Acute Deterioration of Idiopathic Portal Hypertension Requiring Living Donor Liver Transplantation: A Case Report

Abstract

Word count: 1,531 words 2 Key words: idiopathic portal hypertension, liver transplantation 3 AbstractCase reports of severe IPH requiring liver transplantation are very rare. We report the case of a 65-year-old woman who was diagnosed as having idiopathic portal hypertension (IPH). At the age of 60 years, her initial symptom was hematemesis due to ruptured esophageal varices. Computed tomography of the abdomen showed splenomegaly and a small amount of ascites without liver cirrhosis. She was diagnosed as having IPH and followed-up as an outpatient. Five years later, she developed symptoms of a common cold and rapidly progressive abdominal distension. She was found to have severe liver atrophy, liver dysfunction and massive ascites. Living donor liver transplantation was then performed and her postoperative course was uneventful.Histopathological findings of the explanted liver showed collapse and stenosis of the peripheral portal vein. The areas of liver parenchyma were narrow while the portal tracts and central veins were approximate one another, leading to a diagnosis of IPH.There was no liver cirrhosis. The natural history of refractory IPH could be observed in this case. Patients with end stage liver failure due to severe IPH can be treated by liver transplantation.4

Living donor liver transplantation for idiopathic portal hypertension with extrahepatic portal vein stenosis and splenic artery aneurysms: a case report and review of the literature

“…IPH rarely results in neither end-stage liver failure nor requiring liver transplantation. Past case reports for severe IPH requiring liver transplantation were summarized in Table 1 [ 3 , 5 , 9 , 14 – 20 ]. To date, about 50 cases of liver transplantation have been reported for IPH.…”

“…Although most IPH patients have a good prognosis with the treatment for esophageal varices and/or other complications due to portal hypertension, some develop end-stage liver failure and need for liver transplantation [ 3 – 5 ]. It has been reported that about 6% of patients with IPH required liver transplantation [ 6 ].…”