強膜穿孔および視神経萎縮のみられたParry-Romberg症候群の1例

Scleral perforation and optic atrophy in a case of Parry-Romberg syndrome

　33歳男性が，左眼の視力低下と眼球陥凹で受診した。10歳の頃から前額，眼窩，頬部を含む左側の顔面萎縮があった。視力は右1.5，左光覚弁，眼圧は右12mmHg，左2mmHgであった。左側の眼球陥凹，眼瞼萎縮，眼瞼内反，睫毛脱落，散瞳，眼球運動障害，強膜穿孔によると推定される結膜濾過胞があり，眼底には視神経萎縮と乳頭周囲から下方に広がる網脈絡膜萎縮があった。本例は，半側顔面萎縮を中軸病変とするParry-Romberg症候群に視神経萎縮と強膜穿孔が併発したと解釈される。

A 33-year-old male presented with left enophthalmos and visual impairment. He had noticed left facial hemiatrophy involving the forehead, orbit and cheek since the age of about 10 years. His visual acuity was 1.5 right and light perception left. The intraocular pressure was 12 mmHg right and 2 mmHg left. He also showed, besides left facial hemiatrophy, ipsilateral enophthalmos, atrophy of eyelids, entropion, loss of cilia, mydriasis, limitation of eye movement, and conjunctival bleb secondary to scleral perforation. Funduscopy showed ipsilateral optic atrophy and chorioretinal atrophy extending from the peripapillary area to the inferior midperiphery. This case illustrates that Parry-Romberg syndrome, which is characterized by facial hemiatrophy, may be associated with optic atrophy and scleral perforation.