Kp. Vanmeurs et al., LOBAR LUNG TRANSPLANTATION AS A TREATMENT FOR CONGENITAL DIAPHRAGMATIC-HERNIA, Journal of pediatric surgery, 29(12), 1994, pp. 1557-1560
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.