Endovascular Management of Supra Hepatic IVC Budd-Chiari Syndrome in a 12 year old Boy: Case Report

Abstract

Primary Budd-Chiari Syndrome (BCS) is a rare condition affect-ing one in a million adults, even more rare in children. It is a rare form of hepatic venous outpatient obstruction at the supra hepatic Inferior Vena Cava (IVC), the hepatic veins or both. As this syndrome is un-common, especially in paediatric age group, misdiagnosis and delay in diagnosis are frequent. A high index of clinical suspicion along the radiological and histological correlation can lead to early diagnosis and appropriate management. The most common BCS symptom is ascites. Our case was a 12 year old Sudanese boy, suffering of man-ifestations of portal hypertension, ascites, and hepatosplenomegaly. He was treated by Angioplasty and Stenting of the supra hepatic IVC, with good outcome and improvement of symptoms. Published literature on interventional procedure for Budd-Chiari Syndrome in children and adolescents is scare. The management of such cases was not unified in all the reports. Percutaneous endovascular angio plasty and stenting of the suprahepatic IVC