HYDROCEPHALUS

Dr.B.Prakash. MD.DM.,
Prof of Neurology
PSG Hospitals, CBE

INTRODUCTION

HYDROCEPHALUS
From Greek hydrokephalos, from hydr +
kephale
Definition:
An abnormal increase in the amount of cerebrospinal
fluid within the cranial cavity, accompanied by
expansion of the cerebral ventricles, enlargement of
the skull and atrophy of the brain

HYDROCEPHALUS
Most Hydrocephalus are diagnosed during neonatal
period
Overall prevalence : 3-4 per 1000
With treatment not lethal, but not curable
Lifelong medical surveillance
Unpredictable surgical maintenance

HYDROCEPHALUS
Resulting from disturbances in the dynamics of cerebrospinal
fluid (CSF), which may be caused by several diseases.

Choroid Epithelium

CSF Flow path

INCIDENCE
Occurs in 3-4 of every 1000 births
Causes
Congenital:

Mal-development, Intrauterine Infection

Acquired:

Infection, Neoplasm, Hemorrhage

PHYSIOLOGY
CSF is formed by two mechanisms:
Secretion by the choroid plexus
Lymphatic-like drainage by the extracellular fluid in
the brain
CSF circulates thru ventricular system and is absorbed
within subarachnoid spaces by unknown mechanism

OVERVIEW OF CSF
PRODUCTION
The CSF volume of an
average adult is 80 to 160
ml
The ventricular system
holds approximately 20 to
50 ml of CSF
CSF is produced in the
choroid plexuses
Rate of CSF formation 15-35
ml/hr

CSF PRODUCTION
The choroid plexuses are the
source of approximately 80% of
the CSF

The blood vessels in the subependymal regions, and pia also

contribute to the formation of
CSF

CSF
CIRCULATIO
N

Lateral ventricles Foramen of Monro

3rd Ventricle Cerebral Acqueduct

4th Ventricle F. of Magendie & Luschka

Perimedullary and Perispinal subarachnoid spaces upward to the

basal cistern

Superior and lateral surfaces of the cerebral hemispheres

CSF CIRCULATION
The pressure gradient is highest in the
lateral ventricles and diminishes
successively along the subarachnoid
space

Arterial pulsations in the choroid plexuses

drive the fluid from the ventricular system

Peri-ventricular tissues offer minimal

resistance to the flow of CSF

CSF PRESSURE
The CSF volume and
pressure are maintained
every minute by the
systemic circulation

Hypoventilation
in blood PCO2
pH & arteriolar
resistance
cerebral blood flow
CSF pressure

CSF pressure is in
equilibrium with capillary
pressure (arteriolar tone)

Hyperventilation has the

opposite effect

CSF PRESSURE
Normal adult intracranial
pressure 2-8 mmHg

Up to 16 mmHg are considered

normal

ICP higher than 40 mmHg or

lower BP may combine to cause
ischemic damage to the brain

CSF PRESSURE
Increased venous pressure has
a direct effect on CSF pressure

Block of venous flow increases

the volume of cerebral veins,
dural sinuses, and the
subarachnoid space

THE FUNCTION OF THE

CSF
The CSF acts as a water
jacket for the brain and
spinal cord

The 1300 g adult brain

weighs only 45 g when
suspended in CSF

THE FUNCTION OF THE

CSF
The CSF acts like a sink,
effectively flushing out the waste
products, as new fluid is secreted
& reabsorbed

A constant CSF electrolyte

composition helps maintain a,
stable medium for excitable cells
(neurons)

MECHANISMS OF INCREASE ICP

Brain, Blood and CSF are held
in an inelastic container
(cranium, vertebral canal and
dura)

Changes in volume of either

element (Brain, CSF, Blood) is
at the expense of the other two

MECHANISMS OF
FLUID IMBALANCE
HYDROCEPHALUS RESULTS
FROM:

Impaired absorption of CSF within

the subarachnoid space
(communicating hydrocephalus)

Obstruction to the flow of CSF

through the ventricular system (noncommunicating hydrocephalus)

COMMUNICATING VS NON-COMMUNICATING
This classification was based on the imaging findings after

injection of dye into the ventricular system and

simultaneous injection of air into the subarachnoid space

Diffusion of dye into the subarachnoid space and passage

of air into the ventricular space were the criteria for

communicating hydrocephalus

MECHANISMS OF FLUID
IMBALANCE

Both lead to increase accumulation of CSF in the ventricles

Ventricles become dilated and compress the brain.

