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Revista colombiana de Gastroenterología
Print version ISSN 0120-9957
Abstract
CORREA GAVIRIA, Simón; RAMIREZ, Ana Catalina; ESPINOZA HERRERA, Yeinis Paola and RESTREPO GUTIERREZ, Juan Carlos. A Review of Budd Chiari Syndrome. Rev Col Gastroenterol [online]. 2016, vol.31, n.3, pp.242-252. ISSN 0120-9957.
Budd - Chiari Syndrome (BCS) is a rare entity whose incidence is estimated at one in one hundred thousand. It consists of complete or partial obstruction of venous outflow at any location from the small hepatic veins to the hepatic portion of the inferior vena cava. It can be classified according to its etiology into primary BCS when there is venous obstruction and secondary disease when obstruction is attributed to extrinsic compression or invasion due to a lesion outside of the veins such as neoplasms or cysts. In most cases it presents as sudden onset of abdominal pain, ascites and hepatomegaly, but it may be asymptomatic. A definitive diagnosis is established by imaging, but basic laboratory tests and other studies must also be done. Diagnostic imaging techniques include Doppler ultrasonography, computed tomography, magnetic resonance imaging and digital subtraction angiography. The latter is considered to be the gold standard. The first therapeutic measure to be undertaken for these patients is anticoagulation with low molecular weight heparin followed by vitamin K antagonists. Most patients require a multidisciplinary approach and step by step treatment including radiological procedures, balloon enteroscopy, stenting, transjugular intrahepatic portosystemic shunt (TIPS), decompression surgery and finally, liver transplantation
Keywords : Budd-Chiari syndrome; Doppler ultrasonography; CT scan; Digital Subtraction Angiography; Liver Transplant.