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Development
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Clinical scenario: 16-month old boy with abdominal pain, weight loss and occasional diarrhea.
Liver, wedge biopsy:
-- Diffuse severe lobular cholestasis and hepatocellular degeneration associated with a decreased number of portal bile ducts, most compatible with paucity of bile ducts (intrahepatic biliary atresia).
-- No granuloma, ascending cholangitis or biliary cystic ectasia
-- Mild portal fibrosis
-- See microscopic description and Note
Microscopic Description and Note: Sections reveal generous fragments of subcapsular region of the liver with an adequate numbers of portal tracts for evaluation. There is an apparent decrease in the number of bile ducts in these portal tract (less than 0.5 bile ducts per portal tract) with (compensatory) small cholangiolar proliferation. There is a significant mononuclear inflammatory infiltrate (predominantly lymphocytes) at the periphery of the portal tracts. No portal granuloma or ductal bile stasis is identified. Mild portal fibrosis is also noted. The lobular architecture is relatively preserved. The hepatic lobules are remarkable for diffuse severe hepatocellular degeneration associated with prominent lobular cholestasis (both cytoplasmic and canalicular type). Rare extra-medullary hematopoiesis is noted in the sinusoids. The paucity of the bile ducts in the portal tracts is confirmed by immunohistochemical stains for CK19. PASD stain for cytoplasmic hyaline globules is negative. Special stain reveals increase insoluble iron deposition predominantly in the interface (peripheral) zone of the lobule, graded 1-2 out of 4. Rhodamine stain for copper binding protein is negative.
Given the patient's age and hepatic enzyme profile (predominantly as biliary obstructive pattern), the histologic findings are most compatible with the diagnosis of intrahepatic biliary atresia (paucity of ducts syndrome). The differential diagnosis of alpha-1 antitrypsin deficiency, extra-hepatic biliary atresia, congenital hemochromatosis and Wilson's disease are also entertained but are essentially excluded by the combined histological, immunohistochemical and clinical findings. Distinction of syndromic (Alagile's) from nonsyndromic intrahepatic biliary atresia cannot be made at this early stage histologically. Correlation with findings of physical exam and spine imaging would be more helpful. Genetic study of JAGGED-1 (JAG-1) may be considered if also clinically indicated.
Editor's comment:
Including such a long microscopic description and note may seem to contradict recommended report guidelines. However, for pathology diagnosis infrequently encountered (in your own practice), unfamiliar to your colleagues (or yourself), unexpected to the referring clinician, a well structure description with focus on diagnostic features, key ancillary studies and main DDx is very helpful.
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