Background: Wolcott–Rallison syndrome (WRS) is a rare autosomal recessive disorder characterized by neonatal diabetes and acute liver failure. Combined liver–pancreas transplantation offers the potential to address both hepatic failure and insulin dependence. Organ availability and appropriate size matching limits organ usage but previous DCD+NRP use for multivisceral grafts encouraged us to consider a similar approach for this rare disease.

Method: To the best of our knowledge, we describe the first pediatric en-bloc liver–pancreas transplantation in the world from a DCD+NRP donor. The donor was 18-months and 10kg. The donor was on NRP circuit for 1 hour 46 minutes. The lactate trended from 8.7 to 5.2 mmol/L. The organs were used for a 7-year-old female recipient with WRS confirmed by a homozygous mutation in EIF2AK3.  

Results: The organs were procured en-bloc using UW. Engraftment was done by triangulating the upper cava, performing an end-to-end donor portal to recipient SMV anastomosis. An infrarenal aortic conduit was built for arterial reconstruction and a donor to recipient jejunojejunostomy was performed.  The cold ischemia time was 7.5 hours. Insulin was discontinued in the OR and GI function recovered by post-op day 4. Doppler ultrasound confirmed patency of the vessels. Immunosuppression was maintained with tacrolimus, MMF, steroids and Jakafi. She was discharged home on post-op day 15.

Conclusion: This case highlights the feasibility of performing pediatric transplants from pediatric DCD+NRP donors. It also highlights the relevance of adding DCD+NRP as a strategy to increase pediatric donation and transplantation, minimizing mortality on the waiting list.