片眼にPeters奇形を合併した臨床的無眼球の1例

A case of unilateral anophthalmos with Peters'anomaly in the fellow eye

　片眼にPeters奇形を合併し，他眼はMRIで痕跡状の眼球を確認した臨床的無眼球症の1症例を報告した。症例は16歳，男子。出生時，右眼角膜混濁，左眼瞼裂狭少および左眼無眼球と診断された。現在，右眼は瞳孔領を覆う角膜白斑が存在し，虹彩は角膜混濁部の4時と8時部で前癒着し，Peters奇形が疑われた。左眼は，狭小化した結膜嚢から結膜組織様のものがみられ，MRIでは痕跡状の眼球と4直筋が確認された。右眼眼球の大きさ，形態には著変はなかった。片眼にPeters奇形，他眼に臨床的無眼球症を合併した症例はきわめて稀である。本奇形は神経堤細胞の遊走異常が左右別々に程度を変えて起こったために生じたと推測した。

A 16-year-old male had been diagnosed, at birth, as corneal opacity in the right eye and narrow palpebral fissure with anophthalmos in the left. When seen by us, the right corneal opacity was present in the pupillary area. Anterior synechia was present at 4 and 8 o'clock positions. These findings led to the diagnosis of Peters' anomaly. A conjunctiva-like tissue was present in the conjunctival sac deep in the left orbit. Magnetic resonance imaging, MRI, showed a trace of eyeball with thin optic nerve in the left orbit. The right orbit was normal. The unusual association of anophthalmos in one eye and Peters' anomaly in the other appears to be the consequence of abnormal migration of neural crest cells in different ways in either eye.