If this happens before closure of cranial sutures- skull enlarges

In children <10-12, previously closed sutures may open.

NON-COMMUNICATING
HYDROCEPHALUS
There is no communication between the ventricular
system and the subarachnoid space

The commonest cause of this category is aqueduct

blockage or stenosis

CLINICAL FEATURES

INFANCY

Head grows at alarming rate with hydrocephalus.

First sign: Bulging pulsatile fontanels
Tense, non-pulsatile anterior fontanel
Dilated scalp veins, esp. when crying
Thin skull bones with separated sutures
Cracked pot sounds on percussion

INFANCY

Depressed eyes or setting-sun eyes

Eyes downward with sclera visible above

Pupils sluggish with unequal response to

light

Irritability, lethargy, feeds poorly,

Changes in Level of Consc

Arching of back (Opisthotonos)

Lower extremity spasticity

May cry when picked up or rocked

Quiets when allowed to lay still

INFANCY
Brain Stem Compression
Swallowing difficulties, Stridor, Apnea, Aspiration,
Respiratory difficulties and Arm weakness

Lower Brainstem Dysfunction

Difficulty Sucking and Feeding, High-pitched shrill cry

INFANCY
Emesis, Somnolence, Seizures, and Cardio Pulmonary
Distress

Severely affected infants may not survive neonatal period

CHILDHOOD
Features of
Increased ICP / Posterior Neoplasms / Aqueduct Stenosis / SOL

Headache on awakening, improvement following emesis or

sitting
Papilledema, strabismus, and Extrapyramidal signs, ataxia
Irritability, Lethargy, Apathy, Confusion, and often incoherent

SYMPTOMS AND SIGNS

Irritability
Poor feeding

Accelerated head

growth

Headache

Bulging fontanel

Nausea, vomiting

Forced down gaze

Diplopia,
Visual impairment
Dementia
Incontinence
Gait disturbances

Developmental delay
Exotropia
Papilledema
Posturing
Brady cardia
Apnea / Death

AQUEDUCTAL STENOSIS

AQUEDUCTAL STENOSIS
The normal aqueduct measures about 1 mm in
diameter, and is about 11 mm in length.

AQUEDUCTAL STENOSIS
Is the most common cause of congenital
hydrocephalus (43%)
Aqueduct develops about the 6th week of gestation
M:F = 2:1
Mortality : 11-30%

ETIOLOGY OF AQUEDUCTAL
STENOSIS Intrinsic Pathology

Septum Formation:
A membrane of neuroglia occludes
aqueduct
Occurs caudally
Due to 1 developmental defect /

Gliosis of the Aqueduct:

Infectious origin
Marked gliofibrillary response.
The lumen is devoid of
ependyma.

ependymitis
Rarest

Stenosis of the Aqueduct:

Forking of the Aqueduct:

Two channels in midsagittal plane
unable to handle CSF volume
Most often seen with spina bifida

Narrowed aqueduct without

evidence of gliosis.
This may have hereditary basis.

ETIOLOGY OF AQUEDUCTAL STENOSIS

Extrinsic Pathology

Infections
Abscesses

Neoplastic

Pineal tumors
Brainstem gliomas
Medulloblastoma
Ependymoma

Vascular
AVM
Aneurysm
Galen aneurysm

Developmental
Arachnoid cysts

CLINICAL FEATURES OF AQUEDUCTAL

STENOSIS
Obstructive hydrocephalus:
Macrocephaly and/or Intracranial
Hypertension.

Parinaud's syndrome:
Inability to elevate eyes

Collier's sign:
Retraction of the eyelids

IMAGING OF AQUEDUCTAL
STENOSIS
Ultrasonography can detect aqueductal stenosis in utero

IMAGING OF AQUEDUCTAL STENOSIS

CT and MRI. MRI is essential if third ventriculostomy
is to be considered.

TREATMENT OF AQUEDUCTAL
STENOSIS

Remove underlying cause of

obstruction

Third ventriculostomy as initial

treatment of choice.

VP shunt if technical reasons do

not allow third ventriculostomy
or ventriculostomy failure

Aqueductal stent can be placed

if technically feasible. Rarely
done due to risk of brain stem
injury

ARNOLD CHIARI
MALFORMATIONS

COMMON DEFECTS
Arnold-Chiari Malformation (ACM) malformation
Herniation of Cerebellum, Medulla, Pons, And Fourth Ventricle
Into the cervical spinal canal
Through an enlarged Foramen Magnum.

