Intracranial Hemorrhage in Children with Inherited Bleeding Disorders: A Single Center Study in China

Introduction: Intracranial hemorrhage (ICH) is a life threatening condition in children. Inherited bleeding disorders may(IBD) cause ICH

Aim: This single center study aim to identify the incidence, risk factors, and neurological outcome in children with IBD

Method: From 2005 to 2016, 240 children with inherited bleeding disorders hospitalized in Nanfang hospital, department of pediatrics, were evaluated. The ICH episodes were identified by medical history, general physical examination, detailed neurological examination, and CT or MRI exam. The risk factors, location of ICH, management strategies, surgical intervention and outcome were noted.

Results: ICH was confirmed in 54/240 children with IBD. The overall risk of ICH among children with IBD was 22.5% (95% CI: 17.2, 27.8%). 52/54 (96.2%) (95% CI: 91.1, 99.9%) was hemophilic patients. The mean age of patients was 53 months and 18/54 (33.3%) (95% CI: 20.3, 46.3%) had an ICH in the first year of life. 6/52(11.5%) (95% CI: 2.6, 20.5%) hemophilic children had multiple episodes of ICH in which 4 were inhibitor positive.9/52 (17.3%) (95% CI: 6.7, 27.9%) hemophilic children were inhibitor positive while in 24/52(46.1%) (95% CI: 32.1, 60.2%) children the inhibitor was not assessed. 22(36%) (95% CI: 23.7, 48.5%) out of 61 ICH episodes were caused by trauma and 39(63.9%) (95% CI: 51.5, 76.3%) were non-trauma related. Subdural hematoma was most frequently observed. Mortality risk from ICH in children with IBD was 5/54(9.2%) (95% CI: 1.3, 17.2%). 11(22.4%) (95% CI: 10.3, 34.6%) of 49 survivors had known neurological sequelae while 38(77.5%) (95% CI: 65.4, 89.7%) had no documented evidence of neurological impairment.

Conclusion: Hemophilia is the most common primary disease of ICH in children in our center. Risk and consequences of ICH in IBD were high during the first year of life while in older children better outcome may be expected. The optimal management of ICH depends on immediate recognition and prompt replacement therapy.