A 61‐year‐old woman was admitted with acute jaundice. She was in her usual state of health until 2 weeks prior to admission, when she developed progressive jaundice, dull right upper quadrant pain, and lower‐extremity edema. Her medical history included a deep venous thrombosis while taking oral contraceptives and a pulmonary embolism after a surgery. Physical examination revealed icteric sclera and jaundiced skin. She was alert, but oriented to only person and place. She had mild right upper quadrant pain with palpation; she did not have hepatomegaly or ascites. Asterixis and 2+ lower‐extremity edema were present. Laboratory studies showed a total bilirubin of 6.3 mg/dL, a direct bilirubin of 4.7 mg/dL, AST of 487 mg/dL, ALT of 336 mg/dL, and an INR of 1.9; other liver function tests, chemistries, and CBC were normal. Serologies for hepatitis A, B, and C and EBV, HSV, and CMV were negative. An abdominal MRI demonstrated an atrophied left hepatic lobe; associated biliary ducts and vasculature were atretic. Hepatic venogram revealed an acute short‐length right hepatic vein thrombosis partially involving the inferior vena cava, as well as chronic occlusion of all other outflow segments. Acute‐on‐chronic Budd–Chiari syndrome was diagnosed. A stent was placed in the right hepatic vein. Jaundice, edema, and pain improved, but her coagulopathy did not resolve. She was anticoagulated with warfarin and placed on the liver transplant waiting list.
Budd–Chiari syndrome is rare, with an estimated incidence of less than 1 case per million individuals per year. It is diagnosed when hepatic outflow obstruction occurs anywhere between the hepatic veins and the terminal IVC. An underlying hypercoagulable state is identified in 75% of cases; primary myeloproliferative disorders are most commonly identified. Other causes include paroxysmal nocturnal hemoglobinuria, antiphospholipid antibody syndrome, oral contraceptive use, and inherited deficiencies of protein C, protein S, or antithrombin III. Clinical manifestations do not appear until at least 2 hepatic veins are occluded; if obstruction occurs slowly and is accompanied by extensive collateral development, the patient may remain asymptomatic. In this case, the severely atrophied left hepatic lobe suggests a slow thrombosis of the left hepatic vein, resulting in asymptomatic necrosis as collateral vessels developed. The damage was clinically undetectable until the final thrombotic insult occurred. This acute‐on‐chronic presentation of Budd–Chiari syndrome carries a relatively poor prognosis.
Although rare, Budd–Chiari syndrome carries a high mortality rate. Acute, chronic, and acute‐on‐chronic presentations have all been identified. Early identification of the syndrome and diagnosis of the underlying hypercoagulable state allow for early initiation of anticoagulation, which prevents the most severe complications and decreases mortality.
MRI of liver. (1) Atrophy of the left lobe of liver, associated with mild left‐sided biliary ductal dilatation. Status post cholecystectomy. (2) Heterogeneous enhancement of the liver. There are multiple tiny hypervascular foci, which are nonspecific, and could represent perfusion anomalies.