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Prospective Brain Imaging Evaluation of Children with Sickle Cell Trait: Initial Observations¹

¹ From the Departments of Diagnostic Imaging (R.G.S., G.M.H., K.B., J.W.L., K.J.H.), Epidemiology and Biostatistics (X.X.), and Hematology (W.C.W.), St Jude Children’s Research Hospital, 332 N Lauderdale St, Memphis, TN 38105-2794; and Departments of Pediatrics (R.G.S., W.C.W.) and Radiology (R.G.S, J.W.L., K.J.H.), University of Tennessee School of Medicine, Memphis. Received May 20, 2002; revision requested July 16; final revision received October 9; accepted November 5. Supported by the American Lebanese Syrian Associated Charities (ALSAC). R.G.S. supported by grant HL 60022 from the National Institutes of Health. Address correspondence to R.G.S. (e-mail: grant.steen@stjude.org).

View larger version (133K): [in a new window]   Figure 1a. Coronal MR angiograms (34/5; field of view, 20 cm; flip angle, 20°; matrix, 192 x 256) show how measurements were made to establish whether a child had arterial tortuosity. (a) Image shows characterization of basilar artery bowing by means of drawing the basilar chord (left dotted line) from the point of junction of the vertebral arteries to the point of bifurcation of the posterior cerebral arteries. Then a perpendicular distance (between the lines at the arrows) was measured from the basilar chord to a line drawn down the center of the basilar artery (right dotted line) at a point where the artery was farthest from the basilar chord. (b) Image shows characterization of middle cerebral artery bowing by means of locating points (dots) at the confluence of the A1 segment of the anterior cerebral artery and the M1 segment of the middle cerebral artery on each side of the brain, so that a line of reference (upper line) could be drawn through these points. This compensates for head tilt, which could otherwise corrupt the measurement, in the subject. Then the perpendicular distance was measured from the line of reference to the midpoint of the artery (at the point of farthest bowing) of the A1 segment (right arrows) and M1 segment (left arrows) on both sides of the circulation.

View larger version (84K): [in a new window]   Figure 1b. Coronal MR angiograms (34/5; field of view, 20 cm; flip angle, 20°; matrix, 192 x 256) show how measurements were made to establish whether a child had arterial tortuosity. (a) Image shows characterization of basilar artery bowing by means of drawing the basilar chord (left dotted line) from the point of junction of the vertebral arteries to the point of bifurcation of the posterior cerebral arteries. Then a perpendicular distance (between the lines at the arrows) was measured from the basilar chord to a line drawn down the center of the basilar artery (right dotted line) at a point where the artery was farthest from the basilar chord. (b) Image shows characterization of middle cerebral artery bowing by means of locating points (dots) at the confluence of the A1 segment of the anterior cerebral artery and the M1 segment of the middle cerebral artery on each side of the brain, so that a line of reference (upper line) could be drawn through these points. This compensates for head tilt, which could otherwise corrupt the measurement, in the subject. Then the perpendicular distance was measured from the line of reference to the midpoint of the artery (at the point of farthest bowing) of the A1 segment (right arrows) and M1 segment (left arrows) on both sides of the circulation.

View larger version (147K): [in a new window]   Figure 2a. Transverse T2-weighted turbo spin-echo MR images (3,500/17, 102; field of view, 23 cm; matrix, 190 x 256; sections, 19), which are more sensitive to abnormal signal intensity in the brain parenchyma than are T1-weighted images. (a) Image shows periventricular leukoencephalopathy (arrows) in white matter in the centrum semiovale of a 16-year-old boy with hemoglobin AS. (b) Image shows leukoencephalopathy (arrows) in white matter of a second 16-year-old boy with hemoglobin AS.

View larger version (142K): [in a new window]   Figure 2b. Transverse T2-weighted turbo spin-echo MR images (3,500/17, 102; field of view, 23 cm; matrix, 190 x 256; sections, 19), which are more sensitive to abnormal signal intensity in the brain parenchyma than are T1-weighted images. (a) Image shows periventricular leukoencephalopathy (arrows) in white matter in the centrum semiovale of a 16-year-old boy with hemoglobin AS. (b) Image shows leukoencephalopathy (arrows) in white matter of a second 16-year-old boy with hemoglobin AS.

