title: Budd-Chiari syndrome in children: clinical features, percutaneous radiological intervention, and outcome.
abstract: OBJECTIVE
  '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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Departments of aPediatric Gastroenterology bRadiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
authors.forename: Rachana
authors.initials: R
authors.lastname: Kathuria
authors.forename: Anshu
authors.initials: A
authors.lastname: Srivastava
authors.forename: Surender K
authors.initials: SK
authors.lastname: Yachha
authors.forename: Ujjal
authors.initials: U
authors.lastname: Poddar
authors.forename: Sanjay S
authors.initials: SS
authors.lastname: Baijal
mesh_terms: D000293:Adolescent; D000800:Angioplasty, Balloon / Q000379:methods; D006502:Budd-Chiari Syndrome / Q000175:diagnosis* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005260:Female; D005500:Follow-Up Studies; D006503:Hepatic Veins / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D019168:Portasystemic Shunt, Transjugular Intrahepatic / Q000379:methods; D015641:Radiography, Interventional / Q000379:methods*; D015607:Stents; D016896:Treatment Outcome; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging