year-old female presented with painful limitation

of ocular movements of the right eye. Axial CT scan of the orbits
showing moderate thickening of the right medial and lateral
rectus muscles with mild thickening of the left medial and lateral
rectus muscles. Note: Right lateral orbitotomy defect. HPE—
Wegener’s granulomatosis

A B

Figs 14.7A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit shows diffuse ill-defined soft tissue occupying the
intraconal and extraconal space of the left orbit, completely encircling the globe and optic nerve (black arrow) with extension into
the left maxillary sinus. The defect in the floor of the orbit is partly due to the disease process and partly postoperative. HPE—
Wegener’s granulomatosis
190 Section 3 Disorders of Orbit

A B

C D

Figs 14.8A to D: A 44-year-old female presented with bilateral painful proptosis and restricted ocular movements: (A) Axial STIR,
(B) Axial postcontrast T1-weighted image, (C) Coronal T2-weighted image and (D) Noncontrast coronal T1-weighted image
showing a diffuse ill-defined soft tissue mass hypointense in both T1- and T2-weighted image and showing moderate enhancement
on postcontrast scans. HPE—Wegener’s granulomatosis

ORBITAL SARCOIDOSIS
• Sarcoidosis is characterized by noncaseating
granulomatous inflammation involving multiple
organ systems
• The disease is commonly seen in young adults
between 20 and 40 years of age
• Sarcoidosis manifests in acute, subacute and chronic
forms. In the acute form, hilar lymphadenopathy,
erythema nodosum and polyarthritis are seen
• Patients with chronic disease have more incidence of
extrapulmonary involvement
• Ocular involvement is seen in approximately 25 to 50
percent of patients with sarcoidosis, of which orbital
involvement occurs in less than 1 percent of cases
• Signs and symptoms of acute or subacute orbital
inflammation are seen, with pain, proptosis, limited
ocular movements and decreased vision being the
common clinical features
• The lacrimal gland is the most commonly affected
orbital tissue. Lacrimal sac, optic nerve and extraocular
muscles can also be affected by sarcoid granulomata.
DIAGNOSTIC TECHNIQUES
• Diffuse enlargement of the lacrimal gland is seen,
which can be unilateral or bilateral and can resemble
a lymphoproliferative disorder
• Enlargement of the optic nerve may occur
A B
Figs 14.9A and B: (A) Axial CT scan of the orbit and (B) Coronal CT scan of the orbit showing bilateral asymmetric enlargement
of the lacrimal glands—sarcoidosis
Chapter 14 Inflammatory Lesions 191
• Extraocular muscle thickening with perimuscular
infiltration is seen
• It usually displays low signal in T2-weighted images
• There may be associated neurosarcoidosis such as
leptomeningeal thickening, chiasmal thickening or T2-
hyperintense lesions in the periventricular and
subcortical region suggestive of vasculitis
• Appropriate work-up of patients with suspected
sarcoidosis is essential and includes chest
radiography, gallium 67 scan of the head and neck,
pulmonary function tests, serum angiotensin-
converting enzyme levels, serum and urine calcium
levels and tissue biopsy. The Kveim test is rarely used
now.
BIBLIOGRAPHY
1. Gordon LK. Orbital inflammatory disease: A diagnostic
and therapeutic challenge. Eye 2006;20(10):1196-206.
2. Mafee MF, Dorodi S, Pai E. Sarcoidosis of the eye, orbit,
and central nervous system: Role of MR imaging. Radiol
Clin North Am 1999;37(1):73-87, x. Review.
3. Shaikh ZA, Bakshi R, Greenberg SJ, Fine EJ, Shatla A,
Lincoff NS. Orbital involvement as the initial
manifestation of sarcoidosis: Magnetic resonance imaging
findings. J Neuroimaging 2000;10(3):180-3.
4. Simon EM, Zoarski GH, Rothman MI, Numaguchi Y,
Zagardo MT, Mathis JM. Systemic sarcoidosis with bilateral
orbital involvement: MR findings. Am J Neuroradiol
1998;19(2):336-7.
192 Section 3 Disorders of Orbit

A B

Figs 14.10A and B: A 19-year-old male presented with proptosis, restricted ocular movements and signs of inflammation:
(A) Axial contrast-enhanced CT scan and (B) Coronal contrast-enhanced CT scan of the orbit showing hyperdense soft tissue
infiltrating the left medial and inferior rectus muscles

A B

C D E

Figs 14.11A to E: A 30-year-old female who presented with painful sudden loss of vision and ophthalmoplegia, in the left eye,
gave past history of vision loss in the right eye and right facial paralysis that resolved with steroids: (A) Axial T1-weighted MRI and
(B) Axial T2-weighted MRI of the orbits showing an ill-defined extraconal soft tissue lesion in the left lateral orbit, superior orbital
fissure and cavernous sinus. It is isointense on T1 and hyperintense on T2-weighted image with respect to the muscle.
(C) Postgadolinium axial and (D and E) Coronal T1-weighted images show diffuse moderate enhancement of the soft tissue in the
left orbit and superior orbital fissure (white arrows) with leptomeningeal nodular thickening (black arrow in Fig. D)

SJÖGREN’S SYNDROME
INTRODUCTION
Sjögren’s syndrome is a chronic autoimmune disorder.
In the primary form, it is characterized by xeroph-
thalmia, xerostomia and chronic inflammation of
the salivary and lacrimal glands. This is also called
the ‘sicca complex’. The secondary form is defined as
sicca complex with connective tissue disorders. Any
organ can be involved. The disease commonly affects
the eyes, mouth, parotid gland, lungs, kidneys, skin
and nervous system.
CLINICAL PRESENTATION
• The majority of the affected patients are middle aged
and elderly women. Female to male ratio is 9:1
• Patients with lacrimal gland involvement present with
pain and redness similar to dacryoadenitis. Associated
involvement of the parotid or other salivary glands
should raise the possibility of Sjögren’s syndrome
• Patients may present with dry mouth, dry eyes,
arthralgia, myalgia and fatigue.
Laboratory test includes Schirmer’s test, ESR
(elevated), RA factor, complete blood count (anemia and
Chapter 14 Inflammatory Lesions 193
leucopenia), anti-SS-A and anti-SS-B, and antinuclear
antibodies (ANCA) and renal function tests.
Imaging
• MRI is the modality of choice to study the signal
intensity changes in the lacrimal and salivary glands
• The involved glands especially the parotid glands
show loss of homogeneity and increased signal in T2-
weighted images
• The signal changes in the lacrimal gland may be
indistinguishable from other inflammatory disorders
• These patients should be followed-up as they may
develop malignant lymphoma later.
BIBLIOGRAPHY
1. Lzumi M, Eguchi K, Ohki M, Uetani M, Hayashi K, Kita
M. MR Imaging of the parotid gland in Sjögren's
syndrome: A proposal for new diagnostic criteria. Am
Roentengol 1996;166(6):1483-87.
2. Mavragani CP, Moutsopoulos HM. The geoepidemiology
of Sjögren's syndrome. Autoimmun Rev, 2009.
3. Rootman J. Diseases of the orbit: A multidisciplinary
approach, 2nd edn. Lippincott: Williams and Willkins
2003;p. 498.
194 Section 3 Disorders of Orbit

A B

Figs 14.12A to C: (A) Axial T2-weighted image, (B) Axial T1-

weighted image showing diffuse enlargement of the left lacrimal
gland with hyperintense signal in T2-weighted image. Note the
periglandular soft tissue edema and (C) Coronal T2-weighted
image showing enlarged right parotid gland with T2 hyperintense
signal. Biopsy of the left lacrimal gland revealed features
C suggestive of Sjögren’s syndrome

AMYLOIDOSIS
• It is a condition characterized by the deposition of an
amorphous hyaline material called amyloid in various
tissues in the body
• It can involve muscles, skin, nerves, adrenal glands
and sometimes orbit. Involvement of the orbit and
ocular adnexa may occur as a localized isolated
process, as part of primary systemic amyloidosis or
as part of secondary amyloidosis
• Causes of secondary amyloidosis of orbit and ocular
adnexa include:
1. Tuberculosis
2. Rheumatoid arthritis
3. Leprosy
4. Osteomyelitis
5. Multiple myeloma.
Involvement of eyelid is always associated with
systemic disease whereas amyloidosis that occurs in
conjunctiva, lacrimal gland or orbit is often a localized
process without systemic implications.
CLINICAL FEATURES
• Blepharoptosis due to infiltration of levator
• Oculomotor palsies due to involvement of multiple
extraocular muscles
• Lacrimal gland involvement is impossible to clinically
differentiate from other tumors of lacrimal gland
• Lesions have a bleeding tendency due to vascular
fragility from perivascular amyloid.
Chapter 14 Inflammatory Lesions 195
IMAGING
CT Scan
The amyloid deposits simulate pseudotumors, vascular
malformation and other mass lesion and deposits can
occasionally calcify.
MRI Imaging
They have signal intensity similar to skeletal muscles.
Bone destruction has been reported.
BIBLIOGRAPHY
1. Banerjee S, Bogman J, Reuser TT. Amyloid deposition in
the extraocular muscles. Orbit 1999;18(2):105-6.
2. Dinakaran S, Singh AD, Rennie IG. Orbital amyloidosis
presenting as ptosis. Eye 2005;19(1):110-2.
3. Murdoch IE, Sullivan TJ, Moseley I, Hawkins PN, Pepys
MB, Tan SY, Garner A, Wright JE. Primary localized
amyloidosis of the orbit. Br J Ophthalmol 1996;80(12):1083-
6.
4. Okamoto K, Ito J, Emura I, Kawasaki T, Furusawa T, Sakai
K, Tokiguchi S. Focal orbital amyloidosis presenting as
rectus muscle enlargement: CT and MR findings. Am J
Neuroradiol 1998;19(9):1799-801.
5. Taban M, Piva A, See RF, Sadun AA, Quiros PA. Review:
Orbital amyloidosis. Ophthal Plast Reconstr Surg
2004;20(2):162-5.
196 Section 3 Disorders of Orbit

A B

Figs 14.13A and B: (A) Coronal CT and (B) Axial CT of the orbit showing proptosis of the left eye with an ill-defined intraconal and
extraconal soft tissue in the left orbit with multiple calcification within. HPE—amyloidosis

Fig. 14.14: Axial CT scan of the orbit showing bilateral ill-

defined slightly hyperdense right intraconal and left
extraconal soft tissue. HPE—amyloidosis
Chapter 15
Vascular Lesions of the Orbit
Vascular malformations of the orbit arise from the arterial
system, venous system or both.
The Orbital Society has classified vascular
malformations of the orbit based on their hemodynamics
into:
Type I (No flow) lesions: These lesions have a minimal
connection with the vascular system. They are lymph-
angiomas or combined venolymphatic malformations.
Type II (Venous flow) lesions: They are further classified
into distensible and nondistensible venous malformations
depending on their communication with the venous
system and demonstration of distensibility by Valsalva
maneuver.
Type III (High flow) lesions: They include arteriovenous
malformations.
Mulliken and Glowaki have classified the vascular
malformations based on their natural history, growth
pattern and histologic component into the following
groups:
a. Capillary hemangiomas
b. Venous vascular malformations—cavernous
malformations and varices
c. Venous lymphatic malformations—capillary,
cavernous and cystic lymphatic malformations
d. Arterial and arteriovenous malformations,
arteriovenous fistulas and ophthalmic artery
aneurysms
e. Neoplasms—hemangiopericytomas
f. Miscellaneous—Coats’ disease.
Most of the lesions have a characteristic imaging
pattern and thereby help in differentiating them
from other lesions. Imaging plays a very vital role in
diagnosis and management of orbital vascular
malformations.
198 Section 3 Disorders of Orbit
CAPILLARY HEMANGIOMA
• It is the most common orbital tumor in children.
The lesion usually presents in the first few months of
life
• The lesion may be superficial (cutaneous or
conjunctival), deep orbital (behind the orbital septum)
or combined (both dermal and deep components)
• Superficial lesions present as reddish cutaneous
masses which rarely pulsate, and can increase in size
when the child cries
• Deeper lesions can present as proptosis
• Spontaneous involution is known to occur by 5 years
of age.
DIAGNOSTIC TECHNIQUES
Imaging delineates the mass precisely and shows its effect
and relationship to the adjacent structure.
CT Scan
• Shows a homogeneous, moderately defined or
infiltrating lesion with marked enhancement
• Orbital masses appear better defined as compared to
superficial lesions
• Orbital lesions are mostly extraconal, but can be
intraconal
• Occasionally, small phleboliths are seen
• They may extend intracranially through the superior
orbital fissure.
MR Imaging
• MRI is superior to CT scan as it helps to characterize
the lesion. The MR appearance is virtually
pathognomonic. It demonstrates lobular pattern,
septae and vessels within the lesion as well as at the
periphery
• It is isointense to brain tissue on T1-weighted images,
and of moderately increased intensity on T2-weighted
images
• A fat-suppressed T2-weighted image and postcontrast
with fat-suppression will outline the lesion well
• Demonstration of vascular flow voids within the
lesion on MR imaging is characteristic of capillary
hemangiomas
• Involuting lesions are ill-defined with fatty
replacement of the lesion may be noted
• The demonstration of the internal architecture on
MRI helps to differentiate it from venolymphatic
malformations and rhabdomyosarcoma.
BIBLIOGRAPHY
1. Bilanuik LT. Orbital vascular lesions: Role of imaging.
Radiol Clin North Am 1999;37:169-83.
2. Sweet C, Silbergleit R, Mehta B. Primary intraosseous
hemangioma of the orbit: CT and MR appearance. Am J
Neuroradiol 1997;18(2):379-81.
3. Torres C, Kelly JK, Watts FB Jr. Capillary hemangioma of
the orbit: The role of computed tomography. J Am
Osteopath Assoc 1987;87(10):687-91.
 Chapter 15 Vascular Lesions of the Orbit 199

Fig. 15.1: External photograph showing a reddish purple

superonasal mass on the left upper eyelid. This is the most
common site of capillary hemangioma in the orbit

A B

Figs 15.2A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing a brilliantly enhancing pre- and postseptal
inferolateral right orbital lesion enveloping the ocular surface—capillary hemangioma

A B C

Figs 15.3A to C: Superficial and deep capillary hemagioma in different patients: (A) Postcontrast axial CT scan of the obrit,
(B) Axial T2-weighted image and (C) Axial T1-weighted image showing a homogeneous pre- and postseptal orbital lesion.
Note: The flow voids seen within the lesions (arrow) characteristic of capillary hemangioma (combined)
200 Section 3 Disorders of Orbit
CAVERNOUS MALFORMATIONS
• Cavernous malformation is also known as cavernous
hemangioma. It is a benign slowly progressive
vascular lesion in the orbit. It is the most common
primary orbital tumor in adults
• Slowly progressive painless proptosis is the most
common presenting feature. Lesion which is large,
close to the optic nerve and small lesion at the apex
may present with visual dysfunction
• They are more common in women.
DIAGNOSTIC TECHNIQUES
CT Scan
• It appears as a homogeneous well-circumscribed and
well-encapsulated lesion, isodense to slightly
hyperdense on noncontrast scan with marked contrast
enhancement
• The mass is usually intraconal in location, though it
can be present anywhere in the orbit
• The presence of phleboliths is pathognomonic of
cavernous hemangioma, and may be seen as small,
round calcified deposits of varying size. These are also
noted in mixed variety of hemangiolymphangioma
or in long-standing cases of capillary hemangioma of
childhood
• Cavernous hemangioma with large vascular pools or
venous varices may show expansion of the lesion with
Valsalva maneuver, and this is described as stress
proptosis
• It is usually single but can be multiple
• The improved image quality of CT and MR imaging,
along with dynamic CT angiography, MR angiography,
MR venography, and multiphase dynamic contrast
CT/MR imaging, has proven useful to delineate as well
as differentiate various orbital vascular lesions.
MR Imaging
• Cavernous hemangioma appears is isointense on T1-
weighted images and hyperintense on T2-weighted
images. On heavily T2-weighted images, they are
almost isointense to vitreous
• The relation of the hemangioma to the optic nerve is
best demonstrated on MRI
• On multiphasic dynamic postcontrast imaging, poor
enhancement is noted in the early arterial phase with
central filling and progressive filling is noted in the
late venous phase.
Doppler Imaging
On echography, the lesions appear hyperechoic and
heterogeneous, with small areas of very slow flow.
BIBLIOGRAPHY
1. Ansari SA, Mafee MF. Orbital cavernous hemangioma: Role
of imaging. Neuroimaging Clin N Am 2005;15(1):137-58.
2. Tanaka A, Mihara F, Yoshiura T, Togao O, Kuwabara Y,
Natori Y, Sasaki T, Honda H. Differentiation of cavernous
hemangioma from schwannoma of the orbit: A dynamic
MRI study. Am J Roentgenol 2004;183(6):1799-804.
3. Thorn-Kany M, Arrue P, Delisle MB, Lacroix F, Lagarrigue
J, Manelfe C. Cavernous hemangiomas of the orbit: MR
imaging. J Neuroradiol 1999;26(2):79-86.
 Chapter 15 Vascular Lesions of the Orbit 201

Fig. 15.4: Axial CT scan of the orbits showing a well-

circumscribed rounded homogeneous intraconal mass
(arrow)—cavernous hemangioma

A B C

Figs 15.5A to C: (A) Axial CT scan, (B) Coronal CT scan of the orbits showing multiple bilateral lobulated soft tissue enhancing
intra- and extraconal lesions with extension into the cavernous sinus on the left (dotted arrow) and (C) Coronal bone window
showing sclerosis and widening of the diploic space in the right frontal bone and zygoma (arrows)—multiple hemangioma

A B C

Figs 15.6A to C: (A) Coronal T1-weighted image, (B) Axial T2-weighted image and (C) Coronal T2-weighted image showing a
well-encapsulated oval intraconal mass lesion medial to the optic nerve (arrows) having homogeneous isointense signal on T1
and hyperintense signal on T2-weighted image (similar to vitreous)—cavernous hemangioma
202 Section 3 Disorders of Orbit

A B

Figs 15.7A to C: A 21-year-old female presented with visual disturbances in the right eye: (A) Noncontrast axial T1-weighted
image, (B) Axial T2-weighted image and (C) Axial T1 postcontrast fat-suppressed image. A well-defined oval homogeneously
enhancing, T1 isointense and T2 hyperintense lesion (arrow) is seen at the right orbital apex compressing the optic nerve (small
arrow)—cavernous hemangioma
 Chapter 15 Vascular Lesions of the Orbit 203

A B

C D

Figs 15.8A to E: A 30-year-old female presented with proptosis

of the right eye of 2 years duration: (A) Axial T2-weighted fat
suppressed image, (B) Axial T1 image, (C) Axial T1 immediate
postcontrast image, (D) Sagittal T1 delayed postcontrast image
and (E) Axial diffusion weighted images show a well-
circumscribed intraconal lesion in the right orbit displaying
isointense signal in T1-weighted image and hyperintense signal
in T2 and diffusion-weighted images. Postcontrast study shows
slow progressive filling of the lesion in the postcontrast images
E lesion (C and D)—cavernous hemangioma
204 Section 3 Disorders of Orbit
VENOUS LYMPHATIC MALFORMATIONS
• Orbital lymphangioma is a congenital no-flow
vascular lesion and better termed as combined
venolymphatic in nature. They generally present in
infancy or early childhood, but may be present at
birth
• Lymphangioma can be superficial or deep in location.
Mixed superficial and deep lesions can also occur. The
lesion may be single or diffuse
• Superficial lesions are seen in the eyelid or
conjunctiva. Bluish skin discoloration or purplish-red
conjunctival mass may be present
• Deep lymphangioma presents with proptosis, which
may be gradual or sudden. Sudden increase in the
proptosis can be associated with upper respiratory
tract infections or hemorrhage. Optic nerve
compression can occur in these patients when the
hemorrhage or chocolate-colored cyst are close to the
optic nerve
• Change in size of the lesion can occur with variation
in posture
• Combined lymphangiomas are usually large, causing
much cosmetic disfigurement. Associated cheek and
palatal lesions should be looked for
• Most of the lesion consists of lymphatic channels. The
deeper lesions in the orbit may have a prominent
venous component.
DIAGNOSTIC TECHNIQUES
CT Scan
• They are usually unencapsulated, diffuse both intra-
and extraconal in location and generally insinuate
between orbital structures
• Bony remodeling is seen in large lesions
• Little or no enhancement is seen on postcontrast
administration
• They may have a multilocular appearance or may
show cysts of varying sizes seen as well-defined non-
enhancing hypodense areas
• They may be associated with intracranial vascular
anomaly, most commonly a deep venous anomaly is
seen
• Phleboliths may be seen in hemangiolymphangiomas
• Fluid-fluid level can be seen with intralesional
hemorrhage.
MR Imaging
• MRI will show the various components of the
venolymphatic malformation
• The MR signal will depend on the contents of the
cyst fluid. Cyst with subacute hemorrhage and
proteinaceous content will be hyperintense in T1-
weighted images and variable signal in T2-weighted
image
• These usually do not enhance with contrast and thus
can help in differentiating them from other lesions
• Fluid-fluid levels will be seen due to layering of blood
within the cyst
• The lesion appears heterogeneous, with the lymph-
containing areas being hypointense in T1-weighted
images and hyperintense on T2-weighted images
• Areas of subacute hemorrhage may appear hyper-
intense on T1-weighted and can be well appreciated
on MRI scan.
BIBLIOGRAPHY
1. Bond JB, Haik BG, Taveras JL, Francis BA, Numaguchi Y,
Mihara F, Gupta KL. Magnetic resonance imaging of
orbital lymphangioma with and without gadolinium-
contrast enhancement. Ophthalmology 1992;99(8):1318-
24.
2. Rootman J. Vascular malformations of the orbit:
Hemodynamic concepts. Orbit 2003;22(2):103-20.
3. Wendy RK Smoker, Lindell R Gentry, Norbert K Yee,
Deborah L Reede, Jeffrey A Nerad. Vascular lesions of
the Orbit: More than meets the eye. Radiographics 2008;28:
185-204.
 Chapter 15 Vascular Lesions of the Orbit 205

A B

Figs 15.9A and B: (A) Bluish lesion in the right superonasal orbit in a case of combined lymphangioma and (B) Subconjunctival
reddish mass in a superficial lymphangioma

A B

Figs 15.10A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing a well-circumscribed intraconal mass with
blood fluid level within (black arrow) encircling the optic nerve (small white arrow)—venolymphatic malformation with hemorrhage
206 Section 3 Disorders of Orbit

Fig. 15.11: Axial postcontrast CT scan showing multilobulated Fig. 15.13: Axial CT scan of the orbits showing a diffuse pre-
multicystic pre- and postseptal lesion in the right orbit veno- and postseptal intra- and extraconal lesion in the left orbit—
lymphatic malformation (arrow) venolymphatic malformation (arrow)

Fig. 15.12: Axial CT scan showing a medial well-defined Fig. 15.14: Axial plain CT scan of the orbit showing multi-
intraconal mass with small cysts within giving it a heterogeneous lobulated cystic intra- and extraconal lesion (arrow)
appearance (arrow)
 Chapter 15 Vascular Lesions of the Orbit 207

A B
Figs 15.15A and B: (A) Axial postcontrast CT scan and (B) Coronal postcontrast CT scan of the orbits
showing multilobulated intra- and extraconal lesion with small cysts and rounded calcification within a
hemangiolymphangioma (arrows)

A B
Figs 15.16A and B: (A) Coronal T2-weighted MR image and (B) Axial T2-weighted MR image of the orbits shows a preseptal and
postseptal multilobulated intra- and extraconal T2 hyperintense lesion in the left orbit causing proptosis (arrows)

A B
Figs 15.17A and B: (A) Axial T1 MR image and (B) Coronal T2 MR image of the orbits showing multilobula-
ted intra- and extraconal lesion with peripheral T1 hyperintense signal suggestive of subacute blood and
predominantly hyperintense signal on T2-weighted images—hemorrhage within a lymphangioma (arrows)
208 Section 3 Disorders of Orbit
DISTENSIBLE VENOUS MALFORMATIONS
• Distensible venous malformations include orbital
varices: This is a tumor comprising a collection of
venous channels. Primary varix is a congenital lesion,
while the secondary varix is acquired after trauma
• Orbital varices can also be classified as distensible or
nondistensible in nature. Intermittent proptosis is a
classic feature, the prominence of the eye increasing
with straining, stooping or crying
• The presence of recent thrombosis or hemorrhage is
associated with typical clinical findings of rapid
development of proptosis, pain, and decreased
motility
• Atrophy of surrounding orbital fat can lead to
enophthalmos in some cases. Pulsations may be noted
with an associated arteriovenous malformation or
with transmitted intracranial pulsations due to a mass
adjacent to a bony defect.
DIAGNOSTIC TECHNIQUES
CT Scan
• An orbital varix appears as a well-defined elongated
mass lesion within the orbit, often with a club-like
configuration with a tapering end towards the orbital
apex
• Orbital enlargement and phleboliths are features of
long-standing cases
• The density and enhancement of the varices depends
on the patency of its lumen. Thrombosis or
hemorrhage will not show enhancement, whereas
patent lumen will show brilliant enhancement.
MR Imaging
• The MR signal will depend on the presence of flow
within the varix. Patent lumen will appear as an area
of flow void on both T1 and T2-weighted scans
• Contrast enhancement is variable and may be absent
• Slow flow or thrombus will give it a slight hetero-
geneous appearance
• The use of fat saturation with contrast enhancement
on MR imaging helps to delineate the varix better
• There can be an association between intraorbital
venous anomalies and noncontiguous intracranial
venous anomalies; hence these should be looked for.
Doppler and orbital venography are other modes of
diagnosis.
Recommendations
Whenever a varix is suspected, it is mandatory to image
the patient both pre- and post-Valsalva’s maneuver as it
can be easily missed in routine supine scans. CT scan with
contrast is the modality of choice.
BIBLIOGRAPHY
1. Bullock JD, Goldberg SH, Connelly PJ. Orbital varix
thrombosis. Ophthalmology 1990;97(2):251-6.
2. Rootman J. Orbital venous anomalies. Ophthalmology
1998;105(3):387-8.
3. Shields JA, Dolinskas C, Augsburger JJ, Shah HG, Shapiro
ML. Demonstration of orbital varix with computed
tomography and Valsalva maneuver. Am J Ophthalmol
1984;97(1):108-10.
4. Yeatts RP, Driver PJ. Orbital varix. Arch Ophthalmol
1993;111(5):702-3.
 Chapter 15 Vascular Lesions of the Orbit 209

A B

Figs 15.18A and B: (A) Axial postcontrast CT scan and (B) Coronal postcontrast CT scan of the orbit showing a well-defined
club-shaped poorly enhancing mass in the lateral left orbit with widening of the superior orbital fissure (arrow) (thrombosed varix).
This patient had a previous Doppler and MRI scan of orbit suggesting flow within the lesion

A B

Figs 15.19A and B: Axial CT scan of the orbit: (A) Prevalsalva maneuver shows a subtle lesion in the right inferior orbit (arrows)
and (B) With significant increase in size on postvalsalva maneuver
210 Section 3 Disorders of Orbit
ARTERIOVENOUS MALFORMATIONS
• Arteriovenous malformations (AVMs) are anomalous
communications between arterial and venous systems
without interposed capillaries
• These lesions are rarely entirely intraorbital. They can
occur as congenital defects, spontaneously or
following trauma
• Clinically, they can be confused with lymphangiomas
or hemangiomas. Arteriovenous malformations can
be either high or low flow. The larger the shunt, the
more prominent are the clinical findings
• A high flow shunt has clinical features of pulsating
exophthalmos, bruit and marked orbital swelling due
to retrograde flow into the venous system
• A low flow lesion, however, can present clinically
with dilated episcleral, intraocular and orbital vessels
associated with mild proptosis, mild bruit and raised
intraocular pressure.
DIAGNOSTIC TECHNIQUES
• Imaging findings are characteristic in arteriovenous
malformations
• Multiple dilated vessels are seen within and around
the orbit
• MR imaging shows multiple flow voids
• CT angiography or digital subtraction angiography
should be performed to know the entire extent and
feeding arteries and draining veins.
BIBLIOGRAPHY
1. Forbes G. Vascular lesions in the orbit. Neuroimaging Clin
N Am 1996;6(1):113-22.
2. Rootman J. Vascular malformations of the orbit:
Hemodynamic concepts. Orbit 2003;22(2):103-20.
 Chapter 15 Vascular Lesions of the Orbit 211

A B

C D

Figs 15.20A to D: (A and B) Axial postcontrast CT scan and (C and D) Coronal CT scan of orbits showing large dilated blood
vessels in the right orbit, temporal fossa and upper lid with grossly dilated right superior ophthalmic vein (arrow) and aneurysm of
the right cavernous ICA (small arrow) with dilated blood vessels in the prepontine suprasellar cistern
212 Section 3 Disorders of Orbit
ORBITAL HEMANGIOPERICYTOMA
• Hemangiopericytomas are rare, slow growing and
highly vascular tumor, 15% arise in the extracranial
sites of head and neck
• They can occur at any age
• Occurrence in the orbit is more common in males
• They present as slowly progressive painless proptosis.
They can also present with visual symptoms or
motility disturbances
• Histologically, they can be benign or malignant.
Metastasis is uncommon, lung being the most
common site
• Histologically, they are classified into sinusoidal, solid
or mixed.
DIAGNOSTIC TECHNIQUES
CT Scan
• Hemangiopericytomas are well-encapsulated,
lobulated tumors with infiltrative margins
• They are usually extraconal and may cause bony
erosion
• Recurrence is common after surgical excision
• Dynamic postcontrast study shows marked contrast
enhancement in the early arterial phase, a feature
which helps to differentiate from cavernous
hemangiomas
• Bony destruction may be seen
• Angiography best shows the high vascularity of
orbital hemangiopericytomas with dilated feeding
arteries and rapid venous outflow.
MRI
On MRI, hemangiopericytomas are isointense in
T1-weighted images and heterogeneous signal in
T2-weighted images. They can be isointense to the gray
matter on T2-weighted images.
BIBLIOGRAPHY
1. Kikuchi K, Kowada M, Sageshima M. Orbital
hemangiopericytoma: CT, MR, and angiographic findings.
Comput Med Imaging Graph 1994;18(3):217-22.
2. Ruscalleda J, Feliciani M, Avila A, Castaner E, Guardia E,
de Juan M. Neuroradiological features of intracranial and
intraorbital meningeal haemangiopericytomas.
Neuroradiology 1994;36(6):440-5.
 Chapter 15 Vascular Lesions of the Orbit 213

Fig. 15.21: Recurrent hemangiopericytoma. A 30-year-old male

had lateral orbitotomy and an excision of the tumor 2 years
ago, came with recurrence. Axial postcontrast CT scan of the
orbit showing heterogeneously enhancing mass in the left lateral
orbit causing bony destruction (small arrow). Note: The well-
defined lateral orbitotomy defect

A B

Figs 15.22A and B: Recurrent hemangiopericytoma: (A) Axial plain CT scan and (B) Contrast-enhanced CT scan of the orbit
shows well-defined right medial orbital mass lesion with nonenhancing central area of necrosis within. Lateral orbitotomy bony
defect is noted (dotted arrow)
214 Section 3 Disorders of Orbit

A B

Figs 15.23A to C: (A) Axial T1-weighted image, (B) Axial T2-weighted image and (C) Coronal T2-weighted image show a fairly
well-circumscribed left intraconal mass lesion displaying central necrosis with thick irregular wall. HPE—hemangiopericytoma
 Chapter 15 Vascular Lesions of the Orbit 215

ORBITAL LEIOMYOMA
• This is a rare benign smooth muscle tumor arising from
the walls of the orbital vessels, and can occur in any
orbital space. Commonly, manifest with proptosis.
• Further symptoms could be due to compressive effects
of the tumor.
• Pain and increase in proptosis with Valsalva
maneuver are rarely associated.
Differential diagnoses include cavernous heman-
gioma and schwannoma.
BIBLIOGRAPHY
1. Gunduz K, Gunalp I, Erden E, Erekul S. Orbital leiomyoma:
Report of a case and review of the literature. Surv
Ophthalmol 2004;49(2):237-42.

DIAGNOSTIC TECHNIQUES

CT Scan
• Shows a well-defined mass
• Intralesional calcification may be seen in long-
standing cases
216 Section 3 Disorders of Orbit

A B

Figs 15.24A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing a well-defined lobulated extraconal mass in
the right superotemporal region. HPE—leiomyoma
Chapter 16
Pediatric Malignant Orbital Tumors
RHABDOMYOSARCOMA
Rhabdomyosarcoma is the most common pediatric
malignant neoplasm. Six percent of all childhood
malignancies are soft tissue sarcomas. Thirty-to-Forty
percent arise in the head and neck. The orbit may be the
primary site (10%) or may be involved secondarily from
the surrounding structures such as paranasal sinuses,
nasopharynx, pterygopalatine fossa and infratemporal
fossa or may be a site of metastases.
HISTOLOGICAL TYPES
Rhabdomyosarcoma arises from the pleuripotent
mesenchymal cells. There are four histological types seen:
1. Embryonal—most common orbital rhabdomyosar-
coma in children
2. Alveolar
3. Pleomorphic—rare in children and extremely rare in
the orbit
4. Botryoid.
CLINICAL FEATURES
• Mostly occur in the first decade with mean age of
presentation of 6-8 years, but can occur in the older
age group
• Male to female ratio is 5:3
• Child often presents with unilateral rapid progressive
proptosis, pain may or may not be present
• Common sites for metastases are lung, bone marrow,
lymph nodes and brain.
IMAGING FEATURES
• Rhabdomyosarcomas are locally aggressive tumors
and frequently invade the adjacent soft tissues and
bones
• They are usually extraconal, but are usually large at
the time of presentation hence may be both intra- and
extraconal
• Location—embryonal type is mostly seen in the
superior orbit and alveolar type is seen in the inferior
orbit
• Bony erosion, paranasal sinus involvement and
intracranial spread may be seen at the time of
presentation
• MRI with gadolinium is preferred modality as the
tumor extent especially intracranial and extraorbital
spread can be better delineated
• They are usually homogeneous solid lesions
isodense/isointense to the muscles on CT and MRI
(T1-weighted image), with well-defined margins
when small and irregular when large. On a T2-
weighted sequence, the signal may vary depending
on the presence or absence of hemorrhage and
necrosis
• Necrosis and hemorrhage within the lesions are better
seen on MRI. Area of necrosis will appear as T1
hypointense and T2 hyperintense signal and subacute
hemorrhage will appear hyperintense on both T1 and
T2-weighted images
• Bony erosion is better appreciated on CT than on MRI
• On postcontrast study, moderate homogeneous or
heterogeneous enhancement may be noted with areas
of necrosis showing lack of contrast enhancement.
DIFFERENTIAL DIAGNOSIS
• Orbital cellulitis with abscess presents with similar
clinical presentation as a rhabdomyosarcoma. A
contrast-enhanced MRI and diffusion weighted images
218 Section 3 Disorders of Orbit

A B

Figs 16.1A and B: (A) External photograph showing gross proptosis and conjunctival prolapse of the left eye and (B) Axial CT
scan of the orbit of the same patient showing a large heterogeneous mass in the left orbit indenting the globe and causing bony
erosion with a large anterior extraorbital extension

A B C

Figs 16.2A to C: An eight-year-old boy presented with corneal ulcer in the right eye and headache: (A) Axial postcontrast T1-
weighted image, (B) Coronal postcontrast T1-weighted image and (C) Sagittal postcontrast T1-weighted image showing a large
heterogeneous enhancing mass lesion in the right temporal and infratemporal fossa causing bony destruction and extension into
the orbit and cavernous sinus—extraorbital rhabdomyosarcoma with orbital and intracranial extension

Fig. 16.3: Axial CT scan of a 2-year-old child showing a large ill-defined mass
lesion with areas of necrosis appearing hypodense (small arrow), occupying the
entire orbit. Anterior extraorbital extension is seen with bony destruction and
extension into the temporal epidural space (long arrow)

may help in differentiating the two. Presence of bony
erosion and mass like contrast enhancing soft tissue in
the paranasal sinuses favors rhabdomyosarcoma
• Capillary hemangioma: Usually presents at birth.
MRI is helpful in differentiating capillary
hemangioma from rhabdomyosarcoma
• Leukemia and lymphoma: Granulocytic sarcoma
(chloroma) is seen in younger patients with myelo-
genous leukemia. Non-Hodgkin lymphoma is seen
in older age group. Bilateral presentation will help to
differentiate between chloroma and rhabdomyosar-
coma
• Langerhans cell histiocytosis may also present with
rapid proptosis with bony destruction. The pattern
of bony destruction is “punched out” lytic lesion
Chapter 16 Pediatric Malignant Orbital Tumors 219
against moth-eaten or permeative pattern seen in
rhabdomyosarcoma
• Neuroblastoma metastases: Bilateral involvement
with a spiculated bone destruction is characteristic of
neuroblastoma. Presence of sutural calvarial
metastases may also help in differentiation.
BIBLIOGRAPHY
1. Mafee MF, Pai E, Philip B. Rhabdomyosarcoma of the
orbit: Evaluation with MR imaging and CT. Radiol Clin
North Am 1998;36(6):1215-27, xii.
2. Shields JA, Shields CL. Rhabdomyosarcoma: Review for
the ophthalmologist. Surv Ophthalmol 2003;48(1):39-57.
3. Sohaib SA, Moseley I, Wright JE. Orbital rhabdomyosar-
coma: The radiological characteristics. Clin Radiol 1998;
53(5):357-62.
220 Section 3 Disorders of Orbit

A B

Figs 16.4A and B: A two-year-old child presented with rapid onset proptosis of the left eye:
(A) Coronal CT scan and (B) Sagittal MRI scan of the orbit showing a fairly well-marginated
lobulated homogeneous solid mass lesion in the left inferior orbit extending into the inferior
orbital fissure and pterygopalatine fossa (arrow)

A B

C D

Figs 16.5A to D: A 1-year-old child with recent rapid increase in proptosis: (A) Axial CT scan
of the orbit taken at 1 month of age showing an ill-defined hypodense lesion in the intra- and
extraconal space of the left orbit, (B) Axial T1-weighted image, (C) Coronal T2-weighted image
and (D) Post-contrast fat suppressed T1-weighted image of the orbit taken 8 months later
showing a T1 hypointense and T2 heterogeneous lesion occupying the left orbit with marked
contrast enhancement and significant increase in the size of the lesion with intracranial extension
into the cavernous sinus. Note: The expansion and erosion of the lateral wall of the orbit.
HPE—rhabdomyosarcoma

METASTATIC NEUROBLASTOMA
• Neuroblastoma is a malignant tumor of undifferen-
tiated embryonic neural crest cells and can arise from
any site along the sympathetic nervous system,
including the adrenal gland, paraspinal ganglion,
thorax and pelvic region
• This is the most common tumor of infancy and is the
second most common orbital tumor in children after
rhabdomyosarcoma
• Most children have a previous diagnosis of neuro-
blastoma, although 8 percent of patients present
initially with orbital disease
• Twenty percent of patients with neuroblastoma have
orbital metastases at some point in their disease,
usually 3 months after the initial diagnosis
• Bilateral orbital disease is present in 50 percent of
cases. Most orbital metastases occur from the
abdomen
• Proptosis and eyelid ecchymosis due to hemorrhagic
necrosis of the tumors are common presenting
features.
DIAGNOSTIC TECHNIQUES
CT Scan
• Metastatic neuroblastomas are partially encapsulated
hemorrhagic soft tissue masses, with heterogeneous
appearance corresponding to mixed areas of viable
tumor and hemorrhagic necrosis
Chapter 16 Pediatric Malignant Orbital Tumors 221
• Areas of calcification are seen within the tumor
• Lytic bony erosions occur mainly at sutural sites with
a spiculated periosteal reaction. The zygomatic and
temporal bones are most typically involved.
Differential Diagnosis
• Ewing’s sarcoma, Wilms’ tumor and medullo-
blastomas are the other malignant tumors that
metastasize in slightly older children
• In these cases, orbital soft tissue mass with lucent areas
due to necrosis are seen
• Bone destruction, especially involving the sphenoid
bone, can occur.
BIBLIOGRAPHY
1. Grover SB, Pati NK, Saluja S, Bhowmik KT. Solitary
calvarial metastases: An unusual presentation of thoracic
neuroblastoma. Indian J Cancer 2003;40(3):120-2.
2. Rothenberg AB, Berdon WE, D’Angio GJ, Yamashiro DJ,
Cowles RA. Neuroblastoma-remembering the three
physicians who described it a century ago. James Homer
Wright, William Pepper, and Robert Hutchison. Pediatr
Radiol 2009;39(2):155-60.
3. Sterker I, Frerich B. Orbital diseases in childhood. Klin
Monatsbl Augenheilkd 2006;223(1):59-67.
4. Ahmed S, Goel S, Khandwala M, Agrawal A, Chang B,
Simmons IG. Neuroblastoma with orbital metastasis:
ophthalmic presentation and role of ophthalmologists. Eye
2006;20(4):466-70.
222 Section 3 Disorders of Orbit

Fig. 16.6: External photograph showing bilateral eyelid

ecchymosis and subconjunctival hemorrhage in a case of
neuroblastoma

Fig. 16.7: Axial CT scan of the orbit showing a large right orbital
mass with clumps of calcification. Sclerosis of the lateral walls
with spiculated margins (arrows)

Fig. 16.8: Coronal CT scan of the orbits showing an extraconal

mass lesion in the left superior orbit with bony sclerosis and
spiculated appearance involving the roof of the orbit (arrow)
suggestive of metastatic neuroblastoma
 Chapter 16 Pediatric Malignant Orbital Tumors 223

A B

Figs 16.9A to C: (A) Axial CT scan of the orbits

showing an extraconal mass lesion in the right
superior orbit with spiculated bony sclerosis
(arrow), (B) Plain CT scan of the brain showing
bilateral epidural hyperdense soft tissue
(arrows) suggestive of epidural metastases
and (C) Axial CT scan of the abdomen of the
same patient showing a right adrenal mass
with calcification crossing the midline (arrow)
C suggestive of neuroblastoma
224 Section 3 Disorders of Orbit

EWING’S SARCOMA
• It is a rare, highly malignant small round cell • It typically shows a moth eaten or mottled bone
neoplasm of the bone destruction
• It is a primitive neuroectodermal tumor and arises • Onion skin-like periosteal reaction can be seen at
from neuroectodermal cells of the hematopoietic, times, especially in long bones.
vascular, and neural tissues and invades the osseous
tissue (bone) secondarily Magnetic Resonance Imaging
• It comprises 5% of all bone tumors and commonly
• It shows marked heterogeneous signal intensity with
arises from the long bones
areas of hemorrhage and necrosis
• It occurs more commonly in children
• MRI can demonstrate the exact extent of tumor,
• In the head and neck region, Ewing’s sarcoma
therefore, helpful in planning surgery.
typically involves the maxilla, mandible, and skull.
CT is the preferred modality as the pattern of bone
Orbital bone involvement is rare
destruction characteristic of Ewing’s sarcoma is
• There are only 15 reported cases of primary Ewing’s
appreciated in CT bone window settings.
sarcoma of the orbit in the literature
• Primary orbital Ewing’s sarcoma generally present
BIBLIOGRAPHY
with proptosis, visual loss, and retro-orbital pain
• Reciprocal translocation between chromosomes 11 1. Bhatoe HS, Deshpande GU. Primary cranial Ewing's
and 22 is commonly associated with this tumor sarcoma. Br J Neurosurg. 1998;12(2):165-9.
• It is an exquisitely chemosensitive tumor. 2. Kano T, Sasaki A, Tomizawa S, Shibasaki T, Tamura M,
Ohye C. Primary Ewing’s sarcoma of the orbit: Case report.
Brain Tumor Pathol 2009;26(2):95-100.
IMAGING

CT Findings
• Orbital Ewing’s sarcoma appears as an isodense and
well-circumscribed lesion arising from the bone with
heterogeneous enhancement
 Chapter 16 Pediatric Malignant Orbital Tumors 225

A B

Figs 16.10A to C: An 11-year-old female presented with rapid

proptosis of the left eye: (A and B) Axial CT scan of the orbit
in soft tissue and bone window setting respectively and
(C) Coronal CT scan showing lytic lesion in the lateral wall of
the left orbit with irregular periosteal reaction and surroun-
C ding soft tissue. HPE—Ewing’s sarcoma
Chapter 17
Lymphoproliferative Disorders
LYMPHOPROLIFERATIVE DISORDERS
• Lymphoproliferative lesions are a group of reactive
or neoplastic lymphoid proliferations. As the orbit
lacks a native lymphoid system and lymph nodes,
lymphoid processes of the orbit are essentially
extranodal and pathologic in nature
• Lymphoproliferative disorders of the orbit span the
spectrum from reactive lymphoid hyperplasia to
malignant lymphoma. Orbital and adnexal lymphoid
tumors are classified as:
1. Reactive lymphoid hyperplasia
2. Atypical lymphoid hyperplasia
3. Malignant lymphoma
• The distinction between benign and malignant lesions
is made on histopathology.
 Chapter 17 Lymphoproliferative Disorders 227

A B

Figs 17.1A and B: (A) Axial T2-weighted image and (B) Axial T1-weighted image. An ill-defined soft tissue lesion in the left
retrobulbar intra- and extraconal space. The lateral rectus is infiltrated and thickened by the lesion. Extension is seen into the
superior orbital fissure and cavernous sinus. The lesion is isointense in T1 and minimally hyperintense in T2-weighted image with
respect to the extraocular muscles. HPE—reactive lymphoid hyperplasia

A B

Figs 17.2A and B: (A) Axial T2-weighted image and (B) Sagittal T2-weighted image showing bilateral gross asymmetrical
thickening of the extraocular muscles with irregular contour and infiltration of the retrobulbar fat. HPE and immunohistochemistry—
confirmed reactive lymphoid hyperplasia
228 Section 3 Disorders of Orbit
REACTIVE LYMPHOID HYPERPLASIA
• This benign disease has an indolent course. The lesions
are usually firm painless nodular anterior orbital
masses. The orbital involvement may be part of a
multisystem disease.
DIAGNOSTIC TECHNIQUES
• They mimic other lymphoproliferative disorders on
imaging
• The lesion is more heterogeneous
• The margins of the lesions are more infiltrative
• Bone destruction is not a feature.
BIBLIOGRAPHY
1. Nechesniuk SIu, Probatova NA, Grishina EE, Kovrigina
AM, Tupitsyn NN, Sholokhova EN. Lymphoproliferative
diseases of the orbit and appendages of the eye. Arkh Patol
2001;63(1):27-32.
2. Valvassori GE, Sabnis SS, Mafee RF, Brown MS,
Putterman A. Imaging of orbital lymphoproliferative
disorders. Radiol Clin North Am 1999; 37(1):135-50, x-xi.
3. Westacott S, Garner A, Moseley IF, Wright JE. Orbital
lymphoma versus reactive lymphoid hyperplasia: An
analysis of the use of computed tomography in differential
diagnosis. Br J Ophthalmol 1991;75(12):722-5.
 Chapter 17 Lymphoproliferative Disorders 229

A B

Figs 17.3A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing a fairly well-defined superotemporal mass in
the left orbit enveloping the lateral ocular surface. HPE—lymphoma

A B

Figs 17.4A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing a well-marginated heterogeneous mass lesion
in the right lateral orbit. HPE—confirmed lymphoma
230 Section 3 Disorders of Orbit
ORBITAL LYMPHOMA
• Lymphomas account for about 55 percent of the orbital
malignant tumors and orbital lymphoma constitutes
about 1 percent of the non-Hodgkin’s lymphoma
• Most orbital and adnexal lymphomas are low grade
malignant lymphomas arising from mucosa-associated
lymphoid tissue (MALT)
• Orbital involvement may be the only manifestation
of the disease. The diagnosis of orbital disease of any
type mandates a complete work-up for systemic
lymphoma
• We would be primarily discussing Non-Hodgkin’s
lymphoma because of their frequent involvement of
the orbit. It usually presents later in life (50–70 years)
and causes symptoms due to gradually increasing
mass effect
• Proptosis and visible conjunctival mass appearing as
a smooth pink “salmon patch” are the common modes
of presentation
• Pain, headache, erythema and visual loss are not a
characteristic feature but may be seen in high grade
lymphomas. Lacrimal gland, superior and anterior
portion of the orbit, conjunctiva and eyelids are
frequent sites.
DIAGNOSTIC TECHNIQUES
CT Scan Orbits
• They are usually unilateral but can be bilateral. They
can be extraconal or intraconal.
• The lesions are usually well defined, lobulated or
nodular, homogeneous and not encapsulated hence
tends to mold around the adjacent ocular structures
• Four patterns are usually seen—retro-ocular, anterior
preseptal, lacrimal gland involvement and extension
of an adnexal lesion
• Lacrimal gland involvement is diffuse and involves
both the palpebral and orbital lobes (Differential diag-
nosis: Pleomorphic adenoma involves the orbital lobe)
• Lacrimal sac and extraocular muscles can also be
involved
• Calcification is rarely seen, may be seen post-
treatment
• Heterogeneous lesions with bony destruction and
pain are indicative of high-grade lymphomas.
MR Imaging
• The lesion is usually isointense to hyperintense to the
muscles on T1-weighted image and slightly
hyperintense on T2-weighted images
• High grade lymphomas are hypointense on T2-
weighted images
• Moderate enhancement is seen on postgadolinium T1-
weighted images. This is the indicative of high cellularity.
• Restricted diffusion is an seen due to high tumor
cellularity with low ADC values (less than 0.1 × 10–3).
Differential Diagnosis
Idiopathic orbital inflammation—they usually present
with with acute onset and pain. CT density is nonspecific
for both pseudotumor and lymphoma. On MRI, chronic
pseudotumors are isointense to the extraocular muscles
on both T1- and T2-weighted images due to a more fibrosis.
Recommended Imaging Modality
• CT scan both plain and contrast is sensitive but lacks
specificity
• MRI plain and contrast is recommended as it can
characterize the lesion and therefore more specific.
BIBLIOGRAPHY
1. Akansel G, Hendrix L, Erickson BA, Demirci A, Papke A,
Arslan A, Ciftci E. MRI patterns in orbital malignant
lymphoma and atypical lymphocytic infiltrates. Eur J
Radiol 2005;53(2):175-81.
2. Flanders AE, Espinosa GA, Markiewicz DA, Howell DD.
Orbital lymphoma: Role of CT and MRI. Radiol Clin North
Am 1987;25(3):601-13.
3. Sepahdari AR, Aakalu VK, Setabutr P, Shiehmorteza M,
Naheedy JH, Mafee MF. Indeterminate orbital masses:
Restricted diffusion at MR imaging with echoplanar
diffusion-weighted imaging predicts malignancy. Radiol
2010;256(2):554-64.
4. Valvassori GE, Sabnis SS, Mafee RF, Brown MS,
Putterman A. Imaging of orbital lymphoproliferative
disorders. Radiol Clin North Am 1999; 37(1):135-50, x-xi.
5. Weber AL, Jakobiec FA, Sabates NR. Lymphoproliferative
disease of the orbit. Neuroimaging Clin N Am 1996;6(1):93-
111.
6. Yeo JH, Jakobiec FA, Abbott GF, Trokel SL. Combined
clinical and computed tomographic diagnosis of orbital
lymphoid tumors. Am J Ophthalmol 1982;94(2):235-45.
 Chapter 17 Lymphoproliferative Disorders 231

A B

Figs 17.5A and B: (A and B) External photograph of the left eye showing a subconjunctival salmon-pink patch characteristic of
lymphomatous infiltration

Fig. 17.6: Axial CT scan of the orbits showing well-defined

homogeneous mass enveloping the right lateral ocular surface
with posterior extension and typical molding of the lateral ocular
surface (arrow)

Fig. 17.7: Axial CT scan of the orbits showing bilateral disease

with involvement of the medial rectus and optic nerve on the
right and typical molding of the posterior ocular surface on the
left. Note: The left eye was clinically normal
232 Section 3 Disorders of Orbit

Fig. 17.8: Axial CT scan of the orbit showing a well-circumscribed

nodular pre- and postseptal lesion in the left medial orbit

Fig. 17.9: Coronal CT scan of the orbit showing diffuse

enlargement of the right lacrimal gland. HPE—confirmed
lymphoma

A B

Figs 17.10A and B: A 35-year-old patient presented with rapid onset painful proptosis of the right eye: (A) Axial contrast-
enhanced CT scan and (B) Coronal contrast-enhanced CT scan of the orbit showing lobulated heterogeneous mass in the
superotemporal quadrant of the right orbit with erosion of the adjacent roof of the orbit (arrow). HPE—high-grade lymphoma
 Chapter 17 Lymphoproliferative Disorders 233

A B

C D

Figs 17.11A to D: A 44-year-old female presented with painful proptosis of the right eye: (A) Axial T1-weighted image, (B) Axial
T2-weighted image and (C and D) Axial postgadolinium fat suppressed images showing diffuse infiltrating lesion in the right
retrobulbar fat displaying isointense signal in both T1- and T2-weighted images (with respect to the extraocular muscles) with
homogeneous contrast enhancement. HPE—high-grade lymphoma
234 Section 3 Disorders of Orbit

A B

Figs 17.12A to C: A 52-year-old female with painless proptosis of left eye for 7 years: (A) Axial postcontrast CT, (B) Axial T1-
weighted image and (C) Axial T2-weighted image. The CT scan shows a well-defined homogeneous extraconal lesion in the left
medial orbit molding the globe and displacing it laterally. Axial MRI shows a slightly hyperintense mass in T1-weighted image and
homogeneously hypointense in T2-weighted image
 Chapter 17 Lymphoproliferative Disorders 235

A B

Figs 17.13A to C: A 48-year-old female presented with gradual onset proptosis of the right eye: (A) Axial T1-weighted image,
(B) Coronal T2-fat suppressed image and (C) Axial diffusion weighted image showing an ill-defined predominantly extraconal
lesion in the inferolateral aspect of the right orbit. It displays isointense signal in T1-weighted image and intermediate signal in T2-
weighted image with respect to the extraocular muscles. Restricted diffusion noted within the lesion (white arrow)
236 Section 3 Disorders of Orbit

A B

Figs 17.14A to C: (A) Axial CT scan orbit showing

diffuse perioptic lesion along the left optic nerve. Note
ill-defined soft tissue infiltrating the medial fat (arrow),
(B) T1-weighted image showing diffuse perioptic
enhancing lesion along the left optic nerve, an ill-defined
enhancing soft tissue is also seen in the nasal
periocular fat on both sides and (C) Coronal T2-
weighted fat suppressed image showing a homo-
geneous slightly hyperintense perioptic lesion with optic
C nerve (dotted arrow) seen separately

Fig. 17.15: Medial canthal mass with CT. Dynamic cardiogram

(DCG) shows an ill-defined medial canthal mass in the right
orbit (long arrow). The lacrimal sac (short arrow) and canaliculi
are seen separately from the lesion. HPE—lymphoma

LEUKEMIA
• Leukemia is a disease of the bone marrow and
represents a common form of childhood malignancy
• Acute lymphoblastic leukemia (ALL) is more common
in children while acute myeloid leukemia (AML) is
more common in adults. Chronic lymphatic leukemia
(CLL) is a disease of adulthood
• Chronic myelogenous leukemia may occur in children
and has a worse prognosis than CLL. The eye and
adnexae are usually involved in advanced stages of
leukemia. It occurs in children with AML and adults
with CLL. They are an important differential diagnosis
in cases of childhood proptosis
• The onset is usually sudden, with proptosis due to
leukemic soft tissue infiltration (chloroma or
granulocytic sarcoma) or hemorrhage
• Bilateral orbital involvement may be seen. The
orbital manifestations may precede the blood and
bone marrow findings of AML or develop after the
diagnosis.
DIAGNOSTIC TECHNIQUES
• Every tissue of the eye and adnexae can be affected
by leukemic infiltration.
Within the orbit, the fat, lacrimal gland and
extraocular muscles can be involved.
Chapter 17 Lymphoproliferative Disorders 237
CT Scan
• A well-circumscribed enhancing nodular mass may
be seen
• Alternatively, diffuse infiltration of the extraconal or
intraconal spaces can occur
• Subperiosteal space can be involved, with the lateral
wall being more frequently affected
• Bone erosion can occur (Fig. 17.1 B).
MR Imaging
• Leukemic infiltration appears as an area of medium
to low signal which is brighter than vitreous and
isointense to muscles on T1 sequence, with mild
hyperintense signal on T2-weighted images.
BIBLIOGRAPHY
1. Bidar M, Wilson MW, Laquis SJ, Wilson TD, Fleming JC,
Wesley RE, Ribeiro RC, Haik BG. Clinical and imaging
characteristics of orbital leukemic tumors. Ophthal Plast
Reconstr Surg 2007;23(2):87-93.
2. Pui MH, Fletcher BD, Langston JW. Granulocytic sarcoma
in childhood leukemia: Imaging features. Radiology
1994;190(3):698-702.
3. Valvassori GE, Sabnis SS, Mafee RF, Brown MS,
Putterman A. Imaging of orbital lymphoproliferative
disorders. Radiol Clin North Am 1999;37(1):135-50, x-xi.
238 Section 3 Disorders of Orbit

A B

Figs 17.16A and B: A 5-year-old child with painful swelling of one and a half months duration: (A) Axial postcontrast CT scan and
(B) Coronal bone window showing a well-defined mass lesion in the region of the right lacrimal gland with subtle bony erosion
(arrows). HPE—chloroma

A B

Figs 17.17A and B: A 12-year-old female with fever, joint pains and rapid onset proptosis of right eye. (A) Axial postcontrast CT
scan and (B) Coronal postcontrast CT scan of the orbit showing a large irregular heterogeneous mass in the right medial orbit—
chloroma
 Chapter 17 Lymphoproliferative Disorders 239

A B

Figs 17.18A to C: A 5-year-old child with bilateral proptosis, fever and severe anemia: (A) Axial T2-weighted image, (B) Coronal
T1 postcontrast fat suppressed of the orbit, showing lobulated enhancing solid lesions in the extraconal space of the orbits
bilaterally (small white arrows), involvement of the paranasal sinuses and altered marrow of the orbital walls and (C) Axial
postcontrast T1-weighted image of the brain shows an enhancing epidural lesion in the right occipital region (arrow). Peripheral
smear confirmed leukemia
240 Section 3 Disorders of Orbit
PLASMACYTOMA
• These are tumors composed of pure plasma cells. The
plasma cell is actually a B-lymphocyte that has become
modulated to produce large quantities of immuno-
globulin (antibody)
• An important tumor of the plasma cell is multiple
myeloma, a multifocal neoplasm that involves bone
marrow
• However, there are solitary forms of extramedullary
plasmacytomas which are not associated with
systemic myeloma. Any of these solitary or multifocal
variants can occur in the orbit
• Plasmacytoma can be osseous or nonosseous
(extramedullary)
• Age of presentation—above 40 years
• Within bony orbit, there is usually a lytic destructive
process with no sclerotic reaction. These isolated
plasmacytomas are commonly located in the roof and
lateral wall of orbit with extension into the frontal
sinus, frontal bone and orbital cavity
• If plasma cell tumor is diagnosed-on an orbital biopsy,
a complete physical examination, bone scan/PET-CT,
bone marrow biopsy, serum protein electrophoresis
and immunoelectrophoresis of serum and urine must
be done to rule out systemic disease
• Orbit can be involved primarily or from the adnexal
region especially paranasal site, as it is a common site
for extramedullary plasmacytoma.
IMAGING
• Plasmacytomas can cause focal lytic lesion and
expansion of the bone with soft tissue components
• On MRI, they are isointense on T1-weighted images
and moderate signal intensity on T2-weighted images.
There is significant contrast enhancement with central
inhomogeneity.
DIFFERENTIAL DIAGNOSIS—METASTASES
The soft tissue lesions are usually homogeneous and solid.
Necrosis and calcifications is not a feature of plasmacytomas.
BIBLIOGRAPHY
1. Aboud N, Sullivan T, Whitehead K. Primary extramedullary
plasmacytoma of the orbit. Aust NZJ Ophthalmol 1995;23(3):
235-9.
2. Adkins JW, Shields JA, Shields CL, Eagle RC Jr, Flanagan
JC, Campanella PC. Plasmacytoma of the eye and orbit.
Int Ophthalmol 1996-97;20(6):339-43. Review.
3. de Smet MD, Rootman J. Orbital manifestations of plasma-
cytic lymphoproliferations. Ophthalmol 1987;94(8):995-1003.
4. Gonnering RS. Bilateral primary extramedullary orbital
plasmacytomas. Ophthalmol 1987;94(3):267-70.
5. Hamburger HA. Orbital involvement in multiple
myeloma: A new angiographic presentation. J Clin Neuro-
ophthalmol 1984;4(1):25-9.
6. Sen S, Kashyap S, Betharia S. Primary orbital
plasmacytoma: A case report. Orbit 2003;22(4):317-9.
7. Uceda-Montanes A, Blanco G, Saornil MA, Gonzalez C,
Sarasa JL, Cuevas J. Extramedullary plasmacytoma of the
orbit. Acta Ophthalmol Scand 2000;78(5):601-3.
 Chapter 17 Lymphoproliferative Disorders 241

A B

Figs 17.19A to C: (A) Axial CT scan, (B) Coronal CT scan of the orbits showing a lytic lesion in the roof of the right orbit with
surrounding soft tissue in the superior extraconal and (C) Frontal epidural space (dotted arrows)—orbital biopsy conformed
plasmacytoma
Chapter 18
LANGERHANS CELL HISTIOCYTOSIS
(HISTIOCYTOSIS X)
• Langerhans cell histiocytosis is a group of clinical
conditions characterized by proliferation of
Langerhans cells, a histiocyte normally found in the
epidermis and recognized ultrastructurally by tennis
racket-shaped granules known as Birbeck granules
• This term includes eosinophilic granuloma, Hand-
Schüller-Christian disease, and Letterer-Siwe disease. All
these entities have very similar histopathologic features
although clinical manifestations vary and often overlap
• Orbital involvement may vary but the most common
orbital manifestation is a solitary osseous lesion
namely the solitary unifocal eosinophilic granuloma
• Orbital involvement in the more extensive Hand-
Schüller-Christian and Letterer-Siwe variants is less
frequent
• Most cases occur in children between birth and 20 years
of age and usually symptoms are seen before the age
of 5 years. No gender predilection is seen. Clinical
features vary with the clinical form of the disease.
Lesions in children are usually located in the bone or
bone marrow.
EOSINOPHILIC GRANULOMA
• It usually presents with the rapid onset of a painful
tender swelling near the superolateral aspect of the
orbit anteriorly. Unilateral or bilateral proptosis may
be seen
• There is erythema of overlying skin. The presence of
pain, tenderness and erythema differentiate it from a
dermoid
Histiocytic Lesions
• Frontal or zygomatic bones are usually involved.
Isolated sphenoidal involvement may occur
producing an orbital apex syndrome. Some cases
present with involvement of orbital soft tissue without
osseous involvement. Such lesions usually enlarge
and ulcerate the overlying skin.
HAND-SCHÜLLER-CHRISTIAN DISEASE
This represents a multifocal variant of eosinophilic
granuloma. The classic triad of bilateral proptosis,
diabetes insipidus and multiple punched out lesions
in the cranial bones may be seen. Many patients show
only a partial expression of this syndrome.
LETTERER-SIWE DISEASE
This is a fulminant condition characterized by
hepatosplenomegaly, lymphadenopathy and osseous
defects. Orbital involvement is rare but diffuse infiltration
of the uveal tract may be seen.
IMAGING
CT Scan
• Single or multiple osteolytic lesions may be present in
the superolateral and orbital roof regions resulting in
multiple bony defects. The intraosseous lesion has
irregular contour with punched out margins.
Peripheral zone of sclerosis is seen during the healing
phase
 Chapter 18 Histiocytic Lesions 243

A B

C D

Figs 18.1A to D: (A) Axial CT scan of the orbit, (B) Coronal CT scan of the orbit in soft tissue window settings, (C) Axial CT scan
of the brain, and (D) In bone window settings showing a soft tissue lesion in the right superior orbit with erosion of the roof of the
orbit. Figure D shows punched out osteolytic lesions involving the right frontal bone (white arrow) and right temporal bone (dotted
arrow)
244 Section 3 Disorders of Orbit
• Osseous lesions may be seen in sphenoid, temporal,
occipital and parietal as well as facial bones
• Rarely, the lesion may be totally extraosseous. A fairly
well-defined soft tissue mass with moderate-to-
marked postcontrast enhancement is seen
• There may be extension of soft tissue into the epidural
space and temporal fossa and infiltration of temporalis
muscle
• Following spontaneous healing or radiation therapy,
there may be reconstitution of orbital bony defect.
DIFFERENTIAL DIAGNOSIS
Rhabdomyosarcoma, leukemic infiltration, granulocytic
sarcoma or chloroma, metastatic neuroblastoma,
metastatic Wilm’s tumor and metastatic Ewing’s sarcoma.
BIBLIOGRAPHY
1. Banna M, Olutola PS. Orbital histiocytosis on computed
tomography. J Comput Tomogr 1983;7(2):167-70.
2. Cheung N, Selva D, McNab AA. Orbital Langerhans cell
histiocytosis in adults. Ophthalmology 2007;114(8):1569-73.
3. Hidayat AA, Mafee MF, Laver NV, Noujaim S. Langerhans'
cell histiocytosis and juvenile xanthogranuloma of the orbit:
Clinicopathologic, CT, and MR imaging features. Radiol
Clin North Am 1998;36(6):1229-40, xii. Review.
4. Jakobiec FA, Trokel SL, Aron-Rosa D, Iwamoto T, Doyon
D. Localized eosinophilic granuloma (Langerhans' cell
histiocytosis) of the orbital frontal bone. Arch Ophthalmol
1980;98(10):1814-20.
5. Kramer TR, Noecker RJ, Miller JM, Clark LC. Langerhans
cell histiocytosis with orbital involvement. Am J
Ophthalmol 1997;124(6):814-24.
6. LaBorwit SE, Karesh JW, Hirschbein MJ, Dankner SR.
Multifocal Langerhans' cell histiocytosis involving the
orbit. J Pediatr Ophthalmol Strabismus 1998;35(4):234-6.
7. Maccheron LJ, McNab AA, Elder J, Selva D, Martin FJ,
Clement CI, Sainani A, Sullivan TJ. Ocular adnexal
Langerhans cell histiocytosis clinical features and
management: Ocular adnexal Langerhans cell histiocytosis
clinical features and management. Orbit 2006;25(3):169-77.
8. Sarkar S, Singh M, Nag D, Dutta H, Banerjee A, Bhaduri G,
Jha A. A case report of unifocal Langerhans' cell histiocytosis
or eosinophilic granuloma. J Indian Med Assoc
2007;105(4):218-20.
9. Shetty SB, Mehta C. Langerhans cell histiocytosis of the
orbit. Indian J Ophthalmol 2001;49(4):267-8.
10. Stromberg JS, Wang AM, Huang TE, Vicini FA, Nowak
PA. Langerhans cell histiocytosis involving the sphenoid
sinus and superior orbital fissure. Am J Neuroradiol 1995;
16(4 Suppl):964-7.
 Chapter 18 Histiocytic Lesions 245

A B

Figs 18.2A to C: A 42-day-old infant presented with rapid onset

proptosis of the left eye: (A) Axial CT scan of the orbit, (B) Axial
T1-weighted image of the orbit and (C) Axial T2-weighted image
of the orbit shows a fairly well-defined soft tissue lesion in the
left medial orbit without any bony erosion. Differential diagnosis
of rhabdomyosarcoma and histiocytosis was made. HPE—
C histiocytosis
246 Section 3 Disorders of Orbit
JUVENILE XANTHOGRANULOMA
(NEVOXANTHOENDOTHELIOMA)
• Juvenile xanthogranuloma is an idiopathic multifocal
cutaneous eruption which usually occurs in infants,
though young children and adults can also be affected
where typical skin lesions may not be seen
• Elevated sharply demarcated papular or papulono-
dular skin lesions are seen. These may be single or
multiple, measuring 2 to 8 mm and are orange, brown
or golden in color
• The eye can be an extracutaneous site of affection. In
the eye, the iris and ciliary body are most commonly
involved. Xanthogranuloma of the eyelid skin can
occur. The retina, choroid, optic nerve and orbit are
rarely affected
• Edema or fullness of the eyelid, limitation of
extraocular movement, ptosis, localized tumor or
diffuse tumor of iris, iris heterochromia, hyphema,
uveitis and secondary glaucoma are some of the
features of ocular xanthogranuloma
• Rarely pain, lacrimation, dry eye, infection of bulbar
conjunctiva, papilledema and fever may occur.
Unilateral or bilateral proptosis or a nontender
anterior mass at birth or the first few weeks of life is
seen in cases with orbital involvement
• CNS involvement has also been described
• It is characterized histologically by a granulomatous
inflammation with histiocytes that aggregate to form
Touton giant cells, lymphocytes, plasma cells and
occasional eosinophils
• It is a benign and generally self-limited condition,
especially the skin lesions. While systemic prognosis
is excellent, visual prognosis depends on the extent
of intraocular, orbital and optic nerve involvement.
Severe cases may result in orbital sclerosis or fibrosis.
IMAGING
CT Scan
• Orbital CT shows commonly involvement of the
anterior portion of eye
• In case of orbital involvement, usually an irregular
diffuse infiltrating mass is seen that enhances with
contrast
• Marked destruction of the involved orbital bone may
occur. Bone involvement cannot be distinguished
from Langerhans cell histiocytosis radiologically.
MRI Orbit
• A soft tissue infiltrating lesion is seen that enhances
well with contrast
• The lesion is seen to be isointense to brain on T1-
weighted image and variable signal in T2-weighted
images.
DIFFERENTIAL DIAGNOSIS
Rhabdomyosarcoma, capillary hemangioma, idiopathic
orbital inflammation, teratoma, lymphangioma.
BIBLIOGRAPHY
1. Gaynes PM, Cohen GS. Juvenile xanthogranuloma of the
orbit. Am J Ophthalmol 1967;63(4):755-7.
2. Harley RD, Romayananda N, Chan GH. Juvenile
xanthogranuloma. J Pediatr Ophthalmol Strabismus
1982;19(1):33-9.
3. Hidayat AA, Mafee MF, Laver NV, Noujaim S.
Langerhans' cell histiocytosis and juvenile
xanthogranuloma of the orbit: Clinicopathologic, CT, and
MR imaging features. Radiol Clin North Am 1998;36(6):
1229-40, xii. Review.
4. Karcioglu ZA, Sharara N, Boles TL, Nasr AM. Orbital
xanthogranuloma: Clinical and morphologic features in
eight patients. Ophthal Plast Reconstr Surg 2003;19(5):372-
81.
5. Mencia-Gutierrez E, Gutierrez-Diaz E, Madero-Garcia S.
Juvenile xanthogranuloma of the orbit in an adult.
Ophthalmologica. 2000;214(6):437-40.
6. Miszkiel KA, Sohaib SA, Rose GE, Cree IA, Moseley IF.
Radiological and clinicopathological features of orbital
xanthogranuloma. Br J Ophthalmol 2000;84(3):251-8.
7. Shields CL, Shields JA, Buchanon HW. Solitary orbital
involvement with juvenile xanthogranuloma. Arch
Ophthalmol 1990;108(11):1587-9.
8. Shields JA, Shields CL. Clinical spectrum of histiocytic
tumors of the orbit. Trans Pa Acad Ophthalmol
Otolaryngol 1990;42:931-7. Review.
9. Vick VL, Wilson MW, Fleming JC, Haik BG. Orbital and
eyelid manifestations of xanthogranulomatous diseases.
Orbit 2006;25(3):221-5.
 Chapter 18 Histiocytic Lesions 247

A B

Figs 18.3A and B: An adult patient presented with proptosis and pain in the right orbit: (A) Axial CT scan and (B) Coronal CT scan
showing periocular lesion, involvement of the lacrimal gland and intralesional calcification. HPE—juvenile xanthogranulomas
Chapter 19

Lacrimal Gland Lesions

LESIONS OF THE LACRIMAL GLAND
• The lacrimal gland can be affected in various infective, INFLAMMATIONS AND INFECTIONS
inflammatory or neoplastic conditions OF THE LACRIMAL GLAND
• The lacrimal gland tumors are classified into epithelial
• Sarcoidosis
and nonepithelial lesions
• The most common epithelial neoplasms are • Idiopathic nonspecific inflammation
pleomorphic adenoma or benign mixed tumors • Tuberculosis.
• The remainings are malignant carcinomas and include
adenoid cystic carcinoma, mucoepidermoid TUMORS OF THE LACRIMAL GLAND
carcinoma and others. • Pleomorphic adenoma
• Adenoid cystic carcinoma
CONGENITAL LESIONS OF THE LACRIMAL GLAND • Ductal adenocarcinoma
Lacrimal gland aplasia. • Lymphoproliferative disorders.

CONGENITAL LESIONS OF THE LACRIMAL GLAND
LACRIMAL GLAND APLASIA
• This is a very rare condition in which the lacrimal
gland is congenitally absent
• The patient can have dry eye symptoms or congenital
alacrima. It may be associated with aplasia or
hypoplasia of the minor salivary glands or associated
limb and temporal bone abnormalities.
IMAGING
• Shows unilateral or bilateral absence of lacrimal glands
Chapter 19 Lacrimal Gland Lesions 249
• There may be associated hypoplasia of the adjacent
bone.
BIBLIOGRAPHY
1. Kim SH, Hwang S, Kweon S, Kim TK, Oh J. Two cases of
lacrimal gland agenesis in the same family: Clinicoradio-
logic findings and management. Can J Ophthalmol
2005;40(4):502-5.
2. Uleckas JK, Garel L, Milot J, Mathieu-Millaire F. Orbital
CT scan in congenital alacrima. J Pediatr Ophthalmol
Strabismus 1994;31(2):114-7.
250 Section 3 Disorders of Orbit

Figs 19.1A and B: (A) Axial CT scan of orbit and (B) Coronal
reformatted CT scan of orbit showing absent lacrimal gland on
the right side (black arrows). Normal gland is seen on the left
B side (dotted arrow)

INFLAMMATORY LACRIMAL GLAND LESIONS
PSEUDOTUMOR OF THE LACRIMAL GLAND
• Orbital pseudotumor is a nonspecific idiopathic
inflammatory condition for which no identifiable local
or systemic cause can be found
• This is a diagnosis of exclusion. Clinical picture may
include orbital signs of proptosis, diplopia, decreased
vision from optic neuropathy or choroidal folds, and
conjunctival injection
• It can cause myositis, dacryoadenitis or optic
perineuritis
• The typical presentation of acute or subacute
dacryoadenitis is with pain, proptosis, S-shaped upper
eyelid and palpable tender lacrimal gland.
DIAGNOSTIC TECHNIQUES
CT Scan
• Lacrimal gland enlargement is usually diffuse
involving the palpebral and orbital lobe with
preservation of the shape of the gland
• The margins are irregular with periglandular fat
stranding
• Contrast enhancement is a feature
• The mass is confined to the lacrimal gland fossa often
displacing the globe inferomedially. When large, it
can even indent the globe
• Bone destruction is rare
• Thickening of the adjacent extraocular muscles may
be noted.
Chapter 19 Lacrimal Gland Lesions 251
Magnetic Resonance Imaging
• The signal intensity will depend on the stage of the
disease
• In the acute phase, the gland is enlarged, usually
involving both the palpebral and orbital lobes.
T2 hyperintense signal is seen with periglandular fat
stranding. The adjacent extraocular muscles may also
be thickened. Homogeneous enhancement is noted
on the postcontrast study
• In the chronic phase, the T2 signal of the lacrimal gland
will be variable depending on the degree of fibrosis.
Densely fibrotic lesions show marked T2 hypointense
signal.
BIBLIOGRAPHY
1. Gordon LK. Orbital inflammatory disease: A diagnostic and
therapeutic challenge. Eye 2006;20(10):1196-206. Review.
2. Hsuan JD, Selva D, McNab AA, Sullivan TJ, Saeed P,
O'Donnell BA. Idiopathic sclerosing orbital inflammation.
Arch Ophthalmol 2006;124(9):1244-50.
3. Kapur R, Sepahdari AR, Mafee MF, Putterman AM,
Aakalu V, Wendel LJ, Setabutr P. MR imaging of orbital
inflammatory syndrome, orbital cellulitis, and orbital
lymphoid lesions: The role of diffusion-weighted imaging.
Am J Neuroradiol 2009;30(1):64-70.
4. Kitei D, DiMario FJ Jr. Childhood orbital pseudotumor:
Case report and literature review. J Child Neurol 2008;
23(4):425-30.
252 Section 3 Disorders of Orbit

Fig. 19.2: Axial contrast-enhanced CT scan of orbits showing

diffuse enlargement of the palpebral and orbital lobes of the left
lacrimal gland with preservation of the shape (white arrow).
Note: The edema of the adjacent eyelid (white arrow)

A B

Figs 19.3A and B: (A) Axial enhanced CT scan of orbit and (B) Coronal enhanced CT scan of orbit showing diffuse enlargement
of the right lacrimal gland with heterogeneous appearance and globe indentation in a case of acute dacryoadenitis
 Chapter 19 Lacrimal Gland Lesions 253

A B

Figs 19.4A and B: (A) Axial FLAIR image of the orbit and (B) Coronal T2-weighted image showing diffuse enlargement of the
right lacrimal gland with periglandular edema. HPE—chronic nonspecific inflammation

A B

Figs 19.5A and B: (A) Axial CT scan of the orbit and (B) Coronal CT scan of the orbit showing diffuse enlargement of the orbital
and palpebral lobe of the left lacrimal gland with significant periglandular fat stranding—idiopathic orbital inflammation
254 Section 3 Disorders of Orbit

SARCOIDOSIS
• Sarcoidosis is characterized by noncaseating granu- MR Imaging
lomatous inflammation involving multiple organ sys-
It shows unilateral or bilateral enlargement of the lacrimal
tems
gland with diffuse T2 hyperintense signal in the lacrimal
• The lacrimal gland is the most commonly affected
gland.
orbital tissue
• Lesions of sarcoidosis generally show relatively high
OTHER DIAGNOSTIC MODALITIES
Ga-67 uptake and the usefulness of scintigraphy using
this agent in the evaluation of lesion activity is well The characteristic uptake of Ga-67 has a symmetrical
known. accumulation in bilateral lacrimal and salivary glands
which resembles a Panda face (Panda sign) in patients
DIAGNOSTIC TECHNIQUES with sarcoidosis.

CT Scan BIBLIOGRAPHY
Diffuse enlargement of the lacrimal gland is seen, which 1. Sacher M, Lanzieri CF, Sobel LI, Som PM. Computed
can be unilateral or bilateral and can resemble a lympho- tomography of bilateral lacrimal gland sarcoidosis.
proliferative disorder. J Comput Assist Tomogr 1984;8(2):213-5.
 Chapter 19 Lacrimal Gland Lesions 255

A B

Figs 19.6A and B: (A) Axial T1-weighted image and (B) Coronal T2-weighted MR image showing mild prominence of the
palpebral lobe of the left lacrimal gland (dotted arrow) and diffuse soft tissue at left orbital apex and cavernous sinus (white
arrows)—sarcoidosis

A B

Figs 19.7A and B: (A) Coronal T1 postcontrast and (B) Coronal STIR-MR images showing bilateral diffuse enlargement of the
lacrimal gland (arrows) in a patient of sarcoidosis
256 Section 3 Disorders of Orbit
TUBERCULOSIS OF THE LACRIMAL GLAND
• Tuberculosis involving the orbit is rare. Spread may
be from an adjacent lesion or by hematogenous route
• Painless progressive proptosis can occur. Tuberculous
dacryoadenitis presents as a painless progressive
swelling on the lateral aspect of the upper eyelid.
IMAGING
CT Scan
• Shows enlargement of the gland which may be
homogeneous or heterogeneous
• Hypodense areas of necrosis may be seen within
• Bone destruction may be noted.
BIBLIOGRAPHY
1. Bansal RK, Malhotra C, Bhatia R, Chhabra S, Sood S.
Tubercular dacryoadenitis: A case report and review of
literature. Indian J Pathol Microbiol 2006;49(3):385-7.
2. Madge SN, Prabhakaran VC, Shome D, Kim U, Honavar S,
Selva D. Orbital tuberculosis: A review of the literature.
Orbit 2008;27(4):267-77. Review.
3. Madhukar K, Bhide M, Prasad CE, Venkatramayya.
Tuberculosis of the lacrimal gland. J Trop Med Hyg 1991;
94(3):150-1.
4. Sharma A, Pandey SK, Mohan K, Khandelwal N, Gupta A.
Tuberculosis of the lacrimal gland: A case report. J Pediatr
Ophthalmol Strabismus 1998;35(4):237-9.
 Chapter 19 Lacrimal Gland Lesions 257

Fig. 19.8: Axial CT scan of orbits showing bilateral symmetrical

enlargement of the lacrimal glands. HPE—tuberculosis

A B

Figs 19.9A and B: A 75-year-old female presented with sudden onset swelling and proptosis of the right eye with mild pain:
(A) Axial CT scan in soft tissue window showing a well-marginated lesion in the lacrimal fossa showing central hypodense
necrosis (arrow) and (B) Axial CT in bone window setting showing area of osteolysis in the bone (arrow)
258 Section 3 Disorders of Orbit

Figs 19.10A and B: (A) Coronal T2 FRFSE image of a patient

showing central necosis and thick isointense wall extending into
the frontal epidural space and temporal fossa and (B) Axial
diffusion-weighted image showing restricted diffusion in the
lesion—differential diagnosis of tuberculosis and metastases
B was considered. HPE—tuberculosis of the lacrimal gland

LACRIMAL GLAND NEOPLASMS
PLEOMORPHIC ADENOMA
• This is a benign pseudoencapsulated lesion of the
lacrimal gland consisting of two cell types derived
from the epithelial and mesenchymal components
(mixed tumor)
• The tumor produces symptoms due to mass effect
with long-standing history of painless proptosis
• Pain and sudden increase in size is indicative of a
malignant transformation. Malignant transformation
of pleomorphic adenoma (malignant mixed tumor)
is seen in 4 to 24 percent of cases.
DIAGNOSTIC TECHNIQUES
CT Scan
• They are usually well-defined heterogeneous lesions
with cystic spaces, rounded contour and involve
mainly the orbital lobe
• A smooth contour implies encapsulation
• Smooth bony remodeling and fossa formation is noted
in long-standing cases
• Bony erosion is not a feature and if present indicates
malignant transformation
• Calcification is also not a common feature of
pleomorphic adenomas but may be seen in adenoid
cystic carcinomas.
Chapter 19 Lacrimal Gland Lesions 259
MR Imaging
• The tumor shows long T1 and T2 signal characteristics,
and may be heterogeneous on T2-weighted images
• Moderate-to-marked enhancement is seen on MR and
CT images.
Recommendation
CT scan of the orbit, both plain and contrast, is usually
preferred. MRI is needed, if diagnosis is in doubt.
BIBLIOGRAPHY
1. Font RL, Patipa M, Rosenbaum PS, Smith S, Berg L.
Correlation of computed tomographic and histopathologic
features in malignant transformation of benign mixed tumor
of lacrimal gland. Surv Ophthalmol 1990;34(6):449-52.
2. Gunduz K, Shields CL, Gunalp I, Shields JA. Magnetic
resonance imaging of unilateral lacrimal gland lesions.
Graefes Arch Clin Exp Ophthalmol 2003;241(11):907-13.
Epub 2003 Oct 25.
3. Jakobiec FA, Yeo JH, Trokel SL, Abbott GF, Anderson R,
Citrin CM, Alper MG. Combined clinical and computed
tomographic diagnosis of primary lacrimal fossa lesions.
Am J Ophthalmol 1982;94(6):785-807.
4. Mafee MF, Haik BG. Lacrimal gland and fossa lesions:
Role of computed tomography. Radiol Clin North Am
1987;25(4):767-79.
260 Section 3 Disorders of Orbit

Figs 19.11A and B: Pleomorphic adenoma: (A) Axial CT

scan and (B) Coronal CT scan of orbit showing a well-
defined, lobulated mass lesion in the superotemporal
quadrant of the right orbit with areas of necrosis, smooth
B bony remodeling (black arrow) and calcification (white arrow)
 Chapter 19 Lacrimal Gland Lesions 261

Figs 19.12A and B: (A) Axial CT scan and (B) Coronal CT

scan of orbit showing a rounded well-circumscribed hetero-
geneous mass lesion in the region of the left lacrimal gland
B (arrow) with smooth bony remodeling—pleomorphic adenoma
262 Section 3 Disorders of Orbit

Fig. 19.13: A 65-year-old male with proptosis of the right eye

since one year and associated pain since 15 days. Clinically,
gross proptosis with corneal abscess was seen. Coronal CT
scan of orbit showed a large well-defined mass in the lacrimal
gland region with bony erosion of the right orbital roof (arrow).
HPE—malignant mixed lacrimal gland tumor

A B

Figs 19.14A and B: A 65-year-old male with painless gradual progressive proptosis of left eye since 2 years: (A) Axial CT scan
and (B) Coronal CT scan of the orbit showed a large mass in the left orbit with intralesional calcification and bony erosion of the
orbital roof. Note: Grossly phthisical left eye (arrows). HPE—squamous cell differentiation within a pleomorphic adenoma

ADENOID CYSTIC CARCINOMA
• It is the most common malignant epithelial neoplasm
of the lacrimal gland
• There is a propensity for rapid growth with perineural
and vascular invasion. Patients in the younger age
groups are more commonly affected
• Patients present with proptosis and swelling in the
superotemporal anterior orbit. Pain may be the only
symptom suggesting a malignancy
• This is an aggressive tumor with a high-risk of local
recurrence. Late distant spread of the tumor to the
lungs and rarely to the liver has been reported.
DIAGNOSTIC TECHNIQUES
On CT Imaging
• Adenoid cystic carcinomas are well-defined lesions
and at times can simulate pleomorphic adenoma
• They may be very heterogeneous with areas of
necrosis within
• Nodularity and infiltrative nature on imaging are
more diagnostic of carcinoma
• Calcification may be seen within the tumor substance
• A large mass can indent the globe
Chapter 19 Lacrimal Gland Lesions 263
• Lytic destruction of adjacent bone suggests malignant
process
• Malignant lacrimal gland tumors can also cause bony
sclerosis
• Postcontrast CT should be done as the intracranial
spread is best seen on contrast-enhanced CT scan.
MR Imaging
• On MRI, these tumors display low signal intensity on
T1-weighted images and intermediate to high signal
on T2-weighted images
• It is less sensitive to bony erosion, but will show subtle
marrow infiltration and demonstrate intracranial
extension well
• It is useful in intracranial spread and evaluating
patients postexenteration to look for recurrence.
BIBLIOGRAPHY
1. Jakobiec FA, Yeo JH, Trokel SL, Abbott GF, Anderson R,
Citrin CM, Alper MG. Combined clinical and computed
tomographic diagnosis of primary lacrimal fossa lesions.
Am J Ophthalmol 1982;94(6):785-807.
264 Section 3 Disorders of Orbit

Fig. 19.15: A 40-year-old lady with painful proptosis of left

eye of one-year duration with associated decreased vision.
Coronal CT scan of orbit shows a heterogeneous lacrimal
gland mass with areas of necrosis (arrow) indenting the globe
superiorly

Fig. 19.16: Adenoid cystic carcinoma. Coronal CT scan of

orbit showing a heterogeneous lacrimal gland mass with
erosion of the orbital roof and intracranial extension (arrow)
 Chapter 19 Lacrimal Gland Lesions 265

Figs 19.17A and B: (A) Axial CT scan and (B) Coronal CT scan
of orbit showing a heterogeneous mass with calcification and
bony erosion of the adjacent lacrimal bone. Frontal epidural
B extension is seen (arrows)—adenoid cystic carcinoma
266 Section 3 Disorders of Orbit

A B

C D

Figs 19.18A to D: A 40-year-old lady underwent extended orbital exenteration for adenoid cystic carcinoma of the right lacrimal
gland presented 2 years later with right frontal mass: (A) Coronal precontrast T1-weighted MRI scan, (B) Coronal postcontrast
T1-weighted MRI scan of orbit at the level of the apex and (C and D) Anterior orbit level shows postexenteration status of the right
orbit and an enhancing lesion in the right frontal lobe (black arrow) and at the orbital apex, superior orbital fissure (white arrow)—
recurrent adenoid cystic carcinoma

DUCTAL ADENOCARCINOMA
Primary adenocarcinoma of the lacrimal gland is rare. In
contrast, primary adenocarcinomas of the major and
minor salivary glands are much more common.
DIAGNOSTIC TECHNIQUES
CT Scan
• Irregular lacrimal gland mass is seen with infiltrative
margins
• Extensive bony sclerosis and lysis is noted in the
contiguous bone and skip bony lesions are also seen
Chapter 19 Lacrimal Gland Lesions 267
• Contrast-enhanced CT will demonstrate the
intracranial extension.
BIBLIOGRAPHY
1. Katz SE, Rootman J, Dolman PJ, White VA, Berean KW.
Primary ductal adenocarcinoma of the lacrimal gland.
Ophthalmol 1996;103(1):157-62.
2. Krishnakumar S, Subramanian N, Mahesh L, Mohan ER,
Biswas J. Primary ductal adenocarcinoma of the lacrimal
gland in a patient with neurofibromatosis. Eye
2003;17(7):843-5.
268 Section 3 Disorders of Orbit

Fig. 19.19: A 46-year-old male, a known case of neurofibromatosis

presented with gradual proptosis of left eye of 2 years duration.
Axial CT scan of the orbit showing a diffuse infiltrating lesion in the
left orbit with gross proptosis. HPE—ductal adenocarcinoma

Figs 19.20A and B: A 60-year-old male

A B
presented with gradual painless proptosis
of left eye of 2 years duration, associated
with decreased vision: (A) Axial CT scan at
inferior orbital level shows inferior
displacement of the globe by a large
heterogeneous infiltrating mass with
calcification (arrow) and (B) Axial CT scan
at the level of the frontal sinus showing
enhancing soft tissue within the frontal sinus
with extensive mixed lysis and sclerosis of
the walls of the frontal sinus (arrow). HPE—
ductal adenocarcinoma

Figs 19.21A and B: A 58-year-old male was

A B
operated for a benign mixed left lacrimal
gland tumor and 20 years later presented
with proptosis and mild pain: (A) Coronal
CT scan soft tissue window and (B) Bone
window at the level of the frontal sinus
showing enhancing extraconal soft tissue
in the left orbit, frontal sinus and frontal
epidural space with extensive mixed lysis
and sclerosis of the walls of the frontal sinus.
HPE—ductal adenocarcinoma

LYMPHOPROLIFERATIVE DISORDERS
OF LACRIMAL GLAND
These include reactive lymphoid hyperplasia and
lymphoma. The lesions usually present as firm painless
nodular anterior orbital masses.
IMAGING
CT Scan
• In case of reactive lymphoid hyperplasia, the lesion
is more heterogeneous and the margins appear more
infiltrative
• Lymphomatous infiltration of the lacrimal gland is
diffuse and involves both the palpebral and orbital
lobes. The shape of the gland is preserved. The lesion
moulds the globe. Calcification is rarely seen. Bilateral
lesions are possible. Heterogeneous lesions with bony
destruction and pain are indicative of high-grade
lymphomas
Chapter 19 Lacrimal Gland Lesions 269
• In addition, leukemic deposits can also involve the
lacrimal gland. The lacrimal gland mass appears
heterogeneous. Bone erosion can occur. Bilateral
involvement can occur.
BIBLIOGRAPHY
1. Cassidy DT, McKelvie P, Harris GJ, Rose GE, McNab AA.
Lacrimal gland orbital lobe cysts associated with MALT
lymphoma and primary Sjögren’s syndrome. Orbit
2005;24(4):257-63.
2. Farmer JP, Lamba M, Lamba WR, Jordan DR, Gilberg S,
Sengar DP, Bence-Bruckler I, Burns BF. Lympho-
proliferative lesions of the lacrimal gland: Clinico-
pathological, immunohistochemical and molecular genetic
analysis. Can J Ophthalmol 2005;40(2):151-60.
3. Izumi M, Eguchi K, Uetani M, Nakamura H, Takagi Y,
Hayashi K, Nakamura T. MR features of the lacrimal gland
in Sjögren’s syndrome. Am J Roentgenol 1998;170(6):
1661-6.
270 Section 3 Disorders of Orbit

Fig. 19.22: Reactive lymphoid hyperplasia. Axial CT scan of the orbit

showing diffuse enlargement of the orbital lobe of the left lacrimal gland
with preservation of the shape (arrow)

Fig. 19.23: Lymphoma. Coronal CT scan of the orbit showing diffuse

enlargement of the right lacrimal gland (arrow)

Fig. 19.24: Chloroma. An 8-year-old child presented with painless

proptosis of the right eye of 15 days duration and non-resolution of the
lesion after antibiotics and anti-inflammatory. Axial postcontrast CT scan
showing a well-defined mass lesion in the region of the lacrimal gland
(arrow)
Chapter 20
NEUROFIBROMATOSIS OF ORBIT
• Neurofibromatosis is an autosomal dominant condition
• Diagnostic criteria include 6 or more café-au-lait spots of
more than 15 mm in diameter, axillary freckles, cutaneous
neurofibromata and a family history of neurofibromatosis
Visceral neurofibromata, mental disorders and
skeletal abnormalities can occur. Type II neuro-
fibromatosis is associated with schwannomas,
meningiomas, ependymomas and astrocytomas
• Orbital neurofibromatosis is characterized by
hamartomatous overgrowth of nerve sheath tissue. It
involves both soft and bony tissue. They are associated
with optic nerve meningiomas and gliomas, orbital
schwannomas and neurofibromas and sphenoid wing
dysplasia
• Facial deformities can occur. Sagging of the
infraorbital margin and temporal bone defects are
other common bony deformities.
IMAGING
CT Scan
• Moderately enhancing ill-defined soft tissue density
lesion representing plexiform neurofibromas may be
seen in the eyelids, periorbital and intraorbital regions
Sphenoids wing dysplasia may be seen with
varying degree. Gross defects can result in herniation
of the temporal lobe into the orbit resulting in pulsatile
proptosis (Figs 20.1A and B)
Neurogenic Tumors
• Involvement of extraocular muscles appears as a
nodular enlargement
• Expansion of the bony orbit can occur.
MR Imaging
• The lesions appear iso- and hypointense on T1- and
hyperintense on T2-weighted images.
• Orbital and intracanalicular optic nerves are better
appreciated on MRI.
The associated intracranial hamartomatous lesions
in neurofibromatosis type I are better delineated on
MRI.
Discussed in Chapter 51.
BIBLIOGRAPHY
1. Chen JY, Muecke JS, Brown SD. Orbital plexiform
neurofibroma and high axial myopia. Ophthal Plast
Reconstr Surg 2008;24(4):284-6.
2. Jacquemin C, Bosley TM, Svedberg H. Orbit deformities
in craniofacial neurofibromatosis type 1. Am J Neuroradiol
2003;24(8):1678-82.
3. Kapur R, Mafee MF, Lamba R, Edward DP. Orbital
schwannoma and neurofibroma: Role of imaging. Neuro-
imaging Clin N Am 2005;15(1):159-74.
4. Pomeranz SJ, Shelton JJ, Tobias J, Soila K, Altman D,
Viamonte M. MR of visual pathways in patients with
neurofibromatosis. Am J Neuroradiol 1987;8(5): 831-6.
272 Section 3 Disorders of Orbit

Figs 20.1A and B: (A) Axial CT scan and (B) Coronal CT scan
of orbits showing dysplasia of greater wing of right sphenoid
bone with herniation of the temporal lobe through the defect
(small black arrow). Ill-defined soft tissue is noted in the right
pre- and postseptal region and temporal fossa (long black
B arrow)—plexiform neurofibromatosis

ORBITAL SCHWANNOMA
• Schwannoma is a benign proliferation of Schwann
cells, those envelope peripheral nerves. Within the
orbit, sensory and motor nerves can be affected
• The superonasal quadrant of the orbit is more
frequently affected. The most common age of
occurrence is the second to fifth decade
• They may be noted in association with von
Recklinghausen’s disease.
CLINICAL PRESENTATION
• Slowly progressive painless proptosis. Those lesions
with extension into the cavernous sinus may present
with cranial nerve palsies
• Large lesions close to the optic nerve present with
optic nerve dysfunction.
DIAGNOSTIC TECHNIQUES
CT Scan
• Orbital schwannoma appears as a heterogeneous well-
defined encapsulated heterogeneous minimally
enhancing mass, either intraconal or extraconal in
location
• Smooth bony erosion of adjacent walls is seen in long-
standing lesions
• Intracranial extension of the lesion is better apprecia-
ted on contrast-enhanced CT scan (Figs 20.2A and B)
Chapter 20 Neurogenic Tumors 273
• Heterogeneity is a feature of cystic schwannomas
(Figs 20.3 to 20.5).
MR Imaging
• A well-encapsulated heterogeneous soft tissue density
mass which is hypointense iso- to hypointense on T1
(Fig. 20.6A) and hyperintense on T2-weighted images
• Heterogeneous contrast enhancement is seen (Fig.
20.6B).
Differential Diagnosis
Cavernous hemangioma—a dynamic postcontrast will
help in differentiating them.
BIBLIOGRAPHY
1. Kapur R, Mafee MF, Lamba R, Edward DP. Orbital
schwannoma and neurofibroma: Role of imaging. Neuro-
imaging Clin N Am 2005;15(1):159-74.
2. Subramanian N, Rambhatla S, Mahesh L, Menon SV,
Krishnakumar S, Biswas J, Noronha OV. Cystic schwannoma
of the orbit: A case series. Orbit 2005;24:125-9.
3. Tanaka A, Mihara F, Yoshiura T, Togao O, Kuwabara Y,
Natori Y, Sasaki T, Honda H. Differentiation of cavernous
hemangioma from schwannoma of the orbit: A dynamic MRI
study. Am J Roentgenol 2004;183(6):1799-804.
4. Wang Y, Xiao LH. Orbital schwannomas: Findings from
magnetic resonance imaging in 62 cases. Eye 2007.
274 Section 3 Disorders of Orbit

A B

Figs 20.2A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing a well-circumscribed heterogeneous lesion in
the superotemporal quadrant of the right orbit. The lacrimal gland is seen separately (arrow). Note: Cystic degeneration (arrowhead)

A B

Figs 20.3A and B: A 50-year-old man presented with gradual proptosis of the right eye: (A) Axial postcontrast CT scan and
(B) Coronal postcontrast CT scan of the orbit showing a well-circumscribed extraconal hypodense lesion in the right orbit displacing
the superior rectus (white arrow). Also noted is a large mass lesion in the right cavernous sinus and Meckle’s cave (arrow)—
trigeminal schwannoma extending along the frontal nerve—VI division
 Chapter 20 Neurogenic Tumors 275

Fig. 20.4: A 33-year-old male patient presented with rapid painful
proptosis and underwent biopsy. Axial CT scan of the orbit shows
a large heterogeneous mass lesion in the right medial orbit.
Remodeling of adjacent medial orbital wall is seen. Note: Lateral
orbitotomy defect. HPE—malignant schwannoma
Fig. 20.5: Axial CT scan of the orbit showing a well-circumscribed
intraconal mass lesion in the left orbit with cystic degeneration.
HPE—cystic schwannoma (arrow)

A B

Figs 20.6A and B: Neurofibroma: (A) Axial T1-weighted image and (B) Postgadolinium fat suppressed axial T1-weighted image
showing well-circumscribed enhancing intraconal mass displacing the optic nerve (arrows) medially
Chapter 21
FIBROUS HISTIOCYTOMA
• This is the most common mesenchymal tumor of the
orbit in adults (median age—43 years). It can occur in
any age group, including children but is usually seen
in adults in the 5th and 6th decades of life
• It involves muscular, fibrous and fatty tissues. The
tumor can occur in a variety of ocular tissues—eyelids,
conjunctiva, corneoscleral limbus, lacrimal sac and
deeper orbital tissues
• Patients present with symptoms of proptosis (60%),
mass (46%) and decreased vision (25%). Loss of vision,
conjunctival chemosis and oculomotor palsies occur
as the tumor enlarges especially at the apex
• The tumor can be benign, locally aggressive or
malignant. Histologically, a well-circumscribed lesion
with solid yellow-gray appearance is seen and it is
usually made of a mixture of spindle-shaped
fibroblasts and ovoid histiocytes.
IMAGING
CT Scan
• No specific diagnostic feature is seen
• Precontrast imaging shows well-circumscribed round,
or irregular mass of muscle attenuation
• They show moderate enhancement, sometimes with
nonenhancing central necrotic areas
• Difficult to differentiate from neurofibroma.
Fibrous Lesions
MAGNETIC RESONANCE IMAGING
• Nonspecific intermediate signal on T1-weighted image
and high signal intensity on T2-weighted image is seen
• Malignant fibrous histiocytoma may show poor
margins, low intensity septations with heterogeneous
signal on T2-weighted images.
DIFFERENTIAL DIAGNOSIS
Neurolemmoma, hemangiopericytoma, nodular fasciitis,
rhabdomyosarcoma, lymphoma, hemangioma, melanoma,
metastasis.
BIBLIOGRAPHY
1. Bajaj MS, Sethi A, Kashyap S, Thanikachalam, Pushker N.
Locally aggressive orbital fibrous histiocytoma. Indian J
Ophthalmol 2004;52(1):62-4.
2. Bal MS, Singh SP, Jindal K, Thakur KK. Fibrous histio-
cytoma of orbit: A case report. Indian J Pathol Microbiol
2000;43(1):93-5.
3. Dalley RW. Fibrous histiocytoma and fibrous tissue tumors of
the orbit. Radiol Clin North Am 1999;37(1):185-94.
4. Jacomb-Hood J, Moseley IF. Orbital fibrous histiocytoma:
Computed tomography in 10 cases and a review of
radiological findings. Clin Radiol 1991;43(2):117-20.
5. Kargi E, Kargi S, Gun B, Hosnuter M, Altinyazar C, Aktunc
E. Benign fibrous histiocytoma of the eyelid with an
unusual clinical presentation. J Dermatol 2004;31(1):27-31.
 Chapter 21 Fibrous Lesions 277

Figs 21.1A and B: Fibrous histiocytoma: (A) Axial CT scan of

the orbit and (B) Coronal CT scan of the orbit showing a well-
circumscribed intraconal mass lesion in the left orbit inferior
B and temporal to the optic nerve (arrow)
278 Section 3 Disorders of Orbit
6. Rubenstein WA, Gray G, Auh YH, Honig CL,
Thorbjarnarson B, Williams JJ, Haimes AB, Zirinsky K,
Kazam E. CT of fibrous tissues and tumors with sono-
graphic correlation. Am J Roentgenol 1986; 147(5):1067-74.
7. Ros PR, Kursunoglu S, Batlle JF, Sheldon JJ, Glaser J.
Malignant fibrous histiocytoma of the orbit. J Clin
Neuroophthalmol 1985;5(2):116-9.
8. Shinaver CN, Mafee MF, Choi KH. MRI of mesenchymal
chondrosarcoma of the orbit: Case report and review of
the literature. Neuroradiology 1997;39(4):296-301.
Review.
9. Ulloa TK, Anderson SF. Orbital fibrous histiocytoma: Case
report and literature review. J Am Optom Assoc 1999;
70(4):253-60. Review.
 Chapter 21 Fibrous Lesions 279

A B

C D

Figs 21.2A to D: (A) Axial T2-weighted, (B) Axial T1-weighted image, (C) Coronal T2-weighted
and (D) Coronal T1-weighted image, showing a well-circumscribed intraconal mass inferior to
the left optic nerve displaying a hypointense thick capsule with mixed signal in the center of the
lesion. HPE—fibrous histiocytoma

A B

Figs 21.3A and B: (A) Axial T2-weighted image and (B) Axial T1-weighted image of the orbit
showing proptosis of the right eye and a well-defined intraconal mass, isointense in T1-weighted
image and mixed hypo- and hyperintense signal in T2-weighted image, small area of central
necrosis is also noted—fibrous histiocytoma
280 Section 3 Disorders of Orbit
SOLITARY FIBROUS TUMOR
• Solitary fibrous tumor is a recently described entity
in the orbit. It has been described frequently in the
pleura suggesting a mesothelial origin but now
studies favor a mesenchymal origin
• Patients with orbital involvement typically present with
gradual onset of proptosis. It is generally a benign
tumor. A small percentage of tumors behave
aggressively with local recurrence.
IMAGING
CT Orbit
Shows a mild-to-moderately enhancing well-circum-
scribed mass with or without adjacent bony remodeling.
MRI
• Shows an intermediate intensity mass on T1-weighted
images with moderate homogeneous or inhomo-
geneous enhancement after gadolinium. On T2-
weighted images, the mass can be hypointense or
hyperintense
• Dynamic contrast-enhanced MRA shows a hyper-
vascular arterial phase blush.
DIFFERENTIAL DIAGNOSIS
Hemangiopericytoma, schwannoma, neurofibroma,
meningioma, fibrous histiocytoma.
10.
BIBLIOGRAPHY
1. Bernardini FP, de Conciliis C, Schneider S, Kersten RC,
Kulwin DR. Solitary fibrous tumor of the orbit: Is it rare?
Report of a case series and review of the literature.
Ophthalmology 2003;110(7):1442-8. Review.
2. Dalley RW. Fibrous histiocytoma and fibrous tissue
tumors of the orbit. Radiol Clin North Am 1999;37(1):
185-94.
3. Fenton S, Moriarty P, Kennedy S. Solitary fibrous tumor
of the orbit. Eye 2001;15(1):124-6.
4. Festa S, Lee HJ, Langer P, Klein KM. Solitary fibrous tumor
of the orbit: CT and pathologic correlation. Neuroradiology
1999;41(1):52-4.
5. Galie M, Tieghi R, Cavazzini L, Clauser L. Solitary fibrous
tumor of the orbit: A case report. Int J Oral Maxillofac
Surg 2005;34(3):331-3.
6. Gigantelli JW, Kincaid MC, Soparkar CN, Lee AG, Carter
SR, Yeatts RP, Holck DE, Hartstein ME, Kennerdell JS.
Orbital solitary fibrous tumor: Radiographic and histo-
pathologic correlations. Ophthal Plast Reconstr Surg 2001;
17(3):207-14.
7. Hsu SS, Lai PH, Wang JS, Yip CM. Solitary fibrous tumor
of the orbit. J Chin Med Assoc 2004;67(9):483-6.
8. Johnson TE, Onofrey CB, Ehlies FJ. Echography as a
useful adjunct in the diagnosis of orbital solitary fibrous
tumor. Ophthal Plast Reconstr Surg 2003;19(1):68-74.
Review.
9. Krishnakumar S, Subramanian N, Mohan ER, Mahesh L,
Biswas J, Rao NA. Solitary fibrous tumor of the orbit: A
clinicopathologic study of six cases with review of the
literature. Surv Ophthalmol 2003;48(5):544-54. Review.
Romer M, Bode B, Schuknecht B, Schmid S, Holzmann D.
Solitary fibrous tumor of the orbit: Two cases and a review
of the literature. Eur Arch Otorhinolaryngol 2005;
262(2):81-8. Epub 2004 Aug 13.
 Chapter 21 Fibrous Lesions 281

A B

Figs 21.4A and B: A 20-year-old female presented with proptosis of 5 years duration: (A) Axial CT scan and (B) Coronal CT scan
of the orbit showing large well-circumscribed homogeneous slightly hyperdense mass in the left orbit almost filling the orbit

A B

Figs 21.5A and B: (A) Axial T1WI of the orbit and (B) Axial T2-weighted image of the orbit showing a well-defined solid mass
lesion in the superotemporal aspect of the right orbit inseparable from the lacrimal gland. Note: The flow voids within and around
the lesions (arrows). HPE—solitory fibrous tumor
Chapter 22

Secondary Orbital Tumors and

Paraorbital Lesions
The close proximity of the orbit to the paranasal sinus cavities makes it vulnerable to various infective/inflammatory
and neoplastic lesions extending from the sinus to the orbits. The orbit can be involved secondarily from various
benign and malignant lesions of the paranasal sinuses and adnexal regions. The common paranasal sinus lesions
affecting the orbit are infections (discussed in Chapter 13), mucoceles, fibro-osseous lesions and malignant neoplasms
both bony and soft tissue tumors.

