Budd-Chiari syndrome

INTRODUCTION

Pathophysiologic process that results in an interruption or diminution of the normal flow of blood out of the liver, However, as commonly used, the Budd-Chiari syndrome implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava.

ETIOLOGY

An underlying disorder can be identified in over

80 % of patients with the Budd-Chiarisyndrome.

More than one thrombotic risk factors are present in many patients; 46 % had more than one risk factor in one series

A 2009 guideline from the American Association for the Study of Liver Diseases recommends the following approach for investigating causes of Budd-Chiari Syndrome:

• Evaluate for space occupying lesions or malignant tumors compressing or invading the hepatic venous outflow tract with sonography, CT scan, or MRI.

• Seek evidence for ulcerative colitis, celiac disease, and systemic diseases.

• Routinely evaluate for multiple, concurrent risk factors for thrombosis.

CLINICAL MANIFESTATIONS

One of the largest published series included a total of 237 patients who had been treated at four centers (in the United States, the Netherlands, and France) between 1984 and 2001. The following observations were made:

• The median age was 35 (range 13 to 76)

• 67 % were female

• An overt myeloproliferative disorder was present in 23 % (the majority of whom had polycythemia vera)

• The two most common symptoms were ascites (84 %) and hepatomegaly (76 %); 11 patients (5 %) were asymptomatic

• The hepatic outflow obstruction was in the hepatic veins (62 %) inferior vena cava (7 %) or both (31 %); 34 patients (14 %) had associated portal vein thrombosis

Acute disease

• Commonly in women.• Patients usually present with severe right upper

quadrant pain • Hepatomegaly. Jaundice and ascites often develop

rapidly. • Ascites is detectable by ultrasound in more than 90

percent of patients. Variceal bleeding may also occur

• Serum aminotransferase concentrations can range from 100 to 200 int. unit/L to more than 600 int. unit/L

Subacute and chronic disease

• Present for several weeks to more than six months prior to clinical presentation

• Hypertrophy of the caudate lobe of the liver

• Cirrhosis may have developed in the chronically congested liver.

• Patients may then develop ascites, which may be massive.

• Hepatomegaly and abdominal pain are also common.

• encephalopathy is infrequent.

• Hepatopulmonary syndrome has been described in up to 28 % of patients.

• normal or mild to moderate elevation of serum aminotransferases, alkaline phosphat.

• Jaundice is rare.

Diagnosis

• Doppler ultrasonography

• CT scan

• Magnetic resonance imaging

• Venography

• Arteriography

• Liver biopsy

liver biopsy

• In 2009 AASLD recommended liver biopsy ONLY when hepatic venous outflow obstruction cannot be demonstrated by non invasive imaging

11

Management

Medical therapy

• Supportive: ascites

• Anticoagulation: AASLD recommend anticoagulation only in patients with chronic and subacute disease with well compensated liver.

• Thrombolytic therapy: NOT in chronic Bud Chiari and ONLY in patients in whom the clot is well defined on venography

Surgical options

• Angioplasty

• TIPS

• Surgical shunts

• Liver Transplantation

14