全身異常を伴った後部胎生環の6例

Posterior embryotoxon in six cases with systemic anomalies

　全身の先天異常を伴う後部胎生環の6例を検討した。年齢は生後1〜3か月（平均2.2か月），性別は男児3例，女児3例，全例両眼性で，眼圧は正常であった。合併眼異常は強角膜症4例，瞳孔膜遺残2例，合併全身異常は先天性胆道閉鎖症2例，先天性顔面神経麻痺と小耳症，先天性副腎過形成，Hirs—chsprung病，Alagille症候群各1例であった。強角膜症，瞳孔膜遺残，先天性顔面神経麻痺，小耳症，先天性副腎過形成は神経堤細胞の発生異常であり，Hirschsprung病，Alagille症候群は神経堤病に相当する。以上のように，合併した眼および全身異常には神経堤細胞の発生異常が多くみられた。

We reviewed 6 cases of bilateral embryotoxon associated with systemic developmental dis-orders. The series comprised 3 males and 3 females. They were examined 1 to 3 months after birth.Ophthalmologically, sclerocornea was present in 4 cases and persistent pupillary membrane in 2. Intraocular pressure was normal all the 6 cases. As systemic complications, there were 2 cases of congenital atresia of the bile duct and 1 case each of microtia with facial palsy, congenital hyperplasia of adrenal glands,Hirschsprung disease and Alagille syndrome. Developmental anomalies of neural crest cells will result in sclerocornea, persistent pupillary membrane, congenital facial palsy, microtia and congenital hyperplasia of adrenal glands. Hirschsprung disease and Alagille syndrome are regarded as neurocristopathies. The present cases of embryotoxon thus showed various developmental disorders of the neural crest cells.