SINUS MUCOCELE
• This is a mucus collection lined by the mucus-secreting
epithelium of a paranasal sinus
• It occurs when a sinus ostium or a compartment of a
septated sinus becomes obstructed, thus causing the
sinus cavity to be mucus-filled and airless
• The obstruction is often inflammatory in nature, but
may also be due to tumor, trauma, or surgical
manipulation
• It is the most common expansile lesion of the paranasal
sinuses and leads to outward expansion with bony
remodeling
• Initially, the bony structures remain intact, but with
further expansion, deossification may occur
• 60–65 percent of mucoceles reside in the frontal
sinuses, 20–25 percent in the ethmoid sinuses, 5–10
percent in the maxillary sinuses, and 5–10 percent in
the sphenoid sinuses
• Signs and symptoms may last from a few days to years
and are most often due to mass effect
• A mucocele in the frontal sinuses typically leads to
frontal headaches and inferolateral proptosis with
diplopia. Additionally, a superomedial orbital mass
may develop, and the voice may be nasal in quality
• A mucocele in the ethmoid sinuses frequently presents
as lateral proptosis as well as nasal congestion
• A mucocele in the maxillary sinuses causes upward
displacement of the eye, a cheek mass and nasal
congestion
• A mucocele in the sphenoid sinuses can lead to
suboccipital headaches and visual loss.
Mucoceles are usually asymptomatic but pain may
indicate infection.
IMAGING
• Plain radiographs usually reveal sinus enlargement
with opacification. Sinus wall erosion may also be seen
• On CT scan, a mucocele presents as a nonenhancing,
homogeneous and hypodense mass that fills and
expands the entire sinus cavity. Bony dehiscence is
better appreciated on CT scan
• Mucoceles generally do not enhance with contrast,
but acutely inflamed mucopyoceles may show rim
enhancement
• Magnetic resonance imaging: Its appearance on MRI
varies depending on the protein concentration, which
changes overtime. They usually have low-signal
intensity on T1-weighted images and hypo- or
hyperintense T2-weighted images depending on the
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 283

A B

Figs 22.1A and B: Pneumatocele: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing a large expanded frontal sinus
filled with air displacing the globe inferiorly and resulting in proptosis

A B

Figs 22.2A and B: Frontoethmoid mucocele: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing an expanded right
frontoethmoid sinus resulting thinning of the medial wall of orbit and displacing the globe laterally
284 Section 3 Disorders of Orbit
content. On contrast-enhanced MRI, the sinus mucosa
enhances as a thin line surrounding the mucocele
• The differential diagnosis of an expansile mass
involving the sphenoid sinus should include, in
addition to mucoceles, pituitary adenoma, cranio-
pharyngioma, malignant sinus lesion, meningo-
encephalocele, nasopharyngeal tumor and chordoma
• These lesions can usually be differentiated from
mucoceles clinically and radiologically by the
presence of contrast enhancement and an invasive
pattern of bone destruction.
BIBLIOGRAPHY
1. Atlas SW, Bilaniuk LT, Zimmerman RA. Orbit. In: Stark
DD, Bradley WG (Eds). Magnetic resonance imaging.
St Louis: CV Mosby 1988.pp.570-613.
2. Campbell RE, Barone CA, Makris AN, et al. Image
interpretation session: 1993. Fungal mucocele (aspergillosis)
of the sphenoid sinus. Radiographics 1994;14:197-9.
3. Chui MC, Briant TD, Gray T, Horsey WJ, Hudson AR,
Tucker W. Computed tomography of sphenoid sinus
mucocele. J Otolaryngol 1983;12:263-9.
4. Dawson RC 3rd, Horton JA. MR imaging of mucoceles of
the sphenoid sinus. Am J Neuroradiol 1989;10:613-4.
5. Flanders AE, Rao VM. Paranasal sinus mucocele: Unusual
MR manifestations at 1.5 T. Magn Reson Imaging 1989;
7:333-7.
6. Lim CCT, Dillon WP, McDermott MW. Mucocele
involving the anterior clinoid process: MR and CT
findings. Am J Neuroradiol 1999;20:287-90.
7. Moriyama H, Nakajima T, Honda Y. Studies on mucoceles
of the ethmoid and sphenoid sinuses: Analysis of 47 cases.
J Laryngol Otol 1992;106:23-7.
8. Ruelle A, Pisani R, Andrioli G. "Unusual" MRI appearance
of sphenoid sinus mucocele. Neuroradiol 1991;33:352-3.
9. Som PM. CT of the paranasal sinuses. Neuroradiol
1985;27:189-201.
10. Tchoyoson Lim CC, Dillon WP, McDermott MW.
Mucocele involving the anterior clinoid process: MR and
CT findings. Am J Neuroradiol 1999;20:287-90.
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 285

Figs 22.3A and B: (A) Coronal bone

window and (B) Coronal soft tissue CT
scan depicting soft tissue expanding
the sphenoid sinus and compressing
optic nerve on the right side—sphenoid
A B mucocele

A B

Figs 22.4A to D: (A and B) Coronal CT

scan and (C and D) Axial CT scan of
orbit and paranasal sinuses showing a
large expanded sphenoid sinus filled with
homogeneous soft tissue (black arrows)
with dehiscence of medial walls of the
orbits and extension into the orbit
compressing the optic nerves (dotted
C D arrows)
286 Section 3 Disorders of Orbit
FIBRO-OSSEOUS LESIONS
Orbital and paraorbital fibro-osseous lesions share similar
clinical and radiopathologic features. Included in this
spectrum are benign lesions such as fibrous dysplasia,
ossifying fibroma, osteoma, osteoblastoma and
aneurysmal bone cyst. The malignant lesions include
osteosarcoma.
FIBROUS DYSPLASIA
• This is a fibro-osseous lesion considered to be a
hamartomatous malformation. It presumably results
from an idiopathic arrest in maturation of bone at the
woven bone stage
• Normal medullary bone is replaced by structurally
weak fibrous and osseous tissue
• It may involve only one bone (monostotic) or multiple
bones (polyostotic), the latter being found as part of
Albright’s syndrome
• Clinical onset occurs during early life, almost always
in the first or second decade. Cases involving the orbit
are usually not part of the Albright’s syndrome
• The patient characteristically presents with painless
facial asymmetry. Globe displacement is a feature and
the direction of displacement depends on the site of
involvement
• Headache and nasal obstruction may occur. In
advanced cases, there may be visual field defects,
ptosis and epiphora. Optic nerve compression occurs
from lesions involving sphenoid bone
• Cases of fibrous dysplasia are known to undergo
malignant transformation into osteosarcoma,
fibrosarcoma, and other malignant conditions.
IMAGING
CT Scan
• The radiological features depend on the stage of
development and amount of bony matrix within the
lesion. Radiographic changes range from lucent zones
to diffuse areas of sclerosis
• Expansion of involved bone, heterogeneous pattern
of CT densities with ground-glass matrix along with
an intact thin cortex is characteristic
• Periosteal reaction is not a feature of benign fibro-
osseous lesions
• Facial bones and base of skull are preferentially
involved by sclerotic form of disease. Lytic form
occurs in cranial bones.
MRI
The signal in fibrous dysplasia varies. Most often, they
are T1 and T2 hypointense due to sclerosis within the
lesion. Areas of lysis will display variable signal.
Moderate-to-marked contrast enhancement is seen
Differential diagnosis—intraosseous meningioma.
BIBLIOGRAPHY
1. Lupescu I, Hermier M, Georgescu SA, Froment JC. Helical
CT and diagnostic evaluation of craniofacial fibrous
dysplasia. J Radiol 2001;82(2):145-9.
2. Schneider JP, Kosling S, Hantel T, Dietrich J, Herrmann
W. CT and MRI in pronounced dysplastic cranial changes
in the framework of McCune-Albright syndrome. Rofo
1998;168(5):528-30.
3. Shah ZK, Peh WC, Koh WL, Shek TW. Magnetic resonance
imaging appearances of fibrous dysplasia. Br J Radiol
2005;78(936):1104-15. Review.
4. Sharma RR, Mahapatra AK, Pawar SJ, Lad SD, Athale SD,
Musa MM. Symptomatic cranial fibrous dysplasias:
Clinicoradiological analysis in a series of eight operative
cases with follow-up results. J Clin Neurosci 2002;9(4):381-
90.
5. Wenig BM, Mafee MF, Ghosh L. Fibro-osseous, osseous,
and cartilaginous lesions of the orbit and paraorbital
region. Correlative clinicopathologic and radiographic
features, including the diagnostic role of CT and MR
imaging. Radiol Clin North Am 1998;36(6):1241-59,xii.
Review.
6. Wilbur AC, Dobben GD, Linder B. Paraorbital tumors and
tumor-like conditions: Role of CT and MRI. Radiol Clin
North Am 1987;25(3):631-46.
7. Yao L, Eckardt JJ, Seeger LL. Fibrous dysplasia associated
with cortical bony destruction: CT and MR findings.
J Comput Assist Tomogr 1994;18(1):91-4.
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 287

A B C

Figs 22.5A to C: (A and B) Coronal CT scan of the orbit and (C) Axial CT bone window showing expansion of the right frontal
bone and sphenoid bone with a heterogeneous ground-glass matrix. The expanded sphenoid and anterior clinoid process resulting
in bilateral optic canal narrowing (arrows)
288 Section 3 Disorders of Orbit
OSTEOMA
• It is a well-differentiated benign bony neoplasm.
It accounts for 1 to 2 percent of tumors involving the orbit
• Sometimes, it is noted as a coincidental finding on CT
scan. Most osteomas in the orbit arise from bones of
paranasal sinuses and do not produce signs and
symptoms related to the orbit
• They are usually unilateral and solitary but multiple
lesions can occur. It is more commonly diagnosed in
adulthood
• Multiple osteomas are seen in Gardner’s syndrome
• The signs and symptoms vary depending on the
location. While osteomas of frontal sinus are more
likely to produce orbital symptoms, osteomas from
ethmoid, maxillary and sphenoid sinuses are usually
asymptomatic when small
• Osteomas from the orbital roof produce facial
asymmetry, downward displacement of globe and
proptosis but visual impairment is less. Ethmoid bone
osteomas may produce lateral displacement of globe,
mass in the superonasal aspect of orbit and signs of
nasolacrimal duct obstruction
• Maxillary sinus osteomas usually produce no ocular
symptoms but large pedunculated ones cause upward
displacement of globe, proptosis and visual
disturbance
• Sphenoid sinus osteomas cause optic atrophy and
visual loss by encroaching on the optic foramen.
IMAGING
CT Scan
• Rounded, sharply delineated or lobulated radiodense
mass is seen arising from bone and protruding into
the sinus. Sometimes, the borders may be irregular
• The ivory or mature type of osteoma has a density
similar to bone. The fibrous type is less dense and may
closely resemble fibrous dysplasia or ossifying fibroma
• Osteomas from frontal sinus have a broad flat base
with a sessile configuration but those from ethmoid
or maxillary sinus have a smaller base with a
pedunculated or mushroom configuration
BIBLIOGRAPHY
1. Bartlett JR. Intracranial neurological complications of
frontal and ethmoidal osteomas. Br J Surg 1971;58(8):
607-13.
2. Bushan B, Watal G, Ahmed A, Saxena R, Goswami K,
Pathania AG. Giant ivory osteoma of frontal sinus.
Australas Radiol 1987;31(3):306-8.
3. El Kohen A, Lahlou M, Rabeh G, Benjelloun A, Lazrak A,
Jazouli N, Kzadri M. Orbital osteoma: Clinical evaluation
of nine cases. Rev Stomatol Chir Maxillofac 2005;106(1):
7-12.
4. Knothe J, Tellkamp H. Value of computerized tomo-
graphy in paranasal sinus osteoma. Laryngol Rhinol Otol
(Stuttg) 1988;67(1):25-7.
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 289

A B C

Figs 22.6A to C: (A and B) Axial CT bone window settings and (C) Thick MIP (maximum intensity projection) showing a well-
circumscribed dense lesion in the right frontal sinus projecting into the superior orbit suggestive of an osteoma
290 Section 3 Disorders of Orbit
MALIGNANT LESIONS OF THE MAXILLARY SINUS
MAXILLARY SINUS CARCINOMA
• The specific site of origin of antral carcinomas is
thought to have prognostic significance and is
important in planning therapy and in comparing
treatment results
• Antral carcinomas are twice as common in men as
in women and 95 percent occur over the age of 40
years. The radioactive contrast medium thorotrast
is clearly established as an etiologic factor in antral
carcinomas
• 40 to 60 percent of patients have facial asymmetry with
a tumor bulge in the oral cavity and tumor extension
in the nasal cavity
• Facial pain and paresthesia, nonaxial proptosis,
decreased vision, extraocular muscle restriction,
painful ophthalmoplegia (cavernous sinus
involvement), diplopia, lid and conjunctival edema,
tearing, firm lid mass, intraocular invasion and
glaucoma, optic disc swelling and Horner’s syndrome
are some of the presenting features
• Otorhinological features of nasal obstruction and
discharge, enlarged neck nodes, epistaxis, chronic
sinusitis, and difficulty in chewing may also be
present
• Small tumors can be misdiagnosed as chronic
sinusitis, nasal polyposis, tic douloureux.
IMAGING
CT Scan
• All patients have a soft tissue sinus mass and 70 to 90
percent have evidence of bone destruction on plain
films. The characteristic imaging feature is their strong
tendency to destroy bone. Bone remodeling is
uncommon. The area of bone destruction is substantial
compared to the size of tumor mass
• After contrast, the carcinomas have a variable but
slight enhancement
• Sectional imaging is important to better visualize the
extent of spread beyond the sinus cavity.
MRI
• They have an intermediate T1-weighted and slightly
higher T2-weighted signal intensity
• These tumors enhance with contrast
• Sinus carcinomas are fairly homogeneous but larger
tumors may have areas of necrosis and hemorrhage.
Other malignant tumors of the paranasal sinuses like
lymphoma and rhabdomyosarcoma should be ruled
out.
SINONASAL LYMPHOMA
• This tumor is more common in the Asian population
and represents the second most frequent site after
gastrointestinal lymphomas
• Patients present with symptoms related to paranasal
sinus involvement, pain, facial or palatal mass or
ocular symptoms. T-cell type tumors are typically
located in the nasal cavity and have an aggressive
angioinvasive growth pattern with necrosis and bony
erosion
• Younger patients are more commonly affected.
Imaging
• On CT and MR imaging, they appear as bulky soft
tissue masses with moderate enhancement, most often
located in nasal fossa and maxillary sinus and rarely
in frontal and sphenoid sinus
• On MR imaging, they have intermediate signal
intensity on all sequences.
RHABDOMYOSARCOMA (RMS)
• This is a soft tissue sarcoma of mesenchymal
derivation. It is the most common soft tissue sarcoma
in children
• The most common sites in the head and neck are the
orbit, nasopharynx, middle ear and mastoid, sinonasal
cavity, face, neck, larynx and oral cavity in decreasing
order
• It produces rapidly progressive proptosis in children
• Embryonal RMS is the most common type and it is
derived from muscle cell
• RMS is an aggressive bone destructive lesions of
children and adults less than 20 years. It can cause
innocuous problems like chronic sinusitis, proptosis
or a small naso-orbital mass.
Imaging
• On imaging, they usually present with a large mass
lesion with bony remodeling/destruction
• Moderate homogeneous contrast enhancement on CT
scan is seen
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 291

Figs 22.7A and B: An 11-year-old boy

presented with rapid onset right-sided
proptosis of one and half month duration:
(A) Axial CT scan and (B) Coronal CT scan
of the orbits and paranasal sinuses showing
a large hyperdense mass lesion in the right
maxillary sinus causing erosion of the walls
of the maxillary sinus with extension into the
inferior orbit displacing the inferior rectus.
A B HPE—rhabdomyosarcoma

Figs 22.8A and B: (A) Axial CT scan and

(B) Coronal CT scan of the orbits and
paranasal sinuses showing a large
destructive lesion in the maxillary and
ethmoid sinus extending into the nasal
cavity, orbits and frontal epidural space.
A B HPE—squamous cell carcinoma

Figs 22.9A and B: (A) Axial CT scan and

(B) Coronal CT scan of the orbits and
paranasal sinuses showing a large
destructive lesion in the ethmoid and left
maxillary sinus extending into the orbit
resulting in proptosis—carcinoma of
A B maxillary antrum
292 Section 3 Disorders of Orbit
• On MRI, they show intermediate signal on all
sequences, enhance with contrast and are generally
homogeneous
• MRI is the preferred modality as the local extent and
intracranial spread is better delineated on contrast-
enhanced MRI. CT may be complimentary in assessing
the bone erosion.
BIBLIOGRAPHY
1. Ahmad K, Cordoba RB, Fayos JV. Squamous cell
carcinoma of the maxillary sinus. Arch Otolaryngol 1981;
107(1):48-51
2. Bailey BJ, Lawrence RA. Rhabdomyosarcoma of the
maxillary sinus. Trans Am Acad Ophthalmol Otolaryngol
1972;76(5):1375-7.
3. Dulguerov P, Jacobsen MS, Allal AS, Lehmann W,
Calcaterra T. Nasal and paranasal sinus carcinoma: Are
we making progress? A series of 220 patients and a
systematic review. Cancer 2001; 92(12):3012-29.
4. Giordano M, Gilardini P, Puglisi A, Scata G. Case of
rhabdomyosarcoma of the maxillary bone. Minerva
Ortognatod 1990;8(2):117-20.
5. Grieman RB, Katsikeris NF, Symington JM. Rhabdomyo-
sarcoma of the maxillary sinus: Review of the literature
and report of a case. J Oral Maxillofac Surg 1988;46(12):
1090-6. Review.
6. Kanegaonkar G, McDougall J, Grant HR. Rhabdo-
myosarcoma of the maxillary antrum in an adult: A case
report with ultrastructural observations. J Laryngol Otol
1981;95(8):863-72.
7. Sharara N, Muller S, Olson J, Grist WJ, Grossniklaus HE.
Sinonasal undifferentiated carcinoma with orbital
invasion: Report of three cases. Ophthal Plast Reconstr
Surg 2001;17(4):288-92.
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 293

A B

C D

Figs 22.10A to D: (A) Coronal T2-weighted image, (B) T1-weighted image of the paranasal sinuses and (C and D) Axial T2-
weighted image showing soft tissue mass lesion filling the maxillary sinuses with extension into the cheek, retromaxillary space
and right cavernous sinus (arrow). HPE—lymphoma
294 Section 3 Disorders of Orbit
MESENCHYMAL CHONDROSARCOMA
• Chondrosarcoma is a rare tumor of the orbit
• It affects females in the 2nd to 4th decade
• Clinically, patients present with proptosis and pain
• Occasionally, can cause vision loss due to optic nerve
involvement
• It is a highly unpredictable tumor and needs a periodic
evaluation to assess the metastases
• Histopathologically, the tumor is composed of highly
cellular undifferentiated mesenchymal cells and well-
defined cartilage.
IMAGING
CT Scan
• CT reveals well-defined intraconal mass lesion with
dense calcification, but rarely they may not have
calcification and show attenuation similar to extra-
ocular muscles
• A well-defined lytic stippled lesion with calcification
and a wide transition zone is characteristic for
chondrosarcoma involving the bone
MRI Scan
• On T1 images, they show low-to-intermediate signals
and in T2 images, they are isointense to gray matter.
• With contrast, they show heterogeneous enhancement.
BIBLIOGRAPHY
1. Angotti-Neto H, Cunha LP, Oliveira AV, Monteiro ML.
Mesenchymal chondrosarcoma of the orbit. Ophthal Plast
Reconstr Surg 2006;22(5):378-82.
2. Kaur A, Kishore P, Agrawal A, Gupta A. Mesenchymal
chondrosarcoma of the orbit: A report of two cases
and review of the literature. Orbit 2008;27(1):63-7.
Review.
3. Koeller KK. S Mesenchymal chondrosarcoma and
simulating lesions of the orbit. Radiol Clin North Am 1999;
37(1):203-17, xii. Review.
4. Odashiro AN, Leite LV, Oliveira RS, Tamashiro C, Pereira
PR, Miiji LN, Odashiro DN, Burnier MN Jr. Primary orbital
mesenchymal chondrosarcoma: A case report and
literature review. Int Ophthalmol 2009:29(3):173-7. Epub
2008, Jan 10.
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 295

Fig. 22.11: Axial CT scan of the orbit showing intraconal mass

with dense central calcification (arrow)

A B

Figs 22.12A and B: (A) Axial postcontrast T1-weighted and (B) Coronal T2-weighted scans of the same patient showing a well-
circumscribed lesion with moderate peripheral enhancement with nonenhancing central component corresponding to the calcification—
mesenchymal chondrosarcoma
296 Section 3 Disorders of Orbit
NASOPHARYNGEAL CARCINOMA
• Malignancies that originate in the nasopharynx are
rare. Ninety percent of nasopharyngeal tumors are
carcinomas. While squamous cell carcinoma accounts
for 80 percent, adenocarcinoma accounts for the rest
of these tumors
• Nasopharyngeal carcinoma accounts for about
2 percent of head malignancies and can invade the
orbit secondarily
• Men are affected more than women, usually between
the 4th and 6th decades of life. Otorhinological
symptoms include fullness of ear (occlusion of
Eustachian tube), hearing loss, ear discharge, nasal
obstruction, nasal bleeding, nasal discharge and a
neck mass
• Nasal stuffiness and epistaxis usually occur with
anterior lesions while dysphagia and trismus are more
common with posterior lesions
• Direct orbital involvement appears late in the course
of the more posterior tumors. They produce proptosis,
optic disc edema and optic atrophy. The 3rd, 4th, 5th
and 6th cranial nerves can be involved. Painful
ophthalmoplegia may occur
• Loss of vision is either due to intradural spread or
due to involvement of intraorbital or intracanalicular
portion of optic nerve
• Regional metastasis to neck lymph nodes and distant
metastasis to bone, liver and central nervous system
can occur.
IMAGING
CT scan and MRI play complimentary roles in staging
and treatment of patients.
CT Scan
There is usually a recognizable mass that extends into
the sinuses and orbit with marked destruction of bone.
MRI
• Provides excellent visualization of the soft tissue
planes of the nasopharynx and is superior to CT for
detecting perineural spread of tumor and for accurate
tumor mapping
• MR is superior to CT for detecting marrow involvement
• Imaging of cervical lymph nodes is essential in all
patients.
BIBLIOGRAPHY
1. Chang JT, Lin CY, Chen TM, Kang CJ, Ng SH, Chen IH,
Wang HM, Cheng AJ, Liao CT. Nasopharyngeal
carcinoma with cranial nerve palsy: The importance of
MRI for radiotherapy. Int J Radiat Oncol Biol Phys
2005;63(5):1354-60.
2. Chong VF, Mukherji SK, Ng SH, Ginsberg LE, Wee JT,
Sham JS, O'Sullivan B. Nasopharyngeal carcinoma:
Review of how imaging affects staging. J Comput Assist
Tomogr 1999;23(6):984-93.
3. Dickson RI. Nasopharyngeal carcinoma: An evaluation
of 209 patients. Laryngoscope 1981;91(3):333-54.
4. Manavis J, Sivridis L, Koukourakis MI. Nasopharyngeal
carcinoma: The impact of CT-scan and of MRI on staging,
radiotherapy treatment planning, and outcome of the
disease. Clin Imaging 2005;29(2):128-33.
5. Uzcudun AE, Bravo Fernandez P, Sanchez JJ, Garcia
Grande A, Rabanal Retolaza I, Gonzalez Baron M, Gavilan
Bouzas J. Clinical features of pharyngeal cancer: A
retrospective study of 258 consecutive patients. J Laryngol
Otol 2001;115(2):112-8.
6. Wei WI, Sham JS. Nasopharyngeal carcinoma. Lancet
2005;365(9476):2041-54.
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 297

A B C

Figs 22.13A to C: (A) Axial postcontrast T1-weighted MRI, (B) Coronal postcontrast T1-weighted MRI and (C) Sagittal postcontrast
T1-weighted MRI showing an extensive lesion in the right parapharyngeal space with contiguous spread into the right cavernous
sinus—nasopharyngeal carcinoma with intracranial spread
298 Section 3 Disorders of Orbit
PAGET’S DISEASE (OSTEITIS DEFORMANS)
• Paget’s disease is a metabolic bone disease that
involves bone destruction and regrowth which results
in deformity
• The disease is characterized by excessive breakdown
of bone tissue, followed by abnormal bone formation.
The new bone is structurally enlarged, but weakened
and filled with new blood vessels
• The disease may localize to one or two areas within
the skeleton, or become widespread
• Frequently, bones of the pelvis, leg, spine, arm, or the
clavicle are involved. The effect on the skull may
enlarge head size and cause hearing loss, if the cranial
nerves are damaged by the bone growth
• Ophthalmic features include extraocular muscle palsies
(most common is 6M N palsy), angioid streaks,
choroidal sclerosis and unilateral or bilateral proptosis.
Tests that may indicate Paget’s disease include:
• A bone X-ray that shows increased bone density,
thickening, bowing, and overgrowth
• A bone scan
• Elevated serum alkaline phosphatase
• Elevated markers of bone breakdown (for instance,
N-telopeptide).
IMAGING
• Paget’s disease of skull can demonstrate basilar
invagination on sagittal MR
• CT scout film can measure the basilar invagination
• CT/MR of calvarial Paget’s demonstrates trabecular
coarsening, vault thickening and distortion of normal
architecture. MR shows T1 and T2 heterogeneous
signals of abnormally thick calvarium. Paget’s disease
of temporal bone can cause otospongiosis (otosclerosis).
Differential Diagnosis
• Fibrous dysplasia, which may also show elevated serum
calcium and alkaline phosphatase isoenzyme
• Metastases from carcinoma of prostate.
BIBLIOGRAPHY
1. Kumar A, Poon PY, Aggarwal S. Value of CT in diagnosing
non-neoplastic osteolysis in Paget’s disease. J Comput
Assist Tomogr 1993;17(1):144-6.
2. Roberts MC, Kressel HY, Fallon MD, Zlatkin MB, Dalinka
MK. Paget’s disease: MR imaging findings. Radiology
1989;173(2):341-5.
3. Scutellari PN, Giorgi A, De Sario V, Campanati P.
Correlation of multimodality imaging in Paget's disease
of bone. Radiol Med (Torino) 2005;110(5-6):603-15.
4. Tehranzadeh J, Fung Y, Donohue M, Anavim A, Pribram
HW. Computed tomography of Paget's disease of the skull
versus fibrous dysplasia. Skeletal Radiol 1998;27(12):
664-72.
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 299

Figs 22.14A and B: (A) Axial CT scan of

brain (bone window) and (B) Showing diffuse
thickening of calvarium with sclerosis—
A B Paget’s disease (arrow)

A B C

D E

Figs 22.15A to E: Paget’s disease. (A and B) Coronal T2-weighted images, (C) Axial FlAIR, (D) Sagittal T1-weighted image and
(E) Axial T1-weighted showing mixed hyper- and hypointense signal in both T1 and T2 suggestive of sclerotic and fatty marrow
changes. Figures A and E show the narrowing of optic canals on both sides. Patient presented with bilateral gradual progressive
visual loss
300 Section 3 Disorders of Orbit
PNEUMOSINUS DILATANS
• Pneumosinus dilatans is a rare condition of abnormal
dilatation of paranasal sinuses, which contain only
air and are lined by normal mucosa
• There is no associated bone destruction or osseous
hypertrophy. Mostly men in the third decade are
affected but this has also been described at younger
and older ages
• The clinical presentation depends on the sinus
affected. Frontal and maxillary sinus involvement can
produce variable proptosis secondary to orbital
communication. Abnormal pneumatization can lead
to thinning and gross dehiscence of the wall of the
optic canal
• Thus, optic neuropathy can occur because of direct
compressive effect by mucosa or air leading to
ischemic damage
• Sphenoid sinus involvement is the most common
cause for visual loss because of intimate relation with
the optic nerve within the optic canal
• Other clinical features include headache, diplopia,
localized bony tenderness and facial deformity
• Pneumosinus dilatans has been associated with
meningioma and fibro-osseous disease.
Differential diagnosis includes mucocele,
acromegaly.
IMAGING
• CT scan is preferred to MR imaging as bony details
can be accurately assessed on CT
• Abnormal enlargement of the involved sinuses
(sphenoid sinus most commonly) and anterior clinoid
pneumatization are the common neuroimaging
findings seen in these patients
• A coronal CT scan in bone window setting should be
done.
BIBLIOGRAPHY
1. Appelt RA, Withelmi JB, Warder DE, Blackwell SJ. A rare
case of pneumosinus dilatans of the frontal sinus and review
of the literature. Ann Plast Surg 1999;43(6):653-6. Review.
2. Dhillon RS, Williams DC. Pneumosinus dilatans.
J Laryngol Otol 1987;101(8):828-32.
3. Miller NR, Golnik KC, Zeidman SM, North RB.
Pneumosinus dilatans: A sign of intracranial meningioma.
Surg Neurol 1996;46(5);471-4.
4. Reicher MA, Bentson JR, Halbach VV, Lafkin R, Hepler
RS. Pneumosinus dilatans of the sphenoid sinus. Am J
Neuroradiol 1986;7(5):865-8.
5. Skolnick CA, Mafee MF, Goodwin JA. Pneumosinus
dilatans of the sphenoid sinus presenting with visual loss.
J Neuroophthalmol 2000;20(4):259-63.
6. Smith IM, Maran AG, von Haacke NP. Pneumosinus
dilatans. Ann Otol Rhinol Laryngol 1987;96(2 Pt 1):210-2.
7. Spoor TC, Kennerdell JS, Maroon JC, Hepler R, Krohel G.
Pneumosinus dilatans, Klippel-Trenaunay-Weber syndrome,
and progressive visual loss. Ann Ophthalmol 1981;13(1):105-8.
8. Stretch JR, Poole MD. Pneumosinus dilatans as the
aetiology of progressive bilateral blindness. BR J Plast Surg
1992;45(6):469-73.
9. Walker JL, Jones NS. Pneumosinus dilatans of the frontal
sinuses: Two cases and a discussion of its etiology.
J Laryngol Otol 2002;116(5):382-5.
 Chapter 22 Secondary Orbital Tumors and Paraorbital Lesions 301

A B

Figs 22.16A and B: A 32-year-old male with progressive loss of vision in the right eye: (A and B) Coronal CT scan of the orbit
showing hyperpneumatization of the sphenoid and anterior clinoids resulting in narrowing of the optic canals (R > L)

A B

Figs 22.17A and B: (A and B) Coronal T2-weighted images of the orbit at the level of the optic canals showing hyperpneumatization
of the sphenoid sinus resulting in gross optic canal narrowing and optic atrophy in this young patient with bilateral gradual loss of
vision
Chapter 23
Thyroid Eye Disease
THYROID OPHTHALMOPATHY
• Thyroid ophthalmopathy, also known as Graves’
disease, is an autoimmune disorder
• It is the most common orbital disorder in adults and
accounts for 15 to 28 percent of unilateral proptosis
and 80 percent of bilateral proptosis
• Retrobulbar fat and connective tissue infiltration
mainly occurs in type I disease while extraocular
myositis occurs in type II disease
• Thyroid ophthalmopathy is a self-limiting process that
becomes inactive within three to five years of its onset
• Patients with thyroid ophthalmopathy may be
hyperthyroid, hypothyroid or euthyroid
• The ophthalmic signs are lid retraction, lagoph-
thalmos, lid lag on downward gaze, conjunctival
congestion (especially over the insertion of horizontal
recti), conjunctival chemosis, lid edema, starring look,
infrequent blinking, exophthalmos (60%), limitation
of ocular movements, diplopia and elevated intra-
ocular pressure
• One or more extraocular muscles may be involved
simultaneously. Proptosis in thyroid orbitopathy may
result from enlargement of extraocular muscles,
increase in the orbital fat volume with or without
muscle involvement and venous stasis
• Loss of vision can occur due to optic nerve
compression by the increased orbital fat volume and
the thickened extraocular muscles, corneal ulceration
due to severe proptosis and scarring
• Differential diagnosis of thickened extraocular
muscles includes pseudotumor, caroticocavernous
fistula, lymphoma, metastasis and cysticercosis.
IMAGING
CT scan of the orbit is the preferred imaging modality
for evaluation of Graves’ disease as the orbital fat offers
excellent contrast and if planning a orbital wall
decompression, the bony and paranasal sinus anatomy
is better delineated on CT scan.
Imaging Findings
• Increase in the orbital fat volume. It may result in
proptosis and stretching of the optic nerves
• Enlargement of the extraocular muscles is usually
asymmetrical but can be symmetrical. The inferior
rectus is the most common muscle involved followed
by superior muscle complex, medial rectus and
superior oblique. Lateral rectus enlargement is often
less pronounced and in most cases may appear
normal. Enlarged medial rectus may lead to
remodeling of the medial wall
• The enlarged muscles are spindle-shaped with
involvement of the muscle belly and sparing of the
tendons. The margins of the muscle are usually well-
defined
• CT may also reveal muscle enlargement in a clinically
silent eye
• Coronal imaging is mandatory for all patients
suspected to have thyroid eye disease
• Focal areas of hypodensity within the muscles are
suggestive of fatty or mucopolysaccharide deposits
• Mottled increased density may be noted in the retro-
orbital fat due to lymphocytic infiltration and vascular
congestion
 Chapter 23 Thyroid Eye Disease 303

Figs 23.1A to C: (A) External photograph showing severe soft

tissue changes that include eyelid puffiness and retraction,
conjunctival congestion and chemosis, (B) Axial CT scan and
(C) Coronal plain CT scan of the same patient showing fairly
symmetrical thickening of the extraocular muscle bundle on both
sides. The left inferior rectus shows central hypodensity
C suggestive of fatty infiltration (arrow)
304 Section 3 Disorders of Orbit
• Enlargement of the lacrimal gland, and very rarely
enlargement of the optic nerve may be noted.
MR imaging is done to know the disease activity and
optic nerve compression. Muscles with active disease will
have hyperintense signal on T2-weighted and STIR
images. Chronic fibrotic muscles appear hypointense on
T2-weighted images.
Imaging should be done:
• To confirm the diagnosis
• Baseline investigation to look for the involved muscles
• Look for optic nerve compression
• When planning orbital decompression.
BIBLIOGRAPHY
1. Ampudia J, Guardia E, Castrillo P, Cutillas M, Soldevila J,
de Leiva A. Thyroid ophthalmopathy: Clinical and
tomographic study. Nippon Ganka Gakkai Zasshi
1989;93(7):785-9.
2. Baba H, Yoshikawa K, Mizuno T, Inoue T, Inoue Y.
Extraocular muscle aspects of the treatment in dysthyroid
ophthalmopathy. Jpn J Ophthalmol 2001;45(6):622-7.
3. Barrett L, Glatt HJ, Burde RM, Gado MH. Optic nerve
dysfunction in thyroid eye disease: CT. Radiology
1988;167(2):503-7.
4. Ben Simon GJ, Syed HM, Douglas R, McCann JD, Goldberg
RA. Extraocular muscle enlargement with tendon
involvement in thyroid-associated orbitopathy. Am J
Ophthalmol 2004;137(6):1145-7.
5. Dodds NI, Atcha AW, Birchall D, Jackson A. Use of high-
resolution MRI of the optic nerve in Graves'
ophthalmopathy. Br J Radiol 2009;82(979):541-4. Epub
2009, Jan 5.
6. Giaconi JA, Kazim M, Rho T, Pfaff C. CT scan evidence of
dysthyroid optic neuropathy. Ophthal Plast Reconstr Surg
2002;18(3):177-82.
7. Murakami Y, Kanamoto T, Tuboi T, Maeda T, Inoue Y.
Evaluation of extraocular muscle enlargement in dysthyroid
ophthalmopathy. Olympia Eye Hospital, Tokyo, Japan.
8. Nishida Y, Tian S, Isberg B, Hayashi O, Tallstedt L,
Lennerstrand G. Significance of orbital fatty tissue for
exophthalmos in thyroid-associated ophthalmopathy.
Graefes Arch Clin Exp Ophthalmol 2002;240(7):515-20.
Epub 2002 Jun 20.
9. Nishida Y, Tian S, Isberg B, Tallstedt L, Lennerstrand G.
MRI measurements of orbital tissues in dysthyroid
ophthalmopathy. Graefes Arch Clin Exp Ophthalmol
2001;239(11):824-31.
10. Nugent RA, Belkin RI, Neigel JM, Rootman J, Robertson
WD, Spinelli J, Graeb DA. Graves’ orbitopathy: Correlation
of CT and clinical findings. Radiology 1990;177(3):675-82.
11. Ozgen A, Alp MN, Ariyurek M, Tutuncu NB, Can I,
Gunalp I. Quantitative CT of the orbit in Graves’ disease.
Br J Radiol 1999;72(860):757-62.
 Chapter 23 Thyroid Eye Disease 305

Fig. 23.2: Coronal CT scan of the orbit showing asymmetrical

thickening of the extraocular muscles

Fig. 23.3: Axial CT scan of the orbit showing bilateral proptosis

with increase in the orbital fat volume resulting in anterior bowing
of the orbital septum and stretching of the optic nerves.
Herniation of the fat is noted through the superior orbital fissure
(arrow)

Fig. 23.4: Coronal STIR image showing thickening of the

extraocular muscles bilaterally. The thickened left levator
palpebrae superioris muscle (arrow) can be identified separately
from the superior rectus muscle with hyperintense signal in the
inferior, medial and superior muscles bilaterally sign of active
disease
306 Section 3 Disorders of Orbit

Figs 23.5A and B: (A) Preoperative clinical photograph and

(B) Postoperative clinical photograph showing significant
B decrease in the proptosis following orbital decompression

A B

Figs 23.6A and B: (A) Predecompression coronal CT scan and (B) Postdecompression coronal CT scan of the above patient
showing two wall (medial wall and floor) decompression of the orbit with herniation of extraocular muscles and orbital fat into the
adjacent paranasal sinuses resulting in release of optic nerve compression
Chapter 24
• Metastases to the orbit are found in the choroid,
retrobulbar soft tissues, or bony orbit
• Choroidal metastases resulting in retinal detachment
are best evaluated with fundoscopy and ultrasound.
• Retrobulbar metastases can involve both extraconal
and intraconal spaces
• They appear as masses having irregular margins on
plain CT scan and show slight contrast enhancement.
• The greater wing of the sphenoid is the most common
site of bone metastases
• These lesions often have soft tissue components in
both the lateral extraconal space of the orbit and the
middle cranial fossa
• Metastases to the bone and retrobulbar soft tissues cause
exophthalmos (most common), pain, decreasing vision,
periorbital edema, ophthalmoplegia, and diplopia
• The most common primary sources of orbital
metastases are the breast and lung carcinomas,
followed by gastrointestinal and genitourinary sites.
Metastases from prostate are usually sclerotic.
Metastases from thyroid and renal cell carcinoma are
expansile lytic and vascular.
IMAGING
CT Scan
• CT scan with contrast demonstrates extraocular
orbital metastases, showing the extra- or intraconal
Orbital Metastases
location of the mass, invasion or displacement of the
globe, destruction of the orbit, and intracranial
extension
• Most metastases exhibit high attenuation value on plain
CT scans and show slight contrast enhancement. They
usually appear as a discrete mass with irregular
margins, but they can be more diffuse and infiltrating.
MRI Scan
• Compared with CT, MRI provides superior resolution
of both orbital and intracerebral metastases, which are
not uncommon in the setting of an orbital metastasis
• On T1-weighted images, the lesions are isointense to
the muscles and on T2-weighted images, they are
partly hyperintense and isointense to fat.
Contrast-enhanced MRI of the brain and orbit is
recommended in patients with known primary.
BIBLIOGRAPHY
1. Kuo SC, Hsiao SC, Chiou CC, Chen FF, Huang KC.
Metastatic carcinoma of the breast: A case with the unusual
presentation of unilateral periorbital edema. Jpn J
Ophthalmol 2008;52(4):305-7.
2. Wang Y, Yang XJ, Li YY, Hei Y, Xiao LH. Diagnosis and
management of orbital metastatic tumors. Zhonghua Yan
Ke Za Zhi 2008;44(8):687-90.
308 Section 3 Disorders of Orbit

A B

Figs 24.1A and B: A known case of Ca breast, developed proptosis of the right eye: (A) Axial post-
contrast CT and (B) Axial postcontrast fat suppresed T1 image showing a nodular enhancing lesion
in the right lateral rectus muscle

A B

C D

Figs 24.2A to D: (A) Coronal CT of the orbit soft tissue window setting, (B and C) Coronal bone
window settings showing a isodense irregular lobuated mass lesion in the right maxillary sinus, inferior
extraconal space and left superior orbit with bony erosion and (D) Axial CT scan of the brain showing
an epidural enhancing lesion with osteolysis—metastases from lung
 Chapter 24 Orbital Metastases 309

A B

Figs 24.3A and B: (A) Axial postcontrast fat-suppressed T1-weighted image and (B) Coronal postcontrast fat-suppressed T1-
weighted image showing a large brilliantly enhancing lesion around the left greater wing of sphenoid causing extensive osteolysis.
Patient had a large highly vascular thyroid neoplasm
Chapter 25
• Orbital and midfacial fracture usually result from high
acceleration motor vehicle accidents and violent blow
to the face
• Complex and combined fractures involve both the
orbital rim and internal walls.
DIAGNOSTIC TECHNIQUE
CT Scan
• High-resolution axial CT scan is the primary imaging
modality for the evaluation of orbital and ocular
trauma. Both axial and direct coronal views, whenever
possible should be obtained
• Routinely atleast 3 mm axial and direct coronal scans
should obtained for evaluation of orbital fractures. The
sections are studied in bone and soft tissue window
settings
• If the injured patient cannot be positioned for direct
coronal imaging, reconstructed coronal views should
be obtained for evaluation
• With the present generation high speed multislice
spiral imaging, it is possible to get good coronal
isotropic images
• 1 mm high resolution images of the optic canal are
obtained in suspected optic canal fractures
• 2 mm axial scans with overlapping sections are
obtained for evaluation of suspected foreign bodies.
Direct coronal sections are obtained if the foreign body
is located in the inferior or superior orbit or ocular
coats
• In general, a contrast CT scan is not necessary unless
vascular injury is suspected. In this case, a CT
angiogram can be done in the same sitting for
evaluation of the vascular injury, e.g. carotico-
cavernous and dural fistulae or thrombosis of the
superior ophthalmic vein
Orbital Trauma
• Three-dimensional (3D) volume rendering is done for
complex disfiguring facial fractures to help the
maxillofacial surgeon in reconstruction.
Advantages of Spiral CT in Trauma
• Spiral CT acquires images in a short scan time, so they
are useful in pediatric and uncooperative head trauma
patients
• Good quality 3D reconstruction with surface-shaded
display thereby assisting the maxillofacial surgeon in
treatment planning
• CT angiogram can be done in the same sitting when
vascular injury is suspected
• High resolution coronal and sagittal reformations
offer better delineation of the fractures and foreign
bodies.
MR Imaging
• MRI is preferable in optic nerve and muscle injuries.
MR with surface coil technique is superior in
delineation of soft tissue injury to the globe
• In patients with suspected vascular injury, viz carotid
cavernous fistulas, MRI is the preferred imaging
modality
• Useful in imaging wooden foreign bodies.
Disadvantages of MR Imaging
• Bony details are poor
• It is contraindicated in ferromagnetic foreign bodies
• It is a difficult procedure in sick and uncooperative
patients.
ISOLATED ORBITAL FRACTURES
Although isolated fractures may involve any part of the
orbit, they commonly involve the inferior orbital rim.
 Chapter 25 Orbital Trauma 311

A B

Figs 25.1A and B: (A) Plain X-ray (modified Water’s view) showing a displaced fracture floor of the left orbit and teardrop sign
(black arrow) and (B) Coronal CT scan of the orbit in bone window setting of the same patient delineating the fracture and
herniation of the orbital fat into the maxillary sinus (white arrow)

A B

Figs 25.2A and B: (A) Axial CT scan of the orbit and (B) Coronal CT scan of the orbit showing a blow out fracture of the left orbit
with herniation of the orbital fat and entrapment of the inferior rectus (dotted arrow)
312 Section 3 Disorders of Orbit
Superior orbital rim and adjacent roof are other sites
where isolated or comminuted fractures may occur.
Fractures of these sites commonly involve the frontal sinus
resulting in orbital emphysema and pneumocephalus.
Imaging plays a very vital role in the diagnosis and
management of fractures. Conventional X-ray is rarely
done nowadays to diagnose orbitofacial fractures. It may
still be done in rural center where CT scan facilities are
unavailable.
BLOW-OUT AND BLOW-IN FRACTURES
• As the name suggests in orbital blow-out fractures,
there is an outward displacement of the fractured
fragments resulting in enophthalmos. In orbital blow-
in fractures, there is a displacement of the fractured
fragments into the orbit and if significant, will result
in proptosis
• A pure blow-out or blow-in fracture will spare the
orbital rim. The floor and medial wall are most
frequently involved
• Muscle entrapment is frequently noted. The muscle
may be thickened either due to edema or hematoma.
FLOOR FRACTURES
Plain Film Findings of Orbital Floor Fractures
Water's veiw is the recommended view to diagnose floor
fractures.
• Depressed floor fracture extends inferiorly from
medial to lateral side—bony step-off sign
• Globular soft tissue mass projecting inferiorly into the
maxillary sinus from the orbital floor is seen (Tear-
drop sign)
• Opacification of the maxillary sinus can be seen.
CT scan done in axial and coronal planes will provide
the following details:
• Both bony and soft tissue details are seen
• Site and extent of the defect and the amount of
displacement of fracture fragment can be assessed
• Muscle entrapment or tethering can be evaluated
• At times, the patient may have diplopia with no
evidence of muscle entrapment noted on CT scan. In
these cases, small muscle fibers may be caught within
the fracture site with no overt radiographic evidence
of muscle entrapment.
MEDIAL BLOW-OUT FRACTURE
• Approximately, one-third to one-half of orbital
fractures are associated with fractures of the lamina
papyracea
• Isolated medial blow-out fractures are uncommon.
They usually occur in combination with inferior
orbital blow-out fractures.
Plain Films
May not identify the medial wall fractures.
CT Scan
• Depicts the fracture with associated soft tissue injury
• Entrapment of the medial rectus is rarely encountered
• Orbital emphysema occurs most commonly with
medial wall fractures.
ORBITAL ROOF FRACTURES
• Orbital roof fracture accounts for 5 percent of all facial
fractures. It results from direct blow to the superior
orbital rim causing buckling of the orbital roof
• The weakest segment of the roof is near the superior
orbital fissure and optic foramen. Associated injuries
include:
1. Intraorbital injury—ptosis or ocular motility
disorder due to impingement of the bony fragment
on the superior muscle complex or superior
division of the ocular motor nerve
2. Intraocular injury
3. Intracranial injury—dural tear, cerebral contu-
sions, and pneumocephalus.
NASOETHMOID COMPLEX FRACTURES
• Nasoethmoidal fractures result from forces applied
to the midface. Frequently, there is telescoping of the
anterior-most structures resulting in splaying of the
nasal bones and pseudohypertelorism
• There is frequent fracture of the nasolacrimal canal
due to its close proximity. Hence, in nasoethmoid
complex fractures, the lacrimal drainage system
should be evaluated.
ZYGOMATIC COMPLEX FRACTURES
• Zygomatic complex (ZC) fractures most commonly
involve the frontozygomtic suture, zygomatic
arch, anterior wall of the maxilla and midinferior
orbital rim
• There is usually medial, inferior and posterior
displacement of the zygoma
• There may be associated orbital floor fracture adjacent
to the fracture of the orbital rim. ZC injuries are
common and are usually unilateral.
 Chapter 25 Orbital Trauma 313

A B

Figs 25.3A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing a large blow out fracture
of the medial wall with entraped medial rectus (dotted arrow). Also noted is fracture of the lateral wall and floor
of the left orbit with extensive retrobulbar hemorrhage and pneumo-orbit. Left preseptal edema also noted

A B

C D

Figs 25.4A to D: (A and B) Axial CT scan (in bone window setting) and (C and D) Coronal CT scan (in bone
window setting) showing fractures of the roof of the left orbit extending up to the rim. Fractures of the frontal
bone, lateral wall, floor and medial wall of the left orbit are also noted
314 Section 3 Disorders of Orbit
Plain Film (Water’s View)
• Fracture lines are seen involving the inferior orbital
rim, maxillary antrum and zygomatic arch with
separation of the frontozygomatic suture
• Indirect signs are soft tissue density around the
inferior rim and maxillary antrum
• Additionally, submental view may be obtained to
assess the degree of displacement or fracture
instability.
CT Scan
Axial and coronal plane images are useful for detailed
assessment of the fractured fragments, rotation and
extension.
LEFORT TYPE FRACTURES
• LeFort classified facial fractures into types I, II and
III. Pure LeFort type is rare in clinical practice
• More commonly, there is an overlap in the extent of
fractures and the degree of injury is classified as the
more severe fracture type.
Plain Film
It may identify the multiple fractures.
CT Scan
This is crucial to fully evaluate the extent and degree of
fractures, displacement, and comminution.
ORBITAL APEX AND CANAL FRACTURES
• Orbital apex and canal fractures may be isolated or
can occur in combination with other complex fractures
• These fractures may extend into the sphenoid sinus
• Optic nerve damage may be encountered because of
the impaction of the bony fragments on the optic
nerve, due to soft tissue swelling or injury to the optic
nerve (bleeding or shear injury).
TRAUMATIC OPTIC NEUROPATHY
• It is important that diagnosis of traumatic optic
neuropathy must be recognized because immediate
intervention may prevent permanent deficit
• Traumatic optic neuropathy can be categorized into:
– Direct optic neuropathy
– Anterior indirect optic neuropathy
– Posterior indirect optic neuropathy (most
common)
– Chiasmal injury.
CT Scan
• It is the modality of choice
• 1 to 2 mm thick plain axial and coronal CT scan of the
orbits through the optic canal should be obtained.
MR Imaging
• MRI is done to look for soft tissue injury after CT scan
rules out bony fragment impinging on the optic canal
• Edema or contusion of the optic nerve (hyperintense
in T2-weighted images) or perioptic hematoma is
better delineated on MRI.
ORBITAL HEMORRHAGE
• Traumatic orbital hemorrhage may result from direct
transmission of force that tears small orbital vessels
or by indirect transmission where the resulting
fracture fragments disrupt the vessels
• A blunt or penetrating injury may cause traumatic
orbital hemorrhage. It may occur in one of the five
compartments within the orbit: Retrobulbar
(intraconal), extraconal, subperiosteal, sub-Tenon’s
space and within the nerve sheath (subdural). It can
be localized or diffuse
• The clinical signs may include increased intraorbital
pressure, proptosis, restricted ocular movements,
optic nerve dysfunction with afferent pupillary defect,
and possibly retinal vascular occlusion.
CT Scan
• It helps in evaluating the size, extent and location of
orbital hemorrhage
• Retrobulbar hemorrhage usually surrounds the optic
nerve and has a four-leaf clover appearance on coronal
imaging
• Extraconal hemorrhage has a more linear appearance.
• Sub-Tenon’s hemorrhage confirms to the shape of the
globe
• Subdural optic nerve sheath hemorrhage must be
differentiated from extradural (perineural sheath)
hematoma. Epidural hematoma is a localized
intraconal hematoma whereas subdural hemorrhage
appears as a “tram-track” abnormal density surroun-
ding the optic nerve.
MR IMAGING
• MRI is superior to CT in soft tissue delineation and
evaluation of orbital hemorrhage with respect to
extent, size and age of blood
• CT may not be able to differentiate between optic
nerve sheath subdural and extradural hematoma
 Chapter 25 Orbital Trauma 315

A B

Figs 25.5A to C: (A and B) Coronal CT scans of the orbit

and (C) Axial CT scan showing fracture roof of the left orbit
with elevated fragment and dural tear resulting in CSF
collection in the left superior orbit displacing the superior rectus
and levator muscle. Note: Left frontal contusion injury (dotted
C arrow) and multiple preseptal bone fragments
316 Section 3 Disorders of Orbit
• Subperiosteal hemorrhage is linear and limited by
sutural attachments. The roof of the orbit is the most
common site and these hemorrhages occur most often
in children and young adults because of the loose
periosteal attachment. They may be quite large and
result in gross proptosis.
BIBLIOGRAPHY
1. Freund M, Hahnel S, Sartor K. The value of magnetic
resonance imaging in the diagnosis of orbital floor
fractures. Eur Radiol 2002;12(5):1127-33.
2. Go JL, Vu VN, Lee KJ, Becker TS. Orbital trauma.
Neuroimaging. Clin N Am 2002;12(2):311-24.
3. Healy JF. Computed tomography of orbital trauma.
J Comput Tomogr 1982;6(1):1-10.
4. Kolk A, Pautke C, Wiener E, Ploder O, Neff A. A novel
high-resolution magnetic resonance imaging microscopy
coil as an alternative to the multislice-computed
tomography in postoperative imaging of orbital fractures
and computer-based volume measurement. J Oral
Maxillofac Surg 2005;63(4):492-8.
5. Mauriello JA Jr, Lee HJ, Nguyen L. CT of soft tissue injury
and orbital fractures. Radiol Clin North Am 1999;37(1):
241-52.
6. Maus M. Update on orbital trauma. Curr Opin
Ophthalmol 2001;12(5):329-34.
7. Tonami H, Yamamoto I, Matsuda M, Tamamura H, Yokota
H, Nakagawa T, Takarada A, Okimura T. Orbital fractures:
Surface coil MR imaging. Radiology 1991;179(3):789-94.
8. Wiener E, Kolk A, Neff A, Settles M, Rummeny E.
Evaluation of reconstructed orbital wall fractures: High-
resolution MRI using a microscopy surface coil versus
16-slice MSCT. Eur Radiol 2005;15(6):1250-5.
 Chapter 25 Orbital Trauma 317

Fig. 25.6: Coronal CT scans of the orbit following intrathecal

contrast to evaluate CSF leak shows elevated fracture roof of
the right orbit (arrow) and blow out fracture of the floor. Patient
had CSF rhinorrhea due to fracture of the cribriform plate. Note:
The contrast in the subarachnoid space and site of leak (small
arrow)

A B

Figs 25.7A and B: Coronal CT scan of the orbit in bone window setting: (A) Showing fracture of the lateral wall with a blow in
fragment and (B) Showing fracture of the lateral wall at the apex narrowing the apex
318 Section 3 Disorders of Orbit

OPTIC CANAL FRACTURE

Fig. 25.8: Coronal CT scan at the level of the optic canals in

bone window settings shows fracture walls of the left optic canal
and bone fragments within the canal (arrow)

A B

Figs 25.9A and B: Nasoethmoid complex fractures: (A and B) Axial CT of the orbit at the level of the floor showing fractures of the
nasoethmoid and orbital complex. Note: The fracture of the walls of the nasolacrimal canals (arrow)
 Chapter 25 Orbital Trauma 319

ZYGOMATIC COMPLEX FRACTURE

Fig. 25.10: Three-dimensional (3D) CT of the skull showing the left zygomatic arch and zygoma fractures (arrows)

A B

Figs 25.11A and B: Axial CT scans of the orbit showing fracture medial and lateral wall of left orbit with retrobulbar hemorrhage
(dotted arrow) and pneumo-orbit
320 Section 3 Disorders of Orbit

A B

Figs 25.12A and B: (A) Axial CT scan and (B) Coronal reformatted image of a 4-year-old mentally retarded child with history of
repeated trauma showing hyperdense extraconal hematoma in the left superior orbit—subperiosteal hematoma

Fig. 25.13: Axial CT scan of the orbit showing well-defined

hypodense cystic lesion months after the trauma—organized
hematoma (arrow)
SECTION 4
Visual Pathway Lesions:
Optic Nerve Disorders
SECTION OUTLINE
26. Anatomy and Clinical Features
27. Congenital Optic Nerve Anomalies
28. Papilledema
29. Optic Neuritis
30. Infiltrative Optic Neuropathy
31. Traumatic Optic Neuropathy
32. Radiation Optic Neuropathy
Chapter 26
ANATOMY
• The optic nerve is the second cranial nerve and is
derived from an outpouching of the diencephalon
during embryonic development
• The optic nerve is 5 cm long from eye to optic chiasm
• It is divided into four portions:
1. Intraocular
2. Intraorbital
3. Intracanalicular
4. Intracranial
• The optic nerve is ensheathed in all three meningeal
layers (dura, arachnoid, and pia mater)
• The fibers from the retina run along the optic nerve
to nine primary visual nuclei in the brain, resulting
major relay inputs into the primary visual cortex
• The optic nerve is chiefly composed of retinal ganglion
cell axons
• It leaves the orbit via the optic canal, running postero-
medially towards the optic chiasm where there is a
partial decussation of fibers from the nasal visual
fields of both eyes
• Most of the axons of the optic nerve terminate in the
lateral geniculate nucleus from where information is
transmitted to the visual cortex
• Its diameter increases from about 1.5 mm within the
eye, to 3.5 mm in the orbit to 4.5 mm intracranially.
• The optic nerve component lengths are 1 mm in the
intraocular part, 2 to 3 cm in the orbit, 9 mm in the
intracanalicular part and 3 to 16 mm in the intracranial
part before joining the optic chiasm
• In the optic chiasm, more than half of the fibers cross
to form the optic tracts
Anatomy and
Clinical Features
• Most of these fibers terminate in the lateral geniculate
body
• From the lateral geniculate body, fibers of the optic
radiation arises to the visual cortex in the occipital
lobe of the brain.
CLINICAL FEATURES AND EVALUATION
OF OPTIC NERVE DYSFUNCTION
• Any optic nerve disorder causes vision loss, color
vision deficit , relative afferent pupillary defect, visual
field loss, reduced brightness and contrast sensitivity
• Vision loss can vary from mild impairment to no
perception of light
• Vision recording can be done by Snellen’s chart
• Dyschromatopsia is a very common symptom. It is
tested by pseudoisochromatic color plates like
Ishihara or HRR plates
• Presence of RAPD is a hallmark for optic nerve
dysfunction, but it can be appreciated in other causes
of profound vision loss also
• On fundus examination—optic nerve head
appearance can vary from swollen disc to temporal
or diffuse pallor. There are conditions like retrobulbar
neuritis where the optic nerve head appears normal,
but has all signs of optic nerve dysfunction
• Optic nerve field defects can range from enlarged blind
spot, central/centrocecal defects, to altitudinal defects, etc.
• Reduced brightness sensitivity will be complained by
the patients as washed-out appearance
• Contrast sensitivity can be tested by Pelli Robson chart
• Electrophysiological testing—visual evoked
potentials is an objective way of recording the
functional integrity of visual pathways. The p100
324 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
latency and the amplitude of the pathwave are
affected in various optic nerve disorders
• Ocular ultrasound and fundus fluorescein angio-
graphy are useful to differentiate a true optic disc
edema from pseudoedema. On FFA, a swollen disc
will reveal profound disc leak
• Optical coherence tomogram is a newer tool which can
give more details about the retinal nerve fiber layers
(RNFL) and it can be used to measure the swelling of
optic nerve edema and the response to treatment.
IMAGING ANATOMY
• Magnetic resonance imaging is the modality of choice
to image the visual pathways especially the optic
nerves
Fig. 26.1: Axial FIESTA sequence (0.8 mm slice thickness) taken at the
level of the optic nerve showing the intraocular and intraorbital optic nerve
• The imaging plane should be parallel to the optic
nerve, i.e. the neuro-ocular plane (NOP), which is
the plane passing through the optic nerve and the
optic canals with the patient fixing in a primary gaze
• This plane is best to study the details of the intra-
orbital structures as the NOP is parallel to the optic nerve
• On MRI, the optic nerve shows signals similar to white
matter in both T1 and T2-weighted images and the
surrounding CSF in the sheath is of low intensity in
T1 and high intensity in T2-weighted images
• The dural sheath can be differentiated from the CSF
rim only in T2-weighted images. It is seen as a low
signal rim against the bright signal of CSF
• MRI sections obtained parallel to the posterior
commisure-obex (cp-ob) plane are best to evaluate
the details of the intracranial optic nerve.
 Chapter 26 Anatomy and Clinical Features 325

Fig. 26.2: Axial FIESTA demonstrating the intracanalicular optic nerve, chiasm and optic tracts

A B

Figs 26.3A and B: (A) Orientation of NFL in retina and (B) The optic nerve towards chiasm

Fig. 26.4: Optic disc edema Fig. 26.5: Disc pallor

Chapter 27
Congenital Optic Nerve Anomalies
OPTIC DISC COLOBOMA/
MORNING GLORY SYNDROME
• Optic disc coloboma results because of incomplete or
abnormal coaptation of proximal end of embryonic
fissure. It is typically a sharply delimited, glistening
white, bowl-shaped excavation that is decentered
inferiorly in an enlarged optic disc
• It can be unilateral or bilateral. May present as
sporadic cases or autosomal dominant inheritance
• Three types of optic nerve colobomas can occur:
1. Isolated coloboma of the optic nerve
2. Retinochoroidal coloboma
3. Fuchs’ coloboma
• Colobomas have been associated with nonocular
abnormalities including cardiac defects, dysplastic
ears (CHARGE syndrome), facial palsy and trans-
phenoidal encephalocele
• The anomaly may occur in normal-sized eyes as well
as in microphthalmos
• Uncommon, unilateral or bilateral congenital
conditions caused by incomplete closure of the
embryonic fissure such as Meckel’s syndrome
(bilateral ocular disorders associated with
multisystem developmental abnormalities) and
Aicardi’s syndrome (bilateral ocular condition
associated with disorders of cranial, choroidal-
pigment epithelial and skeletal development) can be
associated with it.
CLINICAL FEATURES
• Visual field defects
• Relative afferent pupillary defect (RAPD)
• Signs:
– Enlarged, sharply circumscribed, glistening white
and deeply excavated optic disc which usually
occurs inferiorly
– May mimic glaucomatous cupping in mild cases
– Can develop serous macular detachment
• Other associated ocular findings may include:
– Coloboma of the lens, ciliary body and choroid
– Microphthalmos with orbital cyst
– Persistent hyaloid artery
– Retinal dysplasia
• May also be part of systemic anomalies including:
– CHARGE association (coloboma, congenital heart
disease, choanal atresia, growth retardation and
ear defects)
– Morning glory syndrome is a common anomaly
that phenotypically simulates a coloboma but can
be differentiated by its characteristic features like
normal disc within an excavation, symmetric, with
peripapillary pigmentary disturbances and
anomalous blood vessels, etc.
– Morning glory syndrome can be associated with
basal encephaloceles.
IMAGING (CT/MRI)
• CT and MRI appearance of optic disc coloboma
includes a posterior defect with optic disc excavation
• The morning glory anomaly typically demonstrates
a funnel-shaped deformity of the posterior globe
 Chapter 27 Congenital Optic Nerve Anomalies 327

Fig. 27.1: Fundus photograph of right eye showing optic disc Fig. 27.2: Axial plain CT scan of the orbit showing left optic
coloboma nerve coloboma (black arrow) and conical defect at the right
optic disc (white arrow) suggestive of optic disc coloboma

Fig. 27.3: Axial plain CT scan of the orbit showing left optic
nerve coloboma (black arrow) with a focal defect at the optic
disc

A B

Figs 27.4A and B: (A) Axial FLAIR and (B) Sagittal T1-weighted images of the orbit showing focal outpouching at the optic nerve
head surrounding the optic nerve (black arrows)—optic nerve coloboma
328 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
• The colobomatous cysts associated with coloboma of
the optic nerve head can be identified on CT and MRI
imaging.
MRI of the brain should be done in all patients to
look for CNS malformations especially midline defects.
Recommendation
MRI brain and orbit
BIBLIOGRAPHY
1. Auber AE, O'Hara M. Morning glory syndrome: MR
imaging. Clin Imaging 1999;23(3):152-8.
2. Berk AT, Yaman A, Saatci AO. Ocular and systemic
findings associated with optic disc colobomas. J Pediatr
Ophthalmol Strabismus 2003;40(5):272-8.
3. Cassier C, Kien P, Caille JM. CT images in constitutional
abnormalities of the posterior pole of the eye: Optic disc
coloboma, posterior pole staphyloma. J Neuroradiol
1986;13(1):11-21.
4. Gopal L, Badrinath SS, Kumar KS, Doshi G, Biswas N.
Optic disc in fundus coloboma. Ophthalmology 1996;
103(12):2120-6; discussion 2126-7.
5. Jacobs M, Taylor D. The systemic and genetic significance
of congenital optic disc anomalies. Eye 1991; 5 (Pt 4):470-
5. Review.
6. Mafee MF, Jampol LM, Langer BG, Tso M. Computed
tomography of optic nerve colobomas, morning glory
anomaly, and colobomatous cyst. Radiol Clin North Am
1987;25(4):693-9.
7. Murphy BL, Griffin JF. Optic nerve coloboma (morning glory
syndrome): CT findings. Radiology 1994;191(1):59-61.
 Chapter 27 Congenital Optic Nerve Anomalies 329

Figs 27.5A and B: (A) Axial T1-weighted image

A B
showing bilateral posterior conical deformity with
out-pouchings arising from the (L) anterior optic
nerve suggestive of optic nerve coloboma and
(B) Midsagittal T1-weighted image of the brain in
the same patient showing agenesis of corpus
callosum (arrow)

A B C

Figs 27.6A to C: Disc coloboma with sphenoid encephalocele: (A) Axial plain CT scan of the orbit showing conical defect at the
left optic disc (arrow) suggestive of coloboma, (B) Coronal bone window and (C) Soft tissue of the brain of the same patient at the
level of the sella showing defect in the sphenoid bone with herniation of the pituitary and CSF through the defect consistent with
sphenoid encephalocele (black arrows)

Fig. 27.7: Fundus photograph showing a morning glory disc Fig. 27.8: Morning glory syndrome. Axial plain CT scan of the
that is markedly enlarged, recessed and elevated centrally within orbit showing funnel-shaped defect at the left optic disc (black
confines of funnel-shaped peripapillary excavation. A wide arrow) suggestive of morning glory disc
annulus of chorioretinal pigmentary disturbance surrounds the
disc within the excavation. The blood vessels appear more in
number and arise from periphery of the disc and run an
abnormally straight course over peripapillary retina
330 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
CONGENITAL OPTIC NERVE APLASIA
• It is a rare congenital malformation characterized by
the congenital absence of the optic nerve, central
retinal vessels and retinal ganglion cells that is seen
most often in a unilaterally malformed eye of an
otherwise healthy person
• Bilateral optic nerve aplasia is rare, and is associated
with central nervous system anomalies, and with
other multiple, often severe congenital malformations
• It can be associated with microphthalmos of the
involved eye
• Fundus examination reveals the absence of retinal
vessels, retina and optic disc.
IMAGING
• MRI should be done to evaluate the optic nerve and
brain
• The globe and the bony orbit may appear smaller
• It may show an absent optic nerve and chiasm
• Unilateral optic nerve aplasia will show hemichiasmal
hypoplasia on the ipsilateral side
• Hypopituitarism and ectopic posterior pituitary may
be seen.
BIBLIOGRAPHY
1. Blanco R, Salvador F, Galan A, Gil-Gibernau JJ. Aplasia
of the optic nerve: Report of three cases. J Pediatr
Ophthalmol Strabismus 1992;29(4):228-31.
2. Brodsky MC, Atreides SP, Fowlkes JL, Sundin OH. Optic
nerve aplasia in an infant with congenital hypopituitarism
and posterior pituitary ectopia. Arch Ophthalmol 2004;
122(1):125-6.
3. Chat L, Hertz-Pannier L, Roche O, Boddaert N, Baraton J,
Brunelle F. Value of MRI in the diagnosis of unilateral
optic nerve aplasia: A case report. J Radiol 2002;83 (12 Pt
1):1853-5.
4. Margo CE, Hamed LM, Fang E, Dawson WW. Optic nerve
aplasia. Arch Ophthalmol 1992;110(11):1610-3.
5. Scott IU, Warman R, Altman N. Bilateral aplasia of the
optic nerves, chiasm, and tracts in an otherwise healthy
infant. Am J Ophthalmol 1997;124(3):409-10.
6. Weiter JJ, McLean IW, Zimmerman LE. Aplasia of the optic
nerve and disc. Am J Ophthalmol 1977;83(4):569-76.
 Chapter 27 Congenital Optic Nerve Anomalies 331

A B

C D

Figs 27.9A to D: Bilateral optic nerve aplasia: (A) Axial CT scan and (B) Coronal CT scan at mid-orbit level showing absence of
the optic nerve (arrows), (C) Axial CT scan and (D) Coronal CT scan at the level of the chiasm showing absent chiasm (white
arrows)
332 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
HYPOPLASTIC OPTIC DISC
• Hypoplastic optic disc is characterized by an
abnormally small optic nerve head usually one-half
to one-third of normal size
• It can be bilateral in 56 to 92 percent cases
• The condition may occur as an isolated anomaly in
the normal eye, or in a grossly malformed eye
• It can be associated with a heterogeneous group of
disorders most commonly involving the midline
structures of the brain
• The underlying pathology is the reduction in the
retinal ganglion cells and their nerve fibers. It ranges
from mild to severe depending on the degree of axonal
injury
• Patients have some degree of optic nerve dysfunction
with visual acuity ranging from normal-to-severe
impairment
• It is a clinical diagnosis and is characterized by early
onset strabismus, poor vision, nystagmus and a
relative afferent pupillary defect and peripheral or
arcuate visual field defects
• The small disc appears gray or pale and is often
surrounded by a yellowish mottled peripapillary halo,
bordered by a ring of altered pigmentation, the so
called ‘double-ring sign’. The outer ring is the actual
site of junction between the sclera and lamina cribrosa
and the inner ring represents the termination of an
abnormal extension of retina and pigment epithelium
over the lamina cribrosa
• Systemic associations—neurological malformations
include basal encephaloceles, hypoplasia of cerebellar
vermis, cystic dilatation of fourth ventricle, arachnoid
cysts, posterior fossa cysts and anterior visual
pathway space occupying lesions.
SEPTO-OPTIC DYSPLASIA
(DE MORSIER’S SYNDROME)
• De Morsier’s syndrome (septo-optic dysplasia)
includes a triad of short stature, nystagmus, and
hypoplasia of optic discs and chiasm. Midline
developmental anomalies, including absence of septum
pellucidum, thinning or agenesis of corpus callosum
and dysplasia of anterior third ventricle are seen.
IMAGING FINDINGS
• Absence of septum pellucidum
• Hypoplastic optic nerve (cross-sectional diameter is
reduced to less than 2.9 mm) and chiasm
• Enlarged anterior recess of third ventricle
• Squaring of the frontal horns or box-like frontal horns.
Associated findings: 50 percent have schizencephaly
and some have azygous anterior cerebral artery.
MRI is the imaging modality of choice due to its
greater parenchymal definition and multiplanar
capabilities.
BIBLIOGRAPHY
1. Birkebaek NH, Patel L, Wright NB, Grigg JR, Sinha S, Hall
CM, Price DA, Lloyd IC, Clayton PE. Optic nerve size
evaluated by magnetic resonance imaging in children with
optic nerve hypoplasia, multiple pituitary hormone
deficiency, isolated growth hormone deficiency, and
idiopathic short stature. J Pediatr 2004;145(4):536-41.
2. Brodsky MC, Glasier CM. Optic nerve hypoplasia. Clinical
significance of associated central nervous system
abnormalities on magnetic resonance imaging. Arch
Ophthalmol 1993;111(1):66-74. Erratum in: Arch
Ophthalmol 1993;111(4):491.
3. Hellstrom A, Wiklund LM, Svensson E. Diagnostic value
of magnetic resonance imaging and planimetric
measurement of optic disc size in confirming optic nerve
hypoplasia. J AAPOS 1999;3(2):104-8.
4. Hoyt CS, Good WV. Do we really understand the
difference between optic nerve hypoplasia and atrophy?
Eye 1992; 6 (Pt 2):201-4. Review.
5. Phillips PH, Spear C, Brodsky MC. Magnetic resonance
diagnosis of congenital hypopituitarism in children with
optic nerve hypoplasia. J AAPOS 2001;5(5):275-80.
6. Weiss AH, Kelly JP. Acuity, ophthalmoscopy, and visually
evoked potentials in the prediction of visual outcome in
infants with bilateral optic nerve hypoplasia. J AAPOS
2003;7(2):108-15.
 Chapter 27 Congenital Optic Nerve Anomalies 333

Fig. 27.10: A 15-year-old female presented with no perception of light in

the right eye. Fundus showed a hypoplastic optic disc with dilated and
tortuous retinal vessels

Figs 27.11A and B: (A) Axial CT of

orbit showing small caliber of the
optic nerve bilaterally (black arrow)
and (B) Axial CT scan of the brain
at the level of the lateral ventricles
showing absent septum pellucidum
with squaring of the frontal horns
A B (white arrow)

Figs 27.12A and B: (A) Coronal T1-

weighted image and (B) Sagittal T1-
weighted image of the brain of the
above patient showing hypoplastic
chiasm (white arrows) and absent
septum pellucidum with squaring of
A B the frontal horns (black arrow)
334 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 27.13A to C: (A) Coronal T1-weighted image at the mid-orbit level, (B) Coronal T2-weighted image at the level of the chiasm
and (C) Sagittal T1-weighted image shows small caliber of the optic nerves, chiasm with absent septum pellucidum resulting in
box-like frontal horns—septo-optic dysplasia

OPTIC NERVE MENINGOCELE
• The term optic nerve sheath meningocele has been
proposed by Garrity and Forbes to describe primary
CSF cysts of the optic nerve sheath, without apical
mass or malformation of the cranio-orbital junction
• It occurs in the absence of raised intracranial
pressure
• It is also termed optic hydrops, optic nerve cyst,
arachnoid cyst, perioptic hygroma, and dural ectasia
• It is characterized by a saccular dilatation of the
meninges surrounding the optic nerve
• It can be either primary or secondary in association
with orbital processes, such as meningioma, optic
nerve glioma, and hemangioma and bony orbital
processes, viz osteopetrosis
• Presenting symptoms are often related to involvement
of the optic nerve, with a slow or rapid decrease of
visual acuity.
IMAGING
• CT and MRI studies reveal a tubular-cystic enlargement
of the optic nerve/optic sheath complex. MR will show
the normal optic nerve separately, helping to
differentiating it from cystic optic nerve glioma
Chapter 27 Congenital Optic Nerve Anomalies 335
• It may be associated with empty sella and enlarged
subarachnoid cisterns
• Radiological differential diagnosis should include
optic nerve tumors such as gliomas, meningiomas and
arachnoid cysts involving the optic nerve sheath.
BIBLIOGRAPHY
1. Doi J, Uchino A, Kato A, Koga T, Kudo S. Dural ectasia of
the optic nerve sheath in neurofibromatosis type 1: MRI
manifestations. Radiat Med 1997;15(3):197-8.
2. Garrity JA, Trautmann JC, Bartley GB, Forbes G, Bullock
JD, Jones TW Jr, Waller RR. Optic nerve sheath
meningoceles: Clinical and radiographic features in 13
cases with a review of the literature. Ophthalmology
1990;97(11):1519-31.
3. Jungkim S, Khurshid SG, Fenton S. Dural ectasia of the optic
nerve sheath. Acta Ophthalmol Scand 2005;83(2):266-7.
4. Lovblad KO, Remonda L, Ozdoba C, Huber P, Schroth G.
Dural ectasia of the optic nerve sheath in
neurofibromatosis type 1: CT and MR features. J Comput
Assist Tomogr 1994;18(5):728-30.
5. Lunardi P, Farah JO, Ruggeri A, Nardacci B, Ferrante L,
Puzzilli F. Surgically verified case of optic sheath nerve
meningocele: Case report with review of the literature.
Neurosurg Rev 1997;20(3):201-5.
336 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 27.14A to C: A 2-month-old child presented with bilateral proptosis: (A) Axial CT scan shows bilateral enlarged perioptic
spaces, (B) Axial T1-weighted image and (C) Coronal T2-weighted image shows eccentrically located normal optic nerve (arrows)
with saccular dilatation of the meninges—optic nerve meningocele

OPTIC DISC DRUSEN
• Optic disc drusen is a benign, usually bilateral cause
of pseudopapilledema and is occasionally inherited
as an autosomal dominant condition. Disc drusen are
composed of small conglomerates of mucopoly-
saccharides and proteinaceous material that becomes
calcified with advancing age. They may be
superficially located or deeply buried
• Superficially located drusen are on the surface of the
disc mostly in nasal region or scattered throughout
the disc. Ophthalmoscopically, they appear as
refractile tapioca like bodies
• Deeply buried drusen are located between the surface
of the disc and the lamina cribrosa. They cause
anomalous disc elevation (pseudopapilledema) giving
the disc a yellowish moth-eaten appearance and may
be associated with anomalous retinal artery branching
pattern. They may be seen on retroillumination
• Optic disc drusen may vary in size and number,
smaller drusen appear to coalesce and form larger
aggregates
• Ocular complications: Most patients with optic drusen
are asymptomatic but a few of them can have associated
visual field defects, peripapillary hemorrhages,
subretinal hemorrhages, serous maculopathy, ischemic
optic neuropathy and retinal vascular occlusions
• Systemic associations: Optic nerve drusens are seen
more often in patients with retinitis pigmentosa
(Peripapillary drusen were present in 16 percent of
the patients with Usher syndrome). They are also seen
more frequently in patients of Riley-Smith syndrome,
migraine, space occupying lesions in the brain and
angioid streaks.
IMAGING FINDINGS
• Most sensitive diagnostic technique is A and B scan
ultrasonography, which can demonstrate disc
Chapter 27 Congenital Optic Nerve Anomalies 337
elevation and shows a high reflective spike even at
low gain settings
• CT scan is the imaging modality of choice—the
calcified drusen is usually not more than 1-1.5 mm in
thickness and hence when imaging patients with
suspected drusen, it is advisable to take thin sections,
i.e. 1-2 mm thick
• In early childhood, CT scan should be carefully
evaluated, as the drusen are not calcified enough to
be visible. They may be seen slightly denser lesions
than the optic nerve
• A classical drusen is seen as a discrete, rounded high
density or calcified body that may be confined to the
optic disc surface or found at any level within the
prelaminar zone of the optic nerve
• Optic disc drusen are difficult to visualize by MR
imaging
• Progressive field loss in patient with optic disc drusen
warrants neuroimaging to rule out occult central
nervous system lesions.
Recommended—CT orbit.
BIBLIOGRAPHY
1. Auw-Haedrich C, Staubach F, Witschel H. Optic disc
drusen. Surv Ophthalmol 2002;47(6):515-32. Review.
2. Kheterpal S, Good PA, Beale DJ, Kritzinger EE. Imaging
of optic disc drusen: A comparative study. Eye 1995;9
(Pt 1):67-9.
3. Kurz-Levin MM, Landau K. A comparison of imaging
techniques for diagnosing drusen of the optic nerve head.
Arch Ophthalmol 1999;117(8):1045-9.
4. Neil RM, Nancy JN (Eds). Walsh and Hoyt’s clinical neuro-
ophthalmology: The essentials, 5th edn; 1999.
5. Wilkins JM, Pomeranz HD. Visual manifestations of
visible and buried optic disc drusen. J Neuro-ophthalmol
2004;24(2):125-9.
338 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

Fig. 27.15: Fundus photograph of the right eye showing an

anomalous elevation of disc with anomalous branching pattern
of vessels overlying the disc without obscuration. A zero gain
ultrasonogram detected the presence of optic disc drusen

Fig. 27.16: Plain Axial CT scan of the orbits showing tiny rounded
calcifications at the optic discs bilaterally

Fig. 27.17: Plain Axial CT scan of the orbits showing tiny rounded
calcification at the left optic disc with conical deformity at the
disc—morning glory disc with disc drusen
Chapter 28
• Papilledema is bilateral swelling of the optic disc
secondary to increased intracranial pressure (ICP),
which can be due to a space occupying lesion or
idiopathic raised cerebrospinal fluid (CSF) pressure
• Causes:
1. Intracranial neoplasm
2. Focal or diffuse cerebral edema due to infections,
trauma and vascular
3. Hydrocephalus
4. Craniostenosis
• If none of above causes are found, then the condition
of a benign rise in intracranial pressure (idiopathic
intracranial hypertension) should be suspected
• Patients usually present with symptoms of raised
intracranial pressure such as headache, vomiting and
diplopia (sixth nerve palsy—false localizing sign).
Ocular symptoms include transient loss of vision,
decreased vision, loss of field of vision, etc.
• The vision remains normal till the late stage. The
common visual field defects are the enlarged blind
spots, arcuate defects and constricted fields. The later
two defects are seen in advanced condition
• Clinically, the optic disc will show hyperemia, edema,
hemorrhages and exudates that will be seen in various
stages from early to established and chronic
papilledema.
IMAGING
CT or MRI (preferable) should be done as early as possible
as papilledema is a neurological emergency.
• Imaging plays a crucial role in the diagnosis and
management of these patients
• The cause of papilledema is usually evident on
imaging
Papilledema
• MRI is the modality of choice, as intracranial lesions
are better delineated on MRI without contrast. MRI
can characterize and localize the lesions better than
CT scan
• If MRI cannot be done, then a contrast-enhanced CT
should be done
• If there is no intracranial mass lesion, then one should
look for features suggestive of idiopathic intracranial
hypertension like:
– Widening of the perioptic space (well appreciated
on a T2 fat-suppressed coronal MRI images)
– Empty sella
– Prominent CSF space within the Meckle’s cave
– Slit-like ventricles
– Effacement of cisterns
• MR venogram should be done to rule out cerebral
venous sinus thrombosis in the absence of intracranial
lesions. MRI will show the normal flow void signal in
the dural venous sinuses being replaced by iso- to
hyperintense signal in T1 and T2-weighted images.
MR venogram will show a filling defect corres-
ponding to the signal changes seen on MRI.
RECOMMENDATIONS
• MRI of the brain should be done in patients with
papilledema
• MRI brain and orbit with MR venogram should be
done in all patients suspected to have idiopathic
intracranial hypertension.
BIBLIOGRAPHY
1. Brodsky MC, Glasier CM. Magnetic resonance
visualization of the swollen optic disc in papilledema.
J Neuroophthalmol 1995;15(2):122-4.
340 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

2. Brodsky MC, Vaphiades M. Magnetic resonance imaging
in pseudotumor cerebri. Ophthalmol 1998;105 (9):1686-93.
3. Brodsky MC. MR of papilledema. Am J Neuroradiol 1997;
18(8):1592-4.
4. Friedman DI, Jacobson DM. Idiopathic intracranial
hypertension. J Neuro-ophthalmol 2004;24(2):138-45.
5. Friedman DI. Papilledema and pseudotumor cerebri.
Ophthalmol Clin North Am 2001;14(1):129-47.
6. Friedman DI. Pseudotumor cerebri. Neurosurg Clin N Am
1999;10(4):609-21, viii. Review.
7. Lee AG, Brazis PW. Magnetic resonance venography in
idiopathic pseudotumor cerebri. J Neuro-ophthalmol
2000;20(1):12-3.
8. Leker RR, Steiner I. Features of dural sinus thrombosis
simulating pseudotumor cerebri. Eur J Neurol 1999;6
(5):601-4.
9. Manfre L, Lagalla R, Mangiameli A, Lupo F, Giuffre G,
Ponte F, Cardinale AE. Idiopathic intracranial hyper-
tension: Orbital MRI. Neuroradiol 1995;37(6):459-61.
10. Mathews MK, Sergott RC, Savino PJ. Pseudotumor cerebri.
Curr Opin Ophthalmol 2003;14(6):364-70. Review.
11. Suzuki H, Takanashi J, Kobayashi K, Nagasawa K,
Tashima K, Kohno Y. MR imaging of idiopathic intra-
cranial hypertension. Am J Neuroradiol 2001;22(1):
196-9.

