Open AccessRetinal Hemangioblastoma with Extraocular Extension: Report of Three Cases
Author(s) -
Namita Kumari,
Sima Das,
Anirban Bhaduri,
Arpan Gandhi
Publication year - 2021
Publication title -
ocular oncology and pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.444
H-Index - 10
eISSN - 2296-4681
pISSN - 2296-4657
DOI - 10.1159/000512173
Subject(s) - medicine , cryotherapy , retinal detachment , enucleation , surgery , von hippel–lindau disease , lesion , retinal , ophthalmology , disease , pathology
Retinal hemangioblastoma (RH) is the earliest and most common clinical manifestation in Von Hippel-Lindau (VHL) disease. RH can also present in isolation without any evidence of VHL. Clinical course of RH can be stationary or progress to exudation and chronic retinal detachment requiring surgical intervention. We report 3 cases of aggressive RH with extraocular extension in young males causing painful blind eye requiring enucleation. Two of our cases were bilateral involvement and had systemic manifestations of VHL. The third patient had unilateral involvement with no systemic evidence of VHL. This manifestation of RH is rarely reported. Two of our patients with VHL had early manifestations of RH and had undergone multiple cryotherapy sessions as well as retinal detachment surgery for exudative retinal detachment. This differential should be considered in vascular lesion arising from intraocular structures especially in diagnosed patients of VHL. The cases also highlight the aggressive behaviour and long-term progression of RH in some patients despite early treatment.