Protocol - Stroke Risk in Children with Sickle Cell Disease - TCDi

Description

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.

Specific Instructions

Results from Imaging Transcranial Doppler (TCDi) ultrasound should be interpreted by a medical doctor with expertise in TCDi in sickle cell disease (SCD). Additionally, the TCD results should be compared with published guidelines to determine if the subject should be receiving prophylactic red cell transfusion or require more frequent TCD or other monitoring.

The Working Group does not recommend angle correction of TCDi blood-flow velocities. Cut-points for blood flow velocities are currently based on uncorrected velocities, and new parameters need to be established for angle-corrected velocities. Instead of angle correction, investigators should optimize the velocities with various maneuvers to get the best angle by transducer position.

The Working Group notes that blood-flow velocities indicative of stroke risk can be obtained by either Transcranial Doppler (TCD) ultrasound or Imaging Transcranial Doppler (TCDi) ultrasonography. The choice of TCD or TCDi depends on the expertise and equipment available to investigators.

The Sickle Cell Disease Curative Therapy Working Group recommends that imaging transcranial doppler (TCDi) ultrasonography be performed in sickle cell patients undergoing hematopoietic cell transplant at one time point pre-transplant. Additionally, investigators should indicate whether the results from TCDi were the indication for transplant.

Availability

Available

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Protocol

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 3-millimeter 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.

Personnel and Training Required

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).

Equipment Needs

2 megahertz Doppler ultrasonogram with a 1.8 to 3.6 megahertz transducer.

Requirements

Requirement Category	Required
Major equipment	Yes
Specialized training	Yes
Specialized requirements for biospecimen collection	No
Average time of greater than 15 minutes in an unaffected individual	Yes

Mode of Administration

Clinical examination and physical measurement

Lifestage

Toddler, Child, Adolescent

Participants

Children and adolescents aged 2 years and older with hemoglobin SS or beta-zero thalassemia disease and without a history of clinical stroke.

Selection Rationale

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.

Language

English

Standards

Standard	Name	ID	Source
Human Phenotype Ontology	Sickle Cell Anemia	ORPHA:232	HPO
Human Phenotype Ontology	Anemia	OMIM:603903	HPO
caDSR Form	PhenX PX821002 - Stroke Risk In Children With Sickle Cell Disease Tcdi	6254567	caDSR Form

Derived Variables

None

Process and Review

Not applicable.

Protocol Name from Source

Imaging Transcranial Doppler (TCDi) ultrasonography

Source

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.

General References

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.

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.

Protocol ID

821002

Variables

Export Variables

Variable Name	Variable ID	Variable Description	dbGaP Mapping
PX821002_StrokeRiskChildrenWithSickleCellDiseaseTCDi_Angle_Corrected_MeanVelocities_Automatic
	PX821002060000	Angle-corrected mean velocities by automatic more tracing of the Doppler waveform. show less	N/A
PX821002_StrokeRiskChildrenWithSickleCellDiseaseTCDi_Angle_Corrected_MeanVelocities_Manual
	PX821002070000	Angle-corrected mean velocities by manual more tracing of the Doppler waveform. show less	N/A
PX821002_StrokeRiskChildrenWithSickleCellDiseaseTCDi_Anterior_Cerebral_Artery
	PX821002030000	Highest time-averaged mean blood-flow more velocity in the anterior cerebral artery show less	N/A
PX821002_StrokeRiskChildrenWithSickleCellDiseaseTCDi_Basilar_Artery
	PX821002050000	Highest time-averaged mean blood-flow more velocity in the basilar artery show less	N/A
PX821002_StrokeRiskChildrenWithSickleCellDiseaseTCDi_Distal_Internal_Carotid_Artery
	PX821002020000	Highest time-averaged mean blood-flow more velocity in the distal internal carotid artery show less	N/A
PX821002_StrokeRiskChildrenWithSickleCellDiseaseTCDi_Middle_Cerebral_Artery
	PX821002010000	Highest time-averaged mean blood-flow more velocity in the middle cerebral artery. show less	N/A
PX821002_StrokeRiskChildrenWithSickleCellDiseaseTCDi_Posterior_Cerebral_Artery
	PX821002040000	Highest time-averaged mean blood-flow more velocity in the posterior cerebral artery show less	N/A

SCD Neurology, Quality of Life, and Health Services

Measure Name

Stroke Risk in Children with Sickle Cell Disease

Release Date

July 30, 2015

Definition

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.

Purpose

This measure is used to stratify stroke risk in children to determine eligibility for prophylactic blood transfusion for stroke prevention.

Keywords

stroke, Stroke risk, Blood velocity, Transcranial Doppler ultrasonography, TCD, Imaging Transcranial Doppler ultrasonography, TCDi, Brain, Blood flow, "Neurology, quality of life, and Health Services"

Measure Protocols

Protocol ID	Protocol Name
821001	Stroke Risk in Children with Sickle Cell Disease - TCD
821002	Stroke Risk in Children with Sickle Cell Disease - TCDi

Publications

There are no publications listed for this protocol.