Does Budd-Chiari Syndrome Cause Varices?
Yes, Budd-Chiari syndrome definitively causes varices, including both esophageal and gastric varices, as a direct consequence of portal hypertension resulting from hepatic venous outflow obstruction.
Mechanism of Varix Formation
Budd-Chiari syndrome causes postsinusoidal portal hypertension by obstructing hepatic venous outflow anywhere from the small hepatic venules to the inferior vena cava entrance into the right atrium 1. This venous obstruction leads to:
- Sinusoidal congestion that increases intrahepatic pressure 1
- Portal hypertension with the same complications seen in cirrhosis, including varices 2
- Development of collateral venous pathways including esophageal and gastric varices 3
Clinical Evidence of Varices in Budd-Chiari Syndrome
The presence of varices in Budd-Chiari syndrome is well-documented across multiple studies:
- Esophageal varices occur in approximately 56% of patients with Budd-Chiari syndrome 4
- Upper gastrointestinal bleeding from varices was reported in 22% of a large case series 4
- Variceal bleeding during pregnancy occurred in three women with Budd-Chiari syndrome in one cohort, none of whom had received appropriate primary prophylaxis 5
- Ectopic varices can also develop, with a high rate of recurrent bleeding (42% within 48 hours after TIPS) despite hemodynamic correction 5
Management Implications
Portal hypertension complications, including varices, should be managed following the same guidelines as for cirrhosis 2. This includes:
- Primary prophylaxis with beta-blockers or endoscopic variceal ligation for high-risk varices
- Secondary prophylaxis after variceal bleeding episodes
- TIPS consideration for recurrent variceal bleeding despite adequate endoscopic and medical treatment 5
Critical Clinical Pitfall
The presence of esophageal varices is an independent predictor of major bleeding in Budd-Chiari syndrome patients receiving anticoagulation therapy 6. This creates a management challenge since:
- Anticoagulation should be initiated as soon as possible and continued indefinitely 2
- However, bleeding complications occurred in 47 patients (22.8 per 100 patient-years) in one study, with 15 episodes related to portal hypertension 6
- Reinforced prophylaxis for portal hypertension is essential to improve the benefit-risk ratio of anticoagulation 6
Diagnostic Considerations
When evaluating for varices in Budd-Chiari syndrome:
- Upper endoscopy should be performed to assess for esophageal and gastric varices 7, 8
- Doppler ultrasound may show intrahepatic or subcapsular hepatic venous collaterals (found in >80% of cases) 9
- Varices represent one manifestation of the broader portal hypertension syndrome that includes ascites (present in 83% at diagnosis) and hepatomegaly (67%) 1