A B

C D

Figs 28.1A to D: (A to C) Coronal T2-weighted image at the level of the orbit, Meckle’s cave and sella respectively and (D) Axial T1-
weighted image showing widening of the perioptic CSF spaces bilaterally (black arrow), widened Meckle’s cave (long black
arrow) and empty sella (arrowhead)—idiopathic intracranial hypertension
 Chapter 28 Papilledema 341

A B

Figs 28.2A to C: (A) Axial FLAIR, (B) Coronal T2-weighted image and
(C) Sagittal maximum intensity projection (MIP) of 2D-TOF MR
venogram; Axial FLAIR showing normal flow void of the superior sagittal
sinus being replaced by hyperintense signal (white arrow). Coronal
T2-weighted image showing widening of the perioptic CSF space due
to raised ICP. Sagittal MIP showing absent flow in the superior sagittal
sinus (black arrow) (Fig. C) suggestive of superior sagittal sinus
C thrombosis
342 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 28.3A to C: A 22-year-old male presented with headache and blurring of vision, clinical examination revealed papilledema:
(A) Axial T2-weighted image, (B) Sagittal T2-weighted showing an osteolytic lesion in the occipital bone with an epidural abscess
compressing and invading the adjacent superior sagittal sinus and (C) Sagittal MIP image of MR venogram shows filling defect in
the superior sagittal sinus corresponding to the bony lesion—tuberculous osteitis with epidural abscess
 Chapter 28 Papilledema 343

A B

Figs 28.4A and B: A 30-year-old HIV positive female presented with headache and visual loss: (A) Coronal
postcontrast T1-weighted image and (B) Sagittal postcontrast T1-weighted image showing patchy enhancement
in the suprasellar cistern and ependyma of the fourth ventricle. Narrowing of the cerebral aqueduct is seen
resulting in hydrocephalus (arrow)—tuberculous meningitis

A B

Figs 28.5A and B: A 52-year-old female presented with acute onset severe headache, clinical examination
showed pailledema: (A) Axial FLAIR image at midbrain level showing edema at the dorsal midbrain (arrow) and
altered signal in the (L) temporal cortex (dotted arrow) and (B) Axial contrast T1-weighted image. There is an
enhancing lesion in the dorsal midbrain (arrow) compressing the third ventricle and causing hydrocephalus.
Also note the abnormal meningeal enhancement—systemic examination revealed mass in the breast—a case
of carcinoma breast with meningeal carcinomatosis
344 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 28.6A and B: A 70-year-old male presented with headache and papilledema: (A) Axial T2-weighted image at the level of
the cerebellopontine angle, reveals a large heterogeneous mass lesion in the left CP angle cistern and (B) Axial FLAIR image at
the level of the lateral ventricles reveals hydrocephalus—acoustic neurofibroma causing obstructive hydrocephalus
Chapter 29
It is an acute inflammation of the optic nerve. It may
present with a swollen optic disc (papillitis) or normal
looking optic disc (retrobulbar neuritis).
CAUSES
• Optic neuritis can be associated with systemic
demyelinating diseases. In the absence of associated
systemic demyelination, it is called idiopathic
demyelinating optic neuritis
• Infections tuberculosis, syphilis, mumps, measles,
Lyme disease, HIV infection, toxoplasmosis,
cytomegalovirus or herpes zoster virus and
parainfections
• Inflammatory (sarcoidosis, polyarteritis nodosa,
systemic lupus erythematosus)
• Postvaccination
• Miscellaneous: Acute posterior multifocal placoid
pigment epitheliopathy, histoplasmosis, cysticercosis,
toxoplasmosis, toxocariasis, etc.
IDIOPATHIC DEMYELINATING OPTIC NEURITIS
• It can be acute, chronic or asymptomatic (subclinical)
• Acute type is the most common
• It can affect any age, common between 20 and 50 years
of age
• It can be unilateral (common) or bilateral
• Symptoms are sudden loss of central vision ranging
from blurring of vision to no perception of light, pain
in and around the eyes increasing with ocular
movements and less commonly photopsias
• Signs include decrease in vision, impaired color vision
and contrast sensitivity, relative afferent pupillary
defect, and visual field defects (generalized
depression to central or altitudinal defect).
Optic Neuritis
IMAGING
• CT is not sensitive in identifying optic neuritis, hence
should not be done in patients suspected to have optic
neuritis. CT also lacks the sensitivity to identify the
white matter lesions of multiple sclerosis
• MRI is the imaging modality of choice in patients
clinically suspected to have optic neuritis. MRI is the
most sensitive and specific investigation tool available
in the evaluation of the multiple sclerosis
• The optic nerve may show mild thickening with altered
T2-hyperintense signal. Thin sections with fat
suppression T2-weighted images and STIR sequences
are most sensitive to the altered signal within the optic
nerve. Any part of the optic nerve can be affected,
hence, it is recommended that the coronal T2-weighted
images should be taken up to the optic chiasm
• MRI studies have been performed to identify the most
common sites of optic nerve involvement in optic
neuritis. The nerve can be divided into 5 segments (each
with frequency of involvement), including: (1) Anterior
(45%), abutting the optic disc; (2) Mid-intraorbital
(61%); (3) Intracanalicular (34%); (4) Intracranial
prechiasmatic (5%); and (5) Chiasmatic segments (2%).
Lesions occasionally involve more than one site
• In the acute phase, optic neuritis lesions frequently
enhance, and enhancement is seen best on thin-section
fat-suppressed T1-weighted images. Enhancement
typically is seen in the center of the nerve but may be
peripheral. The nerve may be normal in size or may be
thickened
• Occasionally, there may be a peripheral tram-track
enhancement, which can mimic an optic nerve sheath
meningioma. The presence of a normal appearing optic
nerve within the enhancing nerve sheath is a feature of
optic nerve sheath meningioma, thus will help in
distinguishing the two.
346 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 29.1A to C: (A) Axial postcontrast fat-suppressed

T1-weighted image of the orbit showing thickening of the
left intraorbital optic nerve with homogeneous
enhancement, (B) Axial FLAIR and (C) Sagittal T2 FLAIR
of the same patient showing multiple periventricular
hyperintense plaques typically oriented perpendicular to
the body of the lateral ventricle (Dawson’s fingers)—
C multiple sclerosis with optic neuritis

• Brain imaging is done to look for plaques
• Contrast-enhanced MRI helps to differentiate acute
from chronic lesions when multiple lesions are present
in the brain
• Newer imaging techniques: MR spectroscopy,
magnetization transfer (MT) and diffusion imaging
are currently used to confirm the diagnosis of multiple
sclerosis. MR spectroscopy shows low NAA and
decreased Cr/Cho ratio. Diffusion weighted imaging
helps in differentiating acute from chronic lesions. MT
is a very sensitive technique to identify the plaques
and the age of the lesion. The magnetization transfer
ratio (MTR) demonstrates little change in early MS
plaques but significantly decreased in chronic lesions
• Of patients with optic neuritis and normal brain MRI,
studies suggest that 0-5 percent develop clinically
definite multiple sclerosis compared to 30-50 percent
of patients with three or more periventricular or ovoid
white matter lesions. As a result of the interest in early
intervention using immunomodulation and interferon
therapy for patients with multiple sclerosis, the detection
of additional lesions is likely to be more clinically useful
than the depiction of the optic neuritis lesion
• The most important reason for performing imaging
on isolated optic neuritis is to study the brain for
evidence of demyelination. As a result of the recent
trend towards early interferon therapy in patients
with additional brain lesions, clinicians base their
therapeutic decisions and discussions of prognosis on
the results of brain and, occasionally, cord imaging
studies. It is therefore preferable that all patients with
optic neuritis should have brain and spine imaging.
If brain lesions are detected, then it is preferably to
do a contrast-enhanced MRI with magnetization
transfer technique.
NEUROMYELITIS OPTICA (DEVIC’S DISEASE)
• Primarily seen in children or young adults. Prodromal
symptoms include fever, sore throat and headache.
Visual loss occurs due to damage to anterior visual
pathways and is almost always bilateral, rapid, severe,
Chapter 29 Optic Neuritis 347
not usually associated with pain. Improves in few
weeks. Central scotomas are noted on visual field
examination. Optic disc may show some mild
swelling, and may progress to pallor
• The optic neuritis and spinal cord involvement
usually occurs within 3 months of each other
• MRI of the spine shows areas of abnormal T2
hyperintense signal in the spinal cord which might
involve long segment of the cord. There may be
expansion of the cord simulating neoplasm. Contrast
enhancement may be seen
• Brain imaging is usually normal. If white matter
lesions are seen, then they are usually nonspecific and
not typical as seen in multiple sclerosis.
BIBLIOGRAPHY
1. Beck RW, Arrington J, Murtagh FR, et al. Brain magnetic
resonance imaging in acute optic neuritis. Experience of
the Optic Neuritis Study Group. Arch Neurol 1993;50(8):
841-6.
2. Horsfield MA, Larsson HB, Jones DK, Gass A. Diffusion
magnetic resonance imaging in multiple sclerosis. J Neurol
Neurosurg Psychiatry 1998;64(Suppl 1):S80-4. Review.
3. Lee AG, Lin DJ, Kaufman M, et al. Atypical features
prompting neuroimaging in acute optic neuropathy in
adults. Can J Ophthalmol 2000;35(6):325-30.
4. Miller DH, Newton MR, van der Poel JC, et al. Magnetic
resonance imaging of the optic nerve in optic neuritis.
Neurology 1988;38(2):175-9.
5. Miller DH. Magnetic resonance imaging and spectroscopy
in multiple sclerosis. Curr Opin Neurol 1995;8(3):210-5.
Review.
6. Noseworthy JH, Lucchinetti C, Rodriguez M, Weinshenker
BG. Multiple sclerosis. N Engl J Med 2000;343(13):938-52.
7. The 5-year risk of MS after optic neuritis. Experience of
the Optic Neuritis Treatment Trial: Optic Neuritis Study
Group. Neurology 1997;49(5):1404-13.
8. The clinical profile of optic neuritis. Experience of the Optic
Neuritis Treatment Trial: Optic Neuritis Study Group.
Arch Ophthalmol 1991;109(12):1673-8.
9. Wray SH. Optic neuritis. In: Albert DM, Jakobiec FA (Eds),
Principles and Practice of Ophthalmology. Philadelphia:
WB Saunders Co 1994.pp.2539-68.
348 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 29.2A and B: (A) Axial STIR image of the optic nerve showing diffuse hyperintense in the right intraorbital optic nerve (arrow)
and (B) Axial FLAIR image of the brain showing hyperintense signal in the periventricular parieto-occipital white matter, suggestive
of demyelination (arrow)

A B

Figs 29.3A and B: (A) Coronal T2-weighted image and (B) Postcontrast coronal T1-weighted image showing thickening and
hyperintense signal in the right intracranial prechiasmatic optic nerve (long arrow) (Fig. A) and enhancement in the corresponding
segment of the optic nerve (Fig. B)
 Chapter 29 Optic Neuritis 349

A B

Figs 29.4A to C: (A and B) Sagittal FLAIR showing hyperintense

periventricular plaques and (C) Axial STIR image showing subtle
hyperintense signal in the right optic nerve (white arrow) in a
C case of optic neuritis
350 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

C D

Figs 29.5A to D: Follow-up MRI done one year later: (A and B) Sagittal T2-weighted images and (C and D) Showing increase in
the number of the periventricular plaques with persistent hyperintense signal in the right optic nerve
 Chapter 29 Optic Neuritis 351

A B

Figs 29.6A to C: A 20-year-old female presented with sudden onset

with loss of the left eye of few days duration: (A) Coronal T2-fat-
suppressed image of the orbit showing a thickened left optic nerve
with hyperintense signal, (B) Axial T1 postcontrast with fat-
suppression showing enhancement of the left optic nerve and
(C) Axial diffusion-weighted image showing restricted diffusion in
C the left optic nerve
352 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 29.7A and B: A 12-year-old female presented with bilateral visual loss followed by quadriparesis:
(A) Coronal T2 image of the orbit showing bilateral thin optic nerves with subtle hyperintense signal in
the right optic nerve and (B) Sagittal T2-FSE of the cervical spine showing hyperintense signal in the
upper cervical cord (arrow). The brain-imaging was normal—neuromyelitis optica

A B

Figs 29.8A and B: A 32-year-old male presented with bilateral visual loss following fever 1 month
before: (A) Coronal T2-FSE with fat-suppression of the orbit showing thickened bilateral optic nerves
with hyperintense signal and (B) Coronal T2-FSE at the level of the chiasm showing a thickened chiasm—
postviral demyelination

SARCOIDOSIS
• It is a granulomatous disease of unknown etiology
that involves several organ systems, most commonly
mediastinal lymph nodes, lungs, liver, spleen, skin,
eyes and lacrimal glands
• Clinical presentation is of two types—subacute form
seen in patients under 30 years, particularly women,
possibly accompanied by erythema nodosum,
polyarthritis and hilar lymphadenopathy. These usu-
ally have peripheral and cranial nerve involvement.
The VII cranial nerve and optic nerves are most fre-
quently involved. The second form is in patients over
30 years with involvement of the lung parenchyma
and spread beyond the thorax
• Involvement of the central nervous system is referred
to as neurosarcoidosis. It is an uncommon but severe,
sometimes life-threatening manifestation of sarcoidosis.
It generally occurs only if the disease has had subs-
tantial systemic involvement, and signs of neurologic
involvement usually are seen in patients known to have
active disease. Strictly neurologic forms are seen in fewer
than 10% of patients
• Intracranial involvement may be seen in the form of
sarcoid granuloma, infarction secondary to sarcoid
vasculitis and sarcoid leptomeningitis. Cranial nerves,
pituitary gland, third ventricle, hypothalamus and
rarely pineal gland may be involved
• Mononeuropathy—cranial nerve involvement
– Facial palsy (the most commonly affected cranial
nerve) can be involved either unilaterally or bilat-
erally. Heerfordt’s syndrome is characterized by
fever, uveitis, swelling of the parotid gland, and
facial nerve palsy and represents a type of
neurosarcoidosis. The lesion site has been contro-
versial, but direct nerve compression by parotid
gland swelling or by a lesion within the facial canal
has been assumed
– Impaired taste and smell may occur
– Optic nerve involvement—sarcoidosis is the most
common inflammatory process producing infiltra-
tive optic neuropathy. Optic nerve dysfunction is
the most common neuro-ophthalmic manifesta-
tion of sarcoidosis. The optic nerve may be affected
at any time and may be the site of its initial pre-
sentation. It may produce papilledema,
compressive, ischemic neuropathy or optic neuri-
tis (anterior or retrobulbar). The optic neuritis
results from granulomatous infiltration of any part
Chapter 29 Optic Neuritis 353
of the optic nerve. Signs of optic nerve dysfunc-
tions are present. The optic disc may be markedly
elevated either diffusely or sectorially. The optic
disc has a nodular and solid appearance. Swollen
disc may be difficult to differentiate from demy-
elinating optic neuritis. There is usually evidence
of intraocular inflammation including uveitis,
iritis, choroiditis, and cyclitis. Sarcoid granulomas
infiltrating the optic disc are usually unilateral
associated with decreased vision but may be
bilateral mimicking papilledema.
Other neurological presentations:
– Slurred speech, impaired swallowing, hoarseness
of voice
– Vertigo, sensorineural deafness, tinnitus
– Weakness of trapezius and sternocleidomastoid
muscles
– Tongue deviation and atrophy.
• Peripheral nerve involvement—mononeuropathy,
mononeuropathy multiplex, polyneuropathy
– Sensory neuropathy—characterized by loss of
sensation and abnormal sensation (e.g. tingling,
numbness, painful patches over the thorax,
stocking/glove deficits)
– Motor neuropathy—characterized by weakness,
leading to immobility and joint stiffness.
IMAGING
CT Appearance
• Optic nerve involvement: There may be diffuse
thickening of the optic nerve. Any part of the optic
nerve can be affected. Diffuse enhancement of the
involved optic nerve and sheath is usually noted.
• Brain: Brain imaging may be usually normal. There
is diffuse, irregular thickening of the leptomeninges
with homogeneous enhancement on postcontrast
study. Calcification is not a feature of sarcoidosis.
Brainstem and cerebellar involvement is rare and can
manifest as demyelination, hemorrhagic infarction, or
parenchymal infiltration by granulomatous masses.
• Orbit (discussed in Chapter on Orbit):
– Bilateral lacrimal gland enlargement
– Irregular perioptic thickening
– Involvement of extraocular muscles and orbital
apex may be noted
354 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

C D

E F

Figs 29.9A to F: A 17-year-old male presented with vague pain in the left orbit with decreased vision, this
was followed by headache and bilateral VI nerve palsy: (A) Axial T1-weighted image, (B) Coronal T1-
weighted image, (C) Axial T2-weighted image, (D) Axial postcontrast T1-weighted image and (E and F)
Coronal postcontrast T1-weighted image showing diffuse soft tissue around the cavernous sinus, superior
orbital fissure and right optic nerve thickening at the apex (arrow). Postcontrast scans showing irregular
enhancement of the dura (black arrows) and enhancement of the soft tissue in the cavernous sinus bilaterally
(white arrows)—confirmed sarcoidosis

MR Appearance
• MRI is the imaging modality of choice to evaluate the
optic nerve. Postcontrast fat suppressed images
should be obtained when suspecting optic nerve
lesion. The imaging findings include diffuse
thickening of the optic nerve sheath complex. It is
isointense to the extraocular muscles on short TR/TE
images and minimally hyperintense to the fat on long
TR images usually hyperintense in T2-weighted
images
• Bilateral enlargement of the lacrimal gland may be
noted. They may display hyperintense signal on long
TR images
• Intracranial sarcoid will be best demonstrated on
contrast-enhanced MRI. The MR findings include
diffuse or nodular thickened leptomeninges, nodular
enhancing lesions, hydrocephalus and small areas of
infarction. The thickened meninges may be hyper- or
hypointense on T2-weighted images. A thick and
ragged pial enhancement is indicative of invasion
along perivascular spaces, leading to a meningo-
encephalovasculopathy. Enhancement of linear and
nodular areas from the pial surface into the white
matter indicates infiltration along Virchow-Robin
spaces. Periventricular high-signal lesions are seen on
T2-weighted images
• Spinal cord may show intramedullary lesions
resembling demyelinating disease
• Differential diagnosis for leptomeningeal sarcoid—
tuberculosis or bacterial (pneumococcal) meningitis.
The clinical picture and laboratory tests will help to
differentiate the lesions.
SCREENING FOR SYSTEMIC SARCOIDOSIS
• Chest radiography:
– Radiographic involvement is seen in almost 90%
of patients. Chest radiography is used in staging
the disease
– Stage I disease shows bilateral hilar lymphadeno-
pathy (BHL). Stage II disease shows BHL plus
pulmonary infiltrates. Stage III disease shows
pulmonary infiltrates without BHL. Stage IV
disease shows pulmonary fibrosis
• CT of the thorax:
– CT of the thorax may demonstrate lymphadeno-
pathy or granulomatous infiltration
– Other findings may include small nodules with a
bronchovascular and subpleural distribution,
Chapter 29 Optic Neuritis 355
thickened interlobular septae, honeycombing,
bronchiectasis, and alveolar consolidation
• Fluorodeoxyglucose-positron emission tomography:
(FDG-PET) imaging may show areas of hypermetabo-
lism or hypometabolism in the CNS, and this
information may be compared to a systemic PET scan
in patients with systemic sarcoidosis, thus establish-
ing a firmer diagnosis
• Whole body gallium-67 scanning:
– Two common findings seen in sarcoidosis are the
lambda and panda patterns
– The lambda pattern is produced by uptake of the
right paratracheal and bilateral hilar lymph nodes.
The panda image is produced by symmetric
uptake by the lacrimal and parotid glands.
BIBLIOGRAPHY
1. Christoforidis GA, Spickler EM, Recio MV, Mehta BM.
MR of CNS sarcoidosis: Correlation of imaging features
to clinical symptoms and response to treatment. Am J
Neuroradiol 1999;20(4):655-69.
2. Cooper SD, Brady MB, Williams JP, Pilgreen KL, Harp
DL, Weissmann JR. Neurosarcoidosis: Evaluation using
computed tomography and magnetic resonance imaging.
J Comput Tomogr 1988;12(2):96-9.
3. Frohman LP, Guirgis M, Turbin RE, Bielory L. Sarcoidosis
of the anterior visual pathway: 24 new cases. J Neuro-
ophthalmol 2003;23(3):190-7.
4. Kadakia JK, Collette PM, Sharma OP. Role of magnetic
resonance imaging in neurosarcoidosis. Sarcoidosis
1993;10(2):98-9.
5. Krejchi D, Caldemeyer KS, Vakili ST, Pritz MB.
Neurosarcoidosis resembling meningioma: MRI chara-
cteristics and pathologic correlation. J Neuroimaging
1998;8(3):177-9.
6. Lexa FJ, Grossman RI. MR of sarcoidosis in the head and
spine: Spectrum of manifestations and radiographic
response to steroid therapy. Am J Neuroradiol 1994;15(5):
973-82.
7. Miller DH, Kendall BE, Barter S, Johnson G, MacManus
DG, Logsdail SJ, Ormerod IE, McDonald WI. Magnetic
resonance imaging in central nervous system sarcoidosis.
Neurology 1988;38(3):378-83.
8. Nowak DA, Widenka DC. Neurosarcoidosis: A review of
its intracranial manifestation. J Neurol 2001;248(5):363-72.
Review.
9. Pickuth D, Heywang-Kobrunner SH. Neurosarcoidosis:
Evaluation with MRI. J Neuroradiol 2000;27(3):185-8.
10. Pickuth D, Spielmann RP, Heywang-Kobrunner SH. Role
of radiology in the diagnosis of neurosarcoidosis. Eur
Radiol 2000;10(6):941-4.
356 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 29.10A to C: (A and B) Axial FLAIR showing multiple frontal

and parietal subcortical T2-hyperintense areas suggestive of
vasculitis and (C) Axial postcontrast MRI of orbit (note the
improper fat suppression) showing thickening of the right optic
nerve with enhancement and prominent pituitary gland-
C granulomatous neuritis
 Chapter 29 Optic Neuritis 357

A B

Figs 29.11A and B: (A) Axial T2-weighted image and (B) Axial T1-weighted image showing diffuse thickening of the left optic
nerve with thickening of the posterior sclera (arrow)—sarcoid scleritis and optic neuritis

A B C

Figs 29.12A to C: A case of sarcoidosis: (A) Axial T1-weighted image and (B) T2-weighted image showing T1-isointense and T2-
hyperintense soft tissue at the left superior orbital fissure (long arrow) and cavernous sinus with lateral extraconal soft tissue
(small arrow) and (C) Axial T1-weighted image of the right mastoid showing soft tissue in the right middle ear cavity and mastoid
(white arrow)
358 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

C D

Figs 29.13A to D: Sarcoid meningitis: (A) Axial postcontrast fat-suppressed T1-weighted image,
(B) Coronal postcontrast fat-suppressed T1-weighted image showing diffuse irregular nodular
thickening of the dura (dotted arrow), thickening and enhancement of the intracranial optic nerves
(white arrow), and nodular enhancing extraconal soft tissue in the right superonasal orbit
(black arrow) and (C and D) Coronal FLAIR of brain showing thickening of the optic nerve (white
arrow) and chiasm displaying T2-hyperintense signal and focal area of edema in the right frontal lobe
(black arrow)

INFECTIOUS OPTIC NEURITIS
Inflammation of the optic nerve can result from a direct
infection of the nerve by various infectious agents such
as viruses and bacteria or systemic and central nervous
system (CNS) infection can trigger an immune response.
Distinguishing the two pathogenesis is not possible.
PARAINFECTIOUS OPTIC NEUROPATHY
• It typically follows the onset of a viral or less often a
bacterial infection by 1 to 3 weeks
• More common in children and occurs most often on
an immunologic basis producing demyelination of the
optic nerve
• It is often bilateral
• Optic disc may appear normal or swollen
• It may be seen in patients without neurologic
dysfunction or in association with meningitis,
meningoencephelitis or acute disseminated ence-
phalomyelitis (ADEM). Visual recovery is usually
good even without treatment.
Imaging
• MRI is the imaging modality of choice
• The involved optic nerve will demonstrate hyperin-
tense signal on T2-weighted image and may or may
not show enhancement on postgadolinium studies
• Patients suspected to have ADEM should have
imaging of the brain and spinal cord. T2 hyperintense
lesions are noted typically in the white matter,
brainstem and cerebellum.
HIV OPTIC NEUROPATHY
• HIV optic neuropathy can result from a variety of
infectious processes such as cryptococcal meningitis,
cytomegalovirus infection, tuberculous meningitis,
herpes infection, fungal infection or by HIV virus
itself. Rarely, toxoplasmosis may result in optic
neuropathy
• It can be a direct infection or an immune-mediated
parainfectious process.
Imaging
• The optic nerve will be thickened and may show
diffuse enhancement
• Brain parenchyma may show associated intracranial
diseases which may help in finding causative etiology.
Chapter 29 Optic Neuritis 359
SINUSITIS WITH OPTIC NEURITIS
Optic neuritis following sinusitis in the present era with
aggressive antibiotic therapy is rare. However, in acute
severe sinusitis, spread of infection from the paranasal
sinuses can result in secondary optic neuritis. Infection
originating in the ethmoid and maxillary sinus can result
in obvious signs of orbital inflammation. Spread of
infection from the sphenoid sinus to the posterior optic
nerve at the apex or within the canal can be silent except
for loss of vision. Fungal sinusitis is common in this
setting.
Imaging
CT scan shows opacification of the paranasal sinus with
extension into the orbit. The optic nerve will be thickened
with perineural fat involvement and orbital soft tissue
will be noted. Bony erosion may be seen in fungal
sinusitis. MRI with gadolinium is the imaging modality
of choice. It will show the opacified paranasal sinuses
along with the optic nerve involvement better than CT
scan. The optic nerve involovement may be contiguous,
if the sphenoid sinus is involved. The other paranasal
sinuses will usually cause orbital cellulitis and secondary
optic nerve involvement.
BIBLIOGRAPHY
1. Awerbuch G, Labadie EL, Van Dalen JT. Reversible optic
neuritis secondary to paranasal sinusitis. Eur Neurol
1989;29(4):189-93.
2. Azuma M, Morimura Y, Kawahara S, Okada AA. Bilateral
anterior optic neuritis in adult measles infection without
encephalomyelitis. Am J Ophthalmol 2002;134(5):768-9.
3. Cackett P, Weir CR, McFadzean R, Seaton RA. Optic
neuropathy without retinopathy in AIDS and
cytomegalovirus infection. J Neuro-ophthalmol 2004;
24(1):94-5.
4. Ergene E, Rupp FW Jr, Qualls CR, Ford CC. Acute optic
neuritis: Association with paranasal sinus inflammatory
changes on magnetic resonance imaging. J Neuroimaging
2000;10(4):209-15.
5. Gunduz K, Ozdemir O. Bilateral retrobulbar neuritis
following unilateral herpes zoster ophthalmicus.
Ophthalmologica 1994;208(2):61-4.
6. Hamed LM, Silbiger J, Guy J, Mickle JP, Sibony P, Cossari
A, Andriola M. Parainfectious optic neuritis and
encephalomyelitis: A report of two cases with thalamic
involvement. J Clin Neuroophthalmol 1993;13(1):18-23.
360 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

C D

Figs 29.14A to D: A 6-year-old girl presented with bilateral sudden visual loss following fever: (A) Axial FLAIR, (B) Axial T2-weighted
image showing hyperintense signal in the optic nerve extending up to its intracranial end (arrows) and (C and D) Coronal postcontrast
T1-weighted images showing diffuse enhancement in the intraorbital and intracranial optic nerve (dotted arrows)

7. Jabs DA, Green WR, Fox R, Polk BF, Bartlett JG. Ocular
manifestations of acquired immune deficiency syndrome.
Ophthalmology 1989;96(7):1092-9.
8. Lee LA, Huang CC, Lee TJ. Prolonged visual disturbance
secondary to isolated sphenoid sinus disease. Laryngo-
scope 2004;114(6):986-90.
9. Lee MS, Cooney EL, Stoessel KM, Gariano RF. Varicella
zoster virus retrobulbar optic neuritis preceding retinitis
in patients with acquired immune deficiency syndrome.
Ophthalmology 1998;105(3):467-71.
10. Miller DH, Kay R, Schon F, McDonald WI, Haas LF,
Hughes RA. Optic neuritis following chickenpox in adults.
J Neurol 1986;233(3):182-4.
11. Moorman CM, Anslow P, Elston JS. Is sphenoid sinus
opacity significant in patients with optic neuritis? Eye
1999;13 (Pt 1):76-82.
12. Pless ML, Malik SI. Relapsing-remitting, corticosteroid-
sensitive, varicella zoster virus optic neuritis. Pediatr
Neurol 2003;29(5):422-4.
13. Sadun AA, Pepose JS, Madigan MC, Laycock KA, Tenhula
WN, Freeman WR. AIDS-related optic neuropathy:
Chapter 29 Optic Neuritis 361
A histological, virological and ultrastructural study.
Graefes Arch Clin Exp Ophthalmol 1995;233 (7):387-98.
14. Sanborn GE, Kivlin JD, Stevens M. Optic neuritis secondary
to sinus disease. Arch Otolaryngol 1984;110(12):816-9.
15. Schmidt P. Herpes zoster ophthalmicus with retrobulbar
neuritis: A case report. Acta Ophthalmol (Copenh) 1983;
61(3):501-9.
16. Shayegani A, Odel JG, Kazim M, Hall LS, Bamford N,
Schubert H. Varicella-zoster virus retrobulbar optic neuritis
in a patient with human immunodeficiency virus. Am J
Ophthalmol 1996;122(4):586-8.
17. Strong LE, Henderson JW, Gangitano JL. Bilateral
retrobulbar neuritis secondary to mumps. Am J Ophthalmol
1974;78(2):331-2.
18. Vrabec TR. Posterior segment manifestations of HIV/AIDS.
Surv Ophthalmol 2004;49 (2):131-57. Review.
19. Wang AG, Liu JH, Hsu WM, Lee AF, Yen MY. Optic neuritis
in herpes zoster ophthalmicus. Jpn J Ophthalmol 2000;
44(5):550-4.
20. Yasuda Y, Morita T, Akiguchi I, Kimura J, Kameyama M.
Sphenoid sinus mucocele with recurrent visual disturbance.
Eur Neurol 1992;32(4):225-7.
362 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

C D

Figs 29.15A to D: A 32-year-old HIV positive patient presented with bilateral sudden loss of vision with altered mental status:
(A and B) Sagittal FLAIR and (C and D) Axial FLAIR showing altered signal in the chiasm (arrow) with patchy diffuse hyperintense
signal in the periventricular white matter and diffuse cerebral atrophy—HIV encephalopathy
 Chapter 29 Optic Neuritis 363

A B

Figs 29.16A and B: A 32-year-old female presented with sudden painful loss of vision in the left eye: (A) Axial T2-weighted image
of the orbit and (B) Axial postcontrast T1-weighted image of the orbit showing diffuse thickening of the left optic nerve and
perioptic haziness with infiltration of the optic disc seen as an elevated hypointense lesion (arrow) in T2-weighted image with
moderate contrast enhancement in Figure B. Patient was subsequently tested positive for HIV
364 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

C D

Figs 29.17A to E: (A) Axial T2-weighted image of the orbit,

(B) Coronal T1-weighted image, (C and D) Axial postcontrast
T1-weighted images and (E) Coronal T1-weighted image
showing opacification of the ethmoid and sphenoid sinus with
perioptic soft tissue infiltrating the right optic nerve. On post-
contrast scans, there is diffuse enhancement of the soft tissue
encasing the intracranial optic nerve with patchy enhancement
in the paranasal sinuses—fungal sinusitis with optic nerve
E infiltration
 Chapter 29 Optic Neuritis 365

A B

Figs 29.18A to C: A 46-year-old male presented with sudden

onset painful loss of vision in the left eye: (A) Coronal T2-weighted
image, (B) Axial T2-weighted image and (C) Coronal postcontrast
T1-weighted image with fat-suppression—showing grossly
thickened left optic nerve sheath complex with an elevated nodule
at the optic disc (arrow). Postcontrast study showing enhancement
of the optic nerve and sheath and enhancement of the perioptic
fat. CSF analysis was suggestive of cryptococcal infection in a
C immunocompetent patient
366 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 29.19A to C: A 12-year-old female with past history of

tuberculous meningitis presented with bilateral vision loss:
(A) Axial T2-weighted image, (B) Axial postcontrast fat-
suppressed image showing thickened right ocular Coats’
and right optic nerve and (C) Coronal postcontrast fat-
suppressed image showing soft tissue at the apex
C compressing the optic nerves bilaterally (arrows)
 Chapter 29 Optic Neuritis 367

A B

Figs 29.20A to C: A 27-year-old female presented with sudden

loss of vision in the left eye with headache: (A) Axial T2-weighted
image, (B) Coronal T2-weighted image showing a well-defined
hyperintense cystic lesion with scolex in the left intracranial optic
nerve (arrow) and (C) Axial T1-postcontrast fat-suppressed image
C showing a ring-like enhancement—cysticercosis
Chapter 30
Infiltrative Optic Neuropathy
TUMORS OF THE OPTIC NERVE
PRIMARY AND SECONDARY OPTIC NERVE
SHEATH MENINGIOMA (ONSM)
• Primary ONSMs account for approximately one-third
of primary optic nerve tumors and 5 to 10 percent of
orbital tumors
• The vast majority (90%) of optic nerve sheath tumors
are secondary
• Primary ONSM represents a neoplasia of the
meningothelial cap cells of arachnoid villi and can
develop anywhere along the course of the optic nerve,
from globe to prechiasmal intracisternal optic nerve
• Lesions may be unilateral, bilateral or multifocal with
the latter two subgroups occurring most commonly
in patients with type 2 neurofibromatosis
• Meningiomas extending from other locations and
involving the optic nerve are secondary and may arise
from the cavernous sinus, falciform ligament, clinoid,
sphenoid wing, pituitary fossa, planum sphenoidale,
or olfactory groove
• Age and sex distribution: Female preponderance is
seen. The overall mean age at presentation is cited as
40.8 years, varying from 36.1 years in men to 42.5 years
in women.
Clinical Signs and Symptoms
• Commonly reported clinical manifestations of ONSM
include ipsilateral visual loss, afferent pupillary
defect, color vision disturbance, visual field defect,
proptosis, optic disc edema, motility disturbance, pain
and lower eyelid edema. Patients have symptoms of
gradual or rapid visual loss, diplopia or gaze-evoked
visual obscurations
• Ophthalmoscopic examination may reveal optic nerve
head swelling, contiguous macular edema, nerve
pallor or choroidal folds
• Optic disc swelling accompanied by optic nerve pallor
also may be observed. As visual function declines, the
optic disc edema may resolve, leaving a pale nerve
head. Optociliary shunt vessels occasionally develop
that represent the secondary dilation of pre-existing
retinal to choroidal shunting veins.
Radiological Features
The diagnosis of ONSM relies heavily on imaging
findings. There are several radiographic growth patterns:
Tubular (diffuse, apical expansion, anterior expansion),
globular, fusiform and focal. The tubular patterns,
marked by widening along the length of the nerve sheath,
were subdivided into diffuse expansion, apical expansion
towards the orbital apex or anterior nerve expansion
towards the globe. Globular growth patterns were caused
by exophytic expansion outside the nerve sheath.
Fusiform patterns were spindle-shaped with tapers at the
proximal and distal ends. This type was most likely to be
confused with optic nerve glioma.
Computed Tomography
• Thin CT scans (1.5 mm sections) may reveal regular
or irregular thickening of the nerve sheath meninges
with moderate-to-marked homogeneous enhance-
ment after intravenous contrast infusion
 Chapter 30 Infiltrative Optic Neuropathy 369

Fig. 30.1: Fundus photograph of the right eye of a patient with

optic nerve sheath meningioma showing optociliary shunt
vessels on the disc (arrow)

Fig. 30.2: Axial CT scan of the orbits shows bilateral diffuse

calcification along the optic nerve—bilateral optic nerve sheath
meningiomas
370 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
• On thin section axial CT images, hyperdense
enhancement of the meninges surrounding a
hypodense nerve described classically as a “tram-
track” appearance, is highly characteristic of a
meningioma
• Similarly, on noncontrast CT images, linear
calcification of the nerve sheath is also called a tram-
track calcification. The optic nerve itself appears
normal in size or of a smaller diameter within an area
of thickened meninges, compared to the contralateral
nerve at the same level. The smaller nerve size is the
result of circumferential compression or atrophy and
is a useful differential point
• Calcification may: (1) Surround the nerve and cause
a tram-track appearance on axial and coronal CT scan,
(2) Maintain a punctate diffuse location, or (3) Cause
an en plaque signal along the optic canal, which may
be difficult to distinguish from normal bone
• Calcification may be masked by contrast agents and
should be sought on precontrast soft tissue and bone-
windowed images.
• Hyperpneumatization and hyperostosis of the
adjacent sinus and bone may be noted in meningiomas
located at the apex or within the canal.
Magnetic Resonance Imaging
• Although MRI is less sensitive than CT in the
recognition of calcification, it currently remains the
procedure of choice for diagnosis of ONSM. A contrast-
enhanced fat suppressed T1-weighted images are
mandatory when optic nerve sheath meningioma is
suspected. ONSMs are typically isointense or slightly
hypointense to brain and optic nerve on T1- and
T2-weighted images but may also be hyperin-
tense. Postgadolium shows homogeneous contrast
enhancement study. The calcified component does not
enhance with contrast.
• Contrast-enhanced MRI is recommended in all
patients suspected to have optic nerve sheath
meningioma. It is highly sensitive and specific in
diagnosing and delineating the extent of the lesion. It
will also help in identifying other meningiomas.
Radiographic Differential Diagnosis
Differential diagnosis of perioptic enhancement includes
sarcoidosis, demyelinating optic neuritis or perineuritis,
orbital inflammatory disease of the optic nerve,
lymphoma and optic nerve metastasis.
BIBLIOGRAPHY
1. Carrasco JR, Penne RB. Optic nerve sheath meningiomas
and advanced treatment options. Curr Opin Ophthalmol
2004;15(5):406-10. Review.
2. Dutton JJ. Optic nerve sheath meningiomas. Surv
Ophthalmol 1992;37(3):167-83. Review.
3. Jackson A, Patankar T, Laitt RD. Intracanalicular optic
nerve meningioma: A serious diagnostic pitfall. Am J
Neuroradiol 2003;24(6):1167-70.
4. Kanamalla US. The optic nerve tram-track sign. Radiol
2003;227(3):718-9
5. Lloyd GA. Primary orbital meningioma: A review of 41
patients investigated radiologically. Clin Radiol
1982;33(2):181-7.
6. Mafee MF, Goodwin J, Dorodi S. Optic nerve sheath
meningiomas: Role of MR imaging. Radiol Clin North Am
1999;37(1):37-58, ix. Review.
7. Saeed P, Rootman J, Nugent RA, et al. Optic nerve sheath
meningiomas. Ophthalmol 2003;110:2019-30.
8. Shen TT, Sakai O, Curtin HD, Rizzo JF 3rd. Magnetic
resonance imaging of primary anterior visual pathway
tumors. Int Ophthalmol Clin 2001;41(1):171-80.
 Chapter 30 Infiltrative Optic Neuropathy 371

A B

Figs 30.3A and B: (A) Axial postcontrast CT scan and (B) Coronal postcontrast CT scan of the orbit show proptosis of the left eye
with globular perioptic enhancing mass completely encircling the optic nerve seen as an eccentric-rounded hypodensity within
the enhancing mass (arrow)
372 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 30.4A to C: Right optic nerve sheath meningioma with

extension into the cavernous sinus: (A) Axial CT scan and
(B and C) Coronal CT scan of the orbit in bone window setting
show calcified perioptic mass with hyperostosis of the sphenoid
bone and anterior clinoid and extension into the cavernous
C sinus (arrow)
 Chapter 30 Infiltrative Optic Neuropathy 373

A B

Figs 30.5A and B: (A) Axial T1-weighted image and (B) Contrast MRI of the orbits showing fusiform intraconal mass in the right
orbit encircling the optic nerve (arrow) with homogeneous contrast enhancement

A B

Figs 30.6A and B: (A) Axial T2-weighted image and (B) Postcontrast fat-suppressed T1-weighted image of the orbit showing
diffuse thickening of the optic nerve sheath complex with diffuse enhancement. Note the compressed optic nerve within the lesion
(arrow)
374 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

C D

Figs 30.7A to D: (A) Axial CT scan of the orbit, (B) Coronal CT scan of the orbit showing perioptic mass with tram-track calcification
(arrowhead), (C) Axial T1-weighted image and (D) Postcontrast coronal T1-weighted fat-suppressed image of the orbit showing
diffuse perioptic mass with homogeneous moderate contrast enhancement. Note the normal optic nerve within the lesion (arrow)
 Chapter 30 Infiltrative Optic Neuropathy 375

A B

Figs 30.8A to C: Secondary ONSM: (A) Axial contrast-

enhanced T1-weighted image, (B) Sagittal contrast-enhanced
T1-weighted image and (C) Coronal contrast-enhanced T1-
weighted MRI image of the brain showing plaque-like
enhancement in the tuberculum sellae (white arrows) encasing
the left intracranial optic nerve. Also noted enhancement in
C the intracanalicular optic nerve (black arrow) (Fig. C)
376 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
OPTIC NERVE GLIOMA
• Optic nerve glioma is the most common primary
neoplasm of the optic nerve
• It represents about 17 percent of the orbital tumors
encountered in childhood
• They represent 4 percent of all orbital tumors,
2 percent of all intracranial tumors and 4 percent of
all gliomas
• Optic nerve glioma can be categorized into two
groups—benign optic nerve glioma in children and
aggressive glioma in adults
• In the most instances, optic pathway gliomas arise in
children, and in many cases, affected children have
neurofibromatosis type 1 (NF-1). About 29 percent of
optic pathway gliomas occur in the setting of NF-1.
It can occur in neurofibromatosis type 2 also.
CLINICAL PRESENTATION
• Peak incidence is from 2 to 8 years with 75 percent
manifesting in the first-two decades of life
• There is a slight female preponderance
• Common presentation is proptosis with decreased
visual acuity. They may also present with nystagmus
or strabismus. Afferent pupillary defect is seen on the
involved side
• The optic nerve head may be edematous, infiltrated
with tumor, or atrophic. Rarely, optociliary collateral
vessels may be present
• Optic nerve gliomas are slow-growing lesions without
any significant increase in size over many years.
Spontaneous regression has been reported in patients
with neurofibromatosis
• Intracranial extension may be clinically suspected on
the basis of precocious puberty, somnolence or
diabetes insipidus.
PATHOLOGY
• In the pediatric form, these tumors resemble juvenile
pilocytic astrocytoma. They grow slowly and almost
always are only locally invasive with no tendency for
malignant transformation. They cause fusiform
enlargement of the optic nerve, which is completely
invested by the dura. Hemorrhage and necrosis are
uncommon. Some tumors may invade the leptom-
eninges with growth within the subarachnoid space
resulting in obstruction to the CSF flow and second-
ary dural ectasia. A reactive meningeal hyperplasia
may be incited, making it difficult to distinguish from
a perioptic meningioma
• In the adult form of the disease, the glioma is either
an anaplastic astrocytoma or a glioblastoma
multiforme, arising from abnormal astrocytes.
Nuclear pleomorphism, numerous mitoses and vas-
cular endothelial proliferation are prominent.
IMAGING
• Prior to the introduction of CT and MRI, conventional
radiologic studies included optic foramen views and
polytomography. The optic foramen is enlarged when
the tumor extends into the optic canal. There is smooth
widening of the canal without bony erosion or
sclerosis. Polytomography was introduced, especially
to show the chiasmatic sulcus, tuberculum sellae and
planum sphenoidale. Extension to the chiasm with
formation of bulky mass leads to enlargement and
J-shaped depression of the chiasmal sulcus, flattening
of the tuberculum sellae, undercutting of the anterior
clinoid process and thinning of the optic strut
• MRI with gadolinium would be the preferred
modality as it delineates the posterior extension and
associated findings of NF (discussed later)
• CT is superior in identifying uncommon meningioma
with flecks of calcification, which are rare in optic
pathway glioma. The 2 mm bone window settings
should be taken to see the enlargement of the optic
canal. Normal optic canal measures between 4 and
5 mm.
CT Appearance
• CT findings include well-circumscribed fusiform
enlargement of the optic nerve, which when large
enough will obliterate the intraorbital fat. The
enlargement may be tubular, fusiform or excrescent
with smooth margins and sharp interface. It is
isodense with the brain and shows slight to moderate
enhancement. Areas of hypodensities suggest
mucinous or cystic changes
• Approximately, 50 percent of the lesions demonstrate
enhancement and enhancement is more common with
intracranial (especially retrochiasmatic) extension
• Calcification within the tumor is rare but may occur
following radiation therapy. In case of marked enlarge-
ment of the nerve, kinking and tortousity can be noted
• When tubular enlargement of the intraorbital optic
nerve is seen, the lesion may mimic optic neuritis or
optic nerve meningioma. In this setting, MRI with
gadolinium should be performed.
 Chapter 30 Infiltrative Optic Neuropathy 377

A B

Figs 30.9A to C: (A) Axial CT scan of the orbit and brain showing
proptosis of the left eye with a fusiform well-circumscribed intraconal
mass, (B) Axial CT scan at the level of the chiasm shows posterior
extension resulting in thickened chiasm (arrow) and (C) Axial CT
scan at the level of internal capsule shows bilateral hypodensity
C along the capsular tracts representing dysplastic glial tissue
378 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
MRI Appearance
• It is the imaging modality of choice to evaluate
intracanalicular and intracranial extension of the tumor
and also helps to differentiate from other lesions
• The MR appearance is somewhat variable depending
on whether the tumor grows within the optic nerve
or around the optic nerve into the perineural space.
In the latter case, when the perineural portion
enhances, it may mimic a perioptic meningioma
especially in adolescent or adult patients
• On T1-weighted MRI, optic nerve gliomas are usually
isointense to the cortex and hypointense to white
matter
• On T2-weighted images, they demonstrate a mixed
iso- to hyperintense signal with respect to white
matter and the cortex
• The enhancement pattern of optic nerve glioma is
variable from slight to marked enhancement
• They may be associated with widening of the perioptic
CSF space secondary to the obstruction to the CSF
outflow also termed pseudoectasia
• Large tumors in the suprasellar area may simulate
other suprasellar tumors, but involvement of the
intraorbital optic nerves can help differentiate from
other suprasellar masses
• Patients with NF-1 with optic nerve gliomas (unilate-
ral or bilateral) may have white matter lesions in the
brainstem, cerebellum and thalamus. These lesions do
not usually enhance with contrast and are termed
dysplastic glial tissue or hamartomatous lesions.
Malignant optic nerve gliomas usually occur in adults
with histology consistent with anaplastic astrocytoma.
Preferred Imaging
• MRI is the preferred method for evaluation of optic
pathway glioma to see both the intraorbital lesion and
its intracranial extent
• Gadolinium-enhanced T1-weighted images with fat
saturation should be done to delineate the enhanced
tumor from the orbital fat.
Limitations of MRI
Fine calcification seen in meningiomas can be easily
missed which would help to confirm the diagnosis if there
is a diagnostic dilemma.
Differential diagnosis: Unilateral optic nerve thickening
and enhancement results from other unilateral
inflammatory disorders, such as neuritis, pseudotumor,
sarcoidosis, neoplasms (optic meningioma and leukemia)
and vascular lesions.
BIBLIOGRAPHY
1. Aoki S, Barkovich AJ, Nishimura K, et al. Neuro-
fibromatosis types 1 and 2: Cranial MR Findings. Radiol
1989;172(2): 527-34.
2. Balcer LJ, Liu GT, Heller G, Bilaniuk L, Volpe NJ, Galetta
SL, et al. Visual loss in children with neurofibromatosis
type 1 and optic pathway gliomas: Relation to tumor
location by magnetic resonance imaging. Am J
Ophthalmol 2001;131(4):442-5.
3. Hendrix LE, Kneeland JB, Haughton VM, et al. MR
imaging of optic nerve lesions: Value of gadopentetate
dimeglumine and fat-suppression technique. Am J
Neuroradiol 1990;11(4):749-54.
4. Hollander MD, FitzPatrick M, O'Connor SG, et al. Optic
gliomas. Radiol Clin North Am 1999;37(1):59-71.
5. Kornreich L, Blaser S, Schwarz M, et al. Optic pathway
glioma: Correlation of imaging findings with the presence
of neurofibromatosis. Am J Neuroradiol 2001;22(10):
1963-9.
6. Millar WS, Tartaglino LM, Sergott RC, et al. MR of
malignant optic glioma of adulthood. Am J Neuroradiol
1995;16(8):1673-6.
7. Parsa CF, Hoyt CS, Lesser RL, Weinstein JM, Strother CM,
Muci-Mendoza R, Hoyt WF, et al. Spontaneous regression
of optic gliomas: Thirteen cases documented by serial
neuroimaging. Arch Ophthalmol 2001;119(4):516-29.
8. Thiagalingam S, Flaherty M, Billson F, North K.
Neurofibromatosis type 1 and optic pathway gliomas:
Follow-up of 54 patients. Ophthalmol 2004;111(3):
568-77.
 Chapter 30 Infiltrative Optic Neuropathy 379

A B

Figs 30.10A and B: (A) Axial CT scan of the orbit shows thickening of the right optic nerve with eccentric mass lesion at the apex
and (B) Coronal CT scan at the level of the optic canal shows widening of the right optic canal (white arrow)

A B

Figs 30.11A and B: (A and B) Axial CT scan of the orbit in soft tissue and bone window settings, respectively showing bilateral
intraconal fusiform masses causing bilateral proptosis with widening of the left optic canal (arrow)
380 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 30.12A and B: Axial T2-weighted images showing heterogeneous intraconal mass in the left orbit causing proptosis and
posterior extension into the chiasm and optic tracts (black arrow). Dilatation of the temporal horns is seen (white arrow). T2-
hyperintense signal is noted in the left cerebral peduncle (arrowhead) and bilateral cerebellar peduncle (dotted arrow)—representing
hamartomatous lesions

A B

Figs 30.13A and B: (A) Axial T1-weighted postcontrast image and (B) Coronal T1-weighted postcontrast image of the orbit
showing diffuse thickening and minimal enhancement of the right optic nerve extending posteriorly to involve the intracanalicular
and intracranial optic nerve (arrow)
 Chapter 30 Infiltrative Optic Neuropathy 381

A B

C D

Figs 30.14A to D: Optochismatic glioma: (A) Midsagittal T1-weighted image of the brain showing a large heterogeneous mass
lesion in the suprasellar region extending up to the roof of the third ventricle, (B) Axial T2-weighted image of the orbit showing
thickening of the intraorbital optic nerves bilaterally with widening of the peroptic CSF spaces (secondary dural ectasia), (C) Coronal
T1-weighted image at the level of the orbital apex showing thickening of the optic nerves bilaterally with widened perioptic CSF
spaces and (D) Axial FLAIR image at the level of the chiasm showing a hyperintense mass in the suprasellar cistern with
extension along the retrochiasmal visual pathway
382 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
LEUKEMIC OPTIC NEUROPATHY
• It can occur in patients with acute myelogenous
leukemia, acute lymphocytic, monocytic leukemia,
erythroleukemia and chronic lymphocytic leukemia
• Acute leukemias are responsible for most reported
cases of infiltrative optic neuropathies caused by
lymphoreticular disorders
• About 4 percent of children with acute leukemia have
evidence of infiltrative optic neuropathy
• Patients with infiltrative optic neuropathy in the
setting of chronic leukemia have a more indolent
clinical course than acute leukemias.
CLINICAL FEATURES
• Visual loss may be sudden or gradual. Most patients
with leukemic infiltration of the optic nerve are known
to have leukemia at the time of visual loss or the
patient is found to have asymptomatic disc swelling,
however, in some patients optic neuropathy is the first
evidence of the disease
• It can infiltrate either the prelaminar portion or the
immediate retrolaminar portion of the optic disc
• A whitish fluffy infiltrate obscuring the disc details,
disc edema and peripapillary hemorrhage are usually
seen. In such cases, visual loss is minimal
• Infiltration just posterior to the lamina cribrosa
produce marked decreased in vision with true disc
swelling and variety of field defects. Peripapillary and
peripheral retinal hemorrhages may be seen
• Raised intracranial tension due to central nervous
system infiltration can cause papilledema and leukemic
retinopathy can cause adjacent disc swelling too.
NEUROIMAGING
MRI brain and orbit reveals:
• Diffusely enlarged optic nerve that enhances with
contrast
• There may be diffuse thickening of the dura or
leptomeninges
• Perioptic spaces widening may be seen in patients
who develop papilledema.
BIBLIOGRAPHY
1. Chen CY, Zimmerman RA, Faro S, Bilaniuk LT, Chou TY,
Molloy PT. Childhood leukemia: Central nervous system
abnormalities during and after treatment. Am J Neuro-
radiol 1996;17(2):295-310.
2. Currie JN, Lessell S, Lessell IM, Weiss JS, Albert DM,
Benson EM, Optic neuropathy in chronic lymphocytic
leukemia. Arch Ophthalmol 1988;106(5):654-60.
3. Gordon KB, Rugo HS, Duncan JL, Irvine AR, Howes EL
Jr, O'Brien JM, Carter SR. Ocular manifestations of
leukemia: Leukemic infiltration versus infectious process.
Ophthalmol 2001;108(12):2293-2300.
4. Lin YC, Wang AG, Yen MY, Hsu WM. Leukemic
infiltration of the optic nerve as the initial manifestation
of leukaemic relapse. Eye 2004;18(5):546-50.
5. Mayo GL, Carter JE, McKinnon SJ. Bilateral optic disc
edema and blindness as initial presentation of acute
lymphocytic leukemia. Am J Ophthalmol 2002;134(1):
141-2.
6. Porto L, Kieslich M, Schwabe D, Zanella FE, Lanfermann
H. Central nervous system imaging in childhood
leukemia. Eur J Cancer 2004;40(14):2082-90.
7. Rosenthal AR. Ocular manifestations of leukemia: A
review. Ophthalmol 1983;90(8):899-905.
8. Rudolph G, Haritoglou C, Schmid I, Hochhaus F, Kampik
A. Visual loss as a first sign of adult type chronic myeloid
leukemia in a child. Am J Ophthalmol 2005; 140(4):750-1.
9. Schocket LS, Massaro-Giordano M, Volpe NJ, Galetta SL.
Bilateral optic nerve infiltration in central nervous system
leukemia. Am J Ophthalmol 2003;135(1):94-6.
10. Vazquez E, Lucaya J, Castellote A, Piqueras J, Sainz P,
Olive T, Sanchez-Toledo J, Ortega JJ. Neuroimaging in
pediatric leukemia and lymphoma: Differential diagnosis.
Radiographics 2002;22(6):1411-28. Review.
 Chapter 30 Infiltrative Optic Neuropathy 383

A B

Figs 30.15A and B: A 15-year-old girl who presented with severe headache and
progressive loss of vision and was treated 3 months earlier for tuberculous meningitis.
Fundus photograph of this patient showing elevated edematous angry looking optic
discs with florid peripapillary hemorrhages. MRI brain and blood tests revealed the
presence of acute myeloid leukemia with the lesion infiltrating the prelaminar optic nerve

A B

C D

Figs 30.16A to D: MRI scans of the same patient: (A) Axial postcontrast T1-weighted
image, (B) Axial FLAIR image at the level of the orbit, (C) Coronal postcontrast T1-
weighted image and (D) Axial postcontrast T1-weighted image of the brain showing
thickening of the optic nerves bilaterally with elevation of optic disc (black arrow). Figs C
and D show diffuse nodular enhancement and dural thickening (block arrows) noted
bilaterally
384 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
LYMPHOMA
• CNS involvement in non-Hodgkin’s and Hodgkin’s
lymphoma is rare but can occur in 10 percent of cases
• Of these 5 percent will have optic nerve infiltration at
sometime during the course of their disease
• Infiltration is even less common in Hodgkin’s disease.
Infiltration of the retrobulbar optic nerve and chiasm
is usually seen
• Usually infiltration results from spread of intracranial
tumor and rarely from adjacent paranasal sinuses
• In many cases, the diagnosis of lymphoma is already
known when visual symptoms develop
• Sometimes, visual loss is the presenting sign of the
disease. In such cases, detailed systemic work-up is
recommended
• Visual loss is usually insidious and is slowly progres-
sive but sometimes visual loss can be acute and mimic
optic neuritis or ischemic optic neuropathy.
IMAGING
• The imaging appearance of optic nerve and chiasmal
infiltration by lymphoma is nonspecific. The
infiltrated optic nerve is enlarged and reveals
increased density on CT scanning and enhances after
contrast
• On MR imaging, they are iso-, hypo- or hyperintense
on T1-weighted MRI and hyperintense on T2. Usually,
they enhance well with contrast.
BIBLIOGRAPHY
1. Fierz AB, Sartoretti S, Thoelen AM. Optic neuropathy and
central retinal artery occlusion in non-Hodgkin’s
lymphoma. J Neuro-ophthalmol 2001;21(2):103-5.
2. Howard JG, Lee AG, Garwood M, Link BK, Wooldridge
JE, Kirby P. Optic neuropathy due to anaplastic large cell
lymphoma. Semin Ophthalmol 2004;19(3-4):81-7.
3. Lee LC, Howes EL, Bhisitkul RB. Systemic non-Hodgkin’s
lymphoma with optic nerve infiltration in a patient with
AIDS. Retina 2002;22(1):75-9.
4. Pedraza S, Osuna MT, Guardia R, Abadal M, Teruel J,
Vera-Sancho J, Tarrus J. Infiltration of the optic nerve by
lymphoma. Diagnosis by magnetic resonance imaging.
Rev Neurol 2002;35(11):1027-9.
 Chapter 30 Infiltrative Optic Neuropathy 385

Fig. 30.17: Axial CT scan of the orbit showing diffuse infiltration of the right medial orbit and
the optic nerve by hyperdense soft tissue. Typical moulding by lymphomatous deposits along
the posterior ocular surface is noted on the left
386 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
OPTIC NERVE METASTASES
• Metastases to optic nerve constitute 12 percent of all
the cases of secondary optic nerve tumors. The
diagnosis may be difficult due to their protean
manifestations
• Prompt recognition may help in the detection of a
previously unrecognized systemic malignancy or
allow early treatment.
MECHANISM OF SPREAD
• Hematogenous dissemination of tumor cells is the
most common form of spread. Optic nerve head
involvement by metastases is a rare condition
occurring in only 5 percent of all the ocular metastatic
cases
• The chances of optic nerve, being the only ocular
structure to be affected, is rare, occurring in only 1.3
percent of the cases. The ocular structures that are
usually affected along with optic nerve head are the
choroids and the retina
• In about, 39 percent of the patients, secondaries
develop from intraocular metastases and in 33 percent
of the patient, from direct hematogenous spread to
the optic nerve.
SOURCE OF THE PRIMARY TUMORS
• The most common sources of metastatic carcinoma
to the optic disc are breast carcinomas (43%) in females
and lung carcinomas (27%) in males. Other primary
sources are stomach, pancreas, intestine (3%), kidney
(3%), prostate (3%), melanoma, uterus and ovary
• In 20 percent cases, the primary source is never found
and optic nerve involvement is the first and the only
finding
• Females are more affected than males. The mean age
of presentation is 55 years
• They can have a perineural spread and involve the
adjacent parts in proximity.
CLINICAL FEATURES
• The most common presenting symptom is loss of
vision; the other symptoms being visual field defect,
pain and photopsia depending upon other ocular
structures that might be concurrently involved. The
symptoms will include all features of optic nerve
dysfunction
• Fundus examination may reveal gross disc edema
(84%), adjacent juxtapapillary choroidal involvement
(74%), and distinct nodule (16%)
• Other posterior pole findings, in a case of optic nerve
head metastases, may be secondary disc edema,
buried disc blood vessels (74%), splinter hemorrhages
(42%). Patients may present only with optic nerve
head pathology (26%)
• The chance of other eye being affected is 25 percent
but the chance of optic nerve affection of the other
eye is only 3 percent.
IMAGING
• Contrast-enhanced MRI brain and orbit is the
preferred imaging of choice
• The imaging findings include nodular enhancing
lesion at the disc and optic nerve head, diffuse
thickening of the optic nerve and sheath which may
mimic optic neuritis/perineuritis on imaging
• There may be associated choroidal thickening and
bilateral involvement
• The lesions display iso- to hypointense in T1WI and
hyper/hypointense in T2WI
• Brain imaging should be done with contrast—
presence of intracranial lesions may aid in the
diagnosis in cases with unknown primary.
BIBLIOGRAPHY
1. Biswas J, Ho TC, Bhavsar K. Bilateral metastasis to the
retina, choroids and optic nerve from breast cancer: A
clinicopathological case. Indian J Ophthalmol 2007;55(1):
71-2.
2. Shields JA, Shields CL, Singh AD. Metastatic neoplasms
in the optic disc: The 1999 Bjerrum Lecture, Part 2. Arch
Ophthalmol 2000;118(2):217-24.
3. Takagi T, Yamaguchi T, Mizoguchi T, Amemiya T. A case
of metastatic optic nerve head and retinal carcinoma with
vitreous seeds. Ophthalmologica 1989;199(2-3):123-6.
4. Adachi N, Tsuyama Y, Mizota A, Fujimoto N, Suehiro S,
Adachi-Usami E. Optic disc metastasis presenting as an
initial sign of recurrence of adenoid cystic carcinoma of
the larynx. Eye (Lond) 2003;17(2):270-2.
5. Tamhankar MA, Volpe NJ, Loevner LA, Palmer JN,
Feldman M. Primary sinonasal undifferentiated carcinoma
presenting with bilateral retrobulbar optic neuropathy.
J Neuro-ophthalmol 2007;27(3):189-92.
 Chapter 30 Infiltrative Optic Neuropathy 387

A B

C D

Figs 30.18A to D: A 50-year-old male presented with sudden loss of vision in the left eye associated with headache: (A) Axial T2-
FRFSE image at the level of optic nerve showing thickening of the left optic nerve, (B) Postcontrast fat-suppressed T1-weighted
image showing enhancement of the left optic nerve sheath, (C) Sagittal postcontrast T1-weighted image and (D) Axial postcontrast
T1-weighted image of the brain showing nodular enhancing lesions in the infra- and supratentorial brain parenchyma (arrows)—
subsequently found to have small cell carcinoma of the lung
388 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 30.19A and B: A 40-year-old female, a known case of carcinoma breast presented with bilateral visual loss: (A) Axial
postcontrast T1-weighted image and (B) Coronal T1-fat-suppressed postcontrast image showing enhancing optic nerve sheaths
suggestive of diffuse infiltration
Chapter 31
Traumatic Optic Neuropathy
• Traumatic optic neuropathy (TRON) results from
trauma to the orbital rim or frontal area. May also
result following iatrogenic injury such as endoscopic
sinus surgery or orbital surgery. Rarely results from
orbital hemorrhage (retrobulbar hemorrhage) or
orbital emphysema. They are divided into two types:
1. Direct injury that results from orbital or cerebral
trauma that transgresses normal tissue planes to
disrupt the anatomic and functional integrity of
the optic nerve, e.g. bullet penetrating orbit. Vision
loss is severe, immediate and recovery is unlikely
2. Indirect injury usually results from blunt trauma
to the forehead that results in transmission of force
through the cranium to the restrained
intracanalicular portion of optic nerve. Vision loss
may be delayed and recovery is poor. They are
classified into three types:
i. Optic nerve avulsion—ophthalmoscopic
appearance consists of a partial ring of
hemorrhage or the avulsion can be seen as a
dark crescentic area
ii. Anterior optic neuropathy—injury within
10 mm of the globe. Central retinal artery or
vein occlusion may occur
iii. Posterior optic neuropathy—injury posterior
to entrance of central retinal artery or vein.
CLINICAL FEATURES
• The condition may manifest immediately or within
hours or days following the trauma. Occasionally, the
vision loss may be insidious and in some rare cases,
the patient may not be aware of any visual deficit until
it is detected by routine examination
• Examination reveals variably reduced acuity with
visual field defects. An afferent pupillary defect is
characteristic and dyschromatopsia may be noted in
accordance with the severity of the vision loss
• Initially, a completely normal fundus may be seen. In
other cases, a grossly edematous optic nerve head,
vitreous hemorrhage, venous congestion or retinal
edema may be seen. In the vast majority of cases,
however, optic disc pallor ensues within several weeks
of the injury
• Multisystem trauma or serious brain damage with loss
of consciousness may be present. Periorbital or ocular
hemorrhage, ecchymosis or laceration may be present.
IMAGING
• In TRON, it is preferable to do a CT scan of orbit
with 1 to 2 mm sections through the optic canal in the
axial and coronal planes to rule out canal fracture
compromising the canal. Routine sections of the brain
should also be taken
• CT scan also helps in delineating orbital wall fractures,
orbital and optic nerve sheath hemorrhage and orbital
emphysema, penetrating orbital foreign bodies
• In patients suspected to have traumatic optic
neuropathy, CT scan should be performed followed
by MRI, if the CT scan is normal
• MRI is superior to CT for soft tissue injuries.
OPTIC NERVE AVULSION
• Usually occurs following severe orbital trauma but
numerous cases have reportedly occurred after
seemingly minor ocular or orbital trauma
• The mechanism of avulsion has been postulated to be:
(1) Profound rotation of the globe, causing a fracture
of the nerve from the scleral lamina cribrosa; (2)
Markedly elevated intraocular pressure, resulting in a
blow-out of the optic nerve from the lamina cribrosa;
or (3) A sudden retropulsion of the globe within the
orbit, followed by a sudden anteriorly directed
repositioning that tears the nerve from the lamina
390 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B

Figs 31.1A to C: (A) Axial T2-FSE of the orbit at the level of the
optic nerve using head coil shows a thickened left optic nerve, but
could not confirm the optic nerve avulsion, (B) Axial T2-FSE and
(C) Coronal T2-FSE using a surface coil delineates avulsion of the
C optic nerve from the lamina cribrosa (arrow)

• Orbital CT scanning or MRI may reveal avulsion of
the nerve but frequently shows the optic nerve sheath
to be intact. There may be edema of the optic nerve
head on imaging as an indirect sign of avulsion.
Ophthalmic ultrasonography may also demonstrate
separation of the nerve head from the sclera
• MRI of the orbit using surface coil is recommended.
OPTIC NERVE TRANSECTION
• Usually arises in the context of severe orbital fractures
or penetrating orbital injury or following surgery
(endoscopic sinus surgery)
• Orbital imaging may be used to support the diagnosis.
An MRI is preferable to outline the optic nerve, where-
as CT scan should be done in patients suspected to
have fractures and foreign bodies.
DIFFUSE AND LOCALIZED ORBITAL
HEMORRHAGE (HEMATOMA)
• Nonlocalized orbital soft tissue hemorrhage
frequently follows periorbital or midfacial trauma.
Eyelid and subconjunctival hemorrhage frequently
accompany the condition. Diffuse hemorrhage can
result in displacement of adjacent orbital structures.
The clinical presentation of patients with localized
orbital hemorrhage parallels those with diffuse orbital
hemorrhage
• Diagnosis is facilitated by means of orbital CT
scanning or MRI. CT scan will show diffuse or focal
hyperdense soft tissue in the retrobulbar fat. The
density will vary with duration of hemorrhage. MRI
will help in determining the age of hematoma and its
relationship to the optic nerve.
OPTIC NERVE SHEATH HEMATOMA
• Localized hemorrhage within or along the optic nerve
sheath can result in neuropathic vision loss. Optic
Chapter 31 Traumatic Optic Neuropathy 391
neuropathy can result from a very small quantity of
hemorrhage within the optic nerve sheath , which can
be easily missed on CT scan
• MRI should be done in these patients, after excluding
intraorbital foreign body.
TENSION PNEUMO-ORBITUS (ORBITAL EMPHYSEMA)
• Orbital fractures adjacent to the paranasal sinus can
allow the passage of air into the orbit space and
orbital soft tissues. Hairline fractures, particularly
of the medial orbital wall, occasionally create a
one-way valve effect that entraps this air within the
otherwise compartmentalized orbital space and can
cause vascular insufficiency of the optic nerve and
retina
• CT scan will show the fracture of the orbital wall and
air within orbit (– 1000 HU).
BIBLIOGRAPHY
1. Arkin MS, Rubin PA, Bilyk JR, Buchbinder B. Anterior
chiasmal optic nerve avulsion. Am J Neuroradiol 1996;
17(9):1777-81.
2. Foster BS, March GA, Lucarelli MJ, Samiy N, Lessell S.
Optic nerve avulsion. Arch Ophthalmol 1997;115(5):623-30.
3. Lustrin ES, Brown JH, Novelline R, Weber AL. Radiologic
assessment of trauma and foreign bodies of the eye and
orbit. Neuroimaging Clin N Am 1996;6(1):219-37. Review.
4. Steinsapir KD, Goldberg RA. Traumatic optic neuropathy.
Surv Ophthalmol 1994;38(6):487-518. Review.
5. Steinsapir KD. Traumatic optic neuropathy. Curr Opin
Ophthalmol 1999;10(5):340-2. Review.
6. Tsai HH, Jeng SF, Lin TS, et al. Predictive value of
computed tomography in visual outcome in indirect
traumatic optic neuropathy complicated with periorbital
facial bone fracture. Clin Neurol Neurosurg
2005;107(3):200-6.
7. Zimmer-Galler IE, Bartley GB. Orbital emphysema: Case
reports and review of the literature. Mayo Clin Proc 1994;
69(2):115-21.
392 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

Fig. 31.2: Avulsion of optic nerve with subluxation of the globe—

Axial CT scan of the orbit showing subluxation of the left globe
with avulsion of the optic nerve and medial and lateral rectus
muscles with retrobulbar hemorrhage. Note: The large (L) medial
wall fracture

A B

Figs 31.3A and B: A 19-year-old female presented with sudden loss of vision and esotropia following FESS surgery: (A) Axial
T1-weighted image and (B) Coronal T1-weighted image of the orbit showing fractured right medial wall with nonvisualization of
the medial rectus and irregularity of the medial margin of the optic nerve sheath and nerve suggestive of an incomplete transection
of the optic nerve
 Chapter 31 Traumatic Optic Neuropathy 393

A B

Figs 31.4A and B: Axial CT scan of the orbit: (A) Bone window setting and (B) Soft tissue setting showing fracture of the left optic
canal (white arrow) with fragments compressing the optic nerve. Note: Hemorrhage in the adjacent sinus

A B

Figs 31.5A and B: (A) Axial CT scan of the orbit and (B) Coronal CT scan of the orbit in bone window setting at the level of the optic
canal showing fracture of the left optic canal with fragments within the canal (arrow). Note: Fracture in right sphenoid wing—squamous
temporal bone
394 Section 4 Visual Pathway Lesions: Optic Nerve Disorders

A B C

Figs 31.6A to C: (A) Axial FLAIR, (B) Axial T2 fast spin echo and (C) Axial T1 spin echo showing thickening of the chiasm with T1-
hyperintense signal involving the right half of the chiasm (arrow)—suggestive of chiasmal hemorrhage. Note: The fracture of the
frontal sinus wall with hemosinus

Fig. 31.7: Axial T1-weighted image of the orbit showing a

rounded slightly hyperintense soft tissue in the medial intraconal
compartment (hematoma) displacing the optic nerve. Right
temporal hemorrhagic contusion is also noted (arrow)
 Chapter 31 Traumatic Optic Neuropathy 395

A B

C D

Figs 31.8A to D: (A) Axial FLAIR, (B) Coronal T2-weighted image, (C) Coronal T1-weighted image and (D) Coronal T2-weighted
image of the optic nerve showing T1-hyperintense signal in the right basifrontal region indicative of subacute blood (black arrows).
The right intracranial prechiasmatic optic nerve is thickened with T2-hyperintense signal (white arrow)—optic nerve contusion
(Fig. D)
Chapter 32
Radiation Optic Neuropathy
• It is an ischemic disorder of the optic nerve that leads
to severe irreversible loss of vision months to years
after radiation to brain and orbit
• It occurs after radiation of paranasal sinuses and skull
base malignancies, parasellar meningiomas, cranio-
pharyngioma, frontal gliomas, radiation to intraocular
tumors, after stereotactic radiosurgery, radiation to
thyroid orbitopathy (especially in diabetics). Patients
who have received combined chemotherapy and
radiotherapy and patients with pituitary adenoma
(especially growth hormone secreting adenomas) are
at an increased risk of developing radiation neuropathy
• Pathogenesis (delayed radionecrosis)—primarily a
vascular damage with subsequent neuronal injury.
This leads to ischemic demyelination, reactive
astrocytosis, endothelial hyperplasia, obliterative
endarteritis and fibrinoid necrosis
• The risk of radiation optic neuropathy (RON)
increases at doses over 50 Gy. Tolerance level of optic
nerve is 42 to 50 Gy. In cases, receiving radiation to
PNS and pituitary tumors RON can develop at doses
between 42 and 50 Gy.
SYMPTOMS
There is a rapid and progressive painless loss of vision in
one or both eyes leading to irreversible blindness. Vision
loss may be sequential. Onset is 3 months to 8 years
(usually within 3 years). Eighty-five percent of eyes have
final visual acuity of 20/200 or worse.
SIGNS
Most patients have a normal disc that subsequently
becomes pale over 4 to 6 weeks. The visual field may show
altitudinal defects or central scotomas or bitemporal
hemianopia with radionecrosis of chiasm. It can rarely
present as an anterior optic neuropathy characterized by
disc swelling, hemorrhages and exudates.
IMAGING
MRI with contrast is the imaging modality of choice. Any
part of the optic nerve, optic chiasm and optic tract can
be affected. The involved optic nerve will be thickened
and displays T2-hyperintense signal and will show
enhancement on postcontrast scans. The enhancement
usually resolves in several months at which time visual
function usually stabilizes.
Differential Diagnosis
• Recurrence of primary tumor
• Arachnoiditis
• Radiation-induced parasellar tumors
MRI helps in differentiating RON from the above.
BIBLIOGRAPHY
1. Brown GC, Shields JA, Sanborn G, Augsburger JJ, Savino
PJ, Schatz NJ. Radiation optic neuropathy. Ophthalmol
1982;89(12):1489-93.
2. Ebner R, Slamovits TL, Friedland S, Pearlman JL, Fowble
B. Visual loss following treatment of sphenoid sinus
carcinoma. Surv Ophthalmol 1995;40(1):62-8.
3. Girkin CA, Comey CH, Lunsford LD, Goodman ML, Kline
LB. Radiation optic neuropathy after stereotactic
radiosurgery. Ophthalmol 1997;104(10):1634-43.
4. Guy J, Mancuso A, Beck R, Moster ML, Sedwick LA,
Quisling RG, Rhoton AL Jr, Protzko EE, Schiffman J.
Radiation-induced optic neuropathy: A magnetic
resonance imaging study. J Neurosurg 1991;74(3):
426-32.
5. Hudgins PA, Newman NJ, Dillon WP, Hoffman JC Jr.
Radiation-induced optic neuropathy: Characteristic
appearances on gadolinium-enhanced MR. Am J
Neuroradiol 1992;13(1):235-8.
 Chapter 32 Radiation Optic Neuropathy 397

A B

Figs 32.1A to C: A 48-year-old female presented with sudden

loss of vision in the left eye with history of radiation given for a
pituitary adenoma one year back: (A) Postcontrast coronal
image, (B) Axial fat-suppressed T1-weighted image showing
thickening and enhancement of the left intracranial optic nerve
(arrows) and (C) Coronal T2-weighted scan showing thickening
C of the left intracranial optic nerve with T2-hyperintense signal
398 Section 4 Visual Pathway Lesions: Optic Nerve Disorders
6. Kellner U, Bornfeld N, Foerster MH. Radiation-induced
optic neuropathy following brachytherapy of uveal
melanomas. Graefes Arch Clin Exp Ophthalmol 1993;231
(5):267-70.
7. Kline LB, Kim JY, Ceballos R. Radiation optic neuropathy.
Ophthalmol 1985;92(8):1118-26.
8. Lessell S. Magnetic resonance imaging signs may antedate
visual loss in chiasmal radiation injury. Arch Ophthalmol
2003;121(2):287-8.
9. McClellan RL, el Gammal T, Kline LB. Early bilateral
radiation-induced optic neuropathy with follow-up MRI.
Neuroradiol 1995;37(2):131-3.
10. Piquemal R, Cottier JP, Arsene S, Lioret E, Rospars C,
Herbreteau D, Jan M, Renard JP. Radiation-induced
optic neuropathy 4 years after radiation: Report of a
case followed up with MRI. Neuroradiol 1998;40(7):
439-41.
11. Tachibana O, Yamaguchi N, Yamashima T, Yamashita J.
Radiation necrosis of the optic chiasm, optic tract,
hypothalamus, and upper pons after radiotherapy for
pituitary adenoma, detected by gadolinium-enhanced,
T1-weighted magnetic resonance imaging: Case report.
Neurosurg 1990;27(4):640-3.
12. Young WC, Thornton AF, Gebarski SS, Cornblath WT.
Radiation-induced optic neuropathy: Correlation of MR
imaging and radiation dosimetry. Radiol 1992;185(3):
904-7.
13. Zimmerman CF, Schatz NJ, Glaser JS. Magnetic resonance
imaging of radiation optic neuropathy. Am J Ophthalmol
1990;110(4):389-94.
 Chapter 32 Radiation Optic Neuropathy 399

A B

C D

Figs 32.2A to D: A 38-year-old male with multiple myeloma presented with bilateral sudden loss of vision 3 months following
whole brain radiation: (A) Axial FLAIR and (B to D) Coronal FLAIR at various visual pathway levels showing diffuse hyperintense
signal in nerves, chiasm (long arrow) and optic tract (arrowhead) suggestive of radiation neuropathy
SECTION 5
Disorders of Chiasm
SECTION OUTLINE
33. Anatomy and Clinical Features
34. Chiasmal Disorders in Pediatric: Young Adult
35. Chiasmal Disorders in Middle Age: Elderly
36. Chiasmal Disorders: No Age Predilection
Chapter 33
Anatomy and Clinical Features
INTRODUCTION
• Chiasmal syndromes can result from disease
processes primarily involving the chiasm or lesions
extrinsic to the chiasm causing its compression
• Imaging is challenging as there are variety of disease
processes that involve the chiasm
• The knowledge of imaging anatomy and the
relationship of the chiasm to the surrounding
structures is of utmost importance in identifying the
origin of the pathology for the management.
ANATOMY OF THE OPTIC CHIASM
• The optic chiasm is a flat-oblong structure formed by
union of the intracranial optic nerves
• The optic chiasm has a transverse diameter of 12 to
18 mm, anteroposterior width of 8 mm and 4 mm
thickness
• It lies over tuberculum sellae of sphenoid bone at a
45 degree angle, lower anteriorly than posteriorly and
rest over the pituitary gland (10 mm above) delimited
by the diaphragma sella
• The chiasm is in direct contact with cerebrospinal fluid
anteriorly in subarachnoid space and posteriorly in
the third ventricle. Chiasm forms part of the anterior
inferior wall of the third ventricle
• Bony boundaries of the sella include the tuberculum
sella anteriorly (a small protrusion that continues
anteriorly with planum sphenoidale, this is a common
location for meningioma), the dorsum sella posteriorly
and the anterior and posterior clinoid processes
superiorly
• The cavernous sinuses containing cranial nerves III,
IV, V1, V2 and VI, internal carotid arteries and
sympathetic fibers form the lateral wall of the sella
• When the carotid arteries emerge from the cavernous
sinuses, then they are located beneath and lateral to
the optic nerves. The vessels travel along the lateral
and inferior surfaces of the optic chiasm and branch
into anterior and middle cerebral arteries. The anterior
cerebral arteries cross the superior surface of the optic
nerves and they are connected in the midline by the
anterior communicating artery
• The position of the chiasm can be described by its
relation to the diaphragma sella and pituitary gland
as prefixed, normal or postfixed.
CLINICAL FEATURES
• Visual loss is often the initial manifestation of disorders
involving the optic chiasm in addition to compression
of neighboring structures including pituitary gland and
may produce other systemic manifestations
• Visual loss is usually insidious. Acute visual loss could
be due to rapidly expanding mass, vascular anomalies,
infections or inflammation. This situation can even
mimic retrobulbar neuritis
• Loss of temporal visual fields may result in a inability
to recognize the objects beyond the point of fixation
(Postfixation blindness)
• Diplopia: Because of its close proximity to the caver-
nous sinus, lesions involving the cavernous sinus can
involve the optic chiasm and vice versa causing III,
IV and VI nerve palsies resulting in diplopia.
Binocular diplopia (hemifield slide) may also occur
in patients with loss of sensory fusion despite of
normal ductions and versions
• Nystagmus: See-Saw nystagmus is an uncommon form
of nystagmus may occur in patients with suprasellar
tumors.
404 Section 5 Disorders of Chiasm

Fig. 33.1A: Brain section showing optic chiasm: (1) Pons; Fig. 33.1B: Optic chiasmal orientation of nerve fibers. Blue—
(2) Internal carotid artery; (3) Optic nerve; (4) Optic chiasm; superior fibers and red—inferior fibers
(5) Optic tract; (6) Oculomotor nerve; (7) Basilar artery (Image
copyright—Jerry Vriend)

Fig. 33.1C: Pituitary mass causes superior temporal quadrant Fig. 33.1D: Classic bitemporal hemianopia—due to pituitary
(STQ) defects earlier due to inferior fibers involvement and adenoma
suprasellar masses cause inferior temporal quadrant (ITQ)
defects earlier due to involvement of superior fibers

NEURO-OPHTHALMOLOGIC EXAMINATION
• Visual acuity can be decreased in one or both eyes
• Pupillary light reaction can be abnormal
• Color vision can be affected. Bitemporal hemia-
chromatopsia to red may be localized to the optic
chiasm. This can be tested easily at bedside
• Perimetry is the most important clinical tool for
detecting the chiasmal lesions. The classic visual field
defect is a bitemporal hemianopia because of
involvement of the crossing nasal retinal fibers
• However, precise type of field defect depends on the
anatomy of the optic chiasm and its relation to the
causative lesion. More anterior lesions may also
involve the optic nerves resulting in central scotoma
and more posterior lesions may involve the optic
tracts resulting in a homonymous hemianopia
• Loss of central visual function with contralateral
visual field defect may occur (junctional scotoma)
most commonly from lesion involving the anterior
portion of the chiasm at its junction with intracranial
optic nerve
• Any form of temporal field defect, even if monocular,
can result from chiasmal compression.
OPHTHALMOSCOPE EXAMINATION
• Optic atrophy is seen frequently. It is generally a
horizontal-oriented atrophy (i.e. bow-tie) that
corresponds to the topographic localization of the
nasal retina within the optic nerve
• Papilledema is exceptional, seen only in patients with
pituitary apoplexy or third ventricle obstruction
• Less frequent optic atrophy with increased cup-to-disc
ratio resembling glaucomatous optic atrophy can
occur
• These abnormalities may be present in isolation or
in association with physical changes associated
with endocrine dysfunction. Hypopituitarism can
be the result of compression of normal pituitary
tissue, or impaired blood supply to the normal
tissue or interference with hypothalamic production
and delivery of releasing hormones. Pituitary
hormones excess caused by hypersecreting pituitary
adenomas.
NEUROIMAGING
• High-resolution MRI with contrast is the procedure
of choice in imaging the optic chiasm and provides
excellent imaging of the soft tissues surrounding it
Chapter 33 Anatomy and Clinical Features 405
• Normally on coronal MR images, the body of the
chiasm has dumb-bell shape, and is located in the
middle of the suprasellar cistern
• Posteriorly, the chiasm lies sandwiched between the
third ventricle above and pituitary stalk below outline
by CSF
• On coronal images, the pituitary gland has a flat
superior border
• The pituitary gland displays signal similar to the gray
matter on both T1 and T2-weighted images in adults.
In neonates and infants up to 2 months of age, the
pituitary gland is T1-hyperintense relative to the brain
parenchyma. The posterior pituitary bright spot may
be seen in only two-thirds of the infants
• On sagittal sections, the tilted chiasm is easily
identified above the pituitary. On T1-weighted
images, the anterior pituitary is isointense with the
brain parenchyma. The posterior pituitary is seen as
bright spot
• The normal gland height varies in children,
adolescence, pregnancy and postpartum where the
gland may have a convex upper margin any reach a
height of 10 to 12 mm. The normal gland height is of
up to 6 mm and stalk thickness is 2 to 3 mm
• MRI has replaced CT as per procedure of choice in
imaging the optic chiasm. Soft tissue resolution is
enhanced without interference of bony artifacts. Direct
multiplanar imaging the axial, coronal and sagittal
planes are the advantages of MRI
• Although MRI is the best technique for chiasmal
imaging, CT plays a complementary role for detection
of bony changes and calcification.
Criteria suggestive of chiasmal disease include:
1. Changes in normal size, shape or signal of the
chiasm
2. Presence of abnormal mass with or without
contrast enhancement in the perichiasmal region
3. Distortion of the anterior inferior third ventricle.
OTHER EVALUATIONS
• Endocrinological evaluation should be considered in
all patients with pituitary and infundibular lesions
• Lumbar puncture if neuroimaging suggests an
infectious, inflammatory, or infiltrative disorder
• The chiasmal lesions will be discussed according to the
age groups as—(1) Pediatric, (2) Middle age and (3)
Elderly.
406 Section 5 Disorders of Chiasm

Fig. 33.2: Coronal T2-weighted images anterior to the chiasm Fig. 33.3: Coronal T2-weighted image at the level of the
showing the intracranial optic nerves converging to form the suprasellar cistern showing flat dumb-bell-shaped chiasm in the
chiasm (black arrows). Note: The relation to the cavernous midline. The supraclinoid internal carotid arteries are lateral,
internal carotid arteries (white arrows) infundibulum and pituitary gland are inferior to the chiasm

Fig. 33.4: Sagittal T1-weighted precontrast image showing the

relationship of the chiasm to the pituitary gland. Note: The
posterior pituitary bright spot

BIBLIOGRAPHY
1. Carpenter MB. Core text of neuroanatomy. Baltimore: MD
Williams and Wilkins.
2. Elster AD. Modern imaging of the pituitary. Radiol 1993;
187:1-14.
3. Fujimoto N, et al. Criteria for early detection of temporal
hemianopia asymptomatic pituitary tumors. Eye 2002;16:
731-8.
4. Grant T Liu, Nicholas J Volpe, Steven L Galetta. Neuro-
ophthalmology: Diagnosis and management 2nd edn.
Elsevier: 2010;720.
Chapter 33 Anatomy and Clinical Features 407
5. Osborn AG. Diagnostic neuroradiology. St Louis, Mosby
Year book 1994.
6. Rhoton A. The sellar region. Neurosurg 2002;51:335-74.
7. Rod Foroozan, et al. Chiasmal syndromes. Curr Opin
Ophthalmol 2003;14:325-31.
8. Saunders L, Hupp MD, et al. Magnetic resonance imaging
of optic chiasm. Surv Ophthalmol 1991;36:3.
9. Snell's Anatomy for medical students, 3rd edition.
Chapter 34
Chiasmal Disorders
in Pediatric: Young Adult
The common lesions causing chiasmal disorders in children are tumors such as hypothalamic chiasmatic gliomas
and craniopharyngioma, infections such as tuberculosis. Lesions such as dermoid/epidermoid, histiocytosis and
germinomas are other lesions that can affect the chiasm in children.

HYPOTHALAMIC CHIASMATIC GLIOMA

• Distinction between chiasmatic and hypothalamic
gliomas often depends on the predominant position
of the lesion
• Origin of large lesions cannot be determined as the
hypothalamus and chiasm cannot be identified
separately. Therefore, hypothalamic and chiasmatic
gliomas are discussed as a single entity
• Tumors of chiasmal origin are more aggressive than
those originating from optic nerve
• Seen mostly in children less than 10 years of age with
equal M:F ratio
• Association of hypothalamic—chiasmatic glioma and
neurofibromatosis is seen in 33 percent of the patients
• Histologically, they are pilocytic astrocytomas
• Anaplastic astrocytoma or glioblastoma multiforme
may occur in patients over 50 years
• Clinical presentation—monocular or binocular visual
disturbances, hydrocephalus or hypothalamic
dysfunction
• Diencephalic syndrome is rare but a characteristic
presentation of this tumor comprising of profound
emaciation, locomotor hyperactivity, lack of cutaneous
adipose tissue, euphoria and alertness. Gliomas
presenting with these symptoms tend to be larger and
more aggressive with younger age of presentation.
IMAGING
• These are slow-growing tumors and hence attain a
considerable size at the time of presentation.
Calcification and hemorrhage are rare
• Thickening of optic nerves and presence of a
suprasellar lesion help to differentiate it from other
suprasellar lesions.
CT Scan Findings
• These are usually mixed solid cystic lesions. Cystic
component shows peripheral enhancement. Solid
lesions are isodense and show heterogeneous contrast
enhancement
• Involvement of the optic nerves may be difficult to
appreciate on CT scan unless, there is widening of
the optic canal, an important finding which will help
to differentiate from other suprasellar tumors.
MRI Features
• MRI with gadolinium is the imaging of choice as it
helps in identifying the involvement of the optic
nerves and retrochiasmal visual pathways
• These tumors are usually isointense to hypointense
on T1-weighted images and moderately to markedly
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult 409

Fig. 34.1: A 9-year-old female with bilateral gradual loss of vision. Axial CT scan of the brain showing a large lobulated hypodense
lesion in the suprasellar cistern showing peripheral enhancement. HPE—hypothalamic—chiasmatic glioma
410 Section 5 Disorders of Chiasm
hyperintense in T2-weighted images. Fifty percent
show enhancement with contrast
• They tend to invade the brain along the visual
pathway
• Patients with neurofibromatosis have multiple white
matter lesions along the visual pathways and
brainstem that can mimic tumor extension along the
optic radiations. These are seen as hyperintense foci
on T2-weighted images. Absence of interval growth
and absence of contrast enhancement help to
differentiate these lesions from tumor infiltrations. MR
spectroscopy may be useful in these patients.
DIFFERENTIAL DIAGNOSIS
• Craniopharyngioma—if the lesion is predominantly
cystic (presence of calcification helps in differentiating
the two)
• Optic neuritis—if chiasmal thickening is the only
imaging finding
• Histiocytosis
• Hamartomas.
BIBLIOGRAPHY
1. Alshail E, Rutka JT, Becker LE, Hoffman HJ. Optic
chiasmatic-hypothalamic glioma. Brain Pathol 1997;7(2):
799-806. Review.
2. Davis PC, Hopkins KL. Imaging of paediatric orbit and
visual pathways: CT and MRI. Neuroimaging Clinics of
North America 1999;9(1):93-114.
3. Faerber EN, Roman NV. Central nervous system tumors
of childhood. Radiological Clinics of North America 1997;
35(6):1301-28.
4. Fletcher WA, Imes RK, Hoyt WF. Chiasmal gliomas:
Appearance and long-term changes demonstrated by
computerized tomography. J Neurosurg 1986;65(2):
154-9.
5. Herbert B Newton, Ferenc A Jolesz, Mark G Malkin.
Handbook of Neuro-Oncology Neuroimaging 2007;427-8.
6. Holman RE, Grimson BS, Drayer BP, Buckley EG, Brennan
MW. Magnetic resonance imaging of optic gliomas. Am J
Ophthalmol 1985;100(4):596-601.
7. Jamshidi S, Korengold M, Kobrine AI. Computed
tomography of an optic chiasm glioma in an elderly
patient. Surg Neurol 1984;21(1):83-7.
8. Reider-Groswasser I, Nemet P, Godel V. Glioma of the
anterior optic pathways. Comput Radiol 1985;9(6):351-3.
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult 411

A B

Figs 34.2A and B: A 2-year-old female child presented with see-saw nystagmus: (A) Axial CT scan of brain at the level of the
suprasellar cistern and (B) Showing asymmetric thickening of the chiasm

A B

Figs 34.3A and B: (A) Coronal T1-weighted image and (B) Sagittal T1-weighted image of the brain of the above patient showing
thickened and distorted chiasm (arrows)
412 Section 5 Disorders of Chiasm

A B

C D

Figs 34.4A to D: (A) Axial FLAIR, (B) Axial T2-weighted image, (C) Sagittal T1-weighted image, (D) Coronal T1-weighted image
of the brain showing a large well-circumscribed lobulated mass lesion in the suprasellar cistern displaying hypointense signal in
T1-weighted images and hyperintense signal in T2-weighted images. The chiasm cannot be identified separately.
Coronal T1-weighted image (Fig. D) showing extension into the left optic nerve, which appears thickened (white arrow)—
optochiasmatic glioma
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult 413

A B

Figs 34.5A to C: (A) Sagittal T2-weighted image, (B) Sagittal

T1-weighted image showing T1-hypointense and T2-
hyperintense predominantly cystic mass. Figure A is a right
parasagittal image showing the thickened right intracranial optic
nerve (black arrow) and (C) Coronal T2-weighted image at the
level of the optic canals showing thickened right optic nerve
C (arrow) indicating a optochiasmatic glioma
414 Section 5 Disorders of Chiasm
CRANIOPHARYNGIOMA
• Most common suprasellar mass in children
• It has a bimodal peak. The first peak in the 1st to 2nd
decade and second peak in the 5th to 6th decade
• Histologically, there are two types—admantino-
matous seen in children and papillary seen in adults
and mixed
• Origin: These are epithelial in origin and derived from
remnants of Rathke’s pouch
• Common site: Suprasellar, intrasellar and third ventricle.
Uncommon sites—posterior fossa, pineal region and
nasal cavity
• Manifestation: Headache, visual (gradual decrease in
vision, asymmetric bitemporal defects), endocrine
abnormalities.
IMAGING
• Mostly, well-defined suprasellar mass lesion
• The admantinomatous types are mostly cystic with
calcification and solid components. Ninety percent
show calcification—clumps or peripheral rim
• The papillary type is usually solid and typically lack
calcification and is usually encapsulated. They are
frequently located in the third ventricle.
CT Features
• Plain CT findings: Hypodense cystic suprasellar lesion
with calcification
• Contrast CT: Cystic lesions show peripheral rim
enhancement. Solid craniopharyngiomas will show
heterogeneous moderate enhancement.
MRI Features
• Cystic component is hypointense on T1, if it contains
clear fluid and may be hyperintense in T1, if it contains
cholesterol, blood or proteinaceous fluid
• Solid component is isointense in T1 and hyperintense
in T2-weighted images
• Calcification is hypointense in T1 and T2-weighted
images
• With gadolinium solid component enhances and rim
enhancement is noted in the cystic component.
DIFFERENTIAL DIAGNOSIS
• Rathke’s cleft cyst
• Hypothalamic glioma
• Epidermoid and dermoid
• Arachnoid cyst.
Preferred Imaging
• The CT scan is superior to MR imaging in the detection
of calcification
• The MRI is superior to CT for determining tumor
content and extent. It provides valuable information
about the relationship of the tumor to surrounding
structures
• CT and MR have complementary roles in the
diagnosis of craniopharyngiomas.
BIBLIOGRAPHY
1. Baierl P, Fink U, Mayr B, Bauer WM, Oeckler R. Magnetic
resonance imaging in craniopharyngiomas. The
differential diagnosis of cystic intra- and suprasellar space-
occupying lesions. Rofo 1987;146(5):578-83.
2. Brunel H, Raybaud C, Peretti-Viton P, Lena G, Girard N,
Paz-Paredes A, Levrier O, Farnarier P, Manera L, Choux
M. Craniopharyngioma in children: MRI study of 43 cases.
Neurochirurgie 2002;48(4):309-18.
3. Freeman MP, Kessler RM, Allen JH, Price AC.
Craniopharyngioma: CT and MR imaging in nine cases.
J Comput Assist Tomogr 1987;11(5):810-4.
4. Hamamoto Y, Niino K, Adachi M, Hosoya T. MR and CT
findings of craniopharyngioma during and after radiation
therapy. Neuroradiology 2002;44(2):118-22.
5. Harwood-Nash DC. Neuroimaging of childhood
craniopharyngioma. Paediatric Neurosurgery 1994;21
(suppl 1):2-10.
6. Higashi S, Yamashita J, Fujisawa H, Yamamoto Y, Kadoya
M. "Moustache" appearance in craniopharyngiomas:
unique magnetic resonance imaging and computed
tomographic findings of perifocal edema. Neurosurgery
1990;27(6):993-6.
7. Hillman TH, Peyster RG, Hoover ED, Nair S, Finkelstein
SD. Infrasellar craniopharyngioma: CT and MR studies.
J Comput Assist Tomogr 1988;12(4):702-4.
8. Lee BC, Deck MD. Sellar and juxtasellar lesion detection
with MR. Radiology 1985;157(1):143-7.
9. Molla E, Marti-Bonmati L, Revert A, Arana E, Menor F,
Dosda R, Poyatos C. Craniopharyngiomas: Identification
of different semiological patterns with MRI. Eur Radiol
2002;12(7):1829-36. Epub 2001 Nov 20.
10. Parent AD, Fleischer AS, Marc JA, Ball TI. Giant
craniopharyngioma evaluated by computerized axial
tomography. J Pediatr Surg 1977;12(2):251-3.
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult 415

A B

Figs 34.6A and B: (A) Coronal CT scan and (B) Axial CT scan of the brain showing a well-circumscribed suprasellar cystic lesion
with clumps of calcification at the periphery. HPE—craniopharyngioma

A B

Figs 34.7A and B: (A) Axial contrast-enhanced CT scan and (B) Coronal contrast-enhanced CT scan of the brain showing a
mixed solid and cystic lesion in the suprasellar region with multifocal calcifications within. Note: The ill-defined margins of the
tumor which makes total surgical excision difficult
416 Section 5 Disorders of Chiasm
11. Pigeau I, Sigal R, Halimi P, Comoy J, Doyon D. MRI
features of craniopharyngiomas at 1.5 Tesla: A series of
13 cases. J Neuroradiol 1988;15(3):276-87.
12. Sartoretti-Schefer S, Wichmann W, Aguzzi A, Valavanis
A. MR differentiation of adamantinous and squamous-
papillary craniopharyngiomas. Am J Neuroradiol 1997;
18(1):77-87.
13. Tsuda M, Takahashi S, Higano S, Kurihara N, Ikeda H,
Sakamoto K. CT and MR imaging of craniopharyngioma.
Eur Radiol 1997;7(4):464-9.
14. Van Effenterre R, Boch AL. Craniopharyngioma in adults
and children: A study of 122 surgical cases. J Neurosurg
2002;97(1):3-11.
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult 417

Fig. 34.8: Noncontrast sagittal T1-weighted image of the brain showing

a well-circumscribed lobulated suprasellar mass lesion with mixed signal
intensities. The hyperintense signal (black arrow) represents the high
cholesterol content and hypointense signal (white arrow) represents
the cystic component with clear fluid. Note: Solid elements at the inferior
margin of the lesion (block arrow)

Figs 34.9A and B: (A) Axial T2-

weighted MRI and (B) Axial T1-
weighted image showing a solid
recurrent craniopharyngioma in the
suprasellar cistern in this 50-year-
old patient operated twice for
craniopharyngioma. Note the
bilateral small temporal subdural
A B collections (postoperative)

Figs 34.10A and B: (A) Coronal T1-

weighted image and (B) Sagittal
postcontrast T1-weighted image
showing a well-circumscribed thin-
walled cystic lesion in the supra-
sellar cistern with rim enhancement
noted in the postcontrast image. The
pituitary gland is seen separately
(dotted arrow)—cystic cranio-
A B pharyngioma
418 Section 5 Disorders of Chiasm
GERMINOMA
Germinomas are malignant tumors thought to originate
from neoplastic transformation of embryonic germ cells.
Germinomas account for 0.5 to 2% of primary intracranial
tumors. 20% occur in the suprasellar region.
LOCATION
• Germ cell tumors arise in midline locations, including
the gonads, mediastinum, retroperitoneum, and CNS
• Within the CNS, typical locations for germinomas are
the pineal region (most common), anterior third
ventricle (suprasellar and intrasellar), posterior third
ventricle, and rarely, fourth ventricle. Coexistence of
a suprasellar and pineal mass is highly suggestive of
a germinoma
• Suprasellar germinomas are either primary tumors
or metastatic lesions from the pineal germinomas.
AGE OF PRESENTATION
• Initial tumor symptoms usually begin in childhood,
but, most often, these tumors are diagnosed during
adolescence. Their peak incidence is 2nd to 3rd decade
• Suprasellar germinomas affect males and females
equally. Pineal germinomas have a male predomi-
nance (M:F ratio 9:1).
SIGNS AND SYMPTOMS
• These tumors manifest with symptoms and signs of
increased intracranial pressure, obstructive hydro-
cephalus, or both
• Parinaud’s syndrome is also observed in some cases
• Tumors located in the suprasellar region with potential
involvement of the third ventricle, hypothalamus, and
pituitary are observed more often in females than in
males. These tumors are associated with the classic triad
of symptoms which includes diabetes insipidus (DI),
hypopituitarism, and visual symptoms
• The endocrine work-up should include routine blood
and urine studies for DI, hypogonadism, hypo-
thyroidism, secondary adrenal insufficiency,
hyperprolactinemia, and any evidence of pituitary
dysfunction
• Spinal cord metastasis may be seen in 10 to 15 percent
of patients.
IMAGING STUDIES
CT Scan Findings
• Suprasellar germinomas are nonencapsulated lesions
with an infiltrative pattern. They have a propensity
for subarachnoid seedlings
• They are solid tumors, usually lacking cystic
component, homogeneous, fairly smooth marginated
often involving the infundibulum, optic chiasm, and
third ventricle
• They are isodense with moderate-to-marked contrast
enhancement
• Calcification is a characteristic finding in germinomas
especially pineal germinomas.
MRI Findings
• MRI is superior to CT in identifying the entire extent
of the lesion, simultaneous pineal tumor and CSF
dissemination
• Imaging should include postcontrast screening of the
spine for dissemination
• Germinomas appear isointense in T1-weighted
images and slightly hyperintense on T2-weighted
images, with marked enhancement with contrast
• Cystic degeneration is seen in 33 percent and CSF
seeding in 50 percent of pineal region germinomas
• MR spectroscopy will show a prominent lipid peak.
Recommendation: MRI brain with gadolinium is
recommended with screening of the spine.
BIBLIOGRAPHY
1. Balmaceda C, Modak S, Finlay J. Central nervous system
germ cell tumors. Semin Oncol 1998;25(2):243-50.
2. Capra M, Hargrave D, Bartels U, et al. Central nervous
system tumours in adolescents. Eur J Cancer 2003;39(18):
2643-50.
3. Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura
K, Nakamura O, Tamura A, Sano K. CT and MRI features
of intracranial germ cell tumors. J Neurooncol 1994;
19(3):217-26.
4. Gouliamos AD, Kalovidouris AE, Kotoulas GK,
Athanasopoulou AK, Kouvaris JR,Trakadas SJ, Vlahos LJ,
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult 419

