Journal of Gastrointestinal & Digestive System
Open Access

Abstract

Idiopathic portal hypertension (IPH) is a rare disease characterized by clinical portal hypertension in the absence of a recognizable cause and has a good prognosis, but some cases require liver transplantation. We report the case of a 32-yearold male patient diagnosed with IPH 10 years ago. Clinical signs were splenomegaly, leucothrombocytopenia, and esophageal varices. The histology of the liver biopsy showed portal fibrosis with no evidence of incomplete septal cirrhosis. Due to recurrent episodes of bleeding from esophageal varices, despite band-ligations and performed TIPS procedure, cadaveric liver transplantation was performed 6 years ago. Following liver transplantation, the esophageal varices disappeared but splenomegaly and low blood cells leucothrombocytopenia persisted. The immunosuppression composed of prednisolon, tacrolimus. After 3 years increase in portal vein diameter, which reached over 4 in 2022 with the reccurence of esophageal varices, in December there was a thrombosis of the portal vein, complicated by ascites and bleeding. Anticoagulant therapy for 3 months was unsuccessful. In April 2023, the patient underwent splenectomy. Histopathologically, the liver had obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal cirrhosis. Liver transplantation may be a curative therapy for patients with advanced disease of IPH but the long-term follow-up after transplantation and we need more information on the benefits of one-stage splenectomy during transplantation.

Biography

Aibar Aginbay has been working in the department of hepatology since 2019. During this short time he have seen patients with various liver pathologies, and patients with idiopathic portal hypertension, remains a separate cohort of patients, outwardly indistinguishable from healthy people, but having colossal changes inside. This patient has been seen in his hospital for a long time, and he witnessed his CT picture during that time. After splenectomy they decided to publish this case.