Esophageal atresia in patients with anorectal malformations

The presence of esophageal atresia (EA) in patients with an anorectal malformation (ARM) is well known. The purpose of this work is to find out the most common type of ARM associated to EA and the functional prognostic implication of this association, which has not been described in previous publications.

We reviewed our database for demographic, functional, and associated anomalies data in our patients with EA and ARM, and then compared them with those of our general series of ARM without esophageal atresia.

Out of 1,995 ARM patients, 167 had a concomitant EA (8.3 %). Prostatic fistula was the most common type of defect in the male EA patients (45.9 %) and cloacas were on the female group (57.9 %). EA patients had worse bowel (47 vs. 67 %) and urinary control (56.6 vs. 79.4 %) when compared to the general series (GS). Functional prognosis was significantly worse in cloacas and in patients subjected to re-operations (p < 0.001). EA patients had a 0.52 average sacral ratio and in the GS was 0.65 (p < 0.001). EA patients had a significantly higher incidence of tethered cord (32.3 vs. 17.6 %), cardiac anomalies (32.3 vs. 22.5 %) including VSD (12.5 vs. 4.5 %), hydronephrosis (36.5 vs. 15.4 %), absent kidney (26.3 vs. 10.5 %), duodenal atresia (7.7 vs. 1.7 %), vertebral anomalies (28.1 vs. 14 %), extremity defects (11.3 vs. 3.1 %), tracheal anomalies (6.5 vs. 0.4 %), and developmental delay (5.9 vs. 1.4 %).

The presence of esophageal atresia in ARM patients has a significant, probably coincidental, impact on bowel and urinary control. This association is also related with worse types of ARM defects and with more severe associated anomalies. This association should increase the awareness on the provider in terms of what to expect on functional prognosis and a throughout search for associated anomalies.