Background Pulmonary Langerhans's cell
histiocytosis (PLCH) is a rare cause of
interstitial lung disease in children and more than half of the cases are bilateral. Persistent respiratory distress due to
spontaneous pneumothorax (SP) in bilateral PLCH may refractory to
conservative treatment and posed a great challenge to surgical modalities. A 3-year-old boy with SP due to bilateral PLCH is presented to discuss the surgical options of recurrent and refractory PLCH cases in children. Case Report The patient was admitted to the emergency department with severe respiratory distress and SP. After
chest tube insertion, biopsy from neck mass revealed Langerhans's cell
histiocytosis.
Chemotherapy including
vinblastine and
prednisone was initiated. Due to persistent respiratory difficulty and air leaks,
talc pleurodesis and thoracoscopic
bullae excision with pleural decortication were performed. Two months after the admission, due to
nosocomial infection and severe respiratory distress, extracorporeal membranous oxygenation (ECMO) support was initiated. The patient was died of ECMO complications on 24th day of ECMO. Conclusion Despite the use of
chemotherapy and surgical excision of cystic lesions, bilateral PLCH in children may have lethal outcome. Other treatment options including respiratory support with ECMO and
lung transplantation should be considered as last resort of treatment alternative in persistent cases.