先天性 H 型肛門尿道瘻の2例 : 症例報告と発生学的考察

概要

論文の詳細を見る
Congenital "H-type" ano-urethral fistula is an extemely rare condition which is often accompanied with tracheoesophageal fistula (TEF) and some urological anomalies, but seldom with anal deformitiy. Clinical details of two patients which we experienced recently are reported here, together with the discussion of pathoembryology of this anomaly. One of the patients was 8-year-old boy, and he had hypospadia and undescended testis. Another patient was 4-year-old boy who had urethral stenosis, dysplastic kidney and undescended testis. In the world literatures, 13 cases with congenital "H-type" ano-urethral fistula could be found. Among 15 cases including ours, 7 had TEF and 12 had urological anomalies such as urethral stenosis, ectopic ureteral orifice, cross renal ectopia, and others. Anal anomalies were found in only three cases (1 covered anus, 1 rectal stenosis, 1 anterior perineal anus). Although much to be debated on the pathoembryology of this condition, it seems that the misaligned components of urorectal septum which lead incomplete devision of cloaca play a critical role to make up this rare condition.
日本小児外科学会の論文
1986-12-20