H-type congenital tracheoesophageal fistula: Insights from 70 years of The Royal Children's Hospital experience
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In brief
In brief
Retrospective review of 56 H-type tracheoesophageal fistula cases over 70 years demonstrates diagnostic challenges requiring repeat contrast studies and high surgical morbidity including 22% vocal cord palsy rate. Despite complications, long-term survival exceeds 89% with favorable outcomes when managed through cervical approach.
Written by the GCMD Library team from the article.
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