Learning objectives
1.
To illustrate the embryology of ductal plate malformations
2.
To demonstrate the spectrum of multimodality imaging findings of ductal plate malformations
Background
The ductal plate consists of a layer of bi-potential hepatic precursor cells surrounding portal vein radicals which undergoes remodeling into the intrahepatic biliary tree.
Remodeling of these ductal plates occurs during the 12th embryonic week to form bile canaliculi of various sizes beginning from the hilum to the periphery of the liver with successive development of initially the larger ducts,
the segmental ducts,
the interlobular ducts and finally the smallest bile ductules.
Any surplus bile ducts are degraded or resorbed.
Dysmorphogenesis of ductal plates can...
Findings and procedure details
Large-sized ductal involvement
Ultrasonography findings in large-sized ductal involvement (Caroli's disease and Caroli's syndrome) typically show large anechoic well marginated regionswith echogenic sludge or stones.
MRI cholangiography confirms that these regions communicate with the biliary tree.
Caroli’s disease is limited to the intrahepatic bile ducts,
however extrahepatic biliary dilatation is seen in up to 53% of patients.
This association may be explained by the repeated bouts of cholangitis,
stone formation and stone passage that occurs in these patients.
Contrast-enhanced CT and MRI demonstrate segmental intrahepatic...
Conclusion
A working knowledge of the principles behind the pathogenesis of DPMs is of paramount importance to general and hepatobilary radiologists.
Clinical imaging plays an important role in the correct diagnosis,
stratification of severity,
prognosis,
and follow-up of DPMs.
References
Desmet V.
Pathogenesis of Ductal Plate Abnormalities.
Mayo Clinic Proceedings 1998; 73: 80-89
Wills E,
Roepman R,
Drenth J.
Polycystic liver disease: ductal plate malformation and the primary cilium.
Trends in Molecular Medicine 2014; 20: 261-270
Raynaud P,
Tate J,
Callens C et al.
A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis.
Hepatology 2011; 53: 1959-1966
Huppert S.
A new set of classifications for ductal plate malformations.
Hepatology 2011; 53: 1795-1797
Santiago I,
Loureiro R,
Curvo-Semedo L et al....