Idiopathic Neonatal Hepatitis

 Once known etiologies are excluded, then considered idiopathic



• By mid-2000s, idiopathic neonatal hepatitis (INH) comprised only 15-30% of neonatal cholestasis due to increased ability to detect and diagnose metabolic and genetic disorders previously considered idiopathic




Clinical Issues




• Jaundice

• Hepatomegaly ± splenomegaly

• Bruising/bleeding due to vitamin K deficiency

• Elevated serum total and conjugated bilirubin

• Variably elevated transaminases


Microscopic




• Lobular disarray with giant cell transformation

• Canalicular and hepatocellular cholestasis

• Minimal portal changes

• Preserved bile ducts

• Prominent extramedullary hematopoiesis


Top Differential Diagnoses




• Exclude biliary atresia right away because it requires early surgical intervention
• If neonatal hepatitis-like pattern of injury present, use clinical information and molecular tests to exclude known disorders

image INH is diagnosis of exclusion

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Lobular Giant Cell Change and Extramedullary Hematopoiesis
The characteristic features of idiopathic neonatal hepatitis include lobular giant cell transformation and prominent extramedullary hematopoiesis. Portal changes are minimal, and ductular reaction is absent.


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Cholestasis and Giant Cell Transformation
The lobular hepatocytes show giant cell change and scattered apoptotic hepatocytes image, along with canalicular cholestasis and a cholestatic rosette image .

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CMV Infection
This case of neonatal hepatitis due to CMV shows a few giant cell hepatocytes along with extramedullary hematopoiesis. A single CMV inclusion image was identified.

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Biliary Atresia May Mimic Neonatal Hepatitis
This biopsy from a 9 week old with biliary atresia has features similar to idiopathic neonatal hepatitis, such as giant cell change, but also contains expanded portal tracts with associated ductular reaction.


TERMINOLOGY


Abbreviations




• Idiopathic neonatal hepatitis (INH)


Definitions




• General term for clinical condition manifested by prolonged jaundice in neonates with variable but definable histologic picture
image Clinicopathologic picture is termed neonatal hepatitis syndrome

– Uniform clinical presentation but broad spectrum of causative disease processes

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Idiopathic Neonatal Hepatitis
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