Moyamoya syndrome and stroke among pediatric sickle cell disease patients in Sudan: A cross-sectional study

BACKGROUND: Sickle cell disease (SCD) is autosomal recessive hemolytic anemia due to hemoglobinopathy commonly in Sub-Saharan Africa, and particularly in Sudan. The disease induces a pro-inflammatory cascade in the intimal layer that leads to hyperplasia and progressive stenosis in the major vessel of the circle of Willis. This is associated with the development of Moyamoya collaterals. The aim of this study is to highlight the frequency of Moyamoya syndrome in Sudanese pediatric patients with sickle cell disease presenting with stroke. MATERIALS AND METHODS: A descriptive cross-sectional hospital-based study was conducted in the Department of Hematology at Gaafar Ibnauf Pediatric Tertiary Hospital in Khartoum state in Sudan, in the period between March 2021 and August 2021. Secondary data has been collected from the medical record after ethical approval and informed consent. RESULTS: A total of 104 patients were included in the study. The males were the majority, about 60 (57.7) compared to 44 (42.3%) females. More than half of our study population was in the school-age 54 compared to 42 adolescents and only 8 patients of preschool age. Only 50 out of 104 patients had diagnostic MRA which revealed features of Moyamoya syndrome in 48 (96%) patients. Motor weakness (100%), aphasia (52.9%), and facial palsy (35%) were the major stroke presentations. The left anterior circulation territory was the most common site of moyamoya features involvement in 31 patients out of 48. CONCLUSION: Stroke is a common problem in Sudanese pediatric patients with sickle cell disease, with a very high frequency of Moyamoya vasculopathy among patients with stroke presentation. The burden of Moyamoya syndrome in Sudanese pediatric patients with sickle cell disease is underestimated due to the cost of the available screening and diagnostic tools.