Unclassified bronchopulmonary foregut malformation in a 2-year-old girl

Background Bronchopulmonary foregut malformation (BPFM) is a rare congenital anomaly characterized by a fistula between an isolated portion of respiratory tissue and the esophagus or stomach. Srikanth et al. reported that BPFMs can be categorized into four groups, and that an unclassified BPFM is extremely rare. Herein, we present an unclassified BPFM group III and IV subtype in a 2-year-old girl. Case presentation At a gestational age of 36 weeks, a 1535-g female neonate was born as one of the dichorionic diamniotic twins. She had vertebral abnormality, anovestibular fistula, pulmonary artery sling, small right lung, mediastinal shift with dextrocardia, tracheal stenosis, and radial hemimelia. After birth, she was diagnosed with VACTERL association. She exhibited consolidation of the right upper lobe (RUL) in the neonatal period; however, she had no respiratory symptoms and was kept under observation in an outpatient visit. At 24 months, she was urgently admitted with acute pneumonia, and the upper gastrointestinal series revealed the right upper bronchus arising from the lower esophagus. Therefore, she underwent RUL resection. Intraoperatively, the right lung had no lobulations. RUL was ventilated by the esophageal bronchus (BPFM group III); however, the S6 lesion was ventilated by both the normal bronchial system and esophageal bronchus (close to BPFM group IV). The S6 lesion did not satisfy the definition of group IV as it did not have systemic blood supply. Hence, we decided to preserve the S6 lesion to save lung capacity as much as possible. The esophageal bronchus was transected using a 5-mm stapler. Due to lobulation failure, RUL was resected using an electric scalpel and 5-mm staplers along with the demarcation line by ventilation from the normal bronchus. The postoperative course was uneventful, and the patient could regain oral function without pneumonia or respiratory distress. Conclusions For neonates with repeated consolidation and pneumonia, BPFM must be considered as one of the differential diagnoses. Regarding BPFM treatment, early recognition and imaging are necessary. To determine the resection area of the lung, it is crucial to consider the segment of ventilation from the normal bronchi.