A B

Figs 34.11A and B: (A and B) Axial postcontrast CT scan of the brain showing homogeneous contrast enhancing soild-rounded
lesions in the suprasellar and pineal region in the same patient—confirms germinoma (arrows)
420 Section 5 Disorders of Chiasm
Papavasiliou CG. CT and MR of pineal region tumors.
Magn Reson Imaging 1994;12(1):17-24.
5. Isselbacher KJ, Braunwald E, Wilson JD. Tumors of the
third ventricle and pineal region. In: Isselbacher KJ, Martin
JB, Fauci A, Braunwald E (Eds). Harrison's Principles of
Internal Medicine, 13th edn. New York, NY: McGraw-Hill;
1994;1908, 2265.
6. Lin Y, Gao P. CT and MR imaging of germinomas arising
in basal ganglia and thalamus. Zhonghua Yi Xue Za Zhi
1999;79(6):431-4. Chinese.
7. Moon WK, Chang KH, Han MH, Kim IO. Intracranial
germinomas: Correlation of imaging findings with tumor
response to radiation therapy. Am J Roentgenol 1999;
172(3):713-6.
8. Moon WK, Chang KH, Kim IO, Han MH, Choi CG,
Suh DC, Yoo SJ, Han MC. Germinomas of the basal
ganglia and thalamus: MR findings and a comparison
between MR and CT. Am J Roentgenol 1994;162(6):
1413-7.
9. Ochiai H, Yamakawa Y, Fukushima T, Sato Y, Hayashi T,
Yamada H. Delayed resolution of intracranial germinoma
after radiotherapy: A preliminary study of the correlation
between histology and magnetic resonance imaging.
Neuropathology 2000;20(3):190-6.
10. Scott W. Atlas: Magnetic resonance imaging of the brain
and spine, 4th edn. Lippincott-Williams and Wilkins
2008;(1):1156-8.
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult
ARACHNOID CYST
• Arachnoid cysts are intra-arachnoid cerebrospinal
fluid containing cysts that do not communicate with
the ventricular system
• Cysts in the middle cranial fossa are found more
frequently in males and on the left side
• Arachnoid cysts often are an incidental finding on
imaging and usually patients are asymptomatic even,
if the cyst is quite large
• The most commonly associated clinical features are
headache, calvarial bulging, and seizures, with focal
neurologic signs occurring less frequently. Some may
produce signs such as papilledema, cranial nerve palsies,
hydrocephalus, nystagmus and visual field defects
• The most effective surgical treatment appears to be
excision of the outer cyst membrane and cysto-
peritoneal shunting
• Arachnoid cysts can be associated with Cockayne
syndrome and Menkes disease.
IMAGING
CT Brain
• Arachnoid cysts are isodense to CSF and show
compression of the subarachnoid space. They may be
unilocular or septate and of variable size, although
the septa may not always be visible
• The adjacent brain tissue shows minimal mass effect
• They are characterized by sharp nonenhancing
borders
• Hydrocephalus may be present, depending on the
location of the arachnoid cyst
• CT cisternography yields mixed results, with some
cysts filling with contrast immediately while others
show delayed contrast accumulation or no filling. Non-
communicating and slow-filling cysts are regarded as
true arachnoid cysts while communicating cysts are
regarded as diverticula of the subarachnoid space.
MRI Findings
• On MRI images, arachnoid cysts appear as well-
defined nonenhancing intracranial masses that are
isointense to CSF
• Diagnostic confusion occasionally may arise between
arachnoid cysts and epidermoid tumors. Both masses
may have similar characteristics on T1-weighted
images. The use of proton-density and FLAIR
sequences and high-resolution T2-weighted images
421
allows differentiation between the two lesions in most
patients. The use of diffusion weighted images makes
differentiation between the two masses easier
(epidermoids are bright on diffusion weighted
images). As a result of the CSF content of arachnoid
cysts, signal intensity is low on DWI
• Epidermoid tumors are solid masses, and their T2-
weighted signal is higher than the surrounding CSF
• On FLAIR imaging arachnoid cyst demonstrate low
signal (fluid), whereas epidermoids may demonstrate
variable signal, slightly higher than CSF
CT cisternography may demonstrate the
communication between the subarachnoid space and
the cyst, which is important for surgical planning.
Cranial ultrasonography is an important
diagnostic tool during the first year of life, and
although symptomatic arachnoid cysts are
comparatively rare in infants, ultrasound provides a
noninvasive imaging technique with high yield in the
detection and characterization of cystic masses.
• MRI is the recommended diagnostic procedure of
choice because of its ability to demonstrate the exact
location, and extent.
BIBLIOGRAPHY
1. Dietemann JL, Guessoum M, Schultz A, Zollner G, Sanoussi
S, Maitrot D, Buchheit F. Intrasellar arachnoid cysts:
Computed tomography and MRI. Apropos of two cases.
J Neuroradiol 1997;24(2):168-73.
2. Gosalakkal JA. Intracranial arachnoid cysts in children: A
review of pathogenesis, clinical features, and management.
Pediatr Neurol 2002;26(2):93-8. Review.
3. Lesser RL, Geehr RB, Higgins DD, Greenberg AD. Ocular
motor paralysis and arachnoid cyst. Arch Ophthalmol
1980;98(11):1993-5.
4. McAvoy CE, Best R, Sharkey JA, Gray WJ. Symptomatic
arachnoid cyst presenting as a sixth nerve palsy. Eye 2001;
15(Pt 4):548-50.
5. Miyajima M, Arai H, Okuda O, Hishii M, Nakanishi H,
Sato K. Possible origin of suprasellar arachnoid cysts:
Neuroimaging and neurosurgical observations in nine
cases. J Neurosurg 2000;93(1):62-7.
6. Nomura M, Tachibana O, Hasegawa M, Kohda Y, Nakada
M, Yamashima T, Yamashit J, Suzuki M. Contrast-enhanced
MRI of intrasellar arachnoid cysts: Relationship between
the pituitary gland and cyst. Neuroradiology 1996;38(6):
566-8.
422 Section 5 Disorders of Chiasm

A B

Figs 34.12A and B: (A) Axial CT scan and (B) Coronal CT scan of the brain showing a lobulated CSF signal intensity lesion in the
suprasellar cistern displacing the basilar artery and middle cerebral artery. There is mild dilatation of the lateral ventricles (arrow)
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult
DERMOID INCLUSION CYSTS
• CNS dermoid cysts are considered a non-neoplastic
ectodermal inclusion cyst. Most are thought to
represent a congenital abnormality resulting from the
inclusion of ectodermal elements at the time of neural
tube closure or during the formation of secondary
cerebral vesicles
• Dermoids are mainly unilocular and expand slowly,
enlarging over years or decades, by the accumulation
(within an enclosed space) of cutaneous products
• Dermoid inclusion cysts have a thicker lining, which
may contain dystrophic calcifications
• Dermoid is a rare tumor which comprises less than
1 percent of primary intracranial tumors. It is much
less common (4-9 times) than epidermoid cysts. It
typically presents in patients under thirty years of age,
which is a slightly younger presentation than for
epidermoid cysts
• Clinically, the most common symptoms are seizures
and headaches.
RADIOLOGICAL FEATURES
CT Scans
• Dermoid appears as a well-defined round hypodense
mass
• It typically has an attenuation consistent with fat
(–20 to –40 HU)
423
• In case of rupture, scattered low density fat droplets
may be seen throughout the ventricles and
subarachnoid space
• A fat-fluid level may also be present. Calcifications,
particularly in the capsule are particularly common
• There is no enhancement after contrast adminis-
tration.
MR Imaging
• Typically, demonstrates high signal on T1 and
variable signal on T2. This is consistent with the lipid
and cholesterol which typically collects within the
dermoid cyst. Here may be noted as fine, low signal
structures within the cyst
• If the cyst ruptures, then high-signal droplets on T1
images may be seen scattered throughout the CSF.
Again, a fat/CSF fluid level may also be identified
• As with other fatty masses, chemical shift artifact may
also be present.
BIBLIOGRAPHY
1. Conley FK. Epidermoid and dermoid tumors: Clinical fea-
tures and surgical management. In: Wilkins RH (Ed).
Neurosurgery, 2nd edn. New York: McGraw-Hill 1996;971-6.
2. Wilms G, Casselman J, Demaerel P, et al. CT and MRI of
ruptured intracranial dermoids. Neuroradiology 1991;
33(2):149-51.
424 Section 5 Disorders of Chiasm

A B

Figs 34.13A to C: An operated case of suprasellar dermoid with

recurrence: (A) Sagittal T1-weighted image showing a large
T1-hyperintense lesion in the suprasellar region with posterior
extension into the interpeduncular fossa, (B) Sagittal T2-weighted
image showing T2-hypointense fat-fluid level (arrow) and (C) Axial
C diffusion weighted image showing no restricted diffusion
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult
EPIDERMOID CYSTS
• Epidermoid is a true ectodermal inclusion cysts, lined
by an epithelium
• The cause of epidermoids is same as dermoids which
is failure of surface ectoderm to separate from
underlying structures, sequestration of surface
ectoderm, and implantation of surface ectoderm
• Epidermoid inclusion cysts like dermoids are mainly
unilocular and expand slowly, enlarging over years
or decades, by the accumulation (within an enclosed
space) of cutaneous products
• Epidermoid inclusion cysts have a thin squamous
lining, which only rarely contains calcifications. The
debris consists of mostly keratin, a proteinaceous
material, and some cholesterol, a lipid material
derived from the breakdown of cell membranes.
Epidermoids are often described as pearly tumors
because of the shiny, smooth, waxy character of their
“dry keratin” at gross inspection
• Clinically, epidermoid also shows slight male
predilection, and most patients present in the first four
decades of life
• The most common sites include the cerebellopontine
angle cisterns and parasellar region.
IMAGING
Computed Tomography (CT)
• Epidermoid cysts usually have attenuation and signal
intensity values that roughly parallel those of water
or cerebrospinal fluid
• Epidermoid tumors often have attenuation similar to
CSF but may have hyperattenuation on nonenhanced
images because of high tumoral protein content,
hemorrhage, or cellular debris
• The wall of epidermoid tumors may sometimes
enhance after intravenous administration of contrast
medium
• They have a tendency to grow around available space
• A variant termed as ‘dense’ epidermoids are seen as
hyperdense masses due to high protein content or
saponification of cyst debris to calcium soaps
• Calcification may be seen in 10 percent of the
epidermoids.
425
MRI
• Epidermoid tumors are usually hypointense on T1-
weighted images and hyperintense on T2-weighted
images. These are better delineated on FLAIR images,
i.e. slightly more hyperintense with heterogeneous
signal compared with CSF
• Diffusion weighted images (DWI) are diagnostic as
they display hyperintense signal due a combination
of T2 and diffusion effects. The ADC (apparent
diffusion coefficient) is similar to that of gray matter
and lower than that of CSF. In contrast, arachnoid
cysts or other cystic intracranial lesions do not
show restricted diffusion and follow the CSF signal
on DWI and ADC maps. Therefore, DWI helps in
differentiating epidermoid from arachnoid cyst which
may have similar CT and T1- and T2-MRI appearances
• Differential diagnosis in a child of this age for an extra-
axial mass near the suprasellar region would typically
include:
– Dermoid cyst
– Epidermoids
– Teratoma
– Glioma
– Craniopharyngioma.
BIBLIOGRAPHY
1. Kallmes DF, Provenzale JM, Cloft HJ, McClendon RE.
Typical and atypical MR imaging features of intracranial
epidermoid tumors. Am J Roentgenol 1997;169(3): 883-7.
2. Aribandi M, Wilson NJ. CT and MR imaging features of
intracerebral epidermoid: A rare lesion. Br J Radiol 2008;
81(963):e97-9.
3. Robertson R, Caruso PA, Truwit CL, Barkovich AJ.
Disorders of brain development. In: Atlas SW (Ed).
Magnetic resonance imaging of the brain and spine, 3rd
edn. Philadelphia, PA: Lippincott, Williams and Wilkins,
2002;279-369.
4. Schaefer PW, Grant PE, Gonzalez RG. Diffusion-weighted
MR imaging of the brain. Radiology 2000;217:331-45.
5. Timmer FA, Sluzewski M, Treskes M, van Rooij WJ,
Teepen JL, Wijnalda D. Chemical analysis of an
epidermoid cyst with unusual CT and MR characteristics.
Am J Neuroradiol 1998;19:1111-2.
6. Tsuruda JS, Chew WM, Moseley ME, Norman D.
Diffusion-weighted MR imaging of the brain: Value of
differentiating between extra-axial cysts and epidermoid
tumors. Am J Neuroradiol 1990;11:925-31.
426 Section 5 Disorders of Chiasm

A B

Figs 34.14A and B: (A) Axial CT scan and (B) Coronal CT scan of the brain showing hypodense CSF density soft tissue mass
lesion in the suprasellar region with extension into the adjacent CSF spaces (arrows) suggestive of epidermoid cyst

A B C

Figs 34.15A to C: (A) Axial CT scan, (B) Coronal CT scan of the brain respectively are showing an irregular hypodense suprasellar
lesion insinuating into the adjacent CSF spaces. Note: The linear calcification seen at the inferior aspect on coronal images
(arrow) and (C) Axial diffusion-weighted image showing hyperintense signal—confirming the diagnosis of epidermoid
 Chapter 34 Chiasmal Disorders in Pediatric: Young Adult
HISTIOCYTOSIS X
(EOSINOPHILIC GRANULOMATOSIS)
• This is a group of disorders characterized by
proliferation of Langerhans cell, a histiocyte
(macrophages) normally found in the epidermis and
recognized ultrastructurally by tennis racket-shaped
granules known as Birbeck granules
• This term includes eosinophilic granuloma, Hand-
Schüller-Christian disease, and Letterer-Siwe disease.
All these entities have very similar histopathologic
features although clinical manifestations vary and
often overlap
• Previously known as histiocytosis X now the common
terms are eosinophilic histiocytosis or Langerhans cell
granulomatosis
• Unifocal and multifocal forms of the disease occur.
Unifocal form is a benign disease characterized by
solitary lytic bony lesion. The skull may be involved
but hypothalamic pituitary axis is spared
• Multifocal form is more aggressive and occurs in
childhood. Twenty-five percent of cases have classical
clinical triad of diabetes insipidus, exophthalmos and
lytic bone lesions (Hand-Schüller-Christian syn-
drome). Many patients may show only a partial
expression of this syndrome.
IMAGING
CT Scan Findings
Thickening and enhancement of pituitary gland and stalk
may be seen.
MRI Findings
• Thickening of pituitary stalk displaying hyperintense
signal on T2-weighted images. It usually enhances
well with contrast
• The granulomas may be found in the hypothalamus
or pituitary stalk
• Other cranial imaging findings reported are osseous
lesions and meningeal lesions. The pineal gland
enlargement (>10 mm) or cystic appearance, white
427
matter changes with leukoencephalopathy-like
pattern is also reported.
BIBLIOGRAPHY
1. Asano T, Goto Y, Kida S, Ohno K, Hirakawa K. Isolated
histiocytosis X of the pituitary stalk. J Neuroradiol
1999;26(4):277-80.
2. Broadbent V, Dunger DB, Yeomans E, Kendall B. Anterior
pituitary function and computed tomography/magnetic
resonance imaging in patients with Langerhans cell
histiocytosis and diabetes insipidus. Med Pediatr Oncol
1993;21(9):649-54.
3. Cagli S, Oktar N, Demirtas E. Langerhans' cell histiocytosis
of the temporal lobe and pons. Br J Neurosurg 2004;
18(2):174-80. Review.
4. Crespo-Rodriguez AM, Franco C, Lidon MC, Izquierdo
B, Angulo E, Mazas-Artasona LV. Hypothalamic-
infundibular histiocytosis: Magnetic resonance findings.
Rev Neurol 2004;39(2):125-9.
5. Grois N, Prayer D, Prosch H, Minkov M, Potschger U,
Gadner H. Course and clinical impact of magnetic
resonance imaging findings in diabetes insipidus
associated with Langerhans cell histiocytosis. Pediatr
Blood Cancer 2004;43(1):59-65.
6. Kaltsas GA, Powles TB, Evanson J, Plowman PN,
Drinkwater JE, Jenkins PJ, Monson JP, Besser GM,
Grossman AB. Hypothalamo-pituitary abnormalities in
adult patients with Langerhans cell histiocytosis: Clinical,
endocrinological, and radiological features and response
to treatment. J Clin Endocrinol Metab 2000;85(4):1370-6.
7. Maghnie M, Arico M, Villa A, Genovese E, Beluffi G, Severi
F. MR of the hypothalamic-pituitary axis in Langerhans
cell histiocytosis. Am J Neuroradiol 1992;13(5):
1365-71.
8. Prayer D, Grois N, Prosch H, Gadner H, Barkovich AJ.
MR imaging presentation of intracranial disease associated
with Langerhans cell histiocytosis. Am J Neuroradiol
2004;25(5):880-91.
9. Tien RD, Newton TH, McDermott MW, Dillon WP,
Kucharczyk J. Thickened pituitary stalk on MR images in
patients with diabetes insipidus and Langerhans cell
histiocytosis. Am J Neuroradiol 1990;11(4):703-8.
428 Section 5 Disorders of Chiasm

A B

Figs 34.16A to C: A 8-year-old female presented progressive

loss of vision and diabetes insipidus: (A) Sagittal T1-weighted
image, (B) Axial T2-weighted image and (C) Axial T1-weighted
image showing an ill-defined lesion displaying T1-isointense
and mixed signal in T2 in the suprasellar cistern involving the
C hypothalamo-pituitary axis and chiasm. HPE—histiocytosis
Chapter 35
Chiasmal Disorders in
Middle Age: Elderly
The lesions causing chiasmal disorders in middle age are different from those affecting children, hence they are
discussed separately. The common lesions affecting the middle age and elderly are:
• Pituitary adenoma
• Meningioma
• Rathke's cleft cyst
• Metastases
• Aneurysm.

PITUITARY ADENOMA
• Pituitary adenomas are histologically benign tumors
that arise from the adenohypophysis
• Pituitary adenomas are the most common cause of
chiasmal dysfunction in adults
• Depending on the tumor size, they are classified as
microadenoma (less than 10 mm) or macroadenoma
(more than 10 mm). In general, macroadenomas are
nonfunctional tumors, 50-60 percent of them may
show increased serum prolactin levels. Hence, these
tumors present with compressive symptoms, based
on their size and extent of compression of the
surrounding structures
• Most functioning pituitary adenomas are micro-
adenomas
• They affect all ages, but incidence increases with age,
peaking between the third and sixth decades.
SIGNS AND SYMPTOMS
• Nonocular symptoms include chronic headache,
fatigue, endocrine dysfunction like amenorrhea,
impotence, thyroid, adrenal and gonadal insufficiency
• These tumors are fairly large by the time, they present
to the ophthalmologist. Classical ophthalmic presen-
tation is painless gradual vision loss. Apart from this,
3, 4, 5, 6th cranial nerve palsies can occur, secondary
to compression
• Visual field defects can vary from classical bitemporal
hemianopia (denser superiorly) to central scotoma,
to homonymous hemianopia depending on the size
and direction of growth of the tumor. Asymmetry of
the field defects is the rule
• Microadenomas within the sella usually do not
produce any field defects.
IMAGING
CT Scan
• It is better in visualizing bony sella to look for
expansion or erosion and calcifications
• Macroadenomas are solid lesions usually iso- to
hyperdense with homogeneous contrast enhance-
ment. Necrosis, hemorrhage and calcification may be
seen. Some of the tumors can be purely cystic in which
case differentiating from Rathke’s cleft cyst and
craniopharyngiomas may be difficult, if they are
purely intrasellar
430 Section 5 Disorders of Chiasm

Fig. 35.1: Contrast-enhanced coronal CT scan of the brain

showing a large lobulated mass lesion expanding the bony sella
with suprasellar extension

A B

Figs 35.2A and B: (A) Axial CT scan and (B) Coronal CT scan of the brain showing a large well-circumscribed heterogeneous
lesion in the sella eroding the bony sella with parasellar extension
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly
• Differential diagnosis for suprasellar calcification—
germinomas, craniopharyngiomas, and meningiomas.
MRI Scan
• MRI of the brain and sellar region with multiplanar
thin sections is superior to CT in providing
information regarding chiasmal compression,
parasellar extension and hemorrhage within. Hence,
MRI brain remains the choice of imaging.
Pregadolinium and postgadolinium images are
recommended to delineate the tumor extension into
the cavernous sinus
• Pituitary adenomas are T1-isointense and have
variable T2-signal. They show moderate-to-marked
contrast enhancement with slight-to-moderate degree
of heterogeneity
• Diffusion-weighted imaging and ADC may be useful
in proving information regarding the consistency of
the tumor
• Sagittal section demonstrates the ballooning of the
sella and its relation to the chiasm
• Encasement of the cavernous ICA suggests involve-
ment of the cavernous sinus. If normal pituitary tissue
is seen between the lesion and cavernous sinus, it safely
excludes cavernous sinus involvement
• Hemorrhage is best seen on MRI
• 50 percent of the patients treated with bromocriptine
show T1-hyperintense signal probably attributed to
the presence of hemorrhage within.
MANAGEMENT
• Endocrine function tests (hormone assays) are
mandatory
• Management of the tumors depends upon their
secretory function. Prolactinomas can be treated with
bromocriptine therapy (medical adenomectomy)
• Non-secreting adenoma causing compressive features
need surgical excision. Trans-sphenoidal route is the
common mode of neurosurgical excision. Radiotherapy
431
is useful for tumors that cannot be resected fully or
that recur after surgery
• Patients should be followed up with regular neuro-
ophthalmic examination, endocrinologic and
neuroimaging.
BIBLIOGRAPHY
1. Alberto Pierallini, Francesca Caramia, Carlo Falcone,
Emanuele Tinelli, Amalia Paonessa, Alessia Bernardo
Ciddio, Marco Fiorelli, Federico Bianco, Stefania Natalizi,
Luigi Ferrante, Luigi Bozzao. Pituitary macroadenomas:
Preoperative evaluation of consistency with diffusion-
weighted MR imaging. Initial Experience Radiology
2006;239:223-31.
2. Allen D Elster. Modern imaging of the pituitary gland.
Radiol 1993;187:1-14.
3. Chamlin M, LM Davidoff, et al. Ophthalmologic changes
produced by pituitary tumors. Am J Ophthalmol 1995;40:
353-68.
4. Fatemi N, Dusick JR, de Paiva Neto MA, Kelly DF. The
endonasal microscopic approach for pituitary adenomas
and other parasellar tumors: A 10-year experience.
Neurosurg 2008;63(4 Suppl 2):244-56; discussion 256.
5. Jean-Philippe Cottier, Christophe Destrieux, Laurent
Brunereau, Philippe Bertrand, Laurence Moreau, Michel
Jan, Denis Herbreteau. Cavernous Sinus Invasion by
Pituitary Adenoma: MR Imaging. Radiol 2000;215:463-9.
6. Klauber A, Rasmussen P, Lindholm J. Pituitary adenoma
and visual function: The prognostic value of clinical,
ophthalmological and neuroradiologic findings in 51
patients subjected to operation. Acta Ophthalmol
(Copenh) 1978;56(2):252-6.
7. Lamberts SW, Hofland LJ. Future treatment strategies of
aggressive pituitary tumors. Pituitary 2009;12(3):261-4.
8. Levy A, Lightman SL. Diagnosis and management of
pituitary tumors. BMJ 1994;308:1087-91.
9. Marcus M, Vitale S, Calvert PC, Miller NR. Visual
parameters in patients with pituitary adenoma before and
after trans-sphenoidal surgery. Aust NZJ Ophthalmol
1991;19(2):111-8.
10. Vance ML. Pituitary adenoma: A clinician's perspective.
Endocr Pract 2008;14(6):757-63.
11. Sullivan LJ, O'Day J, McNeill P. Visual outcomes of
pituitary adenoma surgery: St Vincent's Hospital 1968-
1987. J Clin Neuro-ophthalmol 1991;11(4):262-7.
432 Section 5 Disorders of Chiasm

Figs 35.3A and B: (A) Contrast-enhanced axial CT scan

and (B) Coronal CT scan of the brain at the level of the sella
showing a large multilobulated sella lesion with fluid-fluid level
B within suggestive of hemorrhage (arrows)
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly 433

A B C

Figs 35.4A to C: (A) Postcontrast axial, (B) Coronal and (C) Sagittal image of the brain showing a well-circumscribed mass leson
in the sella with suprasellar and left parasellar extension encasing the left cavernous internal carotid artery (arrow). Note: The
chiasmal compression in Figure C (dotted arrow)

A B

Figs 35.5A and B: (A) Coronal CT scan of the sella showing a large well-circumscribed lobulated homogeneously enhancing
solid mass lesion in the sella with a larger suprasellar component causing obstructive hydrocephalus and (B) Sagittal T2-weighted
image of the same patient showing a sella mass and a larger suprasellar component
434 Section 5 Disorders of Chiasm

A B

Figs 35.6A and B: (A) Axial postcontrast T1-weighted image and (B) Coronal postcontrast T1-weighted image of the sella
showing a well-circumscribed homogeneously enhancing sella mass lesion with right parasellar and suprasellar extension

A B

Figs 35.7A and B: (A) Coronal T2-weighted image and (B) T1-weighted image showing mixed solid, cystic sellar mass lesion
with suprasellar extension. Note: Constriction at the diaphragm sella giving a Figure-of-8 appearance (small arrow) and chiasmal
compression (arrow)
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly
MENINGIOMA
• Meningioma is the most common primary tumor of
the central nervous system
• They arise from the arachnoid villi of the meninges
• They are mostly benign but can be locally aggressive
• Meningiomas can occur anywhere along the meninges
of the craniospinal axis
• Women are more often affected than men. The male-
to-female ratio ranges from 1:1.4 to 1:2.8
• Optic nerve sheath and suprasellar/frontal
meningiomas are most frequently seen in ophthal-
mology clinics as they give rise to visual symptoms.
Parasellar meningiomas present with cranial nerve
palsies usually III or VI nerves are involved. The
calvarial meningiomas present to an ophthalmologist
when large enough to produce raised ICT
• Multiple meningiomas are seen in patients with type
II neurofibromatosis
• Small meningiomas are incidentally detected during
cranial imaging.
Classification of meningiomas is based upon the WHO
classification system:
• Benign (Grade I): (90%)—meningothelial, fibrous,
transitional, psammomatous, angioblastic (most
aggressive)
• Atypical (Grade II): (7%)—choroid, clear cell, atypical
• Anaplastic/malignant (Grade III): (2%)—papillary,
rhabdoid, anaplastic.
COMMON SITES
• Parasagittal falx cerebri (50%), sphenoid wing (20%), floor
of the anterior cranial fossa (10%), parasellar region (10%),
tentorium, and cerebellopontine angle cistern region
• The common sella locations are suprasellar and
parasellar, usually arising from the planum sphenoidale,
the tuberculum sella, or from the diaphragm sellae.
Occasionally, they may have a large intrasellar
component. The cavernous sinus is commonly invaded.
Hyperostosis of the planum sphenoid/tuberculum sella
or anterior clinoid process is frequently seen depending
on the location of the tumor.
SIGNS AND SYMPTOMS
The clinical presentation of patients varies depending on
the location of the tumor.
IMAGING
• MRI with gadolinium is the imaging modality of
choice as these tumors are mostly isointense to the
435
gray matter and small lesions can be easily missed.
The exact extent of the tumor is better delineated on
postgadolinium scans
• Plain skull radiograph (not done in the present era)
or lateral topogram of CT may reveal hyperostosis,
increased calvarial vascular markings and intracranial
calcifications.
CT Findings
• Meningiomas are hyperdense and markedly enhance
with contrast. Contrast enhancement is homogeneous
• Calcification and bony hyperostosis is diagnostic of
the tumor
• Small suprasellar, paraclinoid and parasellar can be
easily missed on a plain CT
• Hyperpneumatization of the sphenoid sinus and
clinoid process may be seen in meningiomas around
the tuberculum sella
• It may be difficult to differentiate suprasellar
meningiomas from pituitary adenomas on CT scan if
there is large intrasellar extension.
MR Findings
• Contrast-enhanced MRI is the modality of choice
• They are usually homogeneous solid lesions
isointense to the gray matter but quite often can be
hyperintense on T2-weighted images
• Meningiomas enhance intensely and homogeneously
after injection of gadolinium gadopentetate
• MRI will help in identifying the pituitary gland
separately from the lesion in case of a suprasellar
meningioma
• Dural tail sign may be occasionally seen in parasellar
meningiomas. Gadolinium-enhanced scans should be
obtained to delineate the extent of the lesion and look
for additional lesions at other sites in the brain
• Cystic meningiomas have been reported.
BIBLIOGRAPHY
1. Cappabianca P, Cirillo S, Alfieri A, et al. Pituitary macro-
adenoma and diaphragma sellae meningioma: Differential
diagnosis on MRI. Neuroradiol 1999;41(1):22-6.
2. De Monte F, Al-Mefty O. Meningiomas. In: Kaye AH, Laws
ER (Eds). Brain Tumors: An Encyclopedic Approach.
Ediburgh, Scotland: Churchill Livingstone 1995.pp.675-704.
3. Haddad GF, Al-Mefty O. Meningiomas: an overview. In:
Wilkins RH, Rengachary SS (Eds). Neurosurgery, 2nd edn.
Vol 1. New York, NY: McGraw-Hill 1996;1:833-42.
4. Lusis E, Gutmann DH. Meningioma: An update. Curr
Opin Neurol 2004;17(6):687-92.
436 Section 5 Disorders of Chiasm

A B

Figs 35.8A and B: Coronal postcontrast T1-weighted images showing enhancing suprasellar and left
parasellar lobulated lesion with extension into the left cavernous sinus, En-plaque lesion is extending
along the temporal dura

A B

C D

Figs 35.9A to D: (A) Sagittal T1-weighted image, (B) Coronal T2-weighted image and (C and D) Coronal
T1-weighted image showing a well-circumscribed suprasellar lesion displaying isointense signal in T1 and
T2-weighted image with respect to gray matter. Note: The pituitary gland separate from the tumor (arrow).
Note: The thickening of the right intracranial optic nerve suggestive of extension (white arrow)
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly 437

Figs 35.10A and B: (A) Axial post-

contrast CT scan and (B) Coronal
postcontrast CT scan of the brain
showing a well-circumscribed
lobulated homogeneous modera-
tely enhancing suprasellar mass
lesion. Note: That the sella is not
A B expanded HPE—meningioma

Fig. 35.11: Axial postcontrast CT scan of the brain showing a

well-circumscribed lobulated suprasellar mass lesion showing
marked contrast enhancement. Note: Another enhancing left
tentorial lesion (arrow)—multiple meningioma
438 Section 5 Disorders of Chiasm

Figs 35.12A and B: (A) Axial CT scan

and (B) Coronal non-enhanced CT scan
of the brain showing a calcified lesion in
A B the suprasellar region

Fig. 35.13: Sagittal T1-weighted image showing a well-circums-

cribed, homogeneous suprasellar mass lesion seen separately
from the pituitary gland (arrow)—tuberculum sella meningioma
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly
EMPTY SELLA
Empty sella is defined as a radiological appearance of an
enlarged or deformed sella turcica that is partially or
completely filled with cerebrospinal fluid.
• This term is a misnomer, as the sella is not completely
empty, but the pituitary is always present both
anatomically and functionally
• It is usually an incidental finding and may
occasionally results in abnormal pituitary function
• It can occur at any age with equal sex distribution
• It is more common in women when associated with
idiopathic intracranial hypertension
• Empty sella may be classified as primary or secondary.
Primary empty sella is when there is no surgery or
radiation to the pituitary gland. Empty sella following
such procedure is classified as secondary empty sella
• Pathogenesis: An incomplete sellar diaphragm
predisposes to herniation of subarachnoid CSF into
the sella which may be due to increased intracranial
pressure or due to reduction in the size of the pituitary
gland.
CLINICAL FEATURES
• These patients are usually asymptomatic and are
detected incidentally on cranial imaging
• Visual abnormalities such as decreased visual acuity,
peripheral field constriction, bitemporal hemianopia,
or quadrantinopia may be noted
• Spontaneous CSF rhinorrhea and pseudotumor
cerebri are two syndromes that can be associated with
an empty sella.
IMAGING
X- ray
• The lateral radiograph of the skull may reveal a
normal-sized sella or enlarged sella
• The primary empty sella is frequently confused with
intrasellar neoplasm when lateral skull radiographs
reveal an enlarged sella. In primary empty sella, the
sella shows smooth symmetrical ballooning
439
maintaining the shape of the sella with no bony
erosion.
CT Scan
• On CT, the pituitary fossa is seen to be occupied
largely by CSF rather than a normal gland
• Mild expansion of the bony sella may be noted.
Magnetic Resonance Imaging (MRI)
Mid sagittal T1-weighted image shows the expanded sella
is filled with CSF and thin rim of pituitary gland is seen
flattened against the bony sella.
Recommended imaging—MRI brain.
BIBLIOGRAPHY
1. Beattie AM, Trope GE. Glaucomatous optic neuropathy
and field loss in primary empty sella syndrome. Can J
Ophthalmol 1991;26(7):377-82.
2. Bjerre P. The empty sella: A reappraisal of etiology and
pathogenesis. Acta Neurol Scand 1990;130(Suppl):1-25.
3. Bonneville JF, Dietmann JL. Intrasellar pathology. In:
Radiology of the Sella turcica. Springer Verlag, New York
1981.pp.89-126.
4. Brodsky MC, Vaphiades M. Magnetic resonance imaging
in pseudotumor cerebri. Ophthalmol 1998;105(9):1686-93.
5. Charteris DG, Cullen JF. Binasal field defects in primary
empty sella syndrome. J Neuro-ophthalmol 1996;16(2):
110-4.
6. Elster A. Modern imaging of pituitary. Radiol 1987; 110:
1-14.
7. Kanan CR. The Empty Sella Syndrome. In: The Pituitary
Gland. New York 1987.pp.513-25.
8. Kucharczyk W, Montanera WJ. The sella and parasellar
region. In: Atlas of Magnetic Resonance Imaging of the
Brain and Spine. New York, Raven Press 1996.pp.871-930.
9. McFadzean RM. The empty sella syndrome: A review of
14 cases. Trans Ophthalmol Soc UK 1983;103(Pt 5):537-42.
10. Pocecco M, De Campo O, Marinoni S, et al. High frequency
of empty sella syndrome in children with growth hormone
deficiency. Helv Paediatr Acta 1989;43:295-301.
440 Section 5 Disorders of Chiasm

A B

Figs 35.14A and B: (A) Coronal T2-weighted image and (B) Sagittal T1-weighted image at the level of the sella showing sella
being predominantly occupied by CSF and the pituitary gland compressed against the bony sella—primary empty sella

A B

Figs 35.15A and B: (A) Sagittal T2-weighted image and (B) Sagittal T1-weighted image showing postoperative changes in the
sella resulting in secondary empty sella. Note: The displacement of the chiasm into the sella
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly
METASTASIS
• The common malignancies to metastatize to the sella
include lung, breast, lymphoma, and leukemia
• Most metastases to the pituitary gland are small and
clinically silent. Larger lesions may present with
diabetes insipidus
• The most helpful distinguishing feature is rapid tumor
growth
• Metastatic disease in the pituitary gland accounts for
only 1 percent of all pituitary tumor resections, but
appears to occur more frequently with certain types
of cancer
• Pituitary gland may be involved by hematogenous
or CSF seeding to the dura around the sphenoid sinus
or there may be direct extension from head and neck
neoplasms
• Because of its relative rarity, there are comparatively
few reports offering discussions of diagnosis and
treatment modalities
• In older patients, patients with a history of a malignant
neoplasm, and patients with symptoms such as
diabetes insipidus and ophthalmoplegia, metastasis to
the sella and parasellar region should be strongly
considered.
IMAGING
MRI with gadolinium is the imaging modality of choice.
On imaging following features may be noted:
441
• Thickening of the pituitary stalk
• Enlargement of the pituitary gland
• Invasion of the cavernous sinus or parasellar mass
• Sclerotic changes around the sella turcica
• They may display isointense signal in T1-weighted
images and variable signal on T2-weighted images.
BIBLIOGRAPHY
1. Chaudhuri R, Twelves C, Cox TC, et al. MRI in diabetes
insipidus due to metastatic breast carcinoma. Clin Radiol
1992;46:184-8.
2. Mayr NA, Yuh WT, Muhonen MG, et al. Pituitary
metastases: MR findings. J Comput Assist Tomogr
199;17:432-7.
3. Kimmel DW, O'Neill BP. Systemic cancer presenting as
diabetes insipidus. Clinical and radiographic features of
11 patients with a review of metastatic-induced diabetes
insipidus. Cancer 1983;52:2355-8.
4. Kistler M, Pribram HW. Metastatic disease of the sella
turcica. AJR 1975;123:13-21.
5. Bronwyn E Hamilton, Karen L Salzman, Anne G Osborn.
Anatomic and Pathologic Spectrum of Pituitary
Infundibulum Lesions. AJR 2007;188:223-32.
6. John Komninos, Varvara Vlassopoulou, Despina
Protopapa, Stefanos Korfias, George Kontogeorgos,
Damianos E Sakas, Nicolas C. Thalassinos Tumors
Metastatic to the Pituitary Gland: Case Report and
Literature Review. The Journal of Clinical Endocrinology
and Metabolism 2004;89(2):574-80.
442 Section 5 Disorders of Chiasm

A B

C D
Figs 35.16A to D: A 50-year-old female presented with progressive loss of vision in both eyes and left 6th nerve palsy: (A) Sagittal
T1-weighted image, (B) Axial FLAIR, (C) Coronal T1-weighted image and (D) Coronal T2-weighted image showing a large soft
tissue lesion in the central skull-base lesion with bony destruction and extension into the sella and suprasellar region and left
cavernous sinus. Inferior extension into the nasopharynx noted. HPE—metastasis from thyroid carcinoma
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly 443

A B

C D

Figs 35.17A to D: (A) Coronal T2-weighted image and (B) T1-postcontrast fat-suppressed image showing a
large heterogeneous enhancing mass in the central skull-base causing destruction of the body of the sphenoid
with extension into the sella and suprasellar region. Extension is also seen in the right parasellar region and
infratemporal fossa. Note abnormal marrow signal in the right ramus of mandible (arrow) and (C and D) Coronal
STIR images at the level of the pelvis and thoracolumbar spine, respectively taken as a part of whole body
screening showing multiple metastatic deposits in the skeleton from an unknown primary
444 Section 5 Disorders of Chiasm
RATHKE’S CLEFT CYST
• Rathke’s cleft cysts are congenital, non-neoplastic
sellar and suprasellar cysts derived from remnants of
Rathke’s pouch characteristically lined by a single
layer of ciliated cuboidal or columnar epithelium with
goblet cells
• Rathke’s cleft cysts are cystic sellar and suprasellar
lesions
• Rathke cleft cysts often produce no symptoms and
are incidental findings on radiological examination.
Symptomatic cysts tend to be large and cause
symptoms from compression of the pituitary gland,
pituitary stalk, optic chiasm, or hypothalamus
• Headaches and visual field disturbances arise due to
the mass effect of these tumors. They may also present
with seizures
• The location of the cysts may be entirely suprasellar,
intrasellar or intrasellar with suprasellar extension.
RADIOLOGICAL FINDINGS
CT Scan
• They may be isodense, hyperdense or of mixed
intensity to the gray matter
• Peripheral rim contrast enhancement may be noted
• Calcification may rarely be seen.
MRI Scan
• It is the imaging modality of choice. It will also show
the exact location of cyst with respect to the sella
• They are CSF isointense lesions on T1 and T2-weighted
images or may be T1 and T2-hyperintense (mucoid
type) and are seen predominantly within the sella
• Rim contrast enhancement is occasionally seen
• Calcification is rare.
Differential Diagnosis
• Craniopharyngioma
• Cystic pituitary adenoma.
BIBLIOGRAPHY
1. Billeci D, Marton E, Tripodi M, Orvieto E, Longatti P.
Symptomatic Rathke's cleft cysts: A radiological, surgical
and pathological review. Pituitary 2004;7(3):131-7.
2. Binning MJ, Gottfried ON, Osborn AG, Couldwell WT.
Rathke's cleft cyst intracystic nodule: A characteristic
magnetic resonance imaging findings. J Neurosurg
2005;103(5):837-40.
3. Brassier G, Morandi X, Tayiar E, Riffaud L, Chabert E,
Heresbach N, Poirier JY, Carsin-Nicol B. Rathke's cleft
cysts: Surgical-MRI correlation in 16 symptomatic cases.
J Neuroradiol 1999;26(3):162-71.
4. Byun WM, Kim OL, Kim D. MR imaging findings of
Rathke's cleft cysts: Significance of intracystic nodules.
Am J Neuroradiol 2000;21(3):485-8.
5. Mnif N, Hamrouni A, Iffenecker C, Oueslati S, Fruexer F,
Doyon D, Hamza R. MRI in the diagnosis of Rathke's cleft
cyst. J Radiol 2003;84(6):699-704.
6. Mukherjee JJ, Islam N, Kaltsas G, Lowe DG, Charlesworth
M, Afshar F, Trainer PJ, Monson JP, Besser GM, Grossman
AB. Clinical, radiological and pathological features of
patients with Rathke's cleft cysts: Tumors that may recur.
J Clin Endocrinol Metab 1997;82(7):2357-62.
7. Pisaneschi M, Kapoor G. Imaging the sella and parasellar
region. Neuroimaging Clin N Am 2005;15(1):203-19.
Review.
8. Sumida M, Uozumi T, Mukada K, Arita K, Kurisu K,
Eguchi K. Rathke's cleft cysts: Correlation of enhanced MR
and surgical findings. Am J Neuroradiol 1994;15(3):
525-32.
9. Tominaga JY, Higano S, Takahashi S. Characteristics of
Rathke's cleft cyst in MR imaging. Magn Reson Med Sci
2003;2(1):1-8.
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly 445

A B

C D

Figs 35.18A to D: (A) Axial FLAIR, (B) Coronal T2-weighted image, (C) Coronal T1-weighted image and (D) Sagittal T1-weighted
image showing a well-defined rounded cystic lesion in the suprasellar region almost isointense signal to CSF in all sequences.
The pituitary gland is seen separately from the cyst (arrows). HPE—Rathke’s cleft cyst
446 Section 5 Disorders of Chiasm
PITUITARY APOPLEXY
Apoplexy is defined as a sudden neurologic impairment,
usually due to a vascular process. Pituitary apoplexy is a
clinical syndrome characterized by sudden onset of
headache, visual symptoms, altered mental status, and
hormonal dysfunction due to acute hemorrhage or
infarction of a pituitary gland.
This condition results from an acute, rapid expansion
of a pituitary adenoma or, less commonly, a non-
adenomatous gland into the suprasellar space and
cavernous sinuses from infarction or hemorrhage.
The unusual vascular supply likely contributes to
frequency of pituitary apoplexy.
CLINICAL SIGNS AND SYMPTOMS
• Sudden and marked headache (95% of cases)
• Nausea and vomiting (69%)
• Visual acuity defects (52%) and visual field defects
(64%) that result from upward expansion of the tumor,
which compresses the optic chiasm, optic tracts, or
optic nerve
• Diplopia, ptosis and ocular motility dysfunction (64%)
from involvement of the cranial nerves traversing the
cavernous sinus. The 3rd nerve palsy is more common
that 4th and 6th nerve palsies. Horner’s syndrome
may also occur
• Alteration of consciousness due to diencephalic
compression
• Endocrine abnormalities
• Facial pain or numbness
• Leakage of blood and necrotic tissue into the
subarachnoid space may lead to meningismus, stupor
and coma.
CAUSES
• Two percent of pituitary adenoma present in this way
• Sudden trauma
• Anticoagulation
• Alteration of pressure gradients
• Cardiac surgery
• Diabetic ketoacidosis
• Following bromocriptine
• Radiotherapy
• Postpartum hemorrhage (Sheehan’s syndrome).
IMAGING
• MRI is the most sensitive imaging study for evaluating
the pituitary gland
• CT scan may show a non-enhancing hyperdense sellar
mass
• MRI usually demonstrates a pituitary macroadenoma
with heterogeneous signal characteristics due to
presence of blood. In the first 3-5 days, hemorrhage
within the sella is isointense or hypointense on T1-
weighted images and hypointense on T2-weighted
sequences. Subacute blood, i.e. methemoglobin is
hyperintense in T1- and T2-weighted images
• Hemorrhage may rarely extend into subarachnoid
space and ventricles.
Lumbar puncture demonstrates aseptic meningitis
accompanied by red blood cells.
BIBLIOGRAPHY
1. Ahmad FU, Pandey P, Mahapatra AK. Postoperative
'pituitary apoplexy' in giant pituitary adenomas: A series
of Cases. Neurol India 2005;53(3):326-8.
2. Armstrong MR, Douek M, Schellinger D, Patronas NJ.
Regression of pituitary macroadenoma after pituitary
apoplexy: CT and MR studies. J Comput Assist Tomogr
1991;15(5):832-4.
3. Kyle CA, Laster RA, Burton EM, Sanford RA. Subacute
pituitary apoplexy: MR and CT appearance. J Comput
Assist Tomogr 1990;14(1):40-4.
4. L'Huillier F, Combes C, Martin N, Leclerc X, Pruvo JP,
Gaston A. MRI in the diagnosis of so-called pituitary
apoplexy: Seven cases. J Neuroradiol 1989;16(3):221-37.
Review.
5. Lacomis D, Johnson LN, Mamourian AC. Magnetic
resonance imaging in pituitary apoplexy. Arch
Ophthalmol 1988;106(2):207-9.
6. Reid RL, Quigley ME, Yen SS. Pituitary apoplexy: A
review. Arch Neurol 1985;42(7):712-9.
7. Rogg JM, Tung GA, Anderson G, Cortez S. Pituitary
apoplexy: Early detection with diffusion-weighted MR
imaging. Am J Neuroradiol 2002;23(7):1240-5.
8. Sibal L, Ball SG, Connolly V, James RA, Kane P, Kelly
WF, Kendall-Taylor P, Mathias D, Perros P, Quinton R,
Vaidya B. Pituitary apoplexy: A review of clinical
presentation, management and outcome in 45 cases.
Pituitary 2004;7(3):157-63.
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly 447

A B

Figs 35.19A and B: (A) Coronal T2-weighted image and (B) Sagittal T1-weighted image showing a large lobulated lesion in the
sella with suprasellar extension compressing the chiasm. The lesion is T1-hyperintense and T2-hypointense with areas of low
signal seen within suggestive early subacute hemorrhage. HPE—pituitary apoplexy in a nonadenomatous gland

A B

Figs 35.20A and B: A 40-year-old female presented with sudden onset total ophthalmoplegia in the left eye with headache:
(A) Axial T1-weighted image and (B) Axial T2-weighted image at the sella showing T1-isointense and T2 mixed hypo- and
hyperintense soft tissue in the left cavernous sinus—pituitary apoplexy with cavernous sinus extension
448 Section 5 Disorders of Chiasm

A B

C D

Figs 35.21A to D: Follow-up scan 15 days later: (A and B) Noncontrast T1-weighted images and (C and D) At the level of the sella
showing T1 and T2-hyperintense lesion in the left half of the pituitary displacing the infundibulum to right and extension to the left
cavernous sinus suggestive of subacute blood. Note: A T1-hypointense lesion in the rest of the pituitary suggestive of an adenoma.
Hemorrhage in a pituitary microadenoma—pituitary apoplexy with cavernous sinus
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly
ANEURYSM
INTRODUCTION
The close proximity of the anterior circle of Willis to the
chiasm renders, it vulnerable to compression from
aneurysms arising from the internal carotid and anterior
cerebral arteries. Eighty-five percent of the cerebral
aneurysms arise from the anterior circle of Willis. The
common sites in the anterior circulation are bifurcations
of the internal carotid artery, anterior cerebral artery and
anterior communicating arteries.
CAUSES
• They can occur at any age, but are mostly seen in
adults
• Hypertension, atherosclerosis and trauma are the
main causes of aneurysms anywhere in the body
• Infections give rise to mycotic aneurysms.
Signs and Symptoms
• Headache is the most common and may be the only
presenting feature
• Ruptured aneurysm is a neurological emergency.
These patients usually present with acute onset severe
headache and raised ICT depending on the severity
of the intracranial bleed
• Aneurysms close to the visual pathway that are large
enough will cause compressive symptoms.
IMAGING
• All patients with vascular headaches should have an
MRI with angiogram done
• The choice of CT angiogram versus MR angiogram
for intracranial aneurysms is debatable
• The newer high field strength MR equipment with
refined post processing techniques offer excellent high
resolution MR angiograms and help in identifying
small aneurysms ( 2 mm and above)
• The reported sensitivity of CT angiography lies in the
range of 80-97% depending on the size and location
of an aneurysm
• The advantage of CT angiogram over DSA is its ability
to do 3D-reconstruction which may help in treatment
planning and can avoid a preprocedure DSA in case
of planned for coiling. They can be obtained within
minutes
• Disadvantages of CT angiogram:
– Small perforating arteries ( less than 0.5 mm) may
not be visible
– Difficult to differentiate infundibular dilatation
from aneurysm
449
– Simultaneous visualization of arterial and venous
phases can misinterpret the vein close to the artery
as an aneurysm (especially in the 6 detector and
below CT scanner). Careful examination of source
data will help
– Little value in postoperative evaluation after
clipping due to metal artifacts
– CT angiogram may be difficult in patients with
altered sensorium following SAH.
Advantages of MR Angiogram
• Bone does not interfere with the vascular imaging,
especially in the skull-base and cavernous sinus
• Aneurysms of 3 mm and above the sensitivity of CT
and MR angiogram are comparable, but relatively less
sensitive for smaller aneurysms
• MR angiograms can be performed in postoperative
patients with platinum coils as against CT angiogram,
which will give metal artifacts.
BIBLIOGRAPHY
1. Alberico RA, Patel M, Casey S, Jacobs B, Maguire W,
Decker R. Evaluation of the circle of Willis with three-
dimensional CT angiography in patients with suspected
intracranial aneurysms. Am J Neuroradiol 1995;16:1571-
78; discussion 1579-80.
2. Bernd F Tomandl, Niels C Köstner, Miriam
Schempershofe, Walter J Huk, Christian Strauss, Lars
Anker, Peter Hastreiter. CT Angiography of Intracranial
Aneurysms: A Focus on Postprocessing. Radiographics
2004;24:637-55.
3. Cloft HJ, Joseph GJ, Dion JE. Risk of cerebral angiography
in patients with subarachnoid hemorrhage, cerebral
aneurysm, and arteriovenous malformation: A meta-
analysis. Stroke 1999;30:317-20.
4. Katz DA, Marks MP, Napel SA, Bracci PM, Roberts SL.
Circle of Willis: Evaluation with spiral CT angiography,
MR angiography, and conventional angiography.
Radiology 1995;195:445-9.
5. Mallouhi A, Felber S, Chemelli A, et al. Detection and
characterization of intracranial aneurysms with MR
angiography: Comparison of volume-rendering and
maximum-intensity projection algorithms. Am J Roentgenol
2003;180:55-64.
6. Tomandl B, Hastreiter P, Iserhardt-Bauer S, et al.
Standardized evaluation of CT angiography with remote
generation of 3D videosequences for the detection of
intracranial aneurysms. Radiographics 2003;23:e12.
7. Tsuchiya K, Katase S, Yoshino A, Hachiya J, Yodo K.
Preliminary evaluation of volume-rendered three-
dimensional display of time-of-flight MR angiography in
the diagnosis of intracranial aneurysms. Neuroradiology
2001;43:633-6.
450 Section 5 Disorders of Chiasm

A B

Figs 35.22A to C: A 40-year-old female presented with gradual visual loss in the left eye and field defects: (A) Sagittal T2-weighted
image, (B) Coronal T2-weighted image and (C) Coronal MIP image of a 3D time of flight angiogram showing a large sacular
aneurysm arising from the left supraclinoid internal carotid artery causing compression of the left intracranial optic nerve and
anterior chiasm
 Chapter 35 Chiasmal Disorders in Middle Age: Elderly 451

A B

Figs 35.23A and B: A 52-year-old female presented with bilateral optic atrophy: (A) Axial noncontrast CT scan and (B) Non-
contrast coronal CT scan showing a well-circumscribed hyperdense suprasellar mass and left frontal gliosis—differential diagnosis
included aneurysm and MRI was done

A B

Figs 35.24A and B: MRI of the above patient: (A) Mid-sagittal T1-weighted image and (B) Mid-sagittal T2-weighted image
showing a small ACOM aneurysm (arrow) with perianeurysmal bleed and daughter sac formation compressing the chiasm
Chapter 36
Chiasmal Disorders:
No Age Predilection
The lesions that are common to any age group will be discussed in this chapter. These lesions include tumors such
as lymphoma, leukemia and chordomas and infective lesion such as in tuberculous meningitis.

MENINGITIS
• Meningitis indicates inflammation of the meninges. the cortical or meningeal blood vessels, producing
Clinically, patients present with headache, nuchal inflammation, obstruction, or infarction.
rigidity, photophobia and lumbar puncture reveals
increased number of white blood cells in the cerebro- Signs and Symptoms
spinal fluid (CSF—pleocytosis)
• Basal meningitis causes dysfunction of cranial nerves
• It can be divided as acute/chronic-based on the onset,
III, VI, and VII and also obstructive hydrocephalus
infectious/noninfectious based on the etiology, infec-
due to obstruction of basilar cisterns
tious meningitis is further classified into bacterial/
• Papilledema is the most common visual manifestation
viral/fungal
of TBM. In children, papilledema may progress to
• The incidence of meningitis is presumed to be higher
primary optic atrophy and blindness due to direct
in developing countries
involvement of the optic nerves and chiasma by basal
• The classic presentation of meningitis includes
exudates (i.e. opticochiasmatic arachnoiditis)
fever, headache, neck stiffness, photophobia, nausea,
• In adults, papilledema may progress more commonly
vomiting, and signs of cerebral dysfunction (e.g.
to secondary optic atrophy
lethargy, confusion, coma)
• VI cranial nerve is affected most frequently by TBM,
• Though there are many causes of meningitis, we are
followed by II, III, IV, VII and, less commonly CN,
discussing tuberculous meningitis in this chapter as
VIII, X, XI, and XII.
it is the most common entity encountered in our
country.
Imaging
TUBERCULOUS MENINGITIS Computed Tomography (CT)
Tuberculous meningitis (TBM) is the most common
• Contrast-enhanced CT should be done in supsected
chronic CNS infection in developing countries.
tuberculous meningitis
• It shows leptomeningeal and basal cistern enhance-
Pathogenesis
ments suggestive of exudates
TB bacilli seed to the meninges or brain parenchyma, • Linear periventricular enhancement is present in
tubercles rupturing into the subarachnoid space ependymitis
cause meningitis. A thick gelatinous exudate infiltrates • Obstructive hydrocephalus may eventually develop
 Chapter 36 Chiasmal Disorders: No Age Predilection 453

A B

Figs 36.1A and B: Axial contrast-enhanced CT scan of the brain showing enhancing exudates in the basal cisterns completely
encasing the chiasm (white arrow) with dilatation of the third and lateral ventricles. Infarcts in the left thalamus and internal
capsule (black arrows) are also noted

A B C

Figs 36.2A to C: (A) Axial postcontrast T-weighted image, (B) Coronal FLAIR and (C) Sagittal postcontrast T1-weighted image
showing enhancing exudates in the suprasellar cistern (white arrows) with edema noted in the surrounding medial temporal lobes
and thalamus (black arrows)
454 Section 5 Disorders of Chiasm
• Low-attenuating focal infarcts are seen in the deep
gray matter nuclei, deep white matter, and pons; these
infarcts result from associated vasculitis
• The primary differential diagnoses are fungal
meningitis, bacterial meningitis, carcinomatous
meningitis, and neurosarcoidosis
• The characteristic CT finding of a tuberculoma is a
nodular-enhancing or ring-enhancing lesion with a
non-enhancing hypodense center. Contrast-enhanced
study is essential. Early stages are characterized by
low-density or isodense lesions, often with edema out
of proportion to the mass effect and little
encapsulation. At a later stage, well-encapsulated
tuberculomas appear as isodense or hyperdense
lesions with peripheral ring enhancement
• Parenchymal cerebritis may cause hypoattenuation
with little or no enhancement
• Parenchymal tuberculomas demonstrate various
patterns. Noncaseating granulomas are homo-
geneously enhancing lesions. Caseating granulomas
are rim enhancing; if these have a central calcific focus,
they may form a target-like lesion. Granulomas may
also form a miliary pattern with multiple tiny nodules
scattered throughout the brain. All lesions are
surrounded by vasogenic edema
• The differential diagnosis includes fungal infections,
bacterial infections, neurocysticercosis, and cerebral
metastases.
Magnetic Resonance Imaging
• MRI is more sensitive than CT in determining the
extent of meningeal and parenchymal involvement
• In TBM, gadolinium-enhanced T1-weighted images
demonstrate prominent leptomeningeal and basal
cistern enhancement
• Ependymitis, linear periventricular enhancement is
present
• Hydrocephalus is usually seen
• Infarctions resulting from vasculitis are hyperintense
on T2-weighted images. Diffusion-weighted MRI is
especially sensitive in detecting early ischemic lesions
when findings on the T2-weighted MRIs are normal
• Parenchymal cerebritis may show hyperintensity on
T2-weighted images with little or no enhancement
• Parenchymal tuberculomas demonstrate various
patterns. They are typically hypointense on T2-
weighted images, but they may be hyperintense as
well. The T2-hypointense signal has been attributed
to the lipid content of the tuberculoma. Tuberculomas,
like bacterial cerebral abscesses, have hypointense
walls or rims on T2-weighted MRIs. The cause is
unknown, but free oxygen radicals released by the
inflammatory process are believed to decrease T2-
values. Non-caseating granulomas are homogene-
ously enhancing lesions. Caseating granulomas are
rim enhancing. Granulomas may also form a miliary
pattern with multiple, tiny, enhancing nodules
scattered throughout the brain. Lesions are typically
surrounded by hyperintense edema on T2-weighted
images.
BIBLIOGRAPHY
1. Bhargava S, Gupta AK, Tandon PN. Tuberculous
meningitis: A CT study. Br J Radiol 1982;55(651):189-96.
2. Green PH. Tubercular meningitis. Lancet 1836;2:232-5.
3. Kingsley DP, Hendrickse WA, Kendall BE, et al.
Tuberculous meningitis: Role of CT in management and
prognosis. J Neurol Neurosurg Psychiatry 1987;50(1):30-6.
4. Shah GV. Central nervous system tuberculosis: Imaging
manifestations. Neuroimaging Clin N Am 2000;10(2):
355-74.
5. Stevens DL, Everett ED. Sequential computerized axial
tomography in tuberculous meningitis. JAMA 1978;
239(7):642.
 Chapter 36 Chiasmal Disorders: No Age Predilection 455

A B

C D

Figs 36.3A to D: (A) Coronal T2-weighted image, (B) Coronal T1-weighted image, (C) Axial T1-weighted image and (D) Axial
postcontrast T1-weighted image showing a rounded lesion hypointense in T1 and T2-weighted image with isointense rim. Ring-
like peripheral enhancement is noted. Patient responded to antituberculous treatment and follow-up scan showed resolution of
the lesion
456 Section 5 Disorders of Chiasm
HYPOTHALAMIC HAMARTOMA
• Hypothalamic hamartomas are congenital malfor-
mations consisting of tumor-like collections of normal
brain tissue lodged in an abnormal location
• Hypothalamic hamartomas represent neoplastic
proliferation of well-organized brain tissue similar to
heteropias. Classical location and magnetic resonance
imaging (MRI) features strongly favor the diagnosis
• Hamartomas are not true neoplasms. Pathologically,
they contain the nerve cells that resemble those of the
tuber cinereum along with normal glial cells
• Most of these lesions occur in hypothalamus. Other
reported locations may be the subcortical cerebral
cortex and periventricular region
• Hypothalamic hamartomas are small pedunculated
growths contiguous with posterior hypothalamus,
between the tuber cinereum and mamillary bodies.
They fill the free space between the optic chiasm and
pons and usually do not distort the hypothalamus or
other parts of the base of the brain unless they are
very large
• They occur with equal frequency in males and
females
• Most of patients usually present in the first or second
decade of life
• These patients typically present with precocious
puberty. The larger hamartomas are less likely to
produce precocious puberty. Occasionally, visual
disturbances may be present because of involvement
of optic pathways. Other presenting symptoms may
also include seizures.
IMAGING
• CT: The typical feature is a well-circumscribed round-
shaped isodense soft tissue mass without contrast
enhancement
• MRI: Hypothalamic hamartomas produce chara-
cteristic soft tissue masses isointense to gray matter.
They are homogeneous and sharply marginated by
the surrounding CSF. They do not show enhancement
on postcontrast images
• Calcification is rare and hemorrhage is not described
in these lesions. The anatomic location of these
hamartomas together with signal intensity similar to
gray matter on T1-weighted image and a higher
intensity than gray matter on PDWI and T2-weighted
image strongly supports this diagnosis.
DIFFERENTIAL DIAGNOSIS
• Craniopharyngioma: Calcification is common in
craniopharyngiomas
• Hypothalamic chiasmatic gliomas: Inhomogeneous and
often show enhancement
• Gangliogliomas: Contrast-enhance
• Follow-up: MRI is recommended every 6 to 12 months
for demonstrating lack of growth.
BIBLIOGRAPHY
1. Beningfield SJ, Bonnici F, Cremin BJ. Magnetic resonance
imaging of hypothalamic hamartomas. Br J Radiol
1988;61(732):1177-80.
2. Boyko OB, Curnes JT, Oakes WJ, Burger PC. Hamartomas
of the tuber cinereum: CT, MR and pathologic findings.
Am J Neuroradiol 1991;12(2):309-14.
3. Burton EM, Ball WS Jr, Crone K, Dolan LM. Hamartoma
of the tuber cinereum: A comparison of MR and CT findings
in four cases. Am J Neuroradiol 1989;10(3):497-501.
4. Hahn FJ, Leibrock LG, Huseman CA, Makos MM. The
MR appearance of hypothalamic hamartoma.
Neuroradiology 1988;30(1):65-8.
5. Hubbard AM, Egelhoff JC. MRI imaging of large
hypothalamic hamartomas of two infants. Am J
Neuroradiol 1989;10:1277-9.
6. Lona Soto A, Takahashi M, Yamashita Y, Sakamoto Y,
Shinzato J, Yoshizumi K. MRI findings of hypothalamic
hamartoma: Report of five cases and review of the
literature. Comput Med Imaging Graph 1991;15(6):415-21.
Review.
7. Marliani AF, Tampieri D, Melancon D, Ethier R, Berkovic
SF, Andermann F. Magnetic resonance imaging of
hypothalamic hamartomas causing gelastic epilepsy. Can
Assoc Radiol J 1991;42(5):335-9.
8. Mori K, Handa H, Takeuchi J, Hanakita J, Nakano Y.
Hypothalamic hamartoma. J Comput Assist Tomogr
1981;5(4):519-21.
9. Nakagawa N, Takahashi M, Kohrogi Y, Kodama T,
Matsukado Y. Neuroradiologic findings of hypothalamic
hamartoma with emphasis on computed tomography.
J Comput Tomogr 1986;10(1):77-83.
 Chapter 36 Chiasmal Disorders: No Age Predilection 457

A B

C D

Figs 36.4A to D: A 20-year-old male presented with precocious puberty: (A) Sagittal T1-weighted image SE, (B) Axial T1-
weighted image SE, (C) Coronal T1-weighted image and (D) Coronal T2-weighted image showing a well-circumscribed lesion in
the region of the hypothalamus. It displays hypointense signal in T1 and hyperintense signal in T2-weighted images—hamartoma
of the tuber cinerium
458 Section 5 Disorders of Chiasm
SELLAR AND CHIASMAL LYMPHOMA
• Lymphomatous infiltration of the anterior visual
pathway can occur in Non-Hodgkin’s (NHL) and
Hodgkin’s lymphoma
• CNS involvement in NHL occurs in 10 percent of
cases. While infiltration of optic nerve does occur more
commonly with NHL (5%) than Hodgkin’s
lymphoma, infiltration of chiasm has also been
reported. The infiltration occurs from spread of the
CNS tumor, rarely from adjacent sinuses
• A sellar mass noted in any immunocompromised
individual should lead to suspicion of primary B-cell
lymphoma although this may occur in immuno-
competent individuals as well
• Lymphoma intrinsic to chiasm can occur and presents
with visual loss and endocrine abnormalities.
IMAGING
The appearance of chiasmal infiltration by lymphoma is
nonspecific.
CT Scan
The infiltrated chiasm is enlarged and appears
hyperdense and enhances with contrast.
MRI
• It will appear isointense, hyper- or hypointense on
T1-weighted image and hyperintense on T2-weighted
image
• Homogeneous significant contrast enhancement is
usually seen. Post-therapy, the signal and enhacement
pattern might change. It can show necrosis and
hemorrhage within the lesion.
BIBLIOGRAPHY
1. Lee AG, Tang RA, Roberts D, Schiffman JS, Osborne A.
Primary central nervous system lymphoma involving the
optic chiasm in AIDS. J Neuro-ophthalmol 2001;21(2):95-8.
2. Maiuri F. Primary cerebral lymphoma presenting as
steroid-responsive chiasmal syndrome. Br J Neurosurg
1987;1(4):499-502.
3. Miller NR, Iliff WJ. Visual loss as the initial symptom in
Hodgkin disease. Arch Ophthalmol 1975;93(11):1158-61.
4. Zaman AG, Graham EM, Sanders MD. Anterior visual
system involvement in non-Hodgkin’s lymphoma. Br J
Ophthalmol 1993;77(3):184-7.
 Chapter 36 Chiasmal Disorders: No Age Predilection 459

A B

Figs 36.5A to C: (A and B) Axial postcontrast CT scan of the brain showing moderately enhancing ill-defined suprasellar lesion
with perilesional edema involving the midbrain and thalamus and (C) Sagittal postcontrast T1-weighted image of the brain showing
moderately enhancing suprasellar mass lesion compressing the posterior half of the chiasm—lymphoma
460 Section 5 Disorders of Chiasm
CHORDOMA
• Chordomas are benign tumors arising from the
remnants of the primitive notochord. They are very
rare intracranial tumors (0.5% of primary intracranial
tumors) with a predilection for the clivus
• Though they are benign tumors, because of their
propensity for local invasion and recurrence, their
prognosis is similar to malignant tumors
• They can occur at any age, but are rare below 30 years
of age and have a male predilection
• Presenting features include headache, visual loss,
ataxia, 6th (most common nerve involvement), 7th,
9th, 10th and 11th cranial nerve palsies, dysphagia,
endocrine disturbances like hypogonadism, hyper-
prolactinemia, diabetes insipidus
• Histologically fluid, gelatinous substances, necrotic
areas, calcification and sequestered bone fragments
may be noted which accounts for the variety of signals
produced on MRI
• Chondroid chordomas have hyaline cartilaginous
tissue and due to their low water content produce
shorter T1 and T2 relaxation times on MRI.
RADIOLOGICAL FEATURES
• CT and MRI are complimentary in the diagnosis. While
MRI is inferior to CT in showing calcification and bone
destruction, MRI excels in soft tissue delineation
• On CT scans, chordomas at any site appear as single
or multiple areas of decreased attenuation. If the
chordoma has a significant chondroid component,
focal regions of hyperdensity may be present. The
lesions are expansile and cause destruction of the bone
• On MRI, chordomas are hyperintense on T2 images
and hypointense on T1 images
• Typical features include:
1. Primarily midline location
2. Predilection for the clivus
3. Significant intraosseous component with marked
bone destruction
4. Calcification may be seen on CT
5. Differentiation from a chondrosarcoma cannot be
confidently made on radiological examination.
Plain X-rays are no longer preferred for evaluation
of chordomas. Chordomas show bone expansion, trabe-
culation, rarefaction and calcification.
BIBLIOGRAPHY
1. Doucet V, Peretti-Viton P, Figarella-Branger D, Manera
L, Salamon G. MRI of intracranial chordomas. Extent of
tumour and contrast enhancement: Criteria for differential
diagnosis. Neuroradiology 1997;39(8):571-6.
2. Gehanne C, Delpierre I, Damry N, Devroede B, Brihaye
P, Christophe C. Skull-base chordoma: CT and MRI
features. JBR-BTR 2005;88(6):325-7.
3. Larson TC 3rd, Houser OW, Laws ER Jr. Imaging of cranial
chordomas. Mayo Clin Proc 1987;62(10):886-93.
4. Oot RF, Melville GE, New PF, Austin-Seymour M,
Munzenrider J, Pile-Spellman J, Spagnoli M, Shoukimas
GM, Momose KJ, Carroll R, et al. The role of MR and CT
in evaluating clival chordomas and chondrosarcomas.
Am J Roentgenol 1988;151(3):567-75.
5. Weber AL, Liebsch NJ, Sanchez R, Sweriduk ST Jr.
Chordomas of the skull-base: Radiologic and clinical
evaluation. Neuroimaging Clin N Am. 1994;4(3):515-27.
Review.
Chapter 36 Chiasmal Disorders: No Age Predilection 461

Fig. 36.6: Coronal CT brain showing a large destructive central

skull-base lesion with suprasellar extension with hyperdense
specs seen within suggestive of destroyed bone fragments.
HPE—chordoma (arrow)
462 Section 5 Disorders of Chiasm
PITUITARY ABSCESS
• This is a rare and serious entity. The infection is
usually blood borne or there can be a contiguous
spread from an infected sphenoid or cavernous sinus
• Usually such abscesses occur in the presence of other
sellar masses such as pituitary adenoma, Rathke’s cleft
cyst or craniopharyngiomas
• These sellar lesions may be predisposing factors for
infection. Gram-positive cocci are usually identified
in such abscesses.
CLINICAL FEATURES
• Headache, pituitary insufficiency and visual loss are
common symptoms, similar to a nonfunctioning
pituitary adenoma
• Systemic signs of infection are not common.
IMAGING
CT
• CT features are similar to adenoma. Usually, abscess
may co-exist with adenoma. Establishing correct pre-
operative diagnosis is rare
• A cystic lesion with peripheral enhancement is
characteristic of abscess
MRI
• Plain MRI shows a sellar mass indistinguishable from
adenoma. With contrast, there is rim enhancement of
the mass with persistence of hypointense signal in the
center
• Differential diagnosis: Rathke’s cleft cyst, cranioph-
aryngioma and arachnoid cyst and cystic pituitary
adenoma
• These cyst have characteristic differences on imaging:
– Arachnoid cyst: Usually isointense in all sequences
but heterogeneity can be due to epitheloid
secretions and has no peripheral enhancement
– Rathke’s cyst are hypointense on T1-W images
mostly hyperintense on T2-W images also but can
be iso/hypointense at times. Can have rim
enhancement rarely
– Craniopharyngiomas are partly cystic with nodular
enhancing lesions and calcifications. Purely cystic
lesion will show peripheral enhancement
– In case of any pituitary lesion with heterogeneous
signal and rim enhancement, pituitary abscess
should be considered.
BIBLIOGRAPHY
1. Adams WM, Laitt RD, Thorne JA. MRI and CT in a case
of pituitary abscess. Clin Radiol 1999;54(4):270-1.
2. Erdogan G, Deda H, Tonyukuk V. Magnetic resonance
imaging and computerized tomography images in a case
of pituitary abscess. J Endocrinol Invest 2001;24(11):887-91.
3. Guigui J, Boukobza M, Tamer I, Guichard JP, Wyplosz B,
Reizine D, Merland JJ. Case report: MRI and CT in a case
of pituitary abscess. Clin Radiol 1998;53(10):777-9.
4. Hernandez I, Garcia L, Guinto G, Cabrera L, Mercado M.
Bacterial pituitary abscess: An unusual cause of
panhypopituitarism. Endocr Pract 2002;8(6):424-8.
5. Kabuto M, Kubota T, Kobayashi H, Takeuchi H, Kubota T,
Nakagawa T, Kodera T. MR imaging and CT of pituitary
abscess: Case report and review. Neurol Res 1996;18(6):
495-8.
6. Sabbah P, Bonardel G, Herve R, Marjou F, Hor F, Pharaboz
C, Bauduceau B. CT and MRI findings in primitive
pituitary abscess: A case report and review of literature.
J Neuroradiol 1999;26(3):196-9.
7. Wolansky LJ, Gallagher JD, Heary RF, Malantic GP,
Dasmahapatra A, Shaderowfsky PD, Budhwani N. MRI
of pituitary abscess: Two cases and review of the literature.
Neuroradiology 1997;39(7):499-503.
 Chapter 36 Chiasmal Disorders: No Age Predilection 463

A B

Figs 36.7A and B: (A) Sagittal T1-weighted image and (B) Coronal T2-weighted image showing a well-defined sellar. Suprasellar
lesion demonstrating T1-hyperintense signal and T2-hypointense signal—pituitary abscess was found at surgery. The
T1-hyperintense signal may be due to high proteinaceous content

Fig. 36.8: Intraoperative endoscopic view of the pituitary abscess

of the above patient
SECTION 6
Retrochiasmal Visual
Pathway Lesions
SECTION OUTLINE
37. Anatomy and Clinical Features of the Retrochiasmal
Visual Pathway
38. Congenital Lesions
39. Infections
40. Hydrocephalus
41. Vascular Lesions
42. Intracranial Neoplasms
Chapter 37
Anatomy and Clinical Features
of the Retrochiasmal Visual Pathway
INTRODUCTION
The lesions that affect the visual pathway beyond the
optic chiasm will be discussed in this section.
ANATOMY (FIGURE 37.1A)
OPTIC TRACT
• The optic tract begins in the posterolateral angle of
the chiasm, runs back and lateral between the tuber
cinereum and the anterior perforated substance
• The optic tracts run in close association with the
posterior cerebral arteries and end in the lateral
geniculate body
• Each optic tract contains ipsilateral temporal fibers
and contralateral nasal fibers
• Orientation of the nerve fibers in the optic tract is given
in Figure 37.1B.
LATERAL GENICULATE BODY
• The lateral geniculate body (LGB) is an ovoid cap-shaped
structure composed of both gray and white matter
located on the posterolateral aspect of the pulvinar
• More than 80 percent of ipsilateral optic tract fibers
converge in each lateral geniculate body
• Few of the fibers that do not synapse at LGB reach
the superior colliculus and the pretectal region
• LGB is divided into six layers and alternate layers
receive information from alternate eyes. Another way
of representation is the parvo- and magnocellular
pathways
• The outer four layers are composed of small cells, and
correspondingly, receive inputs from the small
ganglion cells of the retina. These layers are called
the parvocellular layers. The magnocellular layers,
on the other hand, are composed of large cells and
receive their input from large ganglion cells.
OPTIC RADIATIONS
• From the LGB, the fibers reach the primary visual
cortex as the optic radiations
• The optic radiation fibers divide into three bundles—
dorsal, lateral and ventral on their way to the striate
cortex
• The dorsal and lateral bundles pass through
the posterior temporal and parietal lobes to reach the
cortex
• But the ventral bundle makes a loop into the temporal
lobe, anterior and lateral, above and around the
temporal horn of lateral ventricle to reach the cortex.
The anterior deviation of this inferior optic radiation
is known as Meyer’s loop and runs lateral to the
inferior horn of the lateral ventricle (Figure 37.1E)
• The lateral bundle carries more than half of the optic
radiation which represents the hemimaculas of both
the sides and reaches the occipital pole
• The dorsal bundle carries the upper fibers of both eyes
which arise from medial part of LGB—the upper lip
of calcarine fissure
• The ventral bundle carries the lower and peripheral
fibers which originates from the lateral part of the LGB
reaches the lower lip of calcarine fissure.
468 Section 6 Retrochiasmal Visual Pathway Lesions

Fig. 37.1A: Diagrammatic representation of the visual pathway

(Courtesy: www.theodora.com/maps)

Fig. 37.1B: Arrangement of nerve fibers in optic tract

 Chapter 37 Anatomy and Clinical Features of the Retrochiasmal Visual Pathway 469

PRIMARY VISUAL CORTEX AREA-17

• This area, also called as Brodmann’s area, is located
in the superior and inferior lips of calcarine fissure
• The primary visual cortex area-17 is surrounded by
the higher level visual areas—18 and 19 Lesion 3: Right optic tract lesion—left homonymous
• The striate cortex is posteriorly limited by the lunate hemianopia
sulcus
• Primary visual cortex has six layers—4th is divided
into sublaminae and contains the line of Gennari
which represents the histological myelin terminals of
the optic radiations
• The upper lip of calcarine sulcus receives information Lesion 4: Meyer’s loop lesion—left pie in sky appearance
from the contralateral superior fibers (inferior visual
field) and the lower lip receives information from the
contralateral inferior fibers (superior visual field)
• The macula is represented at the occipital pole.

CLINICAL FEATURES OF
RETROCHIASMAL LESIONS
• Unilateral retrochiasmal visual pathway lesions
typically produce contralateral homonymous visual
field defects with preservation of visual acuity.
Associated neurological features may help in
localizing the lesion
• Field defects produced by hemorrhage, inflammation
and ischemia are acute in onset, while those caused
by compression develop more slowly
• Dyschromatopsia manifests earlier than field loss
• Isolated homonymous hemianopia is often a result of
a vascular lesion. Occipital lobe is the most common
site
• Older patients frequently have vascular lesions while
younger patients may have congenital or acquired
nonvascular etiologies, such as neoplasm, abscess, or
demyelinating disease
• Neuroimaging, preferably MRI should be the first
diagnostic procedure to determine the etiology of the
visual field defect
Lesion 1: Optic nerve lesion—complete vision loss
Lesion 2: Chiasmal lesion—bitemporal field loss
Lesion 5: Parietal optic radiations lesion—left pie in floor
appearance
Lesion 6: Occipital cortex lesion—macular sparing homo-
nymous hemianopia
• Vision at the fovea is spared, perhaps because there
is such a large representation of the fovea in the cortex,
or perhaps due to overlapping blood supply
• The loss of vision is not a complete hemifield, but a
notched hemifield. This phenomenon is called as
macular sparing.
OPTIC TRACT LESIONS
• Usually result in an incongruous field defect
contralateral to the lesion
• RAPD is noted in the contralateral eye
• Ipsilateral temporal pallor of the optic disc and
contralateral bow-tie optic atrophy is possible
• If a compressive mass lesion also affects cerebral
peduncle, hemiparesis on the side of hemianopia is
seen.
Causes
• Vascular, demyelination, trauma and brain tumors are
more common
• In adults, the most common cause of unilateral
retrochiasmal visual loss is stroke
470 Section 6 Retrochiasmal Visual Pathway Lesions

Fig. 37.1C: Arrangement of nerve fibers in lateral geniculate body

Fig. 37.1D: Blood supply of LGB

Fig. 37.1E: Orientation of the optic radiation

Fig. 37.1F: Location of fibers at the occipital cortex

 Chapter 37 Anatomy and Clinical Features of the Retrochiasmal Visual Pathway
• A sellar mass can affect optic tract, especially if the
optic chiasm is prefixed. In such cases, blindness or
near blindness occurs on the ipsilateral side with a
temporal visual field defect on the contralateral side.
LATERAL GENICULATE BODY LESIONS
• The dorsal aspect of the LGB subserves the macular
region, the lateral side the superior visual field, while
the medial side the inferior field
• Vascular lesions of the LGB are more likely to respect
anatomic boundaries and produce homonymous
sector defects with sloping borders
• The pupillary reactions are preserved in LGB lesions,
however, hemianopic pattern of optic atrophy may
occur.
Causes
• Anterior choroidal artery infarct can result in upper
and lower congruous homonymous sectoranopia and
associated hemiparesis and hemisensory loss
ipsilateral to hemianopia
• Posterior choroidal artery infarct can result in a
congruous homonymous horizontal sectoranopia and
associated thalamic infarct (impairment of sensation
or central pain on the side ipsilateral to hemianopia).
OPTIC RADIATIONS LESIONS
• Lesions in the internal capsule, temporal lobe, and
parietal lobe may damage the optic radiations
• Parietal lobe lesions may produce contralateral Pie-
in-the-Floor visual field defect. Lesion in the dominant
parietal lobe may have associated Gerstmann
syndrome, viz. finger agnosia, agraphia, acalculia, and
right-left disorientation. Lesion in the nondominant
parietal lobe lesion can have associated contralateral
hemi-neglect, topographic memory loss, and
constructional and dressing apraxia
• Temporal lobe lesion can produce contralateral Pie-
in-the-Sky visual field defect and can have associated
personality change, complex partial seizures and
memory deficits.
OCCIPITAL LOBE LESIONS
• Unilateral occipital lobe lesions cause a contralateral
congruous homonymous hemianopia respecting the
vertical meridian
• PCA territory infarct may produce a macular sparing
hemianopia and this is characteristic of occipital lobe
related hemianopic lesions
• Blood supply to the striate cortex is mostly from the
calcarine branch of posterior cerebral artery (PCA).
471
At the occipital pole, there is anastomosis between
the PCA branches and the superior temporo-occipital
Sylvian artery from middle cerebral artery (MCA).
This dual blood supply to the area for central vision
explains the macular sparing in PCA infarcts
• Other proposed mechanisms for macular sparing in
occipital lobe lesions include bilateral representation
of macula and test artifact due to poor central fixation
by patient
• Homonymous hemianopic central scotoma occurs in
unilateral disturbance at the tip of the occipital lobe
• If the occipital lobe lesion does not involve the anterior
striate cortex, the temporal 30 degrees of the field of
the contralateral eye may be spared. On the other
hand, lesions restricted to the anterior striate cortex
may selectively affect the temporal 30 degrees of
vision of the contralateral eye
• Visual acuity is affected only, if lesions are bilateral
• A proximal PCA occlusion may cause additional
midbrain infarction.
LESIONS AFFECTING THE RETROCHIASMAL
VISUAL PATHWAY
Congenital/Infantile
• Hypoxic ischemic encephalopathy (perinatal hypoxia)
• Inherited metabolic disorders
• Phakomatosis
• TORCH infections
• Neoplasms.
Infections
• Cerebral abscess
• Neurocysticercosis
• Tuberculoma
• Progressive multifocal leukoencephalopathy.
Vascular
• Stroke
• Intracranial hemorrhage
• CNS vasculitis
• Pediatric stroke—Moyamoya disease
• Intracranial vascular malformations.
Demyelination
• Multiple sclerosis
• Acute disseminated encephalomyelitis
• Primary brain tumors
• Secondary brain tumors
• Radiation necrosis
• Trauma.
472 Section 6 Retrochiasmal Visual Pathway Lesions

Fig. 37.1G: Visual pathway lesions affecting the various sites

and the field defect (Courtesy: thalamus.wustl.edu)

Fig. 37.1H: Right homonymous hemianopia

Fig. 37.1I: Left homonymous sectoranopia. Right LGB—lateral

choroidal artery infarct
Chapter 37 Anatomy and Clinical Features of the Retrochiasmal Visual Pathway 473

Fig. 37.1J: Left quadruple sectoranopia—right LGB—anterior

choroidal artery infarct

Fig. 37.1K: Right temporo-occipital infarct

Fig. 37.1L: Left Pie-in-sky appearance

Fig. 37.1M: Right parietal hematoma

474 Section 6 Retrochiasmal Visual Pathway Lesions

Fig. 37.1N: Left Pie-in-floor appearance

Fig. 37.1O: Right occipital infarct (arrow)

Fig. 37.1P: Left homonymous incomplete defect

Chapter 38

Congenital Lesions
HYPOXIC ISCHEMIC ENCEPHALOPATHY

• Hypoxic ischemic encephalopathy (HIE) is a result of Imaging Studies

perinatal hypoxia/hypoperfusion and hypoglycemia
• Head ultrasonography is most useful for the detection
• In reality, it is difficult to establish a clear relationship
of PVL, and may detect basal ganglia lesions. Selective
between perinatal brain injury and hypoxia/ischemia
neuronal injury and parasagittal or watershed lesions
• There are a number of etiologies in the differential
frequently are missed by ultrasound
diagnosis of neurologic dysfunction in the neonate,
• Ultrasonography has poor specificity for differentia-
therefore, the term newborn encephalopathy is
ting between increased echogenicity due to ischemic
alternatively accepted
and hemorrhagic lesions. Ultrasonography may also
• A history of low APGAR score at 1 and 5 minutes is
miss focal and multifocal ischemic lesions, which may
an indicator of HIE and subsequent morbidity
be picked up by CT or MRI. This is especially true in
• HIE has a spectrum of clinical manifestations from
small cortical infarcts. Early, large ischemic infarcts
mild-to-severe. Obtundation, stupor, or coma as well
(e.g. large middle cerebral artery infarct) may be
as muscle hypotonia, altered deep tendon reflexes,
picked up by ultrasonography before detection by CT.
postasphyxial seizures occur in moderate-to-severe
• Cranial CT
grade. Brainstem abnormalities are additionally seen
– CT detects focal, multifocal, and generalized
in the severe grade
ischemic lesions. In the first few days after a severe
• The children may exhibit microcephaly,
hypoxic-ischemic insult, bilateral hypodensities are
developmental delay, cortical visual loss,
seen, which probably reflect both neuronal injury
quadriparesis, spasticity, and seizures
and edema. Diffuse cortical injury is not be detected
• Visual function may be as poor as tunnel vision, light
initially with CT. After a period of days to weeks,
perception or no light perception.
diffuse hypodensity with loss of the gray-white
matter differentiation may appear. Diffuse cerebral
PERIVENTRICULAR LEUKOMALACIA
atrophy with ex-vacuo ventricular dilatation as a
• Periventricular leukomalacia (PVL) is the most typical result of severe hypoxemic insult may take several
lesion in a preterm infant with perinatal hypoxic insult weeks to develop. Atrophy is a consequence of
• Premature and low birth weight infants are cortical and white matter destruction
particularly vulnerable to hypoxic/ischemic injury to – Areas of hypodensity are more challenging to
periventricular white matter interpret in premature infants. CT scanning can
• Neuroimaging frequently demonstrates reduced be performed to more reliably diagnose generali-
amount of periventricular white matter and ventricular zed edema in the premature newborn
dilation. However, the changes may not be evident for – Focal ischemic infarcts may be well visualized by
several months until myelination has occurred. CT after 48 hours. On the first day after a focal
476 Section 6 Retrochiasmal Visual Pathway Lesions
thromboembolic event, the ischemic area may not
be visible by means of CT. A CT scan depicting
hypodensity in the left-middle cerebral artery
distribution on the first day of life suggests
prenatal onset of ischemia. Symptoms of a focal
infarct (usually seizures) on the first day of life with
normal CT findings in a patient, in whom a
hypodensity develops over the first week of life,
suggests perinatal onset of ischemia. Hemorrhagic
conversion of a focal ischemic lesion is uncommon
in the neonatal period, but CT can detect it easily
– CT may also detect hemorrhagic lesions, which
are seen in 10-25 percent of HIE-NE cases,
including intraparenchymal, intraventricular, and
subarachnoid hemorrhages. CT can pick-up basal
ganglia thalamic lesions and selective neuronal
injury, but MRI is more sensitive
– PVL can be visualized on the CT scan around the
frontal horns or posteriorly around the trigonal
area of the lateral ventricles. On the CT scan, PVL
appears as a region of decreased attenuation,
occasionally intermixed with areas of increased
density due to secondary hemorrhage. Careful
interpretation of periventricular hypodensities on
the CT scan is suggested because maturation and
myelination processes lead to an increase in lipid
and protein with a decrease in water content of
the white matter. This explains the findings of
hypodensities in neonates with normal
development
 The long-term changes seen on a CT scan of
patients with PVL are thinning of the peri-
ventricular white matter (especially on the
region of the trigone) and ventriculomegaly
with an irregular outline and deep sulci on the
wall of the lateral ventricles
 Calcification of the areas of white matter
necrosis also may occur over time
– A CT scan demonstrating generalized, diffuse low
attenuation after a hypoxic-ischemic event predicts
both neonatal death and long-term severe
disability, while normal CT findings predicts a
normal or mild disability outcome
• Magnetic resonance imaging
– MRI is very sensitive in detecting focal and
multifocal ischemic lesions
• The following pattern of brain injury noted:
– Cortical injury: The involved region will demons-
trate T1 hypointense signal and T2 hyperintense
signal
– Deep gray matter, perirolandic cortex and
dorsolateral putamen—the predeliction for these
regions has been attributed to high metabolic
activity. These regions demonstrate T1 hyper-
intense signal probably attributed to hemorrhage
– Diffuse brain injury—this results from global
hypoxia
 In the premature infant, there is selective
damage to the periventricular white matter due
to high metabolic activity in the vascular water-
shed zone. This results in periventricular
leukomalacia (PVL). MRI markers of findings
in PVL are thinning and increased T2 signals of
the white matter, especially in the peritrigonal
area and ventricular dilatation. These findings
have a good correlation with spastic diplegia in
premature infants with a history of PVL, but also
are found frequently in term infants after HIE.
Cystic encephalomalacia is seen as cystic
changes in the cortex or deep white matter
 HIE in older children result in infarcts at the
watershed zones
 Diffusion-weighted imaging may help in
detection of the zone of infarction earlier than
conventional MR imaging, but research has
shown that it does not follow the same pattern
or sensitivity as an adult infarction
 Proton MRS may reveal indirect evidence of
neuronal damage by showing a drop of the
N-acetylaspartate-to-choline ratio, which has
some correlations with subsequent neurologic
deficits.
BIBLIOGRAPHY
1. Amiel-Tison C, Ellison P. Birth asphyxia in the full-term
newborn: Early assessment and outcome. Dev Med Child
Neurol 1986; 28(5):671.
2. Azzopardi D, Wyatt JS, Cady EB. Prognosis of newborn
infants with hypoxic-ischemic brain injury assessed by
phosphorus magnetic resonance spectroscopy. Pediatr Res
1989;25(5): 445-51.
3. Barkovich AJ, Westmark K, Partridge C. Perinatal asphyxia:
MR findings in the first 10 days. Am J Neuroradiol 1995;
16(3):427-38.
4. Ekert P, Perlman M, Steinlin M. Predicting the outcome
of postasphyxial hypoxic-ischemic encephalopathy within
4 hours of birth. J Pediatr 1997;131(4):613-7.
5. Lipp-Zwahlen AE, Deonna T, Micheli JL. Prognostic value
of neonatal CT scans in asphyxiated term babies: Low
density score compared with neonatal neurological signs.
Neuropediatrics 1985;16(4):209-17.
 Chapter 38 Congenital Lesions 477

Fig. 38.1: Axial CT scan of the brain at the level of the occipital
lobes showing bilateral occipital hypodensities with dilatation of
occipital horns—consistent with perinatal hypoxic injury in a child
with bilateral optic atrophy

A B

Figs 38.2A and B: (A) Axial T2-weighted image and (B) Axial FLAIR image at the level of the occipital lobes showing bilateral
occipital gliosis consistent with perinatal hypoxic injury
478 Section 6 Retrochiasmal Visual Pathway Lesions
INHERITED METABOLIC DISORDERS
Inherited metabolic disorders that primarily affect the white matter include the classic leukodystrophies such as
adrenoleukodystrophy, Krabbe’s globoid cell and metachromatic leukodystrophy and the other white matter
disorders such Canavan’s disease, Alexander’s disease, Pelizaeus-Merzbacher disease and Cockayne syndrome
ADRENOLEUKODYSTROPHY
• Adrenoleukodystrophy (ALD) is a rare, X-linked,
childhood degenerative disorder of myelin. CNS
demyelination is caused by a defect in the peroxisomal
long-chain fatty acid metabolism
• Leukodystrophies are different from demyelinating
disorders such as multiple sclerosis, in which myelin
is formed normally, but is lost by immunologic
dysfunction or other reasons
• Autosomal recessive forms of the disease do exist, but
are extremely rare.
Childhood Onset Form of ALD
• It occurs between ages of 5 to 10 years
• Long-chain fatty acids accumulate in the brain white
matter and adrenal glands leading to abnormal
behavior and cognition, failure to thrive, visual loss,
degeneration of auditory functions, gait disturbances,
skin hyperpigmentation, and hypoadrenalism.
Neurological and systemic abnormalities precede
visual loss by several years.
Adolescent Onset Form of ALD
• In this form, the spinal cord dysfunction is more
prominent and therefore is called adrenomyelo-
neuropathy
• The patients usually present with weakness and
numbness of the limbs and excretory problems. Most
victims of this form are also males, although female
carriers rarely exhibit similar symptoms.
Diagnosis
• The diagnosis is established by detection of low blood
cortisol levels and increased urinary excretion of C22
to C26 fatty acids
• Genetic analysis of the defective gene should be done.
IMAGING
MRI is the imaging modality of choice. It is useful in the diag-
nosis and follow-up of childhood adrenoleukodystrophy.
• Loes et al. described in five patterns:
1. Pattern 1 is seen in 66 percent of the patients. It
shows predominant involvement of the parieto-
occipital white matter and splenium of the corpus
callosum. It may also include visual and auditory
pathways
2. Pattern 2 is seen in 15.5 percent of the patients.
Mainly seen in adolescent. It shows involvement
of the frontal lobe and genu of the corpus callosum
3. Pattern 3 is seen in 12 percent of the patients. It
showed primary involvement of the frontopontine
or corticospinal projection fibers. It was mainly in
adults
4. Pattern 4 is seen in 1 percent. It showed primary
cerebellar white matter involvement and mainly
in adolescents
5. Pattern 5 is seen in 12.5 percent of the patients. It
showed involvement of the parieto-occipital and
frontal white matter. Seen mainly in children.
• The U-fibers and cortex is spared
• Postgadolinium shows typical enhancement at the
periphery of the lesions corresponding to zone of
active inflammatory demyelination
• Magnetic resonance spectroscopy shows a diminu-
tion in N-acetyl aspartate and an increase in choline
peaks. Spectroscopic changes precede those demons-
trable by MRI.
BIBLIOGRAPHY
1. Bekiesinska-Figatowska M, Tylki-Szymanska A, Walecki
J, Stradomska TJ. MRI findings in an asymptomatic boy
with X-linked adrenoleukodystrophy and his
symptomatic mother. Neuroradiol 2001;43(11):951-2.
2. Eichler FS, Barker PB, Cox C, Edwin D, Ulug AM, Moser
HW, Raymond GV. Proton MR spectroscopic imaging
predicts lesion progression on MRI in X-linked
adrenoleukodystrophy. Neurol 2002;58(6):901-7.
3. Eichler FS, Itoh R, Barker PB, Mori S, Garrett ES, van Zijl
PC, Moser HW, Raymond GV, Melhem ER. Proton MR
spectroscopic and diffusion tensor brain MR imaging in
X-linked adrenoleukodystrophy: Initial experience. Radiol
2002;225(1):245-52.
4. Fatemi A, Smith SA, Dubey P, Zackowski KM, Bastian
AJ, van Zijl PC, Moser HW, Raymond GV, Golay X.
Magnetization transfer MRI demonstrates spinal cord
abnormalities in adrenomyeloneuropathy. Neurol
2005;64(10):1739-45.
5. Hammerschmidt DE. Adrenoleukodystrophy: Abnormal
white matter signal on MRI. J Lab Clin Med 2003; 142(6):
433.
 Chapter 38 Congenital Lesions 479

Figs 38.3A and B: (A) Axial T2-weighted image and (B) Coronal
FLAIR image showing bilateral symmetrical parieto-occipital
periventricular white matter T2-hyperintense signal in a 10-year-old
B child with bilateral poor vision—adrenoleukodystrophy
480 Section 6 Retrochiasmal Visual Pathway Lesions
6. Israel H, Ostendorf F, Stiepani H, Ploner CJ. Spinal cord
atrophy in adrenomyeloneuropathy. Arch Neurol
2005;62(7):1157.
7. Loes DJ, Fatemi A, Melhem ER, Gupte N, Bezman L, Moser
HW, Raymond GV. Analysis of MRI patterns aids
prediction of progression in X-linked adrenoleuko-
dystrophy. Neurol 2003;61(3):369-74.
8. Melhem ER, Loes DJ, Georgiades CS, Raymond GV, Moser
HW. X-linked adrenoleukodystrophy: The role of contrast-
enhanced MR imaging in predicting disease progression.
Am J Neuroradiol 2000;21(5):839-44.
9. Mo YH, Chen YF, Liu HM. Adrenomyeloneuropathy—a
dynamic progressive disorder: Brain magnetic resonance
imaging of two cases. Neuroradiol 2004;46(4):296-300.
Epub 2004 Mar 4.
10. Moser HW, Loes DJ, Melhem ER, Raymond GV, Bezman L,
Cox CS, Lu SE. X-Linked adrenoleukodystrophy: Overview
and prognosis as a function of age and brain magnetic
resonance imaging abnormality: A study involving 372
patients. Neuropediatrics 2000;31(5):227-39. Review.
11. Oz G, Tkac I, Charnas LR, Choi IY, Bjoraker KJ, Shapiro
EG, Gruetter R. Assessment of adrenoleukodystrophy
lesions by high-field MRS in non-sedated pediatric
patients. Neurol 2005;64(3):434-41.
12. Patay Z. Diffusion-weighted MR imaging in leuko-
dystrophies. Eur Radiol 2005;15(11):2284-303. Epub 2005
Jul 15.

