INTRODUCTION: Intracranial haemorrhage (ICH) is the most serious complication in infants and children with severe congenital bleeding disorders causing substantial mortality and long-term neurological morbidity. The neonatal period carries the highest risk, particularly in severe haemophilia and rare factor deficiencies. We review the epidemiology, risk factors, prevention, recognition, management, and long-term outcomes of ICH in children with congenital bleeding disorders.
METHODS: Current evidence from cohort studies, literature reviews, and recent clinical data addressing ICH in childhood is summarized, with attention to evolving prophylactic strategies.
RESULTS: Neonatal ICH occurs in 2%-4% of infants with haemophilia and more frequently in severe FXIII and FX deficiency. Unrecognized bleeding disorders and traumatic delivery-especially with forceps or vacuum-substantially increase risk. Immediate factor replacement prior to diagnostic imaging improves survival and reduces neurological sequelae. After the neonatal period, risk remains elevated, particularly in children <1 year not receiving prophylaxis. Early initiation of prophylaxis, historically limited by challenges with intravenous access, markedly reduces ICH incidence. Emerging non-factor therapies (FVIII mimetics and rebalancing agents) enable earlier and more effective protection. Despite advances in care, 30%-40% of survivors experience long-term neurological impairments with outcomes influenced by bleed location, cerebral shift, age at time of ICH, and treatment delays.
CONCLUSIONS: ICH remains a major threat to children with severe bleeding disorders. Prevention requires early identification of at-risk pregnancies, careful delivery planning, and timely diagnosis of bleeding disorders. Advances in prophylactic therapy offer the potential to significantly reduce ICH incidence and improve long-term outcomes.
}}, author = {{Carcao, Manuel D and Andersson, Nadine G and Gouw, Samantha}}, issn = {{1351-8216}}, language = {{eng}}, month = {{04}}, publisher = {{John Wiley & Sons Inc.}}, series = {{Haemophilia}}, title = {{Prevention, Identification, Management and Long-Term Complications of Intracranial Haemorrhage in Children With Haemophilia and Other Severe Bleeding Disorders-A Practical Guidance}}, url = {{http://dx.doi.org/10.1111/hae.70239}}, doi = {{10.1111/hae.70239}}, year = {{2026}}, }