Clinical management of ruptured intracranial aneurysms in sickle cell disease patients

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Korean J Cerebrovasc Surg. 2026;.jcen.2026.E2026.03.005

Publication date (electronic) : 2026 April 24

doi : https://doi.org/10.7461/jcen.2026.E2026.03.005

Ahmet Hamit Çınkı^,1, Mehmet Özer²

¹Department of Neurosurgery, Faculty of Medicine, Trakya University, Edirne, Turkey

²Department of Neurosurgery, Niğde Training and Research Hospital, Niğde, Turkey

Correspondence to Ahmet Hamit Çınkı Department of Neurosurgery, Faculty of Medicine, Trakya University Koca Sinan, Trakya University Balkan Campus, 22130, Merkez/Edirne, Turkey Tel +90 554 617 85 45 Fax +90 284 236 09 10 E-mail cinkiahmet@gmail.com

Received 2026 March 11; Revised 2026 April 9; Accepted 2026 April 14.

Abstract

INTRODUCTION

Cerebrovascular complications in patients with sickle cell anemia have been recognized since 1923, affecting approximately 26% of this population and consisting primarily of cerebral infarction (75%), intracerebral hemorrhage (20%), and subarachnoid hemorrhage (1–2%) [5]. These events remain leading causes of mortality across all age groups in sickle cell disease. Subarachnoid hemorrhage (SAH) in this setting was first documented in a pediatric patient by Cook in 1930, where no aneurysm was identified at autopsy [8]. From the 1980s onward, case reports—including notable early work by Overby and Rothman in 1985 have described the emergence of intracranial aneurysms in this population [4,20].

Aneurysmal rupture represents the most frequent cause of SAH in sickle cell anemia; nonetheless, leptomeningeal collateral vessel rupture and vaso occlusive crisis–related arterial stenosis or occlusion also contribute. Compared with the general population, aneurysmal hemorrhages in sickle cell patients occur at younger ages and are not typically associated with conventional risk factors such as hypertension, renal disease, or connective tissue disorders [19]. In this patient group, aneurysms are more common in the posterior circulation than in the normal population and are multiple in approximately 43% [9,14]. Brandão et al. analysed 50 aneurysms from English language reports, finding 32.7% situated in the posterior circulation and 58% multiple, with aneurysm sizes ranging between 3–7 mm, smaller than typically seen in the general population [4,8]. Notably, the ophthalmic segment of the internal carotid artery and the superior cerebellar artery are frequently involved [23].

Despite such observations, there remains a paucity of literature on the optimal management of intracranial aneurysms in sickle cell patients. This study aims to address this gap by evaluating advanced neurosurgical techniques and interdisciplinary case management strategies for ruptured cerebral aneurysms in this high-risk group.

MATERIALS AND METHODS

This study was designed to present cases of SAH in patients diagnosed with sickle cell anemia and to evaluate them in the context of the existing literature. All cases included in the study were patients diagnosed with sickle cell anemia who presented with SAH and were treated at the Department of Neurosurgery, Adana City Training and Research Hospital.

Patient data were reviewed retrospectively. Demographic characteristics, clinical presentation, laboratory test results, imaging findings, including computed tomography (CT) and digital subtraction angiography (DSA), as well as treatment modalities, were analyzed. Follow-up DSA evaluations performed 6 to 9 months after treatment were used to assess aneurysm occlusion status according to the Raymond-Roy classification system (Table 1).

Table 1.

Clinical and radiological characteristics of patients with SCD and Aneurysmal SAH

RESULTS

A total of 8 patients with sickle cell disease (SCD) who presented with aneurysmal subarachnoid hemorrhage were included in the study. The mean age was 33.9 years (range, 25–40 years), and 5 patients (62.5%) were male.

The median Glasgow Coma Scale (GCS) score at admission was 14 (range, 9–15). According to the WFNS classification, 4 patients (50%) were Grade I, 3 patients (37.5%) were Grade II, and 1 patient (12.5%) was Grade IV. Based on the Hunt-Hess classification, 2 patients (25%) were Grade I, 4 patients (50%) were Grade II, 1 patient (12.5%) was Grade III, and 1 patient (12.5%) was Grade IV. According to the Fisher classification, 1 patient (12.5%) was Grade I, 2 patients (25%) were Grade II, 2 patients (25%) were Grade III, and 3 patients (37.5%) were Grade IV.