MAPLE SYRUP URINE DISEASE
• It is characterized by failure to catabolize branched
chain amino acids namely leucine, isoleucine, and
valine. The corresponding ketoacids accumulate and
result in urinary excretion of a metabolite with
characteristic odor that resembles maple syrup
• Inheritance is autosomal recessive.
CLINICAL FEATURES
• Typically presentation is within 4-7 days after birth with
severe and rapidly progressive neurological deficits
• Without treatment death occurs within 1 year
• Milder intermediate and intermittent forms have been
described.
IMAGING
• Sequential imaging is essential to follow the natural
course of the disease
• Computed Tomography: Typically are negative
during first few postnatal days. A marked,
generalized diffuse edema appears and remains for
6-7 weeks. In untreated patients, it then decreases and
is transformed into well-demarcated periventricular
white matter disease
• A characteristic intense local edema involves the deep
cerebellar white matter, dorsal brainstem, cerebral
Chapter 38 Congenital Lesions 481
peduncles and posterior limb of internal capsule. Low
densities in the globus pallidus and thalami may also
occur
• MRI shows hyperintense signal in these regions.
BIBLIOGRAPHY
1. Brismar J, Aqeel A, Brismar G, Coates R, Gascon G,
Ozand P. Maple syrup urine disease: Findings on CT and
MR scans of the brain in 10 infants. Am J Neuroradiol
1990;11(6):1219-28.
2. Di Rocco M, Biancheri R, Rossi A, Allegri AE, Vecchi V,
Tortori-Donati P. MRI in acute intermittent maple syrup
urine disease. Neurol 2004;63(6):1078.
3. Jan W, Zimmerman RA, Wang ZJ, Berry GT, Kaplan PB,
Kaye EM. MR diffusion imaging and MR spectroscopy of
maple syrup urine disease during acute metabolic
decompensation. Neuroradiol 2003;45(6):393-9.
4. Parmar H, Sitoh YY, Ho L. Maple syrup urine disease:
Diffusion-weighted and diffusion-tensor magnetic
resonance imaging findings. J Comput Assist Tomogr
2004;28(1):93-7.
5. Sener RN. Diffusion magnetic resonance imaging patterns
in metabolic and toxic brain disorders. Acta Radiol
2004;45(5):561-70.
6. Uziel G, Savoiardo M, Nardocci N. CT and MRI in maple
syrup urine disease. Neurol 1988;38(3):486-8.
482 Section 6 Retrochiasmal Visual Pathway Lesions

Figs 38.4A and B: A known case of maple syrup urine disease

with optic atrophy. Axial CT scan of the brain showing bilateral
frontal and parieto-occipital periventricular hypodensities with
B sparing of the subcortical U fibers and no mass effect
 Chapter 38 Congenital Lesions 483

MUCOPOLYSACCHARIDOSES
• Mucopolysaccharidoses (MPS) includes a group of • Retinal degeneration occurs only when heparin sulfate
inherited metabolic diseases in which there is a defi- is stored (Hurler, Scheie, Hurler-Scheie, Hunter,
ciency of lysosomal enzymes affecting the degradation Sanfilippo syndrome)
of glycosaminoglycans (mucopolysaccharides) • Ophthalmic features include visual loss from corneal
• Types: clouding, optic atrophy, papilledema, progressive
1. Hurler syndrome retinopathy, secondary open angle glaucoma, etc.
2. Hunter syndrome
3. Morquio syndrome CLASSIFICATION OF THE
4. Sanfilippo syndrome MUCOPOLYSACCHARIDOSES
5. Scheie syndrome AND SITES OF INVOLVEMENT
• Hunter’s disease is inherited by sex-linked
Type Eponym Bone Viscera CNS
transmission; all others are inherited by autosomal
recessive transmission IH Hurler + + +
• The clinical picture is that of variable skeletal, visceral, IS Scheie + + –
and central nervous system involvement. The clinical I H/S Hurler-Scheie + + +
manifestations vary for each type of MPS due to II A, B Hunter + + +
differences in specific enzyme defect or in the tissue III A, B, C, D Sanfilippo – – +
localization of the involved enzyme. Ten enzyme IV A, B Morquio + – –
deficiencies have been identified giving rise to VI Maroteaux-Lamy + – +
VII Sly + + +
different syndromes
VIII Diferente + – –
• The diagnosis of a disorder of mucopolysaccharide
catabolism is usually made on the basis of
characteristic clinical findings in association with IMAGING
demonstration of the typical enzymatic deficiency or
• MR findings are quite variable. In few patients, there
detection of elevated urinary GAGs
may be no intracranial abnormality on either MRI or
• A gargoyle-like face and dwarfism are characteristic
CT scan
manifestations of the mucopolysaccharidoses • In cases with mild involvement, thickening of the skull
• Progressive psychomotor retardation, hepato- and slight ventricular enlargement may be the only
splenomegaly, neurosensory hearing loss, cardiac finding. Large head may be noted in infancy
anomalies can also occur • J-shaped sella and small foramen magnum may be
• Morquio’s disease, and far less common Scheie’s and seen
Diferente’s diseases are the only mucopoly- • When the disease is advanced, findings include
saccharidoses in which the patients are not severely hydrocephalus, dural thickening, and periventricular
delayed in development white matter lesions. The periventricular lesions may
• Spinal cord compression is common, especially at the be seen as multiple small areas of bright signal on
upper cervical level and foramen magnum. The cord T2-weighted sequences, and dark areas on T1-
compression results from skeletal narrowing and weighted sequences, reflecting the long T1 and T2
dural thickening from mucopolysaccharide deposits. characteristics of the lesions
Spinal cord compression may also be due to • The punctate lesions are due to perivascular
atlantoaxial subluxation or thoracic gibbus. Meningeal involvement, in which there is a large accumulation of
involvement can cause hydrocephalus vacuolated cells distended with mucopolysaccharide.
• Early death and increased risk for cardiovascular As the disease progresses, the lesions become more
collapse or laryngospasm during generalized widespread and larger, reflecting the development of
anesthesia exists infarcts in demyelination
• Cornea is frequently affected by the abnormal • Bone marrow transplant has been used as a method
glycosaminoglycan (GAG) metabolism because the of therapy in the mucopolysaccharidoses. MRI has
GAGs make up the corneal stromal ground substance been used to follow the progression of patients with
484 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 38.5A to C: A known case of mucopolysaccharides with optic atrophy: (A) Axial FLAIR showing moderate hydrocephalus
with periventricular ooze, (B) Coronal T2-weighted image and (C) Sagittal T1-weighted image showing a J-shaped sella (white
arrow) and aqueductal stenosis (black arrow). Note: Postoperative changes at the craniovertebral junction following posterior
decompression for small foramen magnum

Hurler’s syndrome treated with bone marrow
transplant. Improvement in myelination and
improved gray-white differentiation has been
reported following bone marrow transplant.
BIBLIOGRAPHY
1. Gabrielli O, Polonara G, Regnicolo L, Petroni V, Scarabino
T, Coppa GV, Salvolini U. Correlation between cerebral
MRI abnormalities and mental retardation in patients with
mucopolysaccharidoses. Am J Med Genet A 2004;125(3):
224-31.
2. Kulkarni MV, Williams JC, Yeakley JW, Andrews JL,
McArdle CB, Narayana PA, Howell RR, Jonas AJ.
Magnetic resonance imaging in the diagnosis of the cranio-
cervical manifestations of the mucopolysaccharidoses.
Magn Reson Imaging 1987;5(5):317-23.
3. Lee C, Dineen TE, Brack M, Kirsch JE, Runge VM. The
mucopolysaccharidoses: Characterization by cranial MR
imaging. Am J Neuroradiol 1993;14(6):1285-92.
4. Matheus MG, Castillo M, Smith JK, Armao D, Towle D,
Muenzer J. Brain MRI findings in patients with
Chapter 38 Congenital Lesions 485
mucopolysaccharidosis types I and II and mild clinical
presentation. Neuroradiol 2004;46(8):666-72.
5. Murata R, Nakajima S, Tanaka A, Miyagi N, Matsuoka O,
Kogame S, Inoue Y. MR imaging of the brain in patients
with mucopolysaccharidosis. Am J Neuroradiol 1989;
10(6):1165-70.
6. Parsons VJ, Hughes DG, Wraith JE. Magnetic resonance
imaging of the brain, neck and cervical spine in mild
Hunter’s syndrome (mucopolysaccharidoses type II). Clin
Radiol 1996;51(10):719-23.
7. Rauch RA, Friloux LA 3rd, Lott IT. MR imaging of cavitary
lesions in the brain with Hurler/Scheie. Am J Neuroradiol
1989;10(5 Suppl):S1-3. No abstract available.
8. Taccone A, Tortori Donati P, Marzoli A, Dell’Acqua A,
Occhi M, Gatti R, Leone D. Mucopolysaccharidoses:
Evaluation of the cranium by computed tomography and
magnetic resonance. Radiol Med (Torino) 1992;84(3):
236-41.
9. Zafeiriou DI, Savvopoulou-Augoustidou PA, Sewell A,
Papadopoulou F, Badouraki M, Vargiami E, Gombakis
NP, Katzos GS. Serial magnetic resonance imaging
findings in mucopolysaccharidosis IIIB (Sanfilippo’s
syndrome B). Brain Dev 2001;23(6):385-9.
Chapter 39
CEREBRAL ABSCESS
Brain abscess is the most common focal infection and can
be caused by various pathogens.
ORGANISMS
• Brain abscesses frequently arise secondary to
hematogenous dissemination of an extracranial site,
by direct extension from a contiguous suppurative
focus or secondary to meningitis
• In children, majority of the abscesses are associated
with cyanotic heart disease
• Staphylococcus and Streptococcus are frequently
encountered in immunocompetent individuals
• In neonates, the most frequently implicated
organisms include Citrobacter, Proteus, Pseudomonas,
and Serratia species, as well as Staphylococcus aureus.
These abscesses are often large and have poorly
formed capsules
• Occasionally, organisms other than pyogenic bacteria
cause cerebral abscess. Examples include Myco-
bacterium tuberculosis, non-tuberculous mycobacteria,
fungi, parasites, and Actinomyces and Nocardia species.
PATHOLOGY
• The pathology of abscess evolves through four stages,
viz. early cerebritis, late cerebritis, early capsule
formation, and late capsule formation
1. Early cerebritis is the initial phase of abscess
formation. The initial infection is focal but not
localized. An unencapsulated mass of congested
brain tissue is seen, with regional edema. Scattered
necrotic foci and microscopic petechial
hemorrhages are present, although, no gross tissue
Infections
destruction is seen. Early cerebritis lasts as long as
3 to 5 days
2. After 3 to 5 days, the reaction to the infectious
agent progresses to late cerebritis. The infection
becomes more focal with zones of necrosis. Blood
vessels surrounding the infection proliferate. The
central area of the infection becomes necrotic,
surrounded by a ring of inflammatory cells,
macrophages, granulation tissue, and fibroblasts.
The late cerebritis stage lasts 5 to 14 days
3. With the beginning of the early capsule stage,
collagen and reticulin form a well-delineated
capsule. The central core consists of necrotic and
inflammatory debris. The abscess capsule
increasingly thickens with the addition of more
collagen. With the formation of a well-defined
capsule, the mass effect and surrounding edema
begin to subside. Gliosis around the abscess
periphery further defines the region
4. The wall of a well-defined late abscess consists of
an inner inflammatory layer, a middle collagenous
layer, and an outer gliotic layer. The late capsule
stage may last for months.
RADIOLOGICAL FINDINGS
CT Scan
• CT manifestations of an intracranial abscess depend
on the stage of the abscess formation
• The earliest phase may be related to meningitis, with
no findings on unenhanced CT studies. Enhancement
of the meningeal surfaces is a nonspecific and
inconsistent finding in patients with meningitis
 Chapter 39 Infections 487

Fig. 39.1: A 6-year-old female child presented with high-grade

fever, headache and vomiting. Axial postcontrast CT scan of
the brain showing an irregular thick ring enhancing lesion in the
left thalamic region with extensive perilesional edema causing
mass effect on the ventricles and midline shift
488 Section 6 Retrochiasmal Visual Pathway Lesions
• During early cerebritis, nonenhanced CT scans may
demonstrate normal findings or may show only
poorly marginated subcortical hypodense areas
• Contrast-enhanced CT studies demonstrate an ill-
defined contrast-enhancing area within the
edematous region
• During the early stage of a formed abscess, the lesion
coalesces, with an irregular enhancing rim that
surrounds a central low-attenuating area
• Scans obtained with a time delay following contrast
enhancement in cerebritis may show contrast “filling-
in” the central low-attenuating region. A formed
abscess will not “fill-in” the central portion of the
abscess
• Peripheral edema results in considerable mass effect
with sulcal obliteration.
Differential Diagnosis for Ring Enhancing Lesions
• Metastatic brain tumor
• Some primary brain tumors (particularly, grade 4
astrocytoma)
• Granuloma
• Resolving hematoma.
MRI
• MRI findings of brain abscess vary with time
• Early cerebritis stage: This stage presents as an ill-
defined subcortical hyperintense zone on T2-weighted
imaging. Contrast-enhanced T1-weighted studies
demonstrate poorly delineated enhancing areas
within the isointense-to-mildly hypointense
edematous region
• Late cerebritis stage: During this stage, the central
necrotic area is hyperintense to brain tissue on proton-
density and T2-weighted sequences. The thick
somewhat irregularly marginated rim appears
isointense to mildly hyperintense on spin-echo
T1-weighted images and isointense to relatively
hypointense on proton-density and T2-weighted
scans. Peripheral edema is common. The rim enhances
intensely following contrast administration. Satellite
lesions may be demonstrated
• Early and late capsule stages: During the early and
late capsule stages, the collagenous abscess capsule
is visible prior to contrast as a comparatively thin-
walled isointense-to-slightly hyperintense rim on T1-
weighted images and hypointense on T2-weighted
images. Postcontrast scans show a thin uniform
enhancement usually thinner towards the
ventricular surface. Shrinkage of the necrotic center
and decrease in the capsular hypointensity are more
reliable indicators of healing
• Advanced imaging such as proton spectroscopy and
diffusion: Weighted imaging are now used in
establishing accurate diagnosis. The spectroscopic
findings include elevation of metabolites of bacterial
origin, including acetate, lactate, succinate, cystosolic
acids and amino acids. In contrast, necrotic tumors
demonstrate elevated choline and decreased
N-acetylaspartate. It also helps in evaluating response
to treatment seen as decline of metabolites
• Diffusion-weighted imaging with apparent diffu-
sion coefficient: It may be useful in differentiating
abscess from necrotic tumor. Diffusion-weighted echo
planar images (DWI) demonstrate an abscess as high
signal intensity with a corresponding reduction in the
apparent diffusion coefficient. The brightness on DWI
is related to the cellularity and viscosity of the contents
within the abscess cavity. Tumors with central
necrosis have marked hypointensity on diffusion-
weighted images and much higher apparent diffusion
coefficient values.
BIBLIOGRAPHY
1. Ackermann G, Schoen H, Schaumann R, et al. Rapidly
growing tumor-like brain lesion. Infection 2001;29(5):
278-9.
2. Desprechins B, Stadnik T, Koerts G. Use of diffusion-
weighted MR imaging in differential diagnosis between
intracranial necrotic tumors and cerebral abscesses. Am J
Neuroradiol 1999;20:1252-7.
3. Enzmann DR, Britt RD, Placone R. Staging of human brain
abscess by computed tomography. Radiol 1983;146:703-8.
4. Garg RK, Desai P, Kar M, Kar AM. Multiple ring enhan-
cing brain lesions on computed tomography: An Indian
perspective. J Neurol Sci 2008;266(1-2):92-6.
5. Gaviani P, Schwartz RB, Hedley-Whyte ET, Ligon KL,
Robicsek A, Schaefer P, Henson JW. Diffusion-weighted
imaging of fungal cerebral infection. Am J Neuroradiol
2005;26(5):1115-21.
6. Hartmann M, Jansen O, Heiland S, et al. Restricted
diffusion within ring enhancement is not pathognomonic
for brain abscess. Am J Neuroradiol 2001;22(9):1738-42.
7. Kapsalaki EZ, Gotsis ED, Fountas KN. The role of proton
magnetic resonance spectroscopy in the diagnosis and
categorization of cerebral abscesses. Neurosurg Focus
2008;24(6):E7.
8. Kastrup O, Wanke I, Maschke M. Neuroimaging of
infections of the central nervous system. Semin Neurol
2008;28(4):511-22.
 Chapter 39 Infections 489

A B

C D

Figs 39.2A to D: (A) Axial T2-weighted image, (B) Axial T1-weighted image showing a centrally necrotic lesion with a isointense
rim in T1-weighted image and hypointense rim on T2-weighted of image, (C) Axial diffusion-weighted image showing bright signal
in the necrotic center indicating restricted diffusion and (D) Coronal T1-postcontrast study showing thick irregular rim enhancement
suggestive of an abscess
490 Section 6 Retrochiasmal Visual Pathway Lesions
NEUROCYSTICERCOSIS
• Neurocysticercosis is the most common parasitic
infection of the central nervous system
• Tissue-invading larval forms of the pork tapeworm
Taenia solium (Cysticercus cellulosae) causes
neurocysticercosis
• Humans are the only definitive hosts for Taenia solium
and pigs are the usual intermediate hosts, although
dogs, cats, and sheep can harbor the larval forms
• The adult tapeworm usually resides in the upper
jejunum and sheds eggs, which are excreted in human
feces. These eggs are infective to both humans and
animals
• After ingestion, the eggs embryonate, penetrate the
intestinal wall, and are carried to various tissues,
with a predilection for striated muscle of neck and
trunk
• The encysted larval stage (cysticerci) then develops.
Cysticerci are fluid-filled oval cysts, approximately
1 to 2 cm in diameter, with an internal scolex. These
cysticerci can survive for long periods
• The form of Taenia solium infection that, develops in
humans, depends upon mode of infection. Ingestion
of under cooked pork containing cysticerci leads to
intestinal tapeworms. Infections that cause human
cysticercosis follow the ingestion of Taenia solium eggs,
usually from fecally contaminated food. Autoinfection
is possible
• Neurocysticercosis is a form of the infection when
cysticerci affect the CNS.
CLINICAL FEATURES
• Most patients do not become symptomatic until 5 to
7 years after the initial infection
• The manifestations of neurocysticercosis reflect two
distinct processes, viz local inflammatory response to
the parasite and the local effects of the space-
occupying lesions
• Various forms are asymptomatic, however, some
affected sites can produce symptoms like
parenchymal (seizures 35-96% of patients),
subarachnoid (meningitis and increased intracranial
tension), intraventricular (hydrocephalus), spinal,
ocular and orbital
• The clinical presentation in an individual patient
depends on the number, form and location of the
cysticerci, the extent of associated inflammatory
response and the duration of disease.
DIAGNOSIS
• The definitive diagnosis is by the demonstration of
cysticercus in the involved tissue, but a diagnosis
can be based on a suggestive clinical presentation
supported by compatible results on neuroimaging
• ELISA to detect antibodies to cysticercus has good to
excellent sensitivity and specificity.
IMAGING
• Computed tomography: CT scanning shows the cyst
and granuloma stages of neurocysticercosis. These
cysts can be solitary or multiple and usually are 5 to
20 mm in diameter
• Approximately, 75 percent of children who are
affected with neurocysticercosis have a solitary lesion.
Lesions are located most often in the cortex or at the
gray-white junction
• Approximately, one-half of lesions have a punctate
high density within the ring (scolex). Between 10 and
20 percent of children have no abnormality
• CT scanning is superior to MRI study in detecting
calcification, which can be useful in differentiating the
punctate cyst of neurocysticercosis in the granuloma
wall from other causes of granulomas; however,
calcification is observed less frequently in children
than in adults
• CT scanning can also detect edema around the cyst,
which is associated with the death of the organism.
Magnetic Resonance Imaging
• MRI with gadolinium is the best imaging test overall
for the diagnosis
• MRI is useful for lesions of the spinal cord, posterior
fossa, brainstem, subarachnoid, and ventricles
• Use of contrast also shows larval death, visible as
enhancement of the cyst wall, which indicates that
the cyst has changed into a granuloma. In addition,
MRI (as well as CT scanning) shows any vasogenic
edema around the cyst, indicative of the body’s
inflammatory response to organism death
• MRI can be used as follow-up imaging to document
improvement based on both a decrease in the
granuloma diameter and a resolution of vasogenic
edema.
 Chapter 39 Infections 491

Fig. 39.3: Axial CT scan orbit showing multiple hyperdense

calcified cysticercal lesions in the brain parenchyma bilaterally

A B

Figs 39.4A and B: (A) Axial T2 FRFSE image and (B) Axial T1 spin echo image of the brain showing extensive small cystic
lesions with scolex scattered in the brain parenchyma—neurocysticercosis
492 Section 6 Retrochiasmal Visual Pathway Lesions
BIBLIOGRAPHY
1. Arriada-Mendicoa N, Celis-Lopez MA, Higuera-Calleja J,
Corona-Vazquez T. Imaging features of sellar cysticercosis.
Am J Neuroradiol 2003;24(7):1386-9.
2. Castillo M. Imaging of neurocysticercosis. Semin
Roentgenol 2004;39(4):465-73. Review.
3. Citow JS, Johnson JP, McBride DQ, Ammirati M. Imaging
features and surgery-related outcomes in intraventricular
neurocysticercosis. Neurosurg Focus 2002;12(6):e6.
4. Del Brutto OH. Neurocysticercosis. Semin Neurol
2005;25(3):243-51. Review.
5. Do Amaral LL, Ferreira RM, da Rocha AJ, Ferreira NP.
Neurocysticercosis: Evaluation with advanced magnetic
resonance techniques and in atypical forms. Top Magn
Reson Imaging 2005;16(2):127-44.
6. Hauptman JS, Hinrichs C, Mele C, Lee HJ. Radiologic mani-
festations of intraventricular and subarachnoid racemose
neurocysticercosis. Emerg Radiol 2005;11(3):153-7.
7. Ng SH, Tan TY, Fock KM. The value of MRI in the
diagnosis and management of neurocysticercosis.
Singapore Med J 2000;41(3):132-4.
8. Noujaim SE, Rossi MD, Rao SK, Cacciarelli AA, Mendonca
RA, Wang AM, Coelho FH. CT and MR imaging of
neurocysticercosis. Am J Roentgenol 1999;173(6):1485-90.
Review.
9. Palacios E, Salgado Lujambio P, Rojas Jasso R. Computed
tomography and magnetic resonance imaging of neuro-
cysticercosis. Semin Roentgenol 1997;32(4):325-34. Review.
10. Zee CS, Go JL, Kim PE, DiGiorgio CM. Imaging of
neurocysticercosis. Neuroimaging Clin N Am 2000;10(2):
391-407. Review.
 Chapter 39 Infections 493

A B

Figs 39.5A to C: (A) Axial T1-weighted image, (B) Axial T2-weighted image and (C) Axial T1-postcontrast image showing a well-
defined ring enhancing lesion with scolex and perilesional edema in the right occipital (arrows) in a patient with left field defect
494 Section 6 Retrochiasmal Visual Pathway Lesions
TUBERCULOMA
• The most common parenchymal form of tuberculosis
is a granuloma known as tuberculoma
• It develops following hematogenous spread of
systemic tuberculosis or from extension of CNS
infection into the adjacent parenchyma via cortical
veins or small penetrating arteries.
LOCATIONS
• Tuberculomas may be found in the brain, meninges,
ventricles or subdural space
• Parenchymal disease most often involves cortico-
medullary junction and periventricular regions due
to hematogenous dissemination
• Most tuberculomas are supratentorial
• Parenchymal disease may occur with or without co-
existing meningitis.
PATHOLOGY
The granuloma is composed of central zone of solid
caseation necrosis surrounded by collagenous tissue,
epithelioid cells, multinucleated giant cells, and
mononuclear inflammatory cells. The tuberculoma is
surrounded by parenchymal edema and astrocytic
proliferation. The capsule and necrotic center show plenty
of tubercle bacilli.
CLINICAL FEATURES
Tubercular granulomas present with signs and symptoms
of a space-occupying lesion and may cause fits,
hemiparesis, loss of vision, visual field or ocular motility
defects.
IMAGING
• CT scan: Tuberculomas are seen in very few patients
with TB meningitis
• 10 to 34 percent patients have multiple lesions
• Contrast-enhanced CT shows ring enhancing lesion
with central necrosis and peripheral organization
• 1/3rd patients show target sign with central calci-
fication or punctate enhancement surrounded by
hypodensity and then surrounding rim enhancement.
This is highly suggestive of tuberculosis
• MRI: Tuberculomas are isointense to gray matter on
T1-weighted images with slightly hyperintense rim
• On T2-weighted images, they show variable signal.
They are isointense to hypointense to brain
parenchyma. The T2-hypointensity is due to T2
shortening by paramagnetic free radicals produced
by macrophages distributed throughout the tuber-
culomas. The T2-hypointensity could also be due to
the greater cellular density of the tuberculoma than
the brain. The tuberculomas may be hyperintense on
T2 due to the central liquefactive necrosis in these
lesions
• There is usually mass effect. The edema surrounding
the tuberculoma is minimal when compared to that
seen around a pyogenic abscess of a comparable size.
Edema is also more prominently seen during early
stages of granuloma formation
• With gadolinium, they show intense nodular to ring-
like-enhancement
• Healed granulomas may calcify in about 23 percent
cases. Such calcified granulomas are better seen on
CT than MRI. On MRI, calcified granulomas are
appreciated on gradient echo than spin echo
• On resolution of the CNS TB infection, atrophy may
be seen. The full resolution of the tuberculoma may
take months to years of treatment. The duration for
resolution depends on the size of initial lesion.
BIBLIOGRAPHY
1. Gupta RK, Jena A, Sharma A, Guha DK, Khushu S, Gupta
AK. MR imaging of intracranial tuberculomas. J Comput
Assist Tomogr 1988;12(2):280-5.
2. Gupta RK, Pandey R, Khan EM, Mittal P, Gujral RB,
Chhabra DK. Intracranial tuberculomas: MRI signal
intensity correlation with histopathology and localized
proton spectroscopy. Magn Reson Imaging 1993;11(3):
443-9.
3. Kim TK, Chang KH, Kim CJ, Goo JM, Kook MC, Han MH.
Intracranial tuberculoma: Comparison of MR with
pathologic findings. Am J Neuroradiol 1995;16(9):1903-8.
4. Salgado P, Del Brutto OH, Talamas O, Zenteno MA,
Rodriguez-Carbajal J. Intracranial tuberculoma: MR
imaging. Neuroradiol 1989;31(4):299-302.
5. Saxena S, Prakash M, Kumar S, Gupta RK. Comparative
evaluation of magnetization transfer contrast and fluid
attenuated inversion recovery sequences in brain
tuberculoma. Clin Radiol 2005;60(7):787-93.
6. Wasay M, Kheleani BA, Moolani MK, Zaheer J, Pui M,
Hasan S, Muzaffar S, Bakshi R, Sarawari AR. Brain CT
and MRI findings in 100 consecutive patients with
intracranial tuberculoma. J Neuroimaging 2003;13(3):
240-7.
 Chapter 39 Infections 495

A B

Figs 39.6A to C: (A) Axial T2-weighted image, (B) Axial FLAIR and (C) Axial postcontrast T1-weighted image showing a large
rounded T2-hyperintense lesion in the right corona radiata with perilesional edema causing mass effect on the ventricle.
Note: The central hypointense signal in FLAIR indicating necrotic area (arrow). Postcontrast T1-weighted image showing thick
irregular rim enhancement—tuberculoma with central caseation
496 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 39.7A to D: (A) Axial T2-weighted image at the level of the medulla and (B) Axial T2-weighted image at the level of the lateral
ventricles showing multiple rounded T2-hyperintense lesions in the supra- and infratentorial brain parenchyma, (C) Sagittal
postcontrast image and (D) Coronal postcontrast image showing multiple ring and nodular enhancement with patchy enhancement
in the cervical cord—multiple tuberculomas
 Chapter 39 Infections 497

A B

Figs 39.8A and B: (A) Axial postcontrast T1-weighted image and (B) Coronal postcontrast T1-weighted image showing a thick-
walled irregularly peripherally enhancing lesion with central necrosis in the right optic tract region in a patient of tuberculous
meningitis
498 Section 6 Retrochiasmal Visual Pathway Lesions
PROGRESSIVE MULTIFOCAL
LEUKOENCEPHALOPATHY
• It is a demyelinating viral illness caused by reactivation
of latent human papovavirus, JC or a primary exposure
to JC virus in an immunocompromised host
• Focal or confluent areas of demyelination that vary
greatly in size and distributed throughout the CNS
characterize PML.
PATHOLOGY
The following triad is seen:
a. Multifocal demyelination
b. Hyperchromatic enlarged oligodendroglial nuclei that
contain viral inclusion bodies
c. Enlarged bizarre astrocytes with lobulated hyper-
chromatic nuclei.
CLINICAL FEATURES
• Usually presents insidiously in middle-aged
population
• PML is usually seen in immunocompromised
individuals and occurs in patients with HIV (most
common cause), other immune deficiency states,
chronic infectious or granulomatous diseases,
lymphoproliferative disorders, and myeloprolifera-
tive disease
• Patients present with visual deficits (visual loss,
homonymous hemianopia), and mental impairment
(dementia, confusion, personality change)
• Motor weakness occurs eventually.
DIAGNOSIS
• Definitive diagnosis depends upon identification of
the characteristic pathological changes at biopsy
• The presence of JC virus antigen or genomic DNA can
be confirmed but this alone is not diagnostic unless
accompanied by characteristic pathologic changes.
PROGNOSIS
Usually, there is progressive decline and death within a
few months of diagnosis. Rarely, a spontaneously
relapsing-remitting or static course may be seen.
IMAGING
• CT: Diffuse white matter hypodensities in the
posterior optic radiations which usually do not show
any enhancement
• MRI: It is the imaging modality of choice. Clearly
depicts the extent of white matter involvement
• The lesions are bilateral, asymmetric and are usually
seen in subcortical white matter region with
preference for the parietal lobe. The lesions may focal
but may progress rapidly to involve larger areas and
become confluent
• The lesions are hypointense on T1-weighted images
and hyperintense on T2-weighted images. The lack
of enhancement after contrast due to paucity of
perivenous inflammation differentiates PML from
CNS lymphoma
• A small number of cases exhibit gadolinium
enhancement especially around the lesion border.
Hemorrhage, an uncommon feature has also been
reported.
BIBLIOGRAPHY
1. Armand JP, Dousset V, Franconi JM, Huot P, Mieze S,
Lacoste D, Letenneur L, Caille JM. Progressive multi-
focal leukoencephalopathy: Study of the demyelination by
magnetization transfer. J Radiol 1997; 78(2):131-4.
2. Bergui M, Bradac GB, Oguz KK, Boghi A, Geda C, Gatti
G, Schiffer D. Progressive multifocal leukoence-
phalopathy: Diffusion-weighted imaging and pathological
correlations. Neuroradiol 2004;46(1):22-5. Epub 2003
Oct 31.
3. Carrada-Bravo T. Progressive multifocal leukoencephalo-
pathy: Clinical description and demonstration of the causal
agent. Neurologia 2005;20(8):422-5.
4. Carroll BA, Lane B, Norman D, Enzmann D. Diagnosis of
progressive multifocal leukoencephalopathy by computed
tomography. Radiol 1977;122(1):137-41.
5. Guilleux MH, Steiner RE, Young IR. MR imaging in
progressive multifocal leukoencephalopathy. Am J Neuro-
radiol 1986;7(6):1033-5.
6. Hasan MM, Taylor P. Progressive multifocal leuko-
encephalopathy in a case of chronic lymphocytic
leukaemia. Br J Haematol 2005;130(6):808.
7. Huisman TA, Boltshauser E, Martin E, Nadal D. Diffusion
tensor imaging in progressive multifocal leukoence-
phalopathy: Early predictor for demyelination. Am J
Neuroradiol 2005;26(8):2153-6.
8. Kasner SE, Galetta SL, McGowan JC, Grossman RI.
Magnetization transfer imaging in progressive multifocal
leukoencephalopathy. Neurol 1997;48(2):534-6.
 Chapter 39 Infections 499

A B

C D

Figs 39.9A to D: Axial postcontrast CT scan of the brain (A to D) in an HIV-positive patient showing bilateral asymmetric
nonenhancing white matter hypodensities in the frontal and parietal lobes—progressive multifocal leukoencephalopathy
500 Section 6 Retrochiasmal Visual Pathway Lesions
9. Koeppen S, Lehmann HJ. Progressive multifocal leuko-
encephalopathy: Neurological findings and evaluation of
magnetic resonance imaging and computed tomography.
Neurosurg Rev 1987;10(2):127-32.
10. Levy JD, Cottingham KL, Campbell RJ, Moore GK,
Gyorkey F, Ashizawa T, Goldman AM. Progressive
multifocal leukoencephalopathy and magnetic resonance
imaging. Ann Neurol 1986;19(4):399-401.
11. Mathew RM, Murnane M. MRI in PML: Bilateral
medullary lesions. Neurol 2004;63(12):2380.
12. Sullivan JM, Hahn FJ, Adickes E, Hahn PY, Badakhsh S.
Progressive multifocal leukoencephalopathy (PML): CT,
MRI, and histopathology correlation. Nebr Med J 1990;
75(12):324-8.
13. Tartaglione T, Colosimo C, Antinori A, Vecchiet J, Vural M,
Marano P. Progressive multifocal leukoencephalo-pathy
in patients with AIDS: Findings with computerized
tomography and magnetic resonance. Radiol Med (Torino)
1995;90(1-2):8-15.
14. Thurnher MM, Thurnher SA, Muhlbauer B, Hainfellner
JA, Steuer A, Fleischmann D, Trattnig S, Budka H,
Schindler E. Progressive multifocal leukoencephalopathy
in AIDS: Initial and follow-up CT and MRI. Neuroradiol
1997;39(9):611-8.
15. Trotot PM, Vazeux R, Yamashita HK, Sandoz-Tronca C,
Mikol J, Vedrenne C,Thiebaut JB, Gray F, Cikurel M,
Pialoux G, et al. MRI pattern of progressive multifocal
leukoencephalopathy (PML) in AIDS: Pathological
correlations. J Neuroradiol 1990;17(4):233-54.
 Chapter 39 Infections 501

A B

Figs 39.10A and B: Axial FLAIR image (A and B) in a known HIV-positive patient showing bilateral asymmetrical multifocal
frontal and parietal white matter hyperintense areas. Note: The diffuse cerebral atrophy
502 Section 6 Retrochiasmal Visual Pathway Lesions
MYCOTIC ANEURYSMS
• The term mycotic aneurysm is used to describe any
aneurysm that results from an infectious process that
involves the arterial wall
• These may be caused by septic cerebral embolus that
results in inflammatory destruction of the arterial wall
beginning with the endothelial surface
• A more likely explanation is that the infectious
embolus reaches the adventitia through the vasa
vasorum. Inflammation then disrupts the adventitia and
muscularis, resulting in aneurysmal dilatation
• Mycotic aneurysms account for 2 to 3 percent of
all intracranial aneurysms but may increase in
frequency due to increase in drug abuse and immuno-
compromised state
• Intracranial mycotic aneurysms are rare and occur
with greater frequency in children. They are often
found in vessels distal to circle of Willis.
BIBLIOGRAPHY
1. Kannoth S, Thomas SV, Nair S, Sarma PS. Proposed
diagnostic criteria for intracranial infectious aneurysms.
J Neurol Neurosurg Psychiatry 2008;79(8):943-6.
2. Koch P, Desal HA, Auffray-Calvier E, De Kersaint-Gilly A.
Natural history and management of mycotic intracranial
aneurysm. J Neuroradiol 2005;32(4):258-65.
3. Kovoor JM, Jayakumar PN, Srikanth SG, Sampath S.
Intracranial infective aneurysms: Angiographic evaluation
with treatment. Neurol India 2001;49(3):262-6.
 Chapter 39 Infections 503

Figs 39.11A and B: Axial postcontrast CT brain showing

multiple mycotic aneurysms around the circle of Willis—post-
B meningitis. Note: The hydrocephalus
Chapter 40
The term hydrocephalus is used to describe disturbances
in the formation flow or absorption of cerebrospinal fluid
(CSF) resulting in an increase in CSF volume in the brain.
TYPES
• Acute hydrocephalus occurs over days, subacute over
weeks, and chronic over months or years
• Normal pressure hydrocephalus (NPH) is a term
used to describe dilated ventricles with normal CSF
pressure on lumbar puncture (LP) and the absence
of papilledema in patients over 60 years of age
• Communicating hydrocephalus occurs when a
communication exists between the ventricles and
subarachnoid space. It is caused by overproduction
of CSF (rarely), defective absorption of CSF (most
often), or venous drainage insufficiency (occasionally)
• Obstructive hydrocephalus results from obstruction of
the flow of CSF (intraventricular or extraventricular).
Most cases of hydrocephalus are obstructive, and this
term is used to contrast the hydrocephalus caused by
overproduction of CSF.
CAUSES
• Congenital: Stenoses of the aqueduct of Sylvius, Dandy-
Walker malformation, Arnold-Chiari malformation type
1 and type 2, Bickers-Adams syndrome, etc.
• Acquired causes in infants and children: Posterior
fossa mass lesions like tumors (e.g. medulloblastoma,
astrocytoma), cyst, abscess, or hematoma also can be
the cause
• Acquired causes in adults: Tumors, posthemorrhagic
hydrocephalus (head injury, rupture of an aneurysm,
arteriovenous malformation) and infections.
Hydrocephalus
SYMPTOMS AND SIGNS
• Infants: Sunset sign—forced downward eye position
due to compression by dilated third ventricle and
rostral aqueduct on the pretectal structures
• Adults: They present with headache and vomiting or
unilateral or bilateral abducens palsy, and rarely as
divergence palsy, trochlear nerve palsy, and partial
or complete third nerve palsy.
• They can present as dorsal midbrain syndrome due
to compression of quadrigeminal plates by dilated
third ventricle or aqueductal dilatation or following
shunt malfunction. Features include light near
dissociation, gaze paresis, upbeat nystagmus,
convergence retraction nystagmus, upgaze paralysis,
lid retraction, tonic downward deviation of eyes
• They can present as internuclear ophthalmoplegia or
as nystagmus (downbeat, see-saw, ocular flutter) which
may be false localizing signs. Acute hydrocephalus can
present as pretectal pseudobobbing with “V” pattern
convergence nystagmus
• They can present with vision loss due to several
reasons such as chronic papilledema, chiasmal
compression, occipital lobe infarction, amblyopia and
cortical blindness.
RADIOLOGICAL INVESTIGATIONS
• CT scan can assess the size of ventricles and is cost-
effective imaging modality in follow-up of the patients
• MRI is superior to CT to evaluate the cause of
hydrocephalus. MRI can differentiate NPH from
cerebral atrophy by the CSF flow study.
 Chapter 40 Hydrocephalus 505

B C D

Figs 40.1A and B: (A) Schematic diagram to show the anatomy of the ventricular system, (B) Coronal T1-weighted image, (C) Axial
T1-weighted image and (D) Sagittal T1-weighted image showing the imaging anatomy of the ventricular system. The ventricles are
highlighted in yellow

Figs 40.2A and B: Axial CT scans

(A and B) showing gross dilatation of
the lateral ventricles with ballooning
of third ventricle (dotted arrow) and
narrowing at the aqueduct (white
A B arrow)—aqueductal stenosis
506 Section 6 Retrochiasmal Visual Pathway Lesions
CT/MRI CRITERIA FOR ACUTE HYDROCEPHALUS
• Size of both temporal horns is greater than 2 mm, and
the sylvian and interhemispheric fissures effacement.
In the absence of hydrocephalus, the temporal horns
should be barely visible. In degenerative brain disease,
dilatation of the temporal horns is associated with
enlargement of the perihippocampal fissures
• The ratio between the largest width of the frontal horns
and the internal diameter from inner table to inner table
at this level above 0.5 is considered abnormal in
patients
• Ratio of the largest width of the frontal horns to
maximal biparietal diameter (i.e. Evans ratio) is
greater than 30 percent in acute hydrocephalus
• Enlargement of the frontal horns with acute angle
between their medial walls (septal angle). In atrophy,
the septal angle is obtuse
• Transependymal absorption is seen as periventricular
low density
• Ballooning of frontal horns of lateral ventricles and
third ventricle (i.e. Mickey-mouse ventricles) indicate
aqueductal obstruction
• Upward bowing of the corpus callosum on sagittal
MRI indicate acute hydrocephalus
• Sulcal effacement.
CT/MRI CRITERIA FOR CHRONIC HYDROCEPHALUS
• Temporal horns might be less prominent than in acute
hydrocephalus
• Third ventricle may herniate into the sella turcica
• Sella turcica may be eroded
• Macrocrania (i.e. occipitofrontal circumference >98th
percentile)
• Atrophic corpus callosum (best appreciated on sagittal
MRI).
OTHER RADIOLOGICAL INVESTIGATIONS
• Ultrasound through the anterior fontanelle in infants
is useful for evaluating subependymal and intra-
ventricular hemorrhage and in following infants for
possible development of progressive hydrocephalus
• Radionuclide cisternography can be done in NPH to
evaluate the prognosis with regard to possible
shunting. If a late scan (48-72 hours) shows persistence
of ventricular activity, the patient is more likely to
benefit from shunting (75% chance).
CSF FLOW DYNAMICS
• MRI-CSF flow dynamics can help to identify the level
of obstruction and cause of obstruction in certain cases
• Normal pressure hydrocephalus.
BIBLIOGRAPHY
1. Bradley WG JR, Whittemore AR, Watanabe AS, et al.
Association of deep white matter infarction with chronic
communicating hydrocephalus: Implications regarding
the possible origin of normal-pressure hydrocephalus.
AJNR 1991;12:31-9.
2. Cronqvist S. Hydrocephalus and atrophy. Riv di
Neuroradiol 1990;3 (suppl 2):25-8.
3. Joseph VB, Raghuram L, Korah IP, Chacko AG. MR
ventriculography for the study of CSF flow. Am J
Neuroradiol 2003;24(3):373-81.
4. Kilic K, Czorny A, Auque J, Berkman Z. Predicting the
outcome of shunt surgery in normal pressure
hydrocephalus. J Clin Neurosci 2007;14(8):729-36.
5. O'Brien DF, Hayhurst C, Pizer B, Mallucci CL. Outcomes
in patients undergoing single-trajectory endoscopic third
ventriculostomy and endoscopic biopsy for midline
tumors presenting with obstructive hydrocephalus.
J Neurosurg 2006;105(3 Suppl):219-26.
6. Paciorkowski AR, Greenstein RM. When is enlargement
of the subarachnoid spaces not benign? A genetic
perspective. Pediatr Neurol 2007;37(1):1-7. Review.
7. Persson EK, Anderson S, Wiklund LM, Uvebrant P.
Hydrocephalus in children born in 1999-2002:
Epidemiology, outcome and ophthalmological findings.
Child's Nerv Syst 2007;23(10):1111-8.
8. Ramos-Zuñiga R, Jiménez-Guerra R. Rational
management of transient obstructive hydrocephalus
secondary to a cerebellar infarct. Minim Invasive
Neurosurg 2006;49(5):302-4.
9. Tarnaris A, Kitchen ND, Watkins LD. Noninvasive
biomarkers in normal pressure hydrocephalus: Evidence
for the role of neuroimaging. J Neurosurg 2008.
10. Tu YF, Chuang HY, Huang CC, Chuang CC, Wang SM,
Tsai MC, Chi CH, Chuang MC. Frequency and prediction
of abnormal findings on neuroimaging of infants with
bulging anterior fontanelles. Acad Emerg Med 2005;
12(12):1185-90.
 Chapter 40 Hydrocephalus 507

Fig. 40.3: Axial postcontrast CT scan of the brain showing a Fig. 40.4: Axial CT scan of the brain showing multiple ring
well-defined lobulated mass lesion in the body of the lateral enhancing lesions in the posterior fossa causing compression
ventricles with cystic spaces within and causing dilatation of of the fourth ventricle with dilatation of the third and lateral
the lateral ventricle—central neurocytoma ventricles—tuberculomas causing obstructive hydrocephalus

Figs 40.5A and B: Plain axial CT scan

of the brain showing dilated lateral ventri-
cles with periventricular calcification—
A B congenital cytomegalovirus infection
Chapter 41
CEREBRAL INFARCTION
• The word ‘stroke’ describes a clinical condition
characterized by a sudden onset of neurologic deficit
due to impaired blood supply to the brain. It is the third
leading cause of death. Stroke is most commonly
caused by cerebral infarction secondary to arterial
occlusion
• Atherosclerosis: The principal cause of cerebral
infarction is atherosclerosis and its sequelae. It is a
complex multifactorial process and is the underlying
basis for cerebral thromboembolism in over 90 percent
cases. It is also the most common cause of
craniocerebral vascular stenosis in adults
• It affects large, medium and small arteries and
arterioles. It occurs most commonly and most severely
at the internal carotid artery origin and distal basilar
artery
• Carotid bifurcation is most common site in head and
neck region for atherosclerotic plaques. They are
eccentric focal fibrofatty intimal thickenings.
RISK FACTORS
• Family history, older age, hypertension, diabetes
mellitus, smoking, hypercholesterolemia, cardiac
disease and inherited and acquired hypercoagulable
states
• Ischemic stroke accounts for 40 percent of deaths in
elderly
• Cerebral infarcts occur in >75 percent patients with
carotid occlusion
• 90 percent of large, recent cerebral infarcts are caused
by thromboembolic
Vascular Lesions
• Lacunar infarcts correlate with both hypertension and
atherosclerosis.
CEREBRAL ISCHEMIA
• Cerebral ischemia is significantly diminished blood
flow to all parts (global ischemia) or selected areas
(regional or focal ischemia) of the brain. The normal
cerebral blood flow is in the range of 50–55 ml/100 g of
tissue/minute. When the blood flow falls below a critical
level generally below 10–20 ml/100 g of tissue/min
neuronal loss will occur. When the blood flow falls
below 10 ml/100 g of tissue/minute for a long period,
irreversible damage will occur
• Stroke is a dynamic process in which the location and
degree of cerebral infarction change over time.
STROKE—ETIOLOGY AND TYPES
Cerebral Infarction (80%)—Causes
• Large vessel occlusion (ICA, MCA, PCA)
• Small vessel occlusion (lacunar infarcts)
• Cardiac emboli
• Blood disorders
• Nonatheromatous occlusions (vasculitis, vasculo-
pathy).
Primary Intracranial Hemorrhage (15%)—Causes
• Hypertensive bleeds
• Amyloid angiopathy
• Vascular malformation
• Drugs
• Bleeding diathesis.
 Chapter 41 Vascular Lesions 509

Fig. 41.1: Axial noncontrast CT scan of the brain showing a

wedge-shaped hypodensity in the left occipital lobe causing
mass effect on the occipital horns. Areas of hemorrhage noted
within suggestive of hemorrhagic transformation in PCA infarct
(arrow)

A B

Figs 41.2A and B: (A) Axial FLAIR image showing hyperintense signal in the right occipital lobe and (B) Axial T1-weighted showing
subtle hypointensity in the right occipital lobe in a case of left homonymous hemianopia
510 Section 6 Retrochiasmal Visual Pathway Lesions

Nontraumatic Subarachnoid Hemorrhage (5%)— Subacute Infarct

Causes
• Gyral enhancement is seen on postcontrast study
• Aneurysm • Mass effect and edema present.
• Vascular malformation
• Nonaneurysmal SAH. Chronic Infarct
• Contrast enhancement persists
Miscellaneous
• Mass effect resolves
Dural Sinus/Cerebral Vein Occlusion • Transient calcification can occur (pediatric strokes).
The imaging manifestations of cerebral ischemia vary
Months to Years
with time.
Stroke can be categorized based on the time of onset Encephalomalacic change and volume loss.
of symptoms:
• Hyperacute (0–6 hours) MR FINDINGS
• Acute (6–24 hours) • MRI is tool that is being increasingly used in the
• Subacute (24 hours–2 weeks) diagnosis and management of acute ischemic stroke.
• Chronic (over 2 weeks). It has some limitations, such as high cost, long
scanning duration, and decreased sensitivity in the
ROLE OF CT IN ACUTE INFARCTION detection of subarachnoid hemorrhages
The role of immediate CT in the management of acute • Recent advances in MRI, including higher strength
cerebral infarction is to assess the following: of magnetic field (1.5–3.0 tesla field strength) and
1. To diagnose and exclude intracerebral hemorrhage. newer faster sequences has resulted in early diagnosis
2. To rule out any stroke mimics such as—tumor or of infarction and has therefore revolutionized the
vascular malformations. management resulting in reduced morbidity and
mortality.
FINDINGS ON CT SCAN IN INFARCTION VARIES
WITH THE AGE OF INFARCT MRI Techniques—Diffusion-weighted Imaging

Hyperacute Infarct Diffusion-weighted imaging is now an integral part of

cranial imaging and almost mandatory in all patients
• CT may be normal in 50 to 60 percent of the patients suspected to have cerebrovascular disease.
• Hyperdense artery (25–50%)—usually the middle Because of the increased sensitivity of DWI in
cerebral artery-MCA sign—this occurs with cortical identifying hyperacute stroke, it is now considered a gold
and large, deep MCA infarcts standard in stroke imaging, especially hyperacute and
• Loss of gray-white differentiation in the region of acute strokes
infarction. • Numerous studies have shown that ADCs in ischemic
areas are lower by 50 percent or more than those of
Acute Infarct normal brain areas, and they appear as hypointense
• Subtle hypodense area will be seen in the involved areas on the ADC maps. Studies have demonstrated
arterial territory that changes in the ADC occur as early as 10 minutes
• Loss of gray-white interfaces (insular ribbon sign, following onset of ischemia
obscuration of cortex—medullary white matter • Cytotoxic edema appears following sodium/
border) potassium pump failure, which results from energy
• Sulcal effacement. metabolism failure due to ischemic insult; this occurs
within minutes of the onset of ischemia and produces
an increase in brain tissue water of up to 3 to 5 percent.
Early Subacute Infarct
Reduction in intracellular and extracellular water
• Increasing mass effect molecule movement is the presumed explanation for
• Wedge-shaped low density area that involves both the drop in ADC values
gray and white matter • The diffusion of water molecules is guarded by biologic
• Hemorrhagic transformation may occur (basal ganglia barriers in the brain tissue (e.g. cell membranes and
and cortex are common sites). cellular organelles). The behavior of water molecules
 Chapter 41 Vascular Lesions 511

A B

Figs 41.3A to C: A 41-year-old female presented with left

homonymous hemianopia: (A) Axial FLAIR, (B) Coronal T2-
weighted image and (C) Axial T1-weighted image showing the
right PCA territory infarct as hyperintense signal in T2-weighted
and FLAIR image and not well-appreciated on T1-weighted
C image
512 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 41.4A to C: (A) Axial diffusion weighted image showing a

hyperintense signal in the left occipital lobe also seen on,
(B) Axial T2-weighted image and (C) Axial FLAIR image—
subacute left PCA territory infarct. Note: The mass effect on the
C left occipital horn (white arrow)

is not symmetric and may show uneven distribution
of the ADC when measured in one direction; this
uneven distribution may give a false impression of a
lesion
• ADC values are measured in several directions (3, 6,
or more), and ADC maps are created to produce a
direction-insensitive measurement of the diffusion.
When ADC is measured in 6 or more directions, the
diffusion motion of all the water molecules (i.e. ADC
tensor matrix) can be calculated to create what is called
full diffusion tensor mapping, which can also be used
to visualize white matter tracts
• Reduction in the ADC also occurs in other conditions
such as global ischemia, hypoglycemia, and status
epilepticus; it should always be evaluated in relation
to the clinical condition of the patient
• The acute drop in ADC is gradually normalized to
baseline at 5 to 10 days after ischemia (pseudonormali-
zation); helping in some cases to differentiate between
acute, subacute, and chronic lesions
• Normal DWI in patients with stroke-like symptoms
should trigger further investigation for a nonischemic
cause of the symptoms. DWI has been shown to reveal
diffusion abnormalities in almost 50 percent of
patients with clinically-defined transient ischemic
attacks (TIAs); it tends to be of higher yield at
increasing time intervals from the onset of stroke
symptoms.
Lesions bright on diffusion weighted images:
• Acute and subacute ischemic stroke: Usually takes 7 to
14 days for hyperintensity to subside
• Hemorrhagic stroke: Usually bright on T1WI (subacute
hemorrhage)
• Acute multiple sclerosis plaque: Also bright on FLAIR
and T2WI
• Brain abscess: Ring enhancement on contrast MRI
• Choroid plexus: Intraventricular in location, may be
bilateral
• Epidermoid: Extra-axial in location
• Air-bone interface: Commonly bilateral, in the temporal
bone.
Perfusion-weighted Imaging
• The most commonly used technique is bolus-contrast
tracking (other techniques include blood oxygen level
and arterial spin tagging). The imaging is based on
the monitoring of a nondiffusible contrast material
(gadolinium) passing through brain tissue
• The signal intensity declines as contrast material
passes through the infarcted area and returns to
Chapter 41 Vascular Lesions 513
normal as it exits this area. A curve is derived from
this tracing data (i.e. signal washout curve), which
represents and estimates the cerebral blood volume
(CBV)
• An arterial input function can be derived by measu-
ring an artery in lower brain slices or by measuring
gadolinium concentration that is proportional to the
changes in T2 when gadolinium is used at low doses.
The relative cerebral blood flow (CBF) can be
calculated by using the CBV and the arterial input
function. Then, from the central volume theory, the
relative mean transit time (MTT) can be mapped
• DWI and PWI together have been shown to be
superior to conventional MRI both in early phases and
also up to 48 hours after the onset of stroke. Using
both DWI and PWI is very important because together
they provide information about location and extent
of infarction within minutes of onset; when performed
in series, they can provide information about the
pattern of evolution of the ischemic lesion. This
information may be of great importance in choosing
the appropriate treatment modality as well as in
predicting outcome and prognosis
• The diffusion-perfusion mismatch, i.e. the difference
in size between lesions captured by DWI and PWI,
usually represents the ischemic penumbra which is
the region of incomplete ischemia that lies next to the
core of the infarction. The ischemic penumbra is
regarded as an area that is viable but at risk of
ischemia; it can be saved if appropriate intervention
is promptly instituted
• MRI still has some limitations in its application,
namely, in patients with metal implants and acutely
ill patients requiring close monitoring
• These new techniques, DWI and PWI, together
represent the most exciting areas in MRI for their
potential ability to detect early changes (i.e. within
minutes of the stroke). They are currently used in the
evaluation of thrombolytic and neuroprotective
therapy in acute stroke clinical trials.
MRI Techniques—Magnetic Resonance
Spectroscopy
• Magnetic resonance spectroscopy (MRS) is one of the
recent advances in MR technology; it evaluates meta-
bolic activity and concentration of certain metabolites
in specified areas of the brain. Proton and phosphorus
spectroscopic studies have been performed
• In proton spectroscopy, depression of N-acetylaspartate,
which is considered to be a neuronal marker is the most
consistent finding in acute stroke. This depression may
514 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 41.5A to D: A 6-year-old boy with decreased vision in the right eye: (A) Axial T1-weighted image, (B) Axial T2-weighted
image, (C) Axial FLAIR showing a T1-hypointense and T2-hyperintense altered signal in the left occipital lobe suggestive of
chronic PCA territory infarct and (D) Axial MIP angiogram showing abrupt narrowing of the P2 segment of the left PCA and right
posterior communicating artery continuing as posterior cerebral artery—fetal PCA

occur within hours after the onset of stroke and
continues through the subacute and chronic phases of
the stroke, presumably because of loss of neurons
• Increase in levels of lactate is another important finding
and has been attributed to anaerobic metabolism in
ischemic tissue. Initial studies of other metabolites, such
as choline and creatine, demonstrated decrease in their
levels in acute stroke
• Phosphorus spectroscopy provides information about
energy metabolism and pH, depletion of ATP,
decrease of tissue pH, and increase of the ratio of
inorganic phosphate to phosphocreatine, which has
been reported in both human and animal studies
• Long acquisition times, weak signal, and low spatial
resolution of this technique have limited enthusiasm for
its use in the clinical management of cerebral ischemia.
Magnetic Resonance Angiography
• Magnetic resonance angiography (MRA) is now a part
of the stroke imaging protocol
• MRA technique should be selected appropriately to
identify slow flow distal to the stenotic segment.
Contrast-enhanced MRA is useful in demonstrating
slow flow distal to the stenotic segment, which may
not be detected on the routine MRA studies.
MR FINDINGS IN DIFFERENT STAGES OF STROKE
Immediate
• Absence of normal flow void in the thrombosed artery
• Intravascular contrast enhancement
• Low apparent diffusion coefficients (ADCs)
• Perfusion alterations.
<12 Hours
• Anatomic alterations on T1-weighted images
• Sulcal effacement
• Gyral edema
• Loss of gray-white interfaces.
12 to 24 Hours
• Hyperintensity on T2-weighted images
• Meningeal enhancement adjacent to infarct
• Mass effect.
1 to 3 Days
• Intravascular meningeal enhancement begin decreasing
Chapter 41 Vascular Lesions 515
• Early parenchymal contrast enhancement
• Signal abnormalities striking on T1 and T2 images
• Hemorrhagic transformation may become evident.
4 to 7 Days
• Striking parenchymal contrast enhancement
• Hemorrhage apparent in 25 percent
• Mass effect, edema begin diminishing
• Intravascular, meningeal enhancement disappear.
1 to 8 Weeks
• Contrast enhancement often persists
• Mass effect resolves
• Decrease in abnormal signal on T2-weighted images
sometimes noted (fogging effect)
• Hemorrhagic changes evolve, become chronic.
Months to Years
• Encephalomalacia ensues, volume loss occurs in
affected vascular distribution
• Hemorrhagic residua is seen on gradient imaging
(hemosiderin).
Middle Cerebral Artery (MCA) Infarct
Middle cerebral artery infarcts which is seen in 75 percent
cases of stroke typically shows a wedge-shaped area that
extends from the lateral ventricle to the brain surface and
involves the entire MCA distribution namely the basal
ganglia, deep cerebral white matter, and much of
hemispheric cortex. Sometimes, only the anterior or
posterior division may be involved.
Posterior Cerebral Artery (PCA) Infarct
• Posterior cerebral artery—middle cerebral artery
boundaries are variable particularly in the basal
regions
• In most cases, the PCA supplies the posterior 1/3rd of
the convexity and most of the inferior temporal lobe.
It also supplies the occipital lobe and participates in
supplying the posterior limb of internal capsule and
other deep structures
• Hemispheric PCA infarcts are second in frequency to
MCA occlusions. Common sites are the calcarine
cortex and deep penetrating branches to the thalami,
midbrain and posterior limb of internal capsule.
516 Section 6 Retrochiasmal Visual Pathway Lesions

Figs 41.6A and B: Axial FLAIR images showing well-

demarcated wedge-shaped hyperintense signal in the right
temporal (MCA territory) and right occipital lobe (PCA territory)—
B suggestive of acute infarcts
 Chapter 41 Vascular Lesions 517

Figs 41.7A and B: A 52-year-old male with history of sudden

loss of vision in the left eye: (A) Axial STIR image at the level of
the cavernous sinus showing absent flow void in the left
cavernous ICA with hyperintense signal and (B) Coronal MIP
image of a 2D TOF angiogram showing no flow in the left internal
carotid artery and irregular narrowing of the left middle cerebral
B artery
518 Section 6 Retrochiasmal Visual Pathway Lesions
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HEMORRHAGIC INFARCTION OF BRAIN
• It comprises a pathologic spectrum that ranges from
petechial hemorrhages to frank parenchymal
hematoma
• It can be arterial or venous, cortical or deep,
microscopic or gross.
ARTERIAL INFARCTION
• Hemorrhage almost never occurs as a primary
manifestation of arterial infarction but occurs as a
hemorrhagic transformation of initially ischemic lesions
• Cerebral arterial embolus leads to ischemic insult to
the brain parenchyma and vascular endothelium.
Subsequently, when the clot is disrupted and
circulation is restored, the damaged endothelium
permits blood to extravasate into the previously
ischemic or infarcted parenchyma. This leads to
hemorrhagic transformation
• Hemorrhagic infarction (HI) can occur almost
anywhere in the brain but has predilection to basal
ganglia and cortex. Deep hemorrhagic infarctions are
often associated with proximal middle cerebral artery
occlusion.
IMAGING
CT Scan
• CT scan shows hemorrhagic infarction in 5 to 15
percent of stroke cases. HI is relatively common in
patients with embolic than thrombotic occlusions
• 25 percent of large infarctions show hemorrhagic
transformation
• Hemorrhagic infarction is best identified 24 to 48
hours after the event
• Delayed hemorrhagic infarction may develop if
collateral blood supply through pial arteries increases
following reduction in edema and mass effect
• High density foci within previously ischemic areas
suggest HI.
MRI
• Findings are variable
• Acute HI produces foci of mild cortical low intensity
on T2- and T2*-weighted images. This may be
surrounded by high signal edema
• Subacute hemorrhagic infarcts show hyperintense
signal on T1 and T2-weighted images.
Chapter 41 Vascular Lesions 519
• Chronic hemorrhagic infarcts show hypointense
signal on T2-weighted.
Venous Infarction
• This occurs in association with occlusion of a major
dural sinus. It preferentially affects the white matter
than the cortex
• Venous sinus thrombosis can occur due to trauma,
tumor, infection, hypercoagulable states, pregnancy
and dehydration
• Abrupt occlusion of several cerebral veins or dural
sinuses is necessary to produce a large hemorrhagic
infarct. Slowly progressive venous or dural sinus
occlusion rarely results in tissue necrosis.
FEATURES OF VENOUS SINUS
THROMBOSIS—IMAGING
CT
• The classic finding on a noncontrast scan is the delta
sign, i.e. a triangular hyperdense lesion within the
superior sagittal sinus created by the thrombus.
Another common site of affection is the transverse
sinus. The infarct is seen as patchy foci of edema or
petechial hemorrhage on plain CT
• The empty delta or reverse delta sign is seen on
contrast-enhanced CT scan. The dura around the
affected sinus enhances intensely and the thrombus
appears hypodense. But this sign may not be typical
and is reported less often.
MRI
• MRI in conjunction with MR venogram is now
considered the best noninvasive method of
diagnosing cerebral venous sinus thrombosis. The
diagnosis can usually be made without intravenous
contrast
• Nonconfluent corticomedullary junction hemorrhagic
foci with variable edema may be seen
• Venous infarcts are not in the arterial territory. They
usually involve the white matter. Hemorrhage is a
common feature seen at the initial presentation
• The normal flow void signal of the dural venous
sinuses is replaced by T1 isointense or hyperintense
signal depending on the age of the thrombus
• MRV will confirm the thrombosis. The thrombus will
be seen as a filling defect or as no flow in the involved
sinus.
520 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 41.8A to D: (A and B) Axial T1-weighted image showing extensive wedge-shaped area of mixed T1-hyperintense and
hypointense signal suggestive of hemorrhagic infarct, (C) Coronal T2-weighted image and (D) Axial FLAIR showing heterogeneous
hyperintense signal and the extent of the infarct. Note: The mass effect on the ventricles and midline shift with contralateral
ventricular dilatation
 Chapter 41 Vascular Lesions 521

A B

C D

Figs 41.9A to D: A 20-year-old male presented with severe headache and papilledema: (A) Axial T1-weighted image, (B) Axial
gradient image showing an area of hemorrhage in the left temporal lobe displaying patchy T1-hyperintense signal and blooming
on gradient image and absence of normal flow void signal in the superior sagittal sinus (white arrow)—suggestive of superior
sagittal sinus thrombosis with hemorrhagic venous infarct, (C) Sagittal MIP image of 3D tetralogy of fallot (TOF) venogram
showing no flow in the major portion of the superior sagittal sinus (black arrow) and (D) Follow-up CT scan 3 days later showing
the hemorrhagic venous infarct in the left temporal lobe (arrow)
522 Section 6 Retrochiasmal Visual Pathway Lesions
HYPERTENSIVE INTRACEREBRAL HEMORRHAGE
It is the most common nontraumatic cause of intracranial
hemorrhage in adults and is a significant cause of
morbidity and mortality. Another manifestation of
hypertension is hypertensive encephalopathy.
INCIDENCE
Most nontraumatic spontaneous intracerebral hemorrhage
in elderly patients is associated with hypertension. In some
cases, this occurs from ruptured microaneurysms.
LOCATION
Hypertensive intracerebral hemorrhage (HICH) has a
predilection for areas supplied by penetrating branches of
middle cerebral and basilar arteries. The two-thirds occur
in the basal ganglia. Large hematomas can extend beyond
the putamen to include globus pallidus and internal
capsule. Clot can dissect into the ventricular system and
has poor prognosis when 4th ventricle is involved.
SITES
• Putamen/external capsule
• Thalamus
• Pons
• Cerebellum—originate near the dentate nucleus along
the perforating branches of the superior cerebellar or
posterior inferior cerebellar arteries
• Subcortical white matter
• Midbrain, medulla (rare)
• Spinal cord (rare).
Lobar white matter hemorrhages are seen in 15 to 20
percent cases.
FEATURES
• Active bleeding in hypertensive intracerebral
hemorrhage lasts less than an hour and is followed
by cerebral edema which progresses for 24 to 48 hours
• It has a variable clinical course-death can occur within
48 hours in 25 percent patients
• Delayed neurological deterioration can occur due to
clot expansion with secondary brain herniation in
some cases
• Delayed hemorrhage occurs in some due to persistent
hypertension.
IMAGING
CT Scan
• In acute hyperintensive hematomas, high density
blood will be seen in the locations mentioned above,
viz basal ganglia, thalami, pons or cerebellum
• With contrast subacute hematomas show a peripheral
ring enhancement
• In late stages round or slit like cavities, particularly
in the putamen and external capsule may be seen
• Calcification is uncommon.
MRI
Signal intensity will vary depending on the age of blood
as mentioned earlier.
HYPERTENSIVE ENCEPHALOPATHY
Hypertensive encephalopathy occurs in patients with
markedly elevated blood pressure.
Symptoms
Rapidly progressing symptoms and signs may be noted
such as headache, seizures, visual disturbances, altered
mental status, focal neurologic signs.
Causes
• Pre-eclampsia/eclampsia (most common)
• Chronic renal failure
• Thrombotic thrombocytopenic purpura
• Hemolytic-uremic syndrome
• Systemic lupus erythematosus.
 Chapter 41 Vascular Lesions 523

A B

Figs 41.10A to C: (A) Axial T1-weighted image, (B) Axial T2-

weighted image showing T1-peripheral hyperintense with
central low signal on both T1- and T2-weighted image
suggestive of peripheral subacute hemorrhage
(methemoglobin) with central acute hemorrhage
(deoxyhemoglobin) and (C) Axial T1-weighted image follow-
up scan done one month later shows homogeneous
hyperintense signal suggestive of extracellular methemoglobin
C with reduction in the size of the hemorrhage
524 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 41.11A to C: An 8-year-old male presented with proptosis

of the left eye with congestion and chemosis: (A) Axial FLAIR
image, (B) Sagittal FLAIR image of the brain showing multiple
hyperintense lesions in the cerebellum and frontal and parieto-
occipital subcortical and deep white matter and (C) Axial
postcontrast T1 fat-suppressed image of the orbit showing
proptosis of the left eye, thickened occular coats and periocular
and intraocular hemorrhages—a diagnosis of posterior
reversible encephalopathy syndrome was made. Subsequently,
C the child was noted to have a blood pressure of 210/140 mm Hg

INTRACRANIAL HEMORRHAGE
• Intracranial hemorrhage (ICH) is a common cause of
acute neurologic deterioration and a frequent
indication for emergent neuroimaging
• ICH is commonly arterial in origin
• Sites
– From primary arterial sites
– Smaller vessels around expanding hematoma
margin
– Cortical veins
– Dural venous sinuses.
CT SCAN IN ACUTE HEMORRHAGE
• The electron density determines image contrast. There
is linear relationship between CT attenuation and
hematocrit, hemoglobin concentration and protein
content. As the hematocrit of an acute retracted clot
is around 90 percent and the globin (protein)
component has a high mass density, fresh intra-
cerebral blood clots typically appear hyperdense on
CT when compared to normal brain. The contribution
from iron, calcium and protoporphyrin is negligible
• Appearance of acute blood clot depends upon its
density, volume, and relationship to surrounding
structures. The appearance also varies with slice
thickness, window width and scan angle. Small
petechial hemorrhages or thin linear clots close to
calvarium are best detected with window widths
between 150 and 250
• Density of a clot on CT scan is independent of the
location unless blood is mixed with other fluids, e.g.
cerebrospinal fluid
• Acute epidural and subdural hematoma,
subarachnoid hemorrhage and parenchymal clots are
all hyperdense to brain. Unretracted semiliquid clot
seen with rapidly accumulating blood demonstrates
the so, called swirl sign, i.e. hypodense areas within
the generally hyperdense acute hematoma. Contrast
extravasation is seen, if given during brisk ongoing
hemorrhage.
ATYPICAL CT APPEARANCE IN ACUTE
HEMORRHAGE
• Acute hematomas can be isodense with low
hematocrit as seen in anemia and with abnormal
clotting function leading to failure of clot retraction
• In iatrogenic ICH following thrombolytic therapy, low
density clot with fluid-fluid levels might be apparent.
Chapter 41 Vascular Lesions 525
SUBACUTE HEMORRHAGE
• Early subacute phase begins within few days after the
hemorrhage. Oxidative denaturation of hemoglobin
progresses and deoxyhemoglobin is converted to
methemoglobin. Because the blood clot interior is
profoundly hypoxic, these changes first occur around
periphery and then progress centrally
• Late subacute stage begins after a week. Oxidation of
hemoglobin and cell lysis in the periphery is initiated.
The shrunken RBCs gradually lyse and release
methemoglobin into the extravascular space. Edema
subsides and mass effect reduces. Secondary reactive
changes start in the brain around the clot and are
responsible for the ring enhancement seen on CT and
MR with contrast.
CT SCAN IN SUBACUTE HEMORRHAGE
• The attenuation of the uncomplicated hematoma
decreases at the rate of 1.5 HU per day
• Between 1 and 6 weeks, the subacute hematoma
appears isodense with brain and shows peripheral rim
enhancement with contrast as, there is blood-brain
barrier breakdown in the vascularized capsule that
surrounds the hematoma.
EARLY CHRONIC HEMORRHAGE
• White matter edema surrounding the hematoma
disappears as inflammation regresses
• Vascular proliferation encroaches on the hematoma
cavity causing gradual reduction in its size. Peripheral
reactive astrocytosis becomes pronounced
• At this stage, the clot contains a uniform pool of dilute
extracellular methemoglobin surrounded by a
vascularized wall with activated macrophages. These
macrophages contain 2 iron storage substances—
ferritin and hemosiderin.
LATE CHRONIC HEMORRHAGE
• Chronic hematomas are cystic or slit-like cavities with
a surrounding dense collagenous capsule. Eventually,
this is replaced by a vascularized fibrotic matrix with
ferritin and hemosiderin-laden macrophages
• While the hematoma cavity completely disappears in
children, a permanent residual small scar is often seen
in adults.
526 Section 6 Retrochiasmal Visual Pathway Lesions
CT SCAN IN CHRONIC HEMORRHAGE
• Chronic hematomas are hypodense to brain and high
attenuation within them suggests rebleeding
• Sometimes, a hyperdense area within a low density
collection or fluid-fluid level may be seen
• Rim enhancement occurs around a resolving
hematoma within a few days and disappears by 2 to
6 months
• A target sign is seen on postcontrast CT, if rebleeding
occurs within a resolving hematoma
• Residua of ICH includes slit-like lesions, calcification
and low attenuation foci. Only one percent lesions do
not show any residua.
MRI FACTORS INFLUENCING MR APPEARANCE
OF INTRACRANIAL HEMORRHAGE
• Intrinsic
– Macroscopic structure of clot
– Hemoglobin oxidation state
– Red blood cell morphology
– Protein concentration/hydration
– Size/location of hematoma
– Edema
• Extrinsic
– Pulse sequence
– Field strength.
CLOT AGE AND BLOOD DEGRADATION
PRODUCTS
The relaxivity and susceptibility effects of iron containing
hemoglobin are among the many important factors that
determine signal intensity of intracranial hemorrhage.
Hyperacute Clots and MRI
• Hyperacute clots contain oxyhemoglobin for a few
minutes to hours and this does not affect the T1 or T2
relaxation time. The signal is mainly due to the protein—
rich water content
• Hyperacute clots are typically isointense with gray
matter on T1 and hyperintense on T2.
Acute Clots and MRI
• Hemoglobin desaturation from oxyhemoglobin to
deoxyhemoglobin occurs within few hours and this
deoxyhemoglobin is strongly paramagnetic
• The deoxyhemoglobin appears isointense with brain
parenchyma on T1 regardless of whether it is intra-
or extracellular
• Acute clots with deoxyhemoglobin typically appear
moderately hypointense on balanced scans and
profoundly hypointense on T2 or gradient-refocused
sequences.
Subacute Clots and Methemoglobin (MethHb)
and MRI
• In the early subacute stage, MethHb is contained
within intact RBCs. This T2 shortening results in low
signal on long TR/short TE (proton density-weighted)
scans
• Early subacute clots are profoundly hypointense on
T2-weighted images and gradient refocused
sequences.
Late Subacute Stage and MRI
Hemolysis results in the accumulation of extracellular
MethHb within the hematoma cavity which in free solution
is hyperintense on T1- and T2-weighted images.
Chronic Clots and MRI
• In the early chronic stage, clots are typically homo-
geneously hyperintense on both T1 and T2 and have
a pronounced low signal rim on T2 sequences. Edema
and mass effect gradually disappear
• In late chronic stage, macrophages laden with iron
storage products, viz hemosiderin and ferritin remain
around the margins of clot for years together in adults.
In contrast, the iron storage products are removed in
infants
• Hemosiderin because of strong magnetic
susceptibility effects, ferritin and hemosiderin appear
profoundly hypointense on gradient-echo studies.
BIBLIOGRAPHY
1. Barkovich AJ, Atlas SW. Magnetic resonance imaging of
intracranial hemorrhage. Radiol Clin North Am
1988;26(4):801-20. Review.
2. Hamon M, Leclerc X, Oppenheim C, Gauvrit JY, Meder
JF, Pruvo JP. Neuroimaging characteristics of intracerebral
hematoma. Rev Neurol (Paris) 2005;161(10):997-1006.
Review.
3. Kidwell CS, Chalela JA, Saver JL, Starkman S, Hill MD,
Demchuk AM, Butman JA, Patronas N, Alger JR, Latour
LL, Luby ML, Baird AE, Leary MC, Tremwel M, Ovbiagele
B, Fredieu A, Suzuki S, Villablanca JP, Davis S, Dunn B,
Todd JW, Ezzeddine MA, Haymore J, Lynch JK, Davis L,
Warach S. Comparison of MRI and CT for detection of acute
intracerebral hemorrhage. JAMA 2004;292(15):1823-30.
 Chapter 41 Vascular Lesions 527

A B

Figs 41.12A to C: A 65-year-old male with left homo-

nymous hemianopia: (A) Axial T2-weighted image,
(B) Coronal FLAIR and (C) Axial T1-weighted image
showing a well-defined oval lesion in the right occipital lobe
displaying slight T1-hyperintensity and markedly hypo-
intense in T2-weighted image with perilesional edema and
mass effect on the occipital horn—acute to early subacute
C hematoma
528 Section 6 Retrochiasmal Visual Pathway Lesions
4. Leclerc X, Khalil C, Silvera S, Gauvrit JY, Bracard S, Meder
JF, Pruvo JP. Imaging of non-traumatic intracerebral
hematoma. J Neuroradiol 2003;30(5):303-16. Review.
5. Linfante I, Linas RH, Caplan LR, Warach S. MRI features
of intracerebral hemorrhage within 2 hours from symptom
onset. Stroke 1999;30(11):2263-7.
6. Murai Y, Ikeda Y, Teramoto A, Tsuji Y. Magnetic
resonance imaging-documented extravasation as an
indicator of acute hypertensive intracerebral hemorrhage.
J Neurosurg 1998;88(4):650-5.
7. Parizel PM, Makkat S, Van Miert E, Van Goethem JW,
van den Hauwe L, De Schepper AM. Intracranial
hemorrhage: Principles of CT and MRI interpretation. Eur
Radiol 2001;11(9):1770-83. Review.
8. Salzman C, Tuazon CU.Value of the ring-enhancing sign
in differentiating intracerebral hematomas and brain
abscesses. Arch Intern Med 1987;147(5):951-2.
9. Seidl Z, Obenberger J, Vitak T. Magnetic resonance
imaging of intracerebral hemorrhage. Cas Lek Cesk.
1997;136(24):752-7.
10. Tung GA, Julius BD, Rogg JM. MRI of intracerebral
hematoma: Value of vasogenic edema ratio for predicting
the cause. Neuroradiol 2003;45(6):357-62.
11. Weisberg L. Multiple spontaneous intracerebral hema-
tomas: Clinical and computed tomographic correlations.
Neurol 1981;31(7):897-900.
12. Yamamoto T, Muraki M, Oishi H, Uemura K. Contrast-
enhanced MRI of hypertensive intracerebral hemorrhage
in the peracute stage-extravasation of contrast medium
as detector of growing hematoma. Nippon Rinsho 1993;
(51 Suppl):48-54.
13. Young N, Vladica P, Soo YS, Ho D. Acute intracerebral
hematomas: Assessment for possible underlying cause
with MRI scanning. Australas Radiol 1993;37(4):315-20.
 Chapter 41 Vascular Lesions 529

Fig. 41.13: Axial CT scan of the brain at the level of the thalamus
showing a hyperdense intracerebral hematoma in the right
thalamocapsular region in a known hypertensive patient
presenting with left hemiparesis
530 Section 6 Retrochiasmal Visual Pathway Lesions
CENTRAL NERVOUS SYSTEM (CNS)—VASCULITIS
INTRODUCTION
• Vasculitis confined to the brain, the spinal cord and
meninges is referred to as CNS vasculitis. It is a rare
and one of the most poorly understood forms of
vascular inflammatory disease
• It usually involves the small vessels of the parenchyma
and leptomeninges.
CLASSIFICATION
Clinical Classification
• Primary: When there is no other disease or condition
present that may cause blood vessels to be damaged
it is termed as primary CNS vasculitis. This type is
far more clinically challenging and less well
understood. Few distinguishable but rarer forms
primary CNS vasculitis include Susac and Cogan
syndromes and Eale’s disease
• Secondary: Vasculitis as a part of infectious diseases,
drug exposure (amphetamines, cocaine, etc.),
autoimmune disorders (Wegener’s granulomatosis,
polyarteritis nodosa, etc.), and connective tissue
diseases, systemic lupus erythematosus (SLE),
Sjögren’s syndrome, etc. that may involve the CNS is
termed as secondary CNS vasculitis.
Giant Cell Arteritis
• Over 50-year-old
• Present with headache, abrupt visual loss, jaw
claudication and temporal artery tenderness or
pulsation
• Elevated ESR and positive CRP
• TIA and strokes mainly involve vertebral territory,
less commonly carotid artery.
Polyarteritis Nodosa
• Generalized necrotizing vasculitis of small and
medium-sized arteries. Affects predominantly heart,
kidneys and gastrointestinal tracts
• CNS involvement is uncommon but cortical and
subcortical strokes have been described.
Pathological Classification
Most recently, CNS vasculitis has been classified into two
major subcategories:
1. Primary granulomatous angiitis of the CNS (GACNS)
2. Benign angiopathy of the CNS (BACNS): It is the milder
form of disease and also referred to as reversible
vasoconstrictive (vessel spasm) disease.
DIAGNOSIS
• Perhaps no other form of vasculitis is as difficult to
diagnose as CNS vasculitis. Blood investigations, analysis
of cerebral spinal fluid, CT scan or MRI. All of these tests
can be useful, but they may not sufficiently separate CNS
vasculitis from other forms of neurological disease
• CT scan is less sensitive in detecting the cerebral
ischemia
• MRI is sensitive in identifying the cerebral lesions but
lacks specificity. Most patients of CNS vasculitis would
have a positive MRI. The lesions are typically small and
seen on long TR images and are located in the cortical
gray matter, subcortical white matter, deep white matter,
deep gray matter and cerebellum. The MCA distribution
is more affected than the ACA or PCA or vertebral
territories. Diffusion and perfusion imaging has been
found to sensitive in detecting areas of cerebral
hypoperfusion not seen on conventional MR imaging
• Subarachnoid hemorrhage is an unusual finding in
vasculitis
• Cerebral angiography is considered positive for CNS
vasculitis, if focal or diffuse arterial stenosis,
occlusions, dilatation and beading is detected. The
resolution of angiography cannot detect the
predominant small-vessel disease involved in CNS
vasculitis. It can detect disease in the medium to large
arteries which occurs less frequently in CNS vasculitis.
A positive angiogram indicates CNS vasculitis but
negative angiogram does not exclude the disease
• Biopsy of the brain is the most definitive means of
making the diagnosis of CNS vasculitis. But obviously
because of the invasiveness involved, is the least resorted
technique.
 Chapter 41 Vascular Lesions 531

A B

C D

Figs 41.14A to D: A 32-year-old male patient presented with loss of vision in the left eye: (A and B) Axial FLAIR images showing
patchy T2-hyperintense areas in the left frontal and parietal cortical and subcortical region, (C) Coronal postcontrast image and
(D) Axial postcontrast image showing punctuate and linear enhancement in the parietal region
532 Section 6 Retrochiasmal Visual Pathway Lesions
BIBLIOGRAPHY
1. Abreu MR, Jakosky A, Folgerini M, Brenol JC, Xavier RM,
Kapczinsky F. Neuropsychiatric systemic lupus
erythematosus: Correlation of brain MR imaging, CT, and
SPECT. Clin Imaging 2005;29(3):215-21.
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M, Peltola J, Hietaharju A. Cerebral MRI abnormalities
and their association with neuropsychiatric manifestations
in SLE: A population-based study. Scand J Rheumatol
2005;34(5):376-82.
3. Arroyo HA, Russo RA, Rugilo C. Cerebral vasculitis. Rev
Neurol 2006;42(3):176-86.
4. Aviv RI, Benseler SM, Silverman ED, Tyrrell PN,
Deveber G, Tsang LM, Armstrong D. MR imaging and
angiography of primary CNS vasculitis of childhood.
Am J Neuroradiol 2006;27(1):192-9.
5. MacLaren K, Gillespie J, Shrestha S, Neary D, Ballardie FW.
Primary angiitis of the central nervous system: Emerging
variants. QJM 2005;98(9):643-54. Epub 2005 Jul 22.
6. Moritani T, Hiwatashi A, Shrier DA, Wang HZ,
Numaguchi Y, Westesson PL. CNS vasculitis and
vasculopathy: Efficacy and usefulness of diffusion-
weighted echoplanar MR imaging. Clin Imaging 2004;
28(4):261-70.
7. Peterson PL, Axford JS, Isenberg D. Imaging in CNS lupus.
Best Pract Res Clin Rheumatol 2005;19(5):727-39. Review.
8. Schmidt WA. Use of imaging studies in the diagnosis of
vasculitis. Curr Rheumatol Rep 2004;6(3):203-11. Review.
9. Zhang X, Zhu Z, Zhang F, Shu H, Li F, Dong Y. Diagnostic
value of single-photon-emission computed tomography
in severe central nervous system involvement of systemic
lupus erythematosus: A case-control study. Arthritis
Rheum 2005;53(6):845-9.
 Chapter 41 Vascular Lesions 533

A B

C D

Figs 41.15A to D: Follow-up study done 6 months later: (A) Axial T1-weighted image at the level of the cavernous sinus shows
absence of normal flow void signal in the left ICA with hyperintense signal (arrow), (B and C) Axial T2-weighted images showing
infarcts in the left gangliocapsular region (white arrow), left corona radiate and parietal region (black arrow) and (D) Anteroposterior
view of a two-dimensional phase contrast MRA showing no flow in the left internal carotid artery. The left MCA is supplied by the
left posterior cerebral artery (PCA) via the posterior communication artery (PCOM) (arrow)
534 Section 6 Retrochiasmal Visual Pathway Lesions

Figs 41.16A and B: A 40-year-old female—a known case of

SLE with history of diplopia: (A) Axial FLAIR image and
(B) Coronal FLAIR image showing hyperintense signal in the
ventral midbrain (arrows) with diffuse cerebral atrophy—
B suggestive of vasculitis
 Chapter 41 Vascular Lesions 535

A B

Figs 41.17A to C: A 20-year-old male with retinal vasculitis:

(A and B) Axial FLAIR at the level of the internal capsule and
pons respectively and (C) Coronal T2-weighted images showing
multiple patchy hyperintense areas in the pons (black arrow),
thalami (arrowhead) and internal capsules suggestive of CNS
C vasculitis. Note: Retinal detachment in the left eye
536 Section 6 Retrochiasmal Visual Pathway Lesions
INTRACRANIAL VASCULAR MALFORMATIONS
Intracranial vascular malformations include the
following:
1. Arteriovenous malformations.
2. Cavernous angioma.
3. Venous malformations.
4. Capillary telangiectasia.
ARTERIOVENOUS MALFORMATIONS (AVMS)
They are divided into following types:
1. Brain parenchymal malformations (pial).
2. Dural malformations.
3. Mixed pial-dural AVM.
We would limit our discussion to pial AVMs.
Parenchymal AVMs (Pial)
• AVMs are congenital lesions and contain a complex
network of abnormal vascular channels that consists
of arterial feeders, arterial collaterals, AVM nidus and
enlarged venous outflow channels
• Grossly, they appear as tightly packed masses of
abnormal vascular channels without intervening
normal brain parenchyma. They may contain gliotic
brain and hemorrhagic residua. Vascular steal
phenomenon may cause ischemic and atrophic
changes in the adjacent brain
• Flow-related aneurysms on feeding vessels or within
the AVM nidus itself are seen in 8 to 12 percent cases.
There is no intervening capillary bed in AVMs. Hence,
blood is directly shunted from enlarged feeding
arteries to dilated veins
• Degenerative angiopathic changes such as thrombosis
and stenosis of feeding arteries and draining veins
may be seen.
Location
• The eighty-five percent are supratentorial and are
found in the cerebral hemispheres. Five percent occur
in the posterior fossa
• The typical pial AVM extends from subpial surface
of the brain through the cortex and underlying white
matter.
Incidence/Demography
• Ninty-eight percent lesions are solitary
• Multiple AVMs are seen in Wyburn-Mason and
Rendu-Osler-Weber syndrome
• Both sexes can be affected. Common age of
presentation is between 20 and 40 years and majority
become symptomatic by 50 years.
Manifestations
• Hemorrhage (50%)
• Seizures (25%)
• Mass effect
• Headaches
• Vascular steal phenomenon
• Focal neurological deficits
• Risk of hemorrhage from parenchymal AVM is 2 to 4
percent per year. Each hemorrhagic episode carries
30 percent risk of death and 25 percent risk of long-
term morbidity.
IMAGING
Cerebral Angiography
It is the gold standard for imaging AVM.
Patent AVM
• Parenchymal AVMs appear as tightly packed masses
of enlarged feeding arteries and dilated, tortuous
draining veins with little or no intervening brain
parenchyma
• In the arterial phase of angiogram, AVM appears as a
wedge-shaped lesion with a broad, cortically-based
mass of tangled vessels
• Uncomplicated AVMs have minimal or no mass effect.
Arteriovenous shunting with abnormal early filling
of veins that drain the lesion is characteristic but not
pathognomonic.
Thrombosed AVM
The angiogram may be normal, if the AVM is completely
thrombosed or may show a minimal avascular mass
effect. Very subtle AV shunting may be noted.
Occasionally, arteries with slow blood flow visible into
the late arterial or capillary phases called stagnating
arteries may be seen.
CT Scan
• Patent AVMs are iso- to hyperdense on plain CT.
Intense serpentine enhancement may be seen with
contrast. Sometimes, small AVMs are identified only
after contrast
 Chapter 41 Vascular Lesions 537

A B

Figs 41.18A and B: (A) Axial plain CT scan of the brain showing a hyperdense lesion with calcification in the right frontal lobe
(arrow) and (B) Axial postcontrast CT scan showing multiple dilated tortuous serpiginous vessels in the right frontoparietal lobe
(arrow). Note: The craniotomy defect

Fig. 41.19: Axial postcontrast CT scan of the brain showing

dilated tortuous vessels with intranidal aneurysm (arrow)
538 Section 6 Retrochiasmal Visual Pathway Lesions
• Calcification is seen in 25-30 percent cases
• CT is useful for demonstrating acute hemorrhage,
mass effect and surrounding edema and focal
compression and displacement of normal anatomic
structures by AVMs
• In thrombosed AVMs, calcification is frequent and
variable enhancement is seen.
MRI
• MRI signals are variable and depend on the flow rate
and direction in feeding and draining vessels,
presence of hemorrhage and secondary changes in the
brain
• In patent parenchymal AVMs, tightly packed honey-
comb or flow voids caused by high velocity signal
loss may be seen. Areas of increased signal may be
seen in thrombosed vessels
• Patent AVMs with hyperacute clot show areas of high
velocity signal loss with adjacent hemorrhage that is
typically isointense to brain on T1 and hyperintense
on T2 and low signal on gradient-refocused study.
Subacute hematoma display mixed signal intensity
• Chronic hemorrhage appears as hypointense signal
within and around the AVM on T2-weighted and
gradient—echo images
• Vascular steal phenomenon leads to atrophy and
gliosis of brain that appear as increased T2 signal
within atrophic, shrunken brain.
MR Angiography
• A modified time of flight MRA technique, multiple
overlapping thin slab acquisition (MOTSA) combines
advantages of two- and three-dimensional time of flight
techniques and detects most AVMs. Phase-contrast
MRA allows velocity determination and yields
directional flow information in the vessels feeding the
AVM and those supplying adjacent brain. Small AVMs
can also be detected by phase-contrast MRA.
Dynamic contrast-enhanced 3D, tetralogy of fallot
(TOF) angiogram is useful evaluating patients both
pre- and postradio-surgery.
BIBLIOGRAPHY
1. Aoyama H, Shirato H, Katoh N, Kudo K, Asano T, Kuroda
S, Ishikawa T, Miyasaka K. Comparison of imaging
modalities for the accurate delineation of arteriovenous
malformation, with reference to stereotactic radiosurgery.
Int J Radiat Oncol Biol Phys 2005;62(4):1232-8.
2. Byrne JV. Cerebrovascular malformations. Eur Radiol
2005;15(3):448-52. Review.
3. Gauvrit JY, Leclerc X, Oppenheim C, Munier T, Trystram
D, Rachdi H, Nataf F, Pruvo JP, Meder JF. Three-
dimensional dynamic MR digital subtraction angiography
using sensitivity encoding for the evaluation of intracranial
arteriovenous malformations: A preliminary study. Am J
Neuroradiol 2005;26(6):1525-31.
4. Grinstead JW, Sinha S, Tateshima S, Nien YL, Vinuela F.
Visualization and quantification of flow and velocity fields
in intracranial arteriovenous malformations using phase-
contrast MR angiography. Am J Roentgenol 2006;186(2):
553-5.
5. Kwon BJ, Han MH, Kang HS, Chang KH. MR imaging
findings of intracranial dural arteriovenous fistulas:
Relations with venous drainage patterns. Am J Neuro-
radiol 2005;26(10):2500-7.
6. Nagaraja S, Capener D, Coley SC, Lee KJ, Wilkinson ID,
Kemeny AA, Griffiths PD. Brain arteriovenous
malformations: Measurement of nidal volume using a
combination of static and dynamic magnetic resonance
angiography techniques. Neuroradiol 2005;47(5):387-92.
7. Sato M. MR-digital subtraction angiography in the
diagnosis of cerebral arteriovenous malformations. No To
Shinkei 2005;57(10):902-3.
8. Ziyeh S, Strecker R, Berlis A, Weber J, Klisch J, Mader I.
Dynamic 3D MR angiography of intra- and extracranial
vascular malformations at 3T: A technical note. Am J
Neuroradiol 2005;26(3):630-4.

