Case Report: Retransplantation for Adult-Onset Bilirubin Encephalopathy After Auxiliary Partial Liver Transplant for Crigler-Najjar Syndrome Type 1 in Childhood

Abstract

Crigler-Najjar syndrome (CNS) type 1 is a genetic disorder that leads to severe unconjugated hyperbilirubinemia. Auxiliary partial orthotopic liver transplantation (APOLT) is preferred for metabolic liver diseases as a portion of the native liver is retained. Reported here are details of a 32-year-old male who developed bilirubin encephalopathy due to graft failure 27 years after APOLT performed at 5-year-old for CNS type 1. Initially, the bilirubin level was low, which then gradually increased to 7.3 mg/dL at age 12. At the age of 31, the bilirubin level began to rise rapidly and we submitted a request for deceased donor liver transplantation (DDLT) to the Committee for the Evaluation of Indications for DDLT in Japan. However, that was deemed as not indicated, because unconjugated bilirubin-dominant hyperbilirubinemia alone is generally not considered to cause encephalopathy in adults. Despite the high bilirubin level, the patient remained neurologically asymptomatic until occurrence of acute decompensation. At 32 years old, he was admitted on an emergency basis to our hospital with severe hyperbilirubinemia and neurological symptoms. After managing his general condition with intensive care, DDLT was finally performed, resulting in a drastic resolution of various symptoms. The findings in this case show that bilirubin-induced neurotoxicity in adults may be reversible and indicate that the current liver transplantation criteria used in Japan, which limit eligibility for CNS to pediatric patients, may have contributed to delayed transplantation. Re-evaluation of the transplant eligibility criteria is necessary to allow for a more inclusive approach that encompasses adult patients.