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Erschienen in:
01.12.2015 | Anatomic Bases of Medical, Radiological and Surgical Techniques
Communicating bronchopulmonary foregut malformation type IA: radiologic anatomy and clinical dilemmas
Erschienen in: Surgical and Radiologic Anatomy | Ausgabe 10/2015
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Background
Communicating bronchopulmonary foregut malformation (CBPFM) type IA is extremely rare and is associated with a high mortality rate. This malformation manifests with communication between the lung and the foregut, and this can lead to esophageal atresia and tracheoesophageal fistula (EA-TEF) to the distal pouch.
Purpose
To detail radiographic findings of CBPFM type IA cases and to summarize an appropriate therapeutic strategy for the management of this disorder.
Methods
Medical data for two patients with CBPFM type IA were retrospectively reviewed with regard to radiographic characteristics, therapy, and outcome.
Results
Both cases were initially misdiagnosed due to the presence of EA-TEF. Unusual atelectasis of the lateral lung was observed in chest radiographs, while non-aerated hypoplastic right lung and agenesis of the right main bronchus were detected by computed tomography. A final diagnosis was made by esophagogram. Only one patient survived following surgery.
Conclusion
CBPFM type IA is a rare condition and is extremely difficult to diagnose. However, CBPFM type IA should be suspected in patients manifesting EA and atelectasis of a unilateral lung on a chest radiograph. The decision to perform a pneumonectomy or bronchoplasty depends on the degree of exiting permitted due to pulmonary damage assessed by computed tomography.