CAVERNOUS ANGIOMA
• Cavernous angioma belongs to a group of intracranial
vascular malformations that are developmental
malformations of the vascular bed. These congenital
abnormal vascular connections frequently enlarge
over time.
• Traditionally, intracranial vascular malformations are
grouped into four types, as follows:
1. Arteriovenous shunting malformations
2. Cavernous malformations (cavernous angiomas/
hemangiomas)
3. Venous malformations:
a. Venous angiomas
b. Vein of Galen malformations
c. Venous varix
4. Capillary malformations (or telangiectasias).
• Cavernous angiomas represent approximately
1 percent of intracranial vascular lesions and
15 percent of cerebrovascular malformations
• As a cause of hemorrhage, cavernous angiomas are
far less common than hypertension; nevertheless, as
a cause of hemorrhage, they must be excluded,
especially in young patients. Cavernous angiomas can
also cause a variety of symptoms and neurologic
findings similar to those of tumors
• Cavernous angiomas are typically discrete
multilobulated lesions that contain hemorrhage in
various stages of evolution. Because they are lobulated
and dark red to blue, the lesions grossly resemble
small mulberries
• Cavernous angiomas vary from several millimeters
to several centimeters (usually <3 cm) in diameter
• A familial form of the disorder exists and is inherited
as an autosomal dominant trait with variable
expression. Multiple lesions are more common in the
familial form, occurring in as many as 73 percent of
patients.
CLINICAL FEATURES
• No sex predilection is reported, cavernous angiomas
can occur at any age, but they are most likely to
become clinically apparent in patients aged 20 to
40 years
• Not all cavernous angiomas are associated with
symptoms, but once patients become symptomatic
40 to 50 percent present with seizures, 20 percent
present with focal neurologic deficits, and 10 to 25
percent present with hemorrhage
• Symptoms may progress rapidly, be stable for years;
or wax and wane, as in multiple sclerosis
Chapter 41 Vascular Lesions 539
• Headaches are the most common symptoms. Acute
headaches may result from parenchymal irritation
secondary to gross or repeated extralesional
hemorrhage. Chronic headaches are believed to be the
result of mass effect in slow-growing larger lesions
as a result of repeated intralesional hemorrhage
• The effects of hemorrhage usually result from the
location of the lesion and, at times, their slow
expansion. However, in an exceptional cases, the
hemorrhage may be massive and can also be fatal.
Small hemorrhages in critical locations can have more
severe effects, and thus, they are more likely to
produce symptoms (e.g. brainstem involvement)
• Progressive neurological deficits are more often
associated with cavernous angioma in the infra-
tentorial space and with lesions that demonstrate slow
enlargement because of recurrent bleeds.
LOCATION
• Cavernous angioma can be found in any part of the
brain
• Frontal and temporal lobes are the most common sites
of occurrence, and 80 to 90 percent of the lesions are
supratentorial. The deep cerebral white matter,
corticomedullary junction, and basal ganglia are
common supratentorial sites
• The pons and cerebellar hemispheres are common
posterior fossa sites.
IMAGING
CT Scan
• Nonenhanced CT scan demonstrate cavernous
angioma as focal oval or nodular-appearing lesion that
demonstrates mild-to-moderate increased attenua-
tion, without mass effect on the surrounding brain
parenchyma
• Areas of calcification and hemosiderin deposits in the
walls of the fibrous septa, combined with the
increased blood pool within the lesion, are responsible
for hyperattenuation on noncontrast images
• CT scan demonstrates calcifications in as many as
33 percent of cavernous angioma. If the lesions are
older, they may contain central hypoattenuating
nonenhancing areas, which correspond to cystic
cavities from resorbed hematoma
• Contrast enhancement can vary from minimal to
striking, although 70 to 94 percent of cavernous
540 Section 6 Retrochiasmal Visual Pathway Lesions

Figs 41.20A and B: A 33-year-old female presented with severe

headache and right 3rd nerve palsy: (A) Axial plain CT scan
and (B) Axial contrast-enhanced CT scan of the brain showing
a well-circumscribed—irregular hyperdense nonenhancing
lesion in the right side of the suprasellar cistern—cavernous
angioma. MRI was done and showed hemorrhage within the
B lesion. MRA was normal

malformations demonstrate mild-to-moderate
enhancement after the intravenous administration of
contrast agent. In large part, this enhancement results
from the increased blood pool within the vascular
component. The slightly heterogeneous and mottled
enhancement results from the fibrous intravascular
septa, and the peripheral rim of decreased attenuation
results from the pseudocapsule of gliotic tissue
surrounding the lesion
• Mass effect is not common, unless the lesion is
associated with recent hemorrhage. Cavernous
angioma may not be detected, when they present as
acute intracerebral hematoma on nonenhanced CT
images. After the administration of contrast material,
cavernous angioma may be identified as an area of
nodular enhancement adjacent to the hematoma.
MR Imaging
• On MRI, parenchymal cavernous angioma
demonstrates typical, popcorn like, smoothly
circumscribed, well-delineated complex lesions. The
core is formed by multiple foci of mixed signal
intensities, which represent hemorrhage in various
stages of evolution.
• Acute hematoma containing deoxyhemoglobin is
isointense on T1-weighted images and markedly
hypointense on T2-weighted images. Subacute
hematoma, which contains extracellular methemo-
globin, displays hyperintensity on both T1- and T2-
weighted images because of the paramagnetic effect of
the methemoglobin
• The interspersed fibrous-containing elements
demonstrate mild hypointensity on both T1- and T2-
weighted images because they contain a combination
of calcification and hemosiderin. The heterogeneous
core typically is surrounded completely by a low-
signal-intensity hemosiderin rim on T1-weighted
images. The hypointensity of this rim becomes more
prominent, or blooms, on T2-weighted and gradient-
refocused images because of the magnetic
susceptibility effects
• Typically, cavernous angiomas are not associated with
mass effect or edema and do not demonstrate a
feeding artery or draining vein, except when
associated with other vascular malformations with
similar features. Cavernous angiomas are reported to
be associated with venous malformations, which
typically demonstrate a draining vein. Often,
Chapter 41 Vascular Lesions 541
conventional angiography can be helpful for further
characterization in these mixed cases
• Cavernous malformations are considered
angiographically occult, and when they are evident
on angiographic studies, the findings are nonspecific.
MRI has largely replaced conventional angiography
in the diagnosis of cavernous angioma
• When the lesions occur in combination with other
types of vascular malformations, as they do in as many
as 30 percent of patients with venous angioma, MRI
characteristics become more complicated and less
specific. In these patients, angiography can help
further define the lesions.
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8. Rapacki TF, Brantley MJ, Furlow TW Jr, et al.
Heterogeneity of cerebral cavernous hemangiomas
diagnosed by MR imaging. J Comput Assist Tomogr
1990;14(1):18-25.
9. Sage MR, Blumbergs PC. Cavernous hemangiomas
(angiomas) of the brain. Australas Radiol 2001;45(2):247-
56.
10. TerBrugge KG, Rao KC. Intracranial aneurysms and
vascular malformations. In: Lee SH, Zimmerman RA, Rao
KC (Eds). Cranial MRI and CT, 4th edn. New York:
McGraw-Hill 1999;530-7.
11. Voigt K, Yasargil MG. Cerebral cavernous hemangiomas
or cavernomas. Incidence, pathology, localization,
diagnosis, clinical features and treatment. Review of the
literature and report of an unusual case. Neurochirurgia
(Stuttg) 1976;19(2):59-68.
542 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 41.21A to D: (A) Axial postcontrast T1-weighted image, (B) Coronal postcontrast T1-weighted image showing a rounded
hyperintense signal in the medulla suggestive of subacute hemorrhage, (C) Coronal T2-weighted image and (D) Axial T2-weighted
image with fat supression showing the lesion mixed hyperintense and markedly hypointense confirming blood products—cavernous
angioma

MOYAMOYA DISEASE
• Moyamoya disease (MMD) is a progressive occlusive
disease of the cerebral vasculature with particular
involvement of the circle of Willis
• Moyamoya (means “puff of smoke” in Japanese). The
appearance on angiography of abnormal vascular
collateral networks that develop adjacent to the
stenotic vessels resemble “puff of smoke”
• The exact etiology of Moyamoya disease is unknown.
Some genetic predisposition is apparent because it is
familial 10 percent of the time. The disease may be
hereditary and multifactorial
• Mortality rates of Moyamoya disease are approxi-
mately 10 percent in adults and 4.3 percent in children.
Death is usually from hemorrhage
• About 50-60 percent of affected individuals experience
a gradual deterioration of cognitive function,
presumably from recurrent strokes
• The female-to-male ratio of Moyamoya disease is 1.8:1
CLINICAL FEATURES
• Children may have hemiparesis, monoparesis,
sensory impairment, involuntary movements,
headaches, dizziness, or seizures. Mental retardation
or persistent neurologic deficits may be present
• Adults may have symptoms and signs similar to those
in children, but intraventricular, subarachnoid, or
intracerebral hemorrhage of sudden onset is more
common in adults.
IMAGING
Cerebral angiography is the modality of choice for
diagnosis. The following findings may be seen:
• Stenosis or occlusion at the terminal portion of the
internal carotid artery or the proximal portion of the
anterior or middle cerebral arteries
• Abnormal vascular networks in the vicinity of the
occlusive or stenotic areas
• Bilaterality of the described findings (although some
patients may present with unilateral involvement and
then progress)
Chapter 41 Vascular Lesions 543
• MRI not only reveals areas of infarctions but also
allows direct visualization of these collateral vessels
as multiple small flow voids at the base of brain and
basal ganglia
• MR angiography is used to confirm the diagnosis and
to see the anatomy of the vessels involved. It typically
reveals the narrowing and occlusion of proximal cerebral
vessels and extensive collateral flow through the
perforating vessels demonstrating the classic “puff of
smoke” appearance. Availability of higher field strength
MR and better image quality of MR angiograms has
virtually made conventional angiograms for diagnosis
of Moyamoya disease obsolete
• Contrast-enhanced T1-weighted images are better than
FLAIR images for depicting the leptomeningeal— ivy
sign in Moyamoya disease
• Diffusion-weighted imaging helps in identifying acute
infarcts in patients having multiple infarcts with
recent onset neurological deficit.
BIBLIOGRAPHY
1. Hervé D, Touraine P, Verloes A, Miskinyte S, Krivosic V,
Logeart D, Alili N, Laredo JD, Gaudric A, Houdart E,
Metzger JP, Tournier-Lasserve E, Woimant F. A hereditary
Moyamoya syndrome with multisystemic manifestations.
Neurology 2010;75(3):259-64.
2. Li J, Liu R, Li ZY, Wu DF, Ma XJ, Miao JT. Clinical
manifestations and neuroimaging characteristics of
children with Moyamoya disease. Zhongguo Dang Dai
Er Ke Za Zhi 2010;12(8):637-40.
3. Manjunatha YC, Gupta AK. Moyamoya disease. Indian J
Pediatr 2010;77(7):817.
4. Pandey P, Bell-Stephens T, Steinberg GK. Patients with
Moyamoya disease presenting with movement disorder.
J Neurosurg Pediatr 2010;6(6):559-66.
5. Kim SK, Cho BK, Phi JH, Lee JY, Chae JH, Kim KJ, Hwang
YS, Kim IO, Lee DS, Lee J, Wang KC. Pediatric moyamoya
disease: An analysis of 410 consecutive cases. Ann Neurol
2010;68(1):92-101.
6. Geibprasert S, Pongpech S, Jiarakongmun P, Shroff MM,
Armstrong DC, Krings T. Radiologic assessment of brain
arteriovenous malformations: What clinicians need to
know. Radiographics 2010;30(2):483-501.
544 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 41.22A to D: (A) Axial T2-weighted image at the level of the lateral ventricle showing chronic infarcts in
the bilateral PCA territory (block arrow) and right ACA–MCA watershed territory, (B) Axial FLAIR image at a
higher level also showing bilateral patchy infarcts and tiny speckled flow voids and flow signals in the deep
cerebral parenchyma suggestive of collateral vessels, (C ) Sagittal MIP image and (D) Coronal MIP image
showing narrowing of the terminal internal carotid arteries and proximal ACA and MCA (white arrow).
Note: The deep collaterals giving the appearance of “puff of smoke” (arrow)—moyamoya disease
Chapter 42

Intracranial Neoplasms
INTRACRANIAL TUMORS

INTRODUCTION – Brainstem
– Cerebellar
• Intracranial tumors may involve the brain or other
• Glioblastoma multiforme (WHO grade IV):
structures (e.g. cranial nerves, meninges). The tumors Variants: Giant cell glioblastoma, gliosarcoma
usually develop during early or middle adulthood but • Pilocytic astrocytoma (noninvasive, WHO grade I):
may develop at any age; they are becoming more – Hemispheric
common among the elderly. Brain tumors are found – Diencephalic
in about 2% of routine autopsies. – Optic
– Brainstem
WHO CLASSIFICATION OF – Cerebellar
INTRACRANIAL TUMORS • Subependymal giant cell astrocytoma (noninvasive,
WHO grade I)
• Pleomorphic xanthoastrocytoma (noninvasive,
Neuroepithelial Tumors of the CNS WHO grade I)
2. Oligodendroglial tumors:
1. Astrocytic tumors [glial tumors—categories I-V, • Oligodendroglioma (WHO grade II)
below—may also be subclassified as invasive or non- • Anaplastic (malignant) oligodendroglioma (WHO
invasive, although this is not formally part of the grade III)
WHO system, the noninvasive tumor types are 3. Ependymal cell tumors:
indicated below. Categories in italics are also not • Ependymoma (WHO grade II)
recognized by the new WHO classification system, Variants: Cellular, papillary, epithelial, clear cell,
but are in common use.] mixed
• Astrocytoma (WHO grade II): • Anaplastic ependymoma (WHO grade III)
• Myxopapillary ependymoma
Variants: Protoplasmic, gemistocytic, fibrillary, • Subependymoma (WHO grade I)
mixed 4. Mixed gliomas:
• Anaplastic (malignant) astrocytoma (WHO grade III): • Mixed oligoastrocytoma (WHO grade II)
– Hemispheric • Anaplastic (malignant) oligoastrocytoma (WHO
– Diencephalic grade III)
– Optic • Others (e.g. ependymoastrocytomas)
546 Section 6 Retrochiasmal Visual Pathway Lesions

5. Neuroepithelial tumors of uncertain origin: • Granulocytic sarcoma

• Polar spongioblastoma (WHO grade IV) • Others
• Astroblastoma (WHO grade IV) 3. Germ cell tumors:
• Gliomatosis cerebri (WHO grade IV) • Germinoma
6. Tumors of the choroid plexus: • Embryonal carcinoma
• Choroid plexus papilloma • Yolk sac tumor (endodermal sinus tumor)
• Choroid plexus carcinoma (anaplastic choroid • Choriocarcinoma
plexus papilloma) • Teratoma
7. Neuronal and mixed neuronal-glial tumors: • Mixed germ cell tumors
• Gangliocytoma 4. Tumors of the meninges:
• Dysplastic gangliocytoma of cerebellum • Meningioma
(Lhermitte-Duclos) Variants: Meningothelial, fibrous (fibroblastic),
• Ganglioglioma transitional (mixed), psammomatous, angio-
• Anaplastic (malignant) ganglioglioma matous, microcystic, secretory, clear cell, chordoid,
• Desmoplastic infantile ganglioglioma—desmo- lymphoplasmacyte-rich, and metaplastic subtypes
plastic infantile astrocytoma • Atypical meningioma
• Central neurocytoma • Anaplastic (malignant) meningioma
• Dysembryoplastic neuroepithelial tumor 5. Non-meningothelial tumors of the meninges:
• Olfactory neuroblastoma (esthesioneuroblastoma) • Benign mesenchymal:
Variant: Olfactory neuroepithelioma – Osteocartilaginous tumors
8. Pineal parenchyma tumors: – Lipoma
• Pineocytoma – Fibrous histiocytoma
• Pineoblastoma – Others
• Mixed pineocytoma/pineoblastoma • Malignant mesenchymal:
9. Tumors with neuroblastic or glioblastic elements – Chondrosarcoma
(embryonal tumors): – Hemangiopericytoma
• Medulloepithelioma – Rhabdomyosarcoma
• Primitive neuroectodermal tumors with – Meningeal sarcomatosis
multipotent differentiation – Others
– Medulloblastoma • Primary melanocytic lesions:
Variants: Medullomyoblastoma, melanocytic – Diffuse melanosis
medulloblastoma, desmoplastic medullo- – Melanocytoma
blastoma – Malignant melanoma
– Cerebral primitive neuroectodermal tumor Variant: Meningeal melanomatosis
• Neuroblastoma • Hemopoietic neoplasms:
Variant: Ganglioneuroblastoma – Malignant lymphoma
• Retinoblastoma – Plasmacytoma
• Ependymoblastoma – Granulocytic sarcoma
• Tumors of uncertain histogenesis:
Other CNS Neoplasms – Hemangioblastoma (capillary hemangio-
1. Tumors of the sellar region: blastoma)
• Pituitary adenoma 6. Tumors of cranial and spinal nerves
• Pituitary carcinoma • Schwannoma (neurinoma, neurilemmoma):
• Craniopharyngioma – Cellular, plexiform, and melanotic subtypes
2. Hematopoietic tumors: • Neurofibroma:
• Primary malignant lymphomas – Circumscribed (solitary) neurofibroma
• Plasmacytoma – Plexiform neurofibroma
 Chapter 42 Intracranial Neoplasms 547

• Malignant peripheral nerve sheath tumor (Malignant 10. Cysts and tumor-like lesions:
schwannoma): • Rathke cleft cyst
– Epithelioid • Epidermoid
– Divergent mesenchymal or epithelial diffe- • Dermoid
rentiation • Colloid cyst of the third ventricle
– Melanotic • Enterogenous cyst
7. Local extensions from regional tumors: • Neuroglial cyst
• Paraganglioma (chemodectoma) • Granular cell tumor (choristoma, pituicytoma)
• Chordoma • Hypothalamic neuronal hamartoma
• Chondroma • Nasal glial herterotopia
• Chondrosarcoma • Plasma cell granuloma.
• Carcinoma
8. Metastatic tumors The tumors that we have encountered in our clinical
9. Unclassified tumors practice will be described briefly.
548 Section 6 Retrochiasmal Visual Pathway Lesions
I. ASTROCYTOMA
INTRODUCTION
• Astrocytomas are CNS neoplasms made of astrocytes,
which are one of the primary neuroglial cells present
in the nervous system
• Histologically, they can be classified into three types:
1. Fibrillary, the most common primary brain
tumors, that include low-grade fibrillary,
anaplastic and glioblastoma multiforme,
2. Protoplasmic (rarest), and
3. Pilocytic which could be of adult or juvenile type
• The most widely used classification of astrocytoma is
the WHO four-tiered grading system
• Grade 1 is astrocytomas with excellent prognosis
following surgical excision, e.g. juvenile pilocytic
astrocytoma. Grade 4 is the aggressive glioblastoma
multiforme. In between are astrocytoma (grade 2) and
anaplastic astrocytoma (grade 3)
• Fibrillary astrocytoma may arise anywhere in the
CNS. In children, it is usually seen in brainstem and
hypothalamus. In adults, it is found in the cerebral
hemispheres
• Anaplastic is usually found in the cerebral
hemispheres. It may also be found in the brainstem,
third ventricle and cerebellum
• Glioblastomas are found in the frontal lobe and
brainstem in children. In adults, they are seen in the
frontal lobe and temporal lobe and rarely in the
occipital lobe, cerebellum and pineal gland. They can
be multifocal
• Protoplasmic is limited to the cerebrum
• Pilocytic can be found in the optic nerve, chiasm,
hypothalamus, septum pellucidum, brainstem,
cerebrum and cerebellum. Most of them present in
the first two decades of life.
ETIOLOGY
• With the exception of therapeutic irradiation and,
perhaps, nitroso compounds (e.g. nitrosourea), the
identification of specific causal environmental
exposures or agents has been unsuccessful
• Children receiving prophylactic irradiation for acute
lymphatic leukemia (ALL), have a 22-fold increased
risk of developing astrocytomas, with an interval for
onset of 5-10 years
• Irradiation of pituitary adenomas has been
demonstrated to carry a 16-fold increased risk of
glioma formation
• Evidence also exists for genetic susceptibility to
glioma development.
SIGNS AND SYMPTOMS
The signs and symptoms depend on the site and extent
of tumor growth.
• Headache, visual disturbance, motor impairment,
seizures, sensory anomalies, ataxia, or altered mental
status, cognitive impairment should alert the clinician
for further neurological evaluation
• A thorough neurological examination is required as
these tumors may affect any part of the CNS, including
the spinal cord, and may spread to distant regions of
the CNS
• Special attention should be paid to signs of increased
ICP, such as decreased alertness, cognitive
impairment, papilledema, and ataxia, to determine the
likelihood of mass effect, hydrocephalus, and
herniation
• Localizing and lateralizing signs, including cranial
nerve palsies, hemiparesis, sensory levels, alteration
of deep tendon reflexes (DTRs), and the presence of
pathological reflexes (e.g. Hoffman and Babinski
signs), should be noted. Once neurological
abnormalities are identified, imaging studies should
be sought for further evaluation.
IMAGING STUDIES
• CT and MRI (with and without contrast) are helpful
in the diagnosis and grading of astrocytomas. MRI is
the imaging modality of choice, but a CT scan may be
done when MRI is contraindicated
• On a CT scan, low-grade astrocytomas appear as
poorly defined, homogeneous, low-density masses
without contrast enhancement. However, slight
enhancement, calcification, and cystic changes may
be evident early in the course of the disease
• Anaplastic astrocytomas may appear as low-density
lesions or inhomogeneous lesions, with areas of both
high and low density within the same lesion. Unlike low-
grade lesions, partial contrast enhancement is common
• Astrocytomas generally are hypo- or isointense on T1-
weighted images and hyperintense on T2-weighted
images. While low-grade astrocytomas uncommonly
enhance on MRI, most anaplastic astrocytomas
enhance with paramagnetic contrast agents.

MR diffusion and perfusion and MR spectroscopy
are newer imaging techniques that are now part of
the tumor imaging protocol.
Other Tests
• EEG may be employed to evaluate and monitor
epileptiform activity
• Radionuclide scans, such as positron emission
tomography (PET), PET-CT, single-photon emission
tomography (SPECT), and technetium-based imaging,
can permit study of tumor metabolism and brain
function. PET and SPECT may be used to distinguish
a solid tumor from edema, to differentiate tumor
recurrence from radiation necrosis, and to localize
structures.
• Metabolic activity determined by radionuclide scans
can be used to determine the grade of a lesion.
Hypermetabolic lesions often correspond to higher-
grade tumors.
GLIOBLASTOMA MULTIFORME
• Glioblastoma multiforme (GBM) is the most
malignant glial tumor
• It is composed of poorly differentiated neoplastic
astrocytes
• Glioblastomas primarily affect adults, and they are
located in the cerebral hemispheres. Much less
commonly, GBMs can affect the brainstem in children
and the spinal cord
• These tumors may develop from lower-grade
astrocytomas or anaplastic astrocytomas, but more
frequently, they manifest de novo, without any
evidence of a less malignant precursor lesion
• Males have a slight preponderance over females
(1.5M:1F)
• The clinical history of patients with GBMs usually is
short (< 3 months in > 50% cases). The most common
presentation is a progressive neurologic deficit,
usually motor weakness. Other common
presentations include increased intracranial pressure,
cognitive impairment and seizures
• Neurologic symptoms and signs affecting patients
with glioblastomas can be either general or focal
depending upon the location of the tumor
• Focal signs include hemiparesis, sensory loss, visual
loss, aphasia, etc.
Etiology
• The etiology of glioma essentially remains unknown
• Occurrence of familial brain tumors (e.g. neuro-
fibromatosis 1 or 2) constitutes <1 percent of all the
patients with gliomas
Chapter 42 Intracranial Neoplasms 549
• The only consistently identified risk factor is chronic
exposure to petrochemicals.
Imaging
Imaging studies of the brain are essential to make the
diagnosis of GBM.
• On CT scan, glioblastomas usually appear as
irregularly shaped hypodense lesions with a
peripheral ring-like zone of contrast enhancement and
a penumbra of cerebral edema
• MRI with and without contrast is the study of choice.
These lesions typically have an enhancing ring
observed on T1-weighted contrast-enhanced images
and a broad surrounding zone of edema apparent on
T2-weighted images
• The central hypodense core represents necrosis, the
contrast-enhancing ring is composed of highly dense
neoplastic cells with abnormal vessels permeable to
contrast agents, and the peripheral zone of non-
enhancing low attenuation is vasogenic edema
containing varying numbers of invasive tumor cells
• Several pathological studies have clearly shown that
the area of enhancement does not represent the outer
tumor border because infiltrating glioma cells can be
identified easily within, and occasionally beyond, a
2 cm margin
• Positron emission tomography (PET) scans and
magnetic resonance (MR) spectroscopy can be helpful
to identify glioblastomas in difficult cases, such as
those associated with radiation necrosis or
hemorrhage. On PET scans, increased regional
glucose metabolism closely correlates with cellularity
and reduced survival
• MR spectroscopy demonstrates an increase in the
choline-to-creatine peak ratio, an increased lactate
peak, and decreased N-acetylaspartate (NAA) peak
in areas with glioblastomas.
BIBLIOGRAPHY
1. Byrne TN. Imaging of gliomas. Semin Oncol 1994;21(2):
162-71.
2. Cao Y, Sundgren PC, Tsien CI, Chenevert TT, Junck L.
Physiologic and metabolic magnetic resonance imaging
in gliomas. J Clin Oncol 2006;24(8):1228-35. Review.
3. Chow KL, Gobin YP, Cloughesy T, Sayre JW, Villablanca
JP, Vinuela F. Prognostic factors in recurrent glioblastoma
multiforme and anaplastic astrocytoma treated with
selective intra-arterial chemotherapy. Am J Neuroradiol
2000;21(3):471-8.
4. Dean BL, Drayer BP, Bird CR, Flom RA, Hodak JA, Coons
SW, Carey RG. Gliomas: Classification with MR imaging.
Radiology 1990;174(2):411-5.
550 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.1A and B: Axial postcontrast CT scan of the brain (A and B) at the level of the lateral ventricles showing a well-defined cystic
lesion with rim enhancement and extensive perilesional edema causing mass effect and midline shift (arrow)—cystic astrocytoma

Fig. 42.2: Axial postcontrast CT scan of the brain showing a

heterogeneously enhancing lesion in the right temporal perisyl-
vian region—Grade II astrocytoma (arrow)

5. Fleury A, Menegoz F, Grosclaude P, et al. Descriptive
epidemiology of cerebral gliomas in France. Cancer
1997;79(6):1195-1202.
6. Galanis E, Buckner JC, Novotny P, Morton RF, McGinnis
WL, Dinapoli R, Schomberg P, O'Fallon JR. Efficacy of
neuroradiological imaging, neurological examination, and
symptom status in follow-up assessment of patients with
high-grade gliomas. J Neurosurg 2000;93(2):201-7.
7. Hakyemez B, Erdogan C, Ercan I, et al. High-grade and
low-grade gliomas: Differentiation by using perfusion MR
imaging. Clin Radiol 2005;60(4):493-502.
8. Hammoud MA, Sawaya R, Shi W, Thall PF, Leeds NE.
Prognostic significance of preoperative MRI scans in
glioblastoma multiforme. J Neurooncol 1996;27(1):65-73.
9. Law M, Yang S, Wang H, Babb JS, Johnson G, Cha S,
Knopp EA, Zagzag D. Glioma grading: Sensitivity,
specificity, and predictive values of perfusion MR imaging
and proton MR spectroscopic imaging compared with
conventional MR imaging. Am J Neuroradiol 2003;24(10):
1989-98.
Chapter 42 Intracranial Neoplasms 551
10. Macdonald DR. Low-grade gliomas, mixed gliomas, and
oligodendrogliomas. Semin Oncol 1994;21(2): 236-48.
11. Morantz RA. Low-grade astrocytomas. In: Kaye AH,
Laws ER (Eds). Brain tumors: An encyclopedic approach,
2nd edn. London, England: Harcourt Publishers; 2001.
pp.467-91.
12. Pope WB, Sayre J, Perlina A, Villablanca JP, Mischel PS,
Cloughesy TF. MR imaging correlates of survival in
patients with high-grade gliomas. Am J Neuroradiol 2005;
26(10):2466-74.
13. Recht LD, Bernstein M. Low-grade gliomas. Neurol Clin
1995;13(4):847-59.
14. Riemann B, Papke K, Hoess N, Kuwert T, Weckesser M,
Matheja P, Wassmann H Heindel W, Schober O.
Noninvasive grading of untreated gliomas: A comparative
study of MR imaging and 3-(iodine 123)-L-alpha
methyltyrosine SPECT. Radiology 2002;225(2):567-74.
15. Vandenberg SR, Sampaio Lopez MB. Classification. In:
Berger MS, Wilson CB (Eds). The Gliomas. Philadelphia:
WB Saunders 1999.pp.172-91.
552 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.3A and B: Axial postcontrast CT scan of the brain (A and B) showing a large ill-defined lesion in the left cerebral hemi-
sphere with edema and mass effect on the ventricles—diffuse infiltrating astrocytoma

A B

Figs 42.4A and B: A 22-year-old male presented with papilledema: (A) Axial postcontrast CT scan of the brain showing an
ill-defined non-enhancing lesion in the right temporal-parietal lobe and (B) Coronal FLAIR showing ill-defined margins with relatively
narrow zone of transition—Grade II astrocytoma (white arrows)
 Chapter 42 Intracranial Neoplasms 553

A B

C D

Figs 42.5A to D: A 65-year-old male with history of right homonymous hemianopia: (A) Axial T1-weighted
image showing a necrotic lesion with perilesional edema in the left occipital lobe, (B) Axial FLAIR showing the
extent of the lesion also involving the splenium and (C and D) Contrast-enhanced T1-weighted images showing
heterogeneous peripheral enhancement in the splenium and occipital lobe with areas of necrosis—glioblastoma
multiforme
554 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 42.6A to D: A 55-year-old female presented with right incongruous hemianopia: (A) Axial T2-weighted
image showing a thick-walled centrally necrotic lesion in the left posterior temporal lobe (black arrow) with
perilesional edema (white arrow), (B) Diffusion-weighted image showing high cellular activity in the periphery of
the lesion appearing bright on diffusion weighted image (arrow), (C) Postcontrast T1-weighted image showing
thick irregular wall enhancement and non-enhancing central necrosis and (D) Single voxel PROBE spectroscopy,
voxel placed in the enhancing periphery of the lesion showing reduction in NAA, elevation of lipid-lactate and
mild elevation of choline peak HPE—glioblastoma multiforme

II. OLIGODENDROGLIOMA
• They are relatively rare tumors with a more benign
course
• They are slow growing tumors that carry a 5-year
survival rate of >50 percent. Their peak incidence is
in 4th to 5th decade of life. Patients usually present
with headache and seizures
• They are mostly located in supratentorial locations,
often in the frontal and frontotemporal regions. In
adults, about 30 percent are calcified
• Many of them contain a mixture of cells with astrocytic
and oligodendroglial features. A tumor with a mixed
histological features is termed a mixed gliomas or
oligoastrocytoma
• The greater the oligodendroglial component, the
more benign the behavior of the tumor. On the other
hand, the histological features of mitoses, nuclear
atypia are associated with a more aggressive course
and in that case, the tumor is termed a malignant
oligodendroglioma
• Overall, they are less infiltrative tumors than
astrocytomas, allowing more complete surgical
excision.
IMAGING
Imaging can be diagnostic of such lesions based on
location, and characteristic calcification.
CT
Calcification is a feature of these tumors. Linear or
nodular tumor calcifications can be found in 50-90 percent
of tumors.
Chapter 42 Intracranial Neoplasms 555
MRI
• They are usually located in the frontal lobe. They
are usually heterogeneous. Cystic regions and
hemorrhages may be seen within the tumors
• The heterogeneous appearance can be due to the
calcification
• Edema is not a significant feature of this tumor
• Half the cases, contrast enhancement is heterogeneous.
Calvarial erosion is seen in some and this is not seen
well on MRI unless marrow containing medullary bone
is destroyed.
BIBLIOGRAPHY
1. Brami-Zylberberg F, Grand S, Le Bas JF, Meder JF. MRI
for oligodendrogliomas. Neurochirurgie 2005;51(3-4 Pt
2):273-85. Review.
2. Dutertre G, Leveque C, Delmas JM, Cordoliani YS, Nioche
C. Functional MR imaging and oligodendrogliomas.
Neurochirurgie 2005;51(3-4 Pt 2):323-8.
3. Jager HR, Waldman AD, Benton C, Fox N, Rees J.
Differential chemosensitivity of tumor components in a
malignant oligodendroglioma: Assessment with diffusion-
weighted, perfusion-weighted, and serial volumetric MR
imaging. Am J Neuroradiol 2005;26(2):274-8.
4. Naugle DK, Duncan TD, Grice GP. Oligoastrocytoma.
Radiographics 2004;24(2):598-600.
5. White ML, Zhang Y, Kirby P, Ryken TC. Can tumor contrast
enhancement be used as a criterion for differentiating tumor
grades of oligodendrogliomas? Am J Neuroradiol 2005;
26(4):784-90.
6. White ML, Zhang Y, Smoker WR, Kirby PA, Hayakawa
M, Sickels WJ, Ryken TC, Berbaum K. Fluid-attenuated
inversion-recovery MR imaging in assessment of
intracranial oligodendrogliomas. Comput Med Imaging
Graph 2005;29(4):279-85. Epub 2005 Mar 24.
556 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.7A and B: Axial postcontrast CT scan of the brain showing a cystic lesion with peripheral enhancement and calcification
in the left temporoparietal lobe—oligodendroglioma

A B

Figs 42.8A and B: Axial plain CT scan of the brain showing a small cystic lesion with multiple specks calcification and perilesional
edema—oligodendroglioma

III. CHOROID PLEXUS TUMORS
Tumors involving the choroids plexus are quite rare in
the occurrence.
TUMORS INVOLVING THE CHOROID PLEXUS
• Choroids plexus papilloma
• Choroids plexus carcinoma
• Meningioma
• Metastasis.
Benign Lesions
• Non-neoplastic cysts
• Vascular malformations.
CHOROID PLEXUS PAPILLOMA
• They constitute approximately 0.5 percent of all
intracranial tumors
• They typically appear as cauliflower-like mass
• More than 86 percent of these tumors occur in children
within the first 5 years of life
• Approximately, 40 percent occur in the fourth ventricle
(usually in adults), and another 40 percent in one of
the lateral ventricles. Bilateral tumors are rare
• Extraventricular choroid plexus tumors usually occur
by seeding of the CSF pathways from the primary
neoplasm
• They are irregularly lobulated masses projecting into
the ventricular cavity from the choroid plexus
• Large tumors locally expand the ventricle in which
they are growing and may cause trapping, with
dilation of its more peripheral draining segments
• The tumors may show regions of heavy calcification
with bone formation. Hemorrhage and cystic regions
may develop within the tumor.
Clinical Presentation
Hydrocephalus due to overproduction of cerebrospinal
fluid and/or blockage in the subarachnoid cisterns or
interventricular pathways from adhesions resulting from
tumor hemorrhage.
Imaging
Transcranial ultrasound shows a highly echogenic
intraventricular mass with irregular borders associated
with hydrocephalus. Pulsatile vascular channels may be
seen.
Chapter 42 Intracranial Neoplasms 557
CT Scan
• They appear as isodense or hyperdense on plain CT.
Calcification is seen in 25 percent cases
• Tumor margins are usually slightly irregular,
reflecting the frond-like surface of these neoplasms.
With contrast, they show irregular, heterogeneous
enhancement.
MRI
• They usually have a very lobulated margin and are
iso- to slightly hypointense to gray matter. It may
contain multiple punctate linear and patchy regions
of more marked hypointensity resulting from tumor
calcification and vascularity
• On T2 weighted images, these tumors may show a
very heterogeneous hyperintensity
• Cystic changes may be present within the lesion, and
hydrocephalus may be evident. Enlarged blood
vessels within the tumor may be seen as punctate and
curvilinear regions of hypointensity on both short and
long TR sequences
• Contrast enhancement throughout the tumor may be
heterogeneous in its depending on the degree of
tumor calcification, vascularity, and cystic change
present.
CHOROID PLEXUS CARCINOMA
• Uncommon choroid plexus tumors
• Occurrence in infants and children 2-4 years of age
• They arise in the lateral ventricles. They can infiltrate
the adjacent neural tissue. The regular architecture of
the choroids plexus is lost and cells show mitosis and
histologic atypia
• Clinical presentation is usually with features of
hydrocephalus.
Imaging
Features are nonspecific. Both benign and malignant
choroids plexus neoplasms show focal parenchymal
invasion. CSF dissemination can occur with both.
Imaging features do not distinguish benign from
malignant choroid plexus tumors.
BIBLIOGRAPHY
1. Bhatoe HS, Singh P, Dutta V. Intraventricular
meningiomas: A clinicopathological study and review.
Neurosurg Focus 2006;20:E9.
558 Section 6 Retrochiasmal Visual Pathway Lesions

2. Brown K, Mapstone TB, Oakes WJ. A modern analysis of
intracranial tumors of infancy. Pediatr Neurosurg
1997;26(1):25-32.
3. Do HM, Marx WF, Khanam H, Jensen ME. Choroid plexus
papilloma of the third ventricle: Angiography, preoperative
embolization, and histology. Neuroradiology 2001;43(6):
503-6.
4. Nejat F, Kazmi SS, Ardakani SB. Congenital brain tumors
in a series of seven patients. Pediatr Neurosurg 2008;44(1):
1-8.
5. Talacchi A, De Micheli E, Lombardo C, Turazzi S, Bricolo
A. Choroid plexus papilloma of the cerebellopontine
angle: A twelve patient series. Surg Neurol 1999;51(6):
621-9.

Fig. 42.9: A 10-year-old female with history of headache and

vomiting—axial CT scan of the brain showing a well-defined
markedly enhancing lesion in the left trigone with areas of
necrosis within (arrow)—choroid plexus papilloma

IV. MENINGIOMA
• Meningiomas are mostly benign tumors that originate
from the cells of the meninges, particularly, the
arachnoid cap cells. They may occur in the cranium
or within the spinal canal
• They form 13-18 percent of all primary intracranial
tumors. They are the most common primary nonglial
intracranial tumors
• They are most commonly detected in the middle and
late decades of life. They occur 2-3 times more
frequently in women than men
• Common sites for meningiomas include:
– Parasagittal/falcine (50%)
– Sphenoid wing (20%)
– Floor of the anterior cranial fossa (10%)
– Parasellar region (10%)
– Tentorium
– Cerebellopontine angle cistern region
• The common sella locations are suprasellar and para-
sellar, usually arising from the planum sphenoidale,
the tuberculum sella, or from the diaphragm
sellae. Occasionally, they may have a large intrasellar
component
• The cavernous sinus is commonly invaded
• On histopathology, whorls of calcium salts called
psammoma bodies may be seen in the transitional
type meningioma. Meningiomas contain both
estrogen and progesterone receptors and may become
symptomatic during pregnancy
• Multiple meningiomas are seen in 10 percent of the
patients and may be associated with neurofibro-
matosis, most of them remaining asymptomatic
during life
• Other associations are acoustic neuromas,
neurofibromas, gliomas, pituitary adenomas and
aneurysms
• They usually grow slowly, and they may produce
severe morbidity before causing death.
CLINICAL FEATURES
• Meningiomas produce their symptoms by several
mechanisms. They may cause symptoms by irritating
the underlying cortex, compressing the brain or the
cranial nerves, producing hyperostosis and/or
invading the overlying soft tissues, or inducing
vascular injuries to the brain
• Clinical features vary with the location of the tumor
Chapter 42 Intracranial Neoplasms 559
• Optic nerve: Proptosis, monocular decrease in vision
or blindness, relative afferent pupillary defect
monocular optic nerve head swelling or pallor with
optociliary shunt vessels
• Parasagittal: Monoparesis of the contralateral leg
• Subfrontal: Change in mentation, apathy or
disinhibited behavior, urinary incontinence
• Olfactory groove: Anosmia with possible ipsilateral
optic atrophy and contralateral papilledema (Foster
Kennedy syndrome), visual field defects, seizures, and
headache
• Tuberculum sellae (suprasellar): Compression of the
optic nerves and chiasm producing loss of vision, optic
atrophy and bitemporal/chiasmal or optic tract field
defects
• Cavernous sinus: Multiple cranial nerve deficits (II, III,
IV, V, VI), leading to decreased vision and diplopia
with associated facial numbness, Horner’s syndrome
• Occipital lobe: Contralateral hemianopsia
• Cerebellopontine angle: Decreased hearing with possible
facial weakness and facial numbness
• Spinal cord: Localized spinal pain, Brown-Sequard
(hemispinal cord) syndrome
• Sphenoid wing: Seizures; multiple cranial nerve palsies,
if the superior orbital fissure involved
• Tentorial: May protrude within supratentorial and
infratentorial compartments, producing symptoms by
compressing specific structures within these two
compartments
• Foramen magnum: Paraparesis, sphincter troubles,
tongue atrophy associated with fasciculation
• Intraventricular: Signs of raised intracranial pressure.
IMAGING STUDIES
• Plain skull radiograph may reveal hyperostosis,
increased vascular markings of the skull and
intracranial calcifications
• CT scan: Meningiomas may be isodense or slightly
hyperdense to the brain parenchyma. Isodense lesions
can be easily missed on a plain CT scan. Presence of
mass effect and adjacent bone changes such as
hyperostosis may help in identifying these lesions.
Suprasellar meningiomas may be difficult to
differentiate a pituitary adenoma from a meningioma
in the absence of calcification and expansion of the
sella. On postcontrast study, they demonstrate
moderate-to-marked contrast enhancement
560 Section 6 Retrochiasmal Visual Pathway Lesions
A B
A B
Figs 42.10A and B: Axial post-
contrast T1-weighted image (A and
B) showing an extra-axial left
cerebellopontine angle and petrous
apex lesion extending into the
cavernous sinus (arrows). The
lesion shows homogeneous
marked contrast enhancement.
Note: Postoperative changes in the
left temporal bone—recurrent
cerebellopontine angle and petrous
apex meningioma
Figs 42.11A and B: Axial CT scan
of the brain (A and B) showing
well-circumscribed lobulated mass
lesion with necrotic center and
extensive perilesional edema in
the left frontal parafalcine region.
Mass effect noted on the chiasm
(arrow)—atypical meningioma

• On MR imaging, they are usually isointense to the
gray matter on all sequences but can be hyperintense
T2-weighted images. Meningiomas enhance intensely
and homogeneously after injection of gadolinium
• MRI will help in identifying the pituitary gland
separately from the lesion in case of a suprasellar
meningioma. Dural tail sign may be occasionally seen
in parasellar meningiomas
• Gadolinium-enhanced scans should be obtained to
delineate the extent of the lesion and look for lesions
at other sites in the brain appearing simultaneously
• Multiple meningiomas may be difficult to differentiate
from dural metastasis in elderly patients
• The meningiomas may demonstrate a heterogeneous
intensity pattern that is most evident on the T2-
weighted images. Tumor heterogeneity can be related
to the presence of several factors: tumoral vascularity,
cystic foci, calcifications, and an inherent speckling
and mottling of uncertain etiology
• Cystic spaces is a feature seen in microcystic and
angioblastic varieties of meningioma. They appear on
T1-weighted images as smooth, rounded hypo-
intensities and hyperintense on the long TR/TE
images. Occasionally, subarachnoid cysts develop at
the margins of the tumor that at times may be difficult
to differentiate from the larger peripheral intratumoral
cysts
• Calcifications, when apparent on MRI, appear as
coarse, irregular regions of hypointensity on both T1-
and T2-weighted sequences
Several criteria help to establish the extracerebral
localization of the meningioma.
• A broad, dural-based margin is strongly suggestive,
but not definitive for this localization
• Bony hyperostosis and/or invasion are highly specific
for an extra-axial origin. However, it is infrequently
present
• Another highly specific characteristic for extra-axial
localization is the identification of various anatomic
interfaces interposed between the tumor surface and
the brain surface. Three different anatomic interfaces
may be identified with MRI. These consist of pial
vascular structures, CSF clefts, and dural margins.
With high-resolution and multiplanar MRI, one or
more of these interfaces can usually be identified in
essentially all cases
• In approximately two-thirds of the meningiomas,
vascular rims and CSF clefts are both present at the
brain-tumor interface
Chapter 42 Intracranial Neoplasms 561
• The dural margin interface is seen primarily in
meningiomas of the cavernous sinus. It appears as a
low-intensity rim on all imaging sequences covering
the lateral margin of the tumor and separating it from
the adjacent temporal lobe. Not infrequently, the
tumor can be seen invading through this dural margin
and abutting directly on adjacent brain
• Perilesional edema may be extensive and may be more
apparent on MRI than on CT scanning.
Recommended investigation: Contrast-enhanced MRI.
BIBLIOGRAPHY
1. Bonneville F, Savatovsky J, Chiras J. Imaging of
cerebellopontine angle lesions: An update. Part 2: intra-
axial lesions, skull base lesions that may invade the CPA
region, and non-enhancing extra-axial lesions. Eur Radiol
2007;17(11):2908-20.
2. Cappabianca P, Cirillo S, Alfieri A, et al. Pituitary
macroadenoma and diaphragma sellae meningioma:
differential diagnosis on MRI. Neuroradiology 1999;41(1):
22-6.
3. De Monte F, Al-Mefty O. Meningiomas. In: Kaye AH,
Laws ER (Eds). Brain Tumors: An Encyclopedic Approach.
Edinburgh, Scotland: Churchill Livingstone 1995.pp.
675-704.
4. Haddad GF, Al-Mefty O. Meningiomas: An overview. In:
Wilkins RH, Rengachary SS (Eds). Neurosurgery, 2nd edn.
Vol 1. New York, NY: McGraw-Hill 1996.pp.833-42.
5. Hashiba T, Hashimoto N, Izumoto S, Suzuki T, Kagawa
N, Maruno M, Kato A, Yoshimine T. Serial volumetric
assessment of the natural history and growth pattern of
incidentally discovered meningiomas. J Neurosurg 2009;
110(4):675-84.
6. Lusis E, Gutmann DH. Meningioma: An update (It has
been advocated as an effective management strategy for
small meningiomas and for meningiomas involving the
skull base or the cavernous sinus). Curr Opin Neurol 2004;
17(6):687-92.
7. Miller NR. New concepts in the diagnosis and
management of optic nerve sheath meningioma. J Neuro-
ophthalmol 2006;26(3):200-8.
8. Nagar VA, Ye J, Xu M, Ng WH, Yeo TT, Ong PL, Lim CC.
Multivoxel MR spectroscopic imaging-distinguishing
intracranial tumours from non-neoplastic disease. Ann
Acad Med Singapore 2007;36(5):309-13.
9. Nebbal M, Sindou M. Imaging for the management of
cavernous sinus meningiomas. Neurochirurgie 2008;
54(6):739-49.´
10. Vuèkovicc N, Kozicc D, Vulekovicc P, Vuèkovicc D, Ostojicc J,
Semnic R. MR and MRS characteristics of intraventricular
meningioma. J Neuroimaging 2010;20(3):294-6. Epub 2009
Jan 29.
562 Section 6 Retrochiasmal Visual Pathway Lesions

A B C

Figs 42.12A to C: A 40-year-old female with papilledema: (A) Axial T1-weighted image, (B) Sagittal T1-weighted image and
(C) Axial FLAIR showing a large rounded well-circumscribed lesion in the frontal region displacing the frontal horns and corpus
callosum. Note: The flow voids at the periphery, displaced anterior cerebral arteries (arrow)—anterior falx meningioma

A B

Figs 42.13A and B: Axial postcontrast CT brain (A and B) showing a large well-circumscribed homogeneously enhancing
hyperdense lesion with speckled calcification in the left lateral ventricle (arrows)—intraventricular meningioma. Note: Right shunt
tubes
 Chapter 42 Intracranial Neoplasms 563

Figs 42.14A and B: Coronal

postcontrast CT (A and B) brain
showing a large hyperdense
calcified lesion in the olfactory
groove (white arrows)—olfactory
A B groove meningioma

Figs 42.15A and B: A 40-year-old

male with history of loss of vision
in the right eye. Coronal
postcontrast T1-weighted images
(A and B) showing a lobulated
markedly enhancing lesion in the
right parasellar and temporal
region encasing the right
supraclinoid internal carotid artery
(black arrow) and intracranial optic
nerve. Note: Dural tail sign (white
A B arrow)
564 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 42.16A to D: A 25-year-old female presented with disc edema: (A) Coronal T1-weighted image, (B) Axial T1-weighted
image showing a well-circumscribed extra-axial lesion broad based to the parietal dura (black arrow) with buckling of the white
matter and CSF cleft (white arrow), (C) Axial FLAIR and (D) Coronal T2-weighted image showing the lesion to be hyperintense
with perilesional edema causing mass effect on the lateral ventricles—parietal convexity meningioma
 Chapter 42 Intracranial Neoplasms 565

Figs 42.17A and B: A 45-year-old

lady with headache: (A) Axial T2-
weighted image showing a small
extra-axial lesion on the left parietal
convexity appearing isointense to the
gray matter and (B) Axial postcontrast
T1-weighted image showing homo-
geneous enhancement of the
A B lesion—parietal meningioma

A B

Figs 42.18A to D: (A) Axial T1, (B)

Coronal T2 image showing a well-
defined isointense lesion in T1 and
minimally hyperintense signal in T2-
weighted images in the region of
planum sphenoidale, (C) Postcontrast
sagittal T1 and (D) Coronal T1 images
show homogeneous enhancement of
the lesion, also note the frontal
C D craniotomy defect
566 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 42.19A to D: (A) Axial FLAIR and (B) Coronal T2 images showing a homogeneous extraconal and temporal epidural lesion
displaying isointense signal with respect to the gray matter on T1 and T2 images and homogeneous enhancement after contrast
(C and D) Sphenoid wing meningioma in a patient presenting with proptosis of the left eye
 Chapter 42 Intracranial Neoplasms 567

Figs 42.20A and B: (A) Axial CT scan of the brain

in bone window setting showing hyperostosis of the
tuberculum sella (arrow) and (B) Coronal
postcontrast CT scan showing a well-circumscribed
rounded suprasellar homogeneously enhancing
lesion seen separately from the pituitary fossa—
A B tuberculum sella meningioma

A B

Figs 42.21A to C: A 50-year-old female presented with gradual loss of

vision in both eyes: (A) Sagittal T1-weighted image, (B) Coronal
T1-weighted image showing a well-circumscribed lobulated isointense
suprasellar mass lesion seen separately from the pituitary gland (black
arrow) and compressing the chiasm (dotted arrow) and (C) Axial FLAIR
C showing the lesion to be hyperintense—tuberculum sella meningioma
568 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.22A and B: (A) Postcontrast coronal CT scan showing a well-circumscribed homogeneously enhancing dural-based
lesion in the right temporal lobe and (B) Axial CT scan bone window showing hyperostosis of the adjacent bone (arrows)

Fig. 42.23: A 70-year-old male presented with right fourth nerve

palsy. Axial postcontrast T1-weighted image showing a well-
circumscribed lobulated enhancing lesion along the right
tentorium with dural tail sign (dotted arrow)

V. HEMANGIOPERICYTOMA
• Hemangiopericytoma is a rare tumor and accounts
for less than 1 percent of all primary CNS neoplasms
• It is a morphologic variant of meningioma with
aggressive behavior
• Meningeal hemangiopericytoma is considered by
some to be a subtype of angioblastic meningioma.
However, it arises from pericytes and not from
meningothelial arachnoid cap cells
• It is a highly vascularized tumor with numerous
penetrating blood vessels.
CLINICAL FEATURES
• Its onset is usually around 40 years with a male
preponderance
• The tumor may be benign or malignant or share
characteristics of both
• Orbit and meninges are rare sites of involvement
• Patients with involvement of the orbit may present
with decreased visual acuity, diplopia and pain.
Puffiness of eyelids with a bluish or reddish
discoloration of the ocular adnexa, progressive
unilateral proptosis with downward displacement
of globe are other features
• Intracranial lesions may cause visual field defects,
sensory defects and cranial nerve palsies
• They also have a strong propensity for extraneural
metastasis particularly to the lung and bones, hence
patient should be on regular follow-up.
IMAGING
CT
They are heterogeneous appearing lesions on both plain
and postcontrast CT. Low density cystic or necrotic areas
Chapter 42 Intracranial Neoplasms 569
are common. Strong but inhomogeneous enhancement
is seen with contrast. Bony erosion may be seen.
MRI
The findings are variable. Signs of an extradural mass
are often seen. They are isointense with cortex on T1-
weighted images and slightly hyperintense on proton
density weighted images. It shows strong but inhomo-
geneous enhancement on postcontrast scans. Prominent
vascular channels are frequently identified.
Angiography
They are hypervascular lesions that typically have
prolonged, dense, but heterogeneous tumor stain.
Arteriovenous shunting with early draining veins is
uncommon. Mixed dural-pial vascular supply is typical.
BIBLIOGRAPHY
1. Akiyama M, Sakai H, Onoue H, Miyazaki Y, Abe T.
Imaging intracranial haemangiopericytomas: Study of
seven cases. Neuroradiology 2004;46(3):194-7.
2. Alen JF, Lobato RD, Gomez PA, Boto GR, Lagares A,
Ramos A, Ricoy JR. Intracranial hemangiopericytoma:
Study of 12 cases. Acta Neurochir (Wien) 2001;143(6):
575-86.
3. Ruscalleda J, Feliciani M, Avila A, Castaner E, Guardia E,
de Juan M. Neuroradiological features of intracranial and
intraorbital meningeal haemangiopericytomas.
Neuroradiology 1994;36(6):440-5.
4. Uttley D, Clifton AC, Wilkins PR. Haemangiopericytoma:
A clinical and radiological comparison with atypical
meningiomas. Br J Neurosurg 1995;9(2):127-34.
570 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.24A and B: (A) Axial postcontrast CT scan and (B) Coronal postcontrast CT scan of the brain showing a heterogeneous
brilliantly enhancing left parietal lesion broad based to the partial convexity—HPE hemangiopericytoma

VI. PINEAL GLAND TUMORS
These are uncommon tumors with an incidence of <1
percent of all intracranial neoplasms.
CLINICAL FEATURES
1. Hydrocephalus due to aqueductal compression
2. Endocrine abnormalities
3. Parinaud’s dorsal midbrain syndrome (upgaze palsy,
light near dissociation, convergence abnormalities)
TYPES OF TUMORS
1. Germ cell tumors (germinoma, teratoma)
2. Tumors from pineal parenchymal cells (pinealoma,
pinealoblastoma)
3. Astrocytomas and meningiomas usually extend from
adjacent brainstem.
PINEALOMA
• They are tumors arising from pineal parenchymal cells
(neuroepithelial cells). They are rare tumors
• They are of two types—Pineoblastoma—composed
of immature undifferentiated cells which can show
focal hemorrhage and microscopic necrosis.
Pineocytomas—composed of more differentiated
tumor cells which resemble normal cells in pineal
gland. An intermediate form has also been described
which is composed of both mature and immature cells.
Both can coexist within same tumor. Both contain
melanin
• Pineoblastomas are usually seen in the first decade
and rarely in adults. Pineocytomas are common in
the third decade (usually in adults and elderly). No
gender predilection is seen
• A pinealoma is usually a well-defined mass at the
location of the pineal gland. It may bulge into the
posterior part of the third ventricle or compress the
dorsal mesencephalon and the cerebral aqueduct
• Pineocytomas are slow-growing tumors, are less
cellular and highly vascular. They are well-defined
tumors and do not show infiltration
• Some pineocytomas and all pineoblastomas show
malignant behavior. They may metastasize through
CSF pathways and in the subarachnoid space, they
may infiltrate extensively with leptomeningeal and
subependymal seeding. The prognosis in children
with pinealoblastoma is poor
• Pineoblastoma may be associated with bilateral
retinoblastoma (trilateral retinoblastoma). This is an
Chapter 42 Intracranial Neoplasms 571
inherited syndrome and this diagnosis should be
sought in any child with bilateral retinoblastoma.
Symptoms and Signs
Cases may present with signs of raised intracranial
tension (headache and papilledema), complete or partial
dorsal midbrain syndrome, nerve palsies—third nerve,
fourth nerve, diplopia, skew deviation, nystagmus—
conjugate, dysconjugate, saccadic intrusions, cerebellar
signs, endocrine abnormalities or meningoencephalitis.
Imaging
MRI
• The histological types may not be demonstrated on
MRI. The more important differentiation is between
a pineal neoplasm and a cyst
• Pineal neoplasms are lobulated, solid tumors that
enhance well with contrast
• Cystic lesions should be considered pineal cysts
• Signal intensities vary but pinealoblastomas are
usually isointense to gray matter on long TR/TE spin
echoes. This feature is also seen in PNET tumors due
to paucity of cytoplasm and dense cellularity. The
other differential includes germinoma
• Pineocytomas show higher signal intensity with long
TR/TE sequences
• Both tumors can calcify but intratumoral calcification
is more common with pineocytomas
• Pineal cysts: These are common incidental findings
seen in 40 percent of necropsy specimens. Even large
cysts that compress the dorsal midbrain can be
virtually asymptomatic
• But pineal cysts can develop internal bleeding and
can cause aqueductal compression with secondary
hydrocephalus
• It is important to recognize it as benign and
differentiate it from a pineal neoplasm.
BIBLIOGRAPHY
1. Chang SM, Lillis-Hearne PK, Larson DA, Wara WM,
Bollen AW, Prados MD. Pineoblastoma in adults.
Neurosurgery 1995; 37(3):383-90; discussion 390-1.
2. Gouliamos AD, Kalovidouris AE, Kotoulas GK,
Athanasopoulou AK, Kouvaris JR, Trakadas SJ, Vlahos
LJ, Papavasiliou CG. CT and MR of pineal region tumors.
Magn Reson Imaging 1994;12(1):17-24.
572 Section 6 Retrochiasmal Visual Pathway Lesions
A B
A B
A B
Figs 42.25A and B: (A) Coronal CT
and (B) Axial CT of the brain showing
well-defined homogeneous enhancing
lesion in the pineal region with
obstructive hydrocephalus
Figs 42.26A and B: Contrast-
enhanced: (A) Axial MRI and
(B) Sagittal MRI of brain showing a
well-enhancing lobulated lesion in the
pineal region suggestive of pinealo-
blastoma
Figs 42.27A and B: (A) Axial FLAIR
and (B) Sagittal T1 image showing a
well-defined cystic lesion in the pineal
gland region suggestive of pineal cyst
that was noted incidentally. Empty
sella is also noted

3. Herrick MK, Rubinstein LJ. The cytological differentiating
potential of pineal parenchymal neoplasms (true
pinealomas): A clinicopathological study of 28 tumours.
Brain 1979;102(2):289-320.
4. Korogi Y, Takahashi M, Ushio Y. MRI of pineal region
tumors. J Neurooncol 2001;54(3):251-61. Review.
5. Marcus DM, Brooks SE, Leff G, McCormick R, Thompson
T, Anfinson S, Lasudry J, Albert DM. Trilateral retino-
blastoma: Insights into histogenesis and management.
Surv Ophthalmol 1998;43(1):59-70. Review.
Chapter 42 Intracranial Neoplasms 573
6. Muller-Forell W, Schroth G, Egan PJ. MR imaging in tumors
of the pineal region. Neuroradiology 1988;30(3):224-31.
7. Satoh H, Uozumi T, Kiya K, Kurisu K, Arita K, Sumida
M, Ikawa F. MRI of pineal region tumours: Relationship
between tumors and adjacent structures. Neuroradiology
1995;37(8):624-30.
8. Smirniotopoulos JG, Rushing EJ, Mena H. Pineal region
masses: Differential diagnosis. Radiographics 1992;12(3):
577-96. Review.
9. Tien RD, Barkovich AJ, Edwards MS. MR imaging of
pineal tumors. Am J Roentgenol 1990;155(1):143-51.
574 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.28A to C: (A) Axial T1 image, (B) Sagittal T1 image and

(C) Coronal T1 image showing a well-defined pineal cyst in a 26-year-
old girl who presented with bilateral ptosis and upgaze palsy
C suggestive of dorsal midbrain compression

VII. ACOUSTIC SCHWANNOMAS
• Vestibulocochlear schwannomas are the most
common cerebellopontine angle (CPA) mass and
account for 3/4th of lesions in this location. Acoustic
schwannomas constitute approximately 7 to 8 percent
of all primary intracranial neoplasms
• They most frequently arise from the vestibular portion
of the 8th cranial nerve. These are seen in association
with neurofibromatosis type II disease, in which they
are typically bilateral. The tumors generally involve
the intracanalicular portion of the nerve and in most
instances also demonstrate a mass in the
cerebellopontine angle.
CLINICAL FEATURES
Acoustic schwannomas are more common in men.
• Stage 1: Slowly progressive neurosensory hearing
loss, vertigo, imbalance, tinnitus
• Stage 2: Alteration in facial sensation, facial pain and
headache, trigeminal sensory neuropathy (decreased
corneal reflex, trigeminal neuralgia)
• Stage 3: Constant, severe and widespread cranial
nerve deficits—diplopia, restriction of eye movement,
nystagmus (vestibular, gaze paretic and Brun’s
nystagmus) with oscillopsia, ocular motor nerve
dysfunction, weakness of muscles of mastication, loss
of taste, dysphagia, hoarseness, hemiatrophy of
tongue, skew deviation, horizontal gaze paresis,
ocular dysmetria, cerebellar ataxia (signs of cerebellar
dysfunction), contralateral hemiparesis, hypereflexia
and altered mental status
• Stage 4: Symptoms of increased intracranial tension
occur, if the tumor hampers the normal flow of CSF
or occludes one of the dural sinuses, producing
papilledema or postpapilledemic optic atrophy.
IMAGING
• On MRI, thin-section (3 mm) axial T1-weighted
images will reveal most tumors that are generally
larger than 3 mm in size. The tumors are usually well
demarcated from adjacent CSF on these images and
reveal mild hypointensity
• On long TR images, the tumors are usually
heterogeneously hyperintense. On the long-echo
image, the hyperintensity of the CSF may obscure the
lesion because of similarities in intensity
• Cystic degeneration within the tumor causes hetero-
geneous appearance appreciated on both T1 and T2-
weighted images
Chapter 42 Intracranial Neoplasms 575
• Tumors with marked vascularity or focal nodular
calcifications will reveal significant heterogeneity.
Cystic and/or hemorrhagic regions on MRI is more
typical of acoustic schwannoma than meningioma
• Small tumors that are confined to the internal auditory
canal are best demonstrated on postcontrast study or
using newer sequences such as fast imaging in steady
state aquisition (FIESTA) by GE or constructive
interference in steady state (CISS) by Siemens
• Contrast enhancement may show extension of
cisternal tumor into the canal in the absence of canal
expansion. The contrast enhancement of the tumor
may be homogeneous but is frequently inhomo-
geneous.
• Acoustic schwannomas that do not have an
intracanalicular extension and homogeneously
enhance postcontrast may be difficult to differentiate
from meningioma.
BIBLIOGRAPHY
1. Butti G, Giordana MT, Paoletti P, Schiffer D. Multiple
primary intracranial tumors of different cell types: Asso-
ciation of anaplastic astrocytoma and acoustic neurinoma
with review of the literature. Surg Neurol 1982;18(5):
336-4.
2. Mulhern MG, Aduriz-Lorenzo PM, Rawluk D, Viani L,
Eustace P, Logan P. Ocular complications of acoustic
neuroma surgery. Br J Ophthalmol 1999;83(12):1389-92.
3. Obholzer RJ, Rea PA, Harcourt JP. Magnetic resonance
imaging screening for vestibular schwannoma: Analysis of
published protocols. J Laryngol Otol 2004;118(5):329-32.
4. Rodriguez D, Young Poussaint T. Neuroimaging findings
in neurofibromatosis type 1 and 2. Neuroimaging Clin N
Am 2004;14(2):149-70, vii. Review.
5. Rogers NK, Brand CS. Acoustic neuroma and the eye. Br
J Neurosurg 1997;11(4):292-7.
6. Rosenstock TG, Hurwitz JJ, Nedzelski JM, Tator CH.
Ocular complications following excision of cerebello-
pontine angle tumours. Can J Ophthalmol 1986;21(4):
134-9.
7. Swartz JD. Lesions of the cerebellopontine angle and
internal auditory canal: Diagnosis and differential
diagnosis. Semin Ultrasound CT MR 2004;25(4):332-52.
Review.
8. Van Meter WS, Younge BR, Harner SG. Ophthalmic
manifestations of acoustic neurinoma. Ophthalmology
1983;90(8):917-22.
9. Wright A, Bradford R. Management of acoustic neuroma.
BMJ 1995;311(7013):1141-4. Review.
10. Yoshimoto Y. Systematic review of the natural history of
vestibular schwannoma. J Neurosurg 2005;103(1):59-63.
Review.
576 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 42.29A to D: (A) Axial T2-weighted image, (B) Axial T1-weighted image showing T1 hypointense lesions in the bilateral
cerebellopontine angles (white arrows) with extension into the internal auditory canals (arrow), (C) Axial postcontrast T1-weighted
image and (D) Coronal postcontrast T1-weighted image showing homogeneous marked contrast enhancement

VIII. CNS METASTASIS
• Cerebral metastases are found in 25 percent patients
with systemic malignancy at autopsy
• Any patient with a systemic malignancy who presents
with a new neurologic or visual deficit should be assumed
to have metastatic disease until proven otherwise
• Up to one-third of patients presenting with brain
metastases do not have a known primary
• CNS metastases can involve brain parenchyma,
leptomeninges or skull bones. Spinal cord metastases
are rare
• CNS metastases can occur from several extracranial
primary tumors. Hematogenous spread is the most
common source. Most common primary sites to
metastatize are breast, bronchus, colon and thyroid.
Direct extension of tumor, e.g. from skull-base malig-
nancy and nasopharyngeal tumors can also occur
• The distribution of brain metastases parallels regional
cerebral blood flow. They are commonly located at
gray-white matter junctions and at the border zone
between middle cerebral and posterior cerebral artery
distribution.
CEREBRAL PARENCHYMAL METASTASES
• Usually, they are seen in adults and are uncommon
in children
• Parenchymal metastases accounts for 1/4th to 1/3rd
of all brain tumors
• Patients can be asymptomatic and lesions may be
identified during whole body screening
• Radiotherapy, stereotactic radiosurgery, chemo-
therapy and surgical therapy may all play a role in
management, individually or in combination.
Etiology
Common tumors to metastasize to brain include:
• Lung malignancies (Most common primary site for
brain parenchymal metastases)
• Breast malignancies
• Malignant melanoma
• Gastrointestinal tumors
• Thyroid cancer.
IMAGING
CT Scan
• Usually, they are isodense or hypodense with brain
at gray-white interface. Hyperdense metastasis is seen
with round cell tumors and melanoma
Chapter 42 Intracranial Neoplasms 577
• The metastasis from renal and breast carcinomas,
melanoma and choriocarcinomas are hypervascular
and therefore can bleed
• Cystic and calcified untreated metastasis can occur
from breast and lung cancers and are quite rare.
Edema associated with metastasis can be striking.
With contrast, these lesions usually show strong
enhancement. Both solid and ring patterns can be noted.
MRI
• Signal intensity may vary. They are usually
hypointense to brain on T1-weighted image
• Some nonhemorrhagic metastasis from malignant
melanomas usually are hyperintense on T1 due to the
melanin content. These lesions are usually hypo-
intense on T2-weighted scans
• Multifocal white matter and corticomedullary lesions
on T2-weighted scans are common in metastasis and
may resemble punctate high signal foci seen in elderly
patients
• Most but not all, however, enhance well with contrast.
Solid, rim and mixed enhancement patterns occur.
Multifocal lesions located at gray-white junctions are
typical.
SKULL AND DURAL METASTASES
• Calvarial metastases usually come from breast and
lung cancers. Lesions range from subtle intradiploic
metastasis to large masses with extensive areas of bone
destruction
• Calvarial metastasis can involve the adjacent dura.
Focal dural metastasis without associated calvarial
metastasis can occur. However, dural metastasis is
less common than calvarial metastasis
• The dura matter is an effective barrier to tumor invasion
of the brain. Calvarial metastases, however, can produce
neurological symptoms by compression of adjacent
brain tissue or cranial nerves as they pass through skull
foramina. They may also obstruct venous sinuses
• CT bone window can detect the bony lesions
• Contrast-enhanced MRI is best to locate subtle
intradiploic lesion delineating the extent and
determining the involvement of dura or brain.
LEPTOMENINGEAL METASTASES
• These can be diffuse or widespread or focal
• The basal cisterns are the most common sites
578 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.30A to C: (A and B) Axial contrast-enhanced CT scan of the brain showing multiple ring enhancing
lesions in the frontal, parietal and temporal lobe with extensive perilesional edema and (C) Axial CT scan of the
chest of the same patient shows a necrotic mass in the right middle lobe

• Metastases to the meninges is via the bloodstream.
Rarely, a superficially located parenchymal metastasis
can shed cells directly into the subarachnoid space
• Breast carcinoma, lung carcinoma and melanoma are
the common tumors to metastasize to leptomeninges
• Low-grade meningitis, with symptoms and signs of
damage to cranial nerves at the base of the brain, is
seen. Focal brainstem signs or diffuse encephalopathy
may also develop
• Carcinomatous encephalitis occurs when there is
diffuse tumor spread to cortex and meninges without
formation of a macroscopic mass
• Subtle leptomeningeal and subarachnoid lesions can
be identified on contrast-enhanced MR imaging
• Pial metastases can occur with arachnoid and
subarachnoid metastases
• A cerebrospinal fluid analysis reveals the malignant
cells
DIFFERENTIAL DIAGNOSIS
• Abscess
• Primary neoplasm (glioblastoma multiforme)
• Demyelinating lesions
• Multifocal white matter lesions.
BIBLIOGRAPHY
1. Anand AK, Potts DG. Calcified brain metastases: Demons-
tration by computed tomography. Am J Neuroradiol 1982;
3(5):527-9.
2. Floeter MK, So YT, Ross DA, Greenberg D. Miliary
metastasis to the brain: Clinical and radiologic features.
Neurology 1987; 37(11):1817-8.
Chapter 42 Intracranial Neoplasms 579
3. Gaviani P, Mullins ME, Braga TA, Hedley-Whyte ET,
Halpern EF, Schaefer PS, Henson JW. Improved detection
of metastatic melanoma by T2*-weighted imaging. Am J
Neuroradiol 2006;27(3):605-8.
4. Lassman AB, DeAngelis LM. Brain metastases. Neurol
Clin 2003;21(1):1-23, vii. Review.
5. Lopez-Pousa S, Ojeda B, Lopez-Lopez JJ, Guardia E,
Ruscalleda J, Grau Veciana JM. The correlation between
clinical symptomatology and computerized tomography
in brain metastasis secondary to breast and lung
neoplasias. Comput Tomogr 1981;5(1):17-23.
6. Nayak L, Abrey LE, Iwamoto FM. Intracranial dural
metastases. Cancer 2009;115(9):1947-53.
7. Okamoto K, Ito J, Saito T, Usuda H, Furusawa T, Sakai K,
Tokiguchi S. CT and MR imaging of the "target sign" in
metastatic brain disease. Eur Radiol 2000;10(1):154-6.
8. Posner JB. Diagnosis and treatment of metastases to the
brain. Clin Bull 1974;4(2):47-57.
9. Routh A, Khansur T, Hickman BT, Bass D. Management
of brain metastases: Past, present, and future. South Med J
1994;87(12):1218-26. Review.
10. Ruscalleda J, Lopez-Pousa S, Guardia E, Arroyo I, Roig C,
Faixat J. Clinical symptomatology and computerized
tomography in brain metastasis. Comput Tomogr
1978;2(2):69-77.
11. Schouten LJ, Rutten J, Huveneers HA, Twijnstra A.
Incidence of brain metastases in a cohort of patients with
carcinoma of the breast, colon, kidney, and lung and
melanoma. Cancer 2002;94(10):2698-705.
12. Tang YM, Ngai S, Stuckey S. The solitary enhancing
cerebral lesion: Can FLAIR aid the differentiation between
glioma and metastasis? Am J Neuroradiol 2006;27(3):
609-11.
580 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.31A to C: A 64-year-old lady with known carcinoma thyroid

presented with headache and seizures: (A) Coronal T2-weighted
image, (B) Axial T1-weighted image and (C) Axial FLAIR showing
a rounded well-circumscribed lesion with necrotic center and
hyperintense layering of fluid noted in T1-weighted image
C suggestive of blood (arrow)
 Chapter 42 Intracranial Neoplasms 581

A B C

D E

F G

Figs 42.32A to G: (A to C) Axial FLAIR images showing multiple rounded nodular lesions noted in the
supratentorial brain parenchyma and midbrain, (D) Axial postcontrast T1-weighted image, (E) Coronal
postcontrast T1-weighted image, (F) Sagittal postcontrast T1-weighted image showing multiple nodular
and ring-enhancing lesions cortex and corticomedullary junction and (G) X-ray chest of the patient shows
an ill-defined lesion in the left perihilar region suggestive of bronchogenic carcinoma with metastases
582 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.33A and B: (A) Axial CT scan in soft tissue and (B) Bone window settings in a patient with known carcinoma thyroid. The
contrast-enhanced CT scan shows a expansile hyperdense enhancing epidural and extracranial soft tissue lesion eroding the
bone

IX. PRIMARY CNS LEUKEMIA
• Primary CNS leukemia is extremely rare
• Granulocytic sarcoma is a solid tumor composed
of immature granulocytes that is associated with
systemic leukemia, usually acute myelogenous
leukemia
• Although granulocytic sarcoma can precede the
development of leukemia by a few months, its
occurrence without systemic leukemia is rare.
IMAGING (MRI)
• Usually, an enhancing dural-based mass is seen
• Occasionally, the infundibular stalk and hypo-
thalamus are affected.
Chapter 42 Intracranial Neoplasms 583
BIBLIOGRAPHY
1. Nishiyama T, Johkura K, Johmura Y, Momoo T, Yamada H,
Kuroiwa Y. Encephalitis with MRI abnormality as a
manifestation of central nervous system involvement of adult
T cell leukemia/lymphoma. Eur Neurol 2001;46(4): 218-20.
2. Kitajima M, Korogi Y, Shigematsu Y, Liang L, Matsuoka
M, Yamamoto T, Jhono M, Eto K, Takahashi M. Central
nervous system lesions in adult T-cell leukemia: MRI and
pathology. Neuroradiology 2002;44(7):559-67. Epub 2002
May 30.
3. Taguchi H, Sonobe H, Yamato K, Takeuchi T, Ookawa K,
Kodama H, Ohtsuki Y, Miyoshi I. Central nervous system
involvement of adult T-cell leukemia-lymphoma with
multinucleated giant cells in the brain, skin, and kidney.
Cancer 1993;71(1):133-7.
584 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

E F

Figs 42.34A to F: A 7-year-old child with known leukemia presented with right 6th nerve
palsy: (A) Axial CT scan, (B) Coronal reformation showing a well-marginated homogeneous
enhancing extra-axial dural based lesion in the right middle cranial fossa and paracavernous
region, (C) Axial T1-weighted image and (D) Coronal T2-weighted image showing a well-
defined dural-based lesion in the right middle cranial fossa isointense in T1-weighted image
and hypointense in T2-weighted image and (E and F) Postcontrast T1-weighted images
showing homogeneous enhancement
 Chapter 42 Intracranial Neoplasms 585

A B

Figs 42.35A and B: Axial CT scan of the brain of the above patient showing complete resolution of the lesion postchemotherapy
586 Section 6 Retrochiasmal Visual Pathway Lesions
X. PRIMARY CNS LYMPHOMA
Primary CNS lymphoma presents without evidence of
systemic lymphoma.
INCIDENCE
• It constitutes only about 1 percent of all primary brain
tumors. However, its incidence is increasing in
immunocompromised as well as in immuno-
competent individuals
• AIDS-related primary CNS lymphoma is now more
common than low-grade astrocytomas and as
common as meningioma. Some estimates state that it
has become the most common primary brain
neoplasm, mainly as a result of its frequent occurrence
in the AIDS population
• The groups of people who are at risk of developing
primary CNS lymphoma are:
1. Organ transplant recipients
2. Patients with congenital immunodeficiency
syndromes
3. Those with AIDS and other systemic diseases
associated with immunodeficiency.
SITES OF INVOLVEMENT
• The site of origin of primary CNS lymphoma remains
controversial and unknown, since the CNS does not
have endogenous lymphoid tissue or a lymphatic
circulation
• Focal intracerebral mass is the most common initial
presentation of primary CNS lymphoma in immuno-
competent patients
• In immunocompromised states, the tumor is likely to
be multicentric. The deep basal ganglia, peri-
ventricular regions and corpus callosum are common
sites
• The subarachnoid space is a common site for recurrent
disease
• The supratentorial compartment is involved in
approximately 75 to 85 percent of patients at initial
presentation.
HISTOLOGY
• Primary CNS lymphomas are B cell malignancies of
intermediate to high grade
• Leptomeningeal involvement is present in about 50
percent of cases at some time during the course of the
illness
• The infiltrative edges of the lesions extend along
perivascular spaces and infiltrate blood vessel walls.
SYMPTOMS
Symptoms vary in severity from headaches, focal
neurological deficits to seizures, and stupor.
IMAGING
CT Scan
Ninety percent are moderately hyperdense on CT and
show homogeneous enhancement with contrast. Non-
enhancing tumors are rare and ring enhancement occurs
in immunocompromised individuals.
MRI
• Most focal lymphomas are isointense to slightly
hypointense compared to gray matter on T1-weighted
images and iso- to slightly hyperintense on T2-
weighted images
• The classical imaging findings of parenchymal
lymphoma include masses which involve the deep
gray matter structures, periventricular regions, and
corpus callosum
• It has been reported that up to 75 percent of
lymphomatous masses are seen to be in contact with
ependyma, meninges, or both
• Presence of enhancement along perivascular spaces
on MRI should indicate lymphoma. Differential
diagnosis of sarcoidosis should also be considered
• The extent of edema on MRI is generally less than
that seen in conjunction with primary gliomas or
metastases of similar size. Lymphomatous masses do
not calcify and hemorrhage is distinctly uncommon
on imaging studies
• MRI signal intensity patterns are extremely varied in
focal intracerebral lymphoma, particularly in the AIDS
population. These lesions, when deep and
periventricular, are often isointense to gray matter on
all spin-echo sequences, a finding shared by other
small cell, hypercellular tumors. However, lymphoma
can also be markedly hyperintense on long TR images
• The lymphomas that occur in immunocompetent
patients enhance strongly and homogeneously with
contrast. In AIDS patients, the lesions may appear
heterogeneous with hemorrhage and necrotic foci and

necrotic lesions can show ring enhancement. Reports
indicate that lymphoma in AIDS patients more
commonly appears as multifocal lesions with ring
enhancement and with more prominent edema than
in the general population
• Secondary brain involvement by systemic lymphoma
is indistinguishable from primary CNS lymphoma on
imaging studies, although, as noted above, paren-
chymal involvement is more common in primary
lymphoma. Leptomeningeal seeding, in the absence of
parenchymal involvement, may be difficult to detect
by CT scanning, MRI with IV contrast is more sensitive.
DIFFERENTIAL DIAGNOSIS
• The single most valuable MR finding in the distinction
between lymphoma and toxoplasmosis in AIDS is the
presence of subependymal spread, which is highly
suggestive of lymphoma. Hemorrhage is also very
common in cerebral toxoplasmosis, particularly after
treatment
• If a focal-enhancing parenchymal mass is accompanied
by enhancement along the perivascular spaces,
especially with adjacent meningeal enhancement.
Differential diagnosis includes lymphoma and
sarcoidosis
• If the patient is HIV negative, then other lesions such
as glioblastoma, metastases, sarcoidosis and
cavernous hemangiomas should be considered
Chapter 42 Intracranial Neoplasms 587
• Densely enhancing dural-based lesions could
implicate lymphoma, sarcoidosis, metastases
(especially breast carcinoma), and meningioma.
BIBLIOGRAPHY
1. Buhring U, Herrlinger U, Krings T. MRI features of
primary central nervous system lymphomas at
presentation. Neurology 2001;57(3):393-6.
2. Jahnke K, Schilling A, Heidenreich J, Stein H, Brock M,
Thiel E, Korfel A. Radiologic morphology of low-grade
primary central nervous system lymphoma in immuno-
competent patients. Am J Neuroradiol 2005;26(10):
2446-54.
3. Liao W, Liu Y, Wang X, Jiang X, Tang B, Fang J, Chen C,
Hu Z. Differentiation of primary central nervous system
lymphoma and high-grade glioma with dynamic
susceptibility contrast-enhanced perfusion magnetic
resonance imaging. Acta Radiol 2009;50(2):217-25.
4. Paul T, Challa S, Tandon A, Panigrahi M, Purohit A.
Primary central nervous system lymphomas: Indian
experience, and review of literature. Indian J Cancer
2008;45(3):112-8.
5. Bühring U, Herrlinger U, Krings T, Thiex R, Weller M,
Küker W. MRI features of primary central nervous system
lymphomas at presentation. Neurology 2001;57: 393-6.
6. Kuker W, Nagele T, Thiel E, Weller M, Herrlinger U.
Primary central nervous system lymphomas (PCNSL):
MRI response criteria revised. Neurology 2005;65(7):
1129-31.
588 Section 6 Retrochiasmal Visual Pathway Lesions

A B

Figs 42.36A and B: (A) Coronal postcontrast T1-weighted MRI and (B) Axial postcontrast T1-weighted MRI of the brain showing
bilateral dural based lesion in the cerebellopontine angle and right juxtaventricular region—lymphoma

A B

Figs 42.37A and B: Axial postcontrast CT scan of the brain at the level of the lateral ventricles (A and B) showing homogeneous
enhancing masses in the periventricular region. Note: Central hyperdensity due to hemorrhage from stereotactic biopsy—primary
CNS lymphoma
 Chapter 42 Intracranial Neoplasms 589

A B

Figs 42.38A to C: (A) Axial T2-weighted image and (B and C) Sagittal

T1-weighted images showing frontal extracranial soft tissue swelling
with bony erosion and extension into the frontal epidural space.
C HPE—lymphoma
590 Section 6 Retrochiasmal Visual Pathway Lesions
XI. COLLOID CYST
• Colloid cyst is a benign tumors of unknown cellular
origin
• This tumor is almost always found in the third
ventricle and can be associated with obstructive
hydrocephalus and increased intracranial pressure
• Although it is considered congenital, its presentation
in childhood is rare. The tumor usually presents in
individuals aged 20-50 years. Approximately, 15-
20 percent of all intraventricular masses are colloid
cyst.
CLINICAL FEATURES
• Colloid cyst can obstruct CSF flow. Intermittent
obstruction can result in recurrent episodes of acute
hydrocephalus with signs and symptoms of raised
intracranial pressure
• Vertigo, decreased memory, and behavioral changes
may be associated. In addition, sudden weakness in
the lower limbs resulting in falls without loss of
consciousness has been reported. Sudden death has
also been rarely reported.
IMAGING STUDIES
CT Scan
• Colloid cyst appears homogeneous, with two-third
of them appearing hyperdense to surrounding
parenchyma and one-third appearing isodense to
surrounding parenchyma
• The lesions are well delineated and are usually round
or ovoid. Occasionally, they may have a thin rim of
enhancement after contrast injection, but they are
typically nonenhancing and uncalcified
• The viscosity of cyst contents correlates more closely
to the radiodensity visible on a CT scan, hence they
are easily visible on CT scan
• The viscosity of cyst contents determines the most
appropriate surgical approach. A hyperdense cyst is
more likely to have solid contents and is more difficult
to drain.
MRI
• The appearance of colloid cyst on MRI is variable. The
most common appearance is hyperintense on T1 and
hypointense on T2. The amount of rim enhancement
is variable
• The variable MRI signals do not correlate with the
fluid density of cyst contents. MRI is valuable in
differentiating a colloid cyst from an aneurysm, which
may have a similar appearance on a CT scan
• Recognizing CSF flow artifact at the Monro foramen
from a colloid cyst is important.
BIBLIOGRAPHY
1. Akins PT, Roberts R, Coxe WS, Kaufman BA. Familial
colloid cyst of the third ventricle: Case report and review
of associated conditions. Neurosurgery 1996; 38(2):392-5.
2. Buttner A, Winkler PA, Eisenmenger W, Weis S. Colloid
cysts of the third ventricle with fatal outcome: A report of
two cases and review of the literature. Int J Legal Med
1997;110(5):260-6.
3. Camacho A, Abernathey CD, Kelly PJ, Laws ER. Colloid
cysts: Experience with the management of 84 cases since
the introduction of computed tomography. Neurosurgery
1989;24(5):693-700.
4. Kumar S, Singh AK, Sachdev M. Colloid cyst of third
ventricle: A study of 11 cases. J Indian Med Assoc 1998;
96(11):351,353.
5. Pollock BE, Huston J. Natural history of asymptomatic
colloid cysts of the third ventricle. J Neurosurg 1999;
91(3):364-9.
6. Pollock BE, Schreiner SA, Huston J. A theory on the natural
history of colloid cysts of the third ventricle. Neurosurgery
2000; 46(5):1077-81; discussion 1081-3.
7. Urso JA, Ross GJ, Parker RK, et al. Colloid cyst of the third
ventricle: Radiologic-pathologic correlation. J Comput
Assist Tomogr 1998;22(4):524-7.
 Chapter 42 Intracranial Neoplasms 591

Fig. 42.39: Axial postcontrast CT scan of the brain showing a

well-defined hyperdense lesion in the anterior third ventricle
resulting in mild hydrocephalus

A B

Figs 42.40A and B: (A) Mid-sagittal T1-weighted image and (B) Axial FLAIR image at the level of the foramen of Monro showing
a well-defined oval T1-hyperintense lesion at the anterior third ventricular level with moderate obstructive hydrocephalus—
characteristic of colloid cyst
592 Section 6 Retrochiasmal Visual Pathway Lesions
XII. DERMOID CYSTS
• Dermoid cysts represent a congenital abnormality
resulting from the inclusion of ectodermal elements
at the time of neural tube closure or during the
formation of secondary cerebral vesicles
• Dermoid cyst is a rare tumor, which comprises less
than 1 percent of primary intracranial tumors.
Intracranially, dermoid cyst is much less common
than epidermoid cyst.
PATHOLOGY
• Dermoid cysts are choristomas (tumor-like lesions
composed of normal tissues, the elements of which
are not found normally at the site of involvement)
• Dermoid is a large single or multiloculated cystic
cavity containing keratin and inwardly directed lining
of stratified squamous cell epithelium
• Dermoid cyst can contain all elements of the dermis
including epidermis, hair follicles, sweat glands, and
sebaceous glands. Calcification may develop in the
portion of the walls, and there may be bone and
cartilage within some of the cysts
• Dermoid cysts expand slowly, enlarging over years or
decades, by the accumulation of elements of the dermis
• Dermoid inclusion cysts have a thicker lining that may
contain dystrophic calcification.
LOCATIONS
• Dermoid cysts commonly occur in the orbital and
periorbital region. Intracranial dermoid cysts are very
rare
• The most common intracranial locations of dermoid
cyst are in the midline supratentorially or at the
cerebellopontine angle. They may also have an
intraventricular location arising within the cisterns of
the tela choroidea in the lateral, third, or fourth
ventricular regions.
CLINICAL PRESENTATION
• Dermoid cysts can present either at birth or occur
sporadically
• They typically present in patients less than thirty years
of age
• The most common symptoms are headaches and
seizures
• Not uncommonly, there is a persistent defect in the
overlying skin, with a sinus tract extending into the
intracranial portion.
RADIOLOGICAL FEATURES
CT Scan
• They typically appear as well-defined round
hypodense masses with an attenuation consistent with
fat (–20 to –100 HU)
• In case of rupture, the scattered low density fatty
droplets may be scattered throughout the ventricles
and subarachnoid space
• A fat/CSF fluid level may also be present. Calci-
fications, in the capsule are particularly common
• There is no enhancement after contrast administration.
MRI
• Dermoids typically demonstrate high signal on T1 and
variable signal on T2-weighted images. This is
consistent with the lipid and cholesterol contents of
the lesion. Hair may be noted as fine, low signal
structures within the cyst
• The cysts may rupture into the subarachnoid space.
If the cyst ruptures, then high signal droplets on T1
images may be seen scattered throughout the CSF.
Again, a fat/CSF fluid level may also be identified
• As with other fatty masses, chemical shift artifact may
also be present.
BIBLIOGRAPHY
1. Conley FK. Epidermoid and dermoid tumors: Clinical
features and surgical management. In: Wilkins RH,
Rengachury SS (Eds). Neurosurgery, 2nd edn. New York:
McGraw-Hill 1996.pp.971-6.
2. Huisman TA, Schneider JF, Kellenberger CJ, Martin-Fiori
E, Willi UV, Holzmann D. Developmental nasal midline
masses in children: Neuroradiological evaluation. Eur
Radiol 2004;14(2):243-9.
3. Liu JK, Gottfried ON, Salzman KL, Schmidt RH,
Couldwell WT. Ruptured intracranial dermoid cysts:
Clinical, radiographic, and surgical features. Neuro-
surgery 2008;62(2):377-84; discussion 384.
4. Paller AS, Pensler JM, Tomita T. Nasal midline masses in
infants and children: Dermoids, encephaloceles, and
gliomas. Arch Dermatol 1991;127(3):362-6.
5. Wilms G, Casselman J, Demaerel P, et al. CT and MRI of
ruptured intracranial dermoids. Neuroradiology 1991;
33(2):149-51.
 Chapter 42 Intracranial Neoplasms 593

A B

Figs 42.41A to C: A 40-year-old female presented with right

Vth and VIth nerve palsy: (A) Axial T1-weighted image,
(B) Sagittal T1-weighted image and (C) Coronal T1-weighted
image showing a well-circumscribed hyperintense lesion in the
right cerebellopontine angle compressing the cisternal
segments of the right 6th nerve. On fat-suppressed T1-weighted
images, the lesion became isointense suggesting fatty nature—
C dermoid was noted intraoperatively
594 Section 6 Retrochiasmal Visual Pathway Lesions
XIII. EPIDERMOID CYSTS
• Epidermoid cysts are choristomas (tumor-like lesions
composed of normal tissues, the elements of which
are not found normally at the site of involvement)
• Epidermoid cysts arise from inclusion of ectodermal
epithelial elements at the time of neural tube closure
or during formation of secondary cerebral vesicles
• Both congenital and acquired epidermoid cysts occur
• Congenital epidermoid tumor is actually a non-
neoplastic inclusion cyst
• Acquired epidermoid cysts develop as a result of
trauma. The epidermis is implanted into deeper
underlying tissues and forms a cyst that continues to
desquamate keratin
• Grossly, epidermoid cyst is a large single or multi-
loculated cystic cavity containing keratin and
inwardly directed lining of stratified squamous cell
epithelium.
INCIDENCE
0.2 to 1 percent of all primary intracranial tumors.
PRESENTATION
• Epidermoid cysts can present either at birth or occur
sporadically
• They occur between the ages of 20 and 60 years with
peak incidence in 4th decade. There is no gender
predilection.
LOCATIONS
• Intradural (90%): Occur in the basal subarachnoid
space, off-midline sites are common locations
• 40-50 percent are seen in cerebellopontine angle
• Suprasellar and parasellar (cavernous sinus or middle
cranial fossa) regions account for 7 percent each
• Intra-axial epidermoid cysts are uncommon. The IVth
ventricle is the most common site. Rarely, these
tumors occur in the cerebral hemispheres or brainstem
• Ten percent are extradural, mostly intradiploic.
Primary intradiploic epidermoid occurs in the frontal,
parietal or occipital bones. Involvement of sphenoid
bone is uncommon
• Cranial dermoids and epidermoids are most common
scalp lesions in children.
IMAGING
CT
• Plain CT scans show most epidermoid cysts to be well-
defined lucent appearing lobulated masses with
attenuation similar to CSF
• Calcification is present in 10-25 percent cases
• Occasionally, epidermoids appear hyperdense due to
hemorrhage, high-protein content, saponification of
cyst debris to calcium, or deposition of iron-containing
pigment
• Usually, they do not enhance with contrast although
enhancement at the tumor margin is sometimes
observed
• Intradiploic epidermoids will cause widening of the
diploic space.
MRI
• Epidermoids characteristically insinuate along the
basal cisterns
• They usually follow CSF signal intensity T1 and T2-
weighted images. On FLAIR imaging, they are slightly
brighter than CSF. Diffusion-weighted imaging
helps to differentiate these lesions from arachnoid cyst
as both have similar signal on T1 and T2-weighted
images
• The epidermoids called white epidermoids appear
isointense or even hyperintense to brain on T1-
weighted images. They are noted to have high lipid
content on magnetic resonance spectroscopy
• Epidermoids with long T1 values are called black epider-
moids and have low lipid content on spectroscopy
• On diffusion, the epidermoid will appear bright due
to restricted diffusion and increased apparent
diffusion coefficient (ADC)
 Chapter 42 Intracranial Neoplasms 595

A B

Figs 42.42A to D: (A) Axial T2-

weighted image showing a subtle
lesion in the left cerebellopontine
angle cistern almost isointense to
CSF (black arrows), (B and C) Axial
FLAIR at different levels shows the
lesion to be minimally hyperintense
with respect to CSF and (D) Axial
diffusion weighted image showing
the lesion to be bright confirming
C D the epidermoid nature

Figs 42.43A and B: Coronal T1-

weighted images (A and B)
showing a lobulated suprasellar
lesion slightly hyperintense with
A B respect to CSF—epidermoid
596 Section 6 Retrochiasmal Visual Pathway Lesions
• Epidermoids at the petrous apex should be
differentiated from cholesterol cysts (cholesterol
granulomas). Epidermoids (cholesteatomas) tend to
be dark or intermediate in signal on the T1-weighted
images and bright on T2-weighted images. The
cholesterol granuloma is bright on both sequences but
will often have dark areas within, representing
hemosiderin from current hemorrhage.
BIBLIOGRAPHY
1. Atlas SW. Magnetic Resonance Imaging of the Brain and
Spine, 1996.
2. Gao PY, Osborn AG, Smirniotopoulos JG. Radiologic-
pathologic correlation: Epidermoid tumor of the
cerebellopontine angle. Am J Neuroradiol 1992;13(3):
863-72.
3. Ikushima I, Korogi Y, Hirai T. MR of epidermoids with a
variety of pulse sequences. Am J Neuroradiol 1997;18(7):
1359-63.
4. Kallmes DF, Provenzale JM, Cloft HJ. Typical and atypical
MR imaging features of intracranial epidermoid tumors.
Am J Roentgenol 1997;169(3):883-7.
5. Kato K, Higa T, Ujiie H, Chernov M, Kubo O, Hori T.
Intracranial epidermoid tumor after subcutaneous lipoma
excision. Neurol Med Chir (Tokyo) 2008;48(6):262-5.

RADIATION INJURY
INTRODUCTION
• Radiotherapy has become an important therapeutic
adjunct in the treatment of patients with primary and
metastatic brain tumors
• Radiotherapy to the brain includes high-dose external-
beam radiation, radiosurgery, and radioactive seed
implantation
• Radiation-induced brain injury can occur secondary
to any form of radiotherapy
• Central nervous system radiation has a spectrum of
pathologic (and imaging) manifestations. These
include edema, arteritis, necrosis, mineralizing micro-
angiopathy, progressive leukoencephalopathy and
radiation-induced tumors
• Radiation necrosis is a term for a focal structural lesion
that usually occurs at the original tumor site. Edema
and the presence of tumor render the CNS
parenchyma in the tumor bed more susceptible to
radiation necrosis. However, the pathology is not
limited to necrosis and a spectrum of injury patterns
may occur
• Radiation brain injury can be divided into three
chronological phases:
1. Acute radiation injury: It is transient and occurs
during the radiation or shortly thereafter and is
edematous in nature. It is due to disruption of the
blood-brain barrier
2. Early delayed radiation injury occurs weeks to few
months later and is characterized by demyelina-
tion and vasogenic edema
3. Late radiation injury is characterized by focal or
diffuse coagulation necrosis where ischemia plays
a major role. Late radiation necrosis is dose related,
fatal, and risk increases with high dose (> 6000 rads),
improper dose fractionation and patient’s age
• It is important to distinguish a residual/recurrent
neoplasm from radiation-induced necrosis. Diffe-
rentiation may be difficult based on morphological
features alone.
Clinical Features
• These can be highly variable. Radiation-induced
necrosis usually is a progressive and irreversible
pathological process. Few lesions can stabilize and
may even regress
Chapter 42 Intracranial Neoplasms 597
• Seizures, reappearance of the initial tumor’s
symptomatology and signs of mass effect are usually
evident
• Features of late radiation injury include reduction of
intellectual function, coma, ataxia, decerebration,
personality change, etc.
IMAGING
The appearance of a new lesion several years after radiation
treatment for a primary brain tumor may represent
recurrence of the tumor, radiation-induced demyelination,
radiation necrosis of the brain, ischemic stroke secondary
to radiation-induced vasculopathy, or rarely a secondary
tumor or occult vascular malformation.
CT Scan
• In most cases, CT scan is not helpful in making the
diagnosis of radiation necrosis
• It is most useful in acute, clinical decline of a patient
with brain tumor to differentiate acute hemorrhage
from increased intracranial pressure, obstructive
hydrocephalus, or a herniation syndrome
• Confluent low density white matter lesions within
radiation port might be visible
• Basal ganglia/gyriform subcortical calcium deposits
suggest mineralizing microangiopathy
• The lesions usually do not enhance with contrast
• Necrotizing leukoencephalopathy may have ring-
enhancing lesions
• Whole brain radiation can lead to diffuse white matter
injury. This may appear as hypodense small
periventricular foci on a CT scan.
MAGNETIC RESONANCE IMAGING
Conventional MRI
• MRI signal changes in radiation necrosis cannot be
differentiated from tumor-related changes
• In acute radiation injury, MRI often fails to demonstrate
abnormalities or may show T1- hypointense and T2-
hyperintense lesions in the cerebral white matter, basal
ganglia and cerebral peduncle
• Acute finger-like white matter hyperintense areas are
seen on T2-weighted and FLAIR sequence suggestive
of vasogenic edema
598 Section 6 Retrochiasmal Visual Pathway Lesions

A B

C D

Figs 42.44A to D: A 42-year-old female presented with severe headache one year after radiation for an operated frontal parafalcine
meningioma: (A) Axial T2-weighted preoperative image showing a well-circumscribed right parafalcine meningioma with area of
necrosis within, (B) Axial T2-weighted image one year after surgery and radiation showing extensive white matter hyperintense
signal on the right and (C and D) Axial postcontrast T1-weighted images showing irregular enhancement in the right parietal lobe
away from the site of the meningioma—radiation necrosis

• Focal late radiation injury on MRI may appear as
localized short TR/TE hypointense and long TR
hyperintense lesion and vasogenic edema with or
without mass effect on adjacent tissues
• In chronic radiation, damage, radionecrosis of white
matter can be seen in the immediate vicinity of tumor
or surgical cavity. Most common finding is a hypo-
intense rim on T2-weighted sequences that enhance
well resembling a recurrent or residual tumor
• In chronic radiation damage multiple lesions away
from tumor site may also be seen. These lesions may
demonstrate nodular, linear, curvilinear, “soap-
bubble” or “swiss-cheese” enhancement
• Delayed postradiation MR and CT show bilateral
periventricular changes similar to that of small vessel
disease
• Serial MR examinations are used routinely in the
assessment and surveillance of patients following
radiotherapy. Areas of abnormal contrast enhance-
ment on postcontrast T1-weighted images and the
extent of T2 hyperintensity is evaluated to look for
residual or recurrent disease. However, it is often
difficult to determine whether an enhanced lesion
represents tumor recurrence or not, especially when
the enhancement is initially observed
• Radiation-induced lesions occur within 2 years after
radiation therapy, the same period during which
tumor recurrence is most frequent
• The lack of specificity on conventional MRI has
resulted in other imaging techniques with the hope
of finding a more reliable clinical tool.
Functional MR Imaging
• Functional MR imaging techniques such as MR
spectroscopy, diffusion-weighted imaging and
perfusion-sensitive contrast-enhanced MR imaging
are now used along with conventional MRI to add
more specificity to the diagnosis
• Perfusion MR imaging has made it possible to obtain
measurements of vascularity within brain lesions and
can be acquired during the same session as
conventional MR imaging
• The vascularity of malignant tumor differs
dramatically from that of irradiated brain tissue,
specifically radiation necrosis. The tumor growth
consists of an irregular meshwork of vessels
intermixed with normally existing vessels
• These vessels markedly differ from those of radiation
necrosis, which primarily consist of ischemic changes
Chapter 42 Intracranial Neoplasms 599
caused by the endothelial damage. Thus, tumor
recurrence within irradiated lesions can be
differentiated from regions of radiation necrosis with
perfusion-sensitive contrast-enhanced MR imaging
• If the normalized rCBV ratio of the enhancing lesion
is more than 2.6 tumor recurrence or less than 0.6
radiation necrosis should be strongly suspected
• If the enhancing lesion has a normalized rCBV ratio
between 0.6 and 2.6, 201TI-SPECT may be useful.
Magnetic Resonance Spectroscopy
MR spectroscopy can differentiate tumor from radiation
injury in patients with recurrent contrast-enhancing
intracranial lesions. In these lesions, the Cho/NAA and
Cho/Cr ratios may be the best numeric discriminators.
OTHER MODALITIES
• Functional imaging (PET/SPECT): SPECT and PET
studies (especially FDG-PET) are now widely used
to evaluate the metabolic activities of brain tumors
and are usually helpful in differentiating tumor
recurrence from radiation necrosis
• Radionecrosis is usually hypometabolic (decreased
FDG, Methionine, 201TI uptake compared to healthy
brain parenchyma).
BIBLIOGRAPHY
1. Chan YL, Leung SF, King AD, Choi PH, Metreweli C. Late
radiation injury to the temporal lobes: Morphologic
evaluation at MR imaging. Radiology 1999;213(3):800-7.
2. Chorvath M, Boljesikova E, Pruzincova L, Procka V, Rychly
B, Novotny M, Kalina P, Belan V, Makaiova I, Steno J. Post-
therapeutical changes in the brain: Novel trends in imaging
and their influence on external beam radiotherapy.
Neoplasma 2009;56(2):156-62.
3. Kang TW, Kim ST, Byun HS, Jeon P, Kim K, Kim H, Lee
JI. Morphological and functional MRI, MRS, perfusion and
diffusion changes after radiosurgery of brain metastasis.
Eur J Radiol 2009;72(3):378-80. Epub 2008 Oct 1.
4. Kishimoto R, Mizoe JE, Komatsu S, Kandatsu S, Obata T,
Tsujii H. MR imaging of brain injury induced by carbon
ion radiotherapy for head and neck tumors. Magn Reson
Med Sci 2005;4(4):159-64.
5. Sundgren PC, et al. Metabolic alterations: A biomarker for
radiation-induced normal brain injury—an MR spectro-
scopy study. J Magn Reson Imaging 2009;29(2):291-7.
6. Valk PE, Dillon WP. Radiation injury of the brain. Am J
Neuroradiol 1991;12(1):45-62.
SECTION 7
Efferent Pathway Lesions
SECTION OUTLINE
43. Ocular Motility Disorders
44. Chronic Progressive External Ophthalmoplegia
45. Internuclear Ophthalmoplegia
46. Supranuclear: Gaze Palsy
47. Olivopontocerebellar Atrophy
48. Nystagmus
Chapter 43
Ocular Motility Disorders
• The ocular motor system is separated anatomically
and physiologically into infranuclear (peripheral),
nuclear, internuclear and supranuclear components
• Supranuclear ocular motility disorders can be caused
by lesions in the brainstem, cerebellum, or cerebral
hemispheres
• Internuclear ocular motor disorders are caused by
damage to brainstem pathways that coordinate
movements of the two eyes.
NUCLEAR AND INFRANUCLEAR PATHWAYS
Third Cranial Nerve Pathway
• The oculomotor cranial nerve originates from it is
nucleus (main motor nucleus) located in the midbrain
at the level of superior colliculus
• It has two paired nuclei (for medial and inferior rectus)
and one central unpaired nucleus (for levator
palpebrae superioris and superior rectus) supplying
the extraocular muscles
• The accessory parasympathetic nucleus—Edinger-
Westphal nucleus controls the pupillomotor fibers
• The nerve exits from the interpenduncular fossa
passes through the subarachnoid space and travels
between the posterior cerebral artery and superior
cerebellar artery
• It reaches the lateral wall of the cavernous sinus and
then divides into superior and inferior divisions before
entering the superior orbital fissure
• The divisions then enter the orbit and the superior
division supplies the LPS and superior rectus, the
inferior division supplies the inferior oblique and the
inferior rectus with the parasympathetic innervation
to the ciliary ganglion.
Fourth Nerve Pathway
• The trochlear nerve is the only cranial nerve which
emerges on the dorsal aspect of the brainstem. It has
its nucleus in the periaqueductal gray matter
• The emerging fibers cross around the periaqueductal
gray, decussate at the level of the inferior colliculus
and then across the cavernous sinus lateral and inferior
to the III nerve
• It travels through the superior orbital fissure to supply
the superior oblique muscle
• The superior oblique muscle acts as depressor in
adduction and also a rotator.
Sixth Nerve Pathway
• The abducens nerve nucleus lies in the dorsal aspect
of pons close to the midline, beneath the fourth
ventricle at the level
• The VI nerve leaves the brainstem at the
pontomedullary junction, it runs in the subarachnoid
space along the base of the pons
• It passes through the Dorello’s canal and along the
petrous aspect of the clivus bone
• It pierces the dura and runs in the cavernous sinus inferior
and lateral to the cavernous internal carotid artery and
enters the orbit through the superior orbital fissure
• It supplies the lateral rectus muscle that controls the
abduction movement of eye
• This nerve has the longest course among the cranial
nerve in the subarachnoid space
604 Section 7 Efferent Pathway Lesions

Fig. 43.1: Diagrammatic representation of the course of the III nerve and its important relations to
various structures

Fig. 43.2: Gross anatomy specimen showing the cisternal segment of the
III nerve and its important relations
 Chapter 43 Ocular Motility Disorders 605

Fig. 43.3: Axial T2 FRFSE image. The white dot represents the approximate position of the left
III nerve nucleus at the level of the superior colliculus

Fig. 43.4: Axial FIESTA image showing the cisternal and cavernous segment of the III nerve
606 Section 7 Efferent Pathway Lesions

DIAGRAMMATIC REPRESENTATION OF THE COURSE OF THE IV CRANIAL NERVE

Fig. 43.5: Diagrammatic presentation of the course of the IV nerve

Fig. 43.6: Diagrammatic representation of the course of the cranial nerves III,
IV, V and VI—in the basal cisterns and superior orbital fissure
 Chapter 43 Ocular Motility Disorders 607

Fig. 43.7: Axial T2 FRFSE sequence at the level of the inferior colliculus
showing the approximate location of the IV nerve nucleus

Fig. 43.8: Axial FIESTA image at the level of the IV nerve decussation and also showing
cisternal course of IV nerve

Fig. 43.9: Section of pons showing relation of VI and VII nerve nuclei
608 Section 7 Efferent Pathway Lesions

Fig. 43.10: Axial T2-weighted image at the level of the facial colliculus and VI nerve nucleus

Fig. 43.11: Axial fast imaging employing steady state acquisition (FIESTA) sequence showing
the cisternal segment of the VI nerve

IMAGING
• MRI is the modality of choice to image cranial nerves
• Thin sections should be taken in the axial and coronal
plane to include the entire course of the cranial nerve
to be imaged
• Axial FIESTA (fast imaging employing steady state)
by G Milwakee or CISS (constructive interference in
steady state) sequences by SIEMENS, Germany should
be done in every patients with cranial nerve palsies
• The standard protocol followed at our institution is
(1) Axial FLAIR and DWI sequence for whole brain
(2) 2-3 mm thick axial and coronol T1 (FSPGR) and
T2 FRFSE (3) Oblique sagittal T1 or T2 sequences are
performed depending on the pathology (4) MRA is
performed in all pupils involving III and VI nerve
palsies (5) Contrast-enhanced MRI is done in all
patients with cranial nerve palsies.

