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Cirrhosis

Changed by Mohammad Taghi Niknejad, 4 Jan 2024
Back to revision history
Disclosures - updated 29 Dec 2023: Nothing to disclose

Updates to Article Attributes

Body was changed:

Cirrhosis (plural: cirrhoses) is the common endpoint of a wide variety of chronic liver disease processes which cause hepatocellular necrosis. Cirrhosis can be diagnosed with ultrasound, CT, and MRI, and these imaging modalities can also be used to evaluate for possible complications of cirrhosis, such as portal hypertension or hepatocellular carcinoma.

Epidemiology

The demographics of cirrhosis reflect the underlying causes. Alcoholism and viral hepatitis from intravenous drug use or in an endemic region are the common causes.

The distribution of underlying aetiology will vary regionally, with viral hepatitis being much higher in the developing world, especially in Asia. A typical distribution of causality in Western nations is aas follows 4:

Clinical presentation

The diagnosis is made either at screening for cirrhosis due to known risk factors, elevated elevated liver enzymes, or discovered incidentally in an examination for non-specific symptoms (e.g. right upper quadrant pain). It may also present due to one of its complications:

Pathology

Focal hepatocellular necrosis caused by a variety of insults (see above) is accompanied by the three characteristics of cirrhosis 3:

  1. fibrosis

  2. nodular regeneration

  3. distortion of hepatic architecture

Although traditionally cirrhosis has been divided into micro-and macronodular cirrhosis, many entities begin as micronodular (<3 mm) 9 and and progress to macronodular (e.g. alcoholic cirrhosis), and thus it is of limited utility as a classification scheme 4.

Severity scoring

Radiographic features

Frequent findings in advanced cirrhosis include hypertrophy of the caudate lobe and lateral segments of the left lobe (segments 2 and 3) with concomitant atrophy of the posterior segments (6 and 7) of the right lobe. These changes are likely related to changes in blood flow between the segments. See article:caudate-right lobe ratio (C/RL).

Imaging is not reliable enough to differentiate between various underlying aetiologies.

The main imaging challenge is distinguishing regenerative nodules, siderotic nodules and dysplastic nodules from:

Ultrasound

Ultrasound is a major screening tool for cirrhosis and its complications. It is also useful to aid for biopsy. Appearances include:

  • surface nodularity: 88% sensitive, 82-95% specific 5

  • overall coarse and heterogeneous echotexture

  • segmental hypertrophy/atrophy (see above)

    • caudate width: right lobe width >0.65 (43-84% sensitive, 100% specific 5)

    • reduction of the transverse diameter (<30 mm) of the medial segment of the left lobe (segment 4) 11

  • signs of portal hypertension

Sonoelastography may may also be useful to assess the amount of liver fibrosis 12. Suggested Suggested values for diagnosis are:

  • >7 kPa: advanced fibrosis

  • 12.5-15 kPa: cirrhosis

Contrast-enhanced ultrasound may may have a role in the diagnosis of cirrhosis, as diminished mean hepatic venous transit time is similar to that of perfusion CT 13.

CT

CT is insensitive in early cirrhosis. More established findings include:

MRI

MRI is also insensitive in early cirrhosis but has a significant role in screening cirrhotic livers for small hepatocellular carcinomas (see LI-RADS). MRI findings include:

  • morphologic changes (same as on CT and ultrasound)

  • regenerative nodules (or cirrhotic nodules)

    • T1

      • variable, usually isointense

      • occasionally mildly hyperintense

      • no early enhancement and washout as most supply is from the portal vein 7,9

    • T2

      • usually isointense 9

      • hypointense if siderotic

  • dysplastic nodules

    • maybe of low or high grade, and thus have a variable appearance

    • low-grade nodules will resemble regenerative nodules

    • high-grade nodules will resemble hepatocellular carcinomas 9

  • small hepatocellular carcinoma (HCC)

    • T1 C+ (Gd)

      • arterial phase enhancement and washout 7

      • late enhancing capsule

      • growth in the interval between studies

    • T2: typically mildly or moderately hyperintense

MR angiography or a balanced steady-state free precession sequence may also be used to assess portal vein patency and portosystemic collaterals.

Treatment and prognosis

Treatment depends on the underlying aetiology and presence of complications. One of the key roles of diagnostic radiology is the detection of hepatocellular carcinoma (six-monthly ultrasound should be done for surveillance as per 2018 AASLD (American Association for the Study of Liver Diseases) guidelines in cirrhotic patients to screen for hepatocellular carcinoma development)15. Interventional radiology can be very helpful for the treatment of portal hypertension and its complications (e.g. TIPS, ascites drainage), as well as chemoembolisation or radiofrequency ablation of hepatocellular carcinoma.

Complications

History and etymology

The word cirrhosis derives from the Ancient Greek word "κιρρόειν", meaning "to turn reddish-yellow" or "tawny". The French physician René Laennec (1781-1826) was first to use the term to describe the macroscopic appearance of fibrotic changes in a liver with alcoholic cirrhosis.

