Assessment of Intracranial Hemorrhage Demographics in Children with Hemophilia: A Multicentre Study in China

Objective: Intracranial hemorrhage (ICH) is a severe complication in hemophilia patients with high mortality and disabling sequelae. This study is to provide key insights on the demographics of hemophilic patients with ICH in China.

Method: The database and medical notes ofHemophilia patients who were diagnosed between 1995 and 2014 are reviewed with a goal to find the effective factors which relate to ICH such as age of onset, type, severity, cause, and ICH position, presence of inhibitor and neurological sequelae. A total of 145 hemophilic boys who are diagnosed with ICH and come from multiple hemophilia centers in China, are included.

Result: The median age of ICH is 18 months. 123/145 (85%) are Hemophilia A and 22/145 (15%) are Hemophilia B. Intracranial hemorrhages most commonly occur in patients with severe disease 79/145 (54%), while 58/145 (40%) and 8/145 (5%) in moderate and mild disease respectively. There is obvious trauma history in 53/145 (37%) children. Neurological disabilities are seen only in 34/145 (23%). No deaths from ICH have been reported in our study. Data about the position of ICH is available only in 30% of patients; including involvement of multiple areas is seen in 50%. ICH in epidural space is 14%, brain parenchyma is 14%, subdural is 11% and subarachnoid is 5%.Out of 30 patients inhibitor is found only in 9 cases while in other 115 patients inhibitor data is unavailable. After the onset of ICH only 53/145 (37%) children are put on regular prophylaxis to prevent further episode of ICH.

Conclusion: Risk of ICH is high in patients with severe hemophilia. In most cases of ICH, there is no trauma history and relatively less neurological consequences. About half of children with ICH multiple areas of brain are involved. We need more data to conclude the other risk factors in the incidence of ICH.