Budd–Chiari syndrome in children
| Autor: | Rachana Kathuria, Surender Kumar Yachha, Anshu Srivastava, Ujjal Poddar, Sanjay S. Baijal |
|---|---|
| Rok vydání: | 2014 |
| Předmět: |
Male
medicine.medical_specialty Percutaneous Adolescent medicine.medical_treatment Vena Cava Inferior Budd-Chiari Syndrome Hepatic Veins Radiography Interventional Inferior vena cava Angioplasty Ascites medicine Humans Child Vein Hepatology business.industry Gastroenterology Stent medicine.disease Surgery Treatment Outcome medicine.anatomical_structure medicine.vein Child Preschool Chronic Disease Budd–Chiari syndrome Female Stents Portasystemic Shunt Transjugular Intrahepatic medicine.symptom business Transjugular intrahepatic portosystemic shunt Angioplasty Balloon Follow-Up Studies |
| Zdroj: | European Journal of Gastroenterology & Hepatology. 26:1030-1038 |
| ISSN: | 0954-691X |
| DOI: | 10.1097/meg.0000000000000144 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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