裂孔原性網膜剝離を併発したGoldmann-Favre病の1例

Rhegmatogenous retinal detachment in a case of Goldmann-Favre disease

要約　目的：裂孔原性網膜剝離が発症したGoldmann-Favre病症例の報告。症例：17歳男性が右眼視力低下で受診した。幼時から夜盲があったという。親は血族結婚でなく，家系内に夜盲または視力不良者はいない。矯正視力は右0.03，左0.5で，右眼に耳側上方の網膜円孔から下方にかけて網膜剝離があった。両眼に，硝子体の線維状混濁と液化融解，黄斑部の反射不整，中心窩に向かう放射状の皺襞，眼底周辺部に類円形の色素斑の散在があった。網膜電図は消失型であり，光干渉断層検査（OCT）で，右黄斑部に網膜剝離，左黄斑部に網膜分離があった。経強膜手術で右眼の網膜は復位した。結論：裂孔原性網膜剝離がGoldmann-Favre病に併発する可能性がある。

Abstract.　Purpose：To report a case of Goldmann-Favre disease with unilateral rhegmatogenous retinal detachment. Case and Findings：A 17-year-old male presented with impaired visual acuity in his right eye. He had had night blindness since childhood. There was no consanguineous marriage in his family. His corrected visual acuity was 0.03 right and 0.5 left. The right eye showed an oval retinal hole in the superior-temporal quadrant associated with retinal detachment involving the macula and inferior quadrants. Both eyes showed liquefied and fibrinous-turbid vitreous,irregular macular reflex with folds,and pigment patches in the periphery. Electroretinogram was extinguished in both eyes. Optical coherence tomography（OCT）showed retinal detachment in the right eye and retinoschisis in the left. The retina became reattached in the right eye after scleral buckling procedure. Conclusion：Rhegmatogenous retinal detachment may develop as a complication of Goldmann-Favre disease.