Intracranial Hemorrhage In Patients with Inherited Bleeding Disorder In Children.

Abstract 3657

Intracranial hemorrhage (ICH) is known to be a severe although uncommon complication of inherited bleeding disorder. A 12-year retrospective study was conducted to investigated the episodes of ICH in children with an inherited bleeding disorder who where followed by a regional hemophilia comprehensive center in South Korea. We review cases of ICH events in patients with inherited bleeding disorders in children and investigate characteristics, treatment, and prognosis. Between January 1998 and August 2010, a total of 13 ICHs in 9 patients were identified.

ICHs were occurred for 7 patients with hemophilia A, one patient with hemophilia B, and one patient with factor VII deficiency. All patients were male and severe hemophilia A(2 patients); moderate hemophilia A(5 patients); moderate hemophilia B(1 patients). One of the patients with severe hemophilia A had inhibitors. The median age of patients was 6years 11months old. (range, 7 days≂f15 years old). Types of ICHs were 8 SDH, 2 EDH, 1 intracerebral with IVH, 1 intracerebral with SDH and 1 SDH with SAH. 8 cases were occurred spontaneously and 5 cases were occurred after traumatic events. Presenting symptoms were headache (6 events), seizure (2 events), vomiting (1 event), stuporous mentality (1 event), lethargy (1 event), irritability (1 event) but one patient with traumatic SDH had no symptom. Initial diagnostic modality was CT scan. One patient with severe hemophilia A who had inhibitors was initially treated with FEIBA^® and controlled well. The remaining patients with hemophilia were treated with factor eight/nine concentrate. The patients with factor VII deficiency was treated with recombinant factor VIIa (Novoseven^®). The patient with moderate hemophilia B required surgical evacuation. The mean duration of hospitalization was 12.8 days (range, 3–25 days). ICH was the first significant bleeding event in 3 patients – one with moderate hemophilia A, aged 7 days, and one with moderate hemophilia A, aged 5months, and one with factor VII deficiency, aged 1months. The median follow-up duration was 73 months (range, 4–149 months). Two younger patients had complication with long term sequale like hydrocephalus, cerebral palsy, and epilepsy. For one patient, antibody to factor VIII was developed due to factor administration.

ICH can occur in patient with a severe inherited bleeding disorder and also in patients with moderate hemophilia. Younger patients with ICH may have poor prognosis and neurologic sequale more frequently. Symptoms of younger patients was atypical so their parental recognition and medical diagnosis delayed. The median duration between onset of symptom and administration of factor is 2.6 days(range, 0–8 days). Delayed administration of factor caused the patients to have long term sequale. Rapid and appropriate factor administration is important to treatment course and prognosis.