WOFAPS 2025 8th World Congress of Pediatric Surgery

Betül Beyza Özbal, İbrahim Baytar, Birgül Karaaslan, Fatma Saraç

Basaksehir Cam and Sakura City Hospital, Dep. of Pediatric Surgery, Istanbul, Turkey

Aim:

A case of a male newborn diagnosed with congenital pouch colon-associated anal atresia, complicated by a fistula connecting to the umbilicus, triplication of the vermiform appendix, and congenital stenosis of the bladder neck, is presented.

Method:

The patient underwent emergency surgery at an external center, where the umbilical fistula was excised, an ileal diversion stoma was created, and a vesicostomy was performed. Postoperatively, functional stool passage from the ileostomy and urine output from the vesicostomy were observed. A roughly 2 cm segment between the urethra and bladder neck was completely closed.

At one year of age, the patient was referred for definitive surgery. A posterior sagittal anorectal pull-through procedure was performed. Three appendices and the pouch colon were excised. The ileostomy was reversed, and a J-pouch was created from the ileum. Anoplasty was done directly in place of the anus with anastomosis. The bladder neck was found completely closed, and the vesicostomy was revised. The presence of a fistula from the bladder to the umbilicus and complete closure of the bladder neck at birth suggested congenital autoaugmentation.

Results:

Congenital pouch colon is an extremely rare congenital condition, with very few cases reported in Turkey. It presents with anal atresia and abdominal distension, often accompanied by constipation and vomiting. Most cases involve fistulas with the urinary system and sometimes, as in this case, appendix anomalies. This condition is serious and may lead to megacolon, intestinal perforation, and early mortality.

Conclusion:

This case is notable for three main reasons: its rarity in Turkey , the coexistence of multiple anomalies including umbilical fistula and appendix triplication, and the unique combination of bladder neck closure with bladder-to-umbilicus fistula, which is first reported here. Proper surgical management is crucial to prevent complications. The congenital autoaugmentation feature distinguishes this case from others in the literature.