It may be related with agenesis/atresia of the intrahepatic bile ducts due to ductal plate malformation
. CHF associated with ARPKD should be considered as differential diagnosis in patients with normal OC.
MH is considered a ductal plate malformation
, wherein mesenchymal rests become isolated from the normal portal triad architecture and differentiate independently [3, 4].
Fibrocystic changes to the liver are possibly the only other constant finding [33% to 100%], typically manifesting as impeded development of the intrahepatic biliary system and ductal plate malformation
Findings such as ductal plate malformation
have also been observed in some cases, suggesting an intrauterine insult in the physiopathogenesis of BA, but with no prognostic significance.
Ductal plate malformation results from persistence or absence of remodeling of the embryonic ductal plate during ontogenesis.
Congenital diseases of intrahepatic bile ducts: variations on the theme "ductal plate malformation." Hepatology.
Also known as von Meyenburg complexes, bile duct hamartomas arise from ductal plate malformations
that involve the small interlobular bile ducts.