黄斑部牽引性網膜剝離に進行したBloch-Sulzberger症候群に対する硝子体手術

Vitrectomy for Bloch-Sulzberger syndrome progressing to macular tractional retinal detachment

　8歳女児が右眼視力低下で紹介され受診した。乳児期にBloch-Sulzberger症候群と診断されていた。矯正視力は右0.2，左1.2で，屈折，眼圧，前眼部などに格別の異常所見はなかった。右眼に滲出性網膜剝離と線維増殖膜による黄斑を含む牽引性網膜剝離があり，全周に網膜血管の形成不全があった。左眼には眼底全周に無血管野があった。右眼に経強膜冷凍凝固を行ったが滲出性変化は軽減せず，牽引性網膜剝離が進行したため，硝子体手術を行った。水晶体を切除したのち，後極部の増殖膜を剪刀で切開，除去し，周辺部に向かって後部硝子体剝離を作製した。周辺部の無血管領域では網膜硝子体剝離が強固であった。これにより網膜は復位し，手術の9か月後に矯正視力は術前の0.05から0.2に改善した。本症候群に伴う牽引性網膜剝離では，未熟児網膜症や家族性滲出性硝子体網膜症と同様に周辺部網膜無血管野の硝子体処理が困難であることが特徴的であった。

　A 8-year-old girl was referred to us for failing vision in her right eye. She had been diagnosed with Bloch-Sulzberger syndrome during infancy. Her corrected visual acuity was 0.2 right and 1.2 left. No gross abnormalities were present regarding refraction,intraocular pressure and anterior ocular segment. The right eye showed exudative and tractional retinal detachment involving the macula. The whole peripheral retina had become avascular in both eyes. The retinal detachment progressed in spite of transscleral cryocoagulation,necessitating vitreous surgery. Following lensectomy,the proliferative membrane in the posterior fundus was delaminated and removed. Artificial detachment of posterior vitreous was created towards the periphery in spite of marked vitreoretinal adhesion in the peripheral avascular retinal area. The retina became reattached and the visual acuity improved from 0.05 to 0.2. Presence of marked vitreoretinal adhesion in the periphery appeared to be a characteristic feature of this syndrome similar to retinopathy of prematurity and familial exudative vitreoretinopathy.