抄録
症例は日齢0の男児.在胎32週3日,胎児心音の低下を認め,緊急帝王切開にて出生.外性器異常・鎖肛を認め,当院搬送入院となった.尿膜管より排尿・排便を認めたが,臍帯ヘルニアなどの合併はなく,下腹部は正常皮膚で覆われていた.陰茎欠損と二分陰嚢を認めるも,精巣は陰嚢内に触知可能であった.尿膜管からの造影では膀胱と腸管が描出され外反のない膀胱腸裂と診断し,緊急手術を施行した.開腹所見では,膀胱後壁が回腸により左右に分離されていた.回盲部より肛門側は虫垂も含めて重複しており,結腸は4cmで盲端となっていた.小腸は全長で14cmであった.回腸と膀胱を分離し,単孔式結腸瘻・膀胱皮膚瘻を造設した.その後の染色体検査では正常男性型であり,両親と相談のうえ男性としての性を選択した.術後の栄養管理は短小腸の合併もありかなり困難であった.
We report a rare association of covered cloacal exstrophy with penile agenesis and short bowel as the first case in Japan. A male infant was born at 32 weeks gestation weighing 1,444g. He was noted to have an imperforate anus and anomaly of the external genitalia. He had a lowset umbilicus and passed meconium via the urachus. The pubic bones were separated, but the lower abdominal wall was normal. He had aphallia and bifid scrotum. Laparotomy revealed a ileovesical fissure between the posterior extrophied bladders. Duplicated appendices and colons ended blindly approximately 4cm distal to the fistula. The small intestine was only 14cm in length from the Treitz ligament. The terminal ileum was divided from the bladder for the reconstruction of an end colostomy. The bladder was repaired with a vesicostomy made at the umbilical site.
