Challenges and strategies: treating spontaneous pneumothorax in massive pulmonary langerhans cell histiocytosis in children.

Objective: The objective of this study was to report two cases of successive multiple spontaneous bilateral pneumothorax in children with massive lung involvement due to Langerhans cell histiocytosis (LCH), emphasizing the possibility of this differential diagnosis for the general pediatrician. Additionally, published cases describing pediatric patients with pulmonary manifestations of LCH were reviewed in the literature.

Case description: Case #1: A 3-year-old male patient with a sudden episode of spontaneous right-sided pneumothorax, surgically drained. After 2 months, he experienced two new episodes of contralateral pneumothorax. A pulmonary lymph node biopsy revealed the diagnosis of LCH. He underwent bilateral video-assisted thoracic surgery and mini-thoracotomy with mechanical pleurodesis, in addition to chemotherapy, requiring prolonged hospitalization. Case #2: A 4-year-old boy with progressive dyspnea and wheezing for 5 months. A pulmonary biopsy revealed LCH. He developed significant respiratory distress and right pneumothorax, requiring drainage. Silver nitrate pleurodesis and different chemotherapy regimens were performed. Both patients responded well to multiple chemotherapy treatments, surgeries, and intensive care support.

Comments: LCH is a challenging disease. Its clinical manifestation is variable, and pulmonary involvement occurs in about 10-15% of cases. We consider specialized surgical management and multidisciplinary support essential for the treatment of patients with massive pulmonary LCH. Although rare, massive pulmonary involvement by LCH should be considered in cases of recurrent pneumothorax in children.