THIRD NERVE PALSY
INTRODUCTION
Pupil involving III nerve palsy is a medical emergency
which requires an early accurate diagnosis followed by
an urgent neurosurgical intervention.
CAUSES
Any extrinsic or intrinsic lesion along the course of the
III cranial nerve can cause oculomotor nerve palsy or
dysfunction. Some of the most frequent causes at various
levels along its course are as follows:
Nuclear and Fascicular Level
• Infarction
• Hemorrhage
• Neoplasm
• Infection.
Subarachnoid Portion
• Aneurysm—posterior communicating artery
aneurysm
• Infectious meningitis —bacterial, fungal/parasitic, viral
• Meningeal infiltration
• Carcinomatous/lymphomatous/leukemic infiltration,
granulomatous inflammation (sarcoidosis, lympho-
matoid granulomatosis, Wegener granulomatosis).
Cavernous Sinus Portion
• Tumor—pituitary adenoma, meningioma, schwan-
noma and metastatic carcinoma
• Giant intracavernous aneurysm
• Carotid-cavernous sinus fistula
• Cavernous sinus thrombosis
• Ischemia from microvascular disease in vasa nervosa
• Inflammatory—Tolosa-Hunt syndrome (idiopathic or
granulomatous inflammation).
Orbital Portion
• Inflammatory—orbital inflammatory pseudotumor,
orbital myositis
• Endocrine (thyroid orbitopathy)
• Tumor (e.g. hemangioma, lymphangioma, menin-
gioma).
Chapter 43 Ocular Motility Disorders 609
TRAUMA
Clinical Features
• Patients with III nerve palsies may have binocular
diplopia, ptosis, anisocoria, or a combination of these
findings. If the external muscle dysfunction is
complete, then ipsilateral adduction, elevation, and
depression deficit may be present. The degree of
external muscle dysfunction involvement may be
variable (i.e. partial or complete) and some muscles
may be unaffected
• Nuclear III cranial nerve palsy demonstrates in
addition to the ipsilateral findings, contralateral
partial ptosis and elevation palsy. The contralateral
partial ptosis stems from the bilateral distribution of
innervation to the levator from the caudal central
subnucleus. Ptosis is more complete ipsilateral to the
lesion because function is lost in both the ipsilateral
neural cell bodies and their fibers on the lesioned side,
plus the crossed fibers coursing through the lesion
from the other side. There is partial ptosis contralateral
to the lesion because of the residual integrity of the
uncrossed neural cell bodies and fibers from the
caudal central subnucleus contralateral to the lesion
• Lesions of the III nerve fascicle may also involve other
midbrain structures including:
– The red nucleus-superior cerebellar peduncle
producing contralateral ataxia and cerebellar
tremor (Claude syndrome)
– The cerebral peduncle causing contralateral
hemiparesis (Weber syndrome)
– The red nucleus-substantia nigra producing
contralateral choreiform movements or tremor
(Benedict’s syndrome)
– Due to its close relation to the vessels in the sub-
arachnoid space, the III nerve is susceptible to
compression by an aneurysm. This usually results
in a dilated and unresponsive pupil (i.e. complete
internal dysfunction) because of the superficial
location of the pupil fibers within the subarachnoid
space. Therefore, an isolated pupil-involved III nerve
palsy is an aneurysm until proven otherwise. This is
known as the rule of the pupil
– Lesions of the III nerve in the cavernous sinus
typically but variably involve the other cranial
nerves (e.g. IV nerve and VI nerve), the first
610 Section 7 Efferent Pathway Lesions

A B

Figs 43.12A and B: Pupil involving III nerve palsy: (A) Axial T2-weighted image showing a large oval signal void lesion in the left
suprasellar region compressing the cisternal segment of the III nerve, left optic tract and chiasm and (B) Axial MIP image of 3D
time of flight angiogram showing complex flow in the large aneurysm. DSA (not shown) confirmed the aneurysm to be arising from
the P1 segment of the left posterior cerebral artery

A B

Figs 43.13A and B: A 38-year-old male presented with pupil involving left III nerve palsy: (A) Axial postcontrast fat suppressed
T1- weighted image at the level of the cavernous sinus and (B) Oblique and sagittal postcontrast image showing enhancement of
the cavernous segment of the left III nerve (arrows). Patient had multiple cervical necrotic lymph nodes FNAC proven tuberculosis.
III nerve palsy resolved with antituberculous treatment
 Chapter 43 Ocular Motility Disorders 611

(ophthalmic) branch of the trigeminal nerve, and • As with any pupil-spared ophthalmoplegia,
the oculosympathetic fibers (Horner syndrome) myasthenia gravis should also be considered. In
– Lesions within the superior orbital fissure or in patients without vasculopathic risk factors or in
the orbit produce other ocular motor dysfunction, patients with progressive or unresolved III nerve
optic neuropathy, and proptosis. palsies, neuroimaging should be performed,
preferably cranial contrast-enhanced MRI with
NEUROIMAGING magnetic resonance angiography (MRA) or computed
tomographic angiography (CTA)
• Nonisolated III nerve palsies should undergo
• Patients with pupil sparing should be observed within
neuroimaging [i.e. magnetic resonance imaging (MRI)
the first 24 to 48 hours of onset for delayed pupil
with contrast] directed to the specific areas localized
involvement. If pupil involvement occurs, then further
by the associated neurologic signs and symptoms.
evaluation is warranted. Patients should be evaluated
Patients in whom a meningeal process is suspected
and treated for underlying vasculopathic risk factors
should undergo a lumbar puncture if neuroimaging
(e.g. diabetes mellitus, hypertension, increased
is not diagnostic
cholesterol, and smoking)
• Cerebral angiography may be necessary for patients
• Elderly patients should be questioned regarding
suspected of harboring an underlying aneurysm.
symptoms of giant cell arteritis (e.g. headache, jaw or
Patients with signs of subarachnoid hemorrhage (e.g.
tongue claudication, polymyalgia rheumatica) and
severe headache, change in mental status, other
should have further evaluation as indicated (e.g.
neurologic deficit) often require a noncontrast
erythrocyte sedimentation rate and temporal artery
computed tomography scan, and further imaging to
biopsy).
rule out aneurysm. Patients recovering from a III
• MRI/MRA
nerve palsy may have aberrant regeneration. Aberrant
– MRI is a more sensitive than CT scan for a small
regeneration involves any of the external somatic
brainstem lesion, such as infarction, infection or
branches of the III nerve or the pupil (e.g. lid retraction
tumor
in downgaze or adduction, pupil constriction in
– MRI with contrast, is the procedure of choice to
adduction, or depression)
look for meningeal and dural inflammation and
• Patients with primary (i.e. no prior III nerve palsy) or
infiltration
secondary (i.e. after a III nerve palsy) aberrant
– MRI/MRA should be done in pupil involving III
regeneration should undergo a neuroimaging study
nerve palsy
to exclude a compressive lesion
– Normal MRA is probably not adequate to rule out
• Ischemic III nerve palsies do not produce aberrancy
berry aneurysm causing III cranial nerve palsy.
and the development of aberrant regeneration in a
• CT scan
presumed ischemic palsy should prompt
– CT scan is more sensitive than MRI to demonstrate
neuroimaging
subarachnoid hemorrhage
• An isolated III nerve palsy with a normal pupillary
– CT scan is also better than MRI for demonstrating
sphincter and completely palsied extraocular muscles
calcification within lesions, as may be found in
is rarely caused by an aneurysm. The neurologically
certain tumors and in large aneurysms.
isolated, complete pupil-spared III nerve palsy is
usually due to ischemia and is often caused by
BIBLIOGRAPHY
diabetes mellitus
• Lack of internal muscle dysfunction (pupil-sparing) 1. Eisenkraft B, Ortiz AO. Imaging evaluation of cranial
in diabetic III nerve palsies is presumably due to the nerves 3, 4, and 6. Semin Ultrasound CT MR 2001;22(6):
lack of vascular damage to the periphery of the nerve 488-501.
where the majority of pupillomotor fibers are thought 2. Mark AS, Blake P, Atlas SW, Ross M, Brown D, Kolsky M.
Gd-DTPA enhancement of the cisternal portion of the
to pass. This is in distinction to the pupil involved III
oculomotor nerve on MR imaging. Am J Neuroradiol 1992;
nerve palsy due to aneurysm compressing the
13(5):1463-70.
superficially located pupil fibers. Therefore, 3. Lee AG, Brazis PW. The emerging role of magnetic
neuroimaging may be deferred in adult patients with resonance angiography in the management of patients
known vasculopathic risk factors (e.g. diabetes, with III nerve palsy. Am J Ophthalmol 2000;129(1):
hypertension) who develop an acute isolated, but 115-6.
complete external dysfunction, pupil-sparing III 4. Lee AG, Hayman LA, Brazis PW. The evaluation of isolated
cranial nerve palsy III nerve palsy revisited: An update on the evolving role of
612 Section 7 Efferent Pathway Lesions

A B

Figs 43.14A to C: A 34-year-old male presented with left pupil involving III nerve palsy: (A) Axial T2-weighted image showing a
well-circumscribed flow void arising from the posterior communicating artery (arrow), (B) Sagittal oblique and (C) Sagittal MIP of
3D time of flight angiogram showing a bilobed aneurysm arising from the left posterior communicating aneurysm

magnetic resonance, computed tomography, and catheter
angiography. Surv Ophthalmol 2002;47(2):137-57. Review.
5. Brazis PW. Localization of lesions of the oculomotor nerve:
Recent concepts. Mayo Clin Proc 1991;66(10):1029-35. Review.
6. Mark AS, Casselman J, Brown D, Sanchez J, Kolsky M, Lassen
TC 3rd, Lavin P, Ferraraccio B. Ophthalmoplegic migraine:
Reversible enhancement and thickening of the cisternal
segment of the oculomotor nerve on contrast-enhanced MR
images. Am J Neuroradiol 1998;19(10):1887-91.
Chapter 43 Ocular Motility Disorders 613
7. PY Blake, AS Mark, J Kattah, M Kolsky. MR of oculomotor
nerve palsy. American Journal of Neuroradiology 1995;
16(8):1665-72.
8. Rabadi MH, Beltmann MA. Midbrain infarction presenting
isolated medial rectus nuclear palsy. Am J Med
2005;118(8):836-7.
9. Saeki N, Murai H, Mine S, Yamaura A. Fascicular
arrangement within the oculomotor nerve MRI analysis
of a midbrain infarct. J Clin Neurosci 2000;7(3):268-70.
614 Section 7 Efferent Pathway Lesions

A B

Figs 43.15A to C: A 40-year-old with pupil involving III nerve palsy: (A and B) Coronal MIP showing a small
aneurysm arising from the cavernous segment of the left internal carotid artery with a small neck and (C) Axial
source image showing the neck of the aneurysm (arrow)

A B

Figs 43.16A and B: A 65-year known hypertensive and diabetic with long-standing history of ptosis and
diplopia: (A) Axial T1-weighted image and (B) Axial T2-weighted image showing a T1-hypointense and
T2-hyperintense chronic infarct at the nuclear level of the III nerve (white arrow)
 Chapter 43 Ocular Motility Disorders 615

Fig. 43.17: Axial T2-weighted image at the level of the midbrain

showing infarct in the nucleus and fascicle of the right III nerve
in a patient with III nerve palsy

A B

Figs 43.18A and B: A 58-year-old man presented with acute onset right III nerve plasy: (A) Axial T2-weighted image at the level
of the midbrain showing an oval hyperintense lesion at the III nerve fascicle level and (B) Corresponding diffusion-weighted image
showing restricted diffusion in the lesion suggestive of an acute infarct
616 Section 7 Efferent Pathway Lesions

A B

Figs 43.19A to D: (A and B) Axial post-

contrast CT scans of the brain in a 20-year-
old female with history of headache and
ptosis shows a large well-circumscribed
minimally enhancing hyperdense lesion in
the suprasellar cistern and (C and D) Axial
and Sagittal T1-weighted images showing
a well-circumscribed hyperintense lesion
consistent with subacute blood within—
C D cavernous angioma with bleed

Fig. 43.20: A 8-year-old presented with diplopia and restricted elevation.

Axial plain CT scan of the brain showing a rounded hyperdense calcified
granuloma in the midbrain (arrow)
 Chapter 43 Ocular Motility Disorders 617

A B

Figs 43.21A and B: A 19-year-old female presented with recurrent right III nerve palsy/ophthalmoplegic migraine: (A) Axial
FIESTA image and (B) Coronal T2 FRFSE image showing thickened cisternal segment of the right III nerve. Postcontrast study
did not show any enhancement
618 Section 7 Efferent Pathway Lesions
FOURTH NERVE PALSY
It is the most common cause of acquired vertical
strabismus.
CAUSES
• Idiopathic—most common
• The most common cause of acquired isolated IV nerve
palsy, after idiopathic, is head trauma
• Generally, trauma must be severe with resultant loss
of consciousness
• The possibility of underlying structural abnormalities
should be considered, if IV nerve palsy results
following a minor trauma
• Microvasculopathy secondary to diabetes,
atherosclerosis, or hypertension
• Thyroid ophthalmopathy and myasthenia gravis can
present rarely as isolated IV nerve palsy. These
patients eventually develop other findings,
unmasking the underlying diagnosis
• Tumor, aneurysm, multiple sclerosis, or iatrogenic
injury are other causes of isolated IV nerve palsy can
eventually evolve over time to include other cranial
nerve palsies or neurologic symptoms
• Fourth nerve palsy may become manifest after
cataract surgery.
CLINICAL FEATURES
• In acquired lesions of IV nerve, patients report vertical,
torsional, or oblique diplopia. Diplopia is usually
worse on downgaze and gaze away from side of
affected muscle
• In case of trauma, patients usually report symptoms
immediately after regaining consciousness
• Torsional diplopia and downgaze horizontal diplopia
may be predominant complaints in bilateral palsies
• Patients often adopt a characteristic head tilt, away
from affected side to reduce their diplopia.
Interestingly, some patients develop head tilt toward
side of lesion. This so-called paradoxic head tilt is used
to create a wider separation of images, which allows
the patient to suppress or ignore one image. Old
photographs may provide clear documentation of a
head tilt in congenital IV nerve palsy
• Three-step test can be extremely useful in evaluation
of vertical diplopia caused by a paretic cyclovertical
muscle
• In case of bilateral IV nerve palsy, interpretation of 3-
step test may be confusing
• Excyclotorsion may be measured using double
Maddox rod test.
MANAGEMENT
• This depends on the cause because usually congenital
cases are not symptomatic. It is useful to review the
old photographs as these cases may decompensate at
a later age
• Apart from fasting and postprandial blood sugar,
erythrocyte sedimentation rate, and blood pressure
recording, neuroimaging of the brain and orbit is very
important to rule out any space occupying lesion.
RECOMMENDED IMAGING—MRI BRAIN
• For acquired causes, it is best to wait for six to eight
months. Ischemic, inflammatory or closed injury cases
may recover completely
• Symptomatic patients are prescribed prisms or
occlusion to relieve diplopia. In case of longstanding
deviations (8-12 months), careful orthoptic evaluation,
followed by surgical correction by superior oblique
strengthening, inferior oblique weakening or classical
Harada-Ito procedure is the treatment of choice.
In a patient suspected to have an isolated acquired IV
nerve palsy, MRI should include imaging of the complete
course of the IV nerve and should also include orbit
sections to look at the superior oblique-trochlea complex.
In post-traumatic IV nerve palsies, MRI should include a
thin gradient images of the midbrain to look for small
hemorrhages.
BIBLIOGRAPHY
1. Brazis PW. Palsies of the trochlear nerve: Diagnosis and
localization; recent concepts. Mayo Clin Proc 1993;68(5):
501-9.
2. Miller NR, Newman NJ. Walsh and Hoyt’s Clinical Neuro-
Ophthalmology, 5th edn. 1998;pp.1227-37.
3. Richards BW, Jones FR Jr, Younge BR. Causes and
prognosis in 4,278 cases of paralysis of the oculomotor,
trochlear, and abducens cranial nerves. Am J Ophthalmol
1992;113(5):489-96.
 Chapter 43 Ocular Motility Disorders 619

A B C

Figs 43.22A to C: (A) Axial T2-weighted image, (B) Axial T1-weighted image and (C) Sagittal T1-weighted showing a well-
defined exophytic tectal lesion with areas of necrosis—tectal glioma in a patient with isolated IV nerve palsy

A B C

Figs 43.23A to C: A 75-year-old male with history of isolated right IV nerve palsy: (A) Axial T1-weighted image, (B) Coronal and
(C) Axial postcontrast T1-weighted showing a well-circumscribed T1 isointense lesion with homogeneous enhancement and dural
tail sign (dotted arrow)—right tentorial meningioma
620 Section 7 Efferent Pathway Lesions

A B

Figs 43.24A and B: A 35-year-old lady with long standing IV nerve palsy: (A) Oblique Axial T2-weighted and (B) Axial T1-
weighted images at the level of the midbrain showing a well-defined rounded T1 and T2-hypointense lesion (dotted arrow)
suggestive of calcified granuloma

A B

Figs 43.25A and B: Axial T1-weighted (A and B) at different levels showing a cystic cavity in the periaqueductal region extending
to the right to involve the IV nerve nucleus—post-traumatic gliosis causing IV nerve palsy
 Chapter 43 Ocular Motility Disorders 621

A B

Figs 43.26A to C: A 22-year-old male with history of post-

traumatic IV nerve palsy: (A) Axial CT of the brain showing a
subtle hyperdensity at the left inferior colliculus (arrow) and
(B and C) Axial gradient echo showing hypointense signal in
the inferior colliculus and superior cerebellar peduncles
C bilaterally suggestive of hemorrhagic contusions (arrows)
622 Section 7 Efferent Pathway Lesions
SIXTH NERVE PALSY
It has the longest subarachnoid course of all the cranial
nerves; therefore, its syndromes are similar to those of
the IV nerve because of their long intracranial courses.
CAUSES
• Elevated intracranial pressure can result in downward
displacement of the brainstem, causing stretching of
the VI nerve secondary to its anatomic location within
the Dorello canal. This is believed to be the reason
that about 30 percent of patients with pseudotumor
cerebri have an isolated abducens palsy
• Subarachnoid space lesions (e.g. hemorrhage,
infection, inflammation, space-occupying tumor)
• Inflammatory (postviral, demyelinating)
• Vascular
• Metabolic (vitamin B, Wernicke-Korsakoff syndrome)
• Neoplasm (children)—pontine glioma
• Giant cell arteritis
• Congenital absence of the VI nerve (Duane syndrome)
• Trauma, particularly if it results in a torsional head
motion.
CLINICAL FEATURES
Esotropia, head-turn, diplopia, vision loss, pain, hearing
loss, symptoms of vasculitis, particularly giant cell
arteritis. Physical findings include an esodeviation that
increases on ipsilateral gaze and is often greater at a
distance an isolated abduction deficit. Slowed ipsilateral
saccades, papilledema (if increased intracranial pressure),
nystagmus (usually in children, i.e. secondary to pontine
glioma).
MANAGEMENT
• It is rare to find true congenital VI nerve palsy.
A complete work-up of a VI nerve palsy involves
excluding paresis of other cranial nerves (including
VII and VIII), a ocular motility and evaluating
pupillary reaction.
• Neuroimaging is indicated for any suspected
brainstem lesion to exclude pontine glioma in children
(most have papilledema and nystagmus without other
cranial nerve involvement) and in adults that show
no improvement.
• MRI of the brain should include thin axial section from
the skull base to the cavernous sinus and coronal
sections should include the orbit upto the brainstem.
An axial FIESTA or constructive interference in steady
state (CISS) should be done in patients suspected to
have cranial nerve palsies.
RECOMMENDED IMAGING—MRI BRAIN
• In young adults, a lumbar puncture (LP) for
cerebrospinal fluid (CSF) analysis is done to exclude
meningitis in patients who have no history of diabetes
or hypertension and who have a negative
neuroimaging. Elderly patients should have blood
testing such at erythrocyte sedimentation rate (ESR)
and/or a C-reactive protein to screen for giant cell
(temporal, cranial) arteritis
• Poor or no resolution should prompt a full neurologic
evaluation and a consideration of other possible
diagnoses (e.g. congenital esotropia, Möbius
syndrome, Duane syndrome).
BIBLIOGRAPHY
1. Bendszus M, Beck A, Koltzenburg M, Vince GH,
Brechtelsbauer D, Littan T, Urbach H, Solymosi L. MRI in
isolated VI nerve palsies. Neuroradiology 2001;43(9): 742-
5.
2. Hamilton SR. Neuro-ophthalmology of eye-movement
disorders. Curr Opin Ophthalmol 1999;10(6):405-10.
Review.
3. Holmes JM, Leske DA, Christiansen SP. Initial treatment
outcomes in chronic VI nerve palsy. J AAPOS
2001;5(6):370-6.
4. Richards BW, Jones FR Jr, Younge BR. Causes and
prognosis in 4,278 cases of paralysis of the oculomotor,
trochlear, and abducens cranial nerves. Am J Ophthalmol
1992;113(5):489-96.
 Chapter 43 Ocular Motility Disorders 623

A B

Figs 43.27A to C: A 10-year-old child presented with esotropia of

6 months duration: (A) Axial T2-weighted image showing an ill-
defined lesion expanding the pons, (B) Sagittal T1-weighted image
and (C) Coronal T1-weighted image showing a large lesion
expanding the pons with area of necrosis within (dotted arrow) and
C thrombosis of the basilar artery (white arrow)—brainstem glioma
624 Section 7 Efferent Pathway Lesions

A B

Figs 43.28A and B: (A) Axial T1-weighted and (B) Sagittal T1-weighted image showing a well-circumscribed hyperintense lesion
in the pons suggestive of subacute hematoma (arrow)—patient presented with headache and VI nerve palsy

Fig. 43.29: A known case of Ca breast with history of VI nerve

palsy. Axial T1-weighted image showing a well-circumscribed
necrotic lesion in the pons—metastases (arrow)
 Chapter 43 Ocular Motility Disorders 625

A B

Figs 43.30A and B: (A) Axial postcontrast T1-weighted image and (B) Sagittal postcontrast T1-weighted image showing nodular
enhancing lesions in the region of the facial colliculus and VI nerve nucleus—granulomas (arrow)

Fig. 43.31: A known case of multiple sclerosis. Axial T2-weighted Fig. 43.32: Axial postcontrast CT scan showing a small enhancing
image showing ill-defined T2-hyperintense signal in the pons lesion at the left petrous apex (arrow)—petrous apex meningioma
resulting in VI nerve palsy in a patient presenting with left VI nerve palsy
626 Section 7 Efferent Pathway Lesions
A B
A B
A B
Figs 43.33A and B: Axial T1-weighted
image showing a well-circumscribed
expansile hyperintense lesion at the right
petrous apex (arrow)—cholesterol granu-
loma presenting as VI nerve palsy
Figs 43.34A and B: A 40-year-old male
presented with right VI nerve palsy:
(A) Sagittal and (B) Axial postcontrast T1-
weighted image showing an irregular
lobulated moderately enhancing lesion in
the right jugular foramen with extension into
the cerebellopontine angle and prestyloid
space. Note: Multiple flow voids within
suggestive of glomus tumor (arrows). This
patient had multiple lower cranial nerve
palsies on examination
Figs 43.35A and B: A 45-year-old gentle-
man presented with a right VI nerve palsy:
(A) Postcontrast axial T1 and (B) Sagittal
T1 images showing a uniform ring enhan-
cing lesion with a central scolex suggestive
of cysticercosis in the region of the VI nerve
nucleus
 Chapter 43 Ocular Motility Disorders 627

A B

C D

Figs 43.36A to D: (A and B) Axial CT scans and (C and D) Coronal CT scans in soft tissue and bone window settings
showing an expansile lytic lesion at the petrous apex in a 12-year-old child who presented with a right VI nerve palsy—
cholesteatoma
Chapter 44
Chronic Progressive External
Ophthalmoplegia
• Chronic progressive external ophthalmoplegia
(CPEO) is a disorder characterized by slowly
progressive paralysis of the extraocular muscles
• Patients usually experience bilateral, symmetrical,
progressive ptosis, followed by ophthalmoparesis
months to years later. Ciliary and iris muscles are not
involved
• CPEO is the most frequent manifestation of mito-
chondrial myopathies. CPEO in association with
mutations in mitochondrial DNA (mtDNA) may
occur in the absence of any other clinical sign, but
usually it is associated with skeletal muscle weakness
• Kearns-Sayre syndrome (KSS) is a related mitochon-
drial myopathy that demonstrates the following:
CPEO, onset before age 20, and pigmentary retinopathy
• KSS also has at least one of the following: Cardiac
conduction defects, cerebrospinal fluid (CSF), protein
of greater than 100 mg/dL, and a cerebellar syndrome.
Other abnormalities in KSS can include mental
retardation, Babinski sign, hearing loss, seizures, short
stature, delayed puberty, and various endocrine
disorders
• CPEO also can be a sign in the following disorders:
Oculopharyngeal dystrophy, myasthenia gravis, and
Graves’ disease
• In KSS, both the sexes are equally affected and
manifests before 2nd decade.
RADIOLOGICAL FEATURES
• Magnetic resonance imaging (MRI), computed
tomography (CT), and ultrasound may show,
symmetrical thinning of the extraocular muscles in
CPEO, in contrast to enlarged extraocular muscles
sometimes seen with Graves’ disease. Patients with
CPEO and KSS display a wide spectrum of MRI
findings, to include the following:
– Normal brain
– Cortical and cerebellar atrophy
– Increased T2 signal in subcortical cerebral white
matter, cerebellar white matter, globus pallidi,
thalami, and substantia nigra.
BIBLIOGRAPHY
1. Carlow TJ, Depper MH, Orrison WW Jr. MR of extraocular
muscles in chronic progressive external ophthalmoplegia.
Am J Neuroradiol 1998;19(1):95-9.
2. Fraunfelder FT, Roy FH, Randall J. Chronic progressive
external ophthalmoplegia. In: Fraunfelder FT, Roy FH,
Randall J (Eds), Current Ocular Therapy, 5th edn.
Pennsylvania: WB Saunders. 2000.pp.208-10.
3. Miller NR, Newman NJ. Mitochondrial myopathies
(mitochondrial encephalomyopathies). In: Walsh and
Hoyt's Clinical Neuro-Ophthalmology, 5th edn. Lippincott:
Williams and Wilkins, Baltimore 1998.pp.1378-90.
4. Ogasahara S, Nishikawa Y, Yorifuji S. Treatment of
Kearns-Sayre syndrome with coenzyme Q10. Neurology
1986;36(1):45-53.
5. Peterson PL. The treatment of mitochondrial myopathies
and encephalomyopathies. Biochim Biophys Acta 1995;
1271(1):275-80.
6. Phillips CI, Gosden CM. Leber's hereditary optic
neuropathy and Kearns-Sayre syndrome: Mitochondrial
DNA mutations. Surv Ophthalmol 1991;35(6):463-72.
 Chapter 44 Chronic Progressive External Ophthalmoplegia 629

A B

Figs 44.1A and B: (A) Axial CT scan and (B) Coronal CT scan of the orbit showing thinned out extraocular muscles in a patient
with ptosis and ocular motility restriction

A B

Figs 44.2A and B: (A) Axial and (B) Coronal T1-weighted MRI showing thin muscles in a patient with CPEO (arrow)
Chapter 45
Internuclear Ophthalmoplegia
• The medial longitudinal fasciculus (MLF) connects
nuclei of 3rd, 4th and 6th nerve nuclei
• Many fibers in the medial longitudinal fasciculus
(MLF) carry a conjugate horizontal eye movement
command from the abducens internuclear neuron in
the pons to the medial rectus subdivision of the
oculomotor nuclear complex in the midbrain
• The fibers subserving horizontal gaze in the MLF each
carry commands for all types of conjugate eye
movements.
CLINICAL FEATURES
Lesions of the MLF produce internuclear ophthal-
moplegia (INO). This can result in unilateral or bilateral
INO.
• Unilateral INO is characterized by weakness of
adduction ipsilateral to the side of lesion. The second
cardinal sign is nystagmus on abducting the contra-
lateral eye. This consists of a inward drift followed by a
corrective saccade. The vestibular slow phases, pursuit,
optokinetic movements, saccades and quick phases of
nystagmus can all be affected by MLF lesions
• If convergence is good despite absence of voluntary
adduction, a caudal lesion with preservation of medial
rectus subdivision of oculomotor nuclear complex is
suspected and it is called posterior INO. Absence of
convergence does not imply a rostral lesion
• Bilateral INO manifests with bilateral abduction
weakness and abducting nystagmus. There is also
impaired vertical vestibular and pursuit eye
movements and impaired vertical gaze holding
mechanisms with gaze evoked nystagmus on up or
downgaze
• Skew deviation can occur in unilateral INO but is rare
in bilateral INO. The higher eye is on the side of lesion
and shows intorsion differentiating it from a trochlear
nerve palsy
• Dissociated vertical nystagmus (downbeat in the
ipsilateral eye and torsional in the contralateral eye)
may occur with INO due to passage of posterior
semicircular canal pathways through MLF
• Ipsiversive torsional nystagmus can be seen at times
in INO. This occurs due to lesions affecting the
pathways between the vestibular nuclei and
interstitial nucleus of cajal.
MLF lesions do not affect vision usually. Patients can
present with vertical/horizontal or torsional diplopia.
Patients may also present with oscillopsia.
ETIOLOGY
• Multiple sclerosis (usually bilateral)/postradiation
demyelination
• Brainstem infarction
• Brainstem or 4th ventricular tumors
• Chiari malformation/associated hydrocephalus/
syringobulbia
• Infection (bacterial/viral/AIDS)/meningoencephalitis
• Subdural hematoma/supratentorial AV malforma-
tions
• Nutritional disorders
• Metabolic disorders
• Head trauma
• Drug intoxications
• Cancer
• Progressive supranuclear palsy
• Syphilis.
One must differentiate it from pseudo-INO of
myasthenia and Fisher’s syndrome.
 Chapter 45 Internuclear Ophthalmoplegia 631

Fig. 45.1: Axial T2-weighted image showing a hyperintense

signal in the region of medial longitudinal fasciculus (arrow) in
known patient of multiple sclerosis who presented with INO

Fig. 45.2: Axial T2 MRI scan showing hyperintense signal in

the region of MLF (white arrow) in a 40-year-old male patient
who presented with INO following head injury. Also note the
presence of diffuse right cerebellar atrophy
632 Section 7 Efferent Pathway Lesions
Imaging
MRI brain is the imaging modality of choice focussing
on the brainstem employing thin sections. MRI in normal
individuals can show a thin band of hyperintensity in
the midline which is the nuclei of median raphe and
should not be mistaken for paramedian MLF lesion.
BIBLIOGRAPHY
1. Atlas SW, Grossman RI, Savino PJ, Schatz NJ, Sergott RC,
Bosley TM, Hackney DB, Goldberg HI, Bilaniuk LT,
Zimmerman RA. Internuclear ophthalmoplegia: MR-
anatomic correlation. Am J Neuroradiol 1987; 8(2):243-7.
2. Awerbuch G, Brown M, Levin JR. Magnetic resonance
imaging correlates of internuclear ophthalmoplegia. Int J
Neurosci 1990;52(1-2):39-43.
3. Bronstein AM, Rudge P, Gresty MA, Du Boulay G,
Morris J. Abnormalities of horizontal gaze: Clinical,
oculographic and magnetic resonance imaging findings.
II. Gaze palsy and internuclear ophthalmoplegia. J Neurol
Neurosurg Psychiatry 1990;53(3):200-7.
4. Frohman EM, Zhang H, Kramer PD, Fleckenstein J,
Hawker K, Racke MK, Frohman TC. MRI characteristics
of the MLF in MS patients with chronic internuclear
ophthalmoparesis. Neurology 2001;57(5):762-8.
5. Ohbuchi T, Udaka T, Tokui N, Yamamoto H, Shiomori T,
Fujimura T, Shimizu T, Suzuki H. Clinical and MRI
findings of patients with internuclear ophthalmoplegia.
Nihon Jibiinkoka Gakkai Kaiho 2006;109(2):96-102.
6. Yasukochi H. Magnetic resonance images of traumatic
MLF syndrome. No To Shinkei 2005;57(12):1100-1.
 Chapter 45 Internuclear Ophthalmoplegia 633

A B

C D

Figs 45.3A to D: A 50-year-old male presented with gaze palsy with convergence retraction nystagmus: (A) Axial FLAIR image,
(B) Axial diffusion weighted image, (C) Sagittal T2-weighted and (D) Coronal T2-weighted images at the level of the midbrain
showing an oval lesion in the left half of the midbrain in the region of MLF (white arrows)—suggestive of a subacute infarct
Chapter 46
Supranuclear: Gaze Palsy
HORIZONTAL GAZE PALSY
• The supranuclear fibers for horizontal gaze originate
in the frontal eye fields and travel caudally to
decussate at the pontine-mesencephalic junction
before proceeding to the paramedian pontine reticular
formation (PPRF)
• Thus, horizontal gaze palsy in one direction may
result from a lesion of the contralateral frontomesence-
phalic pathway or a disturbance of the ipsilateral
pontine tegmentum involving the PPRF or abducens
nucleus
• In a frontomesencephalic lesion, the gaze palsy can
be overcome by oculocephalic maneuvers
• When the supranuclear tracts are disturbed, the eyes
will typically deviate in the direction opposite the
hemiparesis
• In pontine lesions, the eyes usually deviate toward
the side of the hemiparesis
• Lesions of the paramedian pontine reticular formation
such as infarction, hemorrhage, tumor, and trauma
cause ipsilateral, conjugate, horizontal gaze palsy
• With acute lesions, the eyes may deviate contralaterally.
Nystagmus in the direction of gaze contralateral to the
affected side occurs. Saccades may be slow
• One and a half syndrome is ipsilateral horizontal gaze
palsy and internuclear ophthalmoplegia due to
combined lesions of the abducens nucleus and/or
PPRF and adjacent medial longitudinal fasciculus on
one side of the brainstem
• There is paralytic pontine exotropia of the eye opposite
side of the lesion.
VERTICAL GAZE PALSY
• The presence of vertical gaze palsy implies a lesion in
the midbrain. The midbrain structure known as the
rostral interstitial nucleus of the medial longitudinal
fasciculus (riMLF) is considered to be the vertical gaze
center
• Bilateral disruption of riMLF may result in a
downgaze palsy or a complete vertical gaze palsy.
Isolated upgaze palsy typically results from a lesion
of the posterior commissure, but may rarely follow a
unilateral thalamus or midbrain disturbance
• Lesions of posterior commissure cause the dorsal
midbrain syndrome characterized by upgaze palsy,
lid retraction, convergence paralysis, persistent
downgaze, light-near dissociation of pupils, down-
beating and convergence-retraction nystagmus
• Common causes of the dorsal midbrain syndrome
include pineal tumors and aqueductal stenosis. Other
etiologies include intramedullary tumors, brainstem
infarctions, vascular malformations, demyelinating
disorders, and infection
• Although a monocular elevation palsy is most
commonly caused by an orbital process such as
thyroid eye disease, a rare supranuclear paresis may
result from a midbrain lesion. The lesion is usually
contralateral to the elevation palsy
• Lesions of the rostral interstitial nucleus of the medial
longitudinal fasciculus, usually infarcts, cause
downgaze palsy, torsional nystagmus and paresis of
downward saccades
• Progressive supranuclear palsy is a degenerative
disease of later life characterized by disturbances of
tone and posture, difficulty with swallowing and
speech and mental slowing with impairment of
vertical saccades and pursuit.
 Chapter 46 Supranuclear: Gaze Palsy 635

A B

Figs 46.1A and B: A case of progressive supranuclear palsy: (A) Axial T2-weighted image and (B) Sagittal T2-weighted image
showing atrophy of the midbrain and superior colliculus
636 Section 7 Efferent Pathway Lesions
HORNER’S SYNDROME
Horner’s syndrome results from an interruption of the
sympathetic nerve supply to the eye, and it is characteri-
zed by the classic triad of miosis (i.e. constricted pupil),
partial ptosis, and loss of hemifacial sweating (i.e.
anhidrosis). Relative enophthalmos may also be seen.
Sympathetic innervation to the eye consists of a
3-neuron arc:
• First-order fibers descend from the ipsilateral
hypothalamus through the brainstem and cervical
cord to T1/T2
• These fibers synapse on ipsilateral preganglionic
sympathetic fibers, exit the cord, travel to the
sympathetic chain as second-order neurons to the
superior cervical ganglion, and then synapse on
postganglionic sympathetic fibers
• The third-order neurons travel via internal carotid
artery to the orbit and innervate the (dilator) radial
smooth muscle of the iris
• Postganglionic sympathetic fibers also innervate the
muscle of Müller within the eyelid. This muscle is
responsible for initiating eyelid retraction during
eyelid opening
• Postganglionic sympathetic fibers responsible for
facial sweating follow the external carotid artery to
the sweat glands of the face. Interruption at any
location along this pathway results in ipsilateral
Horner’s syndrome
• Horner’s syndrome may result from a lesion of the
primary neuron; brainstem stroke or tumor or syrinx
of the preganglionic neuron; trauma to the brachial
plexus; tumors (e.g. Pancoast) or infection of the lung
apex; a lesion of the postganglionic neuron; dissecting
carotid aneurysm; carotid artery ischemia; migraine;
or middle cranial fossa neoplasm.
SYMPTOMS
Symptoms depend on the underlying cause:
• Patients may not be able to completely open the
affected eye and may not sweat on same side of the
face
• Patients with preganglionic lesions may have facial
flushing. This symptom (i.e. Harlequin effect) occurs
in some patients as a result of physical exercise
• Patients with postganglionic lesions may have
ipsilateral orbital pain or a migraine-like headache
• Raeder, a Norwegian Ophthalmologist, described
patients with a combination of orbital pain, miosis,
and ptosis and termed it paratrigeminal syndrome
• If this set of symptoms is associated with lesions of
the cranial nerves (CN) III through VI on the ipsilateral
side, suspect a mass lesion in the middle cranial fossa
(e.g. Raeder paratrigeminal syndrome, type I)
• A benign form characterized by episodic retrobulbar
or orbital pain, with miosis and ptosis but without
associated cranial nerve findings, is considered a
migraine variant (i.e. Raeder paratrigeminal
syndrome, type II)
• When associated with carotid artery dissection,
patients may present with ipsilateral head, neck, or
facial pain.
IMAGING
MRI brain is the recommended imaging of choice but
depending on the localization of the symptoms, MRI of
brain, neck and chest may be indicated. MRA brain and
neck may be needed in case of suspicion of painful
Horner’s secondary to carotid artery pathology.
BIBLIOGRAPHY
1. Albert DM, Jakobiec FA. Principles and Practice of
Ophthalmology 1994;4:2473-4.
2. American Academy of Ophthalmology. Basic and Clinical
Science Course: Neuro-ophthalmology 1999-2000;5:97-9,
109-11.
3. Fraunfelder FT. Drug-induced ocular side effects and drug
interactions. Baltimore: Williams and Wilkins 1996;4:553.
4. Gutman I, Levartovski S, Goldhammer Y, et al. Sixth nerve
palsy and unilateral Horner's syndrome. Ophthalmology
1986;93(7):913-6.
5. Loewenfeld I. The Pupil: Anatomy, physiology and clinical
applications. Iowa State University Press 1993;2:1131-77.
6. Mokhtari F, Massin P, Paques M, et al. Central retinal
artery occlusion associated with head or neck pain
revealing spontaneous internal carotid artery dissection.
Am J Ophthalmol 2000;129(1):108-9.
7. Roy FH. Ocular syndromes and systemic diseases. WB
Saunders 1989.
 Chapter 46 Supranuclear: Gaze Palsy 637

A B

C D

Figs 46.2A to D: A 14-year-old female presented with Horner’s syndrome showing a well-circumscribed mass lesion, hypointense
in T1 and hyperintense signal in T2WI in the right supraclavicular region—ganglioneuroma
Chapter 47
Olivopontocerebellar Atrophy
It is a degenerative disorder characterized by atrophy of
pons, middle cerebellar peduncles and cerebellar
hemispheres. Usually, cerebellar signs predominate.
CLINICAL FEATURES
• Patients with this disorder usually have slow eye
movements or inability to initiate saccades in any
direction. Nystagmus, optic atrophy, retinal or
macular degeneration usually occur
• They may have ataxia, pyramidal signs, extra-
pyramidal signs and posterior column dysfunction.
The inheritance is usually autosomal dominant.
IMAGING
• Small inferior olives and medulla, a small flattened
pons, and atrophic cerebellar hemispheres and vermis
are seen
• High signal intensities on T2-weighted MRI is often
seen in the transverse pontine fibers and brachium
pontis
• The putamen, globus pallidus and substantia nigra
frequently show abnormally low signal intensity on
T2-weighted image.
BIBLIOGRAPHY
1. Giuffrida S, Saponara R, Restivo DA, Trovato Salinaro A,
Tomarchio L, Pugliares P, Fabbri G, Maccagnano C.
Supratentorial atrophy in spinocerebellar ataxia type 2:
MRI study of 20 patients. J Neurol 1999;246(5):383-8.
2. Hamaguchi H, Kanda F, Hosaka K, Fujii M, Chihara K.
Comparison between MRI and 3D-SSP in olivoponto-
cerebellar atrophy and cortical cerebellar atrophy. Rinsho
Shinkeigaku 2004;44(4-5):263-7.
3. Ikuta N. Proton magnetic resonance spectroscopy and single
photon emission CT in patients with olivopontocerebellar
atrophy. Rinsho Shinkeigaku 1998;38(4):289-94. Japanese.
4. Okamoto K, Tokiguchi S, Furusawa T, Ishikawa K,
Quardery AF, Shinbo S, Sasa K. MR features of diseases
involving bilateral middle cerebellar peduncles. Am J
Neuroradiol 2003;24(10):1946-54.
5. Savoiardo M, Grisoli M, Girotti F, Testa D, Caraceni T.
MRI in sporadic olivopontocerebellar atrophy and
striatonigral degeneration. Neurology 1997;48(3):790-2.
6. Schrag A, Rinne JO, Burn DJ, Mathias CJ, Marsden CD,
Brooks DJ, Quinn NP. Olivopontocerebellar atrophy and
multiple system atrophy: Clinical follow-up of 10 patients
studied with PET. Ann Neurol 1998;44(1):151-2.
7. Uchino A, Sawada A, Takase Y, Kudo S. Symmetrical
lesions of the middle cerebellar peduncle: MR imaging
and differential diagnosis. Magn Reson Med Sci 2004;
3(3):133-40.
 Chapter 47 Olivopontocerebellar Atrophy 639

A B

Figs 47.1A to C: (A) Axial T2-weighted image at the level of medulla,

(B) Pons showing marked atrophy of the pons, medulla and
cerebellum—olivopontocerebellar atrophy and (C) Sagittal
T2-weighted image showing atrophy of the cerebellum, medullary
olives, pons corpus callosum. Hyperintense signal is seen in the
C pontine fibers
Chapter 48

Nystagmus
It is defined as a rhythmic biphasic oscillation of the eyes. • Ocular bobbing is typically seen with extensive pontine
It usually results from a defect in the slow movement lesions causing a horizontal gaze palsy, but may be
system. Nystagmus refers to an abnormal to and fro observed with diffuse processes affecting the CNS.
movement of the eyes. Familiarity with the localization of specific nystagmus
types increases the diagnostic acumen of the neuroradio-
TYPES OF NYSTAGMUS logist because high resolution studies can then be tailored
to regions of expected pathology.
• Pendular nystagmus: In pendular nystagmus, the slow
phases are of equal velocity and there are no corrective
TYPES OF NYSTAGMUS—AGE
saccades
• Jerk nystagmus: There is a slow phase followed by a Type of nystagmus Childhood Any age
rapid corrective saccade in the opposite direction. Jerk Latent Vestibular
Many forms of nystagmus have exquisite localizing Peripheral and
value. central
Gaze evoked
CLINICAL SYMPTOMS Physiologic
Pathologic
• Nystagmus may be asymptomatic or produce a Brun’s
jumping of visual environment called oscillopsia. Dissociated
A patient with nystagmus will see the visual Periodic alternating
environment moving in the direction of fast phase Downbeat
• In congenital nystagmus, there is loss of visual acuity Upbeat
Convergence
without oscillopsia
retraction
• Nausea and vomiting may accompany nystagmus in Epileptic
patients with peripheral vestibular disease. Hearing Drug-induced
loss and tinnitus may also be noted in peripheral Optokinetic
vestibular disease See-saw
• In nystagmus of brainstem, origin diplopia, dysarthria, Pendular Spasmus mutans Oculopalatal
facial numbness and dysphagia may be seen Monocular Oculomasticatory
• Other eye oscillations such as ocular dysmetria, nystagmus due to myorhythmia
macrosquare-wave jerks, ocular flutter, and visual deprivation/ See-saw
loss
opsoclonus are often associated with cerebellar
Either pendular Congenital
disorders and/or jerk
 Chapter 48 Nystagmus 641

A B

Figs 48.1A and B: Axial T1-weighted images showing T1-hyperintense signal in the right
cerebellar tonsils in this patient with nystagmus—hemorrhage

A B

C D

Figs 48.2A to D: A 2-year-old child presented with ataxia and nystagmus: (A and C) Axial T1-
and T2-weighted MRI images at the level of the medulla and (B and D) Pons showing diffuse
cerebellar atrophy with normal appearing brainstem
642 Section 7 Efferent Pathway Lesions

LOCALIZING NYSTAGMUS – Cerebellar cortical degenerations

– Ataxia telangiectasia
Type Location
– Spinocerebellar degeneration
Brun’s nystagmus Cerebellopontine angle – Olivopontocerebellar degeneration
Convergence retraction Dorsal mesencephalon
– Friedreich’s ataxia
Dissociated Medial longitudinal fasciculus
Downbeat Craniocervical junction
– Paraneoplastic cerebellar degeneration (remote
Gaze-evoked Vestibular, cerebellum effect of breast, ovary or small cell lung cancer)
Oculopalatal myoclonus Dento-rubro-olivary connections • Vascular diseases:
(central tegmental tract) – Occlusion of posterior inferior cerebellar artery
Periodic alternating Craniocervical junction leading to asymmetric infarction of vestibulocere-
Rebound Cerebellum bellum produces central vestibular disturbance—
See-saw Parasellar, mesencephalon gaze-evoked nystagmus
Spasmus mutans Exclude chiasmal glioma – Infarction of anteroinferior cerebellar artery
Torsional Central vestibular or cerebellum
involvement of labyrinth, vestibular nuclei and
Upbeat Pontomesencephalic,
pontomedullary, cerebellar flocculus.
– Infarction of the superior cerebellar artery
• Mass lesions:
LESIONS OF CEREBELLUM – Tumors—Intra- and extra-axial tumors
• The lesions in the cerebellum can produce varying – Abscesses/cysts
neuro-ocular signs depending on the location of the – Extra-axial hematomas
lesions – Medulloblastomas
• Three principal syndromes can be identified – Tumors within the 4th ventricle
• The syndrome of the dorsal vermis and underlying • Acute cerebellar hemorrhage causes nystagmus, gaze
posterior fastigial nuclei leading to saccadic dysmetria palsy usually towards side of lesion, abducens nerve
and impaired smooth pursuit palsy and skew deviation.
• The syndrome of flocculus and paraflocculus leading
to impaired smooth pursuit and cancellation of LESIONS OF THE THALAMUS
vestibulo-ocular reflex (VOR), fixation suppression of
• The lesions in the thalamus result in disturbances of
caloric nystagmus, gaze-evoked, rebound, centripetal
both horizontal and vertical gazes. The most common
and downbeat nystagmus, postsaccadic drift, inappro-
lesions seen in the thalamus are ischemic and
priate VOR
neoplasm
• The syndrome of nodulus and ventral uvula leading
to periodic alternating nystagmus, positional • The clinical symptoms may vary with the location of
nystagmus, impaired habituation of VOR, increased the lesion in the thalamus and associated involvement
duration of vestibular responses of the midbrain
• Lesions of middle cerebellar peduncle show torsional • Thalamic hemorrhage can cause conjugate deviation
nystagmus during smooth pursuit of the eyes contralateral to the side of lesion may occur.
• Other signs of cerebellar lesions with no localizing Forced downward deviation of the eyes with con-
value include square-wave jerks, esotropia vergence and miosis
(divergence paralysis) with alternating skew • Esotropia occurs in patients with caudal thalamic
deviation, disconjugate saccades with disconjugate lesions
gaze-evoked nystagmus, divergent nystagmus, • In combined lesions of thalamus and midbrain paresis
centripetal nystagmus, and upbeating nystagmus. of convergence occurs
Cerebellar lesions may also compress the brainstem • In posterolateral thalamic infarct disturbances of the
and produce additional signs subjective visual vertical may occur. Disturbances of
• Etiologies: arousal and short-term memory
– Developmental anomalies • Central thalamic lesions can cause double-step
– Arnold-Chiari malformation thalamic saccade paradigms
– Dandy-Walker syndrome • Pulvinar lesions can cause difficulty in shifting gaze
– Degenerative disease and attention into the contralateral hemifield.
 Chapter 48 Nystagmus 643

Figs 48.3A and B: A 1½-year-old child with optic atrophy and

nystagmus: (A) Axial T1-weighted images at the level of the
cerebellum and (B) Lateral ventricle showing absent vermis with
gross asymmetric dilatation of the lateral ventricle (L > R) and
B paucity of the white matter
644 Section 7 Efferent Pathway Lesions
IMAGING
• It is mandatory to know the localization of various
types of nystagmus in order to perform the imaging
focusing on the region of interest.
• MRI is the imaging modality of choice as the posterior
fossa and brainstem are best evaluated by this
modality.
BIBLIOGRAPHY
1. Bronstein AM, Miller DH, Rudge P, Kendall BE. Down-
beating nystagmus: Magnetic resonance imaging and
neuro-otological findings. J Neurol Sci 1987;81(2-3):
173-84.
2. de Seze J, Vukusic S, Viallet-Marcel M, Tilikete C,
Zéphir H, Delalande S, Stojkovic T, Defoort-Dhellemmes
S, Confavreux C, Vermersch P. Unusual ocular motor
findings in multiple sclerosis. J Neurol Sci 2006;243(1-2):
91-5.
3. Jacobs DA, Galetta SL. Neuro-ophthalmology for
neuroradiologists. Am J Neuroradiol 2007;28(1):3-8.
4. Lin CY, Young YH. Clinical significance of rebound
nystagmus. Laryngoscope 1999;109(11):1803-5.
5. Slamovits TL, Gardner TA. Neuroimaging in neuro-
ophthalmology. Ophthalmology 1989;96(4):555-68.
Review
6. Wagner J, Lehnen N, Glasauer S, Rettinger N, Büttner U,
Brandt T, Strupp M. Downbeat nystagmus caused by a
paramedian pontomedullary lesion. J Neurol
2009;256(9):1572-4.
7. Wagner JN, Glaser M, Brandt T, Strupp M. Downbeat
nystagmus: Aetiology and comorbidity in 117 patients.
J Neurol Neurosurg Psychiatry 2008;79(6):672-7.
 Chapter 48 Nystagmus 645

A B

C D

Figs 48.4A to D: A 20-year-old with biplanar nystagmus and left jerk nystagmus OS-RAPD: (A) Axial T1-weighted, (B) Axial
FLAIR showing a well-defined T1- and T2-hyperintense lesion in the quadrigeminal plate suggestive of lipoma, (C) Coronal T2
and (D) T1-weighted image showing the lipoma
646 Section 7 Efferent Pathway Lesions

A B

C D

E F

Figs 48.5A to F: A 1-year-old child presented with see-saw nystagmus: (A) Axial CT scan showing
thickening of the chiasm, (B) Axial T2-weighted, (C) Axial T1-weighted, (D) Coronal T1-weighted images
showing nodular thickening of the left half of the chiasm and (E and F) Sagittal T1 postcontrast image
showing no enhancement of the lesion—differential diagnosis includes chiasmatic glioma and hamartoma
 Chapter 48 Nystagmus 647

A B

Figs 48.6A and B: An 8-year-old boy presented with see-saw nystagmus: (A) Axial postcontrast CT
scan and (B) Coronal postcontrast CT scan of the brain showing a well-defined hypodense poorly
enhancing lesion in the suprasellar region. No calcification seen—craniopharyngioma

A B

Figs 48.7A and B: (A) Axial T2-weighted image and (B) Axial T1-weighted postcontrast image showing
an ill-defined mass in the left middle cerebellar peduncle causing expansion of the left half of the pons
which is suggestive of glioma in a case of nystagmus
648 Section 7 Efferent Pathway Lesions
ARNOLD-CHIARI MALFORMATION
German Pathologist, Hans Chiari described congenital
hindbrain abnormalities in which cerebellar tonsils
descends into the cervical canal.
Four types of Chiari malformations are described in
the literature:
1. Type I
2. Type II
3. Type III
4. Type IV.
TYPE I CHIARI MALFORMATION
• Chiari I malformation (CMI) is characterized by
herniation of the cerebellar tonsils through the
foramen magnum into the cervical spinal canal. The
cerebellar tonsils often are elongated and peg-like.
Mild caudal displacement and flattening or kinking
of the medulla may be present. The vermis cerebelli
and the fourth ventricle are normal or only minimally
deformed.
Criteria for tonsilar ectopia:
First decade of life—6 mm
10 to 30 years—5 mm
30 to 80 years—4 mm
• CMI is not directly associated with other congenital
brain malformations, specifically myelomeningocele.
Clinical Features
Ocular symptoms, including retro-orbital pain, visual
disturbances, photophobia, and diplopia. Nonocular
symptoms are suboccipital headaches, otoneurological
symptoms, syringomyelia-related and spinal cord
dysfunction symptoms and hindbrain compression
symptoms.
Neuroimaging
CT Scan
• A crowded foramen magnum is seen with small or
absent posterior fossa cisterns
• The lateral and third ventricles may be normal. There
may or may not be ventriculomegaly and this depends
on the degree of foramen magnum impaction.
MRI
• MRI has revolutionized the diagnostic evaluation for
CMI. It may be noted an incidental finding in patients
undergoing brain or cervical spine MRI
• Tonsillar position, tonsillar configuration, and many
associated abnormalities are depicted on sagittal and
axial T1- and T2-weighted MRIs
• MRI may not reliably demonstrate abnormal findings
of the skeleton associated with Chiari malformations.
Conventional radiographs or CT are preferred for
skeletal abnormalities
• The degree of tonsillar ectopia is expressed as the
number of millimeters that the tonsils tip below a line
connecting the basion with the opisthion
• Measurements are done on a sagittal T1-weighted
image. Tonsillar tips that extend less than 3 mm below
the foramen magnum are normal
• In Chiari malformation type I pointed, triangular
shaped “peg-like” tonsils are seen that lie more than
5 mm below the foramen magnum.
• Narrowing or obliteration of the retrocerebellar CSF
spaces is observed in an association with a meniscus
sign at the lower pole of the cerebellar tonsils
• Small bony posterior fossa is seen with effaced
cisterns. Short clivus is also seen. Associated
syringohydromyelia is seen in 14 to 75 percent cases
• Narrowing of the CSF pathways at the foramen
magnum, at the C2-3 disc level, and in the posterior
subarachnoid space below the tip of the cerebellar
tonsils
• Obstructed CSF flow across foramen magnum can be
demonstrated on phase contrast CSF flow studies.
• Arnold-Chiari malformations types III and IV are very
rare and mostly patients do not survive. In type III,
patients have cerebellar herniation into high cervical
myelomeningocele. In type IV malformation, patients
have cerebellar agenesis. The III and IV types of
malformations are postulated to have different
etiopathogenesis from the other 2 types (Type I and II).
ARNOLD-CHIARI II
• The Chiari II malformation is a complex congenital
malformation of the brain, nearly always associated
with myelomeningocele
• This condition includes downward displacement of
the medulla, fourth ventricle, and cerebellum into the
cervical spinal canal, as well as elongation of the pons
and fourth ventricle, probably due to a relatively small
posterior fossa
• Neonatal Chiari II malformations continue to result
in significant morbidity and mortality. Hindbrain
dysfunction is the major cause of the mortality
 Chapter 48 Nystagmus 649

Fig. 48.8: Sagittal T1-weighted MRI showing tonsillar ectopia

in a patient who presented with downbeat nystagmus. Syrinx is
noted in the upper cervical cord

A B

Figs 48.9A and B: (A) Sagittal T2-weighted image showing a myelomeningocele (arrow), lumbar syringohydromyelia (dotted arrow)
and tonsilar herniation (black arrow) and (B) Axial FLAIR image of the brain showing hydrocephalus—type II Chiari malformation
650 Section 7 Efferent Pathway Lesions
• Clinical presentations: In Chiari II malformations,
infants, particularly neonates, demonstrate rapid
progressive neurologic deterioration
• A common and striking symptom initially present is
inspiratory stridor when the infant cries. Episodes of
stridor and apnea frequently herald impending
brainstem compromise and subsequent development
of dysphagia or nasal regurgitation, aspiration,
quadriparesis, and opisthotonic posturing.
IMAGING
CT
Skull Abnormalities
• Lacunar skull which is universal at birth and largely
resolves by 2 years of age is seen. This involves inner
and outer tables (squamous bones). This is caused by
a mesenchymal defect and not by a raised intracranial
pressure
• A small posterior fossa with low lying tentorium. The
foramen magnum is usually large and funnel-shaped.
A scalloped petrous pyramid and notched clivus is
also present.
Dural Abnormalities
• These include a fenestrated or hypoplastic falx with
interdigitated gyri and heart-shaped incisura
• CT also demonstrates tectal beaking, cerebellar tissue
wrapping around the brainstem, fenestrations of the
falx, manifestations of hydrocephalus, and shunt
malfunction.
MRI
Ventricles
• The lateral ventricles have pointed anterior horns with
colpocephaly
• The third ventricle is high riding with large massa
intermedia if there is corpus callosal agenesis
• The fourth ventricle is elongated with no posterior
point (fastigium)
• Small posterior fossa is seen
• Towering cerebellum protrudes up through incisura
compresses the midbrain, and causes beaked tectum
• A cascade or waterfall of cerebellum and brainstem
downwards is seen the uvula/nodulus/pyramid of
vermis forms a sclerotic peg. The medulla kinking or
spurring is noted. Seventy percent cases have
Z-shaped cervicomedullary junction
• Twenty-to-ninety percent cases have associated
hydrosyringomyelia.
BIBLIOGRAPHY
1. Amer TA, el-Shmam OM. Chiari malformation type I: A
new MRI classification. Magn Reson Imaging 1997;15(4):
397-403.
2. Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM.
Significance of cerebellar tonsillar position on MR. Am J
Neuroradiol 1986;7(5):795-9.
3. Dure LS, Percy AK, Cheek WR, Laurent JP. Chiari type I
malformation in children. J Pediatr 1989;115(4):573-6.
4. Elster AD, Chen MY. Chiari I malformations: Clinical and
radiologic reappraisal. Radiology 1992;183(2):347-53.
5. Masson C, Colombani JM. Chiari type 1 malformation and
magnetic resonance imaging. Presse Med 2005;34(21):
1662-7. Review.
6. Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS.
Asymptomatic Chiari Type I malformations identified on
magnetic resonance imaging. J Neurosurg 2000;92(6):
920-6.
7. Milhorat TH, Chou MW, Trinidad EM, Kula RW,
Mandell M, Wolpert C, Speer MC. Chiari I malformation
redefined: Clinical and radiographic findings for 364
symptomatic patients. Neurosurgery 1999;44(5):1005-17.
8. Payner TD, Prenger E, Berger TS, Crone KR. Acquired
Chiari malformations: Incidence, diagnosis, and
management. Neurosurgery 1994;34(3):429-34; discussion
434. Review.
9. Pillay PK, Awad IA, Little JR, Hahn JF. Symptomatic
Chiari malformation in adults: A new classification based
on magnetic resonance imaging with clinical and
prognostic significance. Neurosurgery 1991;28(5):639-45.
10. Rauzzino M, Oakes WJ. Chiari II malformation and
syringomyelia. Neurosurg Clin N Am 1995;6(2):293-309.
Review.
11. Ventureyra EC, Aziz HA, Vassilyadi M. The role of cine
flow MRI in children with Chiari I malformation. Childs
Nerv Syst 2003;19(2):109-13.
12. Wolpert SM, Bhadelia RA, Bogdan AR, Cohen AR. Chiari I
malformations: Assessment with phase-contrast
velocity MR. Am J Neuroradiol 1994;15(7):1299-308.
13. Yamazaki Y, Tachibana S, Takano M, Fujii K. Clinical and
neuroimaging features of Chiari type I malformations with
and without associated syringomyelia. Neurol Med Chir
(Tokyo). 1998;38(9):541-6; discussion 546-7.

DANDY-WALKER SYNDROME
• Dandy-Walker (DW) syndrome represents a broad-
spectrum of cystic posterior fossa malformations.
Other anomalies are present in most cases
• This consists of a malformation of the cerebellar
vermis, a membranous cyst of the 4th ventricle, and
malformation of the cerebellar cortex and deep
cerebellar nuclei.
CLINICAL FEATURES
• Ocular: Mild saccadic dysmetria, nystagmus,
strabismus and sometimes normal eye movements
• General: Eighty percent are diagnosed by 1 year
(macrocrania, bulging fontanel, etc.)
• Later: Seizures, developmental delay, poor motor
skills/balance. Early death has been noted to occur.
NEUROIMAGING
General Features
The best diagnostic sign is a large posterior fossa and
CSF cyst and an absent normal 4th ventricle.
The Dandy-Walker spectrum includes:
1. Fourth ventriculocele (10-15% cases):
• The large cyst erodes occipital bone leading to
encephalocele
• Encysted 4th ventricle herniates into the occipital
encephalocele.
2. Classic DW:
• Cystic dilatation of 4th ventricle leading to
enlarged posterior fossa
• Agenesis or hypoplasia of the cerebellar vermis
may be seen superior vermis remnant may be
rotated up over the cyst
• Compressed brainstem
• High tentorium, venous sinus confluence.
3. DW variant (mild form of Dandy-Walker complex):
• Vermian hypoplasia + partial obstruction of 4th
ventricle
• Prominent vallecula (keyhole appearance)
Chapter 48 Nystagmus 651
• Small cyst with normal posterior fossa and
brainstem
4. Mega cisterna magna:
• Enlarged posterior fossa, normal vermis
• Normal 4th ventricle with no cyst or compression.
CT Findings
A large posterior fossa is seen with:
• Variable sized cyst
• Torcular-lambdoid inversion (torcular above lambdoid
suture)
• Occipital bone may be scalloped, remodeled with all
DW types including mega cisterna magna.
MR Findings
Sagittal T1-weighted images shows:
• Floor of the 4th ventricle is present and is open
dorsally to variable-sized CSF-containing cyst
• Vermian remnant is rotated up over cyst
• Elevated torcular with high/steeply sloping tentorium
(classic).
DIFFERENTIAL DIAGNOSIS
• Arachnoid cyst
• Joubert’s anomaly.
BIBLIOGRAPHY
1. Barkovich AJ, et al. Revised classification of posterior fossa
cyst and cyst-like malformations based on the results of
multiplanar MR imaging. AJR 1989;153:1289-300.
2. Lavanya T, Cohen M, Gandhi SV, Farrell T, Whitby EH.
A case of a Dandy-Walker variant: The importance of a
multidisciplinary team approach using complementary
techniques to obtain accurate diagnostic information. Br J
Radiol 2008;81:970.
3. Schmidt MJ, Jawinski S, Wigger A, Kramer M. Imaging
diagnosis: Dandy-Walker malformation. Vet Radiol
Ultrasound 2008;49(3):264-6.
652 Section 7 Efferent Pathway Lesions

A B

Figs 48.10A to C: Axial plain CT scan of the brain showing

absent vermis with keyhole fourth ventricle communicating with
C a posterior fossa cyst—Dandy-Walker syndrome

JOUBERT’S SYNDROME
• Joubert’s syndrome is a rare congenital malformation
of the brain which includes a variable combination of
the central nervous system defect with a distinctive
congenital retinal dystrophy, ocular motor abnormalities
and respiratory abnormalities in early infancy
• It is characterized by the absence or under
development of the cerebellar vermis
• Dysgenesis or absence of cerebellar vermis, creates
the characteristic “molar tooth sign” with deep inter-
peduncular fossa and thickened cerebellar peduncles
• Genetic basis is unknown. Most cases are sporadic
but autosomal recessive inheritance has been
described in some family.
OCULAR ABNORMALITIES
• Congenital retinal dystrophy—abnormal retinal
pigmentation
• Supranuclear ocular motor palsies
• Nystagmus—pendular torsional, see-saw, gaze-
evoked
• Impaired smooth pursuit and saccades
• Ocular motor apraxia
• Inability to cancel vestibulo-ocular reflex
• Strabismus
• Chorioretinal coloboma
• Ptosis.
SYSTEMIC ABNORMALITIES
• Truncal ataxia
• Hypotonia
• Developmental delay
• Mental retardation
• Episodic tachypnea/apnea
• Physical deformities (rare)—polydactyly, cleft lip,
cleft palate, tongue abnormalities
• Seizures (rare).
ELECTROPHYSIOLOGICAL TESTS
Electroretinography: Flash ERG usually shows non-
recordable or highly attenuated response.
Visually evoked potential: Flash and pattern VEP can
be normal.
Chapter 48 Nystagmus 653
NEUROIMAGING
CT/MRI Scan
• The dysgenetic vermis appears split or segmented
and disorganized. The inferior and superior cere-
bellar peduncles are often small and the fourth
ventricular roof appears superiorly convex on sagittal
MR scans. Hydrocephalus is not a feature of this
syndrome
• Dysgenesis or absence of cerebellar vermis
• Dilated IV ventricle
• “Molar tooth” sign is used to describe the appearance
of the midbrain resulting from the deep inter-
peduncular fossa and thickened cerebellar peduncles.
BIBLIOGRAPHY
1. Hentschel F, Marcus A, Klein M, Schmidt MH. Joubert's
syndrome: Computerized tomography and magnetic
resonance tomography. German Rofo 1996;165(2):210-2.
2. Joubert M, Eisenring J, Robb JP, et al. Familial agenesis of
the cerebellar vermis. Neurology 1969;19:813-25.
3. Kendall B, Kingsley D, Lambert SR, Taylor D, Finn P.
Joubert syndrome: A clinico-radiological study.
Neuroradiology 1990;31(6):502-6.
4. Maria BL, Hoang KB, Tusa RJ, Mancuso AA, Hamed LM,
Quisling RG, Hove MT, Fennell EB, Booth-Jones M,
Ringdahl DM, Yachnis AT, Creel G, Frerking B. Joubert
syndrome revisited: Key ocular motor signs with magnetic
resonance imaging correlation. J Child Neurol 1997;12(7):
423-30.
5. Maria BL, Quisling RG, Rosainz LC, Yachnis AT, Gitten J,
Dede D, Fennell E. Molar tooth sign in Joubert syndrome:
Clinical, radiologic, and pathologic significance. J Child
Neurol 1999;14(6):368-76.
6. Padgett KR, Maria BL, Yachnis AT, Blackband SJ. Ex vivo
high-resolution magnetic resonance imaging of the brain
in Joubert's syndrome. J Child Neurol 2002;17(12):911-3.
7. Scott RL, Anthony K, Grestly M, Benton S, Taylor D, et al.
Joubert syndrome. Archives of Ophthalmology 1989;107:
709-13.
8. Sener RN. MR imaging of Joubert's syndrome. Comput
Med Imaging Graph 1995;19(6):481-6.
9. Shen WC, Shian WJ, Chen CC, Chi CS, Lee SK, Lee KR.
MRI of Joubert's syndrome. Eur J Radiol 1994;18(1):30-3.
654 Section 7 Efferent Pathway Lesions

B C

Figs 48.11A to C: External photo a 2-year-old child with Joubert’s syndrome: (A) Showing facial asymmetry and (B and C) Color
fundus photos showing abnormal retinal pigmentation in the above patient
 Chapter 48 Nystagmus 655

A B

C D

Figs 48.12A to D: (A) Axial CT scan, (B) Coronal T2, (C) Axial T2-weighted and (D) Axial T1-weighted image showing absent
vermis with “Molar Tooth” sign (D) (arrow)
656 Section 7 Efferent Pathway Lesions

A B

Figs 48.13A to C: (A) Axial T1-weighted, (B) Axial T2-weighted,

(C) Sagittal T1-weighted. Figure (A) showing absent vermis
with CSF cleft extending up to the fourth ventricle. Figure (B)
C showing the “Molar Tooth” sign (arrow)
SECTION 8
Demyelination
SECTION OUTLINE
49. Primary Demyelination
50. Secondary Demyelination
Chapter 49
Primary Demyelination
Demyelination refers to loss of myelin sheath of the
neurons.
• This can be further divided into primary and
secondary demyelination based on their etiologies:
Primary: Disorders that occur of unknown origin like
multiple sclerosis. Some may be due to genetic factors.
The primary form destroys or damages myelin or
myelin-forming cells and the axons are preserved
early in the early stages
Secondary: Demyelination follows vascular, infectious
or toxic insults. Secondary demyelination, follows
damage to neurons or axons, followed by breakdown
of myelin
• The pattern of involvement vary in size, shape and
distribution, in the speed with which they appear, and
with their ability to recover
• Some may follow viral infections such as measles and
smallpox or vaccination.
MULTIPLE SCLEROSIS
• Multiple sclerosis (MS) is the most common type of
primary demyelinating disorder that affects the long
white tracts of the central nervous system
• Incidence varies considerably in different geographic
area, it is considered rare in Asia and Africa
• Age and sex: Female preponderance of 2:1. It is
uncommon under 10 years of age and the peak
incidence is among young adults 25 to 40 years of age
• Genetic factors probably play a role because the
incidence is approximately 20 times greater in first-
degree relatives of patients with the disease
• Variants of MS: Less common variants of MS are
occasionally seen and differ from classic MS in their
clinical presentation and course as well as
histopathologic findings
• Acute MS (Marburg type): Occurs as an infrequent
variety of MS, most commonly in younger patients.
It is often preceded by fever and typically has
inexorable, rapid progression to death within months.
This fulminant form of MS has also been seen as a
terminal event in classic MS. Pathologic findings of
extensive myelin destruction, severe axonal loss, and
early edema are seen. Treatment is directed at
reducing the inflammation. Although acute fulminant
MS is associated with high morbidity and mortality,
it may respond to aggressive immunosuppressive
therapy
• Neuromyelitis optica (Devic type): It is a syndrome
of acute onset of severe optic nerve and spinal cord
demyelination that appear approximately the same
time and dominate the clinical picture. Often, the
symptoms and signs are not in complete isolation, but
are part of a more generalized disorder. About one-
half of these patients die within several months. It is
clear that several entities can produce this clinical
syndrome, but the term is reserved for those without
other underlying diseases. The relationship of Devic
syndrome to MS is still controversial
• Schilder type, or diffuse sclerosis: Refers to an entity
consisting of extensive, confluent, asymmetric
demyelination of both cerebral hemispheres with
involvement of the brainstem and cerebellum. Late
in the disease, Wallerian degeneration and cavitation
can be seen
• Concentric sclerosis (Balo type): It is a very rare type
of demyelinating disease in which large regions with
alternating zones of demyelinated and myelinated
white matter are found. The myelinated regions may
reflect remyelination, rather than spared normal
myelin. This progressive disease is more often found
in young patients and is more common in the
660 Section 8 Demyelination

A B C

D E F

Figs 49.1A to F: (A to C) Axial FLAIR images of the brain at different levels showing multiple oval lesions of varying sizes in the
periventricular white matter (arrow), (D and E) Sagittal T2-weighted image of the brain showing multiple T2-hyperintense lesions
in the pons and corpus callosum and (F) Axial diffusion weighted image (b = 600) showing hyperintense signal in the right
periventricular lesion

Philippines. Balo concentric sclerosis has a
pathognomonic appearance on both pathology and
MRI
• Diagnosis: Diagnosis of multiple sclerosis is primarily
clinical. The diagnosis of MS is made by identifying
neurologic symptoms that are separated over and
affect different areas (long white tracts) of the central
nervous system. The eye is frequently involved and
may precede, occur concurrently or follow the
development of other signs and symptoms.
Ophthalmic Features
1. Optic neuritis: The most common ocular presentation
occurring in 75 percent of MS patients. May present
as papillitis or retrobulbar neuritis. The patient may
note visual fluctuations after exercise or a hot bath
(Uhthoff’s phenomenon). MRI is of great value in
predicting the development of multiple sclerosis. MS
occurs in 51 percent of patients with 3 or more MS
lesions
2. Fundoscopic abnormalities: Nerve fiber bundle
defects may be noted following an attack of optic
neuritis. Retinitis and periphlebitis occur in 5 to 10
percent of patients with multiple sclerosis. Anterior
uveitis has been noted in 5 percent of patients
3. Involvement of the optic chiasm, optic tracts and
visual radiations is quite rare but has been reported.
The involvement of the above may be marked by
concurrent optic nerve disease
4. Ocular motility disturbances: The patient may note
several attacks of diplopia before an ocular motility
becomes apparent. Supranuclear, nuclear and
fascicular motility disturbances may be noted.
Bilateral internuclear ophthalmoplegias in an
individual < 50 years is highly suggestive of multiple
sclerosis. Other ocular motility disturbances that
have reported include the one and half syndrome,
vertical or horizontal gaze palsy with a skew
deviation. Isolated ocular motor nerve palsies are
unusual but should be considered in the differential
diagnosis of a young adult with no history of trauma.
Nystagmus is common in MS. It may be horizontal,
rotatory or vertical, pendular or jerk. Concomitant
vertical and horizontal nystagmus occurring out of
phase producing circular or elliptical eye movements
is highly suggestive of MS
5. Other system involvement:
• Cerebellar dysfunction—ataxia, dysarthria,
intention tremor, truncal or head titubation,
dysmetria
Chapter 49 Primary Demyelination 661
• Motor symptoms—extremity weakness, facial
weakness (Bell’s palsy), hemiparesis or paraplegia.
• Sensory symptoms: Paresthesias of face or body,
Lhermitte’s sign
• Mental changes—emotional instability, depress-
ion, euphoria
• Sphincter disturbances—frequency, urgency,
hesitancy, incomitance, urinary retention.
Poser’s criteria commonly used for the diagnosis of
multiple sclerosis:
Clinically Definite MS
• 2 attacks and clinical evidence of 2 separate lesions
• 2 attacks, clinical evidence of one and paraclinical
evidence of another separate lesion.
Laboratory Supported Definite MS
• 2 attacks, either clinical or paraclinical evidence of
1 lesion, and cerebrospinal fluid (CSF) immunologic
abnormalities
• 1 attack, clinical evidence of 2 separate lesions and
CSF abnormalities
• 1 attack, clinical evidence of 1 and paraclinical
evidence of another separate lesion, and CSF
abnormalities.
Clinically Probable MS
• 2 attacks and clinical evidence of 1 lesion
• 1 attack and clinical evidence of 2 separate lesions
• 1 attack, clinical evidence of 1 lesion, and paraclinical
evidence of another separate lesion.
Laboratory Supported Probable MS
2 attacks and CSF abnormalities.
RADIOLOGICAL FEATURES
• MRI is the investigation of choice in detecting white
matter lesions in the brain and spinal cord. In multiple
sclerosis, the demyelination may occur anywhere in
the brain but most specific areas are periventricular
white matter, brainstem, cerebellum, and spinal cord.
Because of the inflammation and breakdown of the
blood-brain barrier in MS lesions, the presence of
extravascular fluid leads to hyperintensity on T2-
weighted images. In a patient with MS, MRI typically
demonstrates more than 1 hyperintense white matter
lesion
662 Section 8 Demyelination

A B

C D

Figs 49.2A to D: A 9-year-old boy presented with bilateral divergent squint and poor vision due to optic atrophy in both the eyes:
(A) Axial T1-weighted image, (B) Axial FLAIR showing large lesions in the parietal lobes bilaterally with central hypointense signal
suggestive of necrosis, (C) Coronal T2-weighted image showing the lesions to be hyperintense and (D) Coronal postcontrast
T1-weighted image showing no enhancement within the lesion—tumefactive MS

• The number of lesions paralleling the disease severity,
though not correlating with the clinical signs and
symptoms. It remains the single most important
investigation in prognosticating MS. If no lesions are
detected in optic neuritis in MRI, the risk of
developing MS is as low as 12 percent. These patients
should be followed up every 3 months up to 1 year
• Brain: T1-weighted sequences—not very specific for
detection of demyelination. Acute lesions may not be
seen at all. Low signal in T1-weighted image suggest
chronic lesions. Diffuse cerebral atrophy may be noted
in chronic MS
• Intracranial involvement with MS may appear quite
similar to many other white matter diseases on MRI,
with scattered foci of high intensity in the white matter
on T2-weighted spin-echo images. Contrast
enhancement may be used to add specificity to the
finding of multiple hyperintensities on T2-weighted
images, since the finding of enhancing along
with nonenhancing lesions is quite common in MS
but makes many other diagnoses unlikely. Similarly,
the temporal changes in enhancing and nonenhancing
lesions common in MS cases is very different from
other entities
• Periventricular lesions of MS commonly appear as
linear abnormalities oriented perpendicular to the
lateral ventricle. Although this is common, the
appearance of an MS lesion on MRI is highly variable
and certainly not specific
• MS can also appear as very subtle, diffuse
hyperintensity in the white matter. Moreover, it has
been shown by several studies that quantitative
analysis of relaxation times in “normal appearing”
white matter in MS patients differs from that of normal
volunteers. These nonvisual findings of quantitative
differences in proton relaxation times may be a
reflection of the subtle histologic changes reported by
pathology studies found in the absence of focal
plaques or macroscopic lesions in brain known to be
affected by MS. Finally, it has even been reported that
meningeal enhancement after intravenous contrast
agent administration has been seen in association with
MS
• In the spinal cord, however, on unenhanced T1-
weighted images, depending on their age, MS plaques
appear as areas of isointense signal or low signal.
Plaques may appear as nodules, rings, or arcs and
generally are less than 2 vertebral bodies in length.
Active lesions may show nodular, ring-like or
incomplete ring or arc-like enhancement on post-
contrast studies.
Chapter 49 Primary Demyelination 663
• The characteristic appearance of brain lesions on T2-
weighted images:
1. All lesions appear bright on a T2-weighted image
2. Dawson’s fingers—typically described for ovoid
lesions that occur perpendicular to the ventricles
along the deep medullary veins. Periventricular
lesions are very common in MS
3. The most specific site is the corpus callosum at its
interface with the septum pellucidum
4. Hyperintense signal may also be noted in the optic
nerves and chiasm.
• The cord lesions are hyperintense on T2 sequences.
Focal enlargement of the spinal cord with edema
around the lesions may be seen in acute lesion. Post-
contrast studies will show variable enhancement in
acute lesions. Chronic lesions show focal atrophy and
T1 hypointense signal.
• Optic nerve involvement in MS is quite common.
Patients with optic neuritis should have an MRI which
includes, short tau inversion recovery (STIR) and fat
suppressed fast spin echo T2 images of the optic nerve
up to the chiasm. On a routine spin-echo sequence
without fat suppression the high signal in the optic
nerve will be masked by the retrobulbar fat. There is
strong evidence that optic neuritis is often the first
evidence of MS, being the initial manifestation in
about 20 percent of cases and occurring during the
course of the disease in 50 percent of cases. It is also
estimated that 45 to 80 percent of patients with isolated
optic neuritis go on to develop MS at some point
during the next 15 years, although, most who do
develop MS do so within the first 5 years
• In patients with clinically diagnosed optic neuritis,
many clinicians feel that the role of MRI is two-fold:
(i) to exclude the rarely found alternative cause of the
clinical symptomatology aside from optic neuritis, and
(ii) to detect the presence of cerebral lesions
• Recent data from the Optic Neuritis Treatment Trial
at 2-year follow-up showed that steroid treatment of
acute optic neuritis reduced the risk of developing
MS from 36 to 16 percent only in those patients with
abnormal focal lesions on brain MRI at initial
presentation. Therefore, although it may not be
important to detect the optic nerve lesion in optic
neuritis, MRI appears to be important in prognosis
and in guiding therapy for patients with optic neuritis.
• Newer MRI techniques
• Fluid attenuation inversion recovery (FLAIR): This
is a heavily T2-weighted sequence that dampens
ventricular CSF signal, thereby increasing the contrast
between the lesions and the CSF and aiding detection.
664 Section 8 Demyelination

A B

C D

Figs 49.3A to D: Follow-up scan after one year: (A) Axial FLAIR, (B and D) Axial T2-weighted image and (C) Coronal T2-
weighted image showing partial resolution of the lesions with fresh lesions in the right juxtaventricular region, parietal periventricular
white matter and brainstem (arrows)—tumefactive MS

FLAIR sequence has replaced PD sequence especially
for supratentorial lesions, and now is an integral part
of brain imaging protocol
• Various criteria have evolved in predicting the
development of MS like the Fazeka’s criteria, that
requires the detection of 3 lesions with 2 having the
following characteristics:
1. Infratentorial location
2. Periventricular location
3. Lesion greater than 6 mm
• Other criteria, Tintore and Patty, CT scan has a very
limited role in the diagnosis and management of MS.
Contrast enhancement has been used to enhance the
visualization of lesions, but the sensitivity and
specificity is very poor compared to MRI
• Acute MS (Marburg type) may also present as areas
of clearly defined rings within or surrounding plaques
of demyelination. These rings have signal
characteristics on T1-weighted images consistent with
the presence of paramagnetic material, with slight
increase in signal intensity. Enhancement is typically
seen in the region of these rings. This appearance most
likely represents the presence of free radicals in the
macrophage layer forming the margin of an acute
plaque
• Plaques of Balo concentric sclerosis are striking in
their unique concentric rings of alternating destroyed
and intact myelin
• Treatment with steroids may also be associated with
a marked reduction in lesion enhancement and
morphology. High intensity lesions in MS do not
necessarily indicate demyelination, but rather might
merely reflect transient inflammation. Occasionally,
the plaque will contain a cavity, large enough to
present as a fluid-containing cyst
• With progression of disease, atrophy is apparent, and
increased iron deposition is concomitantly found in
the basal ganglia
• The corpus callosum is especially sensitive to
demyelination related to multiple sclerosis, possibly
due to its intimate neuroanatomic relationship to the
lateral ventricular roofs and its relationship to small
penetrating vessels
• Sagittal MR imaging has been advocated for the
depiction of the majority of the corpus callosal lesions.
In up to 93 percent of MS patients, focal lesions can
be identified in the inferior aspect of the corpus
callosum on sagittal views. Long TR images typically
show focal corpus callosal lesions to best advantage,
Chapter 49 Primary Demyelination 665
but the anatomic distortion with focal thinning in the
inferior aspect of the corpus callosum may be easily
appreciated on T1-weighted lesions in many cases.
The appearance of the corpus callosum involvement
on MRI may be specific for MS, however, ischemic
lesions also are noted to have a virtually identical
appearance
• Note that the anatomic distribution of the lesions
should not be considered key to the diagnosis, since
“exceptional” locations are in fact quite commonly
encountered.
Proton MR Spectroscopy
• Acute MS plaques show reduction in N-
acetylaspartate (NAA), elevation of Cho and presence
of lactate peak
• Decreased levels of N-acetylaspartate (NAA) is seen
in chronic plaques of MS.
Magnetization Transfer (MT) Techniques
• This technique, which can be implemented on a
conventional scanner, exploits differences in
relaxation between immobilized water transiently
bound to macromolecules and water protons not
associated with macromolecules
• The hypothesis underlying this investigation is that
demyelination results in more free water, i.e. a
reduction in the “bound” fraction of water, as
compared to myelinated white matter or intact but
edematous tissue. Selective suppression of
immobilized water is accomplished by the application
of an off-resonance saturation pulse, which saturates
the broad resonance of protons bound to
macromolecules. Transiently bound protons exchange
with free water protons by diffusion. Significant
reduction in MT ratio in MS plaques is noted
• This reduction in MT ratio corresponded to the
presence of abnormal proton MR spectroscopy
resonances that were consistent with active
demyelination.
Diffusion-weighted MR Imaging
• The basis of contrast in diffusion imaging is that
translational molecular motion of water causes a loss
of spin coherence and hence a loss of signal intensity,
when imaged with an appropriately sensitive
technique in the presence of strong magnetic fields
generated by the application of “diffusion gradients”.
666 Section 8 Demyelination

A B

C D

Figs 49.4A to D: (A and B) Axial FLAIR at the level of the lateral ventricles showing multiple tiny periventricular lesions, (C) Coronal
T2-weighted image of the orbit showing T2-hyperintense signal in the left intraorbital nerve (white arrows) and (D) Coronal T1
postcontrast image showing enhancement in the intraorbital optic nerve in a case of left optic neuritis

The unique organizational structure of mature
(myelinated) white matter has been postulated to
cause anisotropy of diffusion, i.e. a directional
restriction of water diffusion, as compared to gray
matter. While it is not clear whether the myelin itself
is the source of the anisotropy, it has been reported
that focal demyelinating lesions in the white matter
lose their anisotropy. Diffusion-weighted imaging
helps to differentiate acute from chronic MS plaques.
Extracerebral Lesions in MS
• Spinal cord plaques are also often demonstrated on
MRI, but MRI may not be as sensitive in the detection
of spinal cord lesions as it is for brain lesions. If a
patient is shown to have an intramedullary lesion that
is suspected to represent spinal cord MS, it is probably
most reasonable to perform a head MRI to screen for
asymptomatic multifocal disease in addition to
scanning the spinal cord
• Routine use of fast spin-echo techniques has become
a standard technique for the intramedullary lesions
of MS in the spinal cord. Gadolinium-contrast
administration will occasionally, demonstrate
enhancement of the acute spinal lesions of MS.
Enhancing MS plaques can be virtually indistingui-
shable from neoplastic lesions and other inflammatory
lesions of the spinal cord, particularly when the spinal
cord is enlarged due to edema. Therefore, clinical
correlation and often serial follow-up scanning is
necessary to formulate a specific diagnosis, especially
in those cases where MRI of the brain is normal.
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668 Section 8 Demyelination

A B

C D

Figs 49.5A to D: (A and B) Sagittal T2-weighted image and (C) Axial FLAIR images showing multiple periventricular and corpus
callosal lesions perpendicular to the body of the lateral ventricles (arrows) and (D) Coronal T2-weighted image showing hyperintense
signal in the right intraorbital optic nerve (white arrow)

18. Paty DW. Magnetic resonance in multiple sclerosis. Curr
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Chapter 49 Primary Demyelination 669
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L, Comi G, Filippi M. MRI quantification of gray and white
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F, Klotz L, Klockgether T, Schild HH. Imaging of
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670 Section 8 Demyelination

A B

Figs 49.6A and B: (A) Sagittal T1-weighted postcontrast MRI showing multiple central necrotic areas with peripheral arc like
enhancement and (B) Single voxel MR spectroscopy at TE = 35 ms the image on the top with voxel on the lesion showing
decrease in NAA and decrease in NAA/Cr ratio. The image at the bottom with voxel on the normal side shows normal spectrogram—
tumefactive MS

A B

Figs 49.7A to C: A 24-year-old female

presented with sudden onset loss of left eye
vision of few days duration: (A) Coronal T2
fat suppressed image of the orbit showing a
thickened left optic nerve with hyperintense
signal, (B) Axial T1 postcontrast with fat
suppression showing enhancement of the left
optic nerve and (C) Axial diffusion-weighted
image showing restricted diffusion in the left
C optic nerve
 Chapter 49 Primary Demyelination 671

Figs 49.8A and B: A 12-year-old female

presented with bilateral visual loss followed
by quadriparesis: (A) Coronal T2 image of
the orbit showing thin optic nerves with subtle
hyperintense signal in the right optic nerve
and (B) Sagittal T2-FSE of the cervical spine
showing hyperintense signal in the upper
cervical cord (arrows). The brain imaging was
A B normal—neuromyelitis optica
Chapter 50
Secondary Demyelination
• Secondary demyelination follows vascular insults,
infectious or toxic insults, trauma and tumors. They
damage neurons or axons, followed by breakdown of
myelin
• Many pathological processes may result in the
breakdown of both together
• The visual pathways, from the optic nerves to the
visual cortex contain myelinated nerve fibers and can
be damaged at any point
• Current classification, therefore, depends upon a
mixture of clinical and pathological features,
including genetics, biochemical and immunological
abnormalities. Some metabolic defects influence the
formation of myelin rather than damage to myelin
once it has been formed
• The toxic demyelination can be following radiation,
chemotherapy and anoxic encephalopathy, age-
related perivascular demyelination
• The infectious causes includes AIDS, progressive
multifocal leukoencephalopathy (PML), acute
disseminated encephalomyelitis (ADEM)
• The infections and radiation brain injury are discussed
in the previous chapters.
TOXIC DEMYELINATION
• Chemotherapeutic agents can cause mineralizing
microangiopathy which is commonly seen in radiation
injury, apart from acute reversible leukoencephalo-
pathy that manifests axonal swelling and edema
• Severe necrotizing leukoencephalopathy can also
occur when the white matter is diffusely damaged
• Common neurotoxic agents are methotrexate,
cisplatin, cytosine, cyclosporin A, asparaginase,
BCNU and arabinoside
• Central pontine myelinolysis alcohol and electrolyte
imbalance can cause
• Alcohol and thiamine deficiency induced toxic
changes are discussed separately later.
IMAGING
Magnetic resonance imaging (MRI) is the choice of imaging:
• In small vessel disease punctate, diffuse
periventricular and basal ganglia lesions which are
hypointense in short TR/TE images and hyperintense
in long TR/TE images are seen. CT reveals these
lesions as hypodense lesions
• Binswanger’s disease has characteristic central white
matter demyelination sparing the subcortical fibers
with multiple basal ganglia lacunar infarcts
• Small vessel disease and chemotoxicity can cause
symmetric bilateral periventricular changes.
Calcifications in and around the small cerebral vessels
is a common finding in toxic demyelination
• Radiation injury can cause focal periventricular white
matter lesions or severe generalized white matter
involvement which are hypointense on T1WI and
hyperintense on T2WI
• Central pontine myelinolysis is seen to have pontine
lesions which is also hypointense on T1 and hyperin-
tense on T2WI, but do not extend to the brainstem
• PML has a characteristic scalloped edge white matter
appearance which shows hyperintensity in long TR
images
• Lack of contrast enhancement can help us to rule out
an infection or a neoplasm
• PET has been successful to certain extent in differen-
tiating tumor recurrence and radiation necrosis
 Chapter 50 Secondary Demyelination 673

A B

Figs 50.1A to C: A 25-year-old HIV +ve man

presented with progressive vision loss: (A) Axial
T2-weighted, (B) Axial diffusion-weighted image
and (C) Coronal white matter lesion. The DWI
shows patchy areas of restricted diffusion in the
right parieto-occipital white (arrows)—progressive
C multifocal leukoencephalopathy
674 Section 8 Demyelination
• Toluene toxicity causes diffuse atrophy of cerebrum,
cerebellum and basal ganglion. MRI shows multiple
hypointense lesions in basal ganglion and thalamus
and hyperintense lesions in the periventricular region.
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management of acute disseminated encephalomyelitis.
J Neuro-ophthalmol 1994;14(4):210-3.
3. Magnano MD, Bush TM, Herrera I, Altman RD. Reversible
posterior leukoencephalopathy in patients with systemic
lupus erythematosus. Semin Arthritis Rheum 2006;
35(6):396-402.
4. Saykin AJ, Ahles TA, McDonald BC. Mechanisms of
chemotherapy-induced cognitive disorders: Neuro-
psychological, pathophysiological, and neuroimaging
perspectives. Semin Clin Neuropsychiatry 2003;8(4):
201-16.
5. Tarasów E, Wierciñska-Drapalo A, Walecki J,
Prokopowicz D. CNS changes in HIV-infected patients:
Magnetic resonance spectroscopy. Neurol Neurochir Pol
2003;37(Suppl 2):21-8.
 Chapter 50 Secondary Demyelination 675

A B

Figs 50.2A to C: A 73-year-old male patient presented with

mild cognitive impairment and imbalance: (A) Postcontrast axial
T1-weighted image, (B) Sagittal T1-weighted image and
(C) Coronal T1-weighted image showing extensive bilateral
enhancing lesions in the supra- and infratentorial white matter.
A follow-up study showed significant resolution of the lesion—
C postinfective/toxic demyelination
676 Section 8 Demyelination
COMMON CNS TOXICITIES
• CNS toxins could be endogenous or exogenous.
Alcohol is a common exogenous neurotoxin. Other
exogenous toxins include lead, mercury, solvent
exposure and chemotherapy
• Toxins cause temporary or permanent disturbance of
brain function in various ways. This can lead to cortical/
cerebellar degeneration and peripheral polyneuropathy
• The toxic effects occur due to depletion of oxidative
energy, nutritional deprivation, disturbances in
neurotransmission, altered ion balance, etc.
CHRONIC ALCOHOLISM
• Various specific processes related to ethanol
intoxication affect the CNS. These include Wernicke’s
encephalopathy, Marchiafava-Bignami disease, and
osmotic myelinolysis
• Ethanol adversely affects vascular, glial, and neural
tissues and causes myelin degeneration
• In chronic ethanol toxicity, patient may have cognitive
problems, recent or remote memory loss, poly-
neuropathy, gait abnormalities and nystagmus due
to cerebellar degeneration.
METHANOL POISONING
• It is a well-recognized optic nerve toxin. Patients
present with sudden profound visual loss with disc
swelling. They may be comatose with metabolic
acidosis. Patients are typically inebriated, and within
6 to 48 hours develop headache, dyspnea, vomiting,
abdominal pain and bilateral visual blurring
• The visual symptoms may be transient. Acute optic
disc edema eventually leads to optic atrophy and
cupping. Histotoxic anoxia in a water-shed area leads
to demyelination of retrobulbar optic nerve.
IMAGING
CT Scan
• Chronic alcoholism—disproportionate superior vermian
atrophy, generalized cerebral and cerebellar atrophy
• Methanol poisoning—bilateral hemorrhagic necrosis
of basal ganglia, especially putamen.
MRI
Chronic Alcoholism
• Chronic use of alcohol results in brain shrinkage. This
shrinkage is most marked in the frontal regions and
especially in older alcoholics
• Other brain regions, vulnerable to shrinkage are
portions of the limbic system and the cerebellum
• Nonspecific deep white matter and periventricular
demyelinating lesions are also seen in chronic
alcoholism. These changes are largely dose dependent.
MRI will show diffuse cerebral and cerebellar atrophy
• The bilateral periventricular demyelinating lesions are
hyperintense on T2-weighted images and FLAIR.
Methanol Poisoning
Bilateral hemorrhagic necrosis of basal ganglia, especially,
of putamen is noted. They display hypointense signal in
T1 and hyperintense signal in T2-weighted images.
BIBLIOGRAPHY
1. Antunez E, Estruch R, Cardenal C, Nicolas JM, Fernandez-
Sola J, Urbano-Marquez A. Usefulness of CT and
MR imaging in the diagnosis of acute Wernicke's encephal-
opathy. Am J Roentgenol 1998;171(4):1131-7.
2. Charness ME. Brain lesions in alcoholics. Alcohol Clin Exp
Res 1993;17(1):2-11. Review.
3. Hund E. Neurologic sequelae of chronic alcoholism.
Anaesthesiol Reanim 2003;28(1):4-7.
4. O'Neill J, Cardenas VA, Meyerhoff DJ. Effects of abstinence
on the brain: Quantitative magnetic resonance imaging
and magnetic resonance spectroscopic imaging in chronic
alcohol abuse. Alcohol Clin Exp Res 2001;25(11):1673-82.
5. Spampinato MV, Castillo M, Rojas R, Palacios E, Frascheri
L, Descartes F. Magnetic resonance imaging findings in
substance abuse: Alcohol and alcoholism and syndromes
associated with alcohol abuse. Top Magn Reson Imaging
2005;16(3):223-30. Review.
6. Vargas MI, Lenz V, Bin JF, Bogorin A, Abu Eid M, Jacques
C, Marin H, Kindo S, Zollner G, Dietemann JL. Brain MR
imaging of chronic alcoholism. J Radiol 2003;84(4 Pt 1):369-
79. Review.
7. Viola A, Nicoli F, Confort-Gouny S, Le Fur Y, Ranjeva JP,
Cozzone PJ. Applications of magnetic resonance
spectrometry (MRS) in the study of metabolic disturbances
affecting the brain in alcoholism. Pathol Biol (Paris)
2001;49(9):718-25. Review.
 Chapter 50 Secondary Demyelination 677

