Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey
Cecilia Morchio,
Alba Ganarin,
Andrea Conforti,
Ernesto Leva,
Giovanni Gaglione,
Gaia Brenco,
Elisa Zambaiti,
Salvatore Fabio Chiarenza,
Tamara Caldaro,
Maurizio Cheli,
Giovanni Boroni,
Elena Sofia Marcandella,
Giovanna Riccipetitoni,
Sebastiano Cacciaguerra,
Vincenzo Di Benedetto,
Valerio Gentilino,
Gabriele Lisi,
Francesco Morini,
Paola Midrio
Affiliations
Cecilia Morchio
School of Pediatric Surgery, University of Florence, 50100 Florence, Italy
Alba Ganarin
Pediatric Surgery Unit, Ca’ Foncello Hospital, 31100 Treviso, Italy
Andrea Conforti
Neonatal Surgery Unit, Medical and Surgical Department of Fetus-Newborn-Infant, Bambino Gesù Children’s Hospital, IRCCS, 00100 Rome, Italy
Ernesto Leva
Pediatric Surgery Unit, Fondazione IRCCS Ca’ Granda—Ospedale Maggiore Policlinico, University of Milan, 20100 Milan, Italy
Giovanni Gaglione
UOC Pediatric Surgery Unit, AORN Santobono-Pausilipon, 80100 Naples, Italy
Gaia Brenco
Pediatric Surgery Unit, IRCCS Giannina Gaslini’s Hospital, 16100 Genova, Italy
Elisa Zambaiti
Department of Pediatric General Surgery, Regina Margherita Children’s Hospital, Azienda Ospedaliero Universitaria Città della Salute e della Scienza, 10100 Turin, Italy
Salvatore Fabio Chiarenza
Department of Pediatric Surgery, San Bortolo Hospital, 36100 Vicenza, Italy
Tamara Caldaro
Digestive Endoscopy and Surgery Unit, Bambino Gesu Children’s Hospital, IRCCS, 00100 Rome, Italy
Maurizio Cheli
Pediatric Surgery Unit, Ospedale Papa Giovanni XXIII, 24100 Bergamo, Italy
Giovanni Boroni
Department of Paediatric Surgery, ASST Spedali Civili di Brescia, 25100 Brescia, Italy
Elena Sofia Marcandella
Paediatric Surgery Unit, Women’s and Children’s Health Department, University of Padua, 35100 Padua, Italy
Giovanna Riccipetitoni
Department of Paediatric Surgery, “V. Buzzi” Children’s Hospital, 20100 Milan, Italy
Sebastiano Cacciaguerra
Department of Pediatric Surgery, Ospedale Garibaldi-Nesima, 95100 Catania, Italy
Vincenzo Di Benedetto
Department of Pediatric Surgery, G. Rodolico—San Marco Hospital, 95100 Catania, Italy
Valerio Gentilino
Division of Pediatric Surgery, Woman and Child Department, “Filippo Del Ponte” Hospital, ASST Sette Laghi, 21100 Varese, Italy
Gabriele Lisi
Pediatric Surgery Unit, Santo Spirito Hospital, University of Chieti-Pescara, 65100 Pescara, Italy
Francesco Morini
Department of Maternal and Child Health and Urological Sciences, La Sapienza University, 00100 Rome, Italy
Paola Midrio
Pediatric Surgery Unit, Ca’ Foncello Hospital, 31100 Treviso, Italy
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.
