[Venous diseases of the liver: what are we talking about?]

Area of Science:

• Hepatology
• Vascular Medicine
• Hematology

Background:

• Vascular liver diseases involve damage to hepatic and perihepatic arteries and veins.
• Key venous forms include Budd-Chiari syndrome (BCS), portal vein thrombosis (PVT), and porto-sinusoidal vascular disease (PSVD).
• These conditions often affect young adults, presenting portal hypertension without underlying cirrhosis.

Purpose of the Study:

• To define and differentiate major venous forms of liver vascular disease.
• To identify common risk factors and patient demographics.
• To highlight the association with portal hypertension in the absence of cirrhosis.

Main Methods:

• Classification based on the location of thrombotic obstruction: hepatic veins (BCS), portal vein (PVT), and intrahepatic microcirculation (PSVD).
• Review of clinical presentations and etiological factors.
• Analysis of associated conditions such as myeloproliferative neoplasms and coagulation disorders.

Main Results:

• BCS involves hepatic vein obstruction.
• PVT involves portal vein obstruction, with a risk of cavernoma formation.
• PSVD affects intrahepatic microcirculation without cirrhosis.
• Myeloproliferative neoplasms are a frequent risk factor, alongside other systemic and local causes.

Conclusions:

• Vascular liver diseases are rare conditions primarily affecting young adults.
• These diseases characteristically cause portal hypertension independent of liver cirrhosis.
• Understanding the specific venous obstruction and associated risk factors is crucial for diagnosis and management.