Redefining Budd-Chiari syndrome: A systematic review.

Area of Science:

• Hepatology
• Vascular Medicine
• Gastroenterology

Background:

• Budd-Chiari syndrome (BCS) encompasses hepatic vein thrombosis (HVT) and hepatic vena cava-Budd Chiari syndrome (HVC-BCS).
• Distinguishing between classical BCS (HVT) and HVC-BCS is crucial for appropriate diagnosis and treatment.
• Previous literature has sometimes conflated these two distinct clinical entities.

Purpose of the Study:

• To systematically review current evidence to determine if classical BCS and HVC-BCS represent the same or different clinical disorders.
• To identify and compare key characteristics, including etiology, clinical presentation, and management, of classical BCS and HVC-BCS.

Main Methods:

• A systematic review of observational studies published between January 2000 and February 2015 was conducted.
• Databases searched included Cochrane Library, CINAHL, MEDLINE, PubMed, and Scopus.
• Studies included adult subjects with primary BCS, hepatic vein outflow tract obstruction, inferior vena cava (IVC) obstruction, or HVT.

Main Results:

• Classical BCS predominantly affects women with pure hepatic vein obstruction, presenting acutely with abdominal pain, ascites, and hepatomegaly.
• HVC-BCS more commonly affects men, with obstruction in both the IVC and hepatic veins, presenting chronically with abdominal pain and abdominal wall varices.
• Myeloproliferative neoplasms (MPN), particularly JAK2V617-F mutation, are a major cause of classical BCS (16%-62%), but less frequent in HVC-BCS (4%-5%).
• Classical BCS responds to medical management, while HVC-BCS often requires percutaneous recanalization as first-line treatment.

Conclusions:

• Current data suggest that classical BCS and HVC-BCS are clinically distinct disorders.
• These conditions share the common feature of disrupted hepatic venous outflow but differ significantly in their clinical profiles.
• Further research may elucidate more precise diagnostic and therapeutic approaches for each entity.