Budd-Chiari syndrome in children: clinical features, percutaneous radiological intervention, and outcome
Rachana Kathuria1, Anshu Srivastava, Surender K Yachha
1Departments of aPediatric Gastroenterology bRadiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Insights
Radiological intervention is effective for pediatric Budd-Chiari syndrome (BCS). This study shows intervention improves outcomes in children with BCS, with hepatic vein block being most common.
Area of Science:
- Pediatric Gastroenterology
- Interventional Radiology
- Vascular Medicine
Background:
- Budd-Chiari syndrome (BCS) in children is rare, with limited literature on treatment outcomes.
- Radiological interventions are standard for adult BCS, but their role in pediatric cases is less defined.
Purpose of the Study:
- To evaluate the clinical characteristics of pediatric Budd-Chiari syndrome.
- To assess the effectiveness and safety of radiological interventions in treating pediatric BCS.
Main Methods:
- Retrospective analysis of 46 children diagnosed with BCS.
- Interventions included angioplasty, stenting of hepatic veins (HV) and inferior vena cava (IVC), and transjugular intrahepatic portosystemic shunt (TIPS) in 25 patients.
- Clinical, biochemical, and radiological follow-up was conducted.
Main Results:
- Hepatic vein (HV) block was the most frequent diagnosis (n=33) in chronic pediatric BCS.
- Radiological interventions were technically successful in 100% of cases, leading to clinical and biochemical improvement.
- Stent patency was observed in 75% of cases at a median follow-up of 6.5 months, with low complication rates.
Conclusions:
- Budd-Chiari syndrome in children commonly presents with hepatic vein block and a chronic course.
- Doppler ultrasonography is a reliable diagnostic tool, achieving 95% diagnostic accuracy.
- Radiological intervention represents a safe and effective therapeutic option for pediatric BCS.
Objectives:
'Radiological intervention' to restore venous patency is the preferred therapy in adults with Budd-Chiari syndrome (BCS). The published literature on pediatric BCS is scarce. This study evaluated the clinical profile and role of a therapeutic radiological intervention in children with BCS.
Patients And Methods:
Forty-six BCS children [29 boys, median age 10.5 (2-16) years] were enrolled. Standard medical therapy was administered to all. A radiological intervention, angioplasty [hepatic vein (HV) (n=3)], stenting [HV (n=18), inferior vena cava (IVC) (n=5)], transjugular intrahepatic portosystemic shunt (TIPS) (n=3), was performed in 25 cases. Clinical, biochemical, and radiological follow-up was carried out.
Results:
Doppler ultrasonography was diagnostic in 95% of cases. All patients had chronic BCS, with ascites in 82.6%, hepatomegaly in 84.8%, splenomegaly in 69.6%, prominent abdominal veins in 69.6%, and variceal bleed in 34.8% cases. The most common site of block was HV (n=33), followed by combined HV and IVC block (n=11), and isolated IVC block (n=2). Eight of 12 (75%) cases had abnormal procoagulant workup. Radiological intervention was technically successful in 100%. Clinical and biochemical improvement was observed in the intervention group. Complications included neck hematoma and hemorrhagic ascites in one patient each. One child in the intervention group (post-TIPS sudden cardiac event) and two children in the nonintervention group [end-stage liver disease (n=1), head injury (n=1)] died. Stent was patent in 15/20 (75%) children over a median follow-up of 6.5 months.
Conclusion:
HV block and a chronic presentation are most common in BCS children. Doppler ultrasonography establishes the diagnosis in 95% of cases. Radiological intervention is an effective and safe therapeutic modality for children with BCS.
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