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

Abstract

Background and purpose: 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.

Patients and methods: 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.

Results and conclusions: 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.

Keywords: Endovascular treatment; Intracranial aneurysms; Sickle-cell anemia.