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Steen RG, Langston JW, Ogg RJ, et al. Ectasia of the basilar artery in children with sickle cell disease: relationship to hematocrit and psychometric measures. J Stroke Cerebrovasc Dis. 1998;7(1):32-43doi: 10.1016/s1052-3057(98)80019-0.
Steen, R. G., Langston, J. W., Ogg, R. J., Manci, E., Mulhern, R. K., & Wang, W. (1998). Ectasia of the basilar artery in children with sickle cell disease: relationship to hematocrit and psychometric measures. Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association, 7(1), 32-43. https://doi.org/10.1016/s1052-3057(98)80019-0
Steen, R G, et al. "Ectasia of the basilar artery in children with sickle cell disease: relationship to hematocrit and psychometric measures." Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association vol. 7,1 (1998): 32-43. doi: https://doi.org/10.1016/s1052-3057(98)80019-0
Steen RG, Langston JW, Ogg RJ, Manci E, Mulhern RK, Wang W. Ectasia of the basilar artery in children with sickle cell disease: relationship to hematocrit and psychometric measures. J Stroke Cerebrovasc Dis. 1998 Jan-Feb;7(1):32-43. doi: 10.1016/s1052-3057(98)80019-0. PMID: 17895054.
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J Stroke Cerebrovasc Dis. 1998 Jan-Feb;7(1):32-43. doi: 10.1016/s1052-3057(98)80019-0.

Ectasia of the basilar artery in children with sickle cell disease: relationship to hematocrit and psychometric measures.

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association

R G Steen, J W Langston, R J Ogg, E Manci, R K Mulhern, W Wang

Affiliations

  1. Department of Diagnostic Imaging, St Jude Children's Research Hospital, Memphis, TN, USA.

PMID: 17895054 DOI: 10.1016/s1052-3057(98)80019-0

Abstract

GOAL: To determine whether children with sickle cell disease (SCD), but without clinical evidence of cerebrovascular disease, have vasculopathy shown by quantitative magnetic resonance angiography (MRA).

METHODS: In a retrospective review of MRA films, we compared 47 SCD patients with 49 control patients. Time-of-flight three-dimensional T1-weighted gradient-echo images were reconstructed, by maximum-intensity projection, to show the basilar artery in coronal view, and basilar volume was calculated from measurements made on films. Basilar volume was correlated with hematocrit and with results of cognitive testing.

FINDINGS: Mean basilar artery volume was 74% larger in SCD patients than in controls (P<.001). If the upper limit of normal is defined as mean adult volume +2 SD (< or =427 mm(3)), 2% (1 of 43) of controls but 37% (17 of 46) of SCD patients exceed this value (chi(2)=19.0; P<.001). Basilar volume correlated inversely with hematocrit (r=-.60; P<.0001), with full-scale IQ (r=-.62; P<.005), and with freedom from distractability (r=-.61; P<.006) in SCD patients. Analysis of basilar artery tissue from a 5-year-old SCD patient showed that basilar dilatation can be associated with pathological changes typical of hypertension.

CONCLUSIONS: Approximately 37% of a heterogenous group of pediatric SCD patients had ectasia of the basilar artery. Quantitative MRA is sensitive to subtle vasculopathy that can go undetected in the qualitative analysis more commonly done. Data suggest that there is a substantial elevation of arteriolar blood volume in pediatric SCD patients, and that such patients may share disease features in common with adult hypertension.

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