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.

Table of contents

1 Signs and symptoms
2 Analysis
3 Diagnosis
4 Treatment
5 Causes
6 Pathophysiology

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.

Analysis

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.

Treatment

With anticoagulant medication, generally unfractioned and warfarin.

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 cerebrovacular accidents.

Pathophysiology

Any obstruction of the venous vasculature of the liver is referred to as Budd-Chiari syndrome, from the venules to the right atrium.

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