European Journal of Pediatrics

, Volume 158, Supplement 3, pp S162–S165 | Cite as

Prevalence and outcome of intracranial haemorrhage in haemophiliacs – a survey of the paediatric group of the German Society of Thrombosis and Haemostasis (GTH)

  • J. Klinge
  • K. Auberger
  • G. Auerswald
  • H. H. Brackmann
  • Ch. Mauz-Körholz
  • W. Kreuz
  • the paediatric group of the German Society of Thrombosis and Haemostasis
HAEMOPHILIA AND VON WILLEBRAND DISEASE

Abstract

A survey among centres of the paediatric group of the GTH was performed to evaluate the prevalence and outcome of haemophiliacs with intracerebral haemorrhage. A questionnaire sent to the centres covered the following points: number of patients with severe, moderate and mild haemophilia A and B; for each patient with ICH: birth date, age at bleeding, aetiology and neurological sequelae. Overall, 30 ICH in 744 haemophiliacs (4.0%) were reported by 17/40 centres (42.5%). There was no significant difference between the prevalence of patients with haemophilia A and B (3.5% vs. 6.3%) and among the age groups. Bleeding was diagnosed within 1 week of birth in 11/27 patients (41%). For 3 patients, no age-related information was given. The most important factor was trauma (17/30 = 57%), either during birth (9/30 = 30%) or later in life (8/30  = 27%). Seizures were common, occurring in 19/30 patients (63%). As 1 patient died after posttraumatic ICH, the neurological outcome of 29 patients could be evaluated. Psychomotor and statomotor retardation and cerebral palsy were reported in 17/29 (59%), 15/29 (51%) and 13/29 (45%) patients respectively. Only 7/29 (24%) showed no neurological sequelae. Severity of deficits was not correlated with birth date but to age at bleeding. Older children showed a better neurological outcome than neonates.

Conclusion The frequency and outcome of ICH in haemophiliacs have not changed in our cohort over the past 20 years. Trauma at birth is an important risk factor for ICH in patients with haemophilia A or B. lntracranial haemorrhages in older children are rare, and a better outcome may be expected.

Key words Haemophilia Intracranial haemorrhage Prevalence Neurological outcome 

Copyright information

© Springer-Verlag Berlin Heidelberg 1999

Authors and Affiliations

  • J. Klinge
    • 1
  • K. Auberger
    • 2
  • G. Auerswald
    • 3
  • H. H. Brackmann
    • 4
  • Ch. Mauz-Körholz
    • 5
  • W. Kreuz
    • 6
  • the paediatric group of the German Society of Thrombosis and Haemostasis
  1. 1.Paediatric Department of the University of Erlangen
  2. 2.Paediatric Department of the University of Munich
  3. 3.Prof. Hess Children's Hospital, Bremen
  4. 4.Haemophilia Centre, University of Bonn
  5. 5.Paediatric Department of the University of Düsseldorf
  6. 6.Paediatric Department of the University of Frankfurt

Personalised recommendations