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title: "Dysmorphic Liver With Abnormal Bile Ducts"
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docid: "1cb8d652-5e83-4ee2-a762-bab56e2d107b"
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authors:
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- key: "6c5a9e0e-9dea-461b-9ad4-c00f5c4c2bbf"
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value: "Atif Zaheer, MD, FSAR"
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- key: "e987d3d3-1206-48d6-824b-3347c2968855"
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value: "Michael P. Federle, MD, FACR"
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breadcrumbs:
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-
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name: "Gastrointestinal"
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slug: "gastrointestinal"
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treeNodeId: "992c2a4d-e0c4-4b82-be00-a05f5f19e3be"
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-
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name: "Differential Diagnosis"
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slug: "differential-diagnosis"
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treeNodeId: "b2773887-e8a2-40b6-86b5-3bd2e3ba7c30"
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-
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name: "Liver"
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slug: "liver"
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treeNodeId: "c1682329-7d88-44f2-bd0f-bccf589e5631"
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-
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name: "Generic Imaging Patterns"
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slug: "generic-imaging-patterns"
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treeNodeId: "4f8b9a53-d6d2-4981-ab07-978042118376"
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-
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name: "Dysmorphic Liver With Abnormal Bile Ducts"
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slug: "dysmorphic-liver-with-abnormal-bil-"
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treeNodeId: null
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category: "Gastrointestinal"
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documentVersionId: "89e10f55-f932-426e-872d-f53c6c08001b"
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imageCount: 24
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lastUpdated: "07/14/22"
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pageDescription: "Dysmorphic Liver With Abnormal Bile Ducts"
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pageKeywords: "Gastrointestinal, Differential Diagnosis, Liver, Generic Imaging Patterns, Dysmorphic Liver With Abnormal Bile Ducts"
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pageTitle: "Dysmorphic Liver With Abnormal Bile Ducts | STATdx"
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enhancedTitle: "Dysmorphic Liver With Abnormal Bile Ducts"
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type: "DDX"
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breadcrumbs:
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- "Gastrointestinal"
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- "Differential Diagnosis"
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- "Liver"
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- "Generic Imaging Patterns"
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- "Dysmorphic Liver With Abnormal Bile Ducts"
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---
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## ESSENTIAL INFORMATION
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- ### Key Differential Diagnosis Issues
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- Dysmorphic liver refers to distortion and scarring of parenchyma
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- May result from inflammation, infection, ischemia, or tumor
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- Or effects of treatment for these conditions
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- Distinguish among dilated ducts, periportal edema, and thrombosed portal or hepatic veins
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- Appearance will vary by modality
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- US: Dilated hepatic artery in cirrhosis may simulate dilated ducts
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- Color Doppler can resolve this issue
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- CT or MR: Periportal edema may simulate dilated ducts
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- Edema usually found surrounding vessels; bile duct lies on only one side
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- CECT: Unopacified veins may simulate ducts
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- Vessels > water attenuation
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- Cholangiography: Intrahepatic ducts may be distorted by masses or regenerating nodules, simulating cholangitis
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- ### Helpful Clues for Common Diagnoses
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- **Primary Sclerosing Cholangitis**
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- Often results in chronic liver damage
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- In patients with primary sclerosing cholangitis (PSC)-induced end-stage cirrhosis, liver is markedly deformed (to much greater extent than with other common causes of cirrhosis)
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- Lobular contour of liver with preferential scarring of periphery
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- Sparing and hypertrophy of caudate and deep right lobe
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- Sometimes to degree simulating central neoplastic mass (pseudotumoral enlargement of caudate)
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- Intrahepatic bile ducts show varying degrees of stricture and dilation with abnormal arborization (branching pattern)
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- Multifocal "beaded" strictures of intra- and extrahepatic ducts with intervening sites of dilated and normal ducts
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- Pruned appearance of biliary tree develops as disease progresses with obliteration of small peripheral ducts
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- Visualization of greater than expected number of peripheral ducts on MRCP is clue to presence of peripheral intrahepatic ductal strictures
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- **Cirrhosis (Mimic)**
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- Rarely causes ductal dilation
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- But may cause pathologic processes that simulate dilated ducts
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- Regenerating nodules may compress and distort intrahepatic ducts
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- **Peribiliary cysts** are dilated peribiliary glands; may simulate dilated ducts ± small cystic masses in portal triads
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- Periportal edema may simulate ducts
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- **Portal Vein Thrombophlebitis (Mimic)**
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- Thrombosed portal vein branches may simulate dilated ducts on CECT or MR
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- Primary thrombosis of portal vein (hypercoagulable states)
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- Produces characteristic distortion of liver that simulates cirrhosis
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- May result from subacute diverticulitis or appendicitis
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- Thrombosed portal veins may simulate dilated ducts
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- Liver may be damaged from infection or ischemia
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- Pyogenic abscesses, liver infarction, volume loss, etc.
