Preventive treatment of unruptured intracranial aneurysms in adult patients with sickle cell anemia: A cohort study

• Published in: Journal of neuroradiology Vol. 50; no. 5; pp. 511 - 517
• Main Authors: Dimancea, A, Mattioni, S, Nouet, A, Drir, M, Santin, A, Marrot, B, Shotar, E, Corcy, C, Bottin, L, Sourour, NA, Premat, K, Alamowitch, S, Carpentier, A, Degos, V, Clarençon, F, Lionnet, F, Lenck, S
• Format: Journal Article
• Language: English
• Published: France Elsevier Masson SAS 01.09.2023
• Subjects: Endovascular treatment; Intracranial aneurysms; Sickle-cell anemia; Endovascular treatment; Intracranial aneurysms; Sickle-cell anemia
• ISSN: 0150-9861
• DOI: 10.1016/j.neurad.2023.02.003

•Intracranial aneurysms are diagnosed in up to 9% of patients with sickle cell anemia, while consensual approaches to treatment of unruptured intracranial aneurysms in this population are still lacking.•A standardized hematological preparation protocol was undertaken before each aneurysm treatment.•Overall, endovascular and surgical treatment seems safe and effective: successful treatment was documented in 95% of aneurysms, while all 6 post-procedural complications were transitory or reversible, without residual deficits at one-year follow-up.•We suggest using stenting as a last therapeutic resort due to the potential high rate of ischemic complications in this population. Intracranial aneurysms are frequent in patients with sickle cell anemia, while subarachnoid hemorrhage is a major cause of death and disability in young adult patients. Several characteristics, such as younger age and smaller size at rupture, may incline therapeutic decision towards exclusion treatments. Clinical guidelines on treatment of unruptured intracranial aneurysms in this population are still missing. We aimed to assess the safety and efficacy of the treatment of unruptured intracranial aneurysm in patients with sickle cell anemia, using an adapted hematological preparation regimen. Adult patients with sickle cell anemia and treated unruptured aneurysms by endovascular therapy or neurosurgery were included in this retrospective cohort study. Treatment decision was reached after multi-disciplinary assessment. A pre-operative blood transfusion protocol was undertaken targeting a HbS below 30%. Demographic data, hematological preparation parameters and clinical and radiological outcomes were documented. Twenty-five procedures were performed in 18 patients encompassing 19 aneurysms treated by embolization and 6 by surgery. Median age at treatment was 34 years-old and median aneurysm dome size was 4.4 mm. Immediate aneurysm exclusion rate was 85.7% after endovascular therapy and 100% after neurosurgery. Median follow-up was 6 months, with all patients being asymptomatic at last follow-up. Two transitory ischemic neurological deficits, as well as four cases of iodine-induced encephalopathy were identified after embolization. No complication occurred after surgery. Endovascular therapy by coiling and neurosurgical treatment of unruptured intracranial aneurysms appears to be safe in patients with sickle cell anemia and should be considered given the specific hemorrhagic risk observed in this population. A rigorous hematological preparation, associated with a dedicated peri‑operative protocol and an adequate therapeutic strategy are essential prerequisites.