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 ?
Ductal plate malformation results from persistence or absence of remodeling of the embryonic ductal plate during ontogenesis.
Also known as von Meyenburg complexes, bile duct hamartomas arise from ductal plate malformations that involve the small interlobular bile ducts.
Two forms have been described: the pure form, which is associated with attacks of cholangitis and intraductal stone formation; and the complex form, which is more common and is associated with other ductal plate malformations.