Stroke Risk in Children with Sickle Cell Disease–TCDi
Date of SC final approval
About the Measure
Domain:
Measure:
Definition:
Purpose:
Sickle Cell Disease – Neurology, Quality of Life, and Health Services
Stroke Risk in Children with Sickle Cell Disease
A clinical test to measure the blood flow velocities in intracranial arteries. Elevated
intracranial arterial blood flow rates are indicative of expanding vessel lesions and a
subsequent increased risk of stroke.
This measure is used to stratify stroke risk in children to determine eligibility for
prophylactic blood transfusion for stroke prevention.
About the Protocol
Description of
Protocol:
Selection
Rationale:
Specific
Instructions:
Protocol Text:
Imaging Transcranial Doppler (TCDi) ultrasonography is an imaging technique that
measures the blood flow velocity in large intracranial arteries. This TCDi protocol covers
how to identify relevant intracranial landmarks and how to record and score TCDi
results.
Transcranial Doppler (TCD) ultrasound is a valid and reliable method that has been
shown in clinical trials to select children for prophylactic blood transfusion. In contrast to
traditional TCD, imaging TCD allows direct visualization of the arteries under
examination.
Results from Imaging Transcranial Doppler (TCDi) ultrasound should be interpreted by a
medical doctor with expertise in TCDi in sickle cell disease (SCD).
Imaging Transcranial Doppler Ultrasonography
Identifying Intracranial Landmarks and Major Cerebral Arteries
Krejza et al. (2000) provide standard parameters for the visualization of the anterior,
middle, and posterior cerebral arteries through the temporal acoustic window in the thin
temporal region of the skull.
Recording Imaging Transcranial Doppler Ultrasonography Results
Examiners should record the highest time-averaged mean blood-flow velocity using a 3millimeter sample volume placed at the point of highest velocity as determined by color
aliasing artifacts in the following arteries:
 middle cerebral artery,
 distal internal carotid artery,
 anterior and posterior cerebral arteries, and
 basilar artery.
Angle-corrected mean velocities can be obtained by automatic or manual tracing of the
Doppler waveform. Uncorrected flow velocities are calculated for each artery as the
product of angle-corrected velocities and the cosine of the recorded angle of insonation
based on the Doppler equation.
Scoring of Uncorrected Flow Velocities
Normal: blood velocities less than 165 centimeters per second in all arteries.
Conditional: blood velocities greater than 165 centimeters per second but less than 200
centimeters per second in all arteries.
Abnormal: blood velocity of 200 centimeters per second or greater in either the internal
carotid artery or the middle cerebral artery.
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Stroke Risk in Children with Sickle Cell Disease–TCDi
Participant:
Source:
Language of
Source:
Personnel and
Training
Required:
Equipment
Needs:
Protocol Type:
Date of SC final approval
Children and adolescents aged 2 years and older with hemoglobin SS or beta-zero
thalassemia disease and without a history of clinical stroke.
Identifying Intracranial Landmarks and Major Cerebral Arteries
Krejza, J., Mariak, Z., Melhem, E. R., & Bert, R. J. (2000). A guide to the identification of
major cerebral arteries with Transcranial Color Doppler Sonography. American Journal
of Roentgenology, 174, 1297–1303.
Recording and Scoring Results
Arkuszewski, M., Krejza, J., Chen, R., Kwiatkowski, J. L., Ichord, R., Zimmerman, R.,
Ohene-Frempong, K., & Melhem, E.R. (2012). Sickle cell disease in children: Accuracy
of imaging transcranial Doppler ultrasonography in detection of intracranial arterial
stenosis. The Neuroradiology Journal, 25(4), 402–410.
English
Imaging Transcranial Doppler (TCDi) ultrasonography should be administered by trained
ultrasonographers and interpreted by clinicians (e.g., radiologists, neurologists,
hematologists) with expertise in TCDi in children with sickle cell disease (SCD).
2 megahertz Doppler ultrasonogram with a 1.8 to 3.6 megahertz transducer.
Clinical examination and physical measurement.
Requirements:
Requirements Category
Common Data
Elements:
General
References:
Required (Yes/No):
Major equipment
Yes
Specialized training
Yes
Specialized requirements for biospecimen
collection
Average time of greater than 15 minutes in an
unaffected individual
TBD by PhenX Staff
No
Yes
Adams, R. J., Brambilla, D., & Optimizing Primary Stroke Prevention in Sickle Cell
Anemia (STOP 2) Trial Investigators. (2005). Discontinuing prophylactic transfusions
used to prevent stroke in sickle cell disease. New England Journal of Medicine 353(26),
2769–2778.
Adams, R. J., McKie, V. C., Brambilla, D., Carl, E., Gallagher, D., Nichols, F. T., Roach,
S., Abboud, M., Berman, B., Driscoll, C., Files, B., Hsu, L., Hurlet, A., Miller, S., Olivieri,
N., Pegelow, C., Scher, C., Vichinsky, E., Wang, W., Woods, G., Kutlar, A., Wright, E.,
Hagner, S., Tighe, F., Lewin, J., Cure, J., Zimmerman, R. A., & Waclawiw, M. A. (1998).
Stroke prevention trial in sickle cell anemia. Controlled Clinical Trials 19(1), 110–129.
Adams, R.J., McKie, V.C., Hsu, L., Files, B., Vichinsky, E., Pegelow, C., Abboud, M.,
Gallagher, D., Kutlar, A., Nichols, F.T., Bonds, D.R., & Brambilla, D. (1998). Prevention
of a first stroke by transfusions in children with sickle cell anemia and abnormal results
on transcranial Doppler ultrasonography. New England Journal of Medicine, 339(1), 5–
11.
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Stroke Risk in Children with Sickle Cell Disease–TCDi
Date of SC final approval
Jones, A., Granger, S., Brambilla, D., Gallagher, D., Vichinsky, E., Woods, G., Berman,
B., Roach, S., Nichols, F., Adams, R. J. (2005). Can peak systolic velocities be used for
prediction of stroke in sickle cell anemia? Pediatric Radiology, 35(1), 66–72.
Krejza, J., Chen, R., Romanowicz, G., Kwiatkowski, J.L., Ichord, R., Arkuszewski, M.,
Zimmerman, R., Ohene-Frempong, K., Desiderio, L., & Melhem, E. R. (2011). Sickle cell
disease and transcranial Doppler imaging: Inter-hemispheric differences in blood flow
Doppler parameters. Stroke, 42(1), 81–86.
Pavlakis, S. G., Rees, R. C., Huang, X., Brown, R. C., Casella, J. F., Iyer, R. V.,
Kalpatthi, R., Luden, J., Miller, S. T., Rogers, Z. R., Thornburg, C. D., Wang, W. C. &
Adams, R. J. (2010). Transcranial doppler ultrasonography (TCD) in infants with sickle
cell anemia: Baseline data from the BABY HUG trial. Pediatric Blood Cancer, 54, 256–
259.
Platt, O. S. (2005). Preventing stroke in sickle cell anemia. New England Journal of
Medicine, 353(26), 2743–2745.
Additional Information About the Measure
Essential Data:
Current Age
Related PhenX
Measures:
Derived Variables:
Keywords/Related
Concepts:
Brain Morphology, Arterial Blood Supply
None
Stroke, Stroke risk, Blood velocity, Transcranial Doppler ultrasonography, TCD,
Imaging Transcranial Doppler ultrasonography, TCDi, Brain, Blood flow
Version 10 – 10/21/09