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

Abstract

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 β-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.