Clinical Presentations of Hepatic Vein Thrombosis
Hepatic vein thrombosis (Budd-Chiari syndrome) typically presents with the triad of abdominal pain, ascites, and hepatomegaly, though clinical presentation can range from asymptomatic to fulminant hepatic failure. 1
Acute Hepatic Vein Thrombosis (Symptoms ≤8 weeks)
Common Clinical Manifestations
- Abdominal pain (particularly right upper quadrant)
- Ascites (present in 83% of patients at diagnosis)
- Hepatomegaly (present in 67% of patients)
- Abdominal distention
- Nausea and vomiting
- Anorexia
- Fever
Less Common Manifestations
- Jaundice (relatively uncommon)
- Gastrointestinal bleeding
- Hepatic encephalopathy
- Lower extremity edema
Laboratory Findings
- Serum aminotransferases: normal to elevated
- Alkaline phosphatase: normal to elevated
- Serum albumin: normal to decreased
- Serum bilirubin: normal to elevated
- Prothrombin time: normal to prolonged
- Ascitic fluid: high protein content (>3.0 g/dL)
- Serum-ascites albumin gradient: ≥1.1 g/dL
Chronic Hepatic Vein Thrombosis (Symptoms >8 weeks)
Chronic cases may be asymptomatic due to the development of venous collaterals, but can present with:
- Portal hypertension manifestations
- Splenomegaly
- Lower extremity edema
- Abdominal pain (less severe than in acute cases)
- Anorexia and nausea
- Marked dilation of subcutaneous veins on the trunk (specific for IVC block) 1
Asymptomatic Presentation
Up to 20% of hepatic vein thrombosis cases may be asymptomatic, particularly when large hepatic venous collaterals have developed 1, 2. These patients typically have:
- No ascites
- No abdominal pain
- Large intrahepatic and portasystemic collaterals
- Better prognosis compared to symptomatic patients 2
Imaging Findings
Doppler Ultrasound Findings (First-line Investigation)
- Absent flow signal in hepatic veins
- Reversed or turbulent flow
- Large intrahepatic or subcapsular collaterals
- Spider-web appearance near hepatic vein ostia
- Absent or flat hepatic vein waveform
- Hyperechoic cord replacing normal vein 1
CT/MRI Findings
- Caudate lobe hypertrophy (in 75% of patients)
- Early homogeneous central enhancement
- Delayed patchy enhancement of liver periphery
- Prolonged contrast retention in periphery 1
Risk Factors to Assess
- Myeloproliferative neoplasms (especially with JAK2V617F mutation)
- Paroxysmal nocturnal hemoglobinuria
- Inherited thrombophilia (protein C, protein S, antithrombin deficiency)
- Factor V Leiden mutation
- Prothrombin G20210A gene variant
- Antiphospholipid syndrome
- Oral contraceptive use
- Recent abdominal surgery
- Abdominal malignancies
- Pancreatitis
- Cirrhosis 1
Diagnostic Approach
Laboratory testing:
- Complete blood count with differential
- PT, aPTT
- Basic metabolic profile
- Hepatic profile
- Serum lactate 1
Imaging:
- Doppler ultrasound (diagnostic sensitivity >75%)
- MRI or CT with vascular contrast if ultrasound is inconclusive
- Venography for uncertain diagnosis or to characterize anatomy before treatment 1
Prognostic Considerations
The severity of presentation correlates with the extent of venous occlusion and development of collaterals. Patients with asymptomatic hepatic vein thrombosis generally have better outcomes, while those with mesenteric vein involvement are at risk for intestinal infarction and have poorer prognosis 1, 2.
Important Clinical Pitfalls
Missing the diagnosis: Hepatic vein thrombosis can be misdiagnosed as other causes of ascites or hepatomegaly. Always consider this diagnosis in patients with unexplained ascites, especially with high protein content.
Overlooking asymptomatic cases: With modern imaging techniques, asymptomatic cases are increasingly detected and should not be dismissed.
Failing to assess for underlying prothrombotic conditions: All patients should be evaluated for underlying thrombophilic disorders, as these impact management and prognosis.
Delayed treatment: Early anticoagulation is critical to prevent clot extension and new thrombotic episodes in symptomatic patients 1, 3.