Treatment of Capillary Hemangioblastoma in Von Hippel-Lindau Disease: Case Report and Online Survey of Treatment Preferences among Experts

Abstract

This case report aims to describe the diagnosis, treatment, and evolution of bilateral, asymmetrical retinal capillary hemangioblastomas treated with argon laser and intravitreal anti-vascular endothelial growth factor and also reports the results of an online survey of treatment preferences among retina and vitreous specialists. A previously healthy 23-year-old female presented to our Retina Department complaining of progressive visual loss in her right eye. Visual acuity at admission was 20/300 in her right eye and 20/20 in her left eye. Anterior segment findings were unremarkable and fundoscopy revealed the presence of retinal capillary hemangioblastomas in both eyes. In the right eye, the hemangioblastoma was associated with pronounced exudation and macular edema; in the left eye, the lesion was quiescent. After a complete anamnesis and genetic counseling, Von Hippel-Lindau disease was diagnosed. Treatment with laser photocoagulation was performed on both eyes. One dose of 0.5 mg intravitreal ranibizumab was applied to the right eye. Two months after treatment, the right eye demonstrated improved visual acuity (20/100). Moreover, an important decrease in tumor dimensions and a reduction of vessel tortuosity was seen in both eyes. At 18 months of follow-up, the patient maintains a good visual acuity without recurrence of the treated tumors. Laser treatment should be considered as the primary treatment option for patients with capillary hemangioblastomas with and without exudation and can be combined with intravitreal antiangiogenics if exudation is significant. Inactive smaller lesions without exudation are likely to have an excellent response to laser treatment alone. Management should be individualized since no consensus between experts has been reached.