H-type congenital tracheoesophageal fistula: Insights from 70 years of The Royal Children's Hospital experience

J Pediatr Surg. 2021 Apr;56(4):686-691. doi: 10.1016/j.jpedsurg.2020.06.048. Epub 2020 Jul 11.

Abstract

Background: The long-term outcomes of H-type tracheoesophageal fistula (TOF), an uncommon variant of esophageal atresia/tracheoesophageal fistula (OA/TOF), are rarely described in the literature. We reviewed our institutional experience of 70 years.

Methods: The Nate Myers Oesophageal Atresia Database was queried for patients with an H-type TOF (1948-2017). Data included presentation, diagnostic workup, surgical management, and outcomes.

Results: Of 1088 patients with OA/TOF, 56 (5.1%) had an H-type TOF. The most common presenting symptoms were cyanotic episodes (68%), choking with feeds (52%), and aspiration pneumonitis (46%). The majority (82%) were symptomatic in the first week of life. Coexisting congenital anomalies were present in 46%: cardiac (13/56, 23%), genitourinary (10/56, 18%), and vertebral/skeletal (9/56, 16%). Patients were consistently diagnosed with prone contrast tube esophagogram (77% sensitivity on the first study and 96% after a second study). The fistula was most commonly approached through a right cervical collar incision. Right vocal cord palsy occurred in 22%, with one case of bilateral palsies. Other complications included leak (5.6%), recurrence (9.3%), stricture (1.9%), and diverticulum (1.9%). Although there was a trend towards a lower recurrence rate when interposition material was used, this was not statistically significant (3.3% vs 16.7%, p = 0.16). Survival in operative cases was 98.2%, and when all diagnosed cases were considered was 89.3%.

Conclusions: We have reported the largest single-center series of H-type TOF. Diagnosis is challenging, and surgical morbidity remains high. Despite this, long-term outcomes are favorable.

Level of evidence: IV.

Keywords: Esophageal atresia; H-type fistula; Tracheoesophageal fistula.

MeSH terms

  • Child
  • Esophageal Atresia* / complications
  • Esophageal Atresia* / surgery
  • Hospitals
  • Humans
  • Retrospective Studies
  • Spine
  • Tracheoesophageal Fistula* / epidemiology
  • Tracheoesophageal Fistula* / surgery