Background: In patients with non-cirrhotic portal vein thrombosis portal cavernoma (PC) might
occur within a few weeks. This leads to portal hypertension and variceal bleeding.
There are few data available on the clinical course and prognosis in this rare disease.
Methods: 46 patients with chronic non-cirrhotic portal vein thrombosis were evaluated in
a retrospective monocentric analysis over a ten-year period (January 1, 2014 – December
31, 2023).
Results: The study included 24 men (52%) and 22 women (48%). Concerning etiology, 24 patients
(52%) had a coagulation disorder, 5 patients (11%) an abdominal inflammatory focus
and 3 patients (6.5%) a sleeve gastrectomy. 34 patients (74%) received therapeutic
anticoagulation. 11 patients (24%) did not develop varices during the follow-up period.
In 24 of the remaining 35 patients (68%), varices were already present at the initial
diagnosis of the PC. Variceal bleeding occurred in only 12 of the 46 patients (26%),
after a median period of 23 months after the initial diagnosis of the PC. Incidence
of variceal bleeding was 10,9%, 13% and 19,6% after 1,3 and 5 years after the diagnosis
of PC, respectively. 27 patients (59%) were treated with non-selective beta blockers.
Only one patient died during the follow-up period.
Conclusions: In chronic non-cirrhotic portal vein thrombosis, varices are often present at the
time of initial diagnosis of PC, but the risk of variceal bleeding appears to be lower
than previously thought. The overall survival of these patients seems not to be limited
with appropriate endoscopic and medical therapy.
