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CHAPTER 47 Doppler Sonography of the Brain in Children 1607

Left ACA

A

B

FIG. 47.14 Anterior Cerebral Artery (ACA) Stenosis in 11-Year-Old Child With Sickle Cell Disease. (A) TCD sonogram demonstrates

abnormally elevated left ACA velocities. (B) Three-dimensional time-of-light MR angiogram demonstrates stenosis at the origin of the A1 segment

of the left ACA (arrow).

When assigning the risk of stroke based on TCD velocities,

it is important to consider how velocities may difer with the

overall health of the patient, patient ethnicity, and the speciic

equipment used compared to those obtained with nonduplex

equipment in the initial STOP studies. When ill, comorbidities

including hypoxia, fever, hypoglycemia, and worsening anemia

can increase the cerebral blood low velocities, making the results

invalid if conditional or abnormal. 56 In these cases, repeating

the study when the patient is no longer ill will help verify the

results. Because diferent ethnicities and haplotypes of the disease

have diferent average velocities, risk of stroke, and prognosis,

it is possible that diferent criteria have to be determined to

assess risk for a speciic ethnicity. 87

Regarding the equipment used, studies have evaluated the

diferences in nonimaging TCD versus imaging TCD techniques.

In studies performed in the early 2000s, nonangle-corrected

velocities obtained with Acuson and ATL TCDI equipment were

approximately 10% lower in the MCA than those obtained with

Nicolet (Vascular, Madison, WI) nonimaging equipment. 20,21

Later studies showed no signiicant diference in TAMM velocity

measurements obtained with General Electric TCDI equipment

compared with Nicolet nonimaging equipment. 22,23 he reasons

for these difering results are likely multifactorial. Early imaging

probes were bulkier, making it more diicult to optimize Doppler

tracings. Padayachee et al. 88 discussed how the TAMM velocity

is quantiied using the Doppler equation. Optimizing the waveform

without angle correction can result in similar results

compared to the nonimaging TCD technique negating the need

for decreasing the threshold for TCDI cutofs. 88 A 10% lower

cutof point for TAMM velocities has been suggested depending

on the protocol and machine used. 13 Besides appropriate curser

placement, instrument settings (volume size, gain, waveform

display) should be optimized. Close attention to technique can

reduce the diferences between velocity data acquired with

diferent ultrasound machines. Centers should be aware of these

potential diferences and perform their own comparison studies

when using various imaging equipment before changing velocity

thresholds for TCDI. 88

Although angle correction with TCDI may be a way to

correct for lower velocities obtained by TCDI compared with

nonimaging TCD, this technique has not yet been validated and

may overestimate stroke risk in children with SCD. 25 herefore

angle correction currently is not recommended when performing

and interpreting TCDI for stroke risk assessment in pediatric

SCD. 12,13

he natural history of TCD velocities in sickle cell patients

continues to be studied. 89-91 he STOP 2 trail was performed

to assess when discontinuation of transfusion therapy was

appropriate. Ater 30 months of transfusion treatment and

normalization of TCD velocities, patients were randomized to

continue or suspend transfusion therapy. STOP 2 demonstrated

that patients in whom transfusion therapy was suspended ater

normalization of TCD have a return to abnormal TCD velocities

with a higher occurrence of stroke, and are more likely to develop

silent clinical infarcts, indicating the need to continue transfusion

treatment. 92,93

Optimal management for children with abnormal velocities

remains problematic, with no consensus regarding long-term

therapy in children with persistently abnormal velocities. he

side efects of prolonged transfusion therapy have clinicians

searching for other methods of improving stroke risk, including

the use of hydroxycarbamide (hydroxyurea) therapy and bone

marrow transplant. 62,94-96 Hydroxyurea with phlebotomy proved

to be useful in the prevention of stroke recurrence during the

SWiTCH protocol, but not as good as transfusion therapy with

chelation. 97,98 Other studies (TWiTCH trial) are evaluating the

use of hydroxyurea in the prevention of primary stroke in patients

with abnormal TCD velocities.

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