Abstract 10251: Intracranial Aneurysms in Sickle Cell Adults Screened by Mra

Abstract

Introduction: Sickle cell disease(SCD) is the most common genetic disease globally. It is an autosomal recessive disease that affects the β-globin chain which most often presents with anemia and vaso-occlusive symptoms. This condition includes multiples genotypes/phenotypes, the most common of which is HbSS (homozygosity for Hemoglobin S); others are compound heterozygosity with HbC variant (HbSC) and HbS-β-thalassemia (HbSβ+/HbSβ0). Stroke occurs in up to 25% of SCD patients and MRI screening is already recommended by the American Society of Hematology. Small case series have shown a higher prevalence of intracranial aneurysms (ICAN) in SCD, and MR angiography (MRA) may therefore be of benefit for the detection of arterial pathology. Hypothesis: There is an increased prevalence of ICAN in adults with SCD; Frequency of anevrysms is associated with SCD genotype and age. Methods: Adult patients from the SCD clinic of our institution were screened with 3T brain MR complemented with MRA in order to accurately determine the prevalence of ICAN. Results: Our study included 245 asymptomatic SCD adults (369 MRIs). Median age was 32; 52% were women. The overall prevalence of ICAN was 0.11 (0.95 CI 0.07-0.15). All aneurysms were saccular ≤ 10 mm. Forty-four aneurysms were detected in 27 patients with a third presenting with multiple aneurysms. Genotype subgroup analysis revealed a higher prevalence of ICAN in SS/Sb0 (0.17; 0.95 CI 0.11-0.25) compared to the SC/Sb+ (0.06; 0.95 CI 0.02-0.11) (p = 0.007). An association between age and ICAN was also statistically significant (p = 0.03) with stratification revealing a higher prevalence of ICAN for >30 years old, 0.15 (0.95 CI 0.09-0.21). In addition, in patients aged 18-30, the prevalence was 0.06 (0.95 CI 0.26-0.12). Conclusions: This is the largest cohort of SCD adults screened specifically with MRA. We confirm with higher confidence the increased prevalence of ICAN in this population which is in the range of other established indications for aneurysm screening such as Familial Intracranial Aneurysm and Autosomal Dominant Polycystic Kidney Disease. This knowledge could therefore impact the existing screening guidelines for SCD patients via systematic inclusion of an MRA sequence in the recommended imaging protocol.