The diagnosis in this issue is cavernous hemangioma of the retina. The patient was referred to a retinologist who confirmed the diagnosis. Neuroimaging found no other lesions. No retinal treatment or change of living routine was required. Follow-up was set for every six months. Visual acuity has remained stable.
Cavernous hemangioma of the retina was first recognized in 1971 as a distinct retinal vascular hamartoma (a benign, disorganized overgrowth of tissue). Cavernous hemangioma is the most common benign orbital tumor of adults, and often presents as a retrobulbar tumor. The slowly progressive tumor resembles a cluster of grapes, and is composed of clusters of thin-walled saccular aneurysms that may be encapsulated by a white or gray fibroglial membrane.1-7
Cavernous hemangiomas usually appear in the second to fourth decade of life, are typically unilateral and are more prevalent in females than males by a 3-2 ratio.5 Retinal lesions are typically 1-2 disc diameters in size, isolated, and widely distributed across the fundus. They can be found anywhere, but are known to follow the course of veins.5 Their growth can be accelerated by pregnancy.
Histopathologic study of cavernous hemangioma indicates that its fibroglial membrane is attached to the internal limiting membrane and is connected with the inner retinal surface. The presence of glial protein, both in the gray membrane and the inner retina, is evidence that the membrane is of glial origin.2,3,5
Retinal cavernous hemangiomas are classified as part of the phacomatoses, a group of lesions characterized by the presence of hamartomas in the skin, eye, central nervous system and viscera.3-6 Although the appearance of cavernous hemangioma is classicoftentimes found on the eyelid as an isolated, light pink, well-circumscribed lesionyou must be able to distinguish it from the other phacomatoses. Various degrees of retinal telangiectasias may be seen in Coats disease and Lebers miliary aneurysms. Since many patients are asymptomatic, the pathognomonic characteristic of cavernous hemangioma is its appearance. It can cause painless, progressive unilateral axial proptosis over 3-5 years.
Visual acuity is affected in cases where the macula is involved or in cases that exhibit spontaneous intravitreal bleeding.16 A visual field defect may arise if the tumor is near the optic nerve head, resulting in an enlarged blind spot.2-5 Other clinical findings may include strabismus, chorioretinal striae/folds, increased IOP, and/or optic atrophy.
Diagnosing cavernous hemangioma requires ruling out involvement of the central nervous system.16 Refer patients for computed tomography and MRI. Most cases of cavernous hemangiomas do not require treatment, but asymptomatic patients should be followed every 6-12 months.1-6
Excision along with vitrectomy is indicated: when vitreous separation or tractional vitreous hemorrhage occurs; when diplopia, proptosis and/or optic nerve compression is present; or if visual function is compromised. Ultrasonography can help you diagnose vitreous separation and the encapsulated tumor.1-6 Other possible treatments may include cryotherapy, laser therapy or diathermy.
Since cavernous hemangioma tends to be familial, family members should be examined, too.16 Family members who show signs of neurological involvement such as seizures, palsies or paresis should be referred for CT scan and MRI. Dilated fundus examinations should be performed biannually on all family members to rule out genetic penetration.3-6
Although this patient simply needed regular monitoring, her condition reminds us that even the so-called routine exam is not always routine. It also reminds us that a dilated fundus exam, even on asymptomatic patients, is necessary to diagnose any underlying problems that may eventually affect the patients vision or have systemic ramifications. And, with conditions such as cavernous hemangioma of the retina, an entire family may be affected. Prompt diagnosis and referral for appropriate testing helps reinforce our role as primary eye care providers.
David Styn, O.D. and Andres Lambaria, O.D., contributed to this case.
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