Yellow is pale: The complications and challenges of late diagnosis of extrahepatic biliary atresia

Authors

  • Shaun SC Ho,

    1. Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital
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  • Wolfram Haller,

    Corresponding author
    1. Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital
      Dr Wolfram Haller, Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Flemington Road, Parkville, Melbourne, Vic. 3052, Australia. Fax: +613 9345 6240; email: wolframhaller@web.de
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  • Anthony G Catto-Smith

    1. Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital
    2. Department of Paediatrics, University of Melbourne
    3. Gut and Liver Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia
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  • Conflict of interest: None.

  • Financial disclosure: None.

Dr Wolfram Haller, Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Flemington Road, Parkville, Melbourne, Vic. 3052, Australia. Fax: +613 9345 6240; email: wolframhaller@web.de

Abstract

Extrahepatic biliary atresia classically presents in the neonatal period with jaundice and pale stools. The lack of bile pigment in stool can be unrecognised, delaying diagnosis and surgical treatment. Vitamin K is given at birth to reduce the risk of haemorrhagic disease of the newborn, but this may be inadequate to prevent the development of coagulopathy secondary to fat soluble vitamin malabsorption. We present the case of a 3 month old infant who presented with an intracerebral haemorrhage and coagulopathy thought to be secondary to fat malabsorption resulting from delayed diagnosis of extrahepatic biliary atresia. This was despite the perinatal administration of intramuscular vitamin K. His parents did not recognise the stool pallor as being abnormal. This case illustrates the importance of educating parents on the significance of pale stools, and also the risk of coagulopathy in extrahepatic biliary atresia despite perinatal intramuscular vitamin K.

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