Retinal capillary hemangioma in a pthisical globe: Late sequelae in a case of Von Hippel-Lindau (VHL) disease presenting with endophthalmitis.

Abstract

Introduction: Retinal capillary hemangioma (RCH) is a benign vascular hamartoma that can occur sporadically or as a manifestation of Von Hippel-Lindau (VHL) disease. If left untreated, it results in adverse ocular complications depending on its location and eventual visual loss.

Case presentation: We present a 50-year-old man who was a known case of VHL with history of left eye vision loss in the left eye at the age of 30 years. He underwent resection of a brain hemangioblastoma 10 years earlier, which was complicated by left facial nerve palsy. He presented with acute left eye pain, redness, and eyelid swelling. He had large corneal infiltrate with hypopyon and obscured fundus view. He was treated medically as a case of left endophthalmitis with no improvement. The eye was eventually eviscerated. Histopathological examination revealed acute necrotizing keratitis, osseous metaplasia, and long-standing RCH.

Discussion: Peripheral RCHs are the most encountered ocular vascular lesion in VHL disease. Other locations close to the optic nerve (juxtapapillary) result in complicated visual loss. The RCH in our VHL case was confirmed 20 years after the history of vision loss in the same eye. The patient also had confirmed surgically treated intracranial hemangioblastoma but didn't seek any ophthalmic care prior to his recent presentation with painful acute keratitis and endophthalmitis.

Conclusion: VHL has multiple organ involvement. Patients suspected or diagnosed with VHL should receive prompt health care counselling to ensure periodic eye examination for control of any intraocular vascular lesions to prevent visual loss.