Salvage External Beam Radiotherapy of Retinal Capillary Hemangiomas Secondary to von Hippel-Lindau Disease: Visual and Anatomic Outcomes

Objective: To examine the visual and anatomic outcomes of patients with retinal capillary hemangiomas secondary to von Hippel-Lindau (VHL) disease treated with globe-salvaging fractionated external beam radiotherapy (EBRT) after progression of hemangiomas despite initial therapy. Design: Retrospective noncomparative consecutive case series. Participants: Review of medical records of 5 patients with retinal capillary hemangiomas secondary to VHL disease. Intervention: External beam radiotherapy delivered to a total dose of 2160 cGy given over 12 daily fractions. Main Outcome Measures: Data on visual acuity and tumor size were collected at baseline, 1, 3, 6, and 12 months after EBRT, and at final follow-up, using echography, fundus photographs, and clinical examination. Results: Six eyes of 5 patients were treated. Mean follow-up was 28.3 months (range, 6-51). Mean baseline visual acuity was 20/70. Mean visual acuity at last follow-up was 20/45 (range, 20/25-20/60). Tumor volume reduction was 27.5 mm³ (range, 0.9-109.6 mm³). Mean percentage reduction in tumor volume was 39.6% (range, 3.3%-71.1%). Conclusions: External beam radiotherapy is a useful option in the treatment of retinal hemangiomas secondary to VHL disease that progress despite standard therapy. External beam radiotherapy leads to improvement in visual acuity, reduction in tumor volume, and stabilization of retinal detachment in most patients treated.