Congenital Hepatic Fibrosis

 1 manifestation of fibropolycystic liver disease






IMAGING




• Biliary hamartomas, polycystic liver disease, choledochal cyst, and Caroli disease
image Often coexist in same patient

• CT or MR: Enlarged dysmorphic liver, left lobe hypertrophy, right lobe atrophy
image ↑ size and number of hepatic arteries

image May develop hypervascular, benign, large, regenerative nodules (nodular regenerative hyperplasia)

image T2WI bright lesions: Hepatic cysts, biliary hamartomas, dilated bile ducts (Caroli disease)

image Varices, ascites, splenomegaly


TOP DIFFERENTIAL DIAGNOSES




• Isolated polycystic liver disease

• Primary sclerosing cholangitis

• Caroli disease

• Isolated biliary hamartomas


CLINICAL ISSUES




• Congenital hepatic fibrosis is always present in patients with autosomal recessive polycystic kidney disease (ARPKD)
image Sometimes with autosomal dominant PKD

image Hepatic fibrosis that exists separately from other liver & renal disease is very rare

• Congenital hepatic fibrosis is variable in severity, age at presentation, and clinical manifestations
image Portal hypertension is usually present by adolescence

• Diagnosis: Coexisting hepatic and renal cysts
image Liver biopsy

• Treatment for moderate to severe fibrosis: Transplantation (hepatic and renal)

image
(Left) Low-power view shows marked portal expansion and numerous irregularly shaped bile ducts image. The lobular architecture in adjacent parenchyma is well maintained with normal central veins image. (Courtesy H. Wang, MD, PhD.)


image
(Right) Axial CECT in a young woman with congenital fibropolycystic disease shows a dysmorphic, heterogeneous liver with several large cystic lesions, representing cystic and fusiform dilation of biliary tree (Caroli disease); note the central dot sign image of Caroli disease.

image
(Left) Axial CECT in a 40-year-old woman shows numerous cysts within the liver, many with the central dot sign image that represents the portal vein branches around which the biliary cystic spaces of Caroli disease are wrapped. This patient had biopsy-proven congenital hepatic fibrosis, which results in hepatic failure and accounts for splenomegaly image.


image
(Right) Coronal CECT in the same patient shows the cystic bile ducts of Caroli disease image with large supernumerary hepatic arteries image and a renal allograft image.


TERMINOLOGY


Abbreviations




• Congenital hepatic fibrosis (CHF)


Definitions




• Part of a spectrum of congenital abnormalities resulting in variable degrees of fibrosis and cystic anomalies of liver and kidneys

• 1 manifestation of fibropolycystic liver disease


IMAGING


General Features




• Best diagnostic clue
image Dysmorphic liver with cysts, abnormal ducts, + signs of portal hypertension
– May show similar changes within kidneys

• Location
image Both lobes of liver

• Size
image Hepatic cystic size varies based on severity of CHF

• Other general features
image CHF is always present in patients with autosomal recessive polycystic kidney disease (ARPKD) and sometimes present with autosomal dominant PKD

image 2 constant features of ARPKD
– Kidney: Tubular ectasia, cysts, and fibrosis

– Liver: Fibrosis (dilated bile ducts, enlarged/fibrotic portal triads) and multiple cysts
image All patients with ARPKD have findings of hepatic fibrosis on biopsy

image Not all patients with hepatic fibrosis have ARPKD

image Relative severity of disease may vary
– Renal or hepatobiliary disease may predominate

image Variants of fibropolycystic liver disease
– CHF, biliary hamartomas, polycystic liver disease, choledochal cyst, and Caroli disease often coexist in same patient

image Hepatic fibrosis that exists separately from other liver and renal diseases is very rare


Radiographic Findings




• ERCP
image ± dilatation of intrahepatic bile ducts


CT Findings




• Mild CHF
image Liver may appear normal

• Moderate to severe fibrosis
image Bile ducts: Normal to irregularly dilated

image Enlarged dysmorphic liver, left lobe hypertrophy, right lobe atrophy

image Splenomegaly and varices

image Increased size and number of hepatic arteries

image May develop hypervascular, benign, large, regenerative nodules (nodular regenerative hyperplasia)
– Similar to those seen in Budd-Chiari syndrome

• Associated polycystic disease of liver and kidney
image Multiple hypodense (water density) hepatic and renal cysts of varied size

image CECT: No enhancement of cyst contents


MR Findings




• MR cholangiography (MRC)
image Bile ducts; variable pattern: Normal, irregular dilation, Caroli pattern

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Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Congenital Hepatic Fibrosis

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