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Quantifying dilated perivascular spaces in children with sickle cell disease
Kristine A Karkoska1, Jahnavi Gollamudi1, Russell P Sawyer2
1Division of Hematology/Oncology, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
Insights
Children with sickle cell disease (SCD) show a high burden of dilated perivascular spaces (dPVS). These dPVS did not correlate with intelligence quotient but showed a decline with blood transfusions in SCD patients.
Area of Science:
- Neurology
- Pediatrics
- Radiology
Background:
- Neurological complications significantly impact children with sickle cell disease (SCD).
- Dilated perivascular spaces (dPVS) are a known marker of cerebral small vessel disease in adults, but their role in pediatric SCD is unexplored.
- Understanding dPVS in pediatric SCD is crucial for assessing neurological disease burden.
Purpose of the Study:
- To quantify the burden of dPVS in children with SCD.
- To investigate the association of dPVS with neurological complications, including silent cerebral infarctions and intelligence.
- To evaluate the effect of blood transfusion therapy on dPVS in pediatric SCD.
Main Methods:
- Utilized international consensus criteria to quantify dPVS in the centrum semiovale and basal ganglia.
- Analyzed T2-weighted MRI scans from 156 children with SCD participating in the Silent Cerebral Infarct Transfusion (SIT) trial.
- Examined relationships between dPVS burden, silent cerebral infarctions, hematological measures, demographics, and full-scale intelligence quotient (FSIQ) scores.
Main Results:
- A high burden of dPVS (60% of participants) was observed in children with SCD.
- No significant association was found between dPVS burden and FSIQ scores.
- Children with high baseline dPVS randomized to blood transfusion showed a moderate decline in dPVS over 36 months, unlike the observation group.
Conclusions:
- Pediatric SCD is associated with a high prevalence of dPVS.
- dPVS in pediatric SCD may have a different pathophysiology than silent cerebral infarcts.
- Further research is needed to elucidate the etiology and clinical significance of dPVS in pediatric SCD.
Abstract:
Sickle cell disease (SCD)-related neurological effects are particularly devastating. Dilated perivascular spaces (dPVS) are a well-described component of cerebral small vessel disease in older adults without SCD. However, the burden and association of dPVS with neurological complications in children with SCD have not been described. In this study, we used the international consensus criteria to quantify dPVS in the centrum semiovale and basal ganglia in T2-weighted magnetic resonance images (MRI) of children with SCD who were randomized as part of the Silent Cerebral Infarct Transfusion (SIT) trial. We examined the relationship between global and/or regional dPVS burden and presence or area of silent cerebral infarctions, hematological measures, demographic variables, and full-scale intelligence quotient (FSIQ) scores. The study included 156 SIT trial participants who had pre-randomization and study exit MRI. Their median age was 9.6 (5-15) years, 39% were female, and 94 (60%) participants had a high dPVS burden. Participants randomized to the blood transfusion arm and who had a high dPVS burden at baseline had a moderate decline in dPVS score over 36 months compared to no change in the observation group. On multivariable logistic regression, intelligence quotient was not associated with dPVS burden. Children with SCD included in the SIT trial have a high burden of dPVS compared to children without SCD. However, dPVS do not appear to have the same pathophysiology of silent cerebral infarcts. Further study is needed to determine both their etiology and clinical relevance.
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