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A5648 - Diagnosis and Treatment of Cryptogenic Organizing Pneumonia in a Child on Extracorporeal Membrane Oxygenation Support
Author Block: E. Sayad1, A. M. Vogel2, R. P. Guillerman3, C. Chartan4, R. Coleman4, K. Patel5, M. A. Musick6, M. D. Silva Carmona1; 1Pediatric Pulmonary, Texas Children's Hospital, Houston, TX, United States, 2Pediatric Surgery, Texas Children's Hospital, Houston, TX, United States, 3Pediatric Radiology, Texas Children's Hospital, Houston, TX, United States, 4Pediatric Critical Care, Texas Children's Hospital, Houston, TX, United States, 5Pediatric Pathology, Texas Children's Hospital, Houston, TX, United States, 6Pediatric Critical Care, Texas Childrens Hospital, Houston, TX, United States.
Background:
Organizing pneumonia is a rare diffuse lung disease that can have a variety of etiologies such as infection, drug reaction, bone marrow transplantation or autoimmune disease. It is defined as cryptogenic organizing pneumonia (COP) if no etiology is identified. The incidence of cryptogenic organizing pneumonia in children is not known. The appearance on chest computed tomography (CT) is highly variable, with the most frequent pattern being patchy ground glass opacities or consolidation in a subpleural or peribronchial distribution. Histopathologic finding are buds of fibroblastic granulation tissue in the lumens of the alveolar spaces and distal bronchioles. The clinical presentation is subacute with cough and dyspnea. Most cases resolve with corticosteroid treatment.
Case report:
We describe a previously healthy 16 months old boy, with recurrent pneumonia for 3 months. He was transferred to our center with respiratory distress, after previously being treated with courses of antibiotics and oral steroids with transient improvement.
He was febrile, tachypneic and tachycardic. A chest CT scan showed extensive multilobar peripheral ground glass opacities, arcifrom bands of peribronchial consolidation of the lower lobes, scattered pulmonary nodules and septal thickening. He developed respiratory failure with refractory hypoxemia despite invasive mechanical ventilation. He was placed on venovenous extracorporeal membranous oxygenation (VV-ECMO). An extensive immunologic, infectious, and rheumatologic evaluation including bronchoscopy with bronchoalveolar lavage was non-diagnostic.
An open lung biopsy, performed on ECMO, showed several amphophilic and cellular foci of fibroblastic proliferation along the alveolar septae. These findings were consistent with COP.
He received IV methylprednisolone pulses (30mg/kg) followed by a daily systemic steroid maintenance dose. He remained on VV-ECMO for 13 days, was extubated after 25 days and was weaned to room air on hospital day 30. Repeat CT scan of the chest then showed near complete resolution of the pulmonary parenchymal disease. Steroids were slowly weaned, and four months later he remained on room air without respiratory problems.
Discussion:
Our case highlights a rare pulmonary disease in pediatrics manifesting with respiratory failure. While ECMO is an established method of supportive care in pediatric hypoxemic respiratory failure, to our knowledge this is the first pediatric case in which lung biopsy was able to be obtained under ECMO leading to the successful diagnosis and treatment of COP.
Conclusion:
This case illustrates that lung biopsy for the diagnosis of diffuse lung disease of unclear etiology and effective diagnosis and treatment of COP are feasible in the setting of ECMO.