Budd-Chiari syndrome
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In medicine (gastroenterology and hepatology), Budd-Chiari syndrome is the clinical picture caused by occlusion of the hepatic vein.
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Signs and symptoms
The syndrome presents with rapidly progressive abdominal pain, hepatomegaly (enlarged liver), ascites, and later the symptoms of hepatic dysfunction: elevated liver enzymes, encephalopathy.
A slower-onset form of hepatic venous occlusion is also recognised; this can be painless.
Often, the patient is known to have a tendency towards thrombosis, while Budd-Chiari syndrome can also be the first symptom of such a tendency.
Diagnosis
When Budd-Chiari syndrome is suspected, measurements are made of liver enzyme levels and other organ markers (creatinine, urea, electrolytes, LDH).
Budd-Chiari syndrome is diagnosed using ultrasound studies of the abdomen, although occasionally more invasive methods have to be used (retrograde angiography). Liver biopsy is sometimes necessary to differentiate between Budd-Chiari syndrome and other causes of hepatomegaly and ascites, such as galactosemia or Reye's syndrome.
Causes
- Primary (75%): thrombosis of the hepatic vein
- Secondary (25%): compression of the hepatic vein by an outside structure (e.g. a tumor)
Many patients (10-40%) have Budd-Chiari syndrome as a complication of polycythemia vera (myeloproliferative disease of red blood cells). Patients suffering from paroxysmal nocturnal hemoglobinuria (PNH) appear to be especially at risk for Budd-Chiari syndrome, more than other forms of thrombophilia: up to 40% develops Budd-Chiari, as well as cerebrovascular accidents.
A related condition is veno-occlusive disease, which occurs in recipients of bone marrow transplants as a complication of their medication. Although its mechanism is similar, it is not considered a form of Budd-Chiari syndrome.
Pathophysiology
Any obstruction of the venous vasculature of the liver is referred to as Budd-Chiari syndrome, from the venules to the right atrium.
Treatment
Treatment is with anticoagulant medication, generally unfractioned heparin and warfarin.
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