Abstract
Intracerebral hemorrhage (ICH) is the leading cause of death in patients with hemophilia. The incidence of ICH is up to 50 times greater than that of the general population, and most frequently occurs in children less than two years of age and among those with the severe forms of the disease. The ideal perioperative management of this patient population, however, remains unclear.
Highlights
Intracerebral hemorrhage (ICH) is the leading cause of death in patients with hemophilia
Intracranial hemorrhage should be suspected in infants and young children who have a known history of hemophilia and present with altered mental status
Failure to maintain adequate factor VIII activity levels with a continuous infusion may be managed with a regimented bolus schedule supplemented with additional boluses prior to invasive procedures
Summary
Spontaneous intracranial hemorrhage is a morbid and potentially fatal sequela of Hemophilia A and requires a heightened level of suspicion in children less than two years of age who present with altered sensorium. Immediate correction of clotting factor deficiency is paramount prior to neurological surgery and in the immediate post-operative period. Inadequate response to a continuous infusion of clotting factor concentrate can be corrected with a regimented bolus schedule titrated to 80-100% factor VIII activity levels; 50 IU/kg given prior to additional invasive procedures was effective in achieving adequate hemostasis intra-operatively
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