Idiopathic Portal Hypertension: Report of a Case

Abstract

Background: Idiopathic portal hypertension (IPH), the socalled Banti’s syndrome, is a condition clinically characterized by splenomegaly, hypersplenism, and increased portal pressure. IPH is uncommon in the Western world. In this article we report on a well-documented case of this rare form of disorder. Case Report: A 27-year-old woman was referred to our hospital with symptoms of severe pancytopenia, massive splenomegaly, and moderate esophageal varices with red color signs despite endoscopic variceal ligation. Upon admission, the hepatic functional reserve was satisfactory (Child-Pugh class A). The color-Doppler ultrasound showed an anatomically normal and non-thrombotic splenoportal venous axis. A complete gastroesophageal devascularization with splenectomy (Hassab-Paquet procedure) was performed. Intraoperatively the liver looked grossly normal while the microscopic examination revealed an inflammatory cell infiltration and fibrosis of the portal tract but no signs of liver cirrhosis. The final diagnosis of IPH was made based on the definitions adopted by the Japan IPH Study Committee. The patient has been asymptomatic for 12 months postoperatively, and hematological indices reached their normal values. The esophageal varices were eradicated. Conclusion: The literature on treatment strategies of esophageal varices due to IPH was reviewed with particular reference to the role of devascularization procedures and splenectomy in the management of this rare condition.