The mean hemoglobin level at admission was 8.47 g/dL (range, 7.1–10.2 g/dL). Aneurysms were most commonly located at the basilar tip (n=4), followed by the middle cerebral artery (MCA) (n=2), internal carotid artery (ICA) (n=2), posterior communicating artery (PCoA) (n=1), vertebral artery (n=1), and anterior communicating artery (AComA) (n=1). Multiple aneurysms were identified in 3 patients (37.5%).

All patients underwent endovascular coil embolization. According to the Raymond-Roy occlusion classification, complete occlusion (Class I) was achieved in 4 patients (50%), residual neck (Class II) in 3 patients (37.5%), and residual aneurysm (Class III) in 1 patient (12.5%).

At follow-up, functional outcomes assessed by the modified Rankin Scale (mRS) showed that 6 patients (75%) had favorable outcomes (mRS 0–2), while 2 patients (25%) had unfavorable outcomes (mRS 3–6). One patient (12.5%) died (mRS 6).

Two cases were selected as illustrative cases to highlight specific clinical and technical features within the series.

Illustrative case 1

A 39-year-old male patient with a known diagnosis of sickle cell anemia presented to the emergency department with a complaint of severe headache. Cranial CT revealed a left-sided sulcal SAH consistent with Fisher Grade 1 (Fig. 1). On admission, the patient’s GCS score was 15 (E4M6V5), World Federation of Neurosurgical Societies (WFNS) Grade I, and Hunt and Hess Grade I. He was alert, oriented, and cooperative, with a stable general condition. He was admitted to the Neurosurgical Intensive Care Unit for further evaluation and management.

Fig. 1.

Axial non-contrast cranial computed tomography (CT) demonstrating a left-sided sulcal subarachnoid hemorrhage, consistent with Fisher Grade 1

Initial laboratory tests showed a hemoglobin (Hb) level of 9 g/dL, hematocrit (Hct) of 26.9%, and a platelet count of 369×103/μL. A hematology consultation was obtained. DSA was performed and demonstrated a saccular aneurysm measuring 12×7 mm located at the basilar tip (Fig. 2). Endovascular coiling of the aneurysm was performed (Fig. 3).

Fig. 2.

Digital subtraction angiography (DSA) demonstrating a saccular aneurysm located at the basilar artery tip

Fig. 3.

Digital subtraction angiography demonstrating successful endovascular coil embolization of the basilar tip aneurysm

On postoperative day 3, the patient developed left upper extremity weakness, which was attributed to cerebral vasospasm. Intra-arterial nimodipine therapy was initiated. By postoperative day 8, a significant improvement in motor strength was observed. The patient was discharged on postoperative day 29 with a mRS score of 2 and was prescribed acetylsalicylic acid (ASA) 300 mg/day.

At the 6-month follow-up, control DSA showed complete occlusion of the aneurysm, classified as Raymond-Roy Occlusion Classification (RROC) Class 1.

Illustrative case 2

A 37-year-old male patient with a known diagnosis of sickle cell anemia presented to the emergency department with complaints of severe headache and syncope. Cranial CT revealed a diffuse SAH consistent with Fisher Grade IV (Fig. 4). On neurological examination, the patient’s GCS score was 14 (E4M6V4), WFNS Grade II, and Hunt and Hess Grade II. His overall condition was moderate, and he was partially disoriented and uncooperative. He was admitted to the Neurosurgical Intensive Care Unit for further evaluation and treatment.

Fig. 4.

Axial non-contrast cranial computed tomography (CT) demonstrating diffuse subarachnoid hemorrhage involving the basal cisterns and cortical sulci, consistent with Fisher Grade IV

Laboratory tests revealed hemoglobin (Hb) of 7.1 g/dL, hematocrit (Hct) of 20.9%, and platelet count of 212×10³/μL. The patient was referred to the hematology department, and red blood cell suspension transfusion was administered based on their recommendations. Subsequently, DSA was performed, which demonstrated a saccular aneurysm measuring 9×4 mm located at the basilar tip (Fig. 5). Endovascular primary coiling with a retrievable stent-assisted technique was successfully performed (Fig. 6).

Fig. 5.

Digital subtraction angiography demonstrating a saccular aneurysm arising from the basilar artery tip

Fig. 6.

