Article Sidebar

Download
PDF

Main Article Content

Abstract


ABSTRACT
Intracranial hemorrhage (ICH) is a major life-threatening complication in hemophiliac patients but rather rarely encountered in sickle cell patients. It often occurs during childhood in response to a minimal head traumatism in hemophilia. Its diagnosis and therapeutic management can be difficult, especially in Africa where coagulation factor replacement is often lacking. We are reporting the case of an 8-year-old boy presenting with severe hemophilia A, sickle disease trait and Down’s syndrome. Six days prior to his admission, the child reported severe headaches and visual disorders leading to a fall with loss of consciousness. Upon clinical examination, an intracranial hypertension syndrome was found. The Brain Computed Tomography revealed multiple intracranial hematomas associated with subarachnoid hemorrhage complicated by hydrocephalus. The patient benefited from a coagulation factor VIII substitution therapy and neurological monitoring. The outcome was favorable and the follow-up Brain CT scan showed a complete resorption of the hematomas. Spontaneous intracranial hematoma in sickle cell disease and hemophilia raises a diagnostic problem and its management remains difficult in Africa due to the unavailability of replacement therapy. Early diagnosis and appropriate management would reduce the morbidity and mortality associated with intracranial hypertension.
RÉSUMÉ
L’hémorragie intracérébrale est une complication redoutable de l’hémophilie, mais elle est rare chez l’enfant drépanocytaire. Elle survient généralement après un trauma minime chez l’hémophile. Le diagnostic et la prise en charge peuvent être difficiles en Afrique du fait du manque de composés sanguins appropriés. Nous reportons le cas d’un garçon de huit ans porteur d’une hémophilie A sévère, d’un trait drépanocytaire et d’un mongolisme. Six jours avant l’admission, l’enfant a signalé des céphalées sévères et des troubles visuels ayant conduit à une chute avec perte de conscience. L’examen clinique a montré un syndrome d’hypertension intracrânienne. Le scanner cérébral a mis en évidence de multiples hématomes intracrâniens et une hémorragie méningée compliquée dar une hydrocéphalie. Le traitement a associé un traitement à base de facteur de coagulation VIII et un monitoring neurologique. Le scanner de contrôle a montré une résolution complète des hémorragies. L’hémorragie cérébrale chez le sujet drépanocytaire hémophile pose des problèmes diagnostiques et son traitement reste délicat. Un diagnostic précoce et une prise en charge adaptée pourraient réduire sa morbidité et sa mortalité qui sont essentiellement liés au syndrome d’hypertension intracrânienne associé.

Keywords

Hemophilia, intracranial hemorrhage, blood coagulation factor, sickle cell disease

Article Details

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

How to Cite
Sambou Natacha, Diop Abdoulaye, Cissé Yakhya, Faye Mohameth, Diouf Cheikh, Ba Momar Codé, & Badiane Seydou Boubacar. (2022). Multiple Spontaneous Intracerebral Hematomas in a Child with Hemophilia and Sickle Cell Trait: A Case Report. HEALTH SCIENCES AND DISEASE, 23(10). https://doi.org/10.5281/hsd.v23i10.3952
Download Citation
Endnote/Zotero/Mendeley (RIS)
BibTeX

References

  1. Kim JB, Jung H-J, Im KS, Han SW, Lee S-H. Urgent craniectomy for subdural hematoma in the 8-month-old infant with unrecognized hemophilia B. Korean J Anesthesiol. janv 2013;64(1):82‑3.
  2. Ghosh K, Nair AP, Jijina F, Madkaikar M, Shetty S, Mohanty D. Intracranial haemorrhage in severe haemophilia: prevalence and outcome in a developing country. Haemophilia. sept 2005;11(5):459‑62.
  3. Gyanesh P, Dhiraaj S. Anesthetic management of a patient with hemophilia A with spontaneous acute subdural hematoma. Journal of Anaesthesiology, Clinical Pharmacology. mars 2013;29(1):117.
  4. Srivastava A, Santagostino E, Dougall A, Kitchen S, Sutherland M, Pipe SW, et al. WFH Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia 26,1-158, 2020
  5. Klinge J, Auberger K, Auerswald G, Brackmann HH, Mauz-Körholz C, Kreuz W. Prevalence and outcome of intracranial haemorrhage in haemophiliacs–a survey of the paediatric group of the German Society of Thrombosis and Haemostasis (GTH). European journal of pediatrics. 1999;158(3): S162–S165.
  6. Cabon I, Hladky JP, Lambillotte A, Cotton A, Dhellemmes P. Uncommon etiology of extradural hematoma. Neurochirurgie 1997; 43:173—6.
  7. Kalala Okito JP, Van damme O, Calliauw L. Are spontaneous epidural haemotomas a rare complication in sickle cell disease? A report of two new cases. Acta Neurochir (Wien) 2004;146: 407—10.
  8. Martinowitz U, Heim M, Tadmor R, Eldor A, Rider I, Findler G, et al. Intracranial Hemorrhage in Patients with Hemophilia. Neurosurgery. 1 mai 1986;18(5):538‑41.
  9. H. Hafidi *, A. Basar, J. Dellamonica, R. Domergue, J. Levraut Acute spontaneous subdural haematoma in a 10-year-old sickle cell disease patient
  10. Hu Q, Zhang A, Liu A, Wang S, Wang Y, Zhang L. A Retrospective Analysis of Intracranial Hemorrhage in Children with Hemophilia A. CURR MED SCI. 1 oct 2018;38(5):875‑9.
  11. Baleh A, Getachew K, Mekonnen A. TRAUMATIC RETROCLIVAL SUBDURAL HEMATOMA IN A CHILD. World Neurosurgery. 1 sept 2019;133.
  12. Darby SC, Kan SW, Spooner RJ, Giangrande PL, Hill FG, Hay CR, et al. Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV. Blood. 2007 ;110(3) :815–825.
  13. Muhammad J. Azhar hémorragie extradurale : une colmplication rare et une manifestation d'AVC dans la drépanocytose
  14. Maataoui H, Fahi A, Oukkache B. Sickle cell trait and haemophilia: A rare association. Pan African Medical Journal. 30 janv 2018;29.
  15. Glenn LD, Lovely RM, Goldsmith JC. Combined sickle cell anemia and mild hemophilia A: successful treatment of hemorrhage with DDAVP. Am J Hematol. mai 1991;37(1):64.
  16. Kumar M, Herring R. Rare combination of homozygous sickle cell disease (Hb SS) and haemophilia B in a paediatric patient. British journal of haematology. 1 oct 1997; 98:780‑1.
  17. Ahmed SG, Ibrahim UA, Kagu MB, Abjah UA. Does sickle cell trait reduce the frequency of spontaneous bleeds in severe haemophilia? The Journal of Haemophilia Practice. 2016;3(2):50–54.
  18. Bullock MR, Chesnut R, Ghajar J, Gordon D, Hartl R, Newell DW, et al. Surgical management of acute subdural hematomas. Neurosurgery. mars 2006;58(3 Suppl): S16-24; discussion Si-iv.