Differential diagnosis

There are severalSeveral conditions that can potentially mimic cirrhosis on imaging 10:

Practical points

  • -<p><strong>Cirrhosis</strong> (plural: cirrhoses) is the common endpoint of a wide variety of chronic liver disease processes which cause hepatocellular necrosis. Cirrhosis can be diagnosed with ultrasound, CT, and MRI, and these imaging modalities can also be used to evaluate for possible complications of cirrhosis, such as <a href="/articles/portal-hypertension">portal hypertension</a> or <a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a>.</p><h4>Epidemiology</h4><p>The demographics of cirrhosis reflect the underlying causes. Alcoholism and viral hepatitis from <a href="/articles/intravenous-drug-user">intravenous drug use</a> or in an endemic region are the common causes.</p><p>The distribution of underlying aetiology will vary regionally, with viral hepatitis being much higher in the developing world, especially Asia. A typical distribution of causality in Western nations is a follows <sup>4</sup>:</p><ul>
  • -<li><p>alcohol: 60-70%</p></li>
  • -<li>
  • -<p><a href="/articles/viral-hepatitis">viral hepatitis</a>: 10%</p>
  • -<ul>
  • -<li><p><a href="/articles/hepatitis-b-virus-1">hepatitis B virus</a> most common in sub-Saharan Africa and Asia <sup>14</sup></p></li>
  • -<li><p><a href="/articles/hepatitis-c-virus">hepatitis C virus</a> most common in the Western countries and Japan <sup>14</sup></p></li>
  • -</ul>
  • -</li>
  • -<li><p>cryptogenic/<a href="/articles/metabolic-dysfunction-associated-steatotic-liver-disease">non-alcoholic steatohepatitis (NASH)</a>: 10-15%</p></li>
  • -<li><p>biliary disease: e.g. <a href="/articles/primary-sclerosing-cholangitis-psc">primary sclerosing cholangitis (PSC)</a>, <a href="/articles/primary-biliary-cirrhosis-pbc">primary biliary cholangitis (PBC)</a>: 5-10%</p></li>
  • -<li><p>metabolic disease: e.g. <a href="/articles/haemochromatosis">hereditary haemochromatosis</a>, <a href="/articles/wilsons-disease">Wilson disease</a>, <a href="/articles/alpha-1-antitrypsin-deficiency-4">alpha-1-antitrypsin deficiency</a>: 5%</p></li>
  • -<li><p><a href="/articles/autoimmune-hepatitis">autoimmune hepatitis</a></p></li>
  • -<li><p>vascular disease: e.g. <a href="/articles/congestive-hepatopathy">congestive hepatopathy</a> (<a href="/articles/congestive-cardiac-failure">right heart failure</a>), <a href="/articles/budd-chiari-syndrome-1">Budd-Chiari syndrome</a>, <a href="/articles/sinusoidal-obstruction-syndrome-1">hepatic veno-occlusive disease</a> (rare)</p></li>
  • -<li><p><a href="/articles/cystic-fibrosis">cystic fibrosis</a>: uncommon but increasing with increased survival</p></li>
  • -<li><p>medications: e.g. methotrexate</p></li>
  • -</ul><h4>Clinical presentation</h4><p>The diagnosis is made either at screening for cirrhosis due to known risk factors, elevated liver enzymes, or discovered incidentally in an examination for non-specific symptoms (e.g. right upper quadrant pain). It may also present due to one of its complications:</p><ul>
  • -<li><p><a href="/articles/acute-liver-failure">liver failure</a></p></li>
  • -<li><p><a href="/articles/ascites">ascites</a></p></li>
  • -<li><p><a href="/articles/portal-hypertension">portal hypertension</a></p></li>
  • -<li><p><a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma (HCC)</a></p></li>
  • -</ul><h4>Pathology</h4><p>Focal hepatocellular necrosis caused by a variety of insults (see above) is accompanied by the three characteristics of cirrhosis <sup>3</sup>:</p><ol>
  • -<li><p>fibrosis</p></li>
  • -<li><p>nodular regeneration</p></li>
  • -<li><p>distortion of hepatic architecture</p></li>
  • -</ol><p>Although traditionally cirrhosis has been divided into micro-and macronodular cirrhosis, many entities begin as micronodular (&lt;3 mm) <sup>9</sup> and progress to macronodular (e.g. alcoholic cirrhosis) and thus it is of limited utility as a classification scheme <sup>4</sup>.</p><h5>Severity scoring</h5><ul>
  • -<li><p>clinical scoring: <a href="/articles/child-pugh-score">Child-Pugh scoring system</a></p></li>
  • -<li><p>biochemical scoring: <a href="/articles/hepascore">hepascore</a></p></li>
  • -<li><p><a href="/articles/metavir-score-1">METAVIR score</a></p></li>
