Diagnostic Ultrasound - Abdomen and Pelvis

Budd-Chiari Syndrome
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Diagnoses: Liver
○ HV/IVC tumor propagation
– Hepatocellular carcinoma (most common); also
cholangiocarcinoma and rarely metastases,
angiosarcoma of IVC
○ Extrinsic HV/IVC compression (stasis &/or thrombosis)
– Hepatocellular carcinoma, hepatic metastasis, adrenal
tumor, adenopathy
○ Centrilobular HV obstruction
– Obstruction of tiny centrilobular veins (hepatic
venoocclusive disease)
– After bone marrow transplantation, antineoplastic
drug use, radiation therapy
○ Congenital-membranous IVC obstruction
– Etiology unclear: Congenital, injury, infection all
hypothesized
– Tapered or membrane-like IVC obstruction
– May present in adulthood; "congenital" questioned
– Japan, India, Israel, South Africa
Gross Pathologic & Surgical Features
• Acute phase
○ Acute findings due to venous outflow obstruction >
hepatic congestion
○ Chronic findings due to ischemia, necrosis, regeneration
• Chronic phase
○ Liver: Nodular, shrunken, may be cirrhotic
○ Atrophy of affected lobes and hypertrophy of caudate
lobe
Microscopic Features
• Acute: Centrilobular congestion, dilated sinusoids
• Chronic: Fibrosis, necrosis, and cell atrophy
CLINICAL ISSUES
Presentation
• Most common signs/symptoms
○ Classical acute Budd-Chiari presentation
– Rapid onset of abdominal pain, liver tenderness,
hepatic dysfunction
– Possible abdominal distention from ascites,
hypotension
– Acute signs/symptoms are variable: Depend on
rapidity of obstructive process, extent of HV
involvement, severity of obstruction, collateralization
○ Chronic signs/symptoms
– RUQ pain, hepatomegaly, hepatic dysfunction
– Splenomegaly, ascites, varicosities
• Other signs/symptoms
○ Acute or chronic lower extremity edema, if IVC
obstructed
Demographics
• Age
○ Any group, but usual onset in young adults
• Gender
○ Females more than males
Natural History & Prognosis
• Complications
○ Acute: Liver failure, shock, pulmonary embolization from
IVC
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○ Chronic:Regeneration/liver dysfunction/failure: Portal
hypertension/variceal bleeding/cirrhosis
○ Congenital-membranous IVC obstruction:Complicated
by hepatocellular carcinoma in 20-40% of cases in Japan
& South Africa
• Prognosis
○ Depends on degree of obstruction, etiology, extent of
liver damage, or collateralization
○ Neoplastic obstruction: Usually fatal
○ Centrilobular obstruction: Variable prognosis ranging
from complete recovery to fulminant hepatic failure and
death
Treatment
• Medical management
○ Anticoagulation, steroids, nutritional therapy
• Transjugular intrahepatic portosystemic shunt (TIPS)
○ Ameliorates intractable ascites
○ Controls intractable, recurrent gastrointestinal
hemorrhage
• Congenital-membranous IVC occlusion
○ Balloon angioplasty, stent insertion
• Liver transplantation; controversial
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
• Narrowed or obliterated HVs/IVC
• Bicolored HVs due to intrahepatic collateralization on color
Doppler ultrasound
SELECTED REFERENCES
1. Patil P et al: Spectrum of imaging in Budd Chiari syndrome. J Med Imaging
Radiat Oncol. 56(1):75-83, 2012
2. Raszeja-Wyszomirska J et al: Primary Budd-Chiari syndrome - a single center
experience. Hepatogastroenterology. 59(118):1879-82, 2012
3. Jayanthi V et al: Budd-Chiari Syndrome. Changing epidemiology and clinical
presentation. Minerva Gastroenterol Dietol. 56(1):71-80, 2010
4. Cura M et al: Diagnostic and interventional radiology for Budd-Chiari
syndrome. Radiographics. 29(3):669-81, 2009
5. Boozari B et al: Ultrasonography in patients with Budd-Chiari syndrome:
diagnostic signs and prognostic implications. J Hepatol. 49(4):572-80, 2008
6. Karaosmanoglu D et al: CT, MRI, and US findings of incidental segmental
distal hepatic vein occlusion: a new form of Budd-Chiari syndrome? J
Comput Assist Tomogr. 32(4):518-22, 2008
7. Aydinli M et al: Budd-Chiari syndrome: etiology, pathogenesis and diagnosis.
World J Gastroenterol. 13(19):2693-6, 2007
8. Bozorgmanesh A et al: Budd-Chiari syndrome: hepatic venous web outflow
obstruction treated by percutaneous placement of hepatic vein stent. Semin
Intervent Radiol. 24(1):100-5, 2007
9. Brancatelli G et al: Budd-Chiari syndrome: spectrum of imaging findings. AJR
Am J Roentgenol. 188(2):W168-76, 2007
10. Buckley O et al: Imaging of Budd-Chiari syndrome. Eur Radiol. 17(8):2071-8,
2007
11. Erden A: Budd-Chiari syndrome: a review of imaging findings. Eur J Radiol.
61(1):44-56, 2007
12. Bargallo X et al: Sonography of Budd-Chiari syndrome. AJR Am J
Roentgenol. 187(1):W33-41, 2006
13. Chaubal N et al: Sonography in Budd-Chiari syndrome. J Ultrasound Med.
25(3):373-9, 2006
14. Brancatelli G et al: Benign regenerative nodules in Budd-Chiari syndrome
and other vascular disorders of the liver: radiologic-pathologic and clinical
correlation. Radiographics. 22(4):847-62, 2002