- Affects 1 in 3000 live births
- Aetiology is unknown but incidence is increased in first degree relatives
- Often associated with a trachea-oesophageal fistula (TOF)
- Various presentations include:
- Oesophageal atresia with TOF - 85%
- Isolated oesophageal atresia - 8%
- Isolated TOF - 4%
- Oesophageal atresia with proximal and distal TOF
- 50% of patients have other congenital abnormalities
- Usually involving the cardiovascular, urogenital or anorectal systems
Clinical features
- Prenatally diagnosed by the finding of polyhydramnios
- Stomach is empty on ultrasound
- Postnatally diagnosed by the neonate drooling or unable to swallow
- Cyanosed during feeding
- Develop aspiration pneumonia
- A 10 Fr nasogastric tube can not be passed more than 10 cm
- On chest x-ray if there is gas in the stomach the is a distal TOF
Management
- Feeding is withheld and suction applied to oesophageal pouch
- Nursed in upright position
- Associated congenital abnormalities are identified
- Surgery required within first 24 hours of life
- Operation involves:
- Right thoracotomy and extrapleural approach
- Azygos vein is divided
- TOF divided
- Oesophagus mobilised and primary anastomosis is usually achieved
- If anastomosis impossible a staged procedure required
- Gastrostomy performed and fistula divided at initial operation
- Oesophagus replaced by colon or stomach after a few months
Complications
- Oesophageal dysfunction
- Dilated proximal pouch
- Gastro-oesophageal reflux
- Anastomotic stricture
- Recurrent fistula
Bibliography
del Rosario J F, Orenstein S R.
Common pediatric esophageal disorders. Gastroenterologist
1998; 6: 104-121.
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