Triple Atresia, Triple Threat? An Unusual Constellation of Primary Surgical Abnormalities

Abstract

We present a case series of two patients with tracheo-oesophageal fistula with oesophageal atresia (TOF/OA), duodenal atresia (DA) and ano-rectal malformation (ARM). This constellation of abnormalities, dubbed triple atresia (TA), is a rare combination with few described cases in the literature. Here we describe our management of these cases, as well as the results of our literature review. Both of our cases had staged surgical procedures and were initially managed with thoracotomy for repair of TOF/OA on day two of life. They subsequently underwent laparotomy for management of their abdominal pathology at day five and seven of life. Both have survived the neonatal period and are awaiting definitive surgery for ARM. Literature review yielded seven cases of TA involving a TOF, DA, and ARM. Four patients underwent staged repair, while three patients underwent repair of TOF/OA, DA and colostomy for ARM at the same time. Of these three patients, two died, representing 22% of the overall cohort. Triple atresia remains a rare subset of patients suspected to have VACTERL association, however mortality may be significantly higher. Our data would suggest a staged approach to be optimal for long term survival.

Triple atresia: Staged repair

“…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.…”

“…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.…”

“…[4] Both Raef and Spitz et al suggest that performing TEF repair as the first surgery yields a better outcome. [1,5].…”

A rare case of oesophageal atresia complicated by duodenal atresia and anorectal malformation successfully treated with multi-stage surgery

“…The incidence of oesophageal atresia ranges from 1 in every 2500 to 4500 live births [ [1] , [2] , [3] ]. Oesophageal atresia is associated with several major complications, the most prevalent being the VACTERL association, wherein the incidence of oesophageal atresia is notably elevated (50–80 %) [ 4 , 5 ]. On the other hand, duodenal atresia has been described in patients presenting with a collection of anomalies consistent with VACTERL [ 6 ].…”

“…On the other hand, duodenal atresia has been described in patients presenting with a collection of anomalies consistent with VACTERL [ 6 ]. Particularly, congenital duodenal atresia is estimated to be present in approximately 3–6 % of neonates with oesophageal atresia, whether accompanied by a tracheoesophageal fistula (TEF) or not [ 5 , [7] , [8] , [9] ]. Furthermore, duodenal atresia has been observed with oesophageal atresia and anorectal malformations.…”