Computer-generated three-dimensional morphology of the hepatic hilar bile ducts in biliary atresia

In biliary atresia (BA), although the intrahepatic bile ducts are reported to appear in the fetal configuration in about 20% to 60% of cases, information about the 3-dimensional (3-D) morphology of the hilar biliary structures is limited. The authors, therefore, have applied the technique of computer-generated 3-D reconstruction, to study the morphology of the hilar biliary structures in patients who have undergone biliary decompression.

Computer-generated 3-D reconstructions were performed from serial sections of the hepatic hilum of 4 BA patients and compared with those obtained from a 3-day old infant who had no apparent biliary disease but died of congenital diaphragmatic hernia (CDH) and with normal human fetal ductal plate at 11 weeks of gestation.

In all 4 BA patients, the hilar bile ducts strongly resembled the ductal plate structures of the fetus rather than the bile ducts of the CDH infant. This leads the authors to suggest that these abnormal biliary structures are the result of improper ductal plate remodeling at the hepatic hilum. In 3 of the BA patients there were some tubular bile ducts that probably are the result of delayed and abnormal attempt at ductal plate remodeling.