Population
Persons with haemophilia of all ages (45 studies, n= 54,470).
Intervention
Summarized evidence on intracranial haemorrhage (ICH) incidence and calculated pooled ICH incidence and mortality with a systematic review and meta-analysis.
Comparison
Outcome
Incidence and mortality were pooled with a Poisson-normal model or a binomial-normal model. The included studies represented 54,470 patients, 809,151 person-years, and 5,326 live births of patients with haemophilia. In persons of all ages, the pooled ICH incidence and mortality rates were 2.3 (95% confidence interval (CI): 1.2-4.8) and 0.8 (95% CI: 0.5-1.2) per 1,000 person-years, respectively. In children and young adults, the pooled ICH incidence and mortality rates were 7.4 (95% CI: 4.9-11.1) and 0.5 (95% CI: 0.3-0.9) per 1,000 person-years, respectively. In neonates, the pooled cumulative ICH incidence was 2.1% (95% CI: 1.5-2.8) per 100 live births. ICH was classified as spontaneous in 35% to 58% of cases.