Path Image
IMAGE DESCRIPTIONS

Proliferation of ductules is seen at the port of the parenchyma and portal tracts; portal edema is not prominent because this patient has been affected for 2 months. Image

ductular cholestasis is seen with ductal structures plugged with insippated bile Image

lobular cholestatis Image

An expanded portal tract shows ductual reaction occurs within portal tracts and along the outside of the lobules Image

portal fibrosis is seen expanding the triads; a minimal degree of periportal fibrosis is present. this infant had choledochocal cyst obstruction for 2 months; it takes months for true septal fibrosis to develop, and years for secondary biliary cirrhosis to form. Image

Case 2 - newborn with markedly elevated bilirubin and absense of bile ducts and giant cell transformation with gigantic hepatocytes Image

no ducts but lots of ductular proliferation at the edge of the lobule Image

fiborosis too Image

another case Image

21 Image

again Image

corresponding trichrome Image

CLINICAL

Infants develop jaundice by the second or third week of life. The infant may gain weight for the first month, but then lose weight and become irritable with worsening jaundice.

TREATMENT

Treatment involves the Kasai procedure, which connects the liver to the small intestine, circumventing the abnormal ducts.

PROGNOSIS

60% of children achieve biliary drainage after Kasai portoenterostomy and have serum bilirubin within the normal range within 6 months. 80% of children who attain satisfactory biliary drainage reach adolescence with a good quality of life without undergoing liver transplantation.

Last updated: 2011-08-01
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