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A spectrum of bronchopulmonary anomalies associated with tracheoesophageal malformations
Insights
Congenital pulmonary and esophageal malformations are rare and often fatal. This study found that while these conditions complicate surgical care, most patients with associated bronchopulmonary anomalies survived.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Congenital Malformations
Background:
- Tracheoesophageal and pulmonary malformations present a rare but severe clinical challenge.
- Associated bronchopulmonary anomalies significantly increase the complexity and mortality risk.
Abstract:
The combination of tracheoesophageal and pulmonary malformations is unusual and reportedly carries a high mortality. We have observed six patients with esophageal atresia and tracheoesophageal fistula and one with a bronchoesophageal fistula who had associated bronchopulmonary anomalies ranging from lobar hypoplasia and agenesis to unilateral pulmonary hypoplasia or agenesis. All of the pulmonary malformations were right-sided. Vertebral or rib anomalies were present in five patients, congenital heart disease in two, imperforate anus in one and one patient had radial aplasia and Pierre Robin syndrome. The bronchopulmonary anomalies complicated the surgical care of tracheoesophageal malformations and required radiologic differentiation from aspiration pneumonia and atelectasis. Six of the seven patients survived. Mortality and morbidity were related to complications and associated cardiac anomalies as well as severity of the lung anomaly.