Moyamoya Disease, a Rare Cause of Recurrent Strokes in an African Sickle Cell Child: Does hydroxyurea have a Role in this Context?


  • Abel Makubi
  • D. Soka
  • J. Makani



Key words. Moyamoya Stroke Sickle Cell Child



Background: Neurological complications are a significant cause of morbidity and mortality in sickle cell patients with reported incidence of stroke in Africa as high as 1·3/100 patient per year(1, 2) . There is an association between sickle cell disease (as well as other hemoglobinopathies) and Moyamoya disease(3). Data on the occurrence of this condition in African sickle patient are scare. Likewise the role of hydroxyurea among patients with both sickle cell anemia and Moyamoya disease in preventing stroke has not yet been studied in Africa.

Case presentation: In the present report, we describe an African child who had a recurrent stroke. She was later diagnosed as having Moyamoya disease while already receiving hydroxyurea.

Conclusion: Moyamoya disease is a rare condition associated with recurrent stroke in African sickle children. The role of hydroxyurea in this context is still unclear.


[1] Izuora GI, Kaine WN, Emodi I. Neurological disorders in Nigerian children with homozygous sickle cell anaemia. East Afr Med J 1989; 66: 653-57.
[2] Amayo EO, Owade JN, Aluoch JR, Njeru EK. Neurological complications of sickle cell anaemia at KNH: a five year retrospective study. East Afr Med J 1992; 69: 660-62.
[3] Fryer RH, Chiriboga CA, Feldstein NA. Sickle cell anemia with moyamoya disease. outcome after EDAS procedure. Paediat Neurol 2003; 29: 124-30.
[4] Smith ER, McClain CD, Heeney M, Scott RM. Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome. Neurosurg Focus 2009; 26(4): E10.
[5] Kawamura K, Cheng Y, Kawamura N, et al. Moyamoya disease In Handbook of Clinical Neurology. Vol 2. Amsterdam, The Netherlands: Elsevier 1989; pp. 293-306.
[6] Kikuta K, Miyamoto S, Kataoka H, et al. An adult case of moyamoya syndrome that developed dural sinus thrombosis associated with protein C deficiency: a case report and literature review. Surg Neurol 2005; 63: 480-84.
[7] Dobson SR, Holden KR, Nietert PJ, Cure JK, Laver JH, Abboud MR. Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events. Blood 2002; 99(9): 3144-50.
[8] Sumoza A, Renate B, Sumoza D, Fairbank V. Hydroxyurea (HU) for Prevention of Recurrent Stroke in Sickle Cell Anemia. Am J Hematol 2002; 71: 161-65.
[9] Greenway A, Russell E, Courtney DT. Long-term results using hydroxyurea/phlebotomy for reducing secondary stroke risk in children with sickle cell anemia and iron overload. Am J Hematol 2011; 86: 357-61.
[10] Ware RE, Zimmerman SA, Sylvestre PB. Prevention of secondary stroke and resolution of transfusional iron overload in children with sickle cell anemia using hydroxyurea and phlebotomy. J Pediatr 2004; 145: 346-52.
[11] Ware RE, Helms RW. Stroke With Transfusions Changing to Hydroxyurea (SWiTCH). Blood 2012; 119: 3925-32.
[12] Uchino K, Johnston SC, Becker KJ, Tirschwell DL. Moyamoya disease in Washington State and California. Neurology 2005; 65: 956-58.
[13] Chiu D, Shedden P, Bratina P, Grotta JC. Clinical features of moyamoya disease in the United States. Stroke 1998; 29: 1347-51.
[14] Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 2008; 79: 900-4.
[15] Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995; 332(20): 1317-22.
[16] Ware RE, Steinberg MH, Kinney TR. Hydroxyurea: an alternative to transfusion therapy for stroke in sickle cell anemia. Am J Hematol 1995; 50(2): 140-3.
[17] Pavlakis SG, ProhovnikI PS, DeVivo DC. Neurologic complications of sickle cell disease. Adv Pediatr 1989: 36: 247-76.
[18] Wang SC, Hong JH, Hsueh C, Chiang CS. High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease. J Pediatr 1991; 118: 377-82.






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