Introduction
Chronic portal thrombosis and/or portal cavernoma is a cavo-portal collateral venous pathway secondary to chronic thrombotic obstruction. The etiologies are numerous and must be systematically investigated. Our work aimed to study the profile of patients with portal cavernoma secondary to a local cause.
Aims & Methods
We conducted a 6-year retrospective, descriptive and monocentric study (2016-2022) including all patients followed for portal cavernoma and who have in their history a notion of abdominopelvic surgery or local non-surgical cause. All patients underwent a comprehensive etiologic workup for thrombosis.
Results
Out of the 56 patients diagnosed with portal cavernoma, 20 had a history of surgery or a local cause, indicating a prevalence of 35.71%. The mean age of the patients was 43.68 years with a range of 20 to 69 years and there was a clear female predominance with a sex ratio F/M of 19.
The clinical presentation of patients with portal cavernoma included acute abdominal pain in 83.3% of cases, digestive haemorrhage in 33.3%, ascites in 33.3% and asthenia in 16.7%. Biologically, cholestasis was observed in 40% of cases, cytolysis in 26.6%, hepatocellular insufficiency in 6.6%, and pancytopenia in 6.6%. Abdominal ultrasound coupled with Doppler was performed in all patients, revealing a dysmorphic liver in 40% and heterogeneous liver in 26.6%. Furthermore, angiography demonstrated extensive portal thrombosis in 20% of patients, and upper endoscopy found esophageal varices in 60%, gastroesophageal varices in 26.6%, and congestive gastropathy in 16.6% of patients.
Local causes of portal thrombosis included abdominal surgery (50%), pelvic surgery (25%), and non-surgical causes (25%). The abdominal surgeries included cholecystectomy (30%), appendectomy (30%), Porto-azygos shunt (20%), cholecystectomy followed by splenectomy (10%), and cholecystectomy followed by liver surgery: resection of the protruding dome for a hydatid cyst (10%). An exhaustive etiological work-up was performed in all patients, revealing various underlying prothrombotic causes such as protein C and/or S deficiency (25%), celiac disease (10%), polycythemia vera (10%), chronic inflammatory bowel diseases (5%), Biermer disease (5%), and Paroxysmal Nocturnal Hemoglobinuria clone mutation (5%). whereas no associated prothrombotic pathology was found in 40% of the patients. In the 40% where no underlying prothrombotic cause was found, the only cause of portal thrombosis was local: pelvic surgery in 20% of all cases, two abdominal surgeries in 10%, and main bile duct stent in 10% of patients.
As for the complications, 4 patients were complicated by portal biliopathy objectified by Cholangio-MRI and one patient died of hepatorenal syndrome.
Conclusion
The local cause of portal cavernoma is rare in the literature and is 4 % in our series. The most frequent surgery found in history is cholecystectomy, but these patients often have another associated prothrombotic factor. In the rare cases where no underlying prothrombotic factor was found, it was most often pelvic surgery followed by the association of 2 abdominal surgeries and a main bile duct stent.