Anorectal malformation, duodenal atresia, and intestinal malrotation: a case report

The combination of anorectal malformation, intestinal malrotation, and duodenal atresia is exceptionally rare. Such cases usually require staged surgical management. A full-term female newborn (3.32 kg), prenatally diagnosed with duodenal atresia, Down syndrome, and imperforate anus without fistula, was admitted. Abdominal x-ray confirmed a double-bubble sign, and ultrasound revealed no other anomalies. A single minimally invasive surgery was planned on day of life three. First step: an exploratory laparoscopy was performed via a paraumbilical port and additional flank ports using 3-mm instruments and a 30-degree scope. Duodenal atresia and intestinal malrotation were identified, and Ladd bands were released. Second step: an intraoperative diagnostic contrast enema was done by a transabdominal puncture, followed by injection of water-soluble contrast. This demonstrated a 5-mm distance between the rectal pouch and the theoretical anal site, without evidence of a fistula. Third step: a side-to-end duodeno-duodenal anastomosis was performed. The anterior walls of both duodenal ends were opened, and a continuous running suture was done using 5-0 polydioxanone, starting on the posterior wall. Next, the patient was placed in prone position for a standard posterior sagittal anorectoplasty. The postoperative course was uneventful. Enteral feeding began on postoperative day six and anal dilatations on postoperative day nine. The patient was discharged home at one month of age having normal function. No reinterventions have been required at one year of follow-up. The combination of anorectal malformation, duodenal atresia, Down syndrome, and intestinal malrotation is very rare. Repairing all defects in a single surgical stage seems safe and effective. • Rare combination of duodenal atresia, malrotation, and anorectal malformation. • Highlights diagnostic challenges in complex neonatal gastrointestinal anomalies • The laparoscopic guided transabdominal contrast study enabled precise intraoperative diagnosis, avoiding staged procedures and allowing definitive treatment in a single operation. • Single step surgical management performed using a minimally invasive approach