View larger version (159K): [in a new window]   Figure 3a. Time-of-flight MR angiograms (34/5; field of view, 20 cm; flip angle, 20°; matrix, 192 x 256) obtained in two boys with hemoglobin AS. (a) Transverse image obtained in a 10-year-old boy. This view is through a 64-mm-thick slab of tissue; stationary protons in the brain parenchyma are nulled out, but moving protons in the blood have high signal intensity. (b) Transverse image obtained in an 11-year-old boy. There is an unusually high degree of signal intensity in the distal vessels (arrows), and this degree of conspicuity suggests very rapid blood flow (30). (c) Coronal image of the middle cerebral arteries obtained in the child in a shows tortuosity (arrow) of the A1 segment. (d) Coronal image of the middle cerebral artery obtained in the child in b shows severe tortuosity (arrow) of the A1 segment on the left side of the patient.

View larger version (158K): [in a new window]   Figure 3b. Time-of-flight MR angiograms (34/5; field of view, 20 cm; flip angle, 20°; matrix, 192 x 256) obtained in two boys with hemoglobin AS. (a) Transverse image obtained in a 10-year-old boy. This view is through a 64-mm-thick slab of tissue; stationary protons in the brain parenchyma are nulled out, but moving protons in the blood have high signal intensity. (b) Transverse image obtained in an 11-year-old boy. There is an unusually high degree of signal intensity in the distal vessels (arrows), and this degree of conspicuity suggests very rapid blood flow (30). (c) Coronal image of the middle cerebral arteries obtained in the child in a shows tortuosity (arrow) of the A1 segment. (d) Coronal image of the middle cerebral artery obtained in the child in b shows severe tortuosity (arrow) of the A1 segment on the left side of the patient.

View larger version (80K): [in a new window]   Figure 3c. Time-of-flight MR angiograms (34/5; field of view, 20 cm; flip angle, 20°; matrix, 192 x 256) obtained in two boys with hemoglobin AS. (a) Transverse image obtained in a 10-year-old boy. This view is through a 64-mm-thick slab of tissue; stationary protons in the brain parenchyma are nulled out, but moving protons in the blood have high signal intensity. (b) Transverse image obtained in an 11-year-old boy. There is an unusually high degree of signal intensity in the distal vessels (arrows), and this degree of conspicuity suggests very rapid blood flow (30). (c) Coronal image of the middle cerebral arteries obtained in the child in a shows tortuosity (arrow) of the A1 segment. (d) Coronal image of the middle cerebral artery obtained in the child in b shows severe tortuosity (arrow) of the A1 segment on the left side of the patient.

View larger version (96K): [in a new window]   Figure 3d. Time-of-flight MR angiograms (34/5; field of view, 20 cm; flip angle, 20°; matrix, 192 x 256) obtained in two boys with hemoglobin AS. (a) Transverse image obtained in a 10-year-old boy. This view is through a 64-mm-thick slab of tissue; stationary protons in the brain parenchyma are nulled out, but moving protons in the blood have high signal intensity. (b) Transverse image obtained in an 11-year-old boy. There is an unusually high degree of signal intensity in the distal vessels (arrows), and this degree of conspicuity suggests very rapid blood flow (30). (c) Coronal image of the middle cerebral arteries obtained in the child in a shows tortuosity (arrow) of the A1 segment. (d) Coronal image of the middle cerebral artery obtained in the child in b shows severe tortuosity (arrow) of the A1 segment on the left side of the patient.

View larger version (84K): [in a new window]   Figure 4a. Coronal time-of-flight MR angiograms (34/5; field of view, 20 cm; flip angle, 20°; matrix, 192 x 256) obtained in two girls with hemoglobin AS show basilar artery. (a) Image shows mild tortuosity (arrow) in a 12-year-old girl. (b) Image shows severe tortuosity (arrow) in a 15-year-old girl.

View larger version (129K): [in a new window]   Figure 4b. Coronal time-of-flight MR angiograms (34/5; field of view, 20 cm; flip angle, 20°; matrix, 192 x 256) obtained in two girls with hemoglobin AS show basilar artery. (a) Image shows mild tortuosity (arrow) in a 12-year-old girl. (b) Image shows severe tortuosity (arrow) in a 15-year-old girl.