A Review of Cavernous Hemangioma of the Retina and Other Vascular Tumors of the Retina and Choroid

Abstract

Vascular tumors of the retina and choroid may be found in isolation or in association with syndromes involving cutaneous and central nervous system lesions. Ophthalmic lesions may be sight-threatening, and may be the presenting sign of systemic disease with life-threatening consequences. The role of the eye care practitioner in establishing a prompt and accurate diagnosis cannot be understated. INTRODUCTION Cavernous hemangioma of the retina is a relatively rare benign vascular tumor typically found unilaterally in Caucasian patients. Its ophthalmoscopic appearance is quite unique: multiple intraretinal aneurysms filled with venous blood resembling a cluster of grapes projecting above the retinal surface. Intravenous fluorescein angiography demonstrates a pathognomonic pattern of early hypofluorescence and late hyperfluorescence, but no leakage, as fluorescein layers above stagnant red blood cells in each individual aneurysm. As the lesion tends to be stable, regular observation is the rule, unless its location, vitreous hemorrhage, or retinal traction compromise vision. Differential diagnoses include other vascular tumors or anomalies of the retina or choroid: arteriovenous malformations, Coats’ disease, capillary hemangiomas, vasoproliferative tumors of the retina, and choroidal hemangiomas. Whether sporadic or part of a syndrome, ophthalmic lesions may be sight-threatening. Associated cutaneous and central nervous system lesions may be life-threatening, making prompt and accurate identification critical. Given that presenting signs and symptoms are often visual, this responsibility may fall upon the eye care practitioner. VASCULAR TUMORS OF THE RETINA AND CHOROID Cavernous Hemangioma of the Retina This is a rare vascular hamartoma, a benign disorganized overgrowth of otherwise normal tissue, typically found unilaterally, primarily in Caucasians. The average age of onset is the early to mid-twenties, with a slight (60:40) predilection for females. These lesions, while most often sporadic, have been classified as part of the phakomatoses, congenital multisystem disorders characterized by the presence of hamartomas in the eye, skin, visceral organs, and central nervous system (CNS). Defects in the tumor suppression gene resulting in uncontrolled cell growth have been implicated in the formation of these (and other) hamartomas. The most common symptoms in patients with CNS lesions include headaches, and seizures and focal neurologic deficits resulting from hemorrhagic stroke and brainstem abnormalities. Ophthalmic cavernous hemangiomas have been reported, albeit infrequently, in isolated cases of blue rubber bleb nevus syndrome (BRBNS, previously known as Gascoyen’s syndrome). Lesions of the skin and gastrointestinal tract, causing hemorrhage and anemia typically beginning in infancy, characterize this autosomal dominant (AD) condition, which has been reported in the literature fewer than 200 times. CNS involvement is rare. Differential diagnoses include other conditions associated with vascular skin lesions, including Sturge-Weber syndrome and von Hippel-Lindau disease. The retinal lesions (Fig. 1) are typically sessile clusters of saccular thin-walled aneurysms within the inner retina filled with dark red venous blood. Their appearance has been likened to that of a bunch of grapes, and they may be found at any location of the retina, including the optic nerve head (ONH). Lesions vary in size, from several disc diameters to those involving multiple quadrants of the fundus. There are no prominent vessels feeding or draining the lesion. Growth is rare, CE Credit Article Clinical & Refractive Optometry is pleased to present this continuing education (CE) article by Dr. Derek MacDonald entitled A Review of Cavernous Hemangioma of the Retina and Other Vascular Tumors of the Retina and Choroid. In order to obtain a 1-hour Council of Optometric Practitioner Education (COPE) approved CE credit, please refer to page __ for complete instructions. D. MacDonald — D. & K. MacDonald & Associates, Waterloo, ON Correspondence to: Dr. Derek MacDonald, D. & K. MacDonald & Associates, 354 King Street North, Waterloo, ON N2J 2Z2; E-mail: derek.macdonald@rogers.com This article has been peer-reviewed.

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Cite this paper

@inproceedings{MacDonald2013ARO, title={A Review of Cavernous Hemangioma of the Retina and Other Vascular Tumors of the Retina and Choroid}, author={Derek MacDonald}, year={2013} }