Oesophageal atresia is a blind-ending oesophagus resulting from congenital closure or absence of the oesophageal lumen. This condition must be identified and corrected within 48 hours to increase the chance of survival. More than 85% of oesophageal atresia cases are associated with a fistula to the trachea (trachea-oesophageal fistula).
Epidemiology
- 1case in 2500-3000 live births.
- There is a 2 % risk of recurrence when sibling is affected.
- Increased in advanced maternal age.
- 50% have one or more associated anomalies (skeletal/vertebral, genitourinary, cardiac, anorectal and others).
Embryology
- The esophagus and trachea are derivatives of the primitive foregut.
- A ventral outpouching is formed from the caudal part of the primitive pharynx. This is the laryngotracheal diverticulum.
- The tracheoesophageal septum is formed by the fusion of the longitudinal tracheoesophageal folds.
- In week 4 – There is a failure of separation in the tracheoesophageal septum leading to the formation of a fistula.
- In week 8- There is a failure of recanalization of the primitive gut leading to the formation of oesophageal atresia.
Classification
- Type A – Esophageal atresia without tracheoesophageal fistula.
- Type B – Esophageal atresia with proximal tracheoesophageal fistula.
- Type C – Esophageal atresia with distal tracheoesophageal fistula (most common type).
- Type D – Esophageal atresia with both proximal and distal tracheoesophageal fistula.
- Type E – Tracheoesophageal fistula without esophageal atresia (H-type).
Associated anomalies
- Cardiovascular defects
- Vertebral defects
- Anorectal malformations
- Limb deformities
- Renal anomalies
Clinical features
- Polyhydramnios prenatally.
- Excessive oral secretions.
- Regurgitation of every feed.
- Choking and cyanosis following the first feed.
- Respiratory compromise.
- Aspiration pneumonia.
Diagnosis
- Inability to passa nasogastric tube
- A coiled nasogastric tube in the upper oesophagus is seen on the x-ray.
Treatment
- The initial treatment goal is to prevent or mitigate the sequelae of aspiration.
- Place the child in an upright position
- Suction the blind pouch
- Administer prophylactic antibiotics
- Definitive treatment is by surgical correction via a thoracotomy.
