Overview of Treatment Options in Budd Chiari Syndrome

The recommended treatment strategy for Budd Chiari Syndrome consists of a step wise approach (see Figure 1, Table 1).

Figure 1: Stepwise Approach to Management of Budd-Chiari Syndrome
**Rotterdam class III:  Scoring system that combines presence of encephalopathy, ascites, prothrombin time, and bilirubin with 5-year survival rate of 89% (95% CI, 79%-99%) for class I, 74% (95% CI, 65%-83%) for class II, and 42% (95% CI, 28%-56%) for class III[7]
 
 Taken from: Khan, F., et al., Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome. Aliment Pharmacol Ther, 2019. 49(7): p. 840-863

1. Treat predisposing conditions and prevent propagation of the clot with anticoagulation:

Back to the case

In the clinical case above, further work up revealed presence of JAK2 mutation, subnormal erythropoietin level and hypercellularity on a bone marrow biopsy – all consistent with a diagnosis of polycythemia vera.

2. Restore patency of thrombosed vein

Back to the case

Our patient was started on IV heparin. IR venogram confirmed occlusion and narrowing of middle and left hepatic vein. He underwent thrombolysis and angioplasty of both veins, with improved blood flow noted on post procedure angiogram. Repeat ultrasound one week later demonstrated flow in all three hepatic veins and liver enzymes returned to baseline values.

3. Creation of shunts can decompress the liver by creating an alternative venous outflow tract

4. Liver transplantation should be considered if:

Table 1: Summary of Treatment Options in Budd Chiari
Adapted from: DeLeve, L.D., et al., Vascular disorders of the liver. Hepatology, 2009. 49(5): p. 1729-64. & Hernández-Gea V, et al. Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. J Hepatol. 2019;71(1):175-199.