網膜剝離を伴うvon Hippel-Lindau病に対する硝子体手術

Vitreous surgery for retinal detachment in a case of von Hippel-Lindau disease

要約　目的：網膜剝離を伴うvon Hippel-Lindau病に対し硝子体手術をした症例の報告。症例：15歳男性。両眼に網膜血管腫，左眼に漿液性網膜剝離を認めた。所見と経過：視力は右眼1.2，左眼0.03であった。右眼周辺部血管腫に網膜光凝固術を施行，左眼は大型の血管腫で網膜剝離もあり，左眼硝子体手術，水晶体摘出術，眼内レンズ挿入術を施行した。術後，左眼の網膜は復位し，術後5か月で左眼視力0.3に回復した。両眼血管腫の活動性が再発し，両眼に網膜光凝固術，右眼に網膜冷凍凝固術を追加した。術後2年後に左眼視力0.5に改善した。結論：網膜剝離を伴うvon Hippel-Lindau病では硝子体手術も有効である。

Abstract.　Purpose：To report a case of von Hippel-Lindau disease who received vitreous surgery for retinal detachment. Case：A 15-year-old male referred to us for retinal detachment in the left eye. He had noticed impaired left vision since 3 years before. His mother and his brother had been diagnosed with von Hippel-Lindau disease. Findings：Corrected visual acuity was 1.2 right and 0.03 left. Both eyes showed three hemangiomas in the periphery. The left eye showed total retinal detachment. The left eye was treated by vitrectomy, lensectomy and implantation of intraocular lens. The retina became reattached with visual acuity improving to 0.3 five months later. Retinal hemangioma became activated in both eyes and was treated by photocoagulation. The right eye received additional cryocoagulation. The patient has been doing well for 2 years until present with visual acuity maintained at 0.5. Conclusion：This case illustrates that vitreous surgery may be effective for retinal detachment as complication of von Hippel-Lindau disease.