Suprahepatic Budd-Chiari syndrome treated with thrombectomy and cavoplasty.
The case of a 42-year-old female with Budd-Chiari syndrome (BCS) and lupus anticoagulant is reported. This patient, who had been on chronic anticoagulants for her lupus anticoagulant, presented with abdominal pain, dyspnea on exertion, engorged abdominal wall venous collaterals, and hepatomegaly. A dynamic computerized tomography of the abdomen showed complete suprahepatic inferior vena caval occlusion at the junction with the right atrium. IVC venogram confirmed the diagnosis and also demonstrated patency of the hepatic veins. Free hepatic venous pressure was 25-26 mm Hg. Histopathologic examination of the liver showed marked central venous congestion with significant bridging fibrosis. The total caval occlusion and overt calcification of the clot precluded radiologic angioplasty, and the patient underwent a successful surgical thrombectomy with cavoplasty utilizing an autologous venous patch. Several weeks following surgery, she was free of symptoms and resumed her usual daily activities. Follow-up venography showed a widely opened cava with normal free hepatic vein pressures. Repeat liver biopsy at 6 months showed complete resolution of the hepatic venous congestion and a decrease in the degree of fibrosis.
Rhee
John
J
Department of Medicine, Division of Gastroenterology and Hepatology, the Starzl Institute University of Pittsburgh, School of Medicine, Pittsburgh, Pennsylvania 15213, USA.
Demetris
Anthony J
AJ
Abu Elmagd
Kareem
K
Rabinovitz
Mordechai
M
D000328:Adult; D001707:Biopsy, Needle; D006502:Budd-Chiari Syndrome / Q000097:blood / Q000000981:diagnostic imaging / Q000601:surgery*; D003097:Collateral Circulation / Q000502:physiology; D005260:Female; D006801:Humans; D019934:International Normalized Ratio; D008099:Liver / Q000473:pathology; D008111:Liver Function Tests; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000000981:diagnostic imaging / Q000601:surgery; D010690:Phlebography; D011183:Postoperative Complications / Q000000981:diagnostic imaging; D017131:Thrombectomy; D014057:Tomography, X-Ray Computed; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging / Q000601:surgery*
D016682:Lupus Coagulation Inhibitor