ductal plate malformation

ductal plate malformation

Malformation consisting of congenital hepatic fibrosis with probable autosomal recessive inheritance.
See also: dysencephalia splanchnocystica.
References in periodicals archive ?
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.
An infant with cholestasis, acholic stool and high GGT levels
MH is considered a ductal plate malformation, wherein mesenchymal rests become isolated from the normal portal triad architecture and differentiate independently [3, 4].
Mesenchymal Hamartoma of the Liver: Complete Excision Always Necessary
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.
Meckel Gruber Syndrome: a case report with emphasis on importance of antenatal diagnosis
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.
Clinical and pathological challenges in the diagnosis of late-onset biliary atresia: four case studies
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.
Cholangiocarcinoma Occurring in a Liver With Multiple Bile Duct Hamartomas (von Meyenburg Complexes)
Also known as von Meyenburg complexes, bile duct hamartomas arise from ductal plate malformations that involve the small interlobular bile ducts.
Imaging of cystic liver lesions in the adult
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