Practice patterns for cerebral imaging for stroke screening and prevention in children with sickle cell anemia (SCA) vary across medical sites, according to a research article published in Pediatric Blood & Cancer.

Children with SCA are at higher risk for stroke and silent cerebral infarct (SCI). Although 2014 guidelines from the National Heart, Lung, and Blood Institute provide prevention recommendations, such as transcranial Doppler ultrasound (TCD) and chronic red cell transfusion (CRCT), the guidelines for preventive imaging are less clear. The guidelines do not provide specific recommendations on preventive magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) for asymptomatic children or for the frequency of neuroimaging for children at high risk of neurological complications.

The study authors surveyed principal investigators at the 28 sites from the Dissemination and Implementation Looking at the Care Environment consortium. The survey covered 37 items related to TCD screening, CRCT initiation, MRI and MRA usage, echocardiograms, developmental-behavioral screening, and immunizations.

The results showed variation in when different sites would order MRI and MRA. Nearly all sites ordered MRI for abnormal TCD results. Recurrent headache was the primary reason for ordering an MRI or MRA with a normal TCD result. However, existing guidelines did not have a standard recommendation for this practice. No sites seemed to have a routine screening schedule for MRI and MRA.


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Nearly half of the surveyed sites obtained an annual MRI and MRA for patients receiving CRCT or hydroxyurea to prevent stroke. Some sites recommended MRI screening when patients were old enough not to need sedation.

The American Society of Hematology (ASH) published cerebrovascular guidelines in April 2020 that provide specific recommendations on obtaining MRI and MRA for screening, prevention and monitoring of neurological complications. The new guidelines recommend obtaining an MRI at least once for school-age children who do not need sedation. However, these guidelines were not published at the time of the study’s survey. The authors expect the ASH guidelines will provide more clarity, as well as more discussion and investigation into neuroimaging for patients with SCA.

Reference

Phillips S, Schlenz AM, Mueller M, Melvin C, Adams RJ, Kanter J (on behalf of the DISPLACE investigators). Practice patterns for neuroimaging and transfusion therapy for management of neurologic complications in sickle cell anemia: DISPLACE consortium. Pediatr Blood Cancer. 2020;e28569. doi:10.1002/pbc.28569