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.