Title
RETINAL CAPILLARY HEMANGIOBLASTOMA: CASE REPORT
Introduction
Retinal capillary hemangioblastomas (RCH) (also known as retinal angiomas) can be a a sign of a von Hippel-Lindau (VHL) disease although they may also be observed as an isolated entity without systemic involvement. RCH is the most frequent and earliest manifestation of the VHL disease and therefore an ophthalmologist is often involved in the care of these patients.
Methods
A 29-YO male was referred with incidental low vision in the OS for 3 months. His vision was 20/25 OD, 20/1000 OS. His fundoscopic examination revealed a reddish, circular mass in the mid-periphery with large feeder vessels, ample retinal and macular oedema and large exsudation in the OS. His OCT showed a significant Cystoid Macular Oedema and the Angiogram revealed capillary drop-outs in the general retina and intense leakage in the tumor site.
Results
Patient, to date, has not yet returned for laser photocoagulation and Intravitreal injection treatment.
Discussion
Retinal capillary Hemangioblastomas (RCH) are highly vascularized tumors that typically present as a red, round, circumscribed mass, more commonly in the midperiphery.1,2 When peripheral, they are often in the superotemporal or inferotemporal quadrants and can easily be found by following their feeder vessel extending from the optic nerve.1 The majority of RCHs are solitary (66%) but they can also present as multiple lesions (33%). 2-4 Most RCHs are asymptomatic and are, therefore, commonly diagnosed incidentally between the ages of one and 40 years, with a mean age of 25.1-3 However, their secondary effects may elicit symptoms. Secondary complications include intraretinal and subretinal exudation in the vicinity of the hemangioma, uncommonly a macular star (a circular distribution of exudates around the macula) and rarely retinal or vitreous hemorrhage (less than 3%).3 Advanced cases may present with tractional or exudative retinal detachment.1,3,4 Symptoms in these instances may include loss of vision, flashes, floaters, photopsias and metamorphopsias.1-3
Keywords
Retinal Capillary Hemangioma, Hemangioblastoma, Von Hippel Lindeau, Retinal tumor,
Area
CLINICAL CASE
Authors
Weider Oliveira Silva, Paulo Kin Takara, Nathale Valente Santiago, Akyioshi Oshima