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- **Cholangiocarcinoma,****Intrahepatic or Hilar**
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- Arising from confluence (Klatskin) or branch ducts (intrahepatic)
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- Ducts are dilated upstream from tumor
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- Liver parenchyma shows volume loss ± visualization of tumor
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- ### Helpful Clues for Less Common Diagnoses
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- **Budd-Chiari Syndrome**
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- Peripheral biliary ducts may get distorted and dilated due to central hepatic hypertrophy
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- Thrombosed hepatic veins may simulate dilated ducts but are more central
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- Liver is distorted with peripheral > central volume loss, scarring, hepatocellular necrosis and steatosis
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- Flip-flop enhancement: Early enhancement of central liver around IVC, decreased peripheral enhancement. Later decreased enhancement centrally with increased enhancement peripherally
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- **Primary Biliary Cholangitis**
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- Irregular and "pruned" ducts leading to vanishing bile duct syndrome as disease progresses
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- Lace-like fibrosis, prominent lymphadenopathy, and hepatomegaly (early) along with positive antimitochondrial antibody test
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- **Chemotherapy Cholangitis**
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- Intraarterial chemotherapy used for primary hepatocellular carcinoma or metastases
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- Liver distortion due to tumors and parenchymal scarring
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- Ducts are damaged and strictured with appearance like primary sclerosing cholangitis
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- **Ascending Cholangitis**
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- May lead to duct strictures; rarely to chronic liver injury
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- More common are hepatic abscesses
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- **AIDS Cholangiopathy**
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- Liver may be distorted by infection &/or tumor
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- Papillary stenosis with proximal common bile duct (CBD) dilation, strictures/ulcerations of CBD, and intrahepatic strictures: Unique to AIDS cholangiopathy
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- **Fibropolycystic Liver Diseases**
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- Encompasses spectrum of related lesions of liver and biliary tract caused by abnormal embryologic development of ductal plates
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- Lesions may occur in isolation or in any combination; may be clinically silent
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- Or, may cause cholangitis, portal hypertension, GI bleeding, infection, etc.
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- Liver may be distorted by congenital fibrosis (simulates cirrhosis) or by scarring (especially with Caroli disease)
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- Bile ducts may be primary site of pathology (e.g., Caroli disease), coexist with primary parenchymal disease (e.g., fibrosis), or be distorted by extrinsic mass effect
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- **Congenital hepatic fibrosis**
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- Dysmorphic liver, portal hypertension
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- Enlarged and possibly supernumerary hepatic arteries
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- **Caroli disease**
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- Dilatation (usually saccular) of large intrahepatic ducts with alternating biliary strictures
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- Liver often progressively damaged by cholangitis, obstruction
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- **Choledochal cyst**
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- Fusiform or cystic dilation of intrahepatic ± extrahepatic bile duct
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- **Biliary hamartomas**
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- Multiple "cysts" of nearly uniform size, up to 15 mm; no biliary connection
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- Mimic cysts on CT and MR but are often echogenic on US
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- Liver and bile ducts are normal unless coexisting disease, such as Caroli or congenital fibrosis
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- **Autosomal dominant polycystic disease, liver**
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- Distorts liver with innumerable cysts
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- Bile ducts intrinsically normal but may have extrinsic compression by cysts