Digital subtraction angiography demonstrating endovascular coil embolization of the basilar tip aneurysm

During postoperative intensive care follow-up, the patient developed aphasia on the third day. Diffusion magnetic resonance imaging (MRI) and cranial CT were subsequently performed. Low- molecular-weight heparin therapy was initiated due to the ischemic areas detected on the diffusion MRI. Treatment for vasospasm was also administered. The patient’s Hb and Hct levels were monitored daily.

The patient was discharged on postoperative day 20 with a mRS score of 2 and was prescribed ASA 300 mg/day. At the 6-month follow-up, control DSA showed complete occlusion of the aneurysm, classified as RROC Class 1.

DISCUSSION

In patients with sickle cell anemia (SCA), vascular injury is thought to begin with endothelial adhesion of sickled erythrocytes, followed by arterial wall degeneration and impaired vascular integrity. These changes, together with chronic hemodynamic stress, may contribute to intracranial aneurysm formation [3,21]. Previous reports have shown that aneurysms in this population tend to occur at younger ages, are often multiple, and may be more frequently located in the posterior circulation than in the general population [4,15,19,21,22,24].

Although SAH in SCA is rare, its clinical presentation is similar to that in the general population. Cranial CT remains the first-line diagnostic tool, but diagnostic sensitivity may be reduced in patients with relatively low hemoglobin levels, and additional evaluation may occasionally be required [1,16]. In our series, aneurysmal SAH was confirmed by vascular imaging in all patients, and 11 aneurysms were identified in 8 patients, including multiple aneurysms in three cases and posterior circulation involvement in nearly half of the lesions.

Peri-procedural management in SCA requires particular attention because hypoxia, acidosis, hypovolemia, hypothermia, and contrast exposure may precipitate sickling-related complications [2,10,11,21]. For this reason, hematology consultation, optimization of hemoglobin status, hydration, oxygenation, and temperature control are essential components of treatment. In our series, all patients were managed with multidisciplinary perioperative planning involving hematology support and transfusion-based strategies when indicated. Peri-procedural hematological management aimed to maintain hemoglobin levels above 9–10 g/dL and reduce HbS percentage below 30% when feasible, in accordance with standard sickle cell management principles. Transfusion strategies were applied based on hematology consultation.

Endovascular treatment offers several practical advantages in this population, including avoidance of craniotomy, earlier mobilization, and potentially lower perioperative morbidity. Prior case reports and small series have increasingly supported coiling as a feasible treatment option in SCA patients [11,17,23]. In the present series, all aneurysms were treated endovascularly, with successful aneurysm occlusion achieved in all cases. This approach was particularly relevant for posterior circulation aneurysms, where microsurgical access may be more challenging [13,18].

Vasospasm remains a major concern after aneurysmal SAH in SCA because arterial narrowing, impaired oxygen delivery, and intravascular sickling may act synergistically to worsen cerebral ischemia [2,6,12]. Early securing of the aneurysm is therefore especially important, as it allows safer implementation of vasospasm-directed therapies. In our cohort, four patients developed clinical and radiological vasospasm and required intra-arterial treatment, underscoring the need for close monitoring in the postoperative period. In patients with SCA, vasospasm management requires particular caution due to the risk of exacerbating sickling under conditions of hypotension or hypoxia. In our series, intra-arterial nimodipine was administered with careful hemodynamic monitoring, and efforts were made to maintain adequate oxygenation and avoid acidosis during treatment.

Stent-assisted techniques and flow diversion should be considered cautiously in this setting. Because SCA is associated with a hypercoagulable state, and because dual antiplatelet therapy may increase hemorrhagic risk in patients with recent SAH, these strategies may be less favorable in ruptured aneurysms unless specifically required by aneurysm morphology [7,8].

The main limitations of this study are its retrospective design, single-center experience, and small sample size. However, aneurysmal SAH in patients with SCA is an uncommon but clinically important condition, and our series adds practical information regarding treatment strategy and perioperative management in this high-risk subgroup.

CONCLUSIONS

Ruptured intracranial aneurysms in patients with sickle cell anemia require careful multidisciplinary management because of the combined hematologic and cerebrovascular risks associated with the disease. In this population, aneurysms may occur at younger ages, may be multiple, and may involve the posterior circulation more frequently than in the general population.

Our experience suggests that endovascular coil embolization is a practical and effective treatment option for these patients, particularly when combined with hematologic optimization and close peri-procedural monitoring. Because vasospasm and ischemic complications remain important concerns, early aneurysm securing and coordinated care with hematology are essential. Larger multicenter studies are needed to better define optimal treatment strategies in this rare clinical setting.

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