デスメ膜破裂より角膜浮腫を生じたHallermann-Streiff症候群の1例

Corneal edema due to rupture of Descemet's membrane in a case of Hallermann-Streiff syndrome

　Hallermann-Streiff症候群に角膜浮腫を生じた1例を経験した。症例は30歳女性で，すでに僚眼は緑内障により著明に視力低下し，患眼は角膜浮腫以外に下方菲薄化が認められた。その他，両眼の小眼球，先天性白内障，眼振，および球状角膜も認められた。角膜保護剤，コルチコステロイド剤，抗生剤，5％高張食塩液の点眼，炭酸脱水酵素阻害剤（ダイアモックス^®）の内服を施行したが効果はなかった。発生1か月後にデスメ膜破裂が認められ，圧迫眼帯も行い，さらに1か月後には浮腫は治癒した。特異的なphotokeratoscope所見は得られなかったが，球状角膜を原因としたデスメ膜破裂より角膜浮腫を生じた1例と考えられた。新鮮例では，保存的療法も効果があるものと思われた。

A 30-year-old female with Hallermann-Streiff syndrome presented with corneal edema and thinning of lower peripheral cornea in the right eye. The left eye showed advanced signs of glaucoma. She had bilateral microphthalmos, congenital cataract, nystagmus and keratoglobus. Systemic acetazolamide and instillation of cornea-protecting agents, corticosteroid, antibiotics and 5% saline proved futile for the corneal edema. One month later, the Descemet's membrane ruptured in the affected eye. Gradual recovery set in after continuous wear of pressure eyepatch for one month. While no specific findings were obtained by photokeratoscopy, the corneal edema appeared to have been induced by ruptured Descemet's membrane due to keratoglobus. Conservative therapy appeared to be effective for fresh corneal lesions in this syn-drome.