Oesophageal atresia and tracheoesophageal fistula are congenital anomalies, occurring during the 4th week of gestation due to failure of foregut separation into trachea and oesophagus.
An oesophageal atresia is a blind-ending oesophagus, while a tracheoesophageal fistula is an abnormal connection between the trachea and the oesophagus. They can be part of other midline congenital anomalies, such as the VACTERL association and CHARGE syndrome.
Classification
| Type | Description |
|---|---|
| Type A (8%) | Oesophageal atresia only |
| Type B (1%) | Oesophageal atresia and proximal tracheoesophageal fistula |
| Type C (84%) | Oesophageal atresia and distal tracheoesophageal fistula |
| Type D (3%) | Oesophageal atresia, and proximal and distal tracheoesophageal fistula |
| Type E (4%) | Tracheoesophageal fistula only (H-type) |
- Causes
- Genetic associations: SHH, SOX2, CHD7, MYCN, FANCB
- Associated conditions
- VACTERL association
- CHARGE syndrome
- Signs and symptoms
- Respiratory distress
- Shortly after birth
- Worsens with feeding
- Regurgitation
- Excessive salivation
- Drooling due to proximal atresia
- Choking with feeding when there is a fistula (types B and D)
- Gastric distension and gastric perforation due to distal tracheoesophageal fistula (types C and D)
- Especially with mechanical ventilation
- Signs of aspiration pneumonia
- Fever
- Tachypnea
- Adventitious lung sounds
- Failure to pass a nasogastric tube
- Mother has a history of polyhydramnios with proximal atresia
- A physical examination of the anus and genitalia is required to screen for associated anomalies
- Respiratory distress
- Differentials
- Oesophageal strictures or webs
- Laryngotracheoesophageal cleft
- Oesophageal duplication
- Tracheal agenesis
- Severe GERD
- Investigations
- Chest or Abdominal X-ray: This is the best initial test
- Blind upper pouch (92%, the nasogastric tube is coiled before reaching the stomach
- Absent gastric bubble
- Air in the stomach suggests a distal tracheoesophageal fistula
- Upper gastrointestinal contrast studies to diagnose type E (H-type) fistulas
- Echocardiogram to screen for concurrent heart defects, since it is associated with the VACTERL sequence
- Renal function tests
- Renal ultrasound
- Spine and limb X-rays
- Chest or Abdominal X-ray: This is the best initial test
- Treatment
- Airway protection
- Intubation if indicated
- Continuous suction of the upper pouch
- Nil per os
- Intravenous fluid
- Intravenous antibiotics
- Intravenous nutrition or gastrostomy
- Consider total parenteral nutrition
- Surgical repair
- Staged repair for low birth weight (< 1500 g): fistula ligation → oesophageal atresia repair
- Fistula ligation or primary esophageal anastomosis
- Foker technique (traction lengthening) and esophagela replacement for a large gap
- NICU care
- Oesophagogram at 5 – 7 days
- Feeds can be started if there are no leaks
- Airway protection
- Complications
- Anastomotic leak
- Recurrent fistula
- Recurrent laryngeal nerve injury
- Oesophageal stricture
- GERD
- Tracheomalacia
- Dysphagia
- Recurrent respiratory infections
- Barrett’s oesophagus and oesophageal cancer