Vitreoretinal Surgery for Severe Retinal Capillary Hemangiomas in Von Hippel-Lindau Disease

Purpose: To evaluate the long-term success rate of vitreoretinal surgery for severe cases of retinal capillary hemangiomas (RCHs) caused by von Hippel-Lindau (VHL) disease. Design: Retrospective case series. Participants: Twenty-three eyes of 21 patients with severe exudative or tractional retinal detachment caused by RCH, who had undergone vitreoretinal surgery. Patients' age at initial surgery ranged from 12 to 47 years (median, 27 years), and the fellow eye was already blind in 6 of 21 patients. Interventions: All eyes underwent pars plana vitrectomy with posterior hyaloid detachment epiretinal membrane dissection and silicone oil or gas injection. In 9 eyes, retinectomy was performed to remove the RCH (group R). In the other 14 eyes, the RCH was treated by laser endophotocoagulation alone or combined with transscleral cryotherapy (group L). Main Outcome Measures: Visual acuity (preoperative, 6 and 18 months postoperatively), rate of RCH recurrence, and postoperative complications. Results: In group R, an average of 2 operations per patient was needed. Six months after surgery, the retina was flat in 8 eyes. Mean follow-up was 8 years. Long-term complications included RCH reproliferation and neovascular glaucoma in 4 eyes, 4 to 8 years after initial surgery. In the remaining 5 eyes, visual acuity ranged from 20/320 to counting fingers 18 months postoperatively. In group L, an average of 1.7 operations was needed. Six months after surgery, the retina was flat in 13 of 14 eyes. Mean follow-up was 4 years. New RCH occurred in 10 eyes and required laser treatment. In the long term, 1 eye became blind after 5 years because of aggressive RCH reproliferation and neovascular glaucoma, and 2 eyes became blind after 10 years because of exudative retinal detachment. In the 11 remaining eyes, visual acuity ranged from 20/320 to 20/20 (median: 20/50) 18 months postoperatively. Conclusions: Vitreoretinal surgery is an effective treatment for severe VHL retinal hemangiomas. Large RCHs were satisfactorily treated by (1) vitrectomy with epiretinal dissection and endolaser photocoagulation or (2) retinectomy for RCH resection, although a high rate of vision-threatening RCH recurrence was observed in the long term. However in most cases, surgery improved or prolonged visual function in these eyes.