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Bleeding as the first symptom of extrahepatic biliary atresia

R H Houwen1, J Bouquet, C M Bijleveld

  • 1Department of Pediatrics, University Hospital Groningen, The Netherlands.

European Journal of Pediatrics
|July 1, 1987
PubMed

Insights

Extrahepatic biliary atresia can present with bleeding, not just jaundice. This condition may cause vitamin K deficiency due to malabsorption, leading to a bleeding diathesis in infants.

Area of Science:

  • Pediatric Surgery
  • Hepatology
  • Neonatology

Background:

  • Extrahepatic biliary atresia (EHBA) is a rare congenital condition affecting bile ducts outside the liver.
  • Classical presentation involves prolonged jaundice, often leading to delayed diagnosis and treatment.
  • Hemorrhagic phenomena are not typically the primary presenting symptom of EHBA.

Observation:

  • This report details four infants with EHBA who primarily exhibited hemorrhagic phenomena.
  • These infants presented with an increased bleeding tendency, rather than the expected prolonged jaundice.
  • The bleeding was attributed to vitamin K deficiency, likely secondary to cholestasis-induced malabsorption.

Findings:

  • Vitamin K deficiency is a significant complication of EHBA, manifesting as a bleeding diathesis.
  • Malabsorption caused by cholestasis in EHBA can impair vitamin K absorption.
  • Hemorrhagic symptoms can be the initial clinical sign of EHBA.

Implications:

  • Clinicians should consider EHBA in infants presenting with unexplained bleeding disorders.
  • Early recognition of EHBA, even without jaundice, is crucial for timely surgical intervention.
  • Investigating vitamin K deficiency in infants with bleeding diathesis may reveal underlying biliary atresia.

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