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Congenital broncho-esophageal fistula: A case report
Barry M Linnane1, Gerard Canny
1Department of Respiratory Medicine, Royal Children's Hospital, Flemington Road, Parkville, Vic. 3052, Melbourne, Australia. barry.linnane@rch.org.au
Insights
This case study details a rare congenital anomaly, bronchopulmonary foregut malformation, in an infant with unique limb and kidney defects. Surgical intervention successfully treated the broncho-esophageal fistula and sequestered lobe.
Area of Science:
- Congenital malformations
- Pediatric surgery
- Embryology
Background:
- Bronchopulmonary foregut malformations (BPFMs) are rare congenital anomalies impacting the respiratory and gastrointestinal tracts.
- Their embryological origins remain debated due to the infrequency of documented cases.
Observation:
- A female infant presented with a malformed right arm, absent right kidney, and aplasia of the right lung.
- At five months, she developed severe respiratory distress, revealing a right broncho-esophageal fistula via contrast study.
- Surgical findings confirmed complete aplasia of the right lung and a fistula connecting the lower esophagus to a sequestered right lobe.
Findings:
- Successful surgical resection of the sequestered right lobe and ligation of the broncho-esophageal fistula were performed.
- The infant recovered well, remaining asymptomatic 13 months post-operation.
- This case, the twelfth documented BPFM, is unique due to associated renal and limb malformations.
Implications:
- The unique combination of anomalies supports the vascular insult theory for the underlying cause of lung maldevelopment.
- This case highlights the importance of early diagnosis and surgical management of BPFMs.
- Further research into the embryological origins of BPFMs is warranted.
Abstract:
Bronchopulmonary foregut malformations are a group of rare congenital anomalies affecting the respiratory and upper gastrointestinal tract. The rarity of these cases means their embryological origin continues to be a source of controversy. We present the case of a female infant, born at term with a malformed right arm, an absent right kidney and aplasia of the right lung. Although initially asymptomatic she presented at 5 months of age in severe respiratory distress. An upper gastro-intestinal contrast study demonstrated a right broncho-esophageal fistula. At surgical resection it was discovered that the right main bronchus ended abruptly just beyond the carina, with total aplasia of the right lung. A bronchoesophageal fistula originating from the lower third of the esophagus communicated with a sequestered right lobe. The lobe was removed and the fistula ligated. The infant remains well 13 months post surgery. This represents only the twelfth case of this rare form of bronchopulmonary foregut malformation. The associated renal and limb malformations make this case unique and may add weight to the theory that the underlying insult to the developing lung is vascular in origin.
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