Title

MULTIPLE RETINAL HEMANGIOBLASTOMAS IN VON HIPPEL LINDAU DISEASE: CASE REPORT USING WIDE FIELD ANGIOGRAPHY

Summary

Purpose: to report a case of peripheral vascular abnormalities using a wide field retinal device Methods: case report Case Report: a 22-year-old, white, female, asymptomatic, who was referred by a neurologist for ophthalmic evaluation due to a systemic disease diagnosed 1 year ago; POx: unremarkable; PFx: 1 sister with the same condition; PMx: cerebellar hemangioblastoma Ophthalmological examination showed visual acuity of 20/20 on OU, normal anterior segment. Fundus examination, showed on OD dilated, tortuous vessels toward 3 hemangioblastomas, one superior nasal, the other peripheral nasal and the largest one in peripheral inferior retina with vitreous membranes. Left fundus, showed a large and tortuous vessel toward to an hemangioblastoma in superior retina. Wide field fluorescein angiography revealed the felling of the “arterial” vessel. OCT was normal in both eyes. An ultrasound was performed to measure hemangioblastomas: all, but the one at 6h on OD, measured less than 1.5 mm in height. The largest one measured 2.6 mm in height. Treatment was initiated with thermal laser photocoagulation in all less than 1.5 mm tumors and cryotherapy using the double freeze-thaw method for the largest one. Discussion: VHL is a hereditary disease and the sole presence of multiple retinal hemangioblastomas is enough to classify as a systemic disease. Tumors, if left untreated, will grow leading to severe tractional retinal detachments with a poor prognosis. Patient should be follow-up for life in search for ocular and systemic new hemangioblastomas.

Area

CLINICAL CASE

Authors

Hideki Barbosa Hirota, Ícaro Augusto Godinho, Patricia Bortolai, Arnaldo Furman Bordon