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Brain Imaging Findings in Pediatric Patients with Sickle Cell Disease¹

¹ From the Depts of Diagnostic Imaging (R.G.S., T.E., G.M.H., K.J.H.), Hematology (W.C.W.), and Biostatistics (X.X.), St Jude Children’s Research Hosp, 332 N Lauderdale St, Memphis, TN 38105-2794; and Depts of Pediatrics (R.G.S., L.W.W., W.C.W.) and Radiology (R.G.S., K.J.H.), Univ of Tennessee School of Medicine, Memphis. Received Jul 29, 2002; revision requested Sep 20; final revision received Nov 25; accepted Jan 14, 2003. Supported by American Lebanese Syrian Associated Charities. R.G.S. supported by grant RO1 HL60022 from the NHLBI. Address correspondence to R.G.S. (e-mail: grant.steen@stjude.org).

PURPOSE: To determine prevalence of imaging abnormalities in the brain of children with sickle cell disease (SCD) and to identify clinical and methodological factors that influence prevalence estimate.

MATERIALS AND METHODS: Magnetic resonance (MR) imaging and MR angiographic findings for 185 patients with SCD examined at St Jude Children’s Research Hospital since 1993 were reviewed. At least two readers independently reviewed images. Standard MR imaging criteria were used to identify lacunae, loss of white matter volume, encephalomalacia, or leukoencephalopathy. Patients were assigned grades to indicate limited or extensive abnormalities. Standard MR angiographic criteria were used to identify arterial tortuosity (limited vasculopathy) and stenosis or occlusion (extensive vasculopathy). Findings were evaluated as a function of patient clinical status (including stroke) and diagnosis. Recent methods (T1- and T2-weighted MR imaging plus fluid-attenuated inversion recovery [FLAIR] at 3-mm section thickness) were compared with older methods (T1- and T2-weighted MR imaging without FLAIR at 5-mm section thickness).

RESULTS: At mean age of 10 years, overall prevalence of infarction, ischemia, or atrophy in patients with SCD was 44% (82 of 185), and prevalence of vasculopathy was 55% (102 of 185), without evidence of a significant referral bias. Twenty-six of 27 patients with clinical stroke had abnormal findings at imaging, but even if patients with stroke were excluded, 35% (56 of 158) had a "silent infarction" (MR imaging–visible injury without clinical stroke), and 49% (78 of 158) had abnormal findings at MR angiography. Patients with clinically severe disease had more abnormalities at MR imaging (P < .001) and MR angiography (P < .004) than did patients with milder disease. Severe vasculopathy was more prevalent in patients with hemoglobin SS than in those with hemoglobin SC (P < .001). Recent imaging methods showed more abnormalities than did older methods (P < .01). With newer methods, 43% (29 of 67) of patients had extensive abnormalities, whereas with older methods, 28% (33 of 116) had extensive abnormalities.

CONCLUSION: Prevalence of ischemic brain injury in pediatric patients with SCD is substantially higher than was previously reported, in part because of improvements in imaging methods.

© RSNA, 2003

Index terms: Brain, infarction, 10.78 • Cerebral angiography, 17.12142 • Cerebral blood vessels, 17.651 • Children, central nervous system, 10.651, 17.651 • Magnetic resonance (MR), vascular studies, 17.12142 • Sickle cell disease (SS, SC), 10.651, 17.651

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