Low mortality from intracranial haemorrhage in paediatric patients with haemophilia

INTRODUCTION: Intracranial haemorrhage (ICH) in patients with haemophilia has an estimated mortality rate of 20%. Advances in haemophilia care have significantly reduced many bleeding complications but it is unclear if these advances have impacted mortality from ICH. AIM: To determine the in-hospital mortality from intracranial ICH in paediatric patients with haemophilia. METHODS: This retrospective multicentre cohort study utilized the Pediatric Health Information System administrative database with data from 43 paediatric tertiary care hospitals in the United States from January 1, 2002-December 31, 2011. Subjects included were male < 21 years of age with an ICD-9-CM code for haemophilia A or B. ICH events were identified using ICD-9-CM codes. RESULTS: There were 8325 admissions for 3133 male subjects with haemophilia. About 271 (3.3%) admissions had an ICH event in 236 (7.5%) individual subjects. The proportion of ICH events was stable over time (P = 0.13). The median age of ICH was 2 years (interquartile range 0.6-7.3). In 28.4% (77/271) of the ICH events the subject had an inhibitor. Twenty-one deaths occurred in the entire cohort (0.7%). Six (28.6%) of these deaths were in patients with an ICH for an ICH mortality rate of 2.5% (6/236). CONCLUSIONS: Mortality from ICH in paediatric patients has significantly improved from prior estimates of 20% to the current estimate of 2.5%. Unfortunately the rate of ICH events remains constant and further efforts are needed to identify alternative strategies of prevention.