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Toxic liver syndrome is a rare condition with multiorgan failure in end-stage liver disease (ESLD), and a two-stage LT following hepatectomy with a prolonged anhepatic phase is an accepted approach to bridge to transplant. This primary approach has not been described for toxic liver syndrome in children with ESLD. We report a 6-year-old boy who developed toxic liver syndrome with multiorgan failure while awaiting LT for ESLD from biliary atresia and failed Kasai at the age of 2 years. Deemed too sick to transplant, he underwent full hepatectomy and portocaval shunt placement. The child was then transplanted hemodynamically stable after an anhepatic phase of 10 h and 30 min. Although his initial graft showed primary liver dysfunction and he needed re-transplantation after 14 days, he was able to leave the hospital 4 months following 2nd LT and is well with a fully working graft 5 years later. Primary two stage LT is feasible in children in dire situations.

One Step at a Time: A Pediatric Case of Primary Two Staged Liver Transplantation in a Child with ESLD