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Esophageal lung with multiple congenital anomalies: conundrums in diagnosis and management
T C Saydam1, G B Mychaliska, M R Harrison
1Department of Surgery, The Fetal Treatment Center, University of California San Francisco, 94143-0570, USA.
Insights
This case highlights a rare combination of esophageal atresia and a distal tracheoesophageal fistula with a bronchopulmonary foregut malformation involving the entire left lung. Accurate anatomical delineation is crucial for surgical planning in such complex congenital anomalies.
Area of Science:
- Pediatric Surgery
- Congenital Malformations
- Thoracic Surgery
Background:
- Communicating bronchopulmonary foregut malformations (CBPFM) represent complex congenital anomalies.
- Accurate anatomical delineation is challenging in these disorders.
- This case presents diagnostic and management conundrums in CBPFM.
Observation:
- A neonate presented with esophageal atresia, tracheoesophageal fistula, and tetralogy of Fallot.
- Initial management involved fistula occlusion and ligation.
- Unexpected left lung inflation via gastrostomy tube suggested an esophageal origin of the left mainstem bronchus.
Findings:
- Autopsy revealed a unilobed left lung arising from the esophagus, esophageal atresia, tracheoesophageal fistula, atretic left mainstem bronchus, tetralogy of Fallot, and DiGeorge syndrome.
- This represents the first reported instance of this specific combination of anomalies.
- Despite surgical intervention, the patient's condition was not salvageable.
Implications:
- Complex CBPFM requires meticulous anatomical assessment for effective surgical planning.
- This case underscores the variability and severity of foregut malformations.
- Further research into the embryology and surgical approaches for rare CBPFM is warranted.
Background/Purpose:
Communicating bronchopulmonary foregut malformations (CBPFM) are a diverse group of potentially devastating congenital anomalies with anatomy that may be difficult to delineate. The authors present a case that illustrates conundrums in the diagnosis and management of these complex disorders.
Methods:
A term baby had esophageal atresia (EA), tracheoesophageal fistula (TEF), and tetralogy of Fallot. Initially, a gastrostomy was performed, and a balloon catheter was inserted through the endotracheal tube to occlude the fistula until the patient was hemodynamically stable. Subsequently, the fistula was ligated. Postoperatively, the left lung collapsed, and bronchoscopy showed an atretic left mainstem bronchus. Repeat thoracotomy showed that the fistula ligation was intact. Air was introduced through the gastrostomy tube, and, surprisingly, the left lung inflated, indicating the left mainstem bronchus arose from the esophagus distal to the ligated TEF.
Results:
Despite reopening this fistula, ventilation remained poor, and support was withdrawn. Autopsy findings confirmed a unilobed left lung arising from the esophagus, EA, TEF, an atretic left mainstem bronchus, tetralogy of Fallot, and DiGeorge syndrome.
Conclusions:
This is the first report of a combination of EA and distal TEF with a second CBPFM involving the esophagus and the entire left lung. Successful correction of these anomalies will require extensive delineation of the anatomy to plan an operative strategy.