Esophageal Atresia and Associated Duodenal Atresia: A Cohort Study and Review of the Literature

Abstract

Introduction Esophageal atresia (EA) is associated with duodenal atresia (DA) in 3 to 6% of cases. The management of this association is controversial and literature is scarce on the topic. Materials and Methods We aimed to (1) review the patients with EA + DA treated at our institution and (2) systematically review the English literature, including case series of three or more patients. Results Cohort study: Five of seventy-four patients with EA had an associated DA (6.8%). Four of five cases (80%) underwent primary repair of both atresia, one of them with gastrostomy placement (25%). One of five cases (20%) had a delayed diagnosis of DA. No mortality has occurred. Systematic Review: Six of six-hundred forty-five abstract screened were included (78 patients). Twenty-four of sixty-eight (35.3%) underwent primary correction of EA + DA, and 36/68 (52.9%) underwent staged correction. Nine of thirty-six (25%) had a missed diagnosis of DA. Thirty-six of sixty-eight underwent gastrostomy placement. Complications were observed in 14/36 patients (38.9 ± 8.2%). Overall mortality reported was 41.0 ± 30.1% (32/78 patients), in particular its incidence was 41.7 ± 27.0% after a primary treatment and 37.0 ± 44.1% following a staged approach. Conclusion The management of associated EA and DA remains controversial. It seems that the staged or primary correction does not affect the mortality. Surgeons should not overlook DA when correcting an EA.

Improving prenatal detection of abdominal supraumbilical anomalies: The sonographic examination of fetal anechoic spaces of upper abdomen revisited

“…Spontaneous in utero gastric perforation has also been described in these cases 67 . The prognosis of duodenal obstruction in association with esophageal atresia is obviously worse than in cases of duodenal obstruction alone 68,69 . Due to the well‐known association between duodenal obstruction and trisomy 21, 16 all these cases should undergo prenatal aneuploidy testing.…”

Concomitant fetal duodenal and esophageal atresia: three‐dimensional reconstruction from magnetic resonance imaging

“…In a recent study evaluating EA associated with DA, only one in five cases of combined malformation was diagnosed prenatally. Of these five cases, two did not present with polyhydramnios despite having EA 7 . The failure to make a prenatal diagnosis may be due to the presence of associated TE fistula, which allows reduction of the pressure in the stomach, producing the double‐bubble sign, and reduces the possibility of formation of a distal esophageal pouch (caused by the obstructed distal dilated portion of the esophagus) which is observed in some cases.…”

“…Due to the scarce studies on the prenatal diagnosis of combined EA and DA, postnatal treatment is often based on case reports, and late (postnatal or postsurgical) diagnosis is likely, leading to lack of consensus on the optimal surgical approach in these cases 6 . Due to the complexity of the condition and late diagnosis, postoperative complications have been reported to occur in 38% and mortality in 41% of cases with concomitant EA and DA 7 .…”

Esophageal Atresia Associated with Congenital Duodenal Obstruction: Turkish Esophageal Atresia Registry (TEAR) Evaluation

“…In the presence of a distal fistula and duodenal obstruction, the risk of pulmonary aspiration is great because the stomach cannot be drained properly before esophageal surgery. 1 This article is protected by copyright. All rights reserved.…”

“…The incidence of esophageal atresia (EA) is 1 in 2.500 to 4.000 births and that of congenital duodenal obstruction is 1 in 5.000 to 10.000 births. 1,2 Both anomalies are known to be coexistent with congenital anomalies of other organ systems and management of both is relatively well-defined as isolated lesions. 3 Congenital duodenal obstruction has been reported to be present in approximately 3 to 6% of newborns with EA with or without tracheoesophageal fistula (TEF).…”