Abstract

A 21-year-old female presented with one-week duration of blurred vision and floaters in her right eye (OD). Best corrected visual acuity (BCVA) in OD was 6/18 and left eye 6/6. Ocular examination revealed multiple retinal hemangioblastomas (RH) in both eyes (OU), along with an epiretinal membrane in OD and minimal vitreous haemorrhage, diagnostic of Von Hippel Lindau (VHL) disease. Initial treatment involved green laser photocoagulation to RH lesions in OU, after systemic screening for VHL. However, OD exhibited aggravated fibro-proliferative vitreo-retinopathy, vitreomacular traction on follow up, despite regression of RH lesions, with a drop in OD BCVA to 6/60. OD underwent pars plana vitrectomy, which resulted in an improvement of the vision to 6/12 at post operative 2 months. The case underscores the complex nature of ocular manifestations in VHL and highlights the critical importance of prompt intervention to preserve vision, with regular follow-up recommended for optimal care.