Hepatobiliary and pancreatic: Extensive thrombosis in Budd–Chiari syndrome

Abstract

A 41-year-old woman presented with a 1-week history of cholangitis. At endoscopic retrograde cholangiography, a benign biliary stricture was identified and treated with a stent. Unfortunately, sepsis persisted leading to the formation of multiple liver abscesses. Despite several courses of intravenous antibiotics, the patient remained bacteremic. To exclude endocarditis, an echocardiogram was carried out and revealed a 2 ¥ 2.4 cm mass in the right atrium. A magnetic resonance scan with T1-weighted images showed that the mass was a pedunculated thrombus that arose from the middle and right hepatic veins and extended into the inferior vena cava (IVC) and right atrium (Fig. 1). With T2-weighted images of the liver, abnormalities included a liver abscess (arrow), thrombosis in the middle hepatic vein (white signal when compared to patent vessels that are black) and edema in the territory of the middle hepatic vein (Fig. 2). Despite the extensive thrombosis, the patient had no clinical features of hepatic venous obstruction. Thrombolysis was excluded as a treatment option because of the risk of fatal pulmonary embolism. A decision was then made to perform an open thrombectomy via the supra-diaphragmatic IVC using general anesthesia with circulatory arrest. In addition, the liver abscesses were aspirated using ultrasound guidance. Histological evaluation revealed a large thrombus infected with fungi. Postoperatively, the patient was treated with a prolonged course of antibiotics. Budd–Chiari syndrome is a heterogenous group of disorders characterized by hepatic vein outflow obstruction. The disorder was first described by George Budd in 1844 and characterized by Hans Chiari in 1899. The natural history of the disorder is highly variable but unless venous obstruction and hepatic congestion are relieved, there is a high risk of hepatic failure and death. There are many predisposing factors, but to the authors’ knowledge, this is the only documented case with biliary sepsis as the causative agent.