18-Month-Old Male Patient Born with Omphalocele, Anorectal Malformation, Ileal Atresia, and Cecum Duplication Referred for Treatment of Short Bowel Syndrome

Case Summary

• 18-mo-old male patient presented for evaluation. He was born at 37 wk with an omphalocele, anorectal malformation, ileal atresia, and cecum duplication.
• On day of life 2, he had laparotomy. During this procedure, surgeons found ileal atresia and internal hernia with ischemia of the ileocecal valve. Surgeons also found cecum and appendix duplication and colon-bladder fistula.
• Duplicated cecum was resected, and an appendectomy was performed.
• Ileostomy and colostomy were performed.
• On day of life 23, an abdominal wall plasty was performed.
• On day of life 35, a cystostomy, but this closed spontaneously.
• He was referred for treatment of short bowel syndrome. At the time of referral, he weighed 6.9kg. His urinary sodium was 8 mEq/L. He had severe malabsorption. He had two stomas that had severe peristomal dermatitis and one enterocutaneous fistula.
• Workup included spinal imaging, which identified spina bifida at T2-T6.
• Sacral ratio anterior-posterior 0.62 and lateral 0.52.
• Distal colostogram showed a colo-bladder fistula 12-15 cm from the distal bowel. Creatinine was 0.3.
• Renal ultrasound showed dilation of the right pelvis and ureter.
• Patient had kidney reflux and left renal agenesis.
• Mag 3 showed obstruction.
• Patient underwent closure of colo-bladder fistula with anorectoplasty, santulli anastomosis of the terminal ileum with the distal colon and right vesicoureteral reimplantation.
• Patient had 100 cm of small bowel from the ligament of Treitz to the proximal colon.
• Patient at 35 mo old started to have urinary sphincter control but had some daytime leaks. He had fecal evacuations through the anus, and he received one enema every day and was clean for 12 h following.
• He typically had two stool evacuation episodes 12 h after the enema. He does not take antidiarrheal medications and his stool nutrient absorption tests are normal.
• One year after surgery, his renal ultrasound did not show evidence of hydronephrosis.

Takeaways

This patient was born with covered cloacal exstrophy. This malformation has some characteristics of cloacal exstrophy but not all the characteristics including short colon and cecal duplication.

During the first operation, it is recommended to ligate the colo-vesical fistula and create a true end colostomy. This allows the ability to do bowel management through the stoma in the future to see if the patient is a candidate for colostomy closure. It is important to incorporate all the bowel into the fecal stream.