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Lobar lung transplantation as a treatment for congenital diaphragmatic hernia
K P Van Meurs1, W D Rhine, W E Benitz
1Department of Pediatrics, Lucile Salter Packard Children's Hospital, Stanford, CA.
Insights
Single-lung transplantation is a feasible treatment for infants with severe congenital diaphragmatic hernia (CDH) and unsalvageable pulmonary hypoplasia. Extracorporeal membrane oxygenation (ECMO) can serve as a bridge to this life-saving procedure.
Area of Science:
- Pediatric Surgery
- Neonatology
- Cardiothoracic Surgery
Background:
- Congenital diaphragmatic hernia (CDH) presents a high mortality rate in infants, even with advanced neonatal intensive care.
- Pulmonary hypoplasia in severe CDH cases often necessitates novel treatment strategies beyond conventional surgical repair and extracorporeal membrane oxygenation (ECMO).
Observation:
- A newborn female with right-sided CDH, initially managed with ECMO and delayed surgical repair, experienced progressive respiratory failure post-repair.
- Despite initial improvements, the infant required escalating ventilator support, indicating persistent hypoxemia and hypercarbia.
Findings:
- Single-lung transplantation was successfully performed using a donor right lung, with partial excision due to size discrepancy, enabling lobar transplantation.
- The infant demonstrated recovery and was discharged at 3.5 months, with ongoing follow-up indicating long-term feasibility.
Implications:
- This case highlights the potential of single-lung transplantation as a viable option for infants with severe CDH and pulmonary hypoplasia.
- Extracorporeal membrane oxygenation (ECMO) can effectively bridge infants to lung transplantation.
- Lobar lung transplantation offers flexibility in donor-recipient size matching for pediatric recipients.
Abstract:
The mortality rate for infants severely affected with congenital diaphragmatic hernia (CDH) remains high despite significant advances in surgical and neonatal intensive care including delayed repair and extracorporeal membrane oxygenation (ECMO). Because of the increasingly successful experience with single-lung transplantation in adults; this approach has been suggested as a potential treatment for CDH infants with unsalvageable pulmonary hypoplasia. The authors report on a newborn female infant who was the product of a pregnancy complicated by polyhydramnios. At birth, she was found to have a right-sided CDH and initially was treated with preoperative ECMO, followed by delayed surgical repair. Despite the CDH repair and apparent resolution of pulmonary hypertension, the infant's condition deteriorated gradually after decannulation, and escalating ventilator settings were required as well as neuromuscular paralysis and pressor support because of progressive hypoxemia and hypercarbia. A lung transplant was performed 8 days after decannulation, using the right lung obtained from a 6-week-old donor. The right middle lobe was excised because of the size discrepancy between the donor and recipient. After transplantation, the patient was found to have duodenal stenosis and gastroesophageal reflux, which required duodenoduodenostomy and fundoplication. The patient was discharged from the hospital 90 days posttransplantation, at 3 1/2 months of age. Currently she is 24 months old and doing well except for poor growth. This case shows the feasibility of single-lung transplantation for infants with CDH, and the potential use of ECMO as a temporary bridge to transplantation. Lobar lung transplantation allowed for less stringent size constraints for the donor lung.