LOBAR LUNG TRANSPLANTATION AS A TREATMENT FOR CONGENITAL DIAPHRAGMATIC-HERNIA

The mortality rate for infants severely affected with congenital diaph ragmatic hernia (CDH) remains high despite significant advances in sur gical and neonatal intensive care including delayed repair and extraco rporeal membrane oxygenation (ECMO). Because of the increasingly succe ssful experience with single-lung transplantation in adults, this appr oach has been suggested as a potential treatment for CDH infants with unsalvageable pulmonary hypoplasia. The authors report on a newborn fe male infant who was the product of a pregnancy complicated by polyhydr amnios. At birth, she was found to have a right-sided CDH and initiall y was treated with preoperative ECMO, followed by delayed surgical rep air. Despite the CDH repair and apparent resolution of pulmonary hyper tension, the infant's condition deteriorated gradually after decannula tion, and escalating ventilator settings were required as well as neur omuscular paralysis and presser support because of progressive hypoxem ia and hypercarbia. A lung transplant was performed 8 days after decan nulation, using the right lung obtained from a 6-week-old donor. The r ight middle lobe was excised because of the size discrepancy between t he donor and recipient. After transplantation, the patient was found t o have duodenal stenosis and gastroesophageal reflux, which required d uodenoduodenostomy and fundoplication. The patient was discharged from the hospital 90 days posttransplantation, at 3 1/2 months of age. Cur rently she is 24 months old and doing well except for poor growth. Thi s case shows the feasibility of single-lung transplantation for infant s with CDH, and the potential use of ECMO as a temporary bridge to tra nsplantation. Lobar lung transplantation allowed for less stringent si ze constraints for the donor lung.