BUDD CHIARI SYNDROME

BY DR.JINO JUSTIN

Budd–Chiari syndrome is a condition caused

by occlusion of the hepatic veins that drains the

liver.

Couinaud classification of

liver anatomy

Divides the liver into eight functionally indepedent

segments.

Each segment has its own vascular inflow, outflow and biliary drainage.

centre of each segment there is a branch of the

portal vein, hepatic artery and bile duct.

periphery of each segment there is vascular

outflow through the hepatic veins.

epidemology

m:f 1:2

3rd and 4th decade

Median age – 35

Location

Hepatic vein 62%

IVC 7%

Both IVC & hepatic veins 31%

Associated portal vein thrombus 14%

Pathogenesis

HEPATIC VEIN THROMBOSIS

Sinusoidal pressure

Sinusoidal flow

Sinusoidal dilatation+Interstitial fluid filtration

Fluid passes through hepatic capsule(Ascitis)

portal vein pressure & perfusion of liver via portal vein

Hypoxia of hepatocyte

inflammatory centrilobular cell necrosis

Release of free oxygen radicals

Atrophy

chronic

weeks of obstruction

fibrosis of centrilobar area

nodular regeneration in periportal area

cirrhosis portovenous

. collateral

cirrhosis

Etiology:

majority of patients have an underlying hematologic

abnormality.

Tumor

Hepatocellular carcinoma

Carcinoma of pancreas

Carcinoma of kidneys

Metastatic disease

Normal biopsy findings do not exclude this entity

Role of imaging:

Evaluation of occlusion of the hepatic veins and inferior vena

cava

Caudate lobe enlargement

Inhomogeneous liver enhancement and

Intrahepatic collateral vessels and hypervascular nodules.

Budd-Chiari syndrome Presents with - acute or

chronic form.

acute - results from an acute thrombosis of the hepatic veins or the IVC

Chronic form is related to fibrosis of the

intrahepatic veins.

Ultrasound findings

Enlargement of the caudate lobe.

Ascitis

Partial or complete inability to see the hepatic

veins ; stenosis with proximal dilatation, and

thrombosis

Narrowing of IVC due to compression by the

enlarged caudate lobe.

Color Doppler studies shows absent or flat or

reversed flow in the hepatic veins,IVC, or both

increased resistive index within the hepatic artery

- >0.75 is seen

Classification of BCS According to

the Level of Obstruction

Type I Obstruction of IVC with or without

secondary hepatic vein occlusion

Type II Obstruction of major hepatic veins

Type III Obstruction of the small centrilobular

venules.

CT:Acute BCS

The liver appears enlarged and swollen with presence of ascites.

decreased peripheral enhancement.

stronger enhancement of the central portion of the liver parenchyma

Thrombosed hepatic veins and IVC appears hypoattenunated.

inferior vena cava is compressed by the enlarged caudate lobe.

liver may have a heterogeneous appearance secondary to hemorrhage and infarction.

CT chronic BCS

Multiple regenerative nodules (diameter of 0.5–4.0 cm)

regenerative nodules are homogeneously hyperattenuating

on arterial phase and remain slightly hyperattenuating on

portal venous phase.

multiple tortuous intrahepatic collaterals may be seen.

CECT

Arterial phase:

--strongly enhancing nodular lesions or may show patchy enhancing areas due to arterio-portal shunting and opacification of portal vein.

The portal phase:

--patchy and mottled type of heterogeneous strong enhancement in the central part of the liver.

--poor or no enhancement in the periphery.

Delayed phase:

--enhancement pattern becomes more homogeneous

HV remain unopacified in all the phases

MRI findings

Regenerative nodules

are bright on T1-

weighted MR images

T2-weighted sequences

usually show

heterogeneously

increased signal intensity

in the peripheral portion

of the liver

Angiographic Findings

spiderweb pattern of

collateral vessels

Presence of thrombus.

long segmental

compression of the

inferior vena cava

caused by caudate

lobe hypertrophy.

Differential Diagnosis

Hepatic Cirrhosis:

Regenerative nodules smaller than BCS

Increased iron content

Patent HV & IVC

Primary sclerosing cholangitis

Chronic cholestatic disease of unknown cause

Mostly associated with ulcerative colitis.

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