Intracranial haemorrhage in a children suffering from heamofilia

Haemophilia is a hereditary X-linked desease manifested in coagulatory hemostasis disorder. Haemophilia A is a recessive mutation in X-chromosome which results in the absence or malfunction of blood clotting factor VIII. Its frequency from 1:5000 to 1:10,000 of born boys. Haemophilia B is caused by the factor IX deficiency (recessive mutation in X-chromosome), with an incidence from 1:25,000 to 1:30,000 of born boys. Frequency of occurrence hemorrhagic stroke in children is 0.7–5.1 (average – 2.9) per 100,000. Reported intracranial hemorrhages (ICH) boys with haemophilia are rare, however, according to foreign authors encountered in 1.7–11.7 % of patients that with appropriate recalculation was 290–2142:100,000 per year. Thus, the risk of ICH patients with hemophilia in 56–420 times higher than the general population. The median age of children with ICH ranges from 5.9 months to 8 years. Operational tactics not developed.

children, hemophilia, intracranial hemorrhage, intracerebral hematoma

1. Silverstein A. Intracranial bleeding in hemophiliacs. Arch Neurol 1960;3:141–57.

2. World Federation of Hemophilia. http://www.wfh.org/index.asp?lang=EN.

3. Antunes S.V., Vicari P., Cavalheiro S., Bordin J.O. Intracranial haemorrhage among a population of haemophilic patients in Brazil. Haemophilia 2003;9:573–7.

4. Eyster M.E., Gill M.F., Blatt P.M. et al. Central nervous system bleeding in hemophiliacs. Blood 1978;51:1179–88.

5. Klinge J., Auberger K., Auerswald G. et al.; The Paediatric Group of the German Society of Thrombosis and Haemostasis. Prevalence and outcome of intracranial haemorrhage in haemophiliacs – a survey of the paediatric group of the German Society of Thrombosis and Haemostasis (GTH). Eur J Pediatr 1999;158(Suppl 3):162–5.

6. Stieltjes N., Calvez T., Demiguel V. et al. Intracranial haemorrhages in French haemophilia patients (1991–2001): clinical presentation, management and prognosis factors for death. Haemophilia 2005;11:452–8.

7. Witmer C., Presley R., Kulkarni R. et al. Associations between intracranial haemorrhage and prescribed prophylaxis in a large cohort of haemophilia patients in the United States. Br J Haematol 2011;152(2):211–6.

8. Zanon E., Iorio A., Rocino A. et al.; The Italian Association of Hemophilia Centers. Intracranial haemorrhage in the Italian population of haemophilia patients with and without inhibitors. Haemophilia 2012;18:39–45.

9. Lynch J.K. Cerebrovascular disorders in children. Curr Neurol Neurosci Rep 2004;4:129–38.

10. Roach E.S., Golomb M.R., Adams R. et al.; American Heart Association Stroke Council; Council on Cardiovascular Disease in the Young. Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke 2008;39(9):2644–91.

11. Nelson M.D. Jr, Maeder M.A., Usner D. et al. Prevalence and incidence of intracranial haemorrhage in a population of children with haemophilia. The Hemophilia Growth and Development Study. Haemophilia 1999;5(5):306–12.

12. Ljung R.C. Intracranial haemorrhage in haemophilia A and B. Br J Haematol 2008;140(4):378–84.

13. Nuss R., Soucie J.M., Evatt B.; Hemophilia Surveillance System Project Investigators. Changes in the occurrence of and risk fators for hemophilia-associated intracranial hemorrhage. Am J Hematol 2001;68(1):37–42.

14. de Tezanos Pinto M., Fernandez J., Perez Bianco P.R. Update of 156 epiodes of central nervous system bleeding in hemophiliacs. Haemostasis 1992;22(5):259–67.

15. Andes W.A., Wulff K., Smith W.B. Head trauma in hemophilia. A propective study. Arch Intern Med 1984;144(10):1981–3.

16. Bentancor N., Lavalle E., Vila V.M. et al. [Intracrnial hemorrhage in hemophiliacs. Study of 10 episodes]. Sangre (Barc) 1992;37(1):43–6.

17. Traivaree C., Blanchette V., Armstrong D. et al. Intracranial bleeding in haemophilia beyond the neonatal period – the role of CT imaging in suspected intracranial bleeding. Haemophilia 2007;13(5):552–9.

18. Revel-Vilk S., Golomb M.R., Achonu C. et al. Effect of intracranial bleeds on the health and quality of life of boys with hemophilia. J Pediatr 2004;144(4):490–5.

19. Ghosh K., Nair A.P., Madkaikar M. et al. Intracranial haemorrhage in severe haemophilia: prevalence and outcome in a developing country. Haemophilia 2005;11:459–62.

20. Mishra P., Naithani R., Dolai T. et al. Intracranial haemorrhage in patients with congenital haemostatic defects. Haemophilia 2008;14:952–5.

21. Yue C.P., Mann K.S. The surgical management of intracranial hematomas in hemophiliac children. Childs Nerv Syst 1986;2(1):5–9.

22. Hoyer L.W. Hemophilia A. N Engl J Med 1994;330(1):38–47.

23. Visconti E.B., Hilgartner M.W. Recognition and management of central nervous system hemorrhage in hemophilia. Paediatrician 1980;9:127–37.

24. Seeler R.A., Imana R.B. Intracranial hemorrhage in patients with hemophilia. J Neurosurg 1973;39:181–5.

25. Davies Jones G.A.B., Preston F.E., Timperley W.R. Neurological complications in clinical haematology. Blackwell, Oxford, 1980. Pp. 143–173.

26. Kasper C.K. Hematologic care. In: Boone D.C. (ed.). Comprehensive management of hemophilia. Davis, Philadelphia, 1976. Pp. 3–17.

27. Martinowitz U., Heim M., Tadmor R. et al. Intracranial hemorrhage in patients with hemophilia. Neurosurg 1986;18(5):538–41.

28. Meyer-Heim A.D., Boltshauser E. Spontaneous intracranial haemorrhage in children: aetiology, presentation and outcome. Brain Dev 2003;25:416–21.

29. Kerr C.B. Intracranial haemorrhage in haemophilia. J Neurol Neurosurg Psychiatry 1964;27:166–73.