Liver Transplantation for Refractory Congenital Cytomegaloviral Hepatitis

Congenital cytomegalovirus (cCMV) is the most common congenital infection. Here, we report on a case of severe, refractory cCMV hepatitis resulting in end-stage liver disease. A male infant born at 37 weeks gestational age presented with petechiae, splenomegaly, and jaundice associated with a direct hyperbilirubinemia, elevated transaminases, and thrombocytopenia. Urine screen was positive for CMV, and he was treated with valganciclovir. He progressed to decompensated cirrhosis with ascites, hypoglycemia, and coagulopathy and was listed for liver transplant at 4 months of age. At 5 months of age, he developed massive hematemesis with hemorrhagic shock and underwent emergent portocaval shunt followed by living donor liver transplant with a left lateral segment graft. Postoperatively, he received CMV immune globulin and intravenous ganciclovir and cleared his viremia by 2 months post-transplant. This case illustrates the diagnostic and management challenges of severe cCMV hepatitis and reports a successful liver transplantation despite active CMV viremia.