家族性滲出性網膜硝子体症での網膜血管と眼底病変との関連

Fundus manifestations and retinal vascular pathology in familial exudative vitreoretinopathy

　家族性滲出性網膜硝子性症を示す81例138眼について，網膜血管異常と眼底病変，視機能との関連を検索した．本症の網膜血管異常を増殖性変化，網膜無血管野の有無から5型に分類して分析した．典型例では広い網膜無血管野，血管吻合，多分岐，咬合不全を呈するが，最軽症例では網膜無血管野がないこと，本症の特微である視野異常は，広い無血管野を示す血管型での検討から，その成因に網膜無血管野，V字型変性が関与しており，本症の遺伝型式については突発性のものがあることが明らかになった．眼底病変と分類された血管型とは，密接な関連を有していることから，網膜硝子体癒着，網膜分離症，黄斑偏位，牽引性網膜剥離は網膜血管異常の続発病変と考えられた．

We investigated the correlation between fundus-copic fundus appearances and retinal vascular pattern in 138 eyes (81 cases) with familial exuda-tive vitreoretinpathy (FEVR). A wide variety of retinal vascular abnormalities were observed in the extreme fundus periphery, including : presence of avascular zone in the extreme periphery, numerous branchings, arteriovenous shunts and neovascularizations. These vascular abnormalities appeared to be the more fundumental lesions in FEVR, while other characteristic features of the disease, i.e. V-shaped chorioretinal degenerations in the temporal sector, ectopia of the macula and tractional retinal detachment, seemed to be secondary to these vascular abnormalities.

A grading system of FEVR into 5 groups is proposed based on these vascular abnormalities. The presence of 'sporadic' FEVR is postulated.