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Bronchopulmonary foregut malformation presenting as extralobar pulmonary sequestration associated with a bronchogenic cyst: an unusual clinical and radiological feature in an adolescent patient.

ABSTRACT: We present a case of symptomatic complex bronchopulmonary foregut malformation (BPFM), including extralobar pulmonary sequestration and a bronchogenic cyst, in the left anterior mediastinum of a 15-year-old boy. Preoperative computed tomography showed a cystic mass with heterogeneous enhancement of adjacent soft tissue components and pleural effusion. We suggested the infected bronchogenic cyst as the first impression. However, pathological examination after surgical resection revealed extralobar pulmonary sequestration and a bronchogenic cyst with unusual manifestation, which was located in the left upper hemithorax and supplied by the pulmonary artery. In patients presenting with a cystic mass with features of inflammation or infection and collateral vasculature, the possibility of a complex bronchopulmonary foregut malformation should be considered in the differential diagnosis.

SUBMITTER: Choo JY

PROVIDER: S-EPMC5542949 | biostudies-other | 2017 Jul

REPOSITORIES: biostudies-other

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Project description:BackgroundA communicating bronchopulmonary foregut malformation (CBPFM) group IB is very rare congenital malformation. Group IB is associated with tracheoesophageal fistula and esophageal atresia (TEF-EA) and a portion of one lung arisen from the esophagus (Gerle et al. in N Engl J Med. 278:1413-1419, 1968). The coexistence of TEF-EA and dextrocardia is also a rare and challenging setting for repair of TEF-EA. Therefore, the thoracoscopic surgery for TEF-EA require the technical devise because of the small operative space. We herein report a rare case of CBPFM group IB with intralobar sequestration of lung and a successful performing of thoracoscopic surgery for EA with dextrocardia in VACTERL association.Case presentationA 2.2-kg term male neonate was born with an anal atresia, coarctation of the aorta, TEF-EA, renal anomalies, radial hemimelia, limb abnormalities (VACTERL association) and hypoplasia of the right lung with dextrocardia. The patient developed respiratory distress after admission. A two-stage operation for the TEF-EA was planned because of multiple anomalies and cardiac condition. In the neonatal period, esophageal banding at the gastroesophageal junction and gastrostomy were performed to establish enteral nutrition. After gaining body weight and achieving a stable cardiac condition, thoracoscopic surgery for TEF-EA was performed. The thoracoscopic findings revealed a small working space due to dextrocardia. To obtain a sufficient working space and to perform secure esophageal anastomosis, an additional 3-mm assistant port was inserted. To close the upper and lower esophagus, anchoring sutures of the esophagus were placed and were pulled to suspend the anastomotic site. Esophageal anastomosis was successfully performed. An esophagogram after TEF-EA surgery showed the connection between the lower esophagus and right lower lung. The definitive diagnosis was CBPFM group IB with intralobar sequestration. The thoracoscopic surgery was performed again for establishing oral intake. After transection of the bronchoesophageal fistula, the patient could perform oral feeding without pneumonia or respiratory distress.ConclusionsCBPFM type IB with intralobar sequestration is a rare condition. CBPFM type IB should be considered for a patients with respiratory symptom after radical operation for TEF-EA. In the present case, suspending the anastomotic site was effective and useful in thoracoscopic surgery for a TEF-EA patient with dextrocardia.

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Bronchopulmonary foregut malformation presenting as extralobar pulmonary sequestration associated with a bronchogenic cyst: an unusual clinical and radiological feature in an adolescent patient.

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