Medical and surgical management of intracranial bleeding in hemophilic children

Abstract

This paper is based on the analysis of 18 hemophiliacs who suffered 28 episodes of intracranial bleeding. Ten cases were subjected to 12 operative procedures and or fontanelle taps. The 13 lesions in these 10 cases were 6 pure subdural hematomas, 5 located in the left side, 4 subdural plus intracerebral hematomas, 1 bilateral extradural plus multiple intracerebral hematomas, 1 single intracerebral and 1 hemorrhagic hydrocephalus followed by meningitis. Three subdural hematomas were neonatal. Eight cases were not operated. One was a subdural hematoma with a high concentration of a circulating anticoagulant and died. Seven cases suffered 14 episodes of subarachnoid bleeding, recurrent in 4 patients. None died. These 18 cases are classified in the following groups: (a) Minor or early forms of cerebromenigeal hemorrhage: 7 cases, all alive. (b)Major forms of progressive intracranial bleeding at one site: 6 episodes of pure subdural bleeding and 1 episode of intracerebral bleeding, in 5 patients, all alive following this episode, 1 died from a second, type (c) episode. (c) Complicated forms of progressive intracranial bleeding at various sites: 1 bilateral extradural and 3 unilateral subdural with multiple intracerebral hemorrhages, 1 subdural and intracerebral hematoma in 5 patients, all dead. (d) Miscellaneous: 1 subdural hematoma unoperated due to circulating anticoagulant, 1 hemorrhagic hydrocephalus plus meningitis, in 2 patients, both died. Factor VIII level must be determined every 12 hours and immediately following major diagnostic or operative procedures. FIOta (tannic acid precipitated Blomback FI-O fraction), an AHG at least ten times more concentrated that plasma was successfully employed in order to increase Factor VIII levels up to 100 percent. This experience indicates that major surgery requires the elevation of Factor VIII close to 100 percent and at least to 50 percent. The EEG, a most valuable diagnostic procedure in these cases, should be routinely used and repeated as many times as necessary. Lumbar puncture and contrasted neuroradiologic procedures can be safely carried out provided that factor VIII (or IX) level is at least 50 percent, but they should be judiciously employed. The extent of the operative exposure should be the smallest one operative with proper visualization and removal of the blood collection. Solid and acute clots usually require a moderate size craniectomy. Liquid and more chronic blood collections can be evacuated through a burr hole and sometimes, in infants, through a fontanelle tap. A flap seems to be unnecessary and undesirable in most cases. Major and complicated intracranial bleeding in hemophiliacs can probably be prevented with early correction of the coagulation factor deficiency in minor or early forms, in cases of minor or major head trauma and in any case of headache lasting for more than 12 hours.