Liver and intrahepatic bile ducts - nontumor
Hepatitis - noninfectious
Neonatal hepatitis

Author: Anthony W.H. Chan, FRCPA (see Authors page)

Revised: 31 October 2017, last major update July 2015

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Neonatal hepatitis[TI] full text[sb]

Cite this page: Chan, A.W.H. Neonatal hepatitis. PathologyOutlines.com website. http://pathologyoutlines.com/topic/liverneonatalhep.html. Accessed December 14th, 2017.
Definition / general
  • Neonatal cholestasis is prolonged jaundice beyond 2 weeks of age; it can be simply classified into extrahepatic and intrahepatic causes (Clin Res Hepatol Gastroenterol 2014;38:263)
  • Extrahepatic biliary atresia is the most common cause of extrahepatic cholestasis
  • Neonatal hepatitis is a nonspecific collective term for intrahepatic cholestasis due to all various etiologies
Terminology
  • Also known as neonatal giant cell hepatitis because of frequent syncytial giant cell formation
Epidemiology
Etiology
  • Intrahepatic neonatal cholestasis accounts for 60 - 70% of all neonatal cholestasis
  • Usual causes include (Front Pediatr 2015;3:43):
    • 9 - 17%: metabolic disorders - alpha-1-antrypsin deficiency, cystic fibrosis and hypopituitarism are common (Am J Surg Pathol 2010;34:1498)
    • 10%: progressive familial intrahepatic cholestasis (PFIC) (J Clin Exp Hepatol 2014;4:25)
    • 10%: preterm (increased incidence of 100 - 200x in infants born before 28 weeks of gestation compared to term infants)
    • 1 - 9%: congenital / neonatal infection - cytomegalovirus, enterovirus, hepatitis B, herpes simplex, human herpesvirus 6, rubella, sepsis, syphilis, toxoplasmosis, varicella
    • 2 - 6%: Alagille syndrome
    • 2%: chromosomal abnormalities - trisomy 18 and Down syndrome (incidence 100x normal term infants)
    • 13 - 30%: idiopathic neonatal hepatitis
Clinical features
  • Prolonged jaundice beyond 2 weeks of age
  • Other clinical features depend on underlying etiology
Diagnosis
  • Based on clinical, laboratory, radiological and histological features
Case reports
Microscopic (histologic) description
  • General nonspecific changes:
    • Lobular changes: giant cell transformation (hepatocytes containing 4 - 10 nuclei), variable lobular inflammatory infiltrate and necrosis (spotty, confluent to bridging), canalicular ± hepatocellular bilirubinostasis, extramedullary hematopoiesis
    • Portal tract changes: variable portal mononuclear inflammatory infiltrate
  • Specific changes depend on underlying etiology
Microscopic (histologic) images

Images hosted on other servers:
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CMV neonatal hepatitis



Images contributed by Dr. Anthony Chan:
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Neonatal hepatitis

Negative stains
Differential diagnosis