Depending on severity of portal hypertension, biliary infection, and renal disease
Top Differential Diagnoses
![image](/wp-content/uploads/2017/04/image01582.jpeg)
A gross photograph of congenital hepatic fibrosis shows white fibrous bands
![image](/wp-content/uploads/2017/04/image01583.jpeg)
![image](/wp-content/uploads/2017/04/image01584.jpeg)
![image](/wp-content/uploads/2017/04/image01585.jpeg)
Marked fibrous portal expansion with bridging fibrosis and numerous ectatic and irregularly shaped bile ducts are present, some of which are characteristically located at the interface with liver parenchyma
![image](/wp-content/uploads/2017/04/image01586.jpeg)
![image](/wp-content/uploads/2017/04/image01587.jpeg)
The aberrantly formed bile ducts are lined by cuboidal biliary epithelium, and some contain inspissated bile
![image](/wp-content/uploads/2017/04/image01588.jpeg)
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
Disease Associations
• Autosomal recessive polycystic kidney disease
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![](https://freepngimg.com/download/social_media/63059-media-icons-telegram-twitter-blog-computer-social.png)
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