We investigated 52 of 457 patients with congenital factor deficiencies with 57 episodes of intracranial haemorrhage (ICH) between 1998 and 2007. There were 38 severe haemophiliacs, 6 with
factor XIII deficiency, 5 with
factor X deficiency, 2
factor V-deficient patients, and 1 with
type 3 von Willebrand disease (VWD). The median age was 8 years (range 1 month-22 years). Most patients were below 15 years of age (86.5%). All patients with
factor X deficiency were between 1 and 5 months of age. ICH was the primary
bleeding episode leading to detection of factor deficiency in 19.2% (five patients with severe
haemophilia and all patients with
factor X deficiency).
Trauma caused
bleeding in 66%. None of the patients with
factor X deficiency had history of prior
trauma. Surgery was performed in five patients with subdural haematomas, all of whom survived. Conservative factor replacement with 100% correction for 3 days followed by 50-60% correction for 7 days was possible in 60% patients.
Seizures requiring prolonged
therapy were noted in eight patients. Death was recorded in 15 patients (29%). Inadequate
therapy in the form of delay or insufficient replacement was noted in 7/15 deaths. ICH was seen in 11.3% of all patients with
coagulation factor deficiencies.
Factor X deficiency presented with ICH at an earlier age. Inadequate replacement
therapy including
delayed treatment caused nearly 50% of all deaths. Most patients can be managed satisfactorily with adequate replacement
therapy alone, with surgery being reserved for those with worsening neurological conditions.