Budd Chiari Syndrome

With a liver pathology of your choice, what techniques are there to investigate the liver and what are their advantages and disadvantages?

Budd Chiari Syndrome
•Clinical term used to describe the manifestation of hepatic venous outflow obstruction •Secondary to hepatic vein thrombosis •Or to the narrowing/occlusion of the inferior vena cava
(Khan 2009,Val DC 2003)

Ostial stenosis

web

thrombus

Abdominal vein Liver

Stomach Gallbladder Enlarged caudate lobe Inferior vena cava

Portal vein

(Menon et al., 2004)

Budd Chiari Syndrome
• Primary type
-Endoluminal venous (I.e. Thrombosis of lumen) • Hypercoagulable state -polycythaemia vera -Protein C deficiency -Antiphospholipid antibody - Medication (female hormones,
Hepatocellular carcinoma) - Extrinsic compression (tumours)
(Kumar and clark, 2005,Brancatelli et al., 2007)

Budd Chiari Syndrome
• Results in portal hypertension • May lead to hepatocellular necrosis and chronic liver disease • Clinically mistaken as liver cirrhosis • Biochemistry test (LFTs) is non-specific • Liver biopsy is nonspecific, and sometimes appear normal
- Peripheral liver may show hepatocellular necrosis, and centrally ‘normal’ liver

• Imaging diagnosis is essential
(Perello A et al., 2002, McCuskey RS, 2000)

• Dependant on cause and acute/chronicity of Budd Chiari syndrome

Budd Chiari Syndrome Image Findings

• Acute:
Normal liver morphology Ascites Obstruction of hepatic vein and inferior vena cava Peripheral hypo/central hyper enhancement of
Federle, Antoine Hakime, Roberto Lagalla, Riccardo Iannaccone, Dominique Valla (2007). Budd-Chiari Syndrome: Spectrum of Imaging Findings. ARJ: 188 Khan Nawaz Ali (2009). Budd-Chiari Syndrome. Consultant Radiologist and Honorary Professor, North Manchester General Hospital Pennine Acute NHS Trust, UK http://emedicine.medscape.com/article/364420-overview (Date Accessed 19/3/2011) Kumar Parveen (2005). Budd Chiari Syndrome. Kumar and Clark’s Clinical medicine. Pg 136-137. 6th edition. London ; W.B. Saunders. McCuskey RS (2000). Morphological mechanisms for regulating blood flow through hepatic sinusoids. Liver;20:3-7 Olliff simon (1996). Radiological diagnosis and treatment of Budd Chiari Syndrome. Journal of Interventional Radiology, 11:9-13 Perelló A, García-Pagán JC, Gilabert R, Suárez Y, Moitinho E, Cervantes F, Reverter JC, Escorsell A, Bosch J, Rodés J (2002). TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy. Hepatology;35(1):132–139 Radiological Society of North America (Radiologyinfo.org, 2011). Radiology Test/ Treatment information. http://www.radiologyinfo.org/ (Date accessed