Abstract
BACKGROUND: Intracranial aneurysms (IAs) are more prevalent and rupture at smaller sizes in adults with sickle cell disease (SCD), compared with the general population, but evidence regarding treatment outcomes remains limited.
OBJECTIVE: To evaluate the safety and efficacy of coil embolization for unruptured IAs in adults with SCD.
METHODS: We retrospectively reviewed consecutive adults with homozygous sickle cell disease (HbSS) who underwent elective coil embolization for unruptured IAs between 2010 and 2023. Clinical, hematological, procedural, and radiological data were analyzed. Primary endpoints were immediate and long term angiographic occlusion and periprocedural complications. Durable occlusion was defined as Raymond-Roy Occlusion Classification class I-II at the last follow-up.
RESULTS: 25 patients with HbSS (median age 50 years; 76% women) with 35 aneurysms were treated. All patients received exchange transfusion to sickle hemoglobin <30%. Immediate Raymond-Roy Occlusion Classification class I-II occlusion was achieved in 33 of 35 lesions (94%; 95% CI 81% to 98%). Two complications (5.7%) occurred: one intraprocedural aneurysm perforation resulting in death 2 days after treatment and one transient ischemic event without permanent deficit. At a median follow-up of 60 months (range 12-156), durable occlusion was observed in 94% (33/35; 95% CI 81% to 98%), with two retreatments. All surviving patients were independent (modified Rankin Scale score of 0-2) at the last follow-up.
CONCLUSIONS: Preventive coil embolization for unruptured IAs in adults with HbSS achieved high rates of durable occlusion with an overall acceptable safety profile in carefully selected cases when applied selectively within expert centers using a standardized hematologic optimization protocol.