Congenital bronchopleural fistula in a newborn: a rare cause of persistent neonatal air leak - a case report.

BACKGROUND: Congenital bronchopleural fistula is an exceptionally rare cause of persistent neonatal air leakage. Most bronchopleural fistulas in newborns are related to mechanical ventilation, infection, or iatrogenic injury. The clinical presentation can suggest pneumothorax commonly seen in premature infants, diagnosis is often delayed, and an ongoing uncontrolled air leak may significantly compromise effective ventilation. CASE PRESENTATION: A female neonate was delivered at 29 weeks of gestation with a birth weight of 1,360 g and Apgar scores of 6, 7, and 9. Immediately after birth, she developed progressive respiratory failure. Bilateral pneumothoraces were diagnosed, including a left tension pneumothorax. Despite two pleural drains and active suction, a continuous air leak persisted at approximately 800 mL/min, corresponding to the infant's entire minute ventilation. Chest computed tomography demonstrated a communication between the left bronchial tree and the pleural cavity, consistent with a bronchopleural fistula. Dedicated CT angiography was not performed because of deterioration and the need to avoid delaying definitive treatment. Selective bronchial intubation, temporary bronchial occlusion with a Fogarty catheter, and minimally invasive options were considered. Owing to respiratory instability despite drainage, surgical treatment was selected. Thoracoscopy identified the lesion, but secure closure could not be achieved thoracoscopically; the procedure was converted to thoracotomy, and the fistula was excised with a segment of adjacent lung tissue. Postoperative bronchoscopy revealed tracheobronchial stenosis and left main bronchial malacia, which were managed conservatively. Genetic testing identified PAI-1/SERPINE1 and MTHFR variants, but these did not establish a recognised syndrome or causal association with the fistula. The postoperative respiratory course was favourable. At 18 months of age, the child had no recurrent pneumothorax, chronic respiratory symptoms, or late surgical complications. CONCLUSION: Persistent high volume air leakage despite adequate pleural drainage should lead to suspect a major bronchial lesion and investigate congenital pulmonary and airway malformations. Conservative and bronchoscopic strategies may be effective in selected stable patients, whereas early surgery may provide definitive control in rapidly deteriorating neonates with a localised structural lesion. This case highlights the diagnostic value of CT, the need for individualised treatment selection, and the possibility of sustained long-term recovery after surgical repair.