ductal plate malformation
Thursday 18 March 2004
Definition: Intrahepatic bile ducts (IHBDs) develop from bipotential liver progenitor cells in contact with the mesenchyme of the portal vein and thus form the ductal plates. The ductal plates are remodeled into mature tubular ducts. Lack of remodeling results in the persistence of periportal epithelial sleeves or "ductal plate malformation" (DPM).
A proposal is that virtually all congenital diseases of IHBDs represent examples of ductal plates malformations (DPM).
autosomal recessive polycystic kidney disease (hepatic ARPKD) (50% of children, 70% of families)
- DPM of interlobular bile ducts associated with tubular dilatation of collecting renal tubules
Caroli disease > DPM of the larger IHBDs
Caroli syndrome > Caroli disease + congenital hepatic fibrosis
von Meyenburg complexes > DPM of smaller interlobular ducts (liver cysts in autosomal dominant polycystic kidney disease)
non-syndromal ductal plate malformation
A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis. Raynaud P, Tate J, Callens C, Cordi S, Vandersmissen P, Carpentier R, Sempoux C, Devuyst O, Pierreux CE, Courtoy P, Dahan K, Delbecque K, Lepreux S, Pontoglio M, Guay-Woodford LM, Lemaigre FP. Hepatology. 2011 Jun;53(6):1959-66. PMID: 21391226
Sergi C, Adam S, Kahl P, Otto HF. Study of the malformation of ductal plate of the liver in Meckel syndrome and review of other syndromes presenting with this anomaly. Pediatr Dev Pathol. 2000 Nov-Dec;3(6):568-83. PMID: 11000335