Rectosigmoid perforation in an anorectal malformation neonate and perineal fistula

Colorectal perforation in infants with anorectal malformation (ARM) is a very rare case with significant morbidity and mortality. Here, we reported the first case of rectosigmoid perforation in an ARM patient with perineal fistula that was previously diagnosed as ARM without fistula. A 3.2 kg-full-term male neonate was admitted to our emergency unit at 24 hours after birth with chief complaint of abdominal distention and imperforate anus without any visualized meconium on the perineum or urine. Physical examination revealed abdominal distention and tenderness, and anal dimple. Cross-table lateral x-rays showed a rectal gas bubble at 5.8 cm from perineal skin, while the babygram revealed massive pneumoperitoneum (i.e., football and falciform ligament signs), indicating peritonitis. Then, we decided to perform exploratory laparotomy and found a colorectal perforation approximately 7 cm along the rectosigmoid colon. We closed the perforation followed by a proximal transverse colostomy. Regrettably, we did not send any specimens of bowel wall for ganglion cells to rule out concomitant Hirschsprung disease. Interestingly, at post-operative day (POD) 2, the meconium was visualized at the perineum (i.e., perineal fistula). Unfortunately, the patient died at POD 11 due to severe sepsis.