Brain Magnetic Resonance Imaging and Angiography in Children with Sickle Cell Anaemia in Uganda in a Cross-Sectional Sample

• Authors: Richard Idro, Makerere University College of Health Sciences; Amelia K Boehme, Columbia University; Michael Kawooya, Makerere University College of Health Sciences; Samson K Lubowa, Makerere University College of Health Sciences; Deogratias Munube, Makerere University College of Health Sciences; Paul Bangirana, Makerere University College of Health Sciences; Robert Opoka, Makerere University College of Health Sciences; Ezekiel Mupere, Makerere University College of Health Sciences; Angela Lignelli, Columbia University; Philip Kasirye, Makerere University College of Health Sciences; Nancy S Green, Columbia University; Frank Minja, Emory University
• Journal Title: JOURNAL OF STROKE & CEREBROVASCULAR DISEASES
• Volume: Volume 31, Number 4
• Publisher: ELSEVIER | 2022-02-11, Pages 106343-106343
• Type of Work: Article | Post-print: After Peer Review
• Publisher DOI: http://doi.org/10.1016/j.jstrokecerebrovasdis.2022.106343
• Subjects: Health Sciences, Medicine and Surgery; Health Sciences, Radiology
• Keywords: Science & Technology; Life Sciences & Biomedicine; Neurosciences; Peripheral Vascular Disease; Neurosciences & Neurology; Cardiovascular System & Cardiology; Silent infarct; Stroke; Pediatrics; Sickle cell; Magnetic resonance imaging; SILENT CEREBRAL INFARCTS; DOPPLER FLOW VELOCITIES; SEVERE MALARIAL ANEMIA; DISEASE; TRANSFUSIONS; HYDROXYUREA; STROKE; HYDROXYCARBAMIDE; RELIABILITY; MULTICENTER

Abstract

Objective: Children with sickle cell anaemia (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 haemoglobin was 7.3±0.9 g/dl. Overall, 13 (16.0%) were malnourished. Infarcts and/or stenoses were detected in 55 (67.9%) participants, with stenoses 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, haemoglobin, 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 haemoglobin (OR 11.8, 95%CI 1.87-74.2). Conclusion: Over half of these SCA children had cerebrovascular infarcts and/or arterial stenoses. 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.