Living donor liver transplantation for neonatal hemochromatosis using non-anatomically resected segments II and III: a case report

Abstract

IntroductionNeonatal hemochromatosis is the most common cause of liver failure and liver transplantation in the newborn. The size of the infant determines the liver volume that can be transplanted safely without incurring complications arising from a large graft. Transplantation of monosegments II or III is a standard method for the newborns with liver failure.Case presentationA three-week old African-American male neonate was diagnosed with acute liver failure secondary to neonatal hemochromatosis. Living-related liver transplantation was considered after the failure of intensive medical therapy. Intra-operatively a non-anatomical resection and transplantation of segments II and III was performed successfully. The boy is growing normally two years after the transplantation.ConclusionNon-anatomical resection and transplantation of liver segments II and III is preferred to the transplantation of anatomically resected monosegements, especially when the left lobe is thin and flat. It allows the use of a reduced-size donor liver with intact hilar structures and outflow veins. In an emergency, living-related liver transplantation should be offered to infants with liver failure secondary to neonatal hemochromatosis who fail to respond to medical treatment.

Neonatal haemochromatosis with reversible pituitary involvement

“…In small infants in whom a left lateral segment is often large for size, liver transplantation using either mono‐segment II or III is a technically challenging, effective option. To reduce the wait time on the list, nonanatomical resection and transplantation of segments II and III have been performed and ABO‐incompatible grafts have been used successfully . In the neonate described in the present report, an ABO‐incompatible LT together with exchange transfusions and the use of rituximab resulted in good control of humoral immunity and favourable outcome.…”