Recognition and early management of Reye's syndrome.

Abstract

Reye's syndrome continues to be associated with a high mortality. Out of 12 cases treated on our intensive care unit over a four year period, seven died, one suffered minimal cerebral damage, and four were normal. Rapid progression through coma stages and high peak ammonia concentrations worsened prognosis. Reye's syndrome was suspected in only 50% of cases at the referring hospital and resulted in prompt referral to the intensive care unit in only one case. Late referral to the unit was associated with a poor outcome. Sudden neurological deterioration followed diagnostic lumbar puncture in six children. Papilloedema was an unreliable sign of raised intracranial pressure and was absent in all cases. While computed tomography of the brain was useful in providing additional evidence of raised intracranial pressure, this could only be confirmed by direct measurement. Lumbar puncture in the presence of rapidly progressive coma should be deferred until raised intracranial pressure has been excluded. To this end, early admission to a paediatric intensive care unit with facilities for computed tomography and monitoring of intracranial pressure is recommended.