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Updated: Mar 3, 2026
Functional Transcranial Doppler Ultrasound for Monitoring Cerebral Blood Flow
Published on: March 15, 2021
Transcranial Doppler screening for stroke risk in children with sickle cell disease: a systematic review
Sara Mazzucco1,2, Marina Diomedi3, Amrana Qureshi4
11 Centre for Prevention of Stroke and Dementia, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
Insights
Transcranial Doppler ultrasound (TCD) effectively screens children with sickle cell disease (SCD) for stroke risk. More research is needed on alternative methods and specific patient groups, especially in Europe.
Area of Science:
- Pediatrics
- Neurology
- Hematology
Background:
- Sickle cell disease (SCD) is a leading cause of stroke in children globally.
- Annual transcranial Doppler ultrasound (TCD) screening is standard for stroke risk in children with SCD, based on the STOP trial.
- Current TCD surveillance may impact stroke risk stratification and clinical management accuracy.
Purpose of the Study:
- To systematically review literature on TCD screening for stroke prevention in pediatric SCD patients.
- To focus on screening practices within European countries.
- To assess the quality of evidence using GRADE methodology.
Main Methods:
- A systematic literature review (CRD42016050549) was conducted.
- Included 33 studies (5 RCTs, 8 non-randomized, 20 observational) in English or French.
- Evidence quality was assessed using Grading of Recommendations Assessment, Development and Evaluation (GRADE).
Main Results:
- TCD is effective for primary stroke prevention screening in children with SCD.
- Limited high-quality evidence exists for alternative screening methods like MRI angiography.
- No data were found on screening effectiveness in children on hydroxyurea or with non-HbSS/HbSβ0 genotypes, nor on European screening rates.
Conclusions:
- High-quality studies are needed on alternative screening methods and their applicability to specific pediatric SCD subgroups.
- Updated guidelines are necessary for uniform, evidence-based surveillance in Europe due to low disease awareness and data gaps.
Abstract:
Background Sickle cell disease (SCD) is one of the most common causes of stroke in children worldwide. Based on the results of the Stroke Prevention Trial in Sickle Cell Anemia (STOP), annual transcranial Doppler ultrasound (TCD) screening for affected children is standard practice. However, the need for TCD surveillance programs could override the accuracy of the screening, affecting the correct stratification of stroke risk and subsequent clinical management of the target population. Aims To shed light on this issue, a systematic review of the literature on TCD screening for children and adolescents with SCD was carried out (CRD42016050549), according to a list of clinically relevant questions, with a particular focus on screening practices in European countries. Quality of the evidence was rated using the grading of recommendations assessment, development and evaluation. Summary of review Thirty-three studies published in English or French were included (5 randomized controlled trials, 8 experimental non-randomized, and 20 observational studies). The quality of the retrieved evidence ranged between low and high, but was rated as moderate or high most of the times. TCD is effective as a screening tool for the primary prevention of stroke in SCD children. There is no high-quality evidence on the effectiveness of alternative screening methods, such as imaging-TCD with or without angle correction or magnetic resonance angiography. No evidence was found on effectiveness of the screening on children on hydroxyurea and with genotypes other than HbSS and HbS/β0. No European data were found on screening rates or adherence of screening practices to the STOP protocol. Conclusions High-quality studies on alternative screening methods that are currently used in real-world practice, and on screening applicability to specific subgroups of patients are urgently needed. Considering the low awareness of the disease in European countries and the lack of data on screening practices and adherence, clinicians need up-to-date guidelines for more uniform and evidence-based surveillance of children with SCD.