  • -</ul><h4>Radiographic features</h4><p>Frequent findings in advanced cirrhosis include <a href="/articles/hypertrophy-of-the-caudate-lobe">hypertrophy of the caudate lobe</a> and lateral <a href="/articles/couinaud-classification-of-hepatic-segments">segments</a> of the left lobe (segments 2 and 3) with concomitant atrophy of the posterior segments (6 and 7) of the right lobe. These changes are likely related to changes in blood flow between the segments. See article: <a href="/articles/caudate-right-lobe-ratio">caudate-right lobe ratio (C/RL)</a>.</p><p>Imaging is not reliable enough to differentiate between various underlying aetiologies.</p><p>The main imaging challenge is distinguishing <a href="/articles/regenerative-liver-nodule">regenerative nodules</a>, <a href="/articles/hepatic-siderotic-nodules">siderotic nodules</a> and <a href="/articles/dysplastic-liver-nodules-1">dysplastic nodules</a> from:</p><ul>
  • -<li><p>small <a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a>: early arterial enhancement with washout</p></li>
  • -<li><p><a href="/articles/hepatic-metastases-1">hepatic metastases</a>: typically high T2</p></li>
  • -</ul><h5>Ultrasound</h5><p>Ultrasound is a major screening tool for cirrhosis and its complications. It is also useful to aid for biopsy. Appearances include:</p><ul>
  • -<li><p>surface nodularity: 88% sensitive, 82-95% specific <sup>5</sup></p></li>
  • -<li><p>overall coarse and heterogeneous echotexture</p></li>
  • -<li>
  • -<p>segmental hypertrophy/atrophy (see above)</p>
  • -<ul>
  • -<li><p>caudate width: right lobe width &gt;0.65 (43-84% sensitive, 100% specific <sup>5</sup>)</p></li>
  • -<li><p>reduction of the transverse diameter (&lt;30 mm) of the medial segment of the left lobe (segment 4) <sup>11</sup></p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>signs of portal hypertension</p>
  • -<ul>
  • -<li>
  • -<p>Doppler flow changes</p>
  • -<ul>
  • -<li>
  • -<p>portal venous system</p>
  • -<ul>
  • -<li><p>enlarged portal vein: &gt;13 mm (42% sensitive, 95-100% specific <sup>6</sup>)</p></li>
  • -<li><p>slow portal venous flow &lt;15 cm/sec</p></li>
  • -<li><p>reversal or to-and-fro portal venous flow</p></li>
  • -<li><p><a href="/articles/portal-vein-thrombosis">portal venous thrombosis </a>+/- <a href="/articles/cavernous-transformation-of-the-portal-vein">cavernous transformation</a></p></li>
  • -<li>
  • -<p>enlarged <a href="/articles/superior-mesenteric-vein">superior mesenteric vein</a> and <a href="/articles/splenic-vein">splenic vein</a>: &gt;10 mm</p>
  • -<ul><li><p>NB: this should be measured during deep inspiration as size can vary</p></li></ul>
  • -</li>
  • -<li><p>loss of respiratory variation in superior mesenteric vein and splenic vein spectral Doppler waveforms</p></li>
  • -<li><p>enlarged <a href="/articles/paraumbilical-veins" title="Paraumbilical veins">paraumbilical vein</a> with hepatofugal flow <sup>17</sup></p></li>
  • -<li><p><a href="/articles/portosystemic-collateral-pathways-1" title="Portosystemic collaterals">portosystemic collaterals</a></p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>hepatic veins</p>
  • -<ul><li><p>portalisation of hepatic vein waveform</p></li></ul>
  • -</li>
  • -<li>
  • -<p>hepatic arteries</p>
  • -<ul>
  • -<li><p>"corkscrew" appearance</p></li>
  • -<li><p>increased velocity (compensating for decreased portal vein flow)</p></li>
  • -</ul>
  • -</li>
  • -</ul>
  • -</li>
  • -<li><p><a href="/articles/splenomegaly">splenomegaly</a></p></li>
  • -<li><p><a href="/articles/ascites">ascites</a></p></li>
  • -<li><p><a href="/articles/diffuse-hepatic-steatosis">fatty change</a> (variable)</p></li>
  • -</ul>
  • -</li>
  • -</ul><p><a href="/articles/elastography">Sonoelastography</a> may also be useful to assess the amount of <a href="/articles/cirrhosis">liver fibrosis</a> <sup>12</sup>. Suggested values for diagnosis are:</p><ul>
  • -<li><p>&gt;7 kPa: advanced fibrosis</p></li>
  • -<li><p>12.5-15 kPa: cirrhosis</p></li>
  • -</ul><p><a href="/articles/contrast-enhanced-ultrasound-2">Contrast-enhanced ultrasound</a> may have a role in the diagnosis of cirrhosis, as diminished mean hepatic venous transit time is similar to that of perfusion CT <sup>13</sup>.</p><h5>CT</h5><p>CT is insensitive in early cirrhosis. More established findings include:</p><ul>
  • -<li>
  • -<p>surface and parenchymal nodularity</p>
