Intracranial Hemorrhage in Patients with Hemophilia

Abstract

Intracranial hemorrhage (ICH) is a life-threatening complication of hemophilia. Seven of the 288 hemophiliacs living in Israel suffered eight episodes of ICH during the years 1972 to 1982. All episodes occurred in hemophilia A patients, with a higher incidence among patients with factor VIII inhibitor. Diagnosis was confirmed by computed tomographic scan in seven of the eight episodes. Four of the 7 patients died despite adequate factor replacement and supportive therapy, probably due to a conservative and hesitant neurosurgical approach. The correction of factor VIII to hemostatic level alone is inadequate in the majority of cases, and there is sudden deterioration in the patient's condition and death. Operation is strongly recommended when no improvement is noted within a few hours.

Diagnosis and treatment of intracranial hemorrhage in children with hemophilia

“…The authors reported that the higher proportion of factor prophylaxis was consistent with practice across hemophiliac centers that routinely administer factor prophylaxis after a documented ICH. The development of inhibitors has been shown in other studies to increase the chances of recurrent ICH [22,36]. The majority of ICH in the pediatric age group is associated with trauma and birth-related factors.…”

Spontaneous intracerebral hemorrhage in hemophiliacs—A treatment dilemma

“…Spontaneous intracranial hemorrhage is a rare complication of hemophilia, with frequency of about 2.2–7.8% [2], and a mortality of 34% [3]. It is a life threatening complication and needs emergent neurosurgical intervention.…”

Intracranial Hemorrhage in Neonates with Unrecognized Hemophilia A: A Persisting Problem

“…In both cases, the presumed trauma initiating the bleed was a difficult or prolonged labor. Both of these cases presented late (days 5 and 4 of life) after a period of apparent well-being, and this is typical of neonatal intracranial hemorrhage in hemophiliacs [8, 9, 13, 17]. Thus, it would appear that the initial hemorrhage was small and well tolerated, but that deterioration was associated with a further episode of hemorrhage and brain swelling.…”

“…Repeated clotting screens should be performed after surgery until satisfactory factor levels have been demonstrated. Postoperative management should aim to increase factor VIII levels to at least 50% of normal [8, 17]or above, and we have found the best way to do this is by initial bolus followed by constant infusion. This should be continued for 2 weeks and is easier to manage through a central venous line [8].…”