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. 2024 Apr 2;11(4):423.
doi: 10.3390/children11040423.

Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey

Cecilia Morchio  1 , Alba Ganarin  2 , Andrea Conforti  3 , Ernesto Leva  4 , Giovanni Gaglione  5 , Gaia Brenco  6 , Elisa Zambaiti  7 , Salvatore Fabio Chiarenza  8 , Tamara Caldaro  9 , Maurizio Cheli  10 , Giovanni Boroni  11 , Elena Sofia Marcandella  12 , Giovanna Riccipetitoni  13   14 , Sebastiano Cacciaguerra  15 , Vincenzo Di Benedetto  16 , Valerio Gentilino  17 , Gabriele Lisi  18 , Francesco Morini  19 , Paola Midrio  2
Affiliations

Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey

Cecilia Morchio et al. Children (Basel). .

Abstract

Background: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF.

Methods: Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010-2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR).

Results: The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day-18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%.

Conclusions: H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

Keywords: congenital tracheoesophageal fistula; h-type esophageal atresia; type E esophageal atresia.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Tracheoesophageal fistula is recognized during tracheoscopy (bulging in the posterior tracheal wall) (A). The fistula is cannulated (B), and then an esophagoscopy is performed to retrieve the wire (C). Right cervicotomy (black dashed line) is performed (D); the guide wire (black arrows), exiting from the mouth, helps recognize the tracheoesophageal fistula.
See this image and copyright information in PMC

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