A case of Langerhans cell histiocytosis presented with pneumothorax.

Pneumothorax (PTX) is an unusual complication of Langerhans cell histiocytosis (LCH) in childhood. Spontaneous PTX is rare in childhood, and it is very rare in infancy. There are no specific recommendations for the treatment of PTX from LCH described in the literature. We are presenting a 19-month-old boy, who suddenly developed left-sided PTX with infiltrations in both lungs. He presented with PTX and skin lesions. He had a prolonged cardiac arrest, and although resuscitation was successful he required continuing ventilatory support (intermittent positive-pressure ventilation). Because he suddenly developed right-sided PTX and died on the second day of the admission, his LCH diagnosis was made only postmortem. So, he did not receive chemotherapy. It is likely that intermittent positive-pressure ventilation during the operation induced the development of much more multiple lung bullae, which subsequently ruptured, and/or it facilitated the development of the right-sided PTX. The patients with PTX and skin lesions, including babies, most likely have LCH and specific chemotherapy should be started in emergency, even before the final diagnosis is achieved.