Congenital H-type tracheoesophageal fistula: A multicenter review of outcomes in a rare disease

Sara C. Fallon, Jacob C. Langer, Shawn D. St. Peter, Kuo Jen Tsao, Caroline M. Kellagher, Dave R. Lal, Jill S. Whitehouse, Diana L. Diesen, Michael D. Rollins, Elizabeth Pontarelli, Marcus M. Malek, Corey W. Iqbal, Jeffrey S. Upperman, Charles M. Leys, Mark L. Wulkan, Sarah J. Hill, Martin L. Blakely, Timothy D. Kane, David E. Wesson

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24 Scopus citations


Objective To perform a multicenter review of outcomes in patients with H-type tracheoesophageal fistula (TEF) in order to better understand the incidence and causes of post-operative complications. Background H-type TEF without esophageal atresia (EA) is a rare anomaly with a fundamentally different management algorithm than the more common types of EA/TEF. Outcomes after surgical treatment of H-type TEF are largely unknown, but many authoritative textbooks describe a high incidence of respiratory complications. Methods A multicenter retrospective review of all H-type TEF patients treated at 14 tertiary children's hospital from 2002–2012 was performed. Data were systematically collected concerning associated anomalies, operative techniques, hospital course, and short and long-term outcomes. Descriptive analyses were performed. Results We identified 102 patients (median 9.5 per center, range 1–16) with H-type TEF. The overall survival was 97%. Most patients were repaired via the cervical approach (96%). The in-hospital complication rate, excluding vocal cord issues, was 16%; this included an 8% post-operative leak rate. Twenty-two percent failed initial extubation after repair. A total of 22% of the entire group had vocal cord abnormalities (paralysis or paresis) on laryngoscopy that were likely because of recurrent laryngeal nerve injury. Nine percent required a tracheostomy. Only 3% had a recurrent fistula, all of which were treated with reoperation. Conclusions There is a high rate of recurrent laryngeal nerve injury after H-type TEF repair. This underscores the need for meticulous surgical technique at the initial repair and suggests that early vocal cord evaluation should be performed for any post-operative respiratory difficulty. Routine evaluation of vocal cord function after H-type TEF repair should be considered. The level of evidence rating Level IV.

Original languageEnglish (US)
Pages (from-to)1711-1714
Number of pages4
JournalJournal of Pediatric Surgery
Issue number11
StatePublished - Nov 2017


  • Congenital esophageal anomaly
  • Neonatal
  • Pediatric
  • Recurrent laryngeal nerve injury
  • Tracheoesophageal fistula

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health


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