  • -<ul>
  • -<li><p><a href="/articles/regenerative-liver-nodule">regenerative nodules</a> (majority): isodense/hyperdense to the rest of liver</p></li>
  • -<li><p><a href="/articles/hepatic-siderotic-nodules">siderotic nodules</a> (minority): hyperdense due to accumulation of iron <sup>6</sup></p></li>
  • -</ul>
  • -</li>
  • -<li><p><a href="/articles/diffuse-hepatic-steatosis">fatty change</a> (variable)</p></li>
  • -<li><p>parenchymal heterogeneity both on the pre and post intravenous contrast scans</p></li>
  • -<li><p>predominantly portal venous supply to <a href="/articles/dysplastic-liver-nodules-1">dysplastic nodules</a></p></li>
  • -<li><p>in advanced cirrhosis, nodular margin and lobar hypertrophy/atrophy may be demonstrated (see above)</p></li>
  • -<li>
  • -<p>signs of portal hypertension</p>
  • -<ul>
  • -<li><p>portal vein enlargement</p></li>
  • -<li><p>portal venous thrombosis +/- <a href="/articles/cavernous-transformation-of-the-portal-vein">cavernous transformation</a></p></li>
  • -<li><p>splenomegaly</p></li>
  • -<li><p>enlarged <a href="/articles/superior-mesenteric-vein">superior mesenteric vein</a> and <a href="/articles/splenic-vein">splenic vein</a></p></li>
  • -<li><p>enlarged <a href="/articles/paraumbilical-veins" title="Paraumbilical veins">paraumbilical vein</a></p></li>
  • -<li><p>portosystemic collaterals</p></li>
  • -</ul>
  • -</li>
  • -<li><p>upper abdominal <a href="/articles/lymph-node-enlargement">lymphadenopathy</a> in end-stage disease <sup>16</sup></p></li>
  • -</ul><h5>MRI</h5><p>MRI is also insensitive in early cirrhosis but has a significant role in screening cirrhotic livers for small hepatocellular carcinomas (see <a href="/articles/li-rads">LI-RADS</a>). MRI findings include:</p><ul>
  • -<li><p>morphologic changes (same as on CT and ultrasound)</p></li>
  • -<li>
  • -<p><a href="/articles/regenerative-liver-nodule">regenerative nodules</a> (or cirrhotic nodules)</p>
  • -<ul>
  • -<li>
  • -<p><strong>T1</strong></p>
  • -<ul>
  • -<li><p>variable, usually isointense</p></li>
  • -<li><p>occasionally mildly hyperintense</p></li>
  • -<li><p>no early enhancement and washout as most supply is from the portal vein <sup>7,9</sup></p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><strong>T2</strong></p>
  • -<ul>
  • -<li><p>usually isointense <sup>9</sup></p></li>
  • -<li><p>hypointense if <a href="/articles/hepatic-siderotic-nodules">siderotic</a></p></li>
  • -</ul>
  • -</li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/dysplastic-liver-nodules-1">dysplastic nodules</a></p>
  • -<ul>
  • -<li><p>maybe of low or high grade, and thus have a variable appearance</p></li>
  • -<li><p>low-grade nodules will resemble regenerative nodules</p></li>
  • -<li><p>high-grade nodules will resemble hepatocellular carcinomas <sup>9</sup></p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>small <a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma (HCC)</a></p>
  • -<ul>
  • -<li>
  • -<p><strong>T1 C+ (Gd)</strong></p>
  • -<ul>
  • -<li><p>arterial phase enhancement and washout <sup>7</sup></p></li>
  • -<li><p>late enhancing capsule</p></li>
  • -<li><p>growth in the interval between studies</p></li>
  • -</ul>
  • -</li>
  • -<li><p><strong>T2:</strong> typically mildly or moderately hyperintense</p></li>
  • -</ul>
  • -</li>
  • -</ul><p><a href="/articles/mr-angiography-2">MR angiography</a> or a balanced steady-state free precession sequence may also be used to assess portal vein patency and portosystemic collaterals.</p><h4>Treatment and prognosis</h4><p>Treatment depends on the underlying aetiology and presence of complications. One of the key roles of diagnostic radiology is the detection of hepatocellular carcinoma (six-monthly ultrasound should be done for surveillance as per 2018 AASLD (American Association for the Study of Liver Diseases) guidelines in cirrhotic patients to screen for hepatocellular carcinoma development) <sup>15</sup>. Interventional radiology can be very helpful for the treatment of portal hypertension and its complications (e.g. <a href="/articles/tips">TIPS</a>, ascites drainage), as well as <a href="/articles/transcatheter-arterial-chemoembolisation">chemoembolisation</a> or <a href="/articles/radiofrequency-ablation">radiofrequency ablation</a> of hepatocellular carcinoma.</p><h5>Complications</h5><ul>
