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A5646 - Recurrent Pneumothoraces in Infant
Author Block: L. A. Lautenbacher, M. Kattan, B. J. Sheares; Pediatric Pulmonology, Columbia University Medical Center, New York, NY, United States.
Introduction: Langerhans Cell Histocytosis (LCH) is a rare disorder characterized by clonal proliferation of Langerhans cells, which clinically presents with single or multisystem involvement. LCH with significant pulmonary involvement is less common in children than adults occurring in about 10% of pediatric cases.Case: A 15-month-old female presented at 12 months of age with a spontaneous pneumothorax followed one month later by prolonged jaundice, transaminitis, and skin rash in the scalp and groin. Abdominal ultrasound, magnetic resonance cholangiopancreatography (MRCP), liver biopsy, and skin biopsy were initially non-diagnostic. Three weeks later, the patient developed bilateral pneumothoraces requiring chest tube placement. Repeat skin biopsy was diagnostic for LCH with positive CD1a and s100. Chest CT at presentation showed multiple bilateral cysts, right sided pneumothorax, and diffuse ground glass opacities. She was started on LCH chemotherapy protocol with prednisone and vinblastine prior to transfer from outside hospital. She was born at 36 weeks gestation without any complications or significant past medical history. There was no significant family history. Physical exam on transfer was remarkable for tachypnea without associated hypoxemia, retractions, and clear breath sounds. She had a prolonged hospitalization with respiratory symptoms including intermittent tachypnea and occasional wheeze in setting of persistent air leaks requiring multiple chest tubes. The lack of improvement in pulmonary status prompted a change in chemotherapy protocol to cytarabine with corticosteroid taper. Course was additionally complicated by corticosteroid-induced hypertension, pseudohyponatremia with hyperlipidemia, and cholestasis. She subsequently developed a large right-sided tension pneumothorax despite multiple chest tubes and underwent total right pleurectomy. Post-operative course after pleurectomy was uncomplicated with chest tube removal without recurrence of pneumothorax.Discussion: Recurrent pneumothoraces is uncommon in LCH in childhood and infancy. Patients with pulmonary LCH have a predisposition to pneumothoraces due to the destructive, cystic changes in lung parenchyma. However, there are no specific management guidelines for recurrent pneumothorax from LCH described in the literature for this age group. This case suggests the diagnosis of LCH should be included in differential diagnosis of recurrent pneumothoraces in childhood and infancy. Pleurectomy may be the only management option in the setting of failed chemotherapy and corticosteroids for pulmonary LCH with recurrent pneumothorax.