{"id":2015,"date":"2009-12-26T13:07:40","date_gmt":"2009-12-26T18:07:40","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=2015"},"modified":"2009-12-26T13:15:35","modified_gmt":"2009-12-26T18:15:35","slug":"buddchiari-syndrome","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2009\/12\/buddchiari-syndrome\/","title":{"rendered":"Budd-Chiari Syndrome"},"content":{"rendered":"

Pathophysiology<\/strong><\/p>\n

1)<\/strong> key event is a decrease of hepatic venous flow 2)<\/strong> classically described as formation of clot at the junction of hepatic veins and vena cava Causes<\/em> – 3)<\/strong> hypercoagulable states (factor V Leiden, lupus, polycythemia vera, myeloproliferative disorders, sickle cell) 4)<\/strong> infectious (amebic liver abscess, hydatid cysts) 5)<\/strong> cancer (hepatocellular carcinoma) 6)<\/strong> pregnancy 7)<\/strong> membranous webs (40% of cases in Far East)<\/p>\n

Signs and Symptoms<\/strong><\/p>\n

1)<\/strong> sudden onset of hepatomegaly 2)<\/strong> abdominal pain 3)<\/strong> ascites 4)<\/strong> liver failure 5)<\/strong> onset can be fulminant (associated with pregnancy), acute, or chronic<\/p>\n

Histology\/Gross Pathology<\/strong><\/p>\n

1)<\/strong> liver parenchymal hemorrhage 2)<\/strong> central zone congestion 3)<\/strong> liver cell necrosis<\/p>\n

Associated Conditions<\/strong><\/p>\n

1)<\/strong> at risk for re-thrombosis, even after transplantation 2)<\/strong> requires lifelong anticoagulation<\/p>\n

Treatment<\/strong><\/p>\n

1)<\/strong> fulminant presentation with liver failure requires liver transplantation 2)<\/strong> chronic presentation with cirrhosis requires liver transplantation Some remaining function and reserve options<\/em> include – 3)<\/strong> thrombolytic agents 4)<\/strong> shunt procedures using a “side-to-side” structure (e.g., mesocaval or atrial-caval shunt that has a differential pressure of at least 10 mmHg 5)<\/strong> transjugular intrahepatic shunt (TIPS) procedure<\/p>\n

Tips for USMLE<\/strong><\/p>\n

if the patient is a 26 year-old white female who has lupus with acute onset of abdominal pain, abdominal distention, and palpable liver, think Budd-Chiari<\/p>\n","protected":false},"excerpt":{"rendered":"

Pathophysiology 1) key event is a decrease of hepatic venous flow 2) classically described as formation of clot at the junction of hepatic veins and vena cava Causes – 3) hypercoagulable states (factor V Leiden, lupus, polycythemia vera, myeloproliferative disorders, sickle cell) 4) infectious (amebic liver abscess, hydatid cysts) 5) cancer (hepatocellular carcinoma) 6) pregnancy […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[63],"tags":[631,630,632,633,635,634],"yoast_head":"\nBudd-Chiari Syndrome - 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