  • -<li><p><a href="/articles/cirrhosis-pulmonary-manifestations">pulmonary complications of cirrhosis</a></p></li>
  • -<li><p><a href="/articles/cirrhosis-musculoskeletal-manifestations">musculoskeletal complications of cirrhosis</a></p></li>
  • -<li><p><a href="/articles/cirrhosis-cns-manifestations">central nervous system (CNS) complications of cirrhosis</a></p></li>
  • -</ul><h4>History and etymology</h4><p>The word cirrhosis derives from the Ancient Greek word "κιρρόειν", meaning "to turn reddish-yellow" or "tawny". The French physician <strong>René Laennec</strong> (1781-1826) was first to use the term to describe the macroscopic appearance of fibrotic changes in a liver with alcoholic cirrhosis.</p><h4>Differential diagnosis</h4><p>There are several conditions that can potentially mimic cirrhosis on imaging <sup>10</sup>:</p><ul>
  • -<li><p><a href="/articles/pseudocirrhosis">pseudocirrhosis</a></p></li>
  • -<li><p>widespread (miliary type)<a href="/articles/hepatic-metastases-1"> liver metastases</a></p></li>
  • -<li><p><a href="/articles/budd-chiari-syndrome-1">Budd-Chiari syndrome</a> (particularly chronic)</p></li>
  • -<li><p><a href="/articles/acute-liver-failure">fulminant hepatic failure</a></p></li>
  • -<li><p><a href="/articles/sarcoidosis-abdominal-manifestations-1">hepatic sarcoidosis</a></p></li>
  • -<li><p><a href="/articles/congenital-hepatic-fibrosis">congenital hepatic fibrosis</a></p></li>
  • -<li><p><a href="/articles/idiopathic-non-cirrhotic-portal-hypertension">idiopathic portal hypertension</a></p></li>
  • -<li><p>early <a href="/articles/primary-biliary-cholangitis">primary biliary cholangitis (PBC)</a></p></li>
  • -<li><p>chronic <a href="/articles/portal-vein-thrombosis">portal vein thrombosis</a></p></li>
  • -<li><p><a href="/articles/nodular-regenerative-hyperplasia">nodular regenerative hyperplasia</a> of the liver</p></li>
  • -</ul><h4>Practical points</h4><ul><li><p><a href="/articles/hepatopulmonary-syndrome">hepatopulmonary syndrome</a> and <a href="/articles/hepatorenal-syndrome">hepatorenal syndrome</a> may develop in cirrhosis</p></li></ul>
  • +<p><strong>Cirrhosis</strong> (plural: cirrhoses) is the common endpoint of a wide variety of chronic liver disease processes which cause hepatocellular necrosis. Cirrhosis can be diagnosed with ultrasound, CT, and MRI, and these imaging modalities can also be used to evaluate for possible complications of cirrhosis, such as <a href="/articles/portal-hypertension">portal hypertension</a> or <a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a>.</p><h4>Epidemiology</h4><p>The demographics of cirrhosis reflect the underlying causes. Alcoholism and viral hepatitis from <a href="/articles/intravenous-drug-user">intravenous drug use</a> or in an endemic region are the common causes.</p><p>The distribution of underlying aetiology will vary regionally, with viral hepatitis being much higher in the developing world, especially in Asia. A typical distribution of causality in Western nations is as follows <sup>4</sup>:</p><ul>
  • +<li><p>alcohol: 60-70%</p></li>
  • +<li>
  • +<p><a href="/articles/viral-hepatitis">viral hepatitis</a>: 10%</p>
  • +<ul>
  • +<li><p><a href="/articles/hepatitis-b-virus-1">hepatitis B virus</a>&nbsp;most common in sub-Saharan Africa and Asia <sup>14</sup></p></li>
  • +<li><p><a href="/articles/hepatitis-c-virus">hepatitis C virus</a> is most common in Western countries and Japan <sup>14</sup></p></li>
  • +</ul>
  • +</li>
  • +<li><p>cryptogenic/<a href="/articles/metabolic-dysfunction-associated-steatotic-liver-disease">non-alcoholic steatohepatitis (NASH)</a>: 10-15%</p></li>
  • +<li><p>biliary disease:&nbsp;e.g. <a href="/articles/primary-sclerosing-cholangitis-psc">primary sclerosing cholangitis (PSC)</a>,&nbsp;<a href="/articles/primary-biliary-cirrhosis-pbc">primary biliary cholangitis (PBC)</a>: 5-10%</p></li>
  • +<li><p>metabolic disease:&nbsp;e.g.&nbsp;<a href="/articles/haemochromatosis">hereditary haemochromatosis</a>,&nbsp;<a href="/articles/wilsons-disease">Wilson disease</a>,&nbsp;<a href="/articles/alpha-1-antitrypsin-deficiency-4">alpha-1-antitrypsin deficiency</a>: 5%</p></li>
  • +<li><p><a href="/articles/autoimmune-hepatitis">autoimmune hepatitis</a></p></li>
