Communicating Bronchopulmonary Foregut Malformation Type IB: Diagnostic and Surgical Challenges

Communicating bronchopulmonary foregut malformations (CBPFM) are extremely rare. We present a complex case of type IB CBPFM with esophageal atresia and distal tracheoesophageal fistula (EA/TOF), duodenal atresia/annular pancreas (DA/AP), and intestinal malrotation who underwent primary repair for EA/TOF on day 3. Bilious aspirates on day 8 prompted an upper gastrointestinal (GI) contrast revealing a duodenal obstruction and communication between the right lung lower lobe and the esophagus (T8-T9 level). DA/AP and malrotation were repaired by a gastrojejunostomy and Ladd's procedure. A repeat contrast swallow identified a 2nd communication from the esophagus into the right lower lobe (T5-T6 level) raising the suspicion of a recurrent TOF. Computed tomography (CT) thorax confirmed above findings with an anomalous blood supply to right lung. An exploratory thoracotomy identified a three-lobed lung. However, the lower lobe was enlarged and connected in two separate locations to the esophagus. The child recovered after the disconnection of the esophageal connections and partial right lower lobectomy. CBPFM are extremely rare anomalies requiring a high index of suspicion, use of an upper GI contrast series, and CT scans for diagnosis. The treatment of choice is resection of the affected lung and disconnection of the esophageal communications.