Intracranial hemorrhage in children with congenital factor deficiencies.

Intracranial hemorrhage (ICH) is a life-threatening situation in childhood. Congenital factor deficiencies (CFD) like hemophilia may cause ICH, and ICH may be the initial presentation in some cases. From 2000 to 2010, 107 children with CFD from Erciyes University, Faculty of Medicine, Department of Pediatric Hematology, were evaluated. The ICH episodes were identified by medical history, general physical examination, detailed neurological examination, and CT or MR scan. The management strategies, surgical intervention, and outcome were noted. Twenty-one episodes of ICH were seen in 18 patients (16.8%) out of 107 CFD patients. The mean age of the patients was 42.1 months. Fourteen out of 18 patients were male, and four were female. Twelve (57.1%) out of 21 ICH episodes were caused by trauma, and nine (42.9%) were non-trauma related. Epidural hematoma was most frequently observed. All patients survived, but four had decrease in intellectual capacity and motor deficit. The optimal management of ICH in children with CFD depends on immediate recognition and prompt replacement therapy to ensure hemostatic balance with adequate surgical intervention.