鼻側網膜ひだを伴う家族性滲出性硝子体網膜症に対し硝子体手術を施行した1例

A case of vitrectomy for familial exudative vitreoretinopathy with nasal retinal folds

要約　目的：家族性滲出性硝子体網膜症（FEVR）は網膜ひだを呈する先天網膜疾患の1つであり，その多くは耳側にみられる。今回筆者らは，鼻側に網膜ひだと網膜剝離を認めるFEVRに対し水晶体温存硝子体手術にてシリコーンオイル（SO）を用いて復位を得た症例を経験したため報告する。

症例：17歳，女性，正期産にて出生。右眼の視野欠損を主訴に近医にてFEVRと診断され，当院を紹介受診した。

所見：右眼の矯正視力（1.2），眼軸長23.11mmで，眼底は鼻側に網膜ひだと，その周囲に網膜剝離を認めていた。蛍光眼底造影検査にて両眼とも周辺部の血管異常を認め，上記と診断し，全身麻酔下にて硝子体手術を施行した。手術では網膜ひだ部に硬い網膜上索状物を認めたため，硝子体鑷子を用いてこれを除去し，またその周囲の菲薄化した網膜に萎縮性網膜円孔を認めたため，同部位に網膜光凝固を行いSOを注入して手術を終了した。術後3か月でSOを抜去し，右矯正視力（1.2）と良好で，網膜の復位を得られている。

結論：認めることが少ない鼻側の網膜ひだを伴うFEVRに対し，水晶体温存硝子体手術でもSOを用いて適切な時期に抜去することで，良好な視力予後を得られる可能性がある。

Abstract　Purpose：Familial exudative vitreoretinopathy（FEVR） is a congenital retinal disorder characterized by retinal folds predominantly observed on the temporal side. We report a case of FEVR presenting with retinal folds and retinal detachment on the nasal side, which was successfully reattached using silicone oil（SO） during crystal-sparing vitreous surgery.

Case：A 17-year-old female, born at full term, presented with a chief complaint of a right visual field defect. She was diagnosed with FEVR at a local hospital and subsequently referred to our institution for further evaluation and management.

Findings：A retinal fold was observed on the nasal side, accompanied by retinal detachment in the surrounding area. The corrected visual acuity in the right eye was 1.2, with an axial length of 23.11 mm. Fluorescein angiography revealed abnormal peripheral vasculature in both eyes, leading to the diagnosis of FEVR. Vitreous surgery was performed under general anesthesia. Intraoperatively, a dense epiretinal membrane was identified within the retinal fold and was excised using vitreous forceps. Additionally, an atrophic retinal hole was detected in the adjacent thinned retina, prompting retinal photocoagulation in the affected area, followed by SO injection to complete the procedure. Three months postoperatively, the SO was removed. Although the retinal fold persisted and a mild posterior subcapsular cataract was observed, the corrected visual acuity in the right eye remained favorable at 1.2, and retinal reattachment was successfully achieved.

Conclusion：In cases of FEVR with nasal retinal folds, a rare presentation, favorable visual prognosis may be achieved through the timely removal of retinal folds using SO during crystalline lens-sparing vitreous surgery.