  • +<li><p>vascular disease:&nbsp;e.g.&nbsp;<a href="/articles/congestive-hepatopathy">congestive hepatopathy</a> (<a href="/articles/congestive-cardiac-failure">right heart failure</a>), <a href="/articles/budd-chiari-syndrome-1">Budd-Chiari syndrome</a>,&nbsp;<a href="/articles/sinusoidal-obstruction-syndrome-1">hepatic veno-occlusive disease</a>&nbsp;(rare)</p></li>
  • +<li><p><a href="/articles/cystic-fibrosis">cystic fibrosis</a>: uncommon but increasing with increased survival</p></li>
  • +<li><p>medications:&nbsp;e.g. methotrexate</p></li>
  • +</ul><h4>Clinical presentation</h4><p>The diagnosis is made either at screening for cirrhosis due to known risk factors,&nbsp;elevated liver enzymes, or discovered incidentally in an examination for non-specific symptoms (e.g. right upper quadrant pain). It may also present due to one of its complications:</p><ul>
  • +<li><p><a href="/articles/acute-liver-failure">liver failure</a></p></li>
  • +<li><p><a href="/articles/ascites">ascites</a></p></li>
  • +<li><p><a href="/articles/portal-hypertension">portal hypertension</a></p></li>
  • +<li><p><a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma (HCC)</a></p></li>
  • +</ul><h4>Pathology</h4><p>Focal hepatocellular necrosis caused by a variety of insults (see above) is accompanied by the three characteristics of cirrhosis <sup>3</sup>:</p><ol>
  • +<li><p>fibrosis</p></li>
  • +<li><p>nodular regeneration</p></li>
  • +<li><p>distortion of hepatic architecture</p></li>
  • +</ol><p>Although traditionally cirrhosis has been divided into micro-and macronodular cirrhosis, many entities begin as micronodular (&lt;3 mm) <sup>9</sup>&nbsp;and progress to macronodular (e.g. alcoholic cirrhosis), and thus it is of limited utility as a classification scheme <sup>4</sup>.</p><h5>Severity scoring</h5><ul>
  • +<li><p>clinical scoring: <a href="/articles/child-pugh-score">Child-Pugh scoring system</a></p></li>
  • +<li><p>biochemical scoring:&nbsp;<a href="/articles/hepascore">hepascore</a></p></li>
  • +<li><p><a href="/articles/metavir-score-1">METAVIR score</a></p></li>
  • +</ul><h4>Radiographic features</h4><p>Frequent findings in advanced cirrhosis include <a href="/articles/hypertrophy-of-the-caudate-lobe">hypertrophy of the caudate lobe</a> and lateral <a href="/articles/couinaud-classification-of-hepatic-segments">segments</a> of the left lobe (segments 2 and 3) with concomitant atrophy of the posterior segments (6 and 7) of the right lobe. These changes are likely related to changes in blood flow between the segments. See article:&nbsp;<a href="/articles/caudate-right-lobe-ratio">caudate-right lobe ratio (C/RL)</a>.</p><p>Imaging is not reliable enough to differentiate between various underlying aetiologies.</p><p>The main imaging challenge is distinguishing <a href="/articles/regenerative-liver-nodule">regenerative nodules</a>, <a href="/articles/hepatic-siderotic-nodules">siderotic nodules</a> and <a href="/articles/dysplastic-liver-nodules-1">dysplastic nodules</a> from:</p><ul>
  • +<li><p>small <a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a>: early arterial enhancement with washout</p></li>
  • +<li><p><a href="/articles/hepatic-metastases-1">hepatic metastases</a>: typically high T2</p></li>
  • +</ul><h5>Ultrasound</h5><p>Ultrasound is a major screening tool for cirrhosis and its complications. It is also useful to aid for biopsy. Appearances include:</p><ul>
  • +<li><p>surface nodularity: 88% sensitive, 82-95% specific <sup>5</sup></p></li>
  • +<li><p>overall coarse and heterogeneous echotexture</p></li>
  • +<li>
  • +<p>segmental hypertrophy/atrophy (see above)</p>
  • +<ul>
  • +<li><p>caudate width: right lobe width &gt;0.65 (43-84% sensitive, 100% specific <sup>5</sup>)</p></li>
  • +<li><p>reduction of the transverse diameter (&lt;30 mm) of the medial segment of the left lobe (segment 4) <sup>11</sup></p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>signs of portal hypertension</p>
  • +<ul>
  • +<li>
  • +<p>Doppler flow changes</p>
  • +<ul>
  • +<li>
  • +<p>portal venous system</p>
  • +<ul>
  • +<li><p>enlarged portal vein: &gt;13 mm (42% sensitive, 95-100% specific <sup>6</sup>)</p></li>
  • +<li><p>slow portal venous flow &lt;15 cm/sec</p></li>
  • +<li><p>reversal or to-and-fro portal venous flow</p></li>
