Case report
Triad of tracheoesophageal fistula-esophageal atresia, pulmonary hypoplasia, and duodenal atresia

https://doi.org/10.1016/j.jpedsurg.2007.01.044Get rights and content

Abstract

We describe 3 cases of tracheoesophageal fistula with esophageal atresia who also had right pulmonary hypoplasia-aplasia and duodenal atresia. We review the current literature and propose that this may be a distinct association.

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Case 1

Case 1 was delivered in good condition at 37 weeks of gestation. Birth weight was 2864 g. Attempts to pass a nasogastric tube (NGT) were unsuccessful. A combined chest and abdominal x-ray (Fig. 1) demonstrated the NGT in the upper esophageal pouch, a large gastric bubble with no distal bowel gas, and opacification of the right lung field. This was consistent with the diagnoses of TEF/EA, DA, and right lung hypoplasia. The TEF/EA and DA were repaired surgically on day 1.

Ventilatory support was

Case 2

Case 2 was a twin delivered electively at 28 weeks of gestation for discordant growth. Birth weight was 808 g. She was intubated and received surfactant. Nasogastric tube could not be passed. Chest and abdominal films demonstrated the NGT in the upper esophagus, gas in the stomach, opacification of the right hemithorax and mediastinal shift, suggestive of TEF/EA, and right lung hypoplasia. She underwent TEF/EA repair on day 2 of life.

Feeds were subsequently introduced but were not tolerated and

Case 3

Case 3 was born at 37 weeks of gestation (twin 2) in 1973. An NGT could not be passed, consistent with EA. Combined abdominal and chest x-rays showed a “double bubble,” suggesting DA and complete opacification of the right hemithorax. He was intubated and ventilated.

Respiratory problems prevented primary surgical repair. A laparotomy was performed on day 3 of life to insert a decompressing gastrostomy and feeding jejunostomy.

A right thoracotomy on day 7 revealed the heart in the right side of

Discussion

The association of TEF/EA with other congenital abnormalities, particularly as part of the VACTERL and CHARGE spectra, is well recognized. Less common is its association with both DA and pulmonary hypoplasia.

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