It is uncommon for infants with hemophilia to have excessive bleeding during the neonatal period. Even if bleeding occurs, it rarely becomes life-threatening, such as in intracranial hemorrhage (ICH). We here report a case of a 4-day-old boy who had intracranial hemorrhage as the first complication of hemophilia B. Computerized axial tomography (CT scan) and ultrasonography were very useful for early diagnosis. Only a few cases of neonatal hemophilia with intracranial hemorrhage have been reported, but the occurrence of this complication is probably more frequent. We reviewed seven cases (including our case) with intracranial hemorrhage as the first manifestation of neonatal hemophilia. Although these infants showed good prognosis as to survival, permanent residual neurological deficits remained in all of them. It is emphasized that intracranial hemorrhage due to hemophilia may occur in neonates even without a family history. Urgent neuroimaging and coagulation studies are necessary for an early and adequate diagnosis.
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