Triple atresia is a rare condition consisting of esophageal atresia, duodenal atresia, and anorectal malformation. It brings dilemmas for surgeons in diagnosing the condition itself and in planning for surgical management, whether to perform surgery in a single stage or in stages for all three anomalies. In staged surgery, there are options of either repairing esophageal atresia first or prioritizing duodenal atresia or colostomy. However, the surgery itself predisposes these neonates to risks of prolonged surgery, hypothermia, and metabolic changes that may affect the prognosis.

A term female neonate with a birth weight of 2.36 kg was referred to us for excessive drooling of saliva. Clinically the baby had no dysmorphism with unremarkable abdominal findings, and normal female genitalia but absent anal opening. Resistance was noted during Ryle’s tube insertion. Infantogram revealed coiling of Ryle's tube at T2-3 vertebra level, prominent gastric bubble, and duodenal cap with no distal bowel gas (Fig. 1). These findings made a provisional diagnosis of Type C esophageal atresia with distal tracheoesophageal fistula, duodenal atresia, and high-type anorectal malformation. Staged surgery began with right thoracotomy on day 3 of life through 4th intercostal space, with an extrapleural approach the fistula was ligated with prolene 5-0, and primary esophageal anastomosis completed using PDS 6-0. Postoperatively, the baby was shifted to NICU, ventilated on low settings, and hemodynamically supported with single inotropes.

On day 7 of life, she underwent laparotomy via a right upper transverse incision. A Type 3 duodenal atresia was identified and Kimura duodeno-duodenostomy was performed. A left transverse colostomy for anorectal malformation was also made. The baby was in NICU on conventional ventilation till day 11 of life. Ryle’s tube feeding was initiated on day 7 postoperative. There was no episode of feeding intolerance. Feeding was gradually stepped up and was discharged with omeprazole after 5 weeks postoperative. Corrective surgery for anorectal malformation was performed at 1 year of age via the posterior sagittal route. There was a rectovaginal fistula noted. The fistula was detached and transfixed. Rectum was mobilized and anorectoplasty was done.

Table 1 depicts a literature review of triple atresia published cases. It shows that seven cases had undergone staged repair of their respective anomalies in which 4 of them underwent laparotomy for bowel pathology first then followed by thoracotomy, ligation of fistula, and esophageal anastomosis. In the other 3 cases from the staged group, thoracotomy was performed prior to laparotomy. Meanwhile, there were 3 cases in the staged group that had concomitant congenital heart disease and in 2 cases, babies’ weights were under 2.0 kg. A single staged surgery was performed in 4 cases of which 3 of them, thoracotomy was performed first and followed by laparotomy. Unfortunately, 2 of them died within one week postoperatively.

This experience convinces us that the decision to perform TEF repair first and followed by laparotomy and stoma is safer and offers better survival. Raef et al favor staged surgery over single-staged surgery as it is found to have a better survival rate.^[1] As outlined in table 1, only one mortality was documented among the staged group. However, as of now, there is no clear guideline available for managing triple atresia as the anomaly remains extremely rare.^{[1], [2], [3]} As to the sequence of staged surgery, we opted to perform TEF repair first as there is a risk of gastric distension due to air venting down from the trachea into the stomach through distal fistula which can also cause gastric perforation.^[4] Both Raef and Spitz et al suggest that performing TEF repair as the first surgery yields a better outcome.^{[1], [5]}