Pediatric spontaneous tension pneumothorax in Langerhans cell histiocytosis

Abstract

Langerhans cell histiocytosis is a rare hematologic disorder caused by the proliferation of specialized dendritic cells. Pulmonary manifestations occur in 20% to 50% of multisystem disease, but isolated pulmonary disease is rare. Spontaneous pneumothorax occurs in up to 10% of pediatric pulmonary cases. We present the case of a 3-year-old boy who presented for tachypnea and chest pain with cough, rhinorrhea, and congestion. He was discharged with a diagnosis of upper respiratory infection. The patient returned to the emergency department and was found to have a tension pneumothorax, which was emergently treated with thoracostomy. The subsequent workup revealed Langerhans cell histiocytosis with pulmonary disease and jaw involvement. Although tension pneumothorax is often associated with trauma, several medical conditions are predispositional.

Keywords: Histiocytosis; Langerhans cell; pediatric emergency medicine; pneumothorax.

Figure 1.

Patient imaging. (a) Chest x-ray showing right-sided tension pneumothorax. (b) Computed tomography demonstrating diffuse cystic disease in the lungs.