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

Autor: Morchio C; School of Pediatric Surgery, University of Florence, 50100 Florence, Italy., Ganarin A; Pediatric Surgery Unit, Ca' Foncello Hospital, 31100 Treviso, Italy., Conforti A; Neonatal Surgery Unit, Medical and Surgical Department of Fetus-Newborn-Infant, Bambino Gesù Children's Hospital, IRCCS, 00100 Rome, Italy., Leva E; Pediatric Surgery Unit, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, University of Milan, 20100 Milan, Italy., Gaglione G; UOC Pediatric Surgery Unit, AORN Santobono-Pausilipon, 80100 Naples, Italy., Brenco G; Pediatric Surgery Unit, IRCCS Giannina Gaslini's Hospital, 16100 Genova, Italy., Zambaiti E; Department of Pediatric General Surgery, Regina Margherita Children's Hospital, Azienda Ospedaliero Universitaria Città della Salute e della Scienza, 10100 Turin, Italy., Chiarenza SF; Department of Pediatric Surgery, San Bortolo Hospital, 36100 Vicenza, Italy., Caldaro T; Digestive Endoscopy and Surgery Unit, Bambino Gesu Children's Hospital, IRCCS, 00100 Rome, Italy., Cheli M; Pediatric Surgery Unit, Ospedale Papa Giovanni XXIII, 24100 Bergamo, Italy., Boroni G; Department of Paediatric Surgery, ASST Spedali Civili di Brescia, 25100 Brescia, Italy., Marcandella ES; Paediatric Surgery Unit, Women's and Children's Health Department, University of Padua, 35100 Padua, Italy., Riccipetitoni G; Department of Paediatric Surgery, 'V. Buzzi' Children's Hospital, 20100 Milan, Italy.; Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy., Cacciaguerra S; Department of Pediatric Surgery, Ospedale Garibaldi-Nesima, 95100 Catania, Italy., Di Benedetto V; Department of Pediatric Surgery, G. Rodolico-San Marco Hospital, 95100 Catania, Italy., Gentilino V; Division of Pediatric Surgery, Woman and Child Department, 'Filippo Del Ponte' Hospital, ASST Sette Laghi, 21100 Varese, Italy., Lisi G; Pediatric Surgery Unit, Santo Spirito Hospital, University of Chieti-Pescara, 65100 Pescara, Italy., Morini F; Department of Maternal and Child Health and Urological Sciences, La Sapienza University, 00100 Rome, Italy., Midrio P; Pediatric Surgery Unit, Ca' Foncello Hospital, 31100 Treviso, Italy.
Jazyk: angličtina
Zdroj: Children (Basel, Switzerland) [Children (Basel)] 2024 Apr 02; Vol. 11 (4). Date of Electronic Publication: 2024 Apr 02.
DOI: 10.3390/children11040423
Abstrakt: 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.
Databáze: MEDLINE