網膜剝離をきたしたStickler症候群の2例

Retinal detachment in two cases of Stickler syndrome

裂孔原性網膜剝離がStickler症候群の2例2眼に起こった。いずれも男性で，年齢は20歳と32歳であった。1例には高口蓋と側彎があり，増殖性硝子体網膜症が発症していた。他の1例には口蓋裂，先天性難聴，膝内症があった。両症例に硝子体手術を行った。2例とも高度の硝子体液化と変性があり，硝子体が面状に網膜と癒着し，双手方による人工的後部硝子体剝離の作製と輪状締結術が必要であった。Stickler症候群に伴う網膜剝離は，異常な網膜硝子体癒着があり，高度な手技による硝子体手術が望ましい。

We treated rhegmatogenous retinal detachment in 2 cases of Stickler syndrome. Both were males aged 20 and 32 years respectively. The first case had arched palate,lateral curvature and proliferative vitreoretinopathy（PVR）. The second had cleft palate,congenital deafness,and internal derangement of knee joints. Both cases received vitreous surgery. As intraoperative findings,both eyes showed firm and extensive vitreoretinal adhesion,requiring artificial posterior vitreous detachment and scleral encircling. These cases illustrate that vitreous surgery may be indicated for retinal detachment in Stickler syndrome because of abnormal vitreoretinal adhesion.