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---
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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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value: "Atif Zaheer, MD, FSAR"
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name: "Dysmorphic Liver With Abnormal Bile Ducts"
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category: "Gastrointestinal"
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|
||||
pageDescription: "Dysmorphic Liver With Abnormal Bile Ducts"
|
||||
pageKeywords: "Gastrointestinal, Differential Diagnosis, Liver, Generic Imaging Patterns, Dysmorphic Liver With Abnormal Bile Ducts"
|
||||
pageTitle: "Dysmorphic Liver With Abnormal Bile Ducts | STATdx"
|
||||
enhancedTitle: "Dysmorphic Liver With Abnormal Bile Ducts"
|
||||
type: "DDX"
|
||||
breadcrumbs:
|
||||
- "Gastrointestinal"
|
||||
- "Differential Diagnosis"
|
||||
- "Liver"
|
||||
- "Generic Imaging Patterns"
|
||||
- "Dysmorphic Liver With Abnormal Bile Ducts"
|
||||
---
|
||||
## ESSENTIAL INFORMATION
|
||||
|
||||
- ### Key Differential Diagnosis Issues
|
||||
|
||||
|
||||
- Dysmorphic liver refers to distortion and scarring of parenchyma
|
||||
- May result from inflammation, infection, ischemia, or tumor
|
||||
- Or effects of treatment for these conditions
|
||||
- Distinguish among dilated ducts, periportal edema, and thrombosed portal or hepatic veins
|
||||
- Appearance will vary by modality
|
||||
- US: Dilated hepatic artery in cirrhosis may simulate dilated ducts
|
||||
- Color Doppler can resolve this issue
|
||||
- CT or MR: Periportal edema may simulate dilated ducts
|
||||
- Edema usually found surrounding vessels; bile duct lies on only one side
|
||||
- CECT: Unopacified veins may simulate ducts
|
||||
- Vessels > water attenuation
|
||||
- Cholangiography: Intrahepatic ducts may be distorted by masses or regenerating nodules, simulating cholangitis
|
||||
- ### Helpful Clues for Common Diagnoses
|
||||
|
||||
|
||||
- **Primary Sclerosing Cholangitis**
|
||||
- Often results in chronic liver damage
|
||||
- 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)
|
||||
- Lobular contour of liver with preferential scarring of periphery
|
||||
- Sparing and hypertrophy of caudate and deep right lobe
|
||||
- Sometimes to degree simulating central neoplastic mass (pseudotumoral enlargement of caudate)
|
||||
- Intrahepatic bile ducts show varying degrees of stricture and dilation with abnormal arborization (branching pattern)
|
||||
- Multifocal "beaded" strictures of intra- and extrahepatic ducts with intervening sites of dilated and normal ducts
|
||||
- Pruned appearance of biliary tree develops as disease progresses with obliteration of small peripheral ducts
|
||||
- Visualization of greater than expected number of peripheral ducts on MRCP is clue to presence of peripheral intrahepatic ductal strictures
|
||||
- **Cirrhosis (Mimic)**
|
||||
- Rarely causes ductal dilation
|
||||
- But may cause pathologic processes that simulate dilated ducts
|
||||
- Regenerating nodules may compress and distort intrahepatic ducts
|
||||
- **Peribiliary cysts** are dilated peribiliary glands; may simulate dilated ducts ± small cystic masses in portal triads
|
||||
- Periportal edema may simulate ducts
|
||||
- **Portal Vein Thrombophlebitis (Mimic)**
|
||||
- Thrombosed portal vein branches may simulate dilated ducts on CECT or MR
|
||||
- Primary thrombosis of portal vein (hypercoagulable states)
|
||||
- Produces characteristic distortion of liver that simulates cirrhosis
|
||||
- May result from subacute diverticulitis or appendicitis
|
||||
- Thrombosed portal veins may simulate dilated ducts
|
||||
- Liver may be damaged from infection or ischemia
|
||||
- Pyogenic abscesses, liver infarction, volume loss, etc.
|
||||
- **Cholangiocarcinoma,****Intrahepatic or Hilar**
|
||||
- Arising from confluence (Klatskin) or branch ducts (intrahepatic)
|
||||
- Ducts are dilated upstream from tumor
|
||||
- Liver parenchyma shows volume loss ± visualization of tumor
|
||||
- ### Helpful Clues for Less Common Diagnoses
|
||||
|
||||
|
||||
- **Budd-Chiari Syndrome**
|
||||
- Peripheral biliary ducts may get distorted and dilated due to central hepatic hypertrophy
|
||||
- Thrombosed hepatic veins may simulate dilated ducts but are more central
|
||||
- Liver is distorted with peripheral > central volume loss, scarring, hepatocellular necrosis and steatosis
|
||||
- Flip-flop enhancement: Early enhancement of central liver around IVC, decreased peripheral enhancement. Later decreased enhancement centrally with increased enhancement peripherally
|
||||
- **Primary Biliary Cholangitis**
|
||||
- Irregular and "pruned" ducts leading to vanishing bile duct syndrome as disease progresses
|
||||
- Lace-like fibrosis, prominent lymphadenopathy, and hepatomegaly (early) along with positive antimitochondrial antibody test
|
||||
- **Chemotherapy Cholangitis**
|
||||
- Intraarterial chemotherapy used for primary hepatocellular carcinoma or metastases
|
||||
- Liver distortion due to tumors and parenchymal scarring
|
||||
- Ducts are damaged and strictured with appearance like primary sclerosing cholangitis
|
||||
- **Ascending Cholangitis**
|
||||
- May lead to duct strictures; rarely to chronic liver injury
|
||||
- More common are hepatic abscesses
|
||||
- **AIDS Cholangiopathy**
|
||||
- Liver may be distorted by infection &/or tumor
|
||||
- Papillary stenosis with proximal common bile duct (CBD) dilation, strictures/ulcerations of CBD, and intrahepatic strictures: Unique to AIDS cholangiopathy
|
||||
- **Fibropolycystic Liver Diseases**
|
||||
- Encompasses spectrum of related lesions of liver and biliary tract caused by abnormal embryologic development of ductal plates
|
||||
- Lesions may occur in isolation or in any combination; may be clinically silent
|
||||
- Or, may cause cholangitis, portal hypertension, GI bleeding, infection, etc.
