Idiopathic portal hypertension in a twin treated with TIPS and consequent splenectomy

SUMMARY Background: Idiopathic portal hypertension is a disorder of unknown aetiology characterized by portal hypertension sec- ondary to splenomegaly, without cirrhosis. There are no re - ports on idiopathic portal hypertension occurring in twins. Variceal haemorrhage, a life threatening manifestation of portal hypertension may be treated with transjugular intra- hepatic portosystemic shunt in the acute setting. case pre - sentation: A 36-year-old woman with severe variceal haem - orrhage and ascites due to idiopathic portal hypertension was admitted to the Gastroenterology Department. Her twin sister underwent a splenectomy at the age of 12 due to sple - nomegaly and haemolytic episodes without further compli - cations. The patient, like her twin sister, had also a history of splenomegaly since her childhood, with haemolytic epi - sodes and need for multiple transfusions. Splenectomy was not preferred for her. In the following years, blood group incompatibilities developed after multiple transfusions that precluded any further blood transfusions. A β-thalassemia trait was also present. At admission, because of active var - iceal haemorrhage we performed a transjugular intrahe - patic portosystemic shunt (TIPS) in an emergency setting. A decline of the portosystemic pressure gradient from 26 to 12 mmHg resulted with no further bleeding and with a sub - sequent reduction of the spleen size from 35 cm to 20 cm in diameter. A transjugular liver biopsy, a few months after TIPS, revealed a mild chronic hepatitis that was attributed to hepatitis c virus infection acquired from transfusions be - fore 1990. A splenectomy was performed and the haemato - logical parameters improved significantly. Despite TIPS ob - struction that occurred later, no further oesophageal varices developed, and there was no need for further transfusions. conclusions: In this patient, idiopathic portal hypertension may have had splenomegaly possibly related to haemolytic episodes as an initial cause, whereas later increased portal vascular resistance developed. In her twin sister, who also had splenomegaly at childhood, there was no development to portal hypertension due to an early splenectomy. emer- gency treatment of the portal hypertension with TIPS, fol - lowed by a later surgical splenectomy was an effective man -