What are the symptoms of an infant born with Tracheoesophageal Fistula (TEF)?

Symptoms of Tracheoesophageal Fistula (TEF) in Newborn Infants

Infants born with TEF classically present with the triad of coughing, choking, and cyanosis during feeding, along with excessive oral secretions and respiratory distress from birth. 1, 2, 3

Primary Presenting Symptoms

Feeding-Related Symptoms

• Coughing and choking episodes during feeds occur in approximately 52% of infants, representing the most characteristic symptom 4
• Cyanosis during feeding manifests in 43.5% of cases as a result of aspiration of oral secretions or milk into the airway 4
• Excessive salivation and inability to handle secretions appears immediately after birth due to the blind-ending esophageal pouch in most TEF types 2

Respiratory Manifestations

• Recurrent pneumonia and respiratory infections develop in 69.6% of infants, often presenting as the initial manifestation 4
• Respiratory distress occurs from aspiration of gastric contents through the fistula into the trachea 2, 5
• Abdominal distension results from air entering the gastrointestinal tract through the fistula, particularly in type C (85% of cases) where the distal esophagus connects to the trachea 5, 3

Type-Specific Presentations

Type C (Most Common - 85% of Cases)

• Immediate inability to pass nasogastric tube due to blind-ending upper esophageal pouch 5
• Excessive drooling and pooling of secretions in the mouth and pharynx from birth 2
• "Three C's": Coughing, Choking, and Cyanosis with first feeding attempt 1, 3

H-Type or Isolated TEF (Less than 4% of Cases)

• Delayed presentation with symptoms appearing during the first month of life rather than immediately at birth 5
• Intermittent symptoms that may be less severe, leading to diagnostic delays 1
• Recurrent aspiration pneumonia as the predominant feature, often diagnosed later in infancy 3, 4

Associated Clinical Findings

Immediate Neonatal Period

• Atelectasis and pneumonia visible on chest imaging from aspiration 3
• Gas in the colon on abdominal radiographs indicating communication between trachea and distal esophagus 3
• Inability to advance feeding tube into the stomach in types with esophageal atresia 2

Airway Complications

• Tracheomalacia is the most frequently identified airway pathology, occurring in 37.4% to 89.2% of patients with EA-TEF, contributing to wheezing, cough, and respiratory symptoms 6, 7
• Laryngeal clefts account for 3.6-12% of additional airway pathologies and may cause aspiration symptoms 6
• Vocal cord paralysis occurs in 3-28% of infants, causing stridor, weak cry, and feeding difficulties 6

Critical Diagnostic Pitfalls

The diagnosis of H-type TEF is frequently delayed because symptoms are non-specific and intermittent. 1, 3 In 47.8% of cases, a single contrast study confirms the diagnosis, but 34.8% require two studies and 17.4% require three repeated studies before visualization of the fistula 4. The rarity of this condition combined with non-specific respiratory symptoms often results in significant delays between symptom onset and definitive diagnosis 1.

Physicians must maintain high clinical suspicion when infants present with the combination of feeding-related coughing and recurrent pneumonia, even if initial imaging is negative. 3, 4 Bronchoscopy can visualize the fistula in cases where contrast studies are inconclusive, with successful cannulation of the fistula achieved in 56.5% of cases 4.