Recognition and management of central nervous system hemorrhage in hemophilia.

18 central nervous system bleeding episodes in 8 patients with hemophilia A, B, or von Willebrand's disease are reviewed. Survival was seen in 17 of the 18 episodes. There was no mortality from uncontrolled bleeding and permanent neurological sequelae were seen in 8 instances. Management consisted of the use of neurological procedures to define the site of bleeding, therapeutic neurological or neurosurgical treatment where indicated, and vigorous factor replacement under laboratory control. The optimal duration of replacement therapy appears to be 14 days. Trauma as a precipitant was found in only 40% of the episodes with no other common etiologic factor.