Diagnostic and management strategies for congenital H-type tracheoesophageal fistula: a systematic review

Pediatr Surg Int. 2021 May;37(5):539-547. doi: 10.1007/s00383-020-04853-3. Epub 2021 Jan 20.


Background: H type tracheoesophageal fistula (H-TEF) is a rare congenital anomaly. Management may be complicated by late diagnosis and variation(s) in the therapeutic strategy. A systematic review of published studies explores the utility of diagnostic studies, operations and postoperative complications.

Methods: Medline and PubMed database(s) were searched for ALL studies reporting H-TEF during 1997-2020. Using PRISMA methodology, manuscripts were screened for eligibility and reporting.

Results: Forty-seven eligible studies were analysed. Primary diagnosis varied widely with surgeons performing oesophagography and trachea-bronchoscopy. Preoperative localisation techniques included fluoroscopy, guidewire placement and catheterisation. A cervical approach (209 of 272 cases), as well as thoracotomy, thoracoscopy and endoscopic fistula ligation, were all described. Morbidity included fistula recurrence (1.7%), leak (2%), tracheomalacia (3.4%) and respiratory sequelae (1%). The major adverse complication in all studies was vocal cord palsy secondary to laryngeal nerve injury (18.5%) yet strikingly few centres routinely reported undertaking vocal cord screening pre or postoperatively.

Conclusion: This study shows that paediatric surgeons record low volume activity with H type tracheoesophageal fistula. Variation(s) in clinical practice are widely evident. Laryngeal nerve injury and its subsequent management warrant special consideration. Care pathways may offset attendant morbidity and define 'best practice.'

Keywords: Clinical outcomes; Congenital oesophageal anomaly; H type tracheoesophageal fistula; Newborns; Paediatric; Recurrent laryngeal nerve injury.

Publication types

  • Systematic Review

MeSH terms

  • Bronchoscopy
  • Female
  • Humans
  • Infant, Newborn
  • Male
  • Postoperative Complications / etiology
  • Postoperative Period
  • Recurrent Laryngeal Nerve Injuries / etiology*
  • Retrospective Studies
  • Thoracoscopy
  • Thoracotomy / adverse effects
  • Trachea / surgery
  • Tracheoesophageal Fistula / diagnosis*
  • Tracheoesophageal Fistula / therapy*
  • Tracheomalacia