LAUSR

Purpose Pleuroparenchymal fibroelastosis (PPFE) is a rare, progressive, restrictive lung disease characterized by hypercarbic respiratory failure. In pediatrics, it has been described in patients with a history of malignancy who have received a bone marrow transplant, chemotherapy, or radiotherapy. It is characterized by pleural thickening, fibrosis, subpleural elastosis, and intraalveolar collagen deposits. Survival is poor and the only therapy is lung transplantation. Little is known about the outcomes after lung transplant for PPFE in pediatric patients. Methods A single-center retrospective chart review of patients that underwent bilateral lung transplant for PPFE between 2010 and 2020 was done. Descriptive and outcomes data were collected and compared to overall survival after lung transplant for our center. Results Nine patients were transplanted for PPFE in the 10-year period. Six were male. The average age at referral for transplant was 12.5 (7 - 16) years. The average at transplant was 13.8 (9 - 16) years. All patients had a history of malignancy; the most common was neuroblastoma (6/9) followed by 1 rhabdomyosarcoma, 1 acute lymphoid leukemia, and 1 acute myeloid leukemia. Seven patients received radiation therapy (non thoracic). Five patients had a history of autologous bone marrow transplantation. The average time from chemotherapy to referral to lung transplant was 9 years (6 - 12). Six patients required donor lung resections. One required delayed chest closure for 48 hours. Currently, 4 patients are alive, 4 deceased, and 1 lost to follow-up due to the transfer of care. The longest surviving patient is 3.7 years post transplant. The median survival after transplantation of the 4 deceased patients was 2.2 (1.3 - 6.3) years. Median survival post lung transplant for any reason is 6.6 years at our center. Conclusion Our experience with PPFE has been reflective of what has been published in the literature. All patients had a previous malignancy. Our limited data indicate worse outcomes compared to our overall center survival. Six patients required donor lung resizing due to the severely restrictive and small thoracic cavity size, resulting in increased morbidity. There is a need for further research to determine if lung transplantation for PPFE is an indicated therapy given the apparent risk of poor outcomes and higher morbidity.