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

Background: Idiopathic portal hypertension is a disorder of unknown aetiology characterized by portal hypertension secondary to splenomegaly, without cirrhosis. There are no reports on idiopathic portal hypertension occurring in twins. Variceal haemorrhage, a life threatening manifestation of portal hypertension may be treated with transjugular intrahepatic portosystemic shunt in the acute setting. Case presentation: A 36-year-old woman with severe variceal haemorrhage 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 splenomegaly and haemolytic episodes without further complications. The patient, like her twin sister, had also a history of splenomegaly since her childhood, with haemolytic episodes 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 beta-thalassemia trait was also present. At admission, because of active variceal haemorrhage we performed a transjugular intrahepatic 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 subsequent 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 before 1990. A splenectomy was performed and the haematological parameters improved significantly. Despite TIPS obstruction 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. Emergency treatment of the portal hypertension with TIPS, followed by a later surgical splenectomy was an effective management option for a follow up period of six years.