Budd chiari syndrome
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Budd-Chiari syndrome is caused by obstruction of the hepatic veins that drain the liver. It presents as either acute or chronic disease. Acute disease results from sudden thrombosis while chronic disease involves fibrosis. Imaging findings include enlarged caudate lobe, ascites, inability to visualize hepatic veins, and collateral vessel formation. Treatment involves identifying the underlying cause of obstruction and considering interventions like stenting or transplant to relieve pressure in severe cases.
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Budd chiari syndrome
- 1. BUDD CHIARI SYNDROME BY DR.JINO JUSTIN
- 2. Budd–Chiari syndrome is a condition caused by occlusion of the hepatic veins that drains the liver.
- 3. 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.
- 5. 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%
- 6. Pathogenesis HEPATIC VEIN THROMBOSIS Sinusoidal pressure Sinusoidal flow Sinusoidal dilatation+Interstitial fluid filtration Fluid passes through hepatic capsule(Ascitis)
- 7. portal vein pressure & perfusion of liver via portal vein Hypoxia of hepatocyte inflammatory centrilobular cell necrosis Release of free oxygen radicals Atrophy
- 8. chronic weeks of obstruction fibrosis of centrilobar area nodular regeneration in periportal area cirrhosis portovenous . collateral cirrhosis
- 9. 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
- 10. 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.
- 11. 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.
- 12. 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
- 17. 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.
- 18. 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.
- 19. 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.
- 20. 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
- 23. 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
- 25. Angiographic Findings spiderweb pattern of collateral vessels Presence of thrombus. long segmental compression of the inferior vena cava caused by caudate lobe hypertrophy.
- 28. 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.
- 29. THANK YOU
Editor's Notes
- The term Budd-Chiari syndrome is applied to the clinical manifestations of hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava and the right atrium regardless of the cause of obstruction
- Fluid exceeds the capacity of lymphatic drainage
- Early diagnosis of Budd-Chiari syndrome is important for establishing appropriate treatment. Because of inhomogeneous distribution of disease in the liver.. myeloproliferative disease is the most common abnormality identified
- In acute disease the liver is enlarged with thrombosed hepatic veins (HV) and ascites, whereas in the chronic form of the disease there may be membranous occlusion of HV and/or the inferior vena cava
- Sagittal sonogram in the epigastric region showing massive caudate lobe enlargement (asterisks) causing compression of the IVC
- Thrombus in the middle and left hepatic veins seen as echogenic areas (thick arrows) in the transverse subcostal image. Narrowing is also seen at the distal end of the middle hepatic vein as it joins the IVC (thin arrow).
- Images through the epigastric region showing an intrahepatic collateral between hepatic
- The Doppler waveform of these HV with intrahepatic collaterals is invariably monophasic as opposed to the normal patent HV which show a triphasic spectral pattern due to transmitted atrial pulsations.
- Occlusion of the hepatic veins Ascites and splenomegaly are usually present Decreased peripheral enhancement caused by portal and sinusoidal stasis
- allowing differential diagnosis from hepatocellular carcinoma
- Contrast-enhanced arterial phase computed tomography images. A: Nodular arterial enhancing lesions indicating nodular regenerative hyperplasia
- Portal venous phase image (A) shows heterogeneous and mottled enhancement pattern with central prominence (arrows) which becomes more homogeneous in delayed phase (B). Oblique reformatted images (C, D) along the axis of the right and left hepatic veins show unopacified hepatic veins (arrowheads).
- Gadolinium-enhanced T1-weighted MR image obtained during arterial phase shows numerous homogeneous hyperintense lesions
- Gadolinium-enhanced T1-weighted MR image showing occlusion of vein draining caudate lobe.
- the presence of thrombus within the hepatic veins or inferior vena cava. Because of hepatic vein obstruction, collateral channels developed within liver.
- Hepatocellular carcinoma causing Budd-Chiari syndrome. CECT scan obtained during portal phase shows hepatocellular carcinoma invading and expanding inferior vena cava (vertical arrow). Note hepatocellular carcinoma satellite lesion (arrowhead) and metastatic lesion (horizontal arrow) involving right chest
- Budd-Chiari syndrome 30 days after receiving liver transplant from live donor. CECT scan obtained during portal phase shows occlusion of branch of middle hepatic vein (arrow) with congestion, edema, and ischemia of anterior part of right lobe of liver. Sharp demarcation (arrowheads) is evident between normal and abnormally drained liver.