Oesophageal atresia with no distal tracheoesophageal fistula: Management and outcomes from a population-based cohort

Abstract

Purpose: To describe the incidence and outcomes to one-year in infants born with oesophageal atresia (OA) with no distal tracheoesophageal fistula within a population cohort.

Methods: A subgroup analysis of a prospective multicentre population cohort study was undertaken describing the outcomes of infants with OA and no tracheoesophageal fistula, (type A) and those with only an upper pouch fistula, (type B).

Main results: Twenty-one of 151 infants in the whole cohort were diagnosed with type A or B oesophageal atresia (14%). Fifteen were type A (71%) and six type B (29%). Infants with type B had a shorter gap length than those with type A: 2.5 vertebral bodies (2-3) vs. 5 (4-6) (p=0.008). All infants with type B OA underwent oesophageal anastomosis, 83% (n=5) as the primary procedure. All infants with type A, underwent staged management. Six (40%) had delayed primary anastomosis and eight required oesophageal replacement (53%). One infant died prior to reconstruction. The median time to delayed primary anastomosis in infants with type A or B OA was 82days (75-89days) (n=7). The median time to oesophageal replacement was 94days (89-147days) (n=8). Median length of stay for infants with type A or B OA from first operation to first discharge was 101days (31-123days).

Conclusions: Infants with type B OA had a shorter gap length and all were managed with oesophageal anastomosis. OA with no distal tracheoesophageal fistula is uncommon at a population level and frequently has a complex course.

Level of evidence: Rating: II.

Keywords: BAPS-CASS; Cohort study; Long gap OA; Oesophageal atresia; Tracheoesophageal fistula.