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Ophthalmological signs as a first clue to an atypical von-Hippel-Lindau presentation

Poster Details

First Author: P.N.Beirão Cardoso Quadrado Gil PORTUGAL

Co Author(s):    E. Costa   J. Pires   R. Matos   M. Mariano         0   0 0   0 0   0 0   0 0

Abstract Details


To report a case of von Hippel-Lindau (VHL) disease with atypical findings.


Ophthalmology Clinic, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal


Case-report of a 79-years old patient with no known personal or familiar history of VHL disease, whose diagnosis was suspected and later established due to the ocular manifestations. Complete ophthalmological examination was performed. Spectral Domain Optic Coherence Tomography was obtained using Cirrus™ HD-OCT Spectral Domain Technology (Zeiss®). Fluorescein angiography and fundus photographs were obtained using Zeiss FF 450 Plus (Carl Zeiss Meditec AG®). Complete screening for further VHL disease lesions comprised a contrast-enhanced computed tomography scan of the brain and spinal cord, urine cathecolamines measurement and sonography and computed tomography scan for abdominal lesions.


A 79-year old female patient presented to the emergency room complaining of sudden, painless loss of vision on the left eye. Best-corrected visual acuity was 0.4 on the right eye and hand movements on the left. In the right eye, a retinal capillary hemangioma was identified in the mid-periphery of the temporal retina, surrounded by hard exudates that involved the macular area. On the left eye, a dense hemovitreous prevented fundus visualization. Three weeks later, a large retinal capillary hemangioma was identified on the left eye, nasal and inferior to the optic disc, with an hemorrhagic component in reabsorption. Both personal and familial history of any typical lesions of the VHL syndrome was negative. On complementary systemic investigation, multiple cysts on the right kidney were identified, with negative brain and spinal CT-scan. Through systematic screening of asymptomatic 1st-degree relatives, one at-risk patient (daughter) was diagnosed with a left renal angiomyolipoma.


We present a rare atypical case of VHL disease in terms of epidemiology and clinical presentation. Age at diagnosis is usually between the 2nd and 3rd decades. To our knowledge this is the oldest newly-diagnosed VHL patient ever published. Also, hemovitreous is not only an uncommon complication of retinal capillary hemangiomas, it is very rarely the first presentation of the VHL syndrome. This case highlights the importance of a multi-systemic approach to ocular diseases and includes VHL disease as a rare etiology in the differential diagnosis of both progressive vision loss (due to exudation) and sudden vision loss (due to hemovitreous), even in older patients with no personal or familial history of VHL disease.

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