Abstract
Arterial ischaemic stroke (AIS) affects at least 10 % of children with sickle cell disease (SCD) by 20 years of age and has a >60 % recurrence rate without intervention. Up to 25 % of people with HbSS phenotype have ischaemic brain injury on MRI – so called “silent infarcts” that, nonetheless, have adverse effects on cognition. Many risk factors are implicated in AIS associated with SCD, including cerebral arteriopathy, anaemia, chronic hypoxaemia, prior chest crisis and high white cell count. Obstructive airways disease (from big tonsils and adenoids), infections, patent foramen ovale and relative hypertension are modifiable risk factors associated with AIS in children with SCD - immunization and penicillin prophylaxis has decreased the prevalence of bacterial meningitis with subsequent stroke. This case provides an illustration of these points in addition to a discussion of treatment strategies.
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References
Roach ES, Golomb MR, Adams R, et al. Management of stroke in infants and children: a scientific statement from a special writing group of American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke. 2008;39:2644–91.
Jordan LC, Casella JF, DeBaun MR. Prospects for primary stroke prevention in children with sickle cell anaemia. Br J Haematol. 2012;157:14–25.
Ware RE, Schultz WH, Yovetich N, et al. Stroke with Transfusions Changing to Hydroxyurea (SWiTCH): a phase III randomised controlled trial for treatment of children with sickle cell anemia, stroke, and iron overload. Pediatr Blood Cancer. 2011;57:1011–7.
Dick M for the UK Forum on Haemoglobin Disorders. Sickle cell disease in childhood: standards and guidelines for clinical care. NHS Sickle Cell and Thalassaemia Screening Programme in partnership with the Sickle Cell Society. London. October 2012.
Hsieh MM, Fitzhugh CD, Tisdale JF, et al. Allogeneic heamtopoietic stem cell transplantation for sickle cell disease: the time is now. Blood. 2011;118:1197–207.
Smith ER, McClain CD, Heeney M, et al. Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome. Neurosurg Focus. 2009;26(4):E10.
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© 2015 Springer-Verlag London
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Niotakis, G., Ganesan, V. (2015). Sickle Cell Disease and Stroke. In: Gill, S., Brown, M., Robertson, F., Losseff, N. (eds) Stroke Medicine. Springer, London. https://doi.org/10.1007/978-1-4471-6705-1_10
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DOI: https://doi.org/10.1007/978-1-4471-6705-1_10
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