  • +<li><p><a href="/articles/portal-vein-thrombosis">portal venous thrombosis </a>+/- <a href="/articles/cavernous-transformation-of-the-portal-vein">cavernous transformation</a></p></li>
  • +<li>
  • +<p>enlarged <a href="/articles/superior-mesenteric-vein">superior mesenteric vein</a> and <a href="/articles/splenic-vein">splenic vein</a>: &gt;10 mm</p>
  • +<ul><li><p>NB: this should be measured during deep inspiration as size can vary</p></li></ul>
  • +</li>
  • +<li><p>loss of respiratory variation in superior mesenteric vein and splenic vein spectral Doppler waveforms</p></li>
  • +<li><p>enlarged <a href="/articles/paraumbilical-veins" title="Paraumbilical veins">paraumbilical vein</a> with hepatofugal flow <sup>17</sup></p></li>
  • +<li><p><a href="/articles/portosystemic-collateral-pathways-1" title="Portosystemic collaterals">portosystemic collaterals</a></p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>hepatic veins</p>
  • +<ul><li><p>portalisation of hepatic vein waveform</p></li></ul>
  • +</li>
  • +<li>
  • +<p>hepatic arteries</p>
  • +<ul>
  • +<li><p>"corkscrew" appearance</p></li>
  • +<li><p>increased velocity (compensating for decreased portal vein flow)</p></li>
  • +</ul>
  • +</li>
  • +</ul>
  • +</li>
  • +<li><p><a href="/articles/splenomegaly">splenomegaly</a></p></li>
  • +<li><p><a href="/articles/ascites">ascites</a></p></li>
  • +<li><p><a href="/articles/diffuse-hepatic-steatosis">fatty change</a>&nbsp;(variable)</p></li>
  • +</ul>
  • +</li>
  • +</ul><p><a href="/articles/elastography">Sonoelastography</a>&nbsp;may also be useful to assess the amount of <a href="/articles/cirrhosis">liver fibrosis</a> <sup>12</sup>.&nbsp;Suggested values for diagnosis are:</p><ul>
  • +<li><p>&gt;7 kPa: advanced fibrosis</p></li>
  • +<li><p>12.5-15 kPa: cirrhosis</p></li>
  • +</ul><p><a href="/articles/contrast-enhanced-ultrasound-2">Contrast-enhanced ultrasound</a>&nbsp;may have a role in the diagnosis of cirrhosis, as diminished mean hepatic venous transit time is similar to that of perfusion CT <sup>13</sup>.</p><h5>CT</h5><p>CT is insensitive in early cirrhosis. More established findings include:</p><ul>
  • +<li>
  • +<p>surface and parenchymal nodularity</p>
  • +<ul>
  • +<li><p><a href="/articles/regenerative-liver-nodule">regenerative nodules</a> (majority): isodense/hyperdense to the rest of liver</p></li>
  • +<li><p><a href="/articles/hepatic-siderotic-nodules">siderotic nodules</a> (minority): hyperdense due to accumulation of iron <sup>6</sup></p></li>
  • +</ul>
  • +</li>
  • +<li><p><a href="/articles/diffuse-hepatic-steatosis">fatty change</a> (variable)</p></li>
  • +<li><p>parenchymal heterogeneity both on the pre and post intravenous contrast scans</p></li>
  • +<li><p>predominantly portal venous supply to <a href="/articles/dysplastic-liver-nodules-1">dysplastic nodules</a></p></li>
  • +<li><p>in advanced cirrhosis, nodular margin and lobar hypertrophy/atrophy may be demonstrated (see above)</p></li>
  • +<li>
  • +<p>signs of portal hypertension</p>
  • +<ul>
  • +<li><p>portal vein enlargement</p></li>
  • +<li><p>portal venous thrombosis +/- <a href="/articles/cavernous-transformation-of-the-portal-vein">cavernous transformation</a></p></li>
  • +<li><p>splenomegaly</p></li>
  • +<li><p>enlarged <a href="/articles/superior-mesenteric-vein">superior mesenteric vein</a> and <a href="/articles/splenic-vein">splenic vein</a></p></li>
  • +<li><p>enlarged <a href="/articles/paraumbilical-veins" title="Paraumbilical veins">paraumbilical vein</a></p></li>
  • +<li><p>portosystemic collaterals</p></li>
  • +</ul>
  • +</li>
  • +<li><p>upper abdominal <a href="/articles/lymph-node-enlargement">lymphadenopathy</a> in end-stage disease <sup>16</sup></p></li>
  • +</ul><h5>MRI</h5><p>MRI is also insensitive in early cirrhosis but has a significant role in screening cirrhotic livers for small hepatocellular carcinomas (see <a href="/articles/li-rads">LI-RADS</a>). MRI findings include:</p><ul>
  • +<li><p>morphologic changes (same as on CT and ultrasound)</p></li>
  • +<li>
  • +<p><a href="/articles/regenerative-liver-nodule">regenerative nodules</a> (or cirrhotic nodules)</p>
  • +<ul>
  • +<li>
  • +<p><strong>T1</strong></p>
  • +<ul>
  • +<li><p>variable, usually isointense</p></li>
  • +<li><p>occasionally mildly hyperintense</p></li>
  • +<li><p>no early enhancement and washout as most supply is from the portal vein <sup>7,9</sup></p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p><strong>T2</strong></p>
