Intracranial Aneurysm in Patients with Sickle Cell Disease: A Systematic Review

Intracranial aneurysm in patients with sickle cell disease is rare, but with an increased incidence in the recent years. This type of intracranial aneurysm possesses distinctive characteristics, and it has been the subject of case reports and series.

We systematically searched relevant publications through PubMed, EMBASE, Web of Science, and Google Scholar up to December 21, 2016. We extracted data about clinical features and outcomes and then conducted a descriptive analysis.

We identified 46 related publications, comprising 111 patients and 218 aneurysms. The mean age was 27 years (range, 5-54 years), and the male:female ratio was 0.96 (52:54). A male predominance existed in children, whereas a female predominance in adults. Age distribution approximated normal distribution, with peak phase between 21 and 30 years. In the child cohort, 13 of 31 (41.9%) aneurysms ruptured, whereas 62 of 79 (78.5%) aneurysms rupturing in the adult cohort. Aneurysms tended to be multiple (45.0%), small (85.6%), and saccular type (90.8%) and they exhibited a preference for posterior circulation (31.7%). Conservation and clipping became the main treatment in children and adults, respectively, but children had more good outcomes (80%) than adults did (67.1%).

Intracranial aneurysms related to sickle cell disease mainly affected patients in their 20s, with a contradictory sex ratio in children and adults. Aneurysms manifested predisposition for multiplicity and posterior circulation. There was no difference between children and adults regarding shape and location of aneurysms. However, children with lower aneurysm rupturing rates had better outcomes than their adult counterparts did.