A B

C D

Figs 50.3A to D: A 36-year-old male with sudden loss of vision following alcohol intake: (A) Axial T1WI, (B) Coronal T2WI, (C) Axial
FLAIR and (D) Axial T2-weighted image showing bilateral putaminal necrosis
678 Section 8 Demyelination
WERNICKE’S ENCEPHALOPATHY
This disorder results from a vitamin B 1 (thiamine)
deficiency. It is characterized by a triad of abnormal
occular motility, gait ataxia and encephalopathy. The
complete triad is seen only in minority of patients.
CLINICAL FEATURES
• Conjugate gaze palsies are seen, usually horizontal
and rarely vertical (especially upward). This is due to
lesions affecting sixth nerve nuclei, pretectal area and
periaqueductal gray matter
• Other neuro-ophthalmic features include nystagmus
due to damage to cerebellar and vestibular structures,
lateral rectus palsy, pupillary abnormalities and rarely
retinal hemorrhages, ptosis and optic neuropathy
• Patients can develop Korsakoff’s psychosis in the
long-run characterized by antegrade and retrograde
amnesia and confabulation.
Causes
• Chronic alcoholism
• Malnourished individuals (prisoners of war)
• Women with hyperemesis gravidarum
• Forced or involuntary starvation, cancer, gastric
plication, chronic renal dialysis
• In thiamine, deficient individuals who receive a
carbohydrate load (thiamine is required for carbo-
hydrate metabolism).
NEUROPATHOLOGIC FINDINGS
Necrosis of nerves and myelin, hypertrophy and
hyperplasia of small blood vessels, pin-point
hemorrhages may occur. These are located symmetrically
in mamillary bodies, superior cerebellar vermis,
hypothalamus, thalamus, midbrain, oculomotor and
vestibular nuclei.
IMAGING
The topographic distribution is characteristic and
involves in the both gray and white matter. The
periventricular regions, the medial thalamic nuclei, massa
intermedia, third ventricular floor and mamillary bodies
are involved. The periaqueductal region, midbrain
reticular formation, and tectal plate are also involved.
MRI
In acute stage, hyperintense areas are seen that surround
the third ventricle and aqueduct. Postcontrast T1-weighted
images may show enhancement around the third ventricle,
aqueduct, and in the mamillary bodies. After vitamin
therapy, resolution of these abnormalities has been noted
in some studies.
Mamillary body and midline cerebellar atrophy with
third ventricular enlargement are seen in chronic stage.
BIBLIOGRAPHY
1. Antunez E, Estruch R, Cardenal C, Nicolas JM, Fernandez-
Sola J, Urbano-Marquez A. Usefulness of CT and MR imaging
in the diagnosis of acute Wernicke's encephalopathy. Am J
Roentgenol 1998;171(4):1131-7.
2. Jover F, Jover R, Martinez A, Caballero O. Use of brain
SPECT imaging in the diagnosis of Wernicke-Korsakoff's
encephalopathy. Rev Neurol 2004;39(6):597-8.
3. Lee ST, Jung YM, Na DL, Park SH, Kim M. Corpus
callosum atrophy in Wernicke's encephalopathy.
J Neuroimaging 2005;15(4):367-72.
4. Liu YT, Fuh JL, Lirng JF, Li AF, Ho DM, Wang SJ.
Correlation of magnetic resonance images with neuro-
pathology in acute Wernicke's encephalopathy. Clin
Neurol Neurosurg 2006;108:682-7.
5. Loh Y, Watson WD, Verma A, Krapiva P. Restricted
diffusion of the splenium in acute Wernicke's
encephalopathy. J Neuroimaging 2005;15(4):373-5.
6. Morcos Z, Kerns SC, Shapiro BE. Wernicke
encephalopathy. Arch Neurol 2004;61(5):775-6.
7. Nolli M, Barbieri A, Pinna C, Pasetto A, Nicosia F.
Wernicke's encephalopathy in a malnourished surgical
patient: Clinical features and magnetic resonance imaging.
Acta Anaesthesiol Scand 2005;49(10):1566-70.
8. Spampinato MV, Castillo M, Rojas R, Palacios E, Frascheri
L, Descartes F. Magnetic resonance imaging findings in
substance abuse: Alcohol and alcoholism and syndromes
associated with alcohol abuse. Top Magn Reson Imaging
2005;16(3):223-30. Review.
9. Unlu E, Cakir B, Asil T. MRI findings of Wernicke
encephalopathy revisited due to hunger strike. Eur J
Radiol Epub 2006;57(1):43-53.
10. White ML, Zhang Y, Andrew LG, Hadley WL. MR
imaging with diffusion-weighted imaging in acute and
chronic Wernicke encephalopathy. Am J Neuroradiol 2005;
26(9):2306-10.
11. Zhong C, Jin L, Fei G. MR imaging of nonalcoholic Wernicke
encephalopathy: A follow-up study. Am J Neuroradiol
2005;26(9):2301-5.
 Chapter 50 Secondary Demyelination 679

A B

Figs 50.4A to C: A 42-year-old male a known alcoholic presented

with ophthalmoplegia and mental confusion following a heavy
alcohol intake: (A and B) Axial FLAIR images at midbrain and
thalamic level showing patchy T2-hyperintense signal in the
midbrain, periaqueductal gray matter, medial temporal lobes and
thalami and (C) Coronal T2-weighted image showing the same
features as above. Note: The lesions are better appreciated on
FLAIR imaging than T2 FSE sequence due to CSF suppression—
C Wernicke’s encephalopathy
SECTION 9
Phacomatosis
SECTION OUTLINE
51. Neurofibromatosis
52. Sturge-Weber Syndrome
53. Tuberous Sclerosis
54. Von Hippel-Lindau Disease
55. Wyburn-Mason Syndrome
Chapter 51

Neurofibromatosis
It is classified as neurofibromatosis type I (NF I) and type • Non-neoplastic hamartomatous lesions in the basal
II neurofibromatosis (NF II). ganglia and white matter
• Neurofibrosarcomas
NEUROFIBROMATOSIS I • Osseous lesion—sphenoid wing dysplasia and sutural
defects
• NF I is also known as classical von Recklinghausen’s • Ocular—buphthalmos, retinal phakomas
disease. It is more common than NF II. This is a true • Vascular—progressive cerebral arterial occlusions,
neurofibromatosis and has prominent clinically aneurysm, vascular ectasia, arteriovenous malfor-
evident cutaneous lesions namely neurofibromas and mation
café-au-lait spots that help in the clinical diagnosis • Macrocephaly.
• It is an autosomal dominant disorder characterized
by the development of peripheral nerve sheath tumors Cutaneous
(neurofibromas) and significant CNS abnormalities • Café–au-lait spots
such as true neoplasms (optic nerve glioma) and • Axillary and intertriginous freckling
dysplastic and hamartomatous/heterotopic lesions • Cutaneous neurofibroma
• There is no racial or sexual predilection. The gene • Plexiform neurofibroma
responsible for this is on the long arm of chromo- • Elephantiasis neuromatosa.
some 7
• Imaging: MRI is the imaging modality of choice and Skeletal
will help in visualizing the various CNS lesions that • Kyphoscoliosis
may not be appreciated on CT. • Lateral thoracic meningocele
• Pseudoarthrosis
Clinical Features • Ribbon ribs
• Focal gigantism
Ocular/Orbit/CNS • Spinal root neurofibroma
• Dysplastic enlargement of spiral foramina and optic
• Lisch nodules (iris hamartomas) and auditory canals.
• Eyelid plexiform neurofibroma
• Optic nerve gliomas and chiasmatic gliomas Vascular/Visceral/Endocrine
• Plexiform neurofibromas (involving the intraorbital • Pheochromocytoma
or branches of cranial nerves III–VI, CN VI most • Parathyroid adenoma
common) • Renal artery stenosis
• Nonoptic gliomas (usually low-grade astrocytomas) • Medullary carcinoma of thyroid.
684 Section 9 Phacomatosis

A B

Figs 51.1A and B: (A) Axial CT scan of the orbit showing a buphthalmic left globe and (B) Axial CT scan in bone window settings
showing dysplasia of the (L) sphenoid wing (arrow)—type I neurofibromatosis

A B

Figs 51.2A and B: (A) Axial T2-weighted image showing heterogeneous mass in the left orbit causing proptosis and (B) Posterior
extension into the chiasm and optic tracts (dotted arrows). Dilatation of the temporal horns is seen (black arrow). T2-hyperintense
signal noted in the left cerebral peduncle and bilateral cerebellar hemisphere (arrowheads)

Imaging
• MRI is the imaging modality of choice. The following
lesions may be seen:
– Hamartomatous and neoplastic lesions (optic
nerve and parenchymal gliomas) and multifocal
signals on T2-weighted MRI representing
abnormal myelination or hamartomas. They are
seen in the brainstem, cerebellar white matter,
dentate nucleus, basal ganglia, periventricular
white matter, optic nerve and optic pathways. In
such lesions, absence of mass effect, edema,
hemorrhage and absence of enhancement with
contrast suggest that they are hamartomas
– Optic nerve gliomas especially those seen in
childhoods are diagnostic of NF I. These are
invariably juvenile pilocytic astrocytomas. Seventy
percent of patients with optic nerve gliomas have
NF I. The presence of bilateral optic nerve gliomas
is considered specific for NF I.
NEUROFIBROMATOSIS II
• This is also an autosomal dominant disorder with no
known racial or sexual predilection
• Multiple cranial nerve schwannomas are the
hallmark. Peripheral and cutaneous neuromas are not
common. Vestibulocochlear nerve schwannomas are
most common. Five percent schwannomas occur in
NF II.
CNS Lesions
• Schwannomas and meningiomas—bilateral acoustic
schwannomas are diagnostic of NF II. They present
in the 2 to 3rd decade. The schwannomas of other
cranial nerves may also be seen. The meningiomas
may be multiple and carry worse prognosis
• Intramedullary spinal cord schwannomas
• Non-neoplastic intracranial calcifications (especially
choroid plexus)
• Cord ependymomas and multilevel schwannomas
• Juvenile subcapsular lens opacities
• Specific type of cutaneous tumor resembling skin tags.
Clinical Features
• Most patients present with hearing loss, followed by
balance problems and seizures
• Vestibular schwannomas in NF II are likely to invade
and even arise from cochlear and facial nerve. So,
preserving hearing and facial nerve function is difficult.
Chapter 51 Neurofibromatosis 685
Imaging
MRI is the imaging modality of choice. The schwannomas
and meningiomas are better delineated with contrast
enhanced MR.
Acoustic Schwannoma
• They invariably arise within the internal acoustic canal
(IAC) or its orifice. Deformity or enlargement of canal
is noted in 70 percent cases on CT scan. They are
hypodense to skeletal muscle and hypo- to isodense
to brain
• They are very vascular and appear heterogeneous due
to cystic degeneration and hemorrhage. With contrast,
they show homogeneous enhancement when small
and heterogeneous enhancement when large.
MRI
• Features of peripheral nerve tumors are nonspecific.
They are smooth, well-demarcated masses that appear
to be mildly heterogeneous in proton density T2-
weighted images. They enhance well with contrast.
In the absence of enlargement on CT, MRI is preferred
as it will show the intracanalicular component.
Meningiomas
On CT, they appear as dural based, homogeneous and
hyperdense to brain. They enhance homogeneously with
contrast. Calcification is better seen on CT.
MRI
They are isointense to gray matter on all pulse sequences.
There may be secondary displacement of adjacent brain
or associated calvarial changes such as hyperostosis or
erosion. They enhance brilliantly with contrast.
BIBLIOGRAPHY
1. Chateil JF, Soussotte C, Pedespan JM, Brun M, Le Manh
C, Diard F. MRI and clinical differences between optic
pathway tumours in children with and without
neurofibromatosis. Br J Radiol 2001;74(877):24-31.
2. Egelhoff JC, Bates DJ, Ross JS, Rothner AD, Cohen BH.
Spinal MR findings in neurofibromatosis types 1 and 2.
Am J Neuroradiol 1992;13(4):1071-7.
3. Evans DG, Huson SM, Donnai D, Neary W, Blair V,
Newton V, Harris R. A clinical study of type 2 neurofibro-
matosis. QJ Med 1992;84(304):603-18.
4. Flexon PB, Nadol JB Jr, Schuknecht HF, Montgomery WW,
Martuza RL. Bilateral acoustic neurofibromatosis
686 Section 9 Phacomatosis

A B C

Figs 51.3A to C: (A) Axial CT scan and (B) Coronal CT scan showing dysplasia of right greater wing of sphenoid bone with
herniation of the temporal lobe through the defect (arrow) and (C) Ill-defined soft tissue is noted in the right pre- and postseptal
region—plexiform neurofibromatosis

A B

Figs 51.4A and B: Type II neurofibromatosis: (A) Axial CT scan of the brain at the level of the optic nerve showing diffuse
thickening of the right optic nerve sheath complex with tram-track calcification—meningioma. Lobulated mass seen in the left
cavernous sinus extending through the Meckle’s cave into the cerebellopontine angle cistern (dotted arrow)—V nerve schwannoma.
Also noted as bilateral acoustic schwannomas (black arrows) and (B) Coronal section of the orbit showing thickened right optic
nerve sheath complex with perioptic calcification

(neurofibromatosis 2): A disorder distinct from von
Recklinghausen's neurofibromatosis (neurofibro-
matosis 1). Ann Otol Rhinol Laryngol 1991;100(10):830-4.
Review.
5. Khong PL, Goh WH, Wong VC, Fung CW, Ooi GC. MR
imaging of spinal tumors in children with neurofibro-
matosis 1. Am J Roentgenol 2003;180(2):413-7.
6. Korf BR. Diagnosis and management of neurofibromatosis
type 1. Curr Neurol Neurosci Rep 2001;1(2):162-7. Review.
7. Korf BR. Neurocutaneous syndromes: Neurofibromatosis
1, neurofibromatosis 2, and tuberous sclerosis. Curr Opin
Neurol 1997;10(2):131-6. Review.
8. Korf BR. Ophthalmological issues in the neurofibromatosis.
J Pediatr Ophthalmol Strabismus 1996;33(4):255-9. Review.
9. Mautner VF, Hartmann M, Kluwe L, Friedrich RE,
Funsterer C. MRI growth patterns of plexiform neuro-
fibromas in patients with neurofibromatosis type 1.
Neuroradiology 2006;1-6 [Epub ahead of print].
10. Menor F, Marti-Bonmati L, Arana E, Poyatos C, Cortina H.
Neurofibromatosis type 1 in children: MR imaging and
follow-up studies of central nervous system findings. Eur
J Radiol 1998;26(2):121-31.
11. Mentzel HJ, Seidel J, Fitzek C, Eichhorn A, Vogt S,
Reichenbach JR, Zintl F, Kaiser WA. Pediatric brain MRI
Chapter 51 Neurofibromatosis 687
in neurofibromatosis type I. Eur Radiol 2005;15(4):814-22.
Epub 2004 Jul 29. Review.
12. Patronas NJ, Courcoutsakis N, Bromley CM, Katzman GL,
MacCollin M, Parry DM. Intramedullary and spinal canal
tumors in patients with neurofibromatosis 2: MR imaging
findings and correlation with genotype. Radiology 2001;
218(2):434-42.
13. Sigorini M, Zuccoli G, Ferrozzi F, Bacchini E, Street ME,
Piazza P, Rossi M,Virdis R. Magnetic resonance findings
and ophthalmologic abnormalities are correlated in
patients with neurofibromatosis type 1 (NF1). Am J Med
Genet 2000;93(4):269-72.
14. Sperfeld AD, Hein C, Schroder JM, Ludolph AC,
Hanemann CO. Occurrence and characterization of peri-
pheral nerve involvement in neurofibromatosis type 2.
Brain 2002;125(Pt 5):996-1004.
15. Sylvester CL, Drohan LA, Sergott RC. Optic-nerve
gliomas, chiasmal gliomas and neurofibromatosis type 1.
Curr Opin Ophthalmol 2006;17(1):7-11.
16. Zacharia TT, Jaramillo D, Poussaint TY, Korf B. MR
imaging of abdominopelvic involvement in neurofibro-
matosis type 1: A review of 43 patients. Pediatr Radiol
2005;35(3):317-22. Epub 2004 Oct 27. Review.
688 Section 9 Phacomatosis

A B

C D

Figs 51.5A to D: (A) Axial CT scan of the brain showing bilateral acoustic schwannomas (black arrows) with left trigeminal
schwannoma (dotted arrow), (B) Coronal bone window shows enlargement of the left foramen rotundum (white arrow) indicating
extension into the V2 division of the V nerve, (C) Axial and (D) Coronal bone window showing widening of the internal auditory
canals bilaterally (dotted arrows)
 Chapter 51 Neurofibromatosis 689

A B

Figs 51.6A to C: (A) Axial T2-weighted images at the level of the optic nerves showing a well-defined
right intraconal mass lesion—optic nerve glioma, (B) At the level of the internal auditory canals showing
bilateral acoustic schwannomas and (C) 2D myelogram image showing multiple intradural filling defects
C suggestive of intradural neurofibromas—a case of type II neurofibromatosis
Chapter 52
Sturge-Weber Syndrome
• The Sturge-Weber syndrome (SWS), also called
encephalotrigeminal angiomatosis, is a neuro-
cutaneous disorder with angiomas involving the
leptomeninges (leptomeningeal angiomas) and skin
of the face, typically in the ophthalmic (V1) and
maxillary (V2) distributions of the trigeminal nerve
• The cutaneous angioma is called a port-wine stain
(PWS). SWS is caused by residual embryonal blood
vessels and their secondary effects on surrounding
brain tissue
• Both sexes are affected equally
• The typical patient presents at birth with facial
angiomas; however, not all children with facial
angiomas and PWS have Sturge-Weber syndrome,
which raises certain diagnostic and prognostic
concerns
• In the “incomplete” forms of SWS, CNS angiomas
occur without cutaneous features. These are
congenital macular lesions that can be progressive;
they may be a light pink color initially and then
progress to a dark red or purple nodular lesion. These
may be isolated to the skin, associated with lesions in
the choroidal vessels of the eye or the leptomeningeal
vessels of the brain, or even located on other body
areas
• A PWS may be difficult to visualize in a patient with
dark skin pigmentation, and therefore, no suspicion
of SWS arises until a seizure or other neurologic
problem develops. Thus, the diagnosis of SWS is not
always straightforward
• SWS occurred only when the port-wine stain involved
the V1 distribution of the trigeminal nerve. No
patients with involvement of the V2 and/or mandi-
bular (V3) area without V1 involvement had SWS.
Patients considered “high-risk” were those with
involvement of the entire V1 area; patients with only
partial involvement of V1 were at low-risk
• The incidence of epilepsy in patients with SWS is 75-90
percent; seizures may be intractable. Seizures result
from cortical irritability caused by cerebral angioma,
through mechanisms of hypoxia, ischemia, and gliosis
• Dual pathology, such as microgyria, also may be
present, which also contributes to epileptogenesis
• Hemiparesis, stroke-like episodes, hemianopia
• Headaches: These occur secondary to vascular disease,
giving symptoms of a migraine headache, considered
“symptomatic migraine”
• Glaucoma and blindness: Glaucoma typically occurs in
SWS only when the PWS involves the eyelids. The
incidence ranges from 30 to 71 percent. Glaucoma may
be present at birth but can develop at any age, even
in adults. Treatment includes yearly examinations,
looking for optic nerve damage (with measurement
of IOP and visual fields) and corneal diameter and
refractive changes in children
• Glaucoma usually occurs only with an ipsilateral facial
PWS, although it may be bilateral when facial
involvement is bilateral. Contralateral glaucoma may
develop, although rarely. Glaucoma also may occur
without neurologic involvement.
SIGNS
• Macrocephaly
• Eye: Buphthalmos, heterochromia of iris, tomato-
catsup color of the fundus (ipsilateral to the nevus
flammeus) with glaucoma, possibility of choroidal
angioma visible with an ophthalmoscope
• Soft-tissue hypertrophy, neurologic signs, develop-
mental delay/mental retardation, learning problems,
 Chapter 52 Sturge-Weber Syndrome 691

Fig. 52.1: External photo face of a 20-year-old gentleman with

Sturge-Weber syndrome showing a unilateral facial angioma
involving the right half of the face. Port-wine stain follows the
trigeminal nerve distribution
692 Section 9 Phacomatosis

A B

C D

Figs 52.2A to D: A known case of Sturge-Weber syndrome: (A and B) Axial CT scan of the orbits, at different levels showing
angiomatous soft tissue in the left eyelid and thickening of the conjunctiva and ocular coats and (C and D) Axial CT scan of the
brain showing dense, gyral calcification in the left frontal, temporal and parieto-occipital region. Cortical atrophy, pneumosinus
dilatans with ipsilateral enlargement of the choroid plexus noted (arrow)

attention deficit hyperactivity disorder, hemiparesis,
visual loss and hemianopia.
Imaging Studies
• Neuroimaging studies: Besides the clinical
examination, neuroimaging studies include skull
radiograph, angiography, CT scan, MRI, MRI with
gadolinium, and functional imaging with SPECT or
positron emission tomography (PET)
• Skull radiograph: The skull X-ray may show the
classical “tram-line,” or “tram-track” or “trolley-track,”
calcifications considered pathognomonic for SWS. With
the advent of CT and MRI, X-rays are no longer done
for visualization of intracranial calcifications. These are
often a late finding and may not be present initially
• Angiography: Angiography does not show the
angioma but demonstrates a lack of superficial cortical
veins, non-filling of dural sinuses, and abnormal,
tortuous veins that course toward the vein of Galen
• Computed tomography: CT scan may show
calcifications in infants and even neonates. The
calcifications in typical cases will follow a gyral
pattern. Other findings include brain atrophy,
ipsilateral choroid plexus enlargement, abnormal
draining veins, and a breakdown of the blood-brain
barrier with seizures
• Magnetic resonance imaging: Although MRI does
not show calcifications, gadolinium enhancement may
show pial angioma; therefore, MRI may permit early
diagnosis of SWS, even in the newborn with a facial
PWS. Sugama et al reported that the most
characteristic finding of SWS on MRI is enhancement
of leptomeningeal angiomas on postgadolinium,
which may show a leptomeningeal angioma (LA), not
seen on CT scan or angiography, however
• Other MRI findings include accelerated myelination
around the LA, a large choroid plexus whose size
correlates with the extent of the LA, and progressive
sinovenous occlusion on MR venography
• Single-photon emission computed tomography: This
measures cerebral blood flow, demonstrates
underperfusion in the area of the pial angioma, and
Chapter 52 Sturge-Weber Syndrome 693
therefore, may detect a latent angioma. With SPECT,
demonstrated hypoperfusion before calcifications,
anomalous drainage, or enhancement developed on
either CT scan or MRI.
BIBLIOGRAPHY
1. Benedikt RA, Brown DC, Walker R, Ghaed VN, Mitchell
M, Geyer CA. Sturge-Weber syndrome: Cranial MR
imaging with Gd-DTPA. Am J Neuroradiol 1993;14(2):
409-15.
2. Elster AD, Chen MY. MR imaging of Sturge-Weber
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685-9.
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radiological CT and MRI findings. Childs Nerv Syst 1993;
9(2):107-9.
5. Marti-Bonmati L, Menor F, Poyatos C, Cortina H. Diagnosis
of Sturge-Weber syndrome: Comparison of the efficacy of
CT and MR imaging in 14 cases. Am J Roentgenol 1992;
158(4):867-71.
6. Sperner J, Schmauser I, Bittner R, Henkes H, Bassir C,
Sprung C, Scheffner D, Felix R. MR-imaging findings in
children with Sturge-Weber syndrome. Neuropediatrics
1990;21(3):146-52.
7. Stimac GK, Solomon MA, Newton TH. CT and MR of
angiomatous malformations of the choroid plexus in
patients with Sturge-Weber disease. Am J Neuroradiol
1986;7(4):623-7.
8. Tournut P, Turjman F, Guibal AL, Revol M, Gilly R, Lapras
C, Froment JC. MRI in Sturge-Weber syndrome. J Neuro-
radiol 1992;19(4):285-92.
9. Truhan AP, Filipek PA. Magnetic resonance imaging: Its
role in the neuroradiologic evaluation of neurofibro-
matosis, tuberous sclerosis, and Sturge-Weber syndrome.
Arch Dermatol 1993;129(2):219-26. Review.
10. Wasenko JJ, Rosenbloom SA, Duchesneau PM, Lanzieri CF,
Weinstein MA. The Sturge-Weber syndrome: Comparison
of MR and CT characteristics. Am J Neuroradiol 1990;
11(1):131-4.
Chapter 53
• Tuberous sclerosis complex (TSC) is the second most
common neurocutaneous disease. It is inherited in an
autosomal dominant pattern, although the rate of
spontaneous mutation is high
• Formerly, characterized by the clinical triad of mental
retardation, epilepsy, and facial angiofibromas, it is
now recognized these patients may present with a
broad range of clinical symptoms due to variable
expressivity
• It may affect many organs, most commonly the brain,
skin, eyes, heart, kidneys, and lungs. Common features
include cortical tubers, subependymal nodules (SENs),
subependymal giant cell astrocytomas (SEGAs), facial
angiofibromas, hypomelanotic lesions (ash-leaf spots)
cardiac rhabdomyomas, and renal angiomyolipomas.
• Many of the clinical symptoms are due to the
development and growth of hamartomas. Overall, the
most common cause of death in these patients status
epilepticus or bronchopneumonia. The next most
frequent cause of death is renal failure. TSC occurs
with equal frequency in all races
• No sex predilection exists in this autosomal dominant
disease. TSC in females tends to have higher
morbidity and mortality rates because the incidence
of lung involvement is higher in females than in males
• It is a congenital disorder, although age at diagnosis
may range from birth to adulthood.
SIGNS AND SYMPTOMS
• Central nervous system symptoms are seizures,
including infantile spasms, and mental retardation.
Other symptoms include autism, aggressive behavior,
schizophrenia, and sleep disturbances. Sleep
disturbances may be displayed by decreased overall
sleep time and frequent nocturnal awakenings
Tuberous Sclerosis
• Dermatologic symptoms: Hypopigmented lesions
(i.e. ash-leaf spots), typically are the first dermatologic
manifestations of TSC. Other lesions that may be noted
are confetti lesions, facial angiofibromas, shagreen
patches, periungual or ungual fibromas, and cafe-au-
lait spots
• Renal symptoms: Flank pain is the most common renal
symptom. Other symptoms include hematuria,
hypertension, and rarely, hemorrhagic shock or renal
failure, which may develop in severe cases. Renal
symptoms are rare during childhood and are not
present in all patients with renal disease
• Cardiac rhabdomyomas may cause heart failure or
arrhythmia, even in the presence of normal echo-
cardiogram findings. Patients with rhabdomyomas
usually are asymptomatic. Rhabdomyomas often
regress with time, although the patient remains at risk
for arrhythmia
• Pulmonary manifestations occur predominantly in
females, although fewer than one percent of females
with TSC have pulmonary pathology. When present,
symptoms include dyspnea, hemoptysis, and
development of spontaneous pneumothorax.
Major and minor diagnostic features of TSC include the
following:
• Major features include cortical tubers, subependymal
nodules, Subependymal astrocytoma, 3 or more ash-
leaf spots, facial angiofibromas or forehead plaques,
shagreen patches, ungual or periungual fibromas in
the absence of trauma, cardiac rhabdomyomas, LAM,
renal angiomyolipomas, or retinal hamartomas
• Minor features include dental pits, gingival fibromas,
confetti skin lesions, bone cysts, hamartomatous rectal
polyps, multiple renal cysts, other nonrenal hamar-
tomas, achromic lesions of the retina, and radial
migration lines of cerebral white matter.
 Chapter 53 Tuberous Sclerosis 695

A B

Figs 53.1A and B: (A) Axial postcontrast CT scan showing a large enhancing lesion at the foramen of Monro (arrow); Subependymal
giant cell astrocytoma and (B) Contrast axial CT at a higher level showing multiple calcified subependymal nodules with cortical
hypodensities (arrow) showing of cortical tubers
696 Section 9 Phacomatosis
IMAGING
• An MRI of the brain is recommended for the detection
and follow-up imaging of cortical tubers,
subependymal nodules and subependymal
astrocytoma, MRI is obtained during the initial
diagnostic work-up and then every 1 to 3 years in
children with TSC. MRI may be performed less
frequently in adults without lesions and as clinically
indicated in adults with lesions. In addition, an MRI
should be done in family members, if results of
physical examinations are negative or are not
definitive for a diagnosis. MRI is preferred over CT
scan due to improved depiction of lesions and the lack
of radiation exposure on repeat examinations
• Cortical tubers, best detected on T2-weighted MRI
sequences, often occur in the gray-white junction. On
T2-weighted images, cortical tubers demonstrate
increased signal intensity and often are wedge-
shaped (tuber) or linear-shaped (radial migration
lines). Conversely, cortical tubers demonstrate
decreased signal intensity on T1-weighted images.
Previously believed to be pathognomonic, cortical
tubers are no longer considered specific for TSC, since
isolated cortical dysplasia may demonstrate similar
radiologic features. A correlation appears to exist
between the number of tubers detected using MRI and
the severity of mental retardation or seizures
• Subependymal nodules are located along the
ventricles and often become calcified. The lesions are
detected best using CT scans, although they
sometimes are noted on MRI or plain radiographs if
calcified. They demonstrate a candle-dripping
appearance
• Subependymal nodules may grow and give rise to
subependymal astrocytomas. It may cause obstruction,
with evidence of hydrocephalus or mass effect in some
patients. The lesions usually appear in the region of
the foramen of Monro, are partially calcified, and often
are larger than 2 cm. MRI is more sensitive in their
detection
• Renal ultrasound usually is preferred over CT scans
and MRI due to availability and cost. Ultrasound is
more sensitive in detection of renal lesions than CT
scans. Ultrasound should be done as the initial
diagnosis or evaluation and it should also include
screening of family members of patients with TSC.
Obtain subsequent surveillance studies in children or
adults with TSC every 1 to 3 years. In those with renal
lesions, follow-up should be done every 6 to 12
months until no further growth occurs or lesions begin
to regress
• Ultrasound, CT, or MRI studies may reveal evidence
of benign or malignant angiomyolipomas, renal cysts,
or, rarely, renal cell carcinoma. Benign angiomyo-
lipomas are found in 50 to 80 percent of patients with
TSC. The lesions usually are bilateral. Angiomyo-
lipomas occur more often in adults with TSC, while
renal cysts occur more commonly in children
• Echocardiography:
– Obtain an echocardiogram at initial evaluation and
in adults with TSC as indicated clinically. In
children with previously detected lesions, obtain
an echocardiogram every 6 to 12 months until
lesions cease growing or begin to regress
– Cardiac rhabdomyomas occur in 50 to 70 percent
of patients with TSC. Tumors almost always
regress as the child ages. Occasionally, lesions are
not detected using echocardiography, although
they may still cause arrhythmia
• Pulmonary CT or plain radiography:
– Obtain a CT scan of the lung in individuals with
TSC as clinically indicated. Obtain pulmonary CT
scans in women with TSC beginning at age 18
years, even in the absence of symptoms.
Pulmonary pathology is almost nonexistent in
males. The average age of onset of pulmonary
symptoms is in the early 30s
– Pulmonary lesions that may be detected on CT
scans include lymphangiomyomatosis, clear cell
tumors, and multifocal multinodular pneumocyte
hyperplasia
– Plain radiographs of the chest may reveal a
honey-comb appearance that is due to the presence
of multiple subpleural cysts.
BIBLIOGRAPHY
1. Adamsbaum C, Merzoug V, Kalifa G. Imaging of CNS
manifestations of tuberous sclerosis in children.
J Neuroradiol 2005;32(3):204-9. Review.
2. Altman NR, Purser RK, Post MJ.Tuberous sclerosis:
Characteristics at CT and MR imaging. Radiology 1988;
167(2):527-32.
3. Baron Y, Barkovich AJ. MR imaging of tuberous sclerosis
in neonates and young infants. Am J Neuroradiol 1999;
20(5):907-16.
4. Braffman BH, Bilaniuk LT, Naidich TP, Altman NR, Post
MJ, Quencer RM, Zimmerman RA, Brody BA. MR imaging
of tuberous sclerosis: Pathogenesis of this phakomatosis,
use of gadopentetate dimeglumine, and literature review.
Radiology 1992;183(1):227-38. Review.
5. Ech-Cherif El, Kettani N, Salaheddine T, El Quessar A, El
Kharras A, El Hassani M, Chakir N, Boukhrissi N,
 Chapter 53 Tuberous Sclerosis 697

A B

C D

Figs 53.2A to D: A 20-year-old male presented with seizures and mental retardation. Axial CT scans at different
levels (A and B) Precontrast; (C) Postcontrast images showing a large lobulated enhancing calcified lesion at
the foramen of Monro—(dotted arrow) with small subependymal calcified nodules (black arrow); (D) At a higher
level shows subcortical hypodensities suggestive of cortical tubers (white arrow)
698 Section 9 Phacomatosis
Benameur M, Jiddane M. Neuro-imaging of tuberous
sclerosis. J Radiol 2006;87(2 Pt 1):109-13.
6. Ergun R, Okten AI, Yaman M, Gezici AR, Taskin Y.
Subependymal giant-cell astrocytoma associated with
tuberous sclerosis: Case report. Neurosurg Rev 1998;21
(2-3):185-8.
7. Gerard G, Weisberg L. Tuberous sclerosis: CT findings and
differential diagnosis. Comput Radiol 1987;11(4):189-92.
8. Griffiths PD, Bolton P, Verity C. White matter abnorma-
lities in tuberous sclerosis complex. Acta Radiol 1998;
39(5):482-6.
9. Inoue Y, Nemoto Y, Murata R, Tashiro T, Shakudo M,
Kohno K, Matsuoka O, Mochizuki K. CT and MR imaging
of cerebral tuberous sclerosis. Brain Dev 1998;20(4):209-
21. Review.
10. Iwasaki S, Nakagawa H, Kichikawa K, Fukusumi A,
Watabe Y, Kitamura K, Otsuji H, Ohishi H, Uchida H.
MR and CT of tuberous sclerosis: Linear abnormalities in
the cerebral white matter. Am J Neuroradiol 1990;11(5):
1029-34.
11. Martin N, Debussche C, De Broucker T, Mompoint D,
Marsault C, Nahum H. Gadolinium-DTPA enhanced MR
imaging in tuberous sclerosis. Neuroradiology 1990;31(6):
492-7.
12. Menor F, Marti-Bonmati L, Mulas F, Poyatos C, Cortina
H. Neuroimaging in tuberous sclerosis: A clinicoradio-
logical evaluation in pediatric patients. Pediatr Radiol
1992;22(7):485-9.
13. Nixon JR, Houser OW, Gomez MR, Okazaki H. Cerebral
tuberous sclerosis: MR imaging. Radiology 1989;170
(3 Pt 1):869-73.
14. Pinto Gama HP, da Rocha AJ, Braga FT, da Silva CJ,
Martins Maia AC Jr, de Campos Meirelles RG, Mendonca
do Rego JI, Lederman HM. Comparative analysis of MR
sequences to detect structural brain lesions in tuberous
sclerosis. Pediatr Radiol 2006;36(2):119-25. Epub 2005 Nov
11.
15. Thibaut H, Parizel PM, Van Goethem J, De Schepper AM.
Tuberous sclerosis: CT and MRI characteristics. Eur J
Radiol 1993;16(3):176-9.
16. Truhan AP, Filipek PA. Magnetic resonance imaging: Its
role in the neuroradiologic evaluation of neurofibro-
matosis, tuberous sclerosis, and Sturge-Weber syndrome.
Arch Dermatol 1993;129(2):219-26. Review.
 Chapter 53 Tuberous Sclerosis 699

A B

Figs 53.3A to C: (A) Axial CT of the orbit showing a small

elevated lesion in the superolateral aspect of the right (arrows)—
retinal hamartoma and (B and C) Axial noncontrast CT at the
level of the lateral ventricle showing hyperdense subependymal
C nodules with varying calcification
700 Section 9 Phacomatosis

A B

Figs 53.4A and B: Axial FLAIR images of the brain in a known case of tuberous sclerosis showing small subependymal nodules
with cortical tubers (arrows)
Chapter 54
Von Hippel-Lindau Disease
• It is an autosomal dominant disorder with incomplete
penetrance
• The clinical diagnosis of von Hippel-Lindau (VHL)
disease is based on the presence of multiple
hemangioblastomas of the CNS, one hemangio-
blastoma plus visceral manifestation, or one central
or visceral manifestation in a patient with an affected
first-order family member
• If a retinal angioma is present it usually becomes
symptomatic in their 20s; patients with hemangio-
blastoma of brain and spinal cord become
symptomatic in their mid-to late 30s; and renal cell
carcinoma develop in their mid-40s
• It is a condition characterized by the association of
retinal and cerebellar angiomas
• The eponym von Hippel-Lindau disease is used when
the disorder involves both the central nervous system
and the retina. The condition is called von Hippel’s
disease when only the retina is involved
• It is transmitted as an autosomal dominant trait with
irregular penetrance. The gene responsible for this is
linked to the DNA markers that map to the short arm
of chromosome 3. The patient usually becomes
symptomatic in the 3rd decade of life though retinal
lesions may be present at birth.
FEATURES
• CNS involvement: The characteristic lesion is a cystic
or solid cerebellar hemangioblastoma (Lindau tumor)
which is responsible for death in most cases. It is
located laterally and posteriorly, is well-demarcated
and completely resectable, but can recurr after
surgery. Symptoms include headache, vomiting,
vertigo and signs of cerebellar dysfunction.
Hemangioblastomas may also be seen in the medulla
oblongata and spinal cord (cervical and thoracic)
• Visceral involvement: Renal cell carcinoma, bilateral
pheochromocytomas, renal and pancreatic cysts,
adenomas of the epididymis, kidneys and liver, cystic
lesions of the lungs, adrenals, bone, omentum, and
mesocolon may be present
• Ophthalmic involvement: Usually, retinal angiomas are
small and peripheral and seen in 40 to 60 percent of
patients. In 1/3rd of patients more than one angioma
can be present. It usually begins as a focal collection
of capillaries that enlarges overtime to form a globular
reddish tumor, 1 to 3 disc diameters in size. A classical
retinal angioma is supplied by a single dilated and
tortuous feeder artery leading from the disc to the
tumor and drained by a vein back to the tumor. In 50
percent cases, lesions are bilateral and are mostly
located in the midperipheral retina, usually in the
inferotemporal quadrant. The angiomas may appear
flat or slightly elevated and may be associated with
retinal exudation and detachment, neovasculariza-
tion, neovascular glaucoma and can lead to phthisis
• On fluorescein angiography, the angiomas show
bright hyperfluorescence with dye leakage from the
incompetent vascular endothelium. The feeder vessel
is seen. Patients with retinal angiomas should undergo
a CT scan or MRI of the head, spinal cord and
abdomen and urine analysis for catecholamines.
Imaging
Von Hippel-Lindau (VHL) disease is a multisystem
disease that is characterized by cysts, angiomas and
neoplasms of the CNS and abdominal viscera. Common
lesions and their incidences are as follows:
• Retinal angiomas (40 to 50%)
• Hemangioblastomas (40 to 80%)
• Cerebellum (75% of hemangioblastomas in VHL)
702 Section 9 Phacomatosis
A B
C D
E F
Figs 54.1A to F: (A) Axial T1 post-
contrast, (B) T2-weighted image, (C)
Sagittal T1-weighted image, (D)
Sagittal T1 postcontrast, (E) Coronal
postcontrast image showing a well-
defined T2 hyperintense brilliantly
enhancing hemangioblastoma in the
posterior fossa with a prominent
feeding vessel at the periphery of the
lesion (arrow in Figure B) and (F)
Sagittal postcontrast T1-weighted
image of the cervical spine showing
two small brilliantly enhancing
intramedullary lesion in the cervical cord
with syrinx formation—suggestive of
cord hemangioblastoma (arrows)
 Chapter 54 Von Hippel-Lindau Disease 703

A B

C D

E F

Figs 54.2A to F: (A and B) Color fundus photograph of a 58-year-old male showing retinal angioma with
exudation and subretinal fluid in the peripheral retina, (C and D) Axial T1-postcontrast and T2-weighted MRI
of the same patient showing well-circumscribed cystic lesion with thick peripheral enhancement suggestive
of cerebellar hemangioblastoma and (E and F) Digital subtraction angiogram of this patient—left vertebral
injection showing tumor vascularity supplied by the basilar branches
704 Section 9 Phacomatosis
• Spinal cord (25% of hemangioblastomas in VHL)
• Visceral cysts and neoplasms (50 to 70%).
Ocular Lesions
Retinal angiomas found in 50 to 60 percent of patients with
VHL. Bilateral in 50 percent of the patients. Diagnosis is
clinical and readily apparent on fundus examination.
CEREBELLAR HEMANGIOBLASTOMAS
Hemangioblastomas are found in two-thirds of the
patients with VHL. Most hemangioblastomas are
detected between 20 to 50 years of age. Ninety percent
occurs in the posterior fossa. The most common location
is the cerebellum, followed by brainstem and spinal
cord. Multiple lesions are considered diagnostic of VHL.
Imaging
• On CT scans, 80 percent of hemangioblastomas appear
cystic, with an isodense, noncalcified mural nodule that
shows strong enhancement after contrast enhancement.
Solid tumors occur in 20 percent of all cases
• On MR scans, hemangioblastomas usually show
prolonged T1 and T2. It may be complex, if hemorrhage
has occurred. An isointense mural nodule that shows
intense contrast enhancement. “Flow voids” in the
afferent and efferent vessels supplying the tumor can
be detected
• Spinal cord hemangioblastomas appear as syrinx-like
cyst with an isointense nodule that enhances strongly
after contrast
• Non-CNS lesions: Visceral cysts and neoplasms are
common in VHL. Renal cell carcinoma is the most
frequent tumor followed by pheochromocytoma
• Prognosis: Large untreated angiomas in the eye carry
poor visual prognosis. Frequent causes of death are
cerebellar hemangioblastoma or renal cell carcinoma.
BIBLIOGRAPHY
1. Choyke PL, Filling-Katz MR, Shawker TH, Gorin MB, Travis
WD, Chang R, Seizinger BR, Dwyer AJ, Linehan WM. von
Hippel-Lindau disease: Radiologic screening for visceral
manifestations. Radiology 1990;174(3 Pt 1):815-20.
2. Choyke PL, Glenn GM, Walther MM, Patronas NJ,
Linehan WM, Zbar B. von Hippel-Lindau disease: Genetic,
clinical, and imaging features. Radiology 1995;194(3):629-
42. Review.
3. Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM,
Rigamonti D. Hemangioblastomas of the central nervous
system in von Hippel-Lindau syndrome and sporadic
disease. Neurosurgery 2001;48(1):55-62; discussion 62-3.
4. Dollfus H, Massin P, Taupin P, Nemeth C, Amara S,
Giraud S, Beroud C, Dureau P, Gaudric A, Landais P,
Richard S. Retinal hemangioblastoma in von Hippel-
Lindau disease: A clinical and molecular study. Invest
Ophthalmol Vis Sci 2002;43(9):3067-74.
5. Fill WL, Lamiell JM, Polk NO. The radiographic
manifestations of von Hippel-Lindau disease. Radiology
1979;133(2):289-95.
6. Filling-Katz MR, Choyke PL, Patronas NJ, Gorin MB,
Barba D, Chang R, Doppman JL, Seizinger B, Oldfield
EH. Radiologic screening for von Hippel-Lindau
disease: The role of Gd-DTPA enhanced MR imaging
of the CNS. J Comput Assist Tomogr 1989;13(5):
743-55.
7. Glasker S. Central nervous system manifestations in VHL:
Genetics, pathology and clinical phenotypic features. Fam
Cancer 2005;4(1):37-42. Review.
8. Herron J, Darrah R, Quaghebeur G. Intracranial
manifestations of the neurocutaneous syndromes. Clin
Radiol 2000;55(2):82-98. Review.
9. Hough DM, Stephens DH, Johnson CD, Binkovitz LA.
Pancreatic lesions in von Hippel-Lindau disease:
Prevalence, clinical significance, and CT findings. Am J
Roentgenol 1994;162(5):1091-4.
10. McCabe CM, Flynn HW Jr, Shields CL, Shields JA, Regillo
CD, McDonald HR, Berrocal MH, Gass JD, Mieler WF.
Juxtapapillary capillary hemangiomas. Clinical features
and visual acuity outcomes. Ophthalmology 2000;107(12):
2240-8.
11. Nelson DR, Yuh WT, Waziri MH, Ryals TJ, Sato Y, Kao
SC, Hawes DR, Williams RD. MR imaging of Hippel-
Lindau disease: Value of gadopentetate dimeglumine.
J Magn Reson Imaging 1991;1(4):469-76.
12. Richard S, Parker F, Aghakhani N, Allegre G, Portier F,
David P, Marsot-Dupuch K. von Hippel-Lindau disease:
Recent advances in genetics and clinical management.
J Neuroradiol 2005;32(3):157-67. Review.
13. Sato Y, Waziri M, Smith W, Frey E, Yuh WT, Hanson J,
Franken EA Jr. Hippel-Lindau disease: MR imaging.
Radiology 1988;166(1 Pt 1):241-6.
14. Sims KB. von Hippel-Lindau disease: Gene to bedside.
Curr Opin Neurol 2001;14(6):695-703. Review.
15. Singh AD, Shields CL, Shields JA. von Hippel-Lindau
disease. Surv Ophthalmol 2001;46(2):117-42. Review.
16. Webster AR, Maher ER, Moore AT. Clinical characteristics
of ocular angiomatosis in von Hippel-Lindau disease and
correlation with germline mutation. Arch Ophthalmol
1999;117(3):371-8.
 Chapter 54 Von Hippel-Lindau Disease 705

A B

Figs 54.3A and B: (A) Precontrast sagittal T1-weighted image and (B) Postcontrast sagittal T1 image showing a large cystic
cerebellar lesion with an isointense brilliantly enhancing eccentric nodule (arrow)—cystic cerebellar hemangioblastoma in a
VHL patient
Chapter 55
Wyburn-Mason Syndrome
• It is the coexistence of arteriovenous (AV) commu-
nication between the retina and the midbrain possibly
resulting from an embryologic lesion of the primitive
anterior vascular plexus during cerebral angiogenesis.
Pathologically, a tract of vascular tissue is found
connecting the AV aneurysm of the retina with the
AV malformation of the midbrain
• Most cases are unilateral. Usually becomes sympto-
matic before the age of 30 years (both ophthalmic and
central nervous system lesions).
FEATURES
• Cutaneous involvement: Vascular nevi in trigeminal
distribution on the affected side, facial angiomas and
pigmented spots on the skin can occur. Sinuses or
mandible involvement can result in epistaxis or
gingival hemorrhage
• CNS involvement: Intracerebral vascular malformation
is most commonly located in the midbrain, ipsilateral
to the retinal lesion. It can cause cerebral or
subarachnoid hemorrhage that can be recurrent and
lead to headache, vomiting, nuchal rigidity, loss of
consciousness. Lesion can expand in the midbrain and
lead to local edema, obstruction of cerebral aqueduct,
dilated lateral and third ventricles, signs of raised
intracranial tension, intermittent hydrocephalus
• Neurological signs: Cases may present with signs of
midbrain lesion, hemiparesis or hemiplegia, cerebellar
dysfunction, Parinaud’s syndrome, epilepsy, mental
changes affecting memory and intelligence, psychosis,
somnolence, irritability, depression
• Ophthalmic involvement: Direct AV communication
exists between enlarged and tortuous artery and vein
usually involving vessels of temporal retina. It can
vary from a single well-defined anastomosis to a large
mass of dilated, convoluted vessels resembling a
tumor.
CLINICAL FEATURES
• Vision varies from normal to no perception of light.
Progressive visual loss may occur due to retinal or
vitreous hemorrhage, microvascular decompensation,
leakage into macular area, compressive optic
neuropathy. Neovascular glaucoma can occur
• Patients may develop a homonymous visual field
defect due to the AV malformation of the visual
pathway. Proptosis can occur due to increased size of
retrobulbar blood vessels. Third nerve palsy
(midbrain involvement) can occur
• Orbital AV malformations may occur and be associated
with orbital bruits.
IMAGING
• Those with suspected intracranial lesions should have
postcontrast CT scan or MRI of the brain and orbits.
CT scan demonstrates the AV malformation as
irregularly hyperdense areas with spot-like contrast
enhancement
• MRI mostly shows the AVM as round or oval lesions
with sponge-like structure of low signal intensity in
T1-weighted sequences or as single-enlarged vessels.
MRI can detect abnormal vessels even in presence of
fresh or older hematoma, and gives the precise
information on size and location of the AVM.
 Chapter 55 Wyburn-Mason Syndrome 707

Fig. 55.1: Montage of the fundus depicting the arteriovenous

malformation in the temporal fundus. This depicts a large area
of convoluted vessels resembling a tumor
708 Section 9 Phacomatosis
BIBLIOGRAPHY
1. Hopen G, Smith JL, Hoff JT, Quencer R. The Wyburn-
Mason syndrome: Concomitant chiasmal and fundus
vascular malformations. J Clin Neuro-ophthalmol 1983;
3(1):53-62.
2. Kim J, Kim OH, Suh JH, Lew HM. Wyburn-Mason
syndrome: An unusual presentation of bilateral orbital and
unilateral brain arteriovenous malformations. Pediatr
Radiol 1998;28(3):161.
3. Nussel F, Wegmuller H, Huber P. Comparison of magnetic
resonance angiography, magnetic resonance imaging and
conventional angiography in cerebral arteriovenous
malformation. Neuroradiology 1991;33(1):56-61.
4. Patel U, Gupta SC. Wyburn-Mason syndrome: A case
report and review of the literature. Neuroradiology 1990;
31(6):544-6. Review.
5. Theron J, Newton TH, Hoyt WF. Unilateral retinocephalic
vascular malformations. Neuroradiology 1974;7(4):185-96.
 Chapter 55 Wyburn-Mason Syndrome 709

A B

C D

E F

Figs 55.2A to F: (A) Axial CT scan, (B) Coronal contrast-enhanced CT scan of the orbit showing irregular enhancing lesion in the
right perioptic region and (C to F) Oblique sagittal MIP views of CT angiogram showing dilated vessels in the orbit, apex and
suprasellar region—suggestive of a vascular malformation in a case of Wyburn-Mason syndrome
710 Section 9 Phacomatosis

Fig. 55.3: Digital subtraction angiography (DSA) of the same

patient—selective right internal carotid angiogram in anteroposterior
projection in the late phase showing multiple vessels around the
cavernous and supraclinoid ICA confirms the vascular malformation

A B

Figs 55.4A and B: MRI images of the above patient: (A) Sagittal and (B) Axial T2-weighted MRI showing prominent flow voids in
perioptic region
SECTION 10
Applications of Interventional
Radiology in Ophthalmology
SECTION OUTLINE
56. Neurovascular Diseases in Ophthalmology:
Diagnosis and Management
Chapter 56
Neurovascular
Diseases in Ophthalmology:
Diagnosis and Management
Broadly, we can classify them as:
1. Caroticocavernous fistula and dural AVMs of
cavernous sinus region.
2. Aneurysms of intracranial arteries like, internal
carotid artery (ICA), anterior or posterior commu-
nicating artery, posterior cerebral artery, etc. giving
ophthalmic symptoms.
3. Orbital vascular malformations: High flow-like
arteriovenous malformations and low flow-like
venous angiomas/pediatric hemangiomas.
4. Occlusive lesions of ICA and ophthalmic vessels.
CAROTICOCAVERNOUS FISTULA (CCF)
These are spontaneous or acquired connections between
the carotid artery and the cavernous sinus and can be
classified as direct or indirect.
Direct CCF represents direct connection between the
ICA and cavernous sinus. They occur as a result of a
trauma causing rupture of ICA into cavernous sinus. The
shearing forces of severe head trauma, often accompanied
by penetrating injury from bony spicules, can cause the
ICA to be torn between its points of dural attachment.
The other causes include rupture of intracavernous ICA
aneurysm into cavernous sinus, collagen deficiency
syndromes, fibromuscular dysplasia, arterial dissection
or direct surgical trauma.
Indirect CCF are actually dural arteriovenous
malformations which are supplied either by dural
branches of external carotid artery (ECA) or dural
branches of ICA or both. They are not fistula per say.
Most of the times, they occur spontaneously. However,
factors associated with their development include
pregnancy, sinusitis, trauma, surgical procedures and
cavernous sinus thrombosis.
Classification of CCF
Type A: Direct rupture of intracavernous ICA into
cavernous sinus.
Type B: Dural arteriovenous malformation between dural
branches of ICA draining into cavernous sinus (Figs 56.1A
to C).
Type C: Dural arteriovenous malformation between dural
branches of ECA draining into cavernous sinus (Figs
56.2A to C).
Type D: Dural arteriovenous malformation between dural
branches of ICA and ECA draining into cavernous sinus.
Another classification depending on therapeutic
strategies is as follows:
Type 1: Post-traumatic direct CCF.
Type 2: CCF caused by rupture of pre-existing intracaver-
nous ICA aneurysm into the cavernous sinus resulting in
two separate holes : one from ICA into the aneurysm and
other from the aneurysm into the cavernous sinus.
Type 3: Dural type of CCF.
Type 4: Combination of Type 3 with either type 1 or type 2.
Pathophysiology and Clinical Presentation of CCF
Clinical features are proptosis, chemosis, venous
retinopathy, secondary glaucoma, decline in vision and
increased intraocular pressure, pulsatile tinnitus,
dysfunction of cranial nerves III, IV, V1, VI. Diplopia and
complete ophthalmoplegia can occur due to cavernous
sinus syndrome.
The clinical features are related to the size, duration,
location of fistula, adequacy and routes of venous
drainage and to the presence of arterial and venous
collateral vessels.
714 Section 10 Applications of Interventional Radiology in Ophthalmology

A B

Figs 56.1A to C: Type B CCF: (A) Arterial phase of lateral

view of left internal carotid angiogram shows an evidence of
CCF. Note: Incidental stenosis of left ICA at that level,
(B) Venous phase of lateral view of left internal carotid
angiogram shows opacification of left superior ophthalmic vein
and (C) Lateral view of left external carotid angiogram shows
C no abnormality
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management
Elevated venous pressure in veins draining the orbit may
produce orbital venous congestion, transudation of
interstitial fluid into the orbit with resultant proptosis,
increased intraocular pressure due to impaired drainage
of aqueous humor and secondary glaucoma. These may
compromise retinal perfusion and result in severely
diminished visual acuity which may or may not be
reversible. Orbital symptoms are frequently related not
only to the degree of shunt but also to the adequacy of
external drainage of the superior ophthalmic vein.
The clinical presentation may not accurately reflect
the pathology.
Emergency treatment is indicated, if the intraocular
pressure rises above 40 mm Hg to prevent permanent
loss of vision and when there is reversal of venous
drainage into the sphenoparietal sinus/cortical veins for
the fear of intracerebral hemorrhage.
Rarely, a unilateral CCF may present with bilateral
orbital symptoms, by supplying the contralateral
cavernous sinus with arterialized blood via the circular
sinus creating free intercavernous communication.
Radiological Evaluation of CCF
Diagnosis is essentially clinical. Gold standard imaging
study is digital subtraction angiography (DSA).
Role of CT and MRI is only in establishing the degree
of associated brain injury and skull fractures.
Evaluation of CCF by DSA:
1. Identifying the type of CCF
2. Size and location of fistula
3. Identification of and confirmation of patency of
outflow pathways of cavernous sinus.
4. Identification of high-risk features like cortical venous
drainage, pseudoaneurysm, cavernous sinus varix.
5. Identification of associated vascular injuries in
traumatic CCF or of ruptured aneurysm.
6. To study cross-circulation through circle of Willis.
Treatment of CCF
Options
i. Spontaneous closure of Type B, C , D can occur.
ii. Intermittent manual carotid compression maneuver
for dural type of low flow fistulae.
iii. Transarterial balloon occlusion of Type A fistulae
using flow directed detachable latex/silicon balloons
(Figs 56.3A to F).
iv. Rarely occlusion of ICA for Type A CCF.
v. Transcatheter coil embolization via an arterial or
venous route using platinum microcoils (Figs 56.4
and 56.5).
715
Complications of Endovascular Treatment of CCF
1. Thromboembolic or ischemic events due to micro-
catheter and balloon manipulation injury to the parent
vessel or inadvertent balloon detachment.
2. Pseudoaneurysm formation due to balloon deflation
or migration.
3. Alteration of arterial flow resulting in hemorrhage,
edema or worsening of ocular symptoms.
4. Jeopardizing supply to cranial nerves during emboli-
zation of dural CCFs.
INTRACRANIAL ANEURYSMS
Small intracranial aneurysms usually present with
subarachnoid hemorrhage but giant (more than 2.5 cm
in size) aneurysms present with mass effect in more than
65-70 percent cases and symptoms are dependent on
aneurysmal location.
All anterior circulation giant intracranial aneurysms
are near the visual pathways and can thus have
symptoms related to sight.
Giant aneurysms of cavernous segment of ICA present
with ophthalmoplegia, retro-orbital headache, facial
sensory loss and sometimes massive epistaxis (Fig. 56.6A).
Paraophthalmic (paraclinoid) ICA aneurysms also
present with retro-orbital headache, visual field defects,
decreased vision secondary to optic nerve/chiasm
compression (Fig. 56.6B).
Carotid bifurcation aneurysms cause visual field
defects, frequently homonymous hemianopia (Fig. 56.6C).
Giant aneurysms of anterior communicating artery
can cause bitemporal hemianopia (Fig. 56.6D).
Posterior communicating artery aneurysms can
present with ptosis due to compression of III nerve even
when they are small in size (Figs 56.6E and 56.7).
Radiological Investigation for Intracranial Aneurysms
CT scan: Presence of subarachnoid hemorrhage can be
seen on CT scan. In giant aneurysms, their size, wall
calcification and thrombus can be depicted well with CT
scan. CT angiogram with 3D reconstructions shows
aneurysms extremely well noninvasively.
MRI and MR angiogram: Aneurysms as small as 3 to 4 mm
can be seen but visualization is flow dependent. If the
blood flow in the aneurysm is poor, the visualization is
suboptimal.
Digital subtraction angiogram: It is the gold standard. It
shows presence, number, location, size, shape, neck and
fundus of the aneurysm. It shows associated vasospasm.
And it is the only imaging modality which shows cross-
circulation through circle of Willis.
716 Section 10 Applications of Interventional Radiology in Ophthalmology

A B

Figs 56.2A to C: Type C CCF: (A) Arterial phase

anteroposterior view of right external carotid angiogram shows
evidence of CCF supplied by dural branches of right internal
maxillary artery, (B) Venous phase of anteroposterior view of
right external carotid angiogram shows drainage to cavernous
sinus and (C) Lateral view of right internal carotid angiogram
C shows no abnormality
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management
Treatment
1. Surgery.
2. Endovascular therapy using coils to occlude
aneurysm sac.
3. In giant aneurysms, parent vessel occlusion using
balloon or coils produces subsequent thrombosis of
the aneurysm. Parent vessel can be occluded only
proximally or trapped. Most of the times, occlusion
distal to the aneurysm is not necessary. However,
presence of good cross-circulation through circle of
Willis is a pre-requisite to perform parent vessel
occlusion. About 90 percent of petrous or intra-
cavernous aneurysms eventually thrombose with
parent vessel occlusion (Figs 56.8 and 56.9). Seventy-
five percent of paraophthalmic aneurysms thrombose
(25% do not thrombose due to retrograde flow from
ophthalmic artery). If the giant aneurysms are
paraclinoid or supraclinoid in location, only 50 percent
will thrombose due to retrograde flow from posterior
communicating artery.
4. When cross-circulation through circle of Willis is
inadequate, parent vessel cannot be occluded for the
treatment of giant aneurysm. In such cases, stent
assisted coiling of the aneurysm is performed,
wherein stent helps to seal the mouth of aneurysm
preventing the coils in the sac from prolapsing into
the parent artery (Figs 56.10A to G)
5. Few giant aneurysms can be treated just by placing
stents across their neck. These stents divert the flow
from aneurysm sac causing progressive thrombosis
of the aneurysm (Figs 56.11A to F)
If the aneurysm does not thrombose completely, the
incidence of bleeding and continued growth is about the
same as for an untreated aneurysm.
Parent vessel occlusion of the ICA for the treatment
of an giant intracavernous aneurysm is a safe and effective
therapy provided, there is good adequate cross-
circulation through circle of Willis, yielding a cure in the
vast majority of cases.
Complications
Complications of parent vessel occlusion are essentially
limited to the ischemic events and are mostly temporary
occurring in 5 to 10 percent of patients. Permanent
deficits, that too late, can occur in about 1 to 3 percent of
patients.
Complications of coil embolization of aneurysmal sac
are aneurysm rupture during procedure, coil malposition,
717
guide catheter complications, distal embolization and
intracranial vascular damage.
ORBITAL VASCULAR MALFORMATIONS
Two major types of superficial vascular abnormalities are
hemangiomas and vascular malformations. Hemangiomas
are benign vascular endothelial tumors in children and
are characterized by phases of growth due to proliferation
of endothelial cells and phases of involution with
spontaneous slow regression.
Vascular malformations are composed of dysplastic
vessels with a normal endothelial turnover. They may be
slow flow (capillary/venous/lymphatic malformations)
and high flow (arteriovenous malformations).
Hemangiomas
Eighty percent of them regress spontaneously. USG and
MRI are useful noninvasive imaging modalities to see
the extent of the tumor and associated lesions.
Angiography is performed only when embolization
is indicated.
The sole indication for more aggressive therapy is a
large extending tumor that has failed medical treatment
(corticosteroid and interferon). They can cause com-
pression of adjacent structures (like eye, nose, etc.), and
can cause heart failure and hemorrhage. Embolization
can be done with occlusion of capillary tumor with micro-
particles after selective catheterization.
Vascular Malformations
It is diagnosed clinically: MRI is the examination of choice
to determine the location and extent of the lesion.
Angiogram is not needed for diagnosis. Three main
criteria for the treatment are cosmetic abnormalities,
functional impairment and social intolerance.
The treatment needs multidisciplinary approach
including dermatologists, plastic surgeons, vascular
surgeons and interventional radiologists.
Venous malformations can be treated using sclerosing
agents like ethibloc, alcohol or even coils or N-butyl-2-
cyanoacrylate can be used (Figs 56.12A to E). Multiple
sessions may be needed.
Arteriovenous malformations need angiography as a
gold standard for diagnosis and to study the angio-
architecture well. These can be treated with embolization
using liquid embolic agents like N-butyl-2- cyanoacrylate
or alcohol (Figs 56.13A to C). For complete cure, surgery
might be needed after embolization for resection of the
lesion.
718 Section 10 Applications of Interventional Radiology in Ophthalmology

A B

C D

E F

Figs 56.3A to F: Transarterial embolization of type A CCF with detachable balloon: (A) Lateral
view of right internal carotid angiogram shows post-traumatic low flow type A caroticocavernous
fistula with venous drainage into anterior and posterior directions, (B) A latex balloon inflated with
contrast material is sitting in the rent in the cavernous segment of internal carotid artery in case of
type A CCF, (C) Check angiogram of right internal carotid artery in lateral view shows excellent
restoration of right internal carotid artery circulation with complete obliteration of fistulous rent by
balloon, (D) Clinical picture of a patient with post- traumatic type A CCF shows excessive chemosis,
proptosis, and complete ophthalmoplegia, (E) 48 hours after obliterating the CCF, there is significant
reduction in chemosis and proptosis. However, ophthalmoplegia persists and (F) Three weeks
follow-up picture shows complete regression of proptosis with minimal residual chemosis. Third
nerve palsy has recovered, however lateral rectus palsy persists. After 3 months of treatment,
patient had complete recovery
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management
OCCLUSIVE LESIONS OF ICA AND CENTRAL
RETINAL VEIN
The stenotic lesions of ICA may cause transient ischemic
attacks, which may have amaurosis fugax as their sole or
one of the symptoms.
Such patients need to be investigated using color
Doppler of carotid arteries followed by CT/MRI/digital
subtraction angiography.
By means of endovascular technique, angioplasty and
stenting of ICA can be performed as a minimal invasive
719
mode of treatment for carotid occlusive diseases (Figs
56.14A to C).
Central retinal vein occlusion (CRVO) is a vascular
retinal problem caused by significant reduction in venous
flow which leads to progressive loss of visual acuity.
It is often seen in patients with atherosclerosis.
To treat recent and severe cases of CRVO, urokinase can
be infused intra-arterially into the ophthalmic artery through
microcatheter. This therapy immediately improves visual
acuity as well as arteriovenous retinal circulatory time. No
alternative therapy is available for this condition.
720 Section 10 Applications of Interventional Radiology in Ophthalmology

A B

C D

Figs 56.4A to D: Treatment of type A CCF with transarterial coil embolization: (A) Right carotid angiogram anteroposterior view
showing type A CCF and contralateral venous drainage, (B) Right carotid angiogram lateral view showing type A CCF and
posterior venous drainage, (C) Lateral view of right carotid angiogram shows anterior venous drainage and (D) Coil embolization—
1st coil
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management 721

E F

G H

Figs 56.4E to H: (E) Coil embolization—2nd coil, (F) Coil embolization—3rd coil, (G) Anteroposterior view of skull showing coil
mass and (H) Lateral view of skull showing coil mass
722 Section 10 Applications of Interventional Radiology in Ophthalmology

I J

Figs 56.4I and J: (I) Anteroposterior view of right carotid angiogram showing complete obliteration of CCF and (J) Lateral view of
right carotid angiogram shows complete obliteration of CCF

A B

Figs 56.5A and B: Transvenous coil embolization of type D CCF: (A) Lateral view of left external carotid angiogram shows
evidence of CCF and (B) Lateral view of left external carotid angiogram shows venous drainage in anterior and posterior (arrow)
directions
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management 723

C D

E F

Figs 56.5C to F: (C) Lateral view of left internal carotid angiogram shows evidence of CCF, (D) Microcatheter is in the fistula
through venous route, (E) 1st coil and (F) 8th coil
724 Section 10 Applications of Interventional Radiology in Ophthalmology

G H

Figs 56.5G to I: (G) 12th coil, (H) Anteroposterior view of left

common carotid angiogram shows obliteration of malformation
and (I) Lateral view of left common carotid angiogram shows
I complete obliteration of malformation
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management 725

A B

C D

Figs 56.6A to E: Intracranial aneurysms presenting with

orbital symptoms: (A) Patient presented with ophthalmic
symptoms. Angiogram showed giant aneurysm of
cavernous segment of left internal carotid artery.
Incidentally, another small saccular aneurysm is noted
arising from left middle cerebral artery bifurcation.
Multiplicity of aneurysms in cerebral circulation is not
uncommon, (B) Giant paraclinoid ICA aneurysm, (C) An
irregular large aneurysm arising from bifurcation of left
internal carotid artery presenting as homonymous
hemianopia, (D) Bilobed anterior communicating artery
aneurysm presenting as bitemporal hemianopia and (E)
Small elongated aneurysm from the junction of left internal
carotid artery and left posterior communicating artery as
seen in this lateral view of left carotid angiogram. Patient
E presented as ptosis (arrows)
726 Section 10 Applications of Interventional Radiology in Ophthalmology

A B

C D

Figs 56.7A to D: Endovascular treatment of dissecting aneurysm of right posterior cerebral artery: (A) Patient presented with
ptosis and ophthalmoplegia. T2-weighted MRI image showing a large irregular aneurysm in the region of right posterior cerebral
artery and (B to D) Left vertebral angiogram shows irregular large dissecting aneurysm arising from P2 segment of right posterior
cerebral artery in anteroposterior, lateral and oblique views, respectively. For dissecting aneurysm, choice of treatment is obliteration
of parent artery
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management 727

E F

Figs 56.7E and F: (E) Coil mass and (F) Post-coiling angiogram shows complete obliteration of aneurysm. Right posterior
cerebral artery was restored through posterior communicating artery (arrows). On six months follow-up, patient's ptosis had
disappeared, vision was normal, however, lateral rectus paresis persisted

A B

Figs 56.8A and B: Giant aneurysm of ICA treated with parent artery occlusion: (A) Giant ‘8’ shaped aneurysm arising from the
cavernous segment of left internal carotid artery projecting into the sphenoid sinus. Patient had ophthalmic symptoms to start with
followed later by intractable epistaxis and (B) After checking that the crosscirculation through the circle of Willis was adequate, left
internal carotid artery was occluded using coils (arrows). Patient's symptoms regressed quickly over next few days
728 Section 10 Applications of Interventional Radiology in Ophthalmology

A B

C D

Figs 56.9A to D: Giant aneurysm of ICA: (A and B) Giant saccular aneurysm is seen arising from cavernous segment of right
internal carotid artery in anteroposterior (A) and lateral views (B); Cross-circulation through circle of Willis was checked prior to
occlusion of right ICA. Cross-circulation was adequate upto venous phase. (C) Hence giant aneurysm was treated by “inside out
coiling”, i.e by deploying coils inside the aneurysm sac as well as in ICA to occlude it permanently; (D) Postembolization right
common carotid angiogram shows occlusion of right ICA (arrows)
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management 729

E F

Figs 56.9E and F: (E) shows filling of right ACA branches through anterior communicating artery on left carotid angiogram after
right ICA occlusion. (F) shows filling of right MCA branches through posterior communicating artery on left vertebral angiogram
after right ICA occlusion (arrows)

A B

Figs 56.10A and B: Stent assisted coiling of giant aneurysm of ICA. Right carotid angiogram shows giant aneurysm from
cavernous segment of right ICA as seen in: (A) Anteroposterior and (B) Lateral views. There was another small aneurysm
proximal to giant aneurysm (arrows)
730 Section 10 Applications of Interventional Radiology in Ophthalmology

C D

Figs 56.10C to E: (C) Since the neck of the aneurysm was

wide and cross-circulation through circle of Willis was not
adequate, aneurysm was treated with stent-assisted coiling.
Stent was deployed covering the necks of both giant and small
aneurysms, (D) Post-treatment anteroposterior and (E) Lateral
views show complete obliteration of giant aneurysm (black
arrow). Small aneurysm is still seen (white arrows). Stent
deployed across the neck of small aneurysm is believed to
divert the flow away from aneurysm arresting its growth and
E promoting its thrombosis
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management 731

F G

Figs 56.10F and G: Coil mass as seen in: (F) Anteroposterior and (G) Lateral views. Stent markers are not radiopaque enough
to see clearly

A B

Figs 56.11A and B: Treatment of giant aneurysm of ICA with flow diversion using stents. Left carotid angiogram shows giant
aneurysm of cavernous segment of ICA in: (A) Anteroposterior view and (B) Lateral view
732 Section 10 Applications of Interventional Radiology in Ophthalmology

C D

E F

Figs 56.11C to F: (C and D) Balloon occlusion test for cross-circulation through circle of Willis shows no cross-circulation through
anterior and posterior communicating arteries. Hence, occlusion of internal carotid artery was not an option, (E) Two stents were
deployed across the aneurysm (arrows) and (F) There is stasis of contrast in aneurysm after deploying stents due to reduction of
flow into aneurysm
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management 733

A B

C D

Figs 56.12A to E: Percutaneous sclerotherapy of orbital

varix (A) Swelling below the lower eyelid on right side
was soft, non-pulsatile and was increasing on bending
forward and with valsalva maneuver (classical of venous
varix), (B) Ultrasound shows sonolucent lesion (with
venous flow in it), (C and D) Under IV sedation, venous
varix was accessed with 23G needle and foamy
sclerosant was injected into it and (E) Three months
E follow-up shows no evidence of venous varix
734 Section 10 Applications of Interventional Radiology in Ophthalmology

A B

C D

E F

Figs 56.13A to F: Orbital arteriovenous malformation (A) Clinical picture showing swelling over right upper
eyelid and eyebrow which was pulsatile. Patient gave trivial history of trauma in childhood, (B) Lateral view
of right common carotid angiogram showing compact medium size nidus in the right orbital region, (C)
Venous phase of the angiogram shows venous drainage into posterior and inferior directions and (D to F)
Lateral view of right internal carotid, right external carotid and left internal carotid angiograms showing their
selective contributions to the nidus (arrows)
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management 735

G H

I J

K L

Figs 56.13G to L: (G to I) In view of superficial location of the nidus and predominant supply from bilateral
ophthalmic arteries, the nidus was percutaneously accessed using 23 G needle and the nidus was embolized
using Histoacryl and Lipiodol mixture, (J and K) Anteroposterior and lateral views of plain X-rays of orbit show
cast of Histoacryl and Lipiodol mixture which exactly conforms to the nidus seen in angiogram and
(L) Postembolization left carotid angiogram shows minimal residual nidus supplied by left ophthalmic artery
736 Section 10 Applications of Interventional Radiology in Ophthalmology

M N

Figs 56.13M to O: (M and N) Lateral and anteroposterior views of right common carotid injections show complete obliteration of
nidus and (O) Patient underwent surgical excision of nidus after embolization. This figure is at 2-year follow-up
 Chapter 56 Neurovascular Diseases in Ophthalmology: Diagnosis and Management 737

A B

Figs 56.14A to C: Carotid stenting: (A) Left common

carotid angiogram shows severe irregular stenosis at its
bifurcation which is extending into the origins of left internal
and external carotid arteries. Patient presented with
transient ischemic attacks including transient loss of vision,
(B) Patient was subjected to carotid angioplasty and
stenting. This figure shows metallic stent in situ and (C)
Check angiogram after stenting shows excellent
restoration of the lumen of left internal and common carotid
C artery
 Index

Page numbers followed by f refer to figure.

A Angioma, cavernous 539, 542f Atypical meningioma 560f

Anomalies Avulsion
Abnormal retinal pigmentation 654f
of globe, congenital 36 of optic nerve 392f
Abscess
optic nerve 326 optic nerve 389
cerebral 486
Anophthalmia 38
orbital 173
Anterior
pituitary 462 B
choroidal artery infarct 473f
subperiosteal 173
falx meningioma 562f Basilar artery 404f
Acoustic
migration of scleral buckle 134f Benign lesions 557
neurofibroma 344
schwannoma 575, 685 Apert’s syndrome 165, 168f Bilateral
Acute Apex and canal fractures, orbital 314 asymmetric enlargement
clots 526 Aphakia 131, 132 of lacrimal glands 191
hemorrhage 525 Aplasia gradual loss of
hydrocephalus 506 congenital 330 vision 301f, 409f
infarct 510 lacrimal 249 occipital gliosis 477
Adenoid cystic Apoplexy, pituitary 446 optic
carcinoma 263, 264f, 165 Aqueductal stenosis 505f atrophy 477f
recurrent 266f Arachnoid cyst 421 nerve aplasia 331f
Adenoma, pituitary 404f, 429 Arnold-Chiari malformation 648 proptosis 166f, 305f
Adrenoleukodystrophy 478, 479f Arterial infarction 519 putaminal necrosis 677f
Adult neoplasms 74 Arteriovenous malformation 210, 536 sclera calcification 116f
Agenesis of corpus callosum 329f orbital 734f symmetrical enlargement of
Aicardi syndrome 107 Artery, cerebral 517f lacrimal glands 257f
Alcoholism, chronic 676 Astrocytoma 548, 552 Blow-out and
Amyloidosis 195, 196f cystic 550f blow-in fractures 312
Aneurysm 449 retinal 89, 90 Bony abnormalities of
intracranial 715 Asymmetric enlargement of orbit, congenital 165
of ica, giant 728f lacrimal glands, bilateral 191 Brain 421
Angiogram of brain 7f Atrophy of midbrain and parenchyma 491
Angiography 569 superior colliculus 635f Brainstem glioma 623
cerebral 536 Atrophy, optic 477f Breast, carcinoma 343f
740 Sankara Nethralaya Atlas of Imaging in Ophthalmology

C Chiasmatic glioma 409f Coats’ disease 102, 103f

and hamartoma 646f Colloid cyst 590, 591f
Calcification, intraocular 113
glioma, hypothalamic 408 Coloboma, optic
Canal fracture, optic 318
Chloroma 238f, 270f disc 326, 327f
Capillary hemangioma 198, 198f, 199f
Cholesteatoma 627f nerve 327, 327f
Carcinoma
Chordoma 460 Communication
breast 343f
Choristoma 158 artery, posterior 533
choroid plexus 557, 558f
Choroid plexus 557 Compression, optic nerve 306f
maxillary sinus 290
carcinoma 557, 558f Confirms germinoma 419f
of maxillary antrum 291f
tumors 557 Congenital
Caroticocavernous fistula 713
Choroidal anomalies of globe 36
Cavernous
artery infarct bony abnormalities
angioma 539, 542f
anterior 473f of orbit 165
hemangioma 201f–203f
lateral 472 cystic eye 36
malformations 200
detachment 111 cytomegalovirus infection 507f
sinus
retinal and 109 lesions 158, 475
portion 609
hemangioma 91, 92f of lacrimal gland 248, 249
thrombosis 173
diffuse 92f optic nerve
CCF, classification of 713
hematoma 112f anomalies 326
Cell
osteoma 95 aplasia 330
arteritis, giant 530
tuberculoma 62f TORCH infection 116f
carcinoma, squamous 291f
Chronic Conjunctival lipodermoids 159f
Cellulitis with phlegmon, orbital 171f
alcoholism 676 Connective tissue system 23
Central
clots and MRI 526 Contusion, optic nerve 395f
nervous system 530
hemorrhage 526 Convergence retraction
neurocytoma 507f
early 525 nystagmus 633f
Cerebellar hemangioblastomas 704
hydrocephalus 506 Convexity
Cerebellopontine angle, recurrent 560f
infarct 510 meningioma, parietal 564f
Cerebellum, lesions of 642
nonspecific inflammation 253f Cranial nerve
Cerebral
progressive external pathway, third 603
abscess 486
ophthalmoplegia 628 Craniofacial dysostosis 165
angiography 536 Ciliochoroidal melanoma 75f Craniopharyngioma 414, 415f, 647f
artery 517f Circulation, intracranial 7f cystic 417f
posterior 514, 533 Classic bitemporal Craniosynostosis 165
atrophy, diffuse 362f, 501f hemianopia 404f Crouzon’s syndrome 165, 166f
infarction 508 Classification of Cryptophthalmia 42
ischemia 508 CCF 713 Cyst, dermoid 161f, 592
parenchymal metastases 577 mucopolysaccharidoses 483 Cystic
vein occlusion 510 Clots astrocytoma 550f
Chiari malformation 648 acute 526 craniopharyngioma 417f
Chiasm and MRI, chronic 526 eye, congenital 36
disorders of 401 CNS schwannoma 275f
optic 403, 404f lesions 685 Cysticercosis 367f
Chiasmal orientation of leukemia primary 583 Cytomegalovirus
nerve fibers, optic 404f metastasis 577 infection, congenital 507f
 Index 741

D Drusen, optic disc 337, 338f F

Ductal adenocarcinoma 267, 268f
Dandy-Walker Falx meningioma, anterior 562f
Dumb-bell dermoid 162f
syndrome 651, 652 Fibro-osseous lesions 286
Dural sinus 510
Dawson’s fingers 346 Fibrous
Dysfunction, optic nerve 323
De Morsier’s syndrome 332 dysplasia 286
Dysplasia, fibrous 286
Demyelinating histiocytoma 276, 277f, 279f
Dysplastic glial tissue 377f
optic neuritis, idiopathic 345 lesions 276
Demyelination 471, 657 Floor fractures 312
primary 659 E Foreign bodies of
secondary 672 eye and orbit 123
Dense central calcification 295f Early Fossa, pituitary 567f
Dermoid chronic hemorrhage 525 Fourth nerve
and epidermoid cyst 160 subacute infarct 510 palsy 618
cyst 161f, 592 Edema pathway 603
inclusion cysts 423 inflammatory 173 Fracture of walls of
Detachment optic disc 325f nasolacrimal canals 318f
choroidal 111 Emphysema, orbital 391 Free-induction decay 9
of retina, choroid and Empty sella 439 Frontoethmoid mucocele 283f
vitreous 109 primary 440f Fungal
retinal 78f, 109, 110f Encephalocele, sphenoid 329f infection of lacrimal sac 179f
Devic’s disease 347 Encephalopathy orbital infections 176
Diffuse HIV 362f sinusitis 364f
and localized orbital hypertensive 522
hemorrhage 391 Endophthalmitis 46
cerebral atrophy 362f, 501f Enhancing lesions, right 488 G
choroidal hemangioma 92f Enlargement of Ganglioneuroma 637f
enlargement of right lacrimal gland, diffuse 270f Gaze palsy 634
right lacrimal gland 270f Eosinophilic Geniculate body, lateral 467, 471
infiltrating astrocytoma 552f granuloma 242 Germinoma 418
right cerebellar atrophy 631f granulomatosis 427 Giant
Digital subtraction angiography 710f Epibulbar osseous aneurysm of ICA 728f
Disc choristoma 115, 116f cell arteritis 530
drusen, with morning glory 338f Epidermoid cyst 425, 594 Gland 438
morning glory 329f dermoid 160 lacrimal 32f
optic 327f Ewing’s sarcoma 224, 225f Glioblastoma
pallor 325f Extensive intraocular multiforme 549, 553, 554f
Disorders of hemorrhage 128f Glioma, optic nerve 376, 689f
chiasm 401 Extraocular Globe rupture 119
optic nerve 321 extension, with Gradual loss of
orbit 139 melanoma 75f vision, bilateral 301f, 409f
Displaced anterior muscles 23 Granuloma 60
cerebral arteries 562 hypoplastic 39f eosinophilic 242
Distensible venous Eye disease, thyroid 302 Granulomatosis, eosinophilic 427
malformations 208 Eyeball injuries 119 Granulomatous neuritis 356f
742 Sankara Nethralaya Atlas of Imaging in Ophthalmology

H Hydrocephalus 504 Infiltrating astrocytoma, diffuse 552f

acute 506 Infiltration, optic nerve 364f
Hemorrhage
chronic 506 Infiltrative optic neuropathy 368
acute 525
Hyperacute infarct 510 Inflammatory
chronic 526
Hypertensive edema 173
intracranial 525, 526
encephalopathy 522 lacrimal gland lesions 251
intraocular 121
intracerebral hemorrhage 522 lesions of eye 46
orbital 314
Hypoplastic Inherited metabolic disorders 478
subacute 525
extraocular muscles 39f Insertion, inferior oblique 24f, 30f
Hamartoma 646f
optic disc 332 Internal carotid artery 404f
hypothalamic 456
Hypothalamic Internuclear ophthalmoplegia 630
of tuber cinerium 457f
chiasmatic glioma 408 Intracerebral haemorrhage,
Hand-Schüller-Christian disease 242
hamartoma 456 hypertensive 522
Head, optic nerve 31f
Hypoxic ischemic Intracranial
Helical scan 4f
encephalopathy 475 aneurysms 715
Hemangioma 717
circulation 7f
cavernous 201f–203f
hemorrhage 525, 526
choroidal 91, 92f
I primary 508
multiple 201f
Idiopathic hypertension, idiopathic 340f
Hemangiopericytoma 214f, 569, 570f
orbital 212 demyelinating optic neuritis 345 neoplasms 545

recurrent 213f intracranial hypertension 340f optic nerve 360f, 563f

Hematoma orbital inflammation 253f tumors 545

choroidal 112f Implants, orbital 133 vascular malformations 536

subacute 624f Inclusion cysts, dermoid 423 Intraocular

subperiosteal 320f Infarct calcification 113
Hemorrhage 641f acute 510 hemorrhage 121
Hemorrhagic chronic 510 neoplasms 63
infarction of brain 519 posterior 515 Intraorbital optic nerve 324f
Heterogeneous Infarction Intraventricular meningioma 562f
lacrimal gland mass 264f cerebral 508 Inversion recovery imaging 13
High-grade venous 519 Iris melanoma 76
lymphoma 232f, 233 Infection Ischemia, cerebral 508
Histiocytoma, fibrous 276, 277f, 279f of lacrimal Isolated orbital fractures 310
Histiocytosis 428f gland, idiopathic 248
HIV sac, fungal 179f
optic neuritis 359
J
encephalopathy 362f
optic neuropathy 359 Infective lesions, orbital 170 Joubert’s syndrome 653
Homonymous Inferior Juvenile xanthogranuloma 246, 247f
hemianopia 509f, 527f, 553f oblique
right 472f insertion 24f, 30f
sectoranopia, left 472 muscle 29f L
Horizontal gaze palsy 634 orbital colobomatous cyst 41 Lacrimal
Horner’s syndrome 636, 637f rectus muscle 25f, 33f gland 32f
Hyaloid detachment, posterior 112f temporal quadrant 404f aplasia 249

lesions of 248
inflammatory 251
neoplasms 259
of congenital 248, 249
of lymphoproliferative
disorders 269
pseudotumor 186f
tuberculosis of 256, 258f
tumors of 248
system 23
Lamina cribrosa 390
Langerhans cell histiocytosis 242
Late chronic hemorrhage 525
Lateral
choroidal artery infarct 472
geniculate body 467, 471
rectus muscles 392f
LeFort type fractures 314
Left
homonymous sectoranopia 472
pie-in-sky appearance 473f
quadruple sectoranopia 473
Leiomyoma 82, 83f, 216f
orbital 215
Leptomeningeal metastases 577
Lesions of
cerebellum 642
CNS 685
congenital 158, 475
eye, inflammatory 46
fibrous 276
lacrimal 248
gland 248
maxillary sinus, malignant 290
ocular 704
orbit, vascular 197
retrochiasmal 469
thalamus 642
Letterer-Siwe disease 242
Leukemia 237
Leukemic optic neuropathy 382
Leukocoria 96
Localized orbital
haemorrhage, diffuse 391
Index 743
Localizing nystagmus 642 Metastases
Location of fibers 470 CNS 577
Lymphatic malformations, venous 204 optic nerve 386
Lymphoma 228f, 229f, 236f, 270f, 384, orbital 307
459f, 588, 589 uveal 84
CNS primary 586, 588f Metastatic
orbital 230 neuroblastoma 221, 222f
Lymphomatous infiltration 231f Methanol poisoning 676
Lymphoproliferative disorders 226 Microphthalmia 40
of lacrimal gland 269 Middle cerebral
artery infarct 515
Migration of
M scleral buckle, anterior 134f
Magnetization transfer Mixed lacrimal
techniques 665 gland tumor, malignant 262f
Malformations, cavernous 200 Molar tooth sign 655f, 656f
Malformations, vascular 717 Morning glory
Malignant disc 329f
lesions of maxillary sinus 290 with disc drusen 338f
mixed lacrimal gland tumor 262f syndrome 326, 329f
orbital tumors, pediatric 217 Motility disorders, ocular 603
schwannoma 275f Moyamoya disease 543, 544f
Maple syrup urine disease 481 Mucocele, sphenoid 285f
Maxillary Mucopolysaccharidoses 483
antrum, of carcinoma 291f classification of 483
carcinoma 290 Multifocal leukoencephalopathy,
sinus 142 progressive 498, 673f
Meckle’s cave 274, 340f, 686f Multiple
Medial blow-out fracture 312 hemangioma 201f
Medulloepithelioma 72, 73f meningioma 437f
Melanocytoma 80, 81 rounded nodular lesions 581f
Melanoma 74, 79 sclerosis 346, 659f
with extraocular extension 75f tuberculomas 496f
Melanomas, uveal 74 Muscle, inferior oblique 29f
Meningeal carcinomatosis 343 Mycotic aneurysms 502
Meningioma 435, 437f, 559, 685 Myositis 184f
multiple 437f
parietal 565f
Meningitis 452
N
sarcoid 358f Nasoethmoid complex
tuberculous 343f, 366f, 452, 497f fractures 312, 318f
Meningocele, optic nerve 335, 336f Nasopharyngeal carcinoma 296
Mesenchymal Neck of aneurysm 614f
chondrosarcoma 294, 295f Necrosis, radiation 66f, 598f
744 Sankara Nethralaya Atlas of Imaging in Ophthalmology

Neoplasms trauma 119 Orbit 36, 142, 146, 230, 280

intracranial 545 Oculomotor nerve 404f disorders of 139
intraocular 63 Olfactory groove meningioma 563f of neurofibromatosis 271
lacrimal 259 Oligodendroglioma 555, 556f protocols 157
pediatric 63 Olivopontocerebellar atrophy 638, 639f Orbital
Nerve 23 Oosterior scleritis 51f abscess 173
aplasia, optic 331f Ophthalmic apex and canal fractures 314
optic 22f, 23, 25f, 26f, 34f, 348, 404f artery 735f arteriovenous malformation 734f
palsy, third 609 vein, superior 173 cellulitis with phlegmon 171f
Nervous system, central 530 Ophthalmopathy, thyroid 302 colobomatous cyst, inferior 41
Neuritis, optic 345, 346, 357 Optic cysticercosis, ocular 53
Neuroblastoma 222f, 223f canal fracture 318 emphysema 391
Neurocysticercosis 490, 491 chiasm 403, 404f fissure, superior 606f
Neurocytoma, central 507f chiasmal orientation of nerve fibers hemangiopericytoma 212
Neuroepithelial tumors of CNS 545 404f hemorrhage 314
Neurofibroma 275 disc 327f implants 133
Neurofibroma, acoustic 344 coloboma 326, 327f infections, fungal 176
Neurofibromatosis 683, 685 drusen 337, 338f infective lesions 170
of orbit 271 edema 325f inflammation, idiopathic 253f
Neurogenic tumors 271 hypoplastic 332 leiomyoma 215
Neuromyelitis optica 347, 352f, 671f nerve 22f, 23, 25f, 26f, 34f, 348, 404f lymphoma 230
Neurovascular diseases in avulsion 389 metastases 307
ophthalmology 713 coloboma 327, 327f portion 609
Nevoxanthoendothelioma 246 compression 306f pseudotumor 183
Nodular heterotopia 106f congenital 326 roof fractures 312
Nonspecific inflammation, chronic 253f contusion 395f sarcoidosis 191
Nontraumatic subarachnoid disorders 321 schwannoma 273
hemorrhage 510 dysfunction 323 teratoma 163
Nystagmus 640 glioma 376, 689f trauma 310
types of 640 head 31f tuberculosis 181
infiltration 364f tumors and paraorbital lesions,
intracranial 360f, 563f secondary 282
O meningocele 335, 336f vascular malformations 717
Obstructive hydrocephalus 344f, 572f metastases 386 Osteitis
Occipital sheath hematoma 391 deformans 298
gliosis, bilateral 477 transection 391 tuberculous 182f, 342f
infarct¸ right 474f tumors of 368 Osteoma 288
lobe lesions 471 neuritis 345, 346, 357 choroidal 95
Occlusive lesions of ICA and central neuropathy
retinal vein 719 HIV 359
radiation 396
P
Ocular
and orbital cysticercosis 53 radiations 467, 471 Paget’s disease 298, 299f
lesions 704 tract 404f, 467, 469 Palsy, fourth nerve 618
motility disorders 603 Optochiasmatic glioma 381, 412f, 413f Panophthalmitis 46, 48f
 Index 745

Papilledema 339 Posterior Reactive lymphoid

Parainfectious
optic neuropathy 359
Parenchyma, brain 491
Parenchymal
metastases, cerebral 577
Parietal
convexity meningioma 564f
hematoma, right 473f
meningioma 565f
Pathway, fourth nerve 603
Pediatric
malignant orbital tumors 217
neoplasms 63
cerebral artery 514, 533
infarct 515
communication artery 533
hyaloid detachment 112f
scleritis 49
staphyloma 45f
Postseptal cellulitis 173
Postviral demyelination 352f
Preseptal cellulitis 173
Primary
and secondary optic nerve
sheath meningioma 368
CNS
hyperplasia 227f, 228, 270f
Rectus muscle
inferior 25f, 33f
lateral 392f
Recurrent
adenoid cystic carcinoma 266f
cerebellopontine angle 560f
hemangiopericytoma 213f
Retinal
and choroidal detachments 109
astrocytoma 89, 90
detachment 78f, 109, 110f
Retinoblastoma 63

Percutaneous sclerotherapy leukemia 583 Retinopathy of prematurity 100

lymphoma 586, 588f Retrobulbar hemorrhage 392f
of orbital varix 733f
demyelination 659 Retrochiasmal
Perinatal hypoxic injury 477f
empty sella 440f lesions 469
Perineuritis 184f
intracranial hemorrhage 508 visual pathway 465, 467
Periventricular leukomalacia 475
Progressive Rhabdomyosarcoma 217, 220f, 290, 291f
Persistent hyperplastic primary
external ophthalmoplegia, Right
vitreous 97, 99f
chronic 628 cerebellar atrophy, diffuse 631f
Petrous apex meningioma 560f
enhancing lesions 488
Phthisis bulbi 117, 118f multifocal
homonymous hemianopia 472f
Pie-in-sky appearance, left 473f leukoencephalopathy 498, 673f
occipital infarct 474f
Pineal gland tumors 571 supranuclear palsy 635f
parietal hematoma 473f
Pinealoblastoma 572f Proptosis, optic 166f, 305f
temporo-occipital infarct 473f
Pinealoma 571 Protocols, orbit 157
tentorial meningioma 619f
Pituitary Pseudotumor
Roof fractures, orbital 312
abscess 462 lacrimal 186f
Rounded nodular lesions, multiple 581f
adenoma 404f, 429 of lacrimal gland 251
apoplexy 446 orbital 183
fossa 567f Putaminal necrosis, optic 677f S
gland 438
Sagittal sinus thrombosis, superior 341f
Plagiocephaly 167
Q Sarcoid
Plasmacytoma 240
meningitis 358f
Pleomorphic adenoma 259, 260f, 261f Quadruple sectoranopia, left 473
scleritis 357
Plexiform
Sarcoidosis 191, 254, 353, 255f, 357f
neurofibromatosis 272f, 686f orbital 191
Pneumatocele 283f
R
Scanning techniques, types of 3
Pneumosinus dilatans 300 Radiation Schwannoma
Polyarteritis nodosa 530 necrosis 66f, 598f acoustic 575, 685
Portion optic 467, 471 cystic 275f
orbital 609 neuropathy 396 malignant 275f
sinus 609 Rathke’s cleft cyst 444, 445f orbital 273
746 Sankara Nethralaya Atlas of Imaging in Ophthalmology
Sclera calcification, optic 116f
Scleral buckle 131
Scleritis 49, 184f
posterior 49
sarcoid 357
Sclerosing pseudotumor 186f
Sclerosis, multiple 346, 659f
Secondary
demyelination 672
optic nerve sheath
meningioma, primary and 368
orbital tumors and
paraorbital lesions 282
Sellar and chiasmal lymphoma 458
Septo-optic dysplasia 332, 334f
Sheath hematoma, optic nerve 391
Simulating retinoblastoma 96
Sinonasal lymphoma 290
Sinus
mucocele 282
thrombosis, venous 519
Sinusitis
fungal 364f
with optic neuritis 359
Sjögren’s syndrome 193, 194f
Skull and dural metastases 577
Slipped and lost muscles 131
Small foramen magnum 484f
Solitary fibrous tumor 280, 281f
Source of primary tumors 386
Sphenoid
encephalocele 329f
mucocele 285f
Spherical implants 133
Spin-echo pulse sequence 13
Spin-lattice relaxation time 12f
Squamous
cell carcinoma 291f
temporal bone 393f
Staging of orbital cellulitis 170
Staphyloma 44
posterior 45f
Stroke 508
Sturge-Weber syndrome 690, 691f, 692f Thyroid
Subacute eye disease 302
hematoma 624f ophthalmopathy 302
hemorrhage 525 Time-of-flight angiogram 13
infarct, early 510 Torch infection 116f
Subarachnoid portion 609 Toxic demyelination 672
Subluxation of globe 392f Tract, optic 404f, 467, 469
Subperiosteal Transection, optic nerve 391
abscess 173 Trauma
hematoma 320f ocular 119
Subretinal hyperdense fluid 120f orbital 310
Superficial lymphangioma 205f Traumatic optic neuropathy 314, 389
Superior Tuberculoma 494
ophthalmic vein 173 choroidal 62f
orbital fissure 606f multiple 496f
sagittal sinus thrombosis 341f Tuberculosis 257f
Supraclinoid internal of lacrimal gland 256, 258f
carotid arteries 406f, 563 orbital 181
Supranuclear palsy, progressive 635f Tuberculous
Symmetrical enlargement meningitis 343f, 366f, 452, 497f
of lacrimal glands, optic 257f osteitis 182f, 342f
Syndrome, morning glory 326, 329f Tuberculum sella meningioma 438f, 567f
System, lacrimal 23 Tuberous sclerosis 694
Systemic sarcoidosis 355 Tumors of
choroid plexus 557
intracranial 545
T lacrimal gland 248
Temporal optic nerve 368
arachnoid cyst 172f types of 571
bone, squamous 393f
quadrant, inferior 404f
U
Temporo-occipital infarct, right 473f
Tenon’s fasciitis 184f Upper cervical cord 649f
Uveal
Tension pneumo-orbitus 391
melanomas 74
Tentorial meningioma, right 619f
Teratoma, orbital 163 metastasis 84
Tetralogy of Fallot 521f
Thalamus, lesions of 642
Third
V
cranial nerve pathway 603 Vascular
nerve palsy 609 lesions of orbit 197
Thrombosis malformations 717
of basilar artery 623 intracranial 536
sinus 173 orbital 717

Vein occlusion, cerebral 510
Veins 23
Venolymphatic malformation 206f
Venous
infarction 519
lymphatic malformations 204
sinus thrombosis 519
Vertical gaze palsy 634
Visual pathway, retrochiasmal 465, 467
Index 747
Vitreous hemorrhage 124f WHO classification of intracranial
Vogt-Koyanagi-Harada syndrome 58 tumors 545
von Hippel-Lindau disease 701 Wyburn-Mason syndrome 706, 709f
W Z
Walker-Warburg syndrome 105 Zygoma fractures 318f
Wegener’s granulomatosis 188, 189f, 190 Zygomatic complex
Wernicke’s encephalopathy 678, 679f fracture 312, 319