  • +<ul>
  • +<li><p>usually isointense <sup>9</sup></p></li>
  • +<li><p>hypointense if <a href="/articles/hepatic-siderotic-nodules">siderotic</a></p></li>
  • +</ul>
  • +</li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p><a href="/articles/dysplastic-liver-nodules-1">dysplastic nodules</a></p>
  • +<ul>
  • +<li><p>maybe of low or high grade, and thus have a variable appearance</p></li>
  • +<li><p>low-grade nodules will resemble regenerative nodules</p></li>
  • +<li><p>high-grade nodules will resemble hepatocellular carcinomas <sup>9</sup></p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>small <a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma (HCC)</a></p>
  • +<ul>
  • +<li>
  • +<p><strong>T1 C+ (Gd)</strong></p>
  • +<ul>
  • +<li><p>arterial phase enhancement and washout <sup>7</sup></p></li>
  • +<li><p>late enhancing capsule</p></li>
  • +<li><p>growth in the interval between studies</p></li>
  • +</ul>
  • +</li>
  • +<li><p><strong>T2:</strong> typically mildly or moderately hyperintense</p></li>
  • +</ul>
  • +</li>
  • +</ul><p><a href="/articles/mr-angiography-2">MR angiography</a> or a balanced steady-state free precession sequence may also be used to assess portal vein patency and portosystemic collaterals.</p><h4>Treatment and prognosis</h4><p>Treatment depends on the underlying aetiology and presence of complications. One of the key roles of diagnostic radiology is the detection of hepatocellular carcinoma (six-monthly ultrasound should be done for surveillance as per 2018 AASLD (American Association for the Study of Liver Diseases) guidelines in cirrhotic patients to screen for hepatocellular carcinoma development)&nbsp;<sup>15</sup>. Interventional radiology can be very helpful for the treatment of portal hypertension and its complications (e.g. <a href="/articles/tips">TIPS</a>, ascites drainage), as well as <a href="/articles/transcatheter-arterial-chemoembolisation">chemoembolisation</a> or <a href="/articles/radiofrequency-ablation">radiofrequency ablation</a> of hepatocellular carcinoma.</p><h5>Complications</h5><ul>
  • +<li><p><a href="/articles/cirrhosis-pulmonary-manifestations">pulmonary complications of cirrhosis</a></p></li>
  • +<li><p><a href="/articles/cirrhosis-musculoskeletal-manifestations">musculoskeletal complications of cirrhosis</a></p></li>
  • +<li><p><a href="/articles/cirrhosis-cns-manifestations">central nervous system (CNS) complications of cirrhosis</a></p></li>
  • +</ul><h4>History and etymology</h4><p>The word cirrhosis derives from the Ancient Greek word "κιρρόειν", meaning "to turn reddish-yellow" or "tawny". The French physician <strong>René Laennec</strong> (1781-1826) was first to use the term to describe the macroscopic appearance of fibrotic changes in a liver with alcoholic cirrhosis.</p><h4>Differential diagnosis</h4><p>Several conditions can potentially mimic cirrhosis on imaging <sup>10</sup>:</p><ul>
  • +<li><p><a href="/articles/pseudocirrhosis">pseudocirrhosis</a></p></li>
  • +<li><p>widespread (miliary type)<a href="/articles/hepatic-metastases-1"> liver metastases</a></p></li>
  • +<li><p><a href="/articles/budd-chiari-syndrome-1">Budd-Chiari syndrome</a>&nbsp;(particularly chronic)</p></li>
  • +<li><p><a href="/articles/acute-liver-failure">fulminant hepatic failure</a></p></li>
  • +<li><p><a href="/articles/sarcoidosis-abdominal-manifestations-1">hepatic sarcoidosis</a></p></li>
  • +<li><p><a href="/articles/congenital-hepatic-fibrosis">congenital hepatic fibrosis</a></p></li>
  • +<li><p><a href="/articles/idiopathic-non-cirrhotic-portal-hypertension">idiopathic portal hypertension</a></p></li>
  • +<li><p>early <a href="/articles/primary-biliary-cholangitis">primary biliary cholangitis (PBC)</a></p></li>
  • +<li><p>chronic <a href="/articles/portal-vein-thrombosis">portal vein thrombosis</a></p></li>
  • +<li><p><a href="/articles/nodular-regenerative-hyperplasia">nodular regenerative hyperplasia</a> of the liver</p></li>
  • +</ul><h4>Practical points</h4><ul><li><p><a href="/articles/hepatopulmonary-syndrome">hepatopulmonary syndrome</a>&nbsp;and <a href="/articles/hepatorenal-syndrome">hepatorenal syndrome</a> may develop in cirrhosis</p></li></ul>
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