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Septicaemia and adrenal haemorrhage in congenital asplenia.
  1. M P Dyke,
  2. R P Martin,
  3. P J Berry
  1. Royal Hospital for Sick Children, Bristol.


    Five patients developed overwhelming infection as a result of congenital asplenia, which was previously unsuspected in all cases. Each illness followed a fulminant course resulting in death within 24 hours. They illustrate the respective roles of Haemophilus influenzae infection (n = 4) and adrenal haemorrhage (n = 4) in this condition. We suggest a management protocol for screening infants with abnormalities of the atria or viscera with splenic ultrasound and examination of a blood film for Howell-Jolly bodies. Vaccination and prophylactic antibiotics should be considered for those at risk. Vigorous use of parenteral antibiotics and steroids in suspected infection is recommended.

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