Fineman_Retina (Color Atlas and Synopsis of Clinical Ophthal

RETINAL CAPILLARY HEMANGIoblastOMA 259

■ Optic disc granuloma ■ Optic disc glioma Diagnostic Evaluation

location, size, and associated complications, especially those that involve the macula. ● In patients with VHL syndrome, the risk of developing associated tumors increases with age. Morbidity and mortality are related to these associated tumors. Median survival is less than 50 years, with renal carcinoma as the leading cause of death. ● Treatment is recommended for tumors with documented growth or effects on visual function. The goal of treatment is to induce resolution of exudation or subretinal fluid. Laser photocoagulation and anti-VEGF ther apy is reserved for smaller tumors ( < 2 mm in diameter). Larger tumors are best treated with cryotherapy or plaque radiotherapy. Photodynamic therapy and proton beam irradiation may be used in certain cases. Vitreoretinal surgery and, rarely, enucleation may be necessary for patients with advanced or uncontrollable pathology, such as retinal detachment. ● Patients with VHL syndrome and their relatives should be examined regularly for life. This includes regular ultrasonography of the kidneys and MRI of the brain and the spinal cord.

● Ocular: Fluorescein angiography is the most helpful ancillary study ( Fig. 6-6 ). In the arterial phase, a prominent, dilated feeder arteriole may be seen. The tumor appears hyperfluorescent early and remains so through the late phases, sometimes leaking dye into the vitreous. Ultrasonography may be helpful in diagnosing lesions of greater than 1 mm and demonstrates acoustic solidity throughout the lesion. ● Systemic: All patients with retinal capillary hemangioma, as well as relatives, should be evaluated for VHL syndrome. Genetic testing for mutations in the VHL gene is available. If genetic testing is not available or practical, MRI of the brain and spinal cord and CT of the chest and abdomen may be helpful to rule out associated tumors. Prognosis and Management ● The natural history is variable, with both progressive enlargement and spontaneous regression having been reported. Visual prog nosis is highly variable and depends on tumor

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