Prevention, Identification, Management and Long-Term Complications of Intracranial Haemorrhage in Children With Haemophilia and Other Severe Bleeding Disorders-A Practical Guidance
(2026) In Haemophilia- Abstract
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... (More)
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.
(Less)
- author
- Carcao, Manuel D ; Andersson, Nadine G LU and Gouw, Samantha
- organization
- publishing date
- 2026-04-16
- type
- Contribution to journal
- publication status
- epub
- subject
- in
- Haemophilia
- publisher
- John Wiley & Sons Inc.
- external identifiers
-
- pmid:41988997
- ISSN
- 1351-8216
- DOI
- 10.1111/hae.70239
- language
- English
- LU publication?
- yes
- additional info
- © 2026 John Wiley & Sons Ltd.
- id
- a9b95a32-b462-44fb-8f53-08115029e5c5
- date added to LUP
- 2026-04-17 08:53:47
- date last changed
- 2026-04-17 08:53:47
@article{a9b95a32-b462-44fb-8f53-08115029e5c5,
abstract = {{<p>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.</p><p>METHODS: Current evidence from cohort studies, literature reviews, and recent clinical data addressing ICH in childhood is summarized, with attention to evolving prophylactic strategies.</p><p>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.</p><p>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.</p>}},
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}},
}