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Sickle cell disease and stroke in a pediatric population. Evidence-based diagnostic evaluation
Nevada Winrow1, Elias R Melhem
1Department of Radiology and Radiological Sciences, Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA.
Insights
Cerebrovascular complications in sickle cell disease (SCD) require careful monitoring. Transcranial Doppler (TCD) is crucial for stroke prevention in children with SCD, complementing CT and MR imaging for stroke assessment.
Area of Science:
- Pediatric Hematology
- Neurology
- Medical Imaging
Background:
- Cerebrovascular complications are frequent in sickle cell disease (SCD), posing significant risks.
- These complications can manifest as clinically apparent or silent events.
Purpose of the Study:
- To review diagnostic tests for cerebrovascular complications in pediatric SCD.
- To evaluate current neuroimaging guidelines and their evidence base.
Main Methods:
- Review of existing literature on diagnostic tests for cerebrovascular complications in pediatric SCD.
- Analysis of evidence supporting neuroimaging guidelines, noting limitations in randomized controlled trials.
Main Results:
- No absolute indications for neuroimaging are established due to limited high-quality evidence.
- CT and MR imaging are primary choices for acute stroke assessment.
- Transcranial Doppler (TCD) is the preferred imaging tool for stroke risk assessment and prevention.
Conclusions:
- Neuroimaging guidelines for pediatric SCD are based on lower-level evidence (Level 2 and 3).
- TCD is integral to routine care for stroke prevention in children with SCD, as per NIH guidelines.
Abstract:
Cerebrovascular complications are common in SCD and constitute a major source of concern to the pediatric hematologist. These complications can be either clinically overt or covert. The authors' review of the diagnostic tests does not offer absolute indications for neuroimaging because most of the evidence is based on studies that are not randomized controlled trials. Imaging guidelines for children have emerged based on the available level 2 and 3 literature, however, CT and MR imaging remain the initial tests of choice for stroke assessment, and TCD is the imaging tool of choice for stroke prevention. Based on guidelines handed down from the NIH, TCD has become a part of routine continuing care of children with SCD.