History and exam
Key diagnostic factors
common
right upper quadrant abdominal pain
Present in many patients (61%) with Budd-Chiari syndrome (classic presentation).[1]
hepatomegaly
Present in many patients (67%) with Budd-Chiari syndrome (BCS; classic presentation).[1] The liver is usually tender in cases of BCS.
ascites
Present in many patients (83%) with Budd-Chiari syndrome (classic presentation).[1]
uncommon
jaundice
Present in many patients with acute Budd-Chiari syndrome.
Other diagnostic factors
common
splenomegaly
Often present in a significant number of patients at time of diagnosis.[3] May be a consequence of the underlying myeloproliferative disease, portal vein thrombosis, or a sequela of cirrhosis (with portal hypertension) that develops in the chronic form of Budd-Chiari syndrome.
uncommon
gastrointestinal bleeding
Occurs as a complication of portal hypertension in chronic form of Budd-Chiari syndrome (cirrhosis) and in cases with portal vein thrombosis.
leg edema
Present in patients with obstruction of the inferior vena cava.
truncal venous collaterals
Present in patients with obstruction of the inferior vena cava.
hepatic encephalopathy
Encephalopathy is manifested by disturbances of consciousness, intellectual function, behavior, and neuromuscular function. This may occur in the case of fulminant and chronic forms of Budd-Chiari syndrome.
Risk factors
strong
female sex
More common in women in their third or fourth decade of life.[15]
myeloproliferative disorders (MPD)
50% of patients are shown to have an underlying MPD, such as polycythemia vera, essential thrombocytosis, or myelofibrosis.[32]
thrombophilic disorders
Factor V Leiden mutation is found in about 30% of patients with Budd-Chiari syndrome.[19]
Other inherited thrombophilic disorders include prothrombin factor gene mutation, methylenetetrahydrofolate reductase mutation, protein C and S deficiency, and antithrombin III deficiency.[20][24]
Acquired thrombophilic conditions include antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, and Behcet syndrome.
weak
oral contraceptive pill use
pregnancy and immediate postpartum period
Pregnancy and immediate postpartum period are associated with hormonal changes, inferior vena cava compression, and physiologic hyperfibrinogenemia, which predisposes to Budd-Chiari syndrome.[6]
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