Vascular lesions of the orbit: A pictorial review

Poster No.: C-503

Congress: ECR 2009
Type: Educational Exhibit
Topic: Head and Neck
Authors: C. L. Fernandez Rey, A. Saiz Ayala, E. Santamarta Liebana, E.
Montes Perez, P. Sanchez Lopez, I. Noval Tuñón; Oviedo/ES
Keywords: angiography, orbit, vascular orbit, angiography MR
DOI: 10.1594/ecr2009/C-503

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Learning objectives

• To illustrate the spectrum of vascular lesions that can involve the orbit.
• To discuss and emphasize the role of MR angiography.

Background

Multiple vascular lesions can affect the orbit including neoplasms, malformations and
shunts. These lesions can manifest with proptosis or diplopia. Imaging differentiation
isessential because the management and prognosis vary greatly. MR angiography
provides useful information in the preoperative evaluation of these lesions.

Imaging findings OR Procedure details

Fig.

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The spectrum of vascular lesions that can involve the orbit include hemangiomas,
cavernous malformations, orbital varices, venous lymphatic malformations,
hemangiopericytomas, hemangioblastomas, vascular metastases, etc. They constitute
the most common group of pathologic conditions of the orbit.

We have made a pictorial review showing the more significant cases and describe the
specific imaging features that can help us in their diagnosis

THE WIDE SPECTRUM OF VASCULAR ORBITARY LESIONS:

Fig.

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Fig.

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Fig.

A/ VENOUS VASCULAR MALFORMATIONS:

In this group we include purely venous lesions and cavernous malformations

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Fig.

ORBITAL VARICES: True orbital varices are congenital venous vascular malformations
that we must distinguish from secondary enlarged veins resulting from arteriovenous
shunts or venous thrombosis. The cardinal clinical feature of true varices is intermitent
proptosis, but occasionally they may cause orbital hemorrhage (spontaneous or
posttraumatic) manifesting as sudden proptosis. Orbital varices usually communicate
with the venous system, therefore an enlargement of these varicous veins results during
maneuvers that increase venous pressure (Valsalva maneuver or prone position)

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Fig.

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Fig.

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Fig.

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Fig.

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Fig.

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Fig.

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Fig.

Key concepts about orbital varices:

• They are the most common cause of spontaneous orbital hemorhage

• Intraconal / extraconal location
• Phleboliths are frequent
• Intermitent proptosis is the major clinical symptom
• You must include images during Valsalva maneuver or prone position in
your studies
• You must rule out thrombosis

CAVERNOUS HEMANGIOMA: Encapsulated venous malformation that is the most

common orbital lesion in adults. The most frequent location is the intraconal space and
the principal symptom is progressive painless proptosis

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Fig.

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Fig.

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Fig.

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Fig.

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Fig.

Key concepts about cavernous hemangiomas:

• They are the most common orbital vascular lesions in adults

• They are low-flow venous or arterio-venous malformations
• Intraconal space is the most frequent location
• Occasionally they present phleboliths
• High intensity sygnal on T2WI and progressive enhancement at multiphase
dynamic CECT are characteristic
• May produce expansion of the orbital walls and optic nerve displacement
• You must distinguish cavernous hemangioma from capillary hemangioma
and hemangiopericytoma

B/ VENOUS-LYMPHATIC MALFORMATIONS:

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Fig.

LYMPHANGIOMAS: Uncommon hamartomatous lymphatic and venous malformations

that preferently affect children.

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Fig.

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Fig.

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Fig.

Key concepts about lymphangiomas:

• They are no flow or low-flow vascular malformations

• May be superficial (conjunctiva) or deep (intraconal)
• Frequently trans-spatial location
• Fluid-fluid levels or hemorrhagic content is more suggestive
• The principal symptom is progressive painless proptosis

C/ VASCULAR NEOPLASMS:

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Fig.

CAPILLARY HEMANGIOMAS: They are the most frequent orbital vascular tumors in
children.

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Fig.

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Fig.

Again we must distinguish between two vascular disorders: capillary hemangioma

and cavernomatous hemangioma. The first one is a true neoplasm that consists
in prolipherating endothelial cells tumor that frequently affects the preseptal space.
Cavernous hemangioma is a common vascular malformation in adults and are most
commonly located in the intraconal space.

