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Victor Grech, John Torpiano, Cecil Vella
CaseRepClinPractRev 2002; 3(3):148-150
Background: Hemophilia A is an X-linked dominant condition leading to deficiency of factor 8 in affected males, with easy bruising and bleeding. Benign intracranial hypertension is a condition characterized by headaches;
raised cerebrospinal fluid pressure with normal composition in the absence of ventricular dilatation or an intracranial mass lesion; and neurological symptoms and signs of raised intracranial pressure.
Case report: We report a five-year-old hemophilic boy who sustained blunt head injury, and went on to develop headache, papilledema and other symptoms and signs of raised intracranial pressure. This was due to the development of severe benign intracranial hypertension as demonstrated on lumbar puncture manometry. The diagnosis was obfuscated by the presence of a small intracranial hematoma diagnosed on CT scan. He responded
rapidly to conservative standard treatment with infusions of factor 8 and tranexamic acid.
Conclusion: Benign intracranial hypertension should be excluded in all individuals with an underlying bleeding diathesis, who develop severe headaches and other symptoms and signs of raised intracranial pressure after
blunt head injury, even in the presence of an intracranial hemorrhage, even when such a hemorrhage is of a minor nature.