TYPES OF ACM
Type I
Extension of the Cerebellar tonsils into the foramen magnum

Type II (Classic)
Extension of cerebellum and Brainstem accompanied by
Myelomeningocele

Type III (Serious)

The cerebellum and brain stem protrude, or herniate, through the
foramen magnum and into the spinal cord. Part of the fourth ventricle
may also protrude through the hole

Type IV
Incomplete or underdeveloped cerebellum (Cerebellar Hypoplasia)

COMMUNICATING
HYDROCEPHALUS

CAUSES OF COMMUNICATING
HYDROCEPHALUS

Overproduction of CSF

Blockage of CSF circulation

Blockage of CSF resorption

Hydrocephalus ex-vacuo

Normal pressure
hydrocephalus

OVERPRODUCTION OF CSF

Excessive secretion of CSF by the choroid plexus

Choroid plexus papilloma
Choroid plexus carcinoma

BLOCKAGE OF CSF
CIRCULATION
Could be at any level of the CSF circulation.
At the level of the foramen of monro, unilateral / bilateral
Colloid cyst of the third ventricle
Tumors of the third ventricle.

DANDY WALKER
SYNDROME
Congenital blockage of foramina of Lushka and
Magendie

Associated with Agenesis of Cerebellar Vermis

Female predominance (3:1)

Bulging Occiput, Nystagmus, Ataxia, Cranial Nerve

palsies

No occlusion of ventricles

BLOCKAGE OF CSF
RESORPTION
Poor resorption of CSF into the venous sinuses
Scarring of the Arachnoid Villi
Post meningitis or hemorrhage

CVT

HYDROCEPHALUS EXVACUO
CSF pressure is normal

Shrinkage of brain substance

Damage to the brain
Stroke or other form of injury
Chronic neuro degeneration

RASMUSSEN'S
ENCEPHALITIS Prog
hemispheric atrophy
Unknown origin.
Increasing seizures
Progressive hemiplegia.
Smaller hemisphere is
abnormal
Lateral ventricle is larger in
smaller

NORMAL PRESSURE
HYDROCEPHALUS
Normal pressure hydrocephalus (NPH)
Due to a gradual blockage of the CSF
Unusual cause for dementia
Occur as a complication of infection or
hemorrhage

NORMAL PRESSURE
HYDROCEPHALUS

Ventricles enlarge
The CSF pressure is normal
NPH is characterized by

Gradual memory loss

(dementia)
Balance disorder (ataxia)
Urine incontinence
General slowing of activity.

Symptoms progressively
worsen
Over weeks

Improvement noted
immediately after the
removal of spinal fluid

TREATMENT OF
HYDROCEPHALUS

THERAPEUTIC
MANAGEMENT
Goals:
Relieve hydrocephaly
Treat complications
Manage psychomotor
problems
Usually surgical

VENTRICULO PERITONEAL
SHUNT
Shunt systems include three components:
Ventricular catheter
One way valve
Distal catheter

The ventricular catheter

Straight piece of tube
Closed on the proximal end
With multiple holes upto 2cm for the entry of CSF

SHUNT MATERIALS
Shunts are composed of Silastic material made
from silicone.

SHUNT MALFUNCTION
COMPLICATIONS OF VP
SHUNT
Shunt malfunction
Blockage
Infection

MALFUNCTION

BLOCKADE OR
INFECTION

Related to growth
Headache / Fever
Replace with longer catheter

Drowsiness / Convulsions
Increased head
circumference
Bulging fontanelle

TREATMENT OF
HYDROCEPHALUS
The VA shunt
Must be accurately located
Requires frequent revisions
Distal end position to be maintained
Infection may be more serious

VENTRICULO PLEURAL
SHUNT

If both the VPS & VAS do not function to absorb CSF the
shunt have to placed in the pleural space

POST-OP CARE
Observe for signs of Increased ICP
Assess pupil size
Blood pressure may be variable due to BS hypoxia
Abdominal distention
due to CSF peritonitis or post-op ileus due to catheter placement.

DRUGS
The choroid plexus shares many ion pumps and
enzyme systems with renal tubular epithelium
Acetazolamide (Diamox)
Furosemide (Lasix)

THANK YOU