Monitoring and Management of Increased Intracranial Pressure in Reye Syndrome: Results in 29 Children

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Abstract
The outcome of 29 children with Reye syndrome admitted to Yale-New Haven Hospital since initiating a regimen incorporating intracranial pressure (ICP) monitoring, controlled respiration, and administration of mannitol and barbiturates was reported. The majority of the children were in stage 3 comma and 24/29 had ICP monitored via an intraventricular catheter. Evidence of severe disease characterized by blood ammonia concentrations > 500 .mu.g/100 ml, and difficulty controlling ICP manifested by the need to administer numerous doses of mannitol were exhibited by 17 of the children, and longer hospitalizations were required for them. The earliest and most indicator of severity was the degree of elevation of the blood ammonia concentration; other laboratory values (SGOT [serum glutamic oxaloacetic transaminase], lactic dehydrogenase, creatine phosphokinase), while related to each other, did not reliably predict severity. ICP monitoring was maintained for a median duration of 8 days and ICP was most difficult to control for the 1st few days of the illness. Children [2] died during the acute illness (2/29, mortality 6.9%) and there were significant neurologic residua in 3 children (10.3%). The outcome using the present management technqiue was clearly superior to previous management when considering the most severely affected children. Prior to ICP monitoring (1967-1974) a 60% mortality was experienced in children with blood ammonia > 500 .mu.g/100 ml; in this present series two deaths and two children with significant residual were noted in the group of 17 children with blood ammonia > 500 .mu.g/100 ml, an overall salvage rate of 11/15 (73.3%). It is not possible to determine which measure or combination of measures is necessary or whether addition to or modification of the treatment protocol might be more effective. Resolution of such issues must await the results of a prospective, multi-institutional collaborative project.