Bronchopleural fistula in a 5- years old child with novel CARMIL 2 mutation: A rare disease and a rare case

A five year girl had eczema and allergic rhinitis in the past, presented with a history of cough, shortness of breath for the last one month. Her chest -X-ray showed a left side pleural effusion, and a computed tomographic scan (CT) of the chest showed left side hydropneumothorax. Left side 21 Fr drain was inserted. Her clinical condition deteriorated despite antimicrobial therapy, and she required mechanical ventilatory support due to respiratory distress. She also developed a right-sided pneumothorax that was managed by inserting a 21 Fr chest drain. A video-assisted thoracoscopic VATS procedure was done to staple the lung bullae and drain the empyema. Her post-operative chest X-ray showed good lung expansion. Pleural fluid culture report was positive for candida. She was commenced on antifungal microbial therapy. Two days later, she developed again left side pneumothorax, which was again managed by left intercostal drain. We were unable to wean her off from mechanical ventilatory support due to a significant air leak due to bronchopleural fistula. A posterolateral thoracotomy was performed, and the bronchopleural fistula was closed. She was extubated the next day, and intercostal drains were removed on the 4th post-operative day.