We describe eleven children (median age 10.5 months) with Budd Chiari syndrome in whom radiological intervention was attempted. The commonest presentation was ascites. Venogram of hepatic veins revealed an ostial block in 5, long segment block in 3, extrinsic compression from a diffuse hepatoblastoma in 1; intrinsic IVC narrowing in 1, IVC and hepatic venous block in 1 patient. In 5 with ostial blocks a dilatation or stent placement was possible with resolution of ascites. In one patient, the hepatopulmonary syndrome completely resolved. Long segment blocks were dilated with no resolution of the ascites in two and one patient died immediately post procedure. IVC stents were placed in 2 who had resolution of ascites and oedema feet. Of the 6 patients with successful interventions, four are well (follow up 1 m to 3 years), one lost to follow up and one died 6 months later. Only one of the 5 patients with failed interventions is alive for 2 ½ years, controlled on diuretics. The disease pattern in children is similar to that seen in Western adults with hepatic vein blocks seen often in infancy. Interventional radiological procedures are successful in short ostial blocks, long term outcome needs to be studied.

Pedgastro 2005 - Conference Abstracts.Pediatric Oncall [serial online] 2006 [cited 15 March 2006(Supplement 3)];3. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/
budd_chiari_syndrome.asp