Brain magnetic resonance imaging and angiography in children with sickle cell anemia in Uganda in a cross-sectional sample

Document Type

Article

Department

Paediatrics and Child Health (East Africa)

Abstract

Objective

Children with sickle cell anemia (SCA) are highly susceptible to cerebrovascular injury. We performed brain magnetic resonance imaging and angiography (MRI-MRA) in Ugandan children with SCA to identify structural cerebrovascular abnormalities and examine their relationship to standardized clinical assessments.

Methods

A sub-sample (n=81) was selected from a cross-sectional study of children attending SCA clinic, including 52 (64.2%) with and 29 (35.8%) without clinically detected abnormalities. Clinical evaluation included assessment for prior stroke, cognitive testing and cerebral arterial transcranial Doppler (TCD) flow velocity. MRI-MRA scans were interpreted by at least two neuroradiologists.

Results

Mean age was 6.5±2.7 years, with 39 (48.1%) female. Mean hemoglobin was 7.3±0.9 g/dl. Overall, 13 (16.0%) were malnourished. Infarcts and/or stenosis were detected in 55 (67.9%) participants, with stenosis primarily in the anterior circulation. Infarcts were seen in those with normal 17/29 (58.6%) or abnormal 34/52 (65.4%) clinical testing (p=0.181). Neither abnormal MRI nor MRA was associated with age, sex, hemoglobin, or malnutrition. Abnormal MRA was highly associated with infarcts (p<0.0001). Participants with abnormal imaging had two-fold higher proportion of stroke on exam and/or impaired cognition. Stroke on exam was strongly associated with an imaging abnormality after adjusting for age, sex, malnutrition, and hemoglobin (OR 11.8, 95%CI 1.87-74.2).

Conclusion

Over half of these SCA children had cerebrovascular infarcts and/or arterial stenosis. Cerebrovascular disease was frequently undetectable by clinical assessments. While rarely available in under-resourced settings, MRI-MRA brain imaging is an important tool for defining SCA cerebrovascular disease and for assessing impact of clinical intervention trials

Comments

This work was published before the author joined Aga Khan University.

Publication (Name of Journal)

WB Saunders

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