Surgical management of persistent pneumothorax in patient with Langerhan’s histiocytosis: case report

Ariela L. Zenilman, Linda T. Li, Andrew Silverman, Eva M. S. Notis, Michael Weiner, Vincent Duron


Langerhans cell histiocytosis (LCH) is a rare disease that involves multiple organ systems and may cause secondary pneumothorax in children. Management of luteinizing hormone (LH) involves multiple medical disciplines and primarily relies on long-term chemotherapeutic agents. Surgery, namely total pleurectomy, is rarely necessary and is described only in a few case reports, but to our knowledge, has not been described using the thoracoscopic approach in a 1-year old patient. We discuss a 1-year old child who initially presented to an outside hospital with respiratory distress and was found to have pneumothoraces, jaundice, and a rash. Bilateral chest tubes were placed and the diagnosis of LCH was secured by skin biopsy. She was started on weekly vinblastine and prednisone, but had a prolonged hospital course due to a persistent right pneumothorax and a continuous air leak for which she was transferred to our institution. Her treatment course was changed to cytarabine however her air leak persisted. Computed tomography of her chest confirmed extensive, bilateral involvement of both lungs with large cystic spaces throughout the lung parenchyma, and a sizeable pneumothorax, despite chest tube drainage. At 8 weeks of treatment, she underwent a right video-assisted thoracoscopic total pleurectomy. She was extubated on postoperative day 1. She continued to have an air leak and a small pneumothorax for several days after surgery until she achieved pleural apposition and resolution of her air leak at postoperative day 7. We maintained her chest tube to suction for another 7 days and her chest tube was removed successfully after a water seal test. She continues on her cytarabine therapy. This case describes the first report of a thoracoscopic total pleurectomy in a 1-year-old child with LCH.