Pediatr Pathol 1988;8(1):1-15

Syndromatic paucity of interlobular bile ducts: hepatic histopathology of the
early and endstage liver

Hashida Y, Yunis EJ

Children's Hospital of Pittsburgh, University of Pittsburgh, Pennsylvania.

Abstract
Morphologic findings of the liver in syndromatic paucity of intrahepatic bile
ducts (SPIHBD) during infancy include paucity of interlobular bile ducts,
features of "giant cell hepatitis," dilated lymphatics and veins in the portal
tract, perisinusoidal fibrosis, and bile duct epithelial changes with a
concentric layering of mesenchymal cells around bile ducts reminiscent of renal
dysplasia. The latter change is characteristic of SPIHBD. Although the disease
is characterized by paucity of bile ducts, morphometric studies show paucity of
interlobular bile ducts in less than half of the patients during infancy.
Reduced numbers of portal tracts and increased percentage of portal tracts
devoid of bile ducts are more constant findings. It was impossible to predict
from the early biopsy which patients would develop more severe portal fibrosis.
Later in the disease portal fibrosis is variable and unevenly distributed, being
more severe near the hilum regardless of the prior performance of a Kasai-type
operation or the state of patency of the extrahepatic bile ducts. Hypoplasia of
the extrahepatic bile ducts is the usual finding in SPIHBD, but if atresia of
extrahepatic bile ducts is associated with intrahepatic paucity of bile ducts,
the hepatic histopathology is that of PIHBD. Recognition of PIHBD would avoid
unwarranted surgical procedures