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Budd-Chiari Syndrome

Phuong L. Doan, MD

University of North Carolina HospitalsMorning Report

April 22, 2005

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Case- QC

41M w/ RUQ pain X 3 months Constant, progressive

Nausea, no vomiting, not assoc. w/ food

Bilateral lower quadrant compressible masses

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Case- PMH

Bilateral LE DVT 1999 Without travel hx

Hypercoagulable evaluation unremarkable

Microcytic anemia, hgb 8- 10 g/dL Negative endoscopies and bone marrow biopsy

Hematuria

Gynecomastia, 1999

Bilateral fine needle aspirations negative for

malignancy

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Case- Physical Exam

Afebrile No resp distress

No JVD, no hepatojugular reflux Abd obese, nontender, enlarged liver,

spleen, no ascites, B LQ subcutaneous

compressible masses

Calves symmetric, nontender

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Outline

Definition Pathogenesis

Causes Clinical presentation

Differential diagnosis Diagnostic laboratories, imaging

Management

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Budd-Chiari Syndrome

Hepatic outflow obstruction

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Pathogenesis

Outflow obstruction Increase sinusoid pressure and portal

hypertension

Decrease hepatic portal venous perfusion

Hypoxia! free radical production and

oxidative injury Centrilobular hepatocyte necrosis, fibrosis,

hyperplasia, and cirrhosis

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Pathogenesis

Central vein

Sinusoids

Hepatocyte

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Pathology

Central veins

Sinusoids

Thrombus

Normal Thrombosis

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Common Causes

Hypercoagulable state

Hematologic abnormalities:

Myeloproliferative disorders:

Polycythemia Vera, essential thrombocythemia,myelofibrosis

Paroxysmal nocturnal hemoglobinuria

Antiphospholipid antibody syndrome Factor V Leiden

Protein C, S, and antithrombin III deficiency

Prothrombin gene mutation Methylenetetrahydrofolate reductase mutation

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Uncommon Causes

Tumor invasion

Hepatocellular

Renal cell

Adrenal

Miscellaneous

Infection: TB, aspergillosis, syphilis

Trauma Inflammatory bowel disease

Idiopathic

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Clinical Presentation

Fulminant, acute, subacute, chronic Fulminant: hepatic encephalopathy

Acute: hepatic necrosis, intractable ascites, no

collateral vessels

Subacute: minimal ascites, hepatic and portal

collaterals with sinusoid decompression

Chronic: cirrhosis, hepatosplenomegaly,

esophagogastric varices, collaterals

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Collateral Vessels- QC

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Chronic Budd-Chiari Syndrome

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Differential Diagnosis

Cardiac: tricuspid regurgitation, constrictivepericarditis, right atrial myxoma

Hepatitis

Choleycystitis

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Diagnosis

Laboratories: AST, ALT > 5x nl: fulminant and acute

Elevated AP, T bili; decreased serum albumin

High serum-ascitic fluid albumin gradient

Ascitic fluid total protein > 2.5 g/dL

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Diagnosis- Imaging

Doppler ultrasonography of liver > 85% sensitivity and specificity

Contrast-enhanced computed tomography

Magnetic resonance imaging

Hepatic venography

Portacaval venous pressure gradient Transjugular liver biopsy

(Echocardiography)

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Computed Tomography

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Venography

Collaterals

Venography

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Medical Management

Negative sodium balance Anticoagulation

Thrombolytics Angioplasty

Transjugular intrahepatic portosystemic

shunts (TIPSS)

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TIPSS

Indicated in bridge to liver transplantation, acute BCS,

subacute BCS if low portocaval pressure gradient

Complicated with shunt stenosis, liver transplantation

interference

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Surgical Management

Shunts:

peritoneovenous

portosystemic

mesocaval

74-95% survival at 5 yrs

Liver transplantation

> 95% survival at 5 yrs Indicated in fulminant hepatic failure, cirrhosis,

portosystemic shunt failure, favorable prognosis ofunderlying disease

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Case- Follow up

Restarted on heparin and coumadin Pain, nausea resolved

Subcutaneous collaterals diminished overtwo days

Home on coumadin

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References Bogin et al. Budd-Chiari syndrome: in evolution. Eur J

Gastroenterol Hepatol. 2005; 17:33-5. Denninger et al. Cause of portal or hepatic venous

thrombosis in adults: the role of multiple concurrent

factors. Hepatology 2000; 31: 587-591. Menon et al. The Budd-Chiari syndrome. NEJM 2004;

350: 578-585.

Ruh et al. Management of Budd-Chiari syndrome.Digestive Diseases and Sciences 2005; 50: 540-546.

Zettoun et al. Outcome of Budd-Chiari syndrome: amultivariate analysis of factors related to survival

including surgical portosystemic shunting. Hepatology1999; 30: 84-89.