What anticoagulant should be given to a child with portal hypertension and cirrhosis who develops Budd-Chiari syndrome?

Anticoagulation for Budd-Chiari Syndrome in Children with Cirrhosis and Portal Hypertension

Children with Budd-Chiari syndrome and cirrhosis should receive immediate and indefinite anticoagulation with low molecular weight heparin (LMWH) followed by vitamin K antagonist (VKA) therapy, targeting an INR of 2-3, regardless of the presence of portal hypertension. 1

Initial Anticoagulation Strategy

• Start LMWH immediately upon diagnosis for at least 5-7 days while simultaneously initiating oral VKA therapy 1
• LMWH can be discontinued once INR reaches therapeutic range (2-3) for two consecutive measurements 1
• Anticoagulation should be initiated as soon as possible to prevent clot extension and new thrombotic episodes 1
• Continue anticoagulation indefinitely, as this is a lifelong requirement for Budd-Chiari syndrome 1

Critical Pre-Treatment Requirements

Before initiating anticoagulation, you must:

• Screen for esophageal varices using upper endoscopy 2
• Ensure adequate prophylaxis for portal hypertension-related bleeding is in place 1
• Implement appropriate variceal management (beta-blockers or endoscopic band ligation) if varices are present 1, 2

This step is crucial because bleeding complications historically occurred in up to 50% of patients when portal hypertension was inadequately managed, though more recent data shows only 17% bleeding rates with proper prophylaxis 1.

Anticoagulant Selection in Pediatric Cirrhosis

For children with cirrhosis, the preferred regimen is:

• LMWH bridging to VKA (warfarin) as the standard approach 1
• DOACs are not recommended in pediatric populations due to lack of safety and efficacy data in children
• In adults with Child-Pugh A or B cirrhosis, DOACs would be acceptable 1, but this evidence does not extend to pediatric patients

The EASL guidelines specifically recommend VKA therapy for Budd-Chiari syndrome, making this the gold standard 1.

Management of Thrombocytopenia

If the child has thrombocytopenia from portal hypertension:

• Do not withhold anticoagulation for platelet counts >50 × 10⁹/L 1
• For platelet counts 25-50 × 10⁹/L, consider dose reduction based on thrombus burden (e.g., full dose for extensive hepatic vein thrombosis, reduced dose for limited thrombosis) 1
• For platelet counts <25 × 10⁹/L, evaluate risk of thrombus extension versus bleeding risk, considering platelet transfusion support during initial treatment period 1

Concurrent Treatment Considerations

Address the underlying prothrombotic disorder simultaneously:

• Investigate for myeloproliferative neoplasms, inherited thrombophilias, or other hypercoagulable states 1
• Early treatment of underlying conditions (e.g., cytoreductive therapy for MPNs) improves outcomes 1

Manage portal hypertension complications:

• Treat ascites and varices following standard cirrhosis management guidelines 1
• Consider TIPS if medical therapy with anticoagulation fails 1
• Evaluate for liver transplantation if poor prognostic features develop 1

Monitoring Protocol

• Monitor INR closely, targeting 2-3 throughout treatment 1
• Perform Doppler ultrasound early after treatment initiation, then every 6 months to assess hepatic vein patency 1
• Assess for bleeding complications at each visit, particularly gastrointestinal bleeding 3
• Evaluate liver function regularly, as disease severity predicts bleeding outcomes 3

Critical Pitfalls to Avoid

Common errors that increase bleeding risk:

• Failing to screen for and treat varices before anticoagulation 1, 2
• Excessive anticoagulation (INR >3) - this contributed to 27% of bleeding episodes in one series 3
• Inadequate monitoring during invasive procedures 1
• Interrupting anticoagulation prematurely, which risks thrombus extension 1

Bleeding during anticoagulation:

• Most major bleeding (43% in one study) was related to invasive procedures for BCS treatment 3
• Presence of esophageal varices is the strongest independent predictor of spontaneous bleeding 3
• Temporarily reduce or hold anticoagulation for major bleeding, but resume as soon as hemostasis is achieved 3

Escalation Pathway

If anticoagulation alone fails (persistent symptoms, thrombus extension):

1. Consider angioplasty with stenting if focal hepatic vein stenosis is present 1
2. Proceed to TIPS if angioplasty is not feasible or fails 1
3. Evaluate for liver transplantation if TIPS fails or contraindications exist 1

All children with Budd-Chiari syndrome should be managed at centers with expertise in vascular liver diseases and access to liver transplantation 1.