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