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

Abstract

Congenital portal vein agenesis, known as the Abernathy's malformation, is associated with focal nodular hyperplasia and adenomas due 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. She was also noted to have a congenital absence of the portal vein with the splenic vein joining the superior mesenteric vein before emptying into the suprarenal inferior vena cava. There was no evidence of cirrhosis or portal hypertension. A biopsy of the largest mass revealed hepatocellular adenoma. Because of the size, location and multiplicity of the lesions, we elected to proceed with the orthotopic liver transplantation. The patient was granted 22 MELD points and placed on the UNOS waiting list. She subsequently underwent deceased donor liver transplant using a piggyback technique. After completion of the hepatic vein anastomosis the portal vein was stapled off the IVC. Donor and recipient portal veins were anastomosed in the end-to-end fashion. While the anastomosis was being completed, a large parenchymal laceration was noted through the splenic hilum. Repair of the laceration was attempted but was unsuccessful. Patient underwent splenectomy. This complication is very rare. It has been described with clamping of the portal vein for liver resections. It occurs in patients with the normal liver parenchyma without cirrhosis, portal hypertension or collaterals. She did well postoperatively with good liver function and was discharged home on the postoperative day 5.