Imaging Pearls ❯ Liver ❯ Budd Chiari
|
-- OR -- |
|
- "Chronic Budd-Chiari syndrome is also characterized by the development of multiple regenerative nodules, which can be viewed as a response to a focal loss of portal perfusion and hyperarterialization in areas with pre- served hepatic venous outflow. These nodules are usually multiple and have a typical diameter of 0.5–4.0 cm. On multiphasic helical CT, regenerative nodules are markedly and homogeneously hyperattenuating on arte- rial phase images and remain slightly hyperattenuating on portal venous phase images, allowing differential diagnosis from hepatocellular carcinoma."
Hepatic morphology abnormalities: beyond cirrhosis.
Mamone G, Cortis K, Sarah A, Caruso S, Miraglia R
Abdom Radiol (NY). 2018 Jul;43(7):1612-1626 - “The diagnosis of cirrhosis can be reached on the basis of established hepatic morphological changes. However, some other conditions can mimic cirrhosis. The aim of this pictorial essay is to review the CT and MRI appearances of hepatic morphology abnormalities in the cirrhotic liver and other diseases, describing pathologic conditions that can mimic cirrhosis, with useful tips for the differential diagnosis. Mimickers of cirrhosis include congenital hepatic fibrosis, Caroli disease, Budd-Chiari Syndrome, hepatoportal sclerosis, cavernous transformation of the portal vein, pseudocirrhosis from metastatic disease, acute liver failure, post-therapeutic morphologic changes in the liver, and infective conditions including schistosomiasis and oriental cholangiohepatitis. Recognizing the hepatic morphological changes in images can help radiologists to diagnose cirrhosis and other diseases in early stages.”
Hepatic morphology abnormalities: beyond cirrhosis.
Mamone G, Cortis K, Sarah A, Caruso S, Miraglia R
Abdom Radiol (NY). 2018 Jul;43(7):1612-1626 - “In subacute or chronic Budd-Chiari syn- drome, the morphologic changes in the liver are the result of the type of venous involve- ment, and portosystemic and intrahepatic collateral vessels are often found. Contrast-enhanced CT is useful for depicting regions of hypoperfused liver parenchyma.”
Hepatic morphology abnormalities: beyond cirrhosis.
Mamone G, Cortis K, Sarah A, Caruso S, Miraglia R
Abdom Radiol (NY). 2018 Jul;43(7):1612-1626 - “In acute Budd-Chiari syndrome, the morphologic features of the liver usually are normal, and occlusion of the hepatic veins with severe ascites is the typical finding. The liver exhibits patchy, decreased peripheral enhancement caused by portal and sinusoidal stasis and stronger enhancement of the central portion of the liver parenchyma. The thrombosed hepatic veins are hypoattenuating, and the inferior vena cava is compressed by the enlarged caudate lobe. Ascites and splenomegaly are usually present.”
Budd-Chiari Syndrome: Spectrum of Imaging Findings
Brancatelli G, Vilgrain V, Federle MP et al.
AJR 2007; 188:W168–W176 - “The liver exhibits patchy, decreased peripheral enhancement caused by portal and sinusoidal stasis and stronger enhancement of the central portion of the liver parenchyma. The thrombosed hepatic veins are hypoattenuating, and the inferior vena cava is compressed by the enlarged caudate lobe. Ascites and splenomegaly are usually present.”
Budd-Chiari Syndrome: Spectrum of Imaging Findings
Brancatelli G, Vilgrain V, Federle MP
AJR 2007; 188:W168–W176 - "In subacute or chronic Budd-Chiari syn- drome, the morphologic changes in the liver are the result of the type of venous involve- ment, and portosystemic and intrahepatic col- lateral vessels are often found. Contrast-enhanced CT is useful for depicting regions of hypoperfused liver parenchyma."
Budd-Chiari Syndrome: Spectrum of Imaging Findings
Brancatelli G, Vilgrain V, Federle MP
AJR 2007; 188:W168–W176 - “Common causes of BCS include inherited and acquired hypercoagulable states. Inherited hypercoagulable states such as factor V Leiden mutation, protein C deficiency, protein S deficiency, the prothrombin G20210A mutation, and antithrombin III deficiency are common causes of hepatic vein thrombosis resulting in BCS. Acquired prothrombotic states such as myeloproliferative disorders (e.g., polycythemia vera, paroxysmal nocturnal hemoglobinuria, essential thrombocytosis, agnogenic myeloid metaplasia, and myelofibrosis) account for more than 50% of BCS cases. Other prothrombotic conditions, such as antiphospholipid syndrome, have also been mentioned. Other conditions have been proposed as risk factors for the development of BCS, including Behçet disease, hypereosinophilic syndrome, and ulcerative colitis and pregnancy, malnutrition, and the use of oral contraceptives. “
Budd-Chiari Syndrome
Hector Ferral, George Behrens, Jorge Lopera
AJR 2012; 199:737–745 - Budd Chiari Syndrome: Causes
- Inherited and acquired hypercoagulability states (i.e. factor V Leiden mutation)
- Myeloproliferative disorders (polycythemia vera, PNH, myelofibrosis)
- Pregnancy
- Malnutrition
- Use of oral contraceptives - Budd Chiari Syndrome: Facts
- Clinical presentation of BCS may be fulminant (5%), acute (20%), and subacute or chronic (60%) [13]. BCS may be asymptomatic in approximately 15–20% of cases.
- Approximately 75–80% of patients with BCS will have clinical manifestations; the most common include fever, abdominal pain, abdominal distention, ascites, liver failure, lower extremity edema, gastrointestinal bleeding, and encephalopathy - Budd Chiari Syndrome: Facts
- Fulminant Budd Chiari Syndrome
- Acute Budd-Chiari Syndrome
- Subacute Budd-Chiari Syndrome
- Chronic Budd-Chiari Syndrome - "CT findings in acute BCS may include nor- mal liver morphology, patchy enhancement, an enlarged caudate lobe, a compressed IVC, the absence of hepatic veins, and ascites. The patchy enhancement seen on CT scans of patients with BCS is related to the stasis in the sinusoids and portal vein and is associated with increased en- hancement of the central portion of the liver parenchyma."
Budd-Chiari Syndrome
Hector Ferral, George Behrens, Jorge Lopera
AJR 2012; 199:737–745 - “Chronic BCS is associated with an alteration in the morphology of the liver and regenerative nodule formation. These nodules typically show hyperattenuation in the arterial phase and remain hyperattenuating in the portal phase; patchy enhancement may also be seen in this stage of the disease.”
Budd-Chiari Syndrome
Hector Ferral, George Behrens, Jorge Lopera
AJR 2012; 199:737–745
- Acute Budd Chiari Syndrome: CT Findings
- absence of hepatic vein opacification
- hypertrophied caudate lobe may be found in 75% of the cases
- the liver may have a mottled appearance along with late enhancement of the periphery of the liver and around the hepatic veins - Chronic Budd Chiari Syndrome: CT Findings
- shrunken liver sparing the caudate lobe with intrahepatic collaterals.
- collateralized routes seen in BCS are the left renal hemiazygos pathway, inferior phrenic, pericardiophrenic collaterals, and superficial collaterals of the abdominal wall
- Regenerative nodules common and may be multiple