Tracheoesophageal fistula

Tracheoesophageal fistula
Updated: 02/20/2019
© Jun Wang, MD, PhD

General features
  • Either congenital or acquired
  • Commonly associated with esophageal atresia
  • May leads to severe and fatal pulmonary complications
  • Commonly associated with other developmental abnormalities
Pathogenesis
  • Tracheoesophageal septum abnormality
  • Incomplete separation of esophagus from laryngotracheal tube
Clinical presentations
  • May be asymptomatic if fistula is small
  • Presentation of esophageal atresia, if present: Polyhydramnios of mother, excess oral secretion, choke during suckling
  • Coughing and choking associated with feeding
  • Recurrent pneumonia
Key pathological features
  • Type A: Isolated esophageal atresia without fistula
  • Type B: Esophageal atresia with proximal fistula
  • Type C: Esophageal atresia with distal fistula
  • Type D: Esophageal atresia with proximal and distal fistula
  • Type E: Isolated fistula, may be referred to as “H type”
Vacterl syndrome
  • Vertebral abnormalities: hemivertebrae, scoliosis, rib deformities
  • Anorectal abnormalities: imperforate anus, cloacal deformities
  • Cardiac defects: ventricular septal defect, tetralogy of Fallot, etc
  • TracheoEsophageal fistula
  • Renal anomalies: agenesis, etc
  • Limb deformities: radial dysplasia, etc
  • Usually at least three of these abnormalities
Diagnosis
  • Clinical symptoms, radiologic studies
Treatment
  • Surgery



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