姉弟例における瞼裂縮小症の治療経験

Treatment of blepharophimosis in a pair of siblings

　先天性瞼裂縮小症は，両眼性の瞼裂縮小，逆内眼角贅皮，眼瞼下垂を三主徴とする先天性奇形である。我々は，祖父，父にも瞼裂縮小症のある姉弟2例に対して治療した。まず水平瞼裂幅拡大を日的としてMustardé法を行い，6か月後に垂直瞼裂幅拡大を目的として　Friedenwald—Guyton法を施行した。2年後の計測で，姉の水平瞼裂幅は概当年齢の平均値なみに拡大し，内眼角間距離は平均値より短くなった。弟では最初のMustardé法が効いていず，水平瞼裂幅の拡大と内眼角間距離の短縮はなかった。今回の手術を通してMustardé法が奏効すれば，吊り上げ術でも十分自然な表情が得られると思われた。

We attempted surgical correction of blepharo-phimosis syndrome in a female child at the age of 3 years 11 months and her brother at the age of 3 years 6 months. The condition was characterized by bilateral blepharophimosis, epicanthus inversus and blepharoptosis. The father and the grandfather of the siblings manifested the same condition.

We performed the Mustarde technique for epicanthus inversus and, 6 months later, theFriedenwald-Guyton technique for blepharoptosis. When evaluated 2 years later, the first case showed normal value for the horizontal length of palpebral fissures. The distance between the medial angles was shorter than normal. In the second case, epicanthus inversus persisted in spite of Mustarde technique. Due correction of epicanthus inversus appeared to be a prerequisite for the ensuing eyelid elevating surgery to succeed.