SESSION TITLE: Surgery Cases
SESSION TYPE: Case Reports
PRESENTED ON: Sunday, March 23, 2014 at 09:00 AM - 10:00 AM
INTRODUCTION: Cystic Fibrosis (CF) is a disease which can develop lung destruction and cause respiratory insufficiency in children. Lobar lung transplantation from adult donors has arisen as a treatment in some cases of respiratory failure in pediatric patients.
CASE PRESENTATION: A 9 years old female was admitted to the pediatric ICU due to a refractive respiratory failure. She was diagnosed of CF at the age of 18 months with homozygous F508Δ mutation. She had bilateral bronchiectasis colonized by MRSA and Pseudomona aeruginosa. Her evolution was torpid, indicated by more than 6 admissions in a year. Besides, she was on the waiting list for over 12 months as she did not have a suitable donor. Therefore we had to accept an adult donor. She needed respiratory support with invasive ventilation and was included into the Urgent Transplant Code. After six days she received a bilateral lobar lung transplant from a cadaver adult donor. The surgery was done with extracorporeal membrane oxygenation (ECMO) support. Both native lungs were resected and the procedure was completed from left side to right side. The donor lower left and right lobes were transplanted and anastomoses performed in this order: bronchus, pulmonary vein, and pulmonary artery. The postoperative evolution was satisfactory and the patient was discharged from ICU at day 8 and discharged from the hospital at day 40. Two years after the lobar lung transplant she is well and can run and play as similar-age children. Her FEV1 is 1’01 (67% of predicted) and her FVC 1’36 (60% of predicted).
DISCUSSION: CF patients on the waiting list for lung transplant can develop severe hypoxemic life-threatening situations. Pediatric patients have a higher mortality rate on waiting lists due to the scarcity of donors, especially in the end stage of CF. In the 90’s the first lobar transplantations were performed using live donors. Despite these years of experience of live lung transplants, cadaver lobar transplants are not frequent. Although it is a surgical practice that has demonstrated to increase survival in pediatric CF patients.
CONCLUSIONS: This case illustrates the difficulty in acquiring an adequate number of donors to serve pediatric lung transplantation candidates. Using adult lung lobes in our patient had a successful outcome. Our team proposes that cadaver adult donors should be taken into account in order to reduce the waiting list mortality rate experienced by this age group.
Reference #1: Bonnette P. Lung transplantation for cystic fibrosis: satisfactory results in specialized centres. Eur J Cardiothorac Surg. 2012 Feb;41(2):440-1.
Reference #2: Algar F.J, Moreno P, Cano J.R, Espinosa D, Alvarez A, Cerezo F, et al. Urgency-Code Lung Transplantation for Cystic Fibrosis: Experience and Results. Transplant Proc. 2008 40, 3067-3069.
Reference #3: Espinosa D, Algar F. J, Moreno P, Illana J, Álvarez A, Cerezo F, et al. Experience of the Reina Sofia Hospital in Lobar Lung Transplantation. Transplant Proc. 2010 42, 3214-3216.
DISCLOSURE: The following authors have nothing to disclose: Juan Pablo Reig, Amparo Sole, Emilio Ansótegui, Juan Escrivá, Juan Antonio Pastor
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