Congenital H-type tracheoesophageal fistula: A multicenter review of outcomes in a rare disease.
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.
Journal of pediatric surgery
Child, Preschool; Esophagoplasty; Female; Humans; Incidence; Infant; Infant, Newborn; Laryngoscopy; Male; Postoperative Complications; Rare Diseases; Recurrence; Recurrent Laryngeal Nerve Injuries; Reoperation; Retrospective Studies; Tracheoesophageal Fistula; Tracheostomy
TEF; Congenital esophageal anomaly; Neonatal; Pediatric; Recurrent laryngeal nerve injury; Tracheoesophageal fistula
Fallon, Sara C.; Langer, Jacob C.; St Peter, Shawn D.; Tsao, KuoJen; Kellagher, Caroline M.; Lal, Dave R.; Whitehouse, Jill S.; Diesen, Diana L.; Rollins, Michael D.; Pontarelli, Elizabeth; Malek, Marcus M.; Iqbal, Corey W.; Upperman, Jeffrey S.; Leys, Charles M.; Wulkan, Mark L.; Hill, Sarah J.; Blakely, Martin L.; Kane, Timothy D.; and Wesson, David E., "Congenital H-type tracheoesophageal fistula: A multicenter review of outcomes in a rare disease." (2017). Manuscripts, Articles, Book Chapters and Other Papers. 834.