Clinical management of ruptured intracranial aneurysms in sickle cell disease patients

Sickle cell disease (SCD) is a hereditary hemoglobinopathy associated with various cerebrovascular complications. Although ischemic stroke is the most common manifestation, subarachnoid hemorrhage (SAH) secondary to ruptured intracranial aneurysms represents a rare but life-threatening condition in this population. Data regarding the optimal management of aneurysmal SAH in SCD patients remain limited.

We retrospectively analyzed eight patients with confirmed SCD who were diagnosed with aneurysmal SAH and treated at a tertiary referral center. Clinical severity was assessed using the Glasgow Coma Scale (GCS), World Federation of Neurosurgical Societies (WFNS) grade, Hunt-Hess grade, and Fisher score. Aneurysm characteristics, including size, location, and multiplicity, were recorded. All patients underwent endovascular coil embolization. Hematologic parameters and peri-procedural multidisciplinary management strategies were reviewed. Aneurysm occlusion status was evaluated using the Raymond-Roy Occlusion Classification (RROC) at 6-month follow-up.

A total of 11 aneurysms were identified in 8 patients (mean age: 34.5 years; 5 males, 3 females). Three patients (37.5%) had multiple aneurysms. Six aneurysms (54.5%) were located in the anterior circulation and five (45.5%) in the posterior circulation. Four patients (50%) developed clinical and radiological vasospasm requiring intra-arterial therapy. All aneurysms were successfully treated with endovascular coiling. At discharge, modified Rankin Scale (mRS) scores ranged from 0 to 6. At 6-month follow-up, complete occlusion (RROC Class I) was achieved in the majority of treated aneurysms. Multidisciplinary perioperative management, including hematology consultation and transfusion strategies, was applied in all cases.

Ruptured intracranial aneurysms in patients with SCD require careful multidisciplinary management due to disease-specific hematologic and vascular risks. Endovascular coil embolization appears to be a safe and effective treatment modality in this population. Early aneurysm securing combined with optimized hematologic management may improve clinical outcomes and reduce procedure-related complications.