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- **Recurrent Pyogenic Cholangitis**
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- Usually in Asian, poorly nourished populations
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- Liver distorted by chronic infection and biliary obstruction (abscesses, cirrhosis)
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- Bile ducts enormously dilated by pus and stones
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- **Hepatic Hydatid Disease**
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- Cysts may distort liver
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- Cysts may communicate with ducts, leading to cholangitis
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## Images
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### Selected Images
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**Primary Sclerosing Cholangitis**
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*Axial CECT shows a small liver with deep scars and lobular contour, typical of cirrhosis due to primary sclerosing cholangitis (PSC). Note the irregular dilation of intrahepatic ducts <img src='img/arrows/WS.png' alt='white solid arrow'/> and esophageal varices <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
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**Primary Sclerosing Cholangitis**
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*Axial CECT shows a small liver with deep scars and lobular contour, typical of cirrhosis due to primary sclerosing cholangitis (PSC). Note the irregular dilation of intrahepatic ducts <img src='img/arrows/WS.png' alt='white solid arrow'/> and esophageal varices <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
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**Primary Sclerosing Cholangitis**
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*ERCP shows segmental strictures <img src='img/arrows/WS.png' alt='white solid arrow'/> and "diverticula" <img src='img/arrows/WO.png' alt='white open arrow'/> involving the intra- and extrahepatic bile ducts, resulting in a beaded appearance of the ducts.*
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**Primary Sclerosing Cholangitis**
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*Coronal MRCP MIP in a 37-year-old man with PSC shows extensive intrahepatic strictures with alternating sites of narrowing and ductal dilatation.*
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**Hepatitis**
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*Axial CECT in a 47-year-old woman with autoimmune hepatitis shows evidence of periportal edema <img src='img/arrows/WC.png' alt='white curved arrow'/> that might be mistaken for dilated bile ducts, except that the lucent band extends completely around the portal vein branches.*
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**Peribiliary Cysts**
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*Axial CECT in a patient with advanced cirrhosis and ascites shows saccular and spherical cystic lesions <img src='img/arrows/WS.png' alt='white solid arrow'/> (peribiliary cysts) paralleling the portal triads.*
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**Peribiliary Cysts**
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*Axial CECT shows cirrhotic morphology of the liver with reduced size, wide fissures, and ascites noted. The portal vein branches <img src='img/arrows/BC.png' alt='black curved arrow'/> are surrounded by a collar of low density, some of which probably represents periportal edema; however, there are also discrete, low-density focal lesions <img src='img/arrows/WS.png' alt='white solid arrow'/> that represent periportal cysts within the bile duct walls.*
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**Portal Vein Thrombophlebitis (Mimic)**
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*Axial arterial-phase CECT shows hyperperfusion of the anterior right lobe of the liver <img src='img/arrows/BS.png' alt='black solid arrow'/> due to thrombosis of the anterior branch of the right portal vein. The thrombosed intrahepatic branches <img src='img/arrows/WS.png' alt='white solid arrow'/> might be mistaken for dilated bile ducts.*
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**Portal Vein Thrombophlebitis (Mimic)**
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*Axial CECT in a patient with transient hepatic attenuation difference (THAD) <img src='img/arrows/BS.png' alt='black solid arrow'/> due to thrombosis of the anterior right portal vein shows the increased size of the right hepatic artery <img src='img/arrows/BC.png' alt='black curved arrow'/> that is compensating for the decreased flow through the portal vein.*
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**Cholangiocarcinoma, Intrahepatic or Hilar**
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*Axial CECT shows a heterogeneous left lobe mass <img src='img/arrows/WO.png' alt='white open arrow'/> that obstructs intrahepatic ducts <img src='img/arrows/BC.png' alt='black curved arrow'/> and causes volume loss of the left lobe. This was a multifocal cholangiocarcinoma.*
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**Budd-Chiari Syndrome**
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*Axial CECT shows thrombosed hepatic veins <img src='img/arrows/WS.png' alt='white solid arrow'/> simulating dilated ducts. Ascites, peripheral hepatic damage, and central hypertrophy are typical findings of Budd-Chiari.*
