Congenital Pouch Colon Complicated by Persistent Descending Mesocolon

Abstract

Congenital pouch colon (CPC), which is one of the anorectal malformations with pouch formation of the colon, is rare in Japan. Persistent descending mesocolon (PDM) is a syndrome with the displacement of the left colon with a shortened mesocolon and radial distribution of the inferior mesenteric artery. No case of CPC complicated by PDM has been reported. A boy was born with anal atresia. X-ray examination showed a huge colon gas. After 15 h, laparoscopy showed that the left colon is fixed to the midline, and the sigmoid colon extended like a pouch, which suggested Type-4 CPC with PDM. Colostomy was carried out at the oral edge of the pouch to preserve the normal sigmoid colon, although Type-4 CPC commonly requires the excision of the pouch at the first operation. Contrast enema of the pouch colon showed a colourethral fistula. MRI showed closed spinal dysraphism with myelocystocele. Because of repeated stoma prolapse, we performed laparoscopic pull through after the excision of the pouch and the ligation of the colourethral fistula on the 47th day. The control of defecation is good two years after the last operation. Because CPC has not been well recognized in Japan, an operative strategy based on a precise diagnosis is difficult. However, CPC is accompanied by multiple anatomical malformations in the gastrointestinal, urological, gynecological, skeletal, and neurospinal areas.