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. Author manuscript; available in PMC: 2010 Aug 10.
Published in final edited form as: Am J Med Genet C Semin Med Genet. 2009 Nov 15;151C(4):296–306. doi: 10.1002/ajmg.c.30225

Figure 1.

Figure 1

Artist’s rendering of normal bile duct development in comparison with the ductal plate malformation and corresponding liver histopathology showing normal portal triad and congenital hepatic fibrosis. The ductal plate initially forms as a sleeve-like structure around the portal vein branches. Normal remodeling of the ductal plate involves resorption of parts of this structure and migration of the remodeled ducts centrally closer to the portal vein (left panel at bottom). The liver biopsy at 10× magnification shows a normal portal tract with sections of portal vein, bile duct, and hepatic artery. Defective remodeling, termed the ductal plate malformation, is characterized by retention of excessive numbers of bile duct remnants in their original peripheral interrupted ring-like position (right panel at bottom). The biopsy with CHF shows persistence of bile duct remnants (magnification = 40×). (Liver biopsies are from Potter’s pathology of the fetus, infant and child, 2nd edition, Ed: Gilbert-Barness E. Mosby Elsevier.)