Orthotopic Liver Transplantation for Hepatic Adenoma in a Patient with Portal Vein Agenesis

Congenital portal vein agenesis, known as the Abernathy malformation, was first described in 1793. It is associated with focal nodular hyperplasia and adenomas due to abnormal hepatic regeneration secondary to abnormal or absent flow to the liver. We report a case of a 25-year-old Caucasian female with a complicated medical history including idiopathic membranoproliferative glomerulonephritis, hypertension, hyperlipidemia, Sweet's syndrome, acute febrile neutrophilic dermatosis, who incidentally was diagnosed with multiple liver nodules, the largest 14 cm in diameter, arising at the junction of the left and right hepatic lobes. She was also noted to have a congenital absence of the portal vein. There was no evidence of cirrhosis or portal hypertension. Liver function tests and  - fetoprotein were within normal limits. A biopsy of the largest mass revealed hepatocellular adenoma. Because of the size, location, and multiplicity of the lesions, we elected to proceed with orthotopic liver transplantation. She subsequently underwent deceased donor liver transplantation using the piggyback technique. Despite dividing the portal vein shunt after completion of the caval anastomosis and minimizing the portal vein clamp time, her spleen spontaneously ruptured, for which splenectomy was performed. The patient did well postoperatively with good liver function.