Key concepts about capillary hemangiomas:

• They are the most common benign neoplasm in children

• Preseptal and extraconal spaces are the most frequent locations
• Flow voids are very suggestive
• Involution
• Not pseudocapsule
• High sygnal intensity on T2WI
• Prominent tumor blush on angiography
• You must distinguish from cavernous hemangiomas

HEMANGIOPERICYTOMAS: Highly vascular neoplasms derived from pericytes of

Zimmerman. They show lobulated and encapsulated appearance like cavernous

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hemangiomas, but usually have infiltrative and irregular margins with adjacent bone
erosion.

Fig.

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Fig.

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Fig.

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Fig.

Key concepts about hemangiopericytomas:

• Potential malignant tumor

• Rare in the orbit
• Important tumor blush in angiography
• You must suspect hemangiopericytoma when margins are irregular and
infiltrative

VASCULAR METASTASES: The most common origin tumors are breast and lung in
adults and retinoblastoma and neuroblastoma in chidhood, but typical hypervascular
metastases are secondary to thyroid, renal and melanoma neoplasms

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Fig.

D/ ARTERIAL AND ARTERIO-VENOUS LESIONS:

Angiography studies are required in these type of vascular conditions for precise location
and treatment planning.

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Fig.

ANEURYSM OF THE OPHTHALMIC ARTERY: Ophthalmic artery aneurysms are rare

and they sometimes extend through the optic canal.

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Fig.

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Fig.

ARTERIOVENOUS MALFORMATIONS: Abnormal shunts between arteries and veins

without an intervening capillary bed. Orbitary and facial involvement is uncommon.

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Fig.

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Fig.

CAROTID-CAVERNOUS FISTULA: Abnormal communication between cavernous sinus

and artery branches from external-internal carotid. May be spontaneous or secondary
to trauma. Frequently it conduces to secondary orbital varices (in fact they are the most
common extraorbital shunt that produce changes within the orbit).

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Fig.

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Fig.

E/ VASCULAR ORBITAL LESIONS RELATED SYNDROMES:

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Fig.

STURGE WEBER SYNDROME: In this syndrome multiple and diffuse hemangiomas

may involve the orbit (choroid, ciliary body, iris, episclera, conjunctiva and limbus) and
maxillary area

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Fig.

KLIPPEL TRENAUNAY SYNDROME: Congenital disease with capillary-venous-

lymphatic malformations associated

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Fig.

WYBURN MASON SYNDROME: Congenital nonherediatry syndrome wich courses

with unilateral arteriovenous malformations involving midbrain and orbit. Vascular
malformations result from abnormal persistence of embryogenic vascular tissue. Facial
telangiectasias and deformities are often associated.

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Fig.

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Fig.

SINUS PERICRANII:Abnormal communication between intracranial venous sinus and

epicranial varicosies veins

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Fig.

F/ VASCULAR OSSEOUS CONDITIONS:

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Fig.

ANEURYSMATIC OSSEOUS CYST:

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Fig.

OSSEOUS HEMANGIOMA:

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Fig.

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Fig.

MR ANGIOGRAPHY: AN USEFUL TOOL IN THE STUDY OF VASCULAR

ORBITARY LESIONS

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Fig.

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Fig.

Conclusion

Imaging studies are essential for the evaluation of the vascular processes affecting the
orbit.

Location, age patient and characteristics on MR imaging are key facts that can help
narrow the differential diagnosis, thereby helping to guide patient treatment.

Personal Information

Cristina Fernandez Rey

Email address: cristinarey80@hotmail.com

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Department of Radiology, Hospital Universitario Central de Asturias, Oviedo, Spain

References

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and Jeffrey A. Nerad. Vascular Lesions of the Orbit: More than Meets the
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• W.S. Tan, A.C. Wilbur M.D., M. F. Mafee, The Role of the Neuroradiologist
in Vascular Disorders Involving the Orbit, The radiologic clinics of North
America, Imaging in ophtahalmology part II: , 849-861, July 1987
• Fordham LA, Chung CJ, Donnely LF, Imaging of congenital vascular and
lymphatic anomalies of the head and neck, Neuroimaging clinics of North
America, Imaging in ophthalmology 10:1: 117-136, 2000
• Harnsberger, Wiggins, Hudgins, Michel, Swartz, Davidson, Macdonal;
Glastonbury, Curé, Branstetter. Diagnostic Imaging. Head and Neck.
Amyrsis 2005

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