Budd Chiari Syndrome

Dr Ira Shah

doi:10.7199/ped.oncall.2014.50

Budd Chiari Syndrome

Dr Ira Shah.

Medical Sciences Department, Pediatric Oncall, Mumbai, India.

ADDRESS FOR CORRESPONDANCE
Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056

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Clinical Problem
A 3 months old girl presented with ascitis. There is no jaundice. Ultrasound abdomen with colour doppler showed ascitis with hepatomegaly with non-visualisation of hepatic veins. CT angiography showed thrombosis of all 3 hepatic veins suggestive of Budd-Chiari syndrome {BCS}.

How should such a child be managed_?

Discussion
The traditional approach to treatment involves systemic thrombolysis in all BCS patients without a specific contraindication. Surgical portosystemic shunt or TIPS is performed in progressive cases of BCS or as a bridge to transplantation. Patients with acute or fulminant BCS are best served with liver transplantation. Recently, there have been an increasing number of successful reports involving endovascular techniques including angioplasty and stent placement specially for hepatic webs. {1} Surgical shunting can lead to chronic hepatic encephalopathy. Balloon angioplasty of HV thrombosis has shown variable results throughout the world, restricting its use to short-segment HV thrombosis only. Angioplasty in infants is difficult due to small size of vessels. Thus, the choice of therapy would be difficult in this child considering the age. This child underwent dilatation of hepatic veins transjugularly but had no response. She then underwent portocaval shunt but continued to have ascitis. A liver biopsy at time of surgery showed nodular liver with cirrhosis. She was subsequently advised liver transplant but could not undergo same and died by 10 months of age.

Compliance with ethical standards

Funding: None

Conflict of Interest: None

References

Bozorgmanesh A,Selvam D,Caridi J. Budd-Chiari Syndrome: Hepatic Venous Web Outflow Obstruction Treated by Percutaneous Placement of Hepatic Vein Stent. Semin Intervent Radiol. 2007; 24: 100–105 [CrossRef] [PubMed] [PMC free article]

DOI: https://doi.org/10.7199/ped.oncall.2014.50

Cite this article as:
Shah I. Budd Chiari Syndrome. Pediatr Oncall J. 2014;11: 95. doi: 10.7199/ped.oncall.2014.50