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**Budd-Chiari Syndrome**
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*Dilatation of the biliary tree in the periphery of the liver <img src='img/arrows/WS.png' alt='white solid arrow'/> is due to caudate lobe hypertrophy <img src='img/arrows/WO.png' alt='white open arrow'/> and resulting narrowing of the biliary tree in the central liver as well as peripheral volume loss.*
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**Primary Biliary Cholangitis**
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*Coronal MRCP in a 58-year-old woman with primary biliary cholangitis shows signs of advanced disease with decreased visualization of the peripheral intrahepatic ducts (vanishing bile duct syndrome).*
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**Fibropolycystic Liver Diseases**
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*Axial CECT shows a dysmorphic liver with irregular dilation of intrahepatic ducts <img src='img/arrows/WC.png' alt='white curved arrow'/>. On lower sections, hepatic arteries were enlarged, and small, cystic kidneys were noted. All findings were related to fibropolycystic disease of the liver and kidneys, including congenital hepatic fibrosis.*
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**Caroli Disease**
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*Axial T2 MR shows cystic and irregular cylindrical dilation of intrahepatic bile ducts, characteristic of Caroli disease. Progressive liver failure resulted in transplantation.*
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**Recurrent Pyogenic Cholangitis**
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*Axial CECT shows dilated ducts <img src='img/arrows/WS.png' alt='white solid arrow'/> with a large pigment calculus <img src='img/arrows/CS.png' alt='cyan solid arrow'/> and a small liver abscess <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
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**Hepatic Hydatid Disease**
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*Coronal CECT shows 2 large, cystic masses <img src='img/arrows/WS.png' alt='white solid arrow'/> with daughter cysts and a densely calcified cyst <img src='img/arrows/BS.png' alt='black solid arrow'/>. Bile ducts <img src='img/arrows/BC.png' alt='black curved arrow'/> are dilated due to communication of one of the cysts with the ducts.*
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### Additional Images
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**Cirrhosis (Mimic)**
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*Axial CECT shows a cirrhotic liver with ascites and splenomegaly. Intrahepatic ducts are irregularly dilated <img src='img/arrows/BS.png' alt='black solid arrow'/> in this case of mixed PSC and autoimmune hepatitis.*
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**Chemotherapy Cholangitis**
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*Axial CECT shows surgical absence of the lateral segment (resected metastasis) and a dilated bile duct <img src='img/arrows/WO.png' alt='white open arrow'/> following intraarterial chemotherapy.*
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**Chemotherapy Cholangitis**
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*Transhepatic cholangiogram shows irregular strictures <img src='img/arrows/WS.png' alt='white solid arrow'/> of the common hepatic and intrahepatic ducts as a result of intraarterial <img src='img/arrows/WC.png' alt='white curved arrow'/> chemotherapy for liver metastases.*
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**Caroli Disease**
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*Axial CECT in a patient with Caroli disease and congenital hepatic fibrosis shows dysmorphic liver and classic cystic dilation of intrahepatic ducts with central dot sign.*
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**Cholangiocarcinoma, Intrahepatic or Hilar**
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*Axial T2 MR shows gross dilation of the intrahepatic bile ducts <img src='img/arrows/WS.png' alt='white solid arrow'/>. There is no large mass <img src='img/arrows/WC.png' alt='white curved arrow'/> evident at the site of obstruction, typical of a hilar (Klatskin) tumor.*
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**Peribiliary Cysts**
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*Transverse US shows sonolucent saccular and spherical cystic lesions paralleling the portal triads, representing peribiliary cysts in this patient with cirrhosis and ascites.*
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**Hepatitis**
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*Axial CECT shows a mottled, distorted liver due to acute and chronic hepatitis. Periportal edema <img src='img/arrows/WS.png' alt='white solid arrow'/> might be mistaken for dilated ducts.*
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**Chemotherapy Cholangitis**
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*Axial CECT shows necrotic metastases <img src='img/arrows/BS.png' alt='black solid arrow'/> (from colon cancer) and mild dilation of the bile ducts <img src='img/arrows/BC.png' alt='black curved arrow'/>, a result of intraarterial chemotherapy.*
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