Intractable constipation in an adult with megarectosigmoid following repair of low-Type anorectal malformation

A 55-year-old male patient received an anoplasty at infancy for low-Type anorectal malformation, and intractable constipation was observed since. The patient presented with colitis at the emergency department. Computed tomography revealed feces with a megarectosigmoid of diameter 18 cm. Hirschsprung disease was suspected on the basis of the imaging findings. Therefore, a rectal biopsy was performed, which indicated the presence of normal ganglion cells. Bowel management was implemented with one large-volume enema to empty the huge rectal pouch daily. The patient was free of constipation and had an improved nutritional status with appropriate weight gain in 3 months. A laparoscopic Swenson pull-Through was performed. The hospital course was uneventful. Enemas, loperamide, and water-soluble fibers were required for frequent soiling because of postoperative hypermotile colon. The treatments were weaned off gradually. At the latest follow-up (2 years postoperatively), the patient had regular voluntary bowel movements with complete bowel control.