|
||||
- Liver may be distorted by congenital fibrosis (simulates cirrhosis) or by scarring (especially with Caroli disease)
|
||||
- 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
|
||||
- **Congenital hepatic fibrosis**
|
||||
- Dysmorphic liver, portal hypertension
|
||||
- Enlarged and possibly supernumerary hepatic arteries
|
||||
- **Caroli disease**
|
||||
- Dilatation (usually saccular) of large intrahepatic ducts with alternating biliary strictures
|
||||
- Liver often progressively damaged by cholangitis, obstruction
|
||||
- **Choledochal cyst**
|
||||
- Fusiform or cystic dilation of intrahepatic ± extrahepatic bile duct
|
||||
- **Biliary hamartomas**
|
||||
- Multiple "cysts" of nearly uniform size, up to 15 mm; no biliary connection
|
||||
- Mimic cysts on CT and MR but are often echogenic on US
|
||||
- Liver and bile ducts are normal unless coexisting disease, such as Caroli or congenital fibrosis
|
||||
- **Autosomal dominant polycystic disease, liver**
|
||||
- Distorts liver with innumerable cysts
|
||||
- Bile ducts intrinsically normal but may have extrinsic compression by cysts
|
||||
- **Recurrent Pyogenic Cholangitis**
|
||||
- Usually in Asian, poorly nourished populations
|
||||
- Liver distorted by chronic infection and biliary obstruction (abscesses, cirrhosis)
|
||||
- Bile ducts enormously dilated by pus and stones
|
||||
- **Hepatic Hydatid Disease**
|
||||
- Cysts may distort liver
|
||||
- Cysts may communicate with ducts, leading to cholangitis
|
||||
|
||||
|
||||
## Images
|
||||
|
||||
|
||||
### Selected Images
|
||||
|
||||
|
||||
**Primary Sclerosing Cholangitis**
|
||||
*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'/>.*
|
||||
|
||||
|
||||
**Primary Sclerosing Cholangitis**
|
||||
*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'/>.*
|
||||
|
||||
|
||||
**Primary Sclerosing Cholangitis**
|
||||
*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.*
|
||||
|
||||
|
||||
**Primary Sclerosing Cholangitis**
|
||||
*Coronal MRCP MIP in a 37-year-old man with PSC shows extensive intrahepatic strictures with alternating sites of narrowing and ductal dilatation.*
|
||||
|
||||
|
||||
**Hepatitis**
|
||||
*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.*
|
||||
|
||||
|
||||
**Peribiliary Cysts**
|
||||
*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.*
|
||||
|
||||
|
||||
**Peribiliary Cysts**
|
||||
*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.*
|
||||
|
||||
|
||||
**Portal Vein Thrombophlebitis (Mimic)**
|
||||
*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.*
|
||||
|
||||
|
||||
**Portal Vein Thrombophlebitis (Mimic)**
|
||||
*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.*
|
||||
|
||||
|
||||
**Cholangiocarcinoma, Intrahepatic or Hilar**
|
||||
*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.*
|
||||
|
||||
|
||||
**Budd-Chiari Syndrome**
|
||||
*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.*
|
||||
|
||||
|
||||
**Budd-Chiari Syndrome**
|
||||
*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.*
|
||||
|
||||
|
||||
**Primary Biliary Cholangitis**
|
||||
*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).*
|
||||
|
||||
|
||||
**Fibropolycystic Liver Diseases**
|
||||
*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.*
|
||||
|
||||
|
||||
**Caroli Disease**
|
||||
*Axial T2 MR shows cystic and irregular cylindrical dilation of intrahepatic bile ducts, characteristic of Caroli disease. Progressive liver failure resulted in transplantation.*
|
||||
|
||||
|
||||
**Recurrent Pyogenic Cholangitis**
|
||||
*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'/>.*
|
||||
|
||||
|
||||
**Hepatic Hydatid Disease**
|
||||
*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.*
|
||||
|
||||
|
||||
### Additional Images
|
||||
|
||||
|
||||
**Cirrhosis (Mimic)**
|
||||
*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.*
|
||||
|
||||
|
||||
**Chemotherapy Cholangitis**
|
||||
*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.*
|
||||
|
||||
|
||||
**Chemotherapy Cholangitis**
|
||||
*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.*
|
||||
|
||||
|
||||
**Caroli Disease**
|
||||
*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.*
|
||||
|
||||
|
||||
**Cholangiocarcinoma, Intrahepatic or Hilar**
|
||||
*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.*
|
||||
|
||||
|
||||
**Peribiliary Cysts**
|
||||
*Transverse US shows sonolucent saccular and spherical cystic lesions paralleling the portal triads, representing peribiliary cysts in this patient with cirrhosis and ascites.*
|
||||
|
||||
|
||||
**Hepatitis**
|
||||
*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.*
|
||||
|
||||
|
||||
**Chemotherapy Cholangitis**
|
||||
*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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||||
|
||||
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Reference in New Issue
Block a user