Congenital biliary dilatation in autosomal dominant adult polycystic disease of the liver and kidneys.

Specimens of autopsied livers and biliary tracts of three patients with autosomal dominant adult polycystic disease of the liver and kidneys were examined morphologically with adjunct postmortem cholangiography. The cholangiograms revealed nonobstructive diffuse dilatation of intrahepatic bile ducts in all cases. Macroscopic examination confirmed the nonobstructive ductal dilatation and also showed a number of cysts in all cases. Microscopically, the dilated bile duct walls were composed of fibrous walls and were free of other pathologic changes, suggesting that the biliary dilatation was congenital in origin. Small microscopic bile ducts, however, were not dilated and were free of ductal plate malformation. In addition, Meyenburg's complexes and liver cysts not communicating with the biliary tract lumen were seen. It was suggested that adult polycystic disease, Meyenburg's complexes, and congenital intrahepatic biliary dilatation could coexist in some patients.