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[Transcranial color Doppler in children with sickle cell disease]
S Verlhac1, F Bernaudin, P Brugières
1Service d'imagerie médicale, Centre Hospitalier Intercommunal, Créteil. suzanne.verlhac@chicreteil.fr
Insights
Children with sickle cell disease face risks of cerebral vascular disease. Transcranial Doppler screening can detect stenosis, and transfusions significantly reduce stroke risk.
Area of Science:
- Pediatric Neurology
- Vascular Medicine
- Hematology
Background:
- Cerebral vascular disease in children with sickle cell disease involves progressive stenosis of large arteries at the skull base.
- Transcranial Doppler (TCD) offers a sensitive, specific, and cost-effective method for detecting this stenosis.
- Standard stenosis diagnostic criteria differ for children with sickle cell disease due to elevated baseline velocities.
Purpose of the Study:
- To highlight the importance of early detection of cerebral vasculopathy in children with sickle cell disease.
- To emphasize the utility of transcranial Doppler in identifying at-risk individuals.
- To underscore the effectiveness of transfusion therapy in mitigating stroke risk.
Main Methods:
- Utilizing transcranial Doppler to assess cerebral artery velocities in pediatric sickle cell disease patients.
- Applying specific diagnostic criteria adjusted for sickle cell disease.
- Monitoring stroke risk based on mean velocity thresholds and transfusion protocols.
Main Results:
- A mean velocity exceeding 2 m/s predicts a 40% risk of stroke within three years.
- Transfusion programs maintaining hemoglobin S below 30% reduce stroke risk to 2%.
- Early detection via TCD enables timely intervention.
Conclusions:
- Regular transcranial Doppler screening from age one or two is crucial for children with sickle cell disease.
- Identifying cerebral vasculopathy before overt stroke prevents long-term deficits.
- Prompt initiation of appropriate treatment, such as transfusions, is vital for reducing stroke incidence.
Abstract:
Cerebral vascular disease in children with sickle cell disease is characterized by progressive stenosis of large arteries of the skull base, which can be detected sensitively, specifically and unexpensively by transcranial Doppler. Because sickle cell disease is responsible for basal high velocities, criteria used for diagnosis of stenosis are different than those used in non sickled children and moreover in adults. Mean velocity higher than 2 meters per second is predictive of a 40% risk for stroke in the three following years, but transfusion program maintaining hemoglobin S under 30%, reduces the risk to 2%. It is important to test each child with sickle cell disease by the age of one or two years, in order to detect cerebral vasculopathy before overt stroke and its residual deficits and to initiate appropriate treatment