LIVER-TRANSPLANTATION AND SPLENECTOMY IN IDIOPATHIC PORTAL-HYPERTENSION

Idiopathic portal hypertension (IPH) was diagnosed in a 30-year-old ma n. Clinical signs were splenomegaly, leucothrombocytopenia, and esopha geal varices of fourth degree. The histology of the liver biopsy showe d portal fibrosis with no evidence of cirrhosis. No causing agent or k nown disease could be found for the histopathological and clinical fea tures. Due to a Severe deterioration of general condition and a declin e of synthetic liver function, liver transplantation and splenectomy w ere performed. The histological examination of the explanted liver rev ealed features of IPH, demonstrating portal fibrosis and dilated vesse ls adjacent to portal tracts; no cirrhosis was found. The postoperativ e recovery was without any severe complications. The duration of hospi talization was 28 days. Following liver transplantation the esophageal varices disappeared and leucocytes, platelets as well as parameters o f hepatic synthesis reached normal values. Initially, the immun-suppre ssion was composed of prednisolon, tacrolimus, and antibodies against IL-2 receptors (BT 563) and was later continued with prednisolon and t acrolimus. Within the follow-up observation of 26 months, there was no evidence for graft rejection, severe infection, or occurrence of port al hypertension. Up till now the patient is in good condition with nor mal graft function. Liver transplantation may be a curative therapy fo r patients with advanced disease of IPH but the long-term follow-up af ter transplantation has to show whether IPH can reoccur.