家族性滲出性硝子体網膜症と裂孔原性網膜剥離多数例の検討

Familial exudative vitreoretinopathy and rhegmatogenous retinal detachment

　FEVRに併発した網膜剥離85例101眼の臨床像を検討した。全101眼のうち90眼（89％）が裂孔原性網膜剥離であり，FEVR病型では全101眼のうち周辺部変性型が83眼（82％）と大多数を占めた。FEVRによる裂孔原性網膜剥離の初回手術眼83眼のうち68眼（82％）の主裂孔は網膜有血管野と無血管野との間の境界線に接する無血管野に存在した。FEVRによる裂孔原性網膜剥離の初回手術眼の再剥離原因の多くは，バックルによる網膜隆起辺縁の境界線に沿って形成される新裂孔や網膜固着術による過凝固部に生じる新裂孔によるものであった。FEVRに併発する裂孔原性網膜剥離に対する治療は，境界線を含む網膜無血管野の全領域をバックルによる網膜隆起の周辺側に乗せる広範囲強膜内陥術と適度な光凝固による二段階法が最も適切と考えられた。

We reviewed a consecutive series of 85 cases, 101 eyes, with familial exudative vitreoretinopathy (FEVR) and retinal detachment. The series com-prised rhegmatogenous retinal detachment (RRD) 90 eyes, 89%, traction retinal detachment 3 eyes and total retinal detachment resulting in blindness 8 eyes. Out of 90 eyes with RRD, 7 eyes had been operated elsewhere. In 68 eyes of the remaining 83 eyes, 82%, retinal breaks were located immediately outer to the borderline between vascular and avas-cular retina in the periphery. In the other 15 eyes, breaks were located in the vicinity of the border-line. All the 83 eyes were operated by scleral buck-ling procedure and external drainage of the sub-retinal fluid. Reattachment resulted in 68 out of the 83 eyes, 82%, after the initial surgery. Recurrences developed in the other 15 eyes, 18%, necessitating a total of 28 reoperations. Recurrences were due to newly developed retinal breaks located near the borderline in 13 of these eyes and due to prolifer-ative vitreoretinopathy in the other 2 eyes. Even-tual reattachment was obtained in 82 eyes, 99%. We advocate wide episcleral buckling and encircling covering all the avascular area in the periphery for eyes with RRD due to FEVR.