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Neuroimaging

ORIGINAL RESEARCH

Intracranial aneurysms in sickle cell anemia: clinical and imaging findings S Ali Nabavizadeh,1,2 Arastoo Vossough,1,2 Rebecca N Ichord,3 Janet Kwiatkowski,4 Bryan A Pukenas,1 Michelle J Smith,5 Philip B Storm,6 Eric L Zager,5 Robert W Hurst1,2 For numbered affiliations see end of article. Correspondence to Dr Robert W Hurst, Department of Radiology, Hospital of University of Pennsylvania, Perelman School of Medicine of the University of Pennsylvania, 19104, PA USA; robert.hurst@uphs. upenn.edu Received 29 November 2014 Revised 12 February 2015 Accepted 18 February 2015

ABSTRACT Background Cerebral aneurysm formation is one of the cerebrovascular complications of sickle cell disease. Objective To report the clinical and imaging findings of intracerebral aneurysms and their treatment in pediatric and adult patients with sickle cell disease. Methods Review of clinical data via chart abstraction and radiologic features at the University of Pennsylvania and Children’s Hospital of Philadelphia from 2000 to 2014 and review of the literature since 1942. Results Nineteen patients with aneurysms (2.7%) were found in 709 imaged patients, including 1.2% of imaged children and 10.8% of adults. A total of 44 aneurysms were detected (52.6% with multiple aneurysms, overall 2.3 per patient), 35 (79.5%) in the anterior circulation and 9 in the posterior circulation (20.4%). Thirty-eight unruptured aneurysms ranging in size from 2 to 6 mm and six ruptured aneurysms ranging in size from 3 to 9 mm in diameter were found. Of the patients with ruptured aneurysms, two were treated by stent-assisted coiling, two by clipping, and one patient with coiling. In the group without a rupture, one patient was treated by coil embolization and one patient with a peripheral middle cerebral artery aneurysm was treated by aneurysmectomy. Three pediatric patients with a previously normal MR angiogram demonstrated new aneurysm formation during the study. Conclusions Adult patients with sickle cell disease have a high prevalence of aneurysm formation. Both pediatric and adult patients with sickle cell disease tend to develop multiple aneurysms with frequent involvement of atypical locations, in both anterior and posterior circulations.

INTRODUCTION

To cite: Nabavizadeh SA, Vossough A, Ichord RN, et al. J NeuroIntervent Surg Published Online First: [please include Day Month Year] doi:10.1136/ neurintsurg-2014-011572

Cerebrovascular diseases are common complications in patients with sickle cell disease. Ischemia and intracranial hemorrhage are the most common neurologic complications of sickle cell disease. Cerebral aneurysms have been described in patients with sickle cell disease and have been shown to have different distributions and prevalence than in the general population.1 There is a paucity of published information about cerebral aneurysms and their treatment in patients with sickle cell disease. Aside from two large case series that were published in the early nineties,2 3 the literature on this subject is primarily limited to case reports and small case series.4–26 This study aimed to investigate the clinical and imaging findings of intracerebral aneurysms

and their treatment in pediatric and adult patients with sickle cell disease.

METHODS AND PATIENTS A retrospective review of brain MR angiography (MRA), CT angiography (CTA), and cerebral angiograms in patients with sickle cell disease at our institutions from 2000 to 2014 was performed. A keyword search of the picture archiving and communications system identified patients with sickle cell anemia and cerebral aneurysms using related keywords. Inclusion criteria were (1) diagnosis of hemoglobinopathy, including HgbSS, HgbSC, and HgbS-β thalassemia, confirmed by a hematologist; (2) finding of a cerebral aneurysm on routine surveillance imaging or in the evaluation of subarachnoid hemorrhage; (3) imaging available for analysis. Departmental databases from the Sickle Cell Anemia Program and Stroke Program were queried to identify cases. Medical records and imaging were also reviewed to determine inclusion criteria and treatments received. The study was performed with approval of the institutional review board and was compliant with guidelines of the Health Insurance Portability and Accountability Act.

Imaging protocol and analysis CTA was performed on various multidetector CT scanners (Siemens, Erlangen, Germany), and MRI was performed on 1.5 T or 3 T magnets (Siemens) in two institutions. The technical parameters of these various scanner models varied over the course of the 14 years. CTA imaging, in general, consisted of submillimeter collimation axial scanning after dynamic injection of intravenous contrast using a timed delay triggered from the aortic arch or common carotid arteries. MRI sequences varied among patients, but generally included precontrast T1-weighted, T2-weighted, fluid-attenuated inversion recovery, echo-planar spin-echo diffusionweighted imaging, and either T2* gradient-echo susceptibility or susceptibility-weighted imaging, and three-dimensional (3D) time-of-flight MRA. Catheter angiography (DSA) was performed using dedicated biplane neuroangiographic units (Siemens, Germany) with transfemoral arterial access. The vascular imaging studies (CTA, MRA and cerebral angiography) were reviewed and consensus reached by two board-certified neuroradiologists. For CTA and MRA, various visual assessments of available images, including source axial CTA and

Nabavizadeh SA, et al. J NeuroIntervent Surg 2015;0:1–7. doi:10.1136/neurintsurg-2014-011572

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Neuroimaging Table 1 Demographics, location, and treatment of patients with sickle cell disease and cerebral aneurysms Patient No

No. of aneurysms

Ruptured aneurysm

Location/size of ruptured aneurysm

No. of unruptured aneurysms

Location/size of unruptured aneurysms

Treatment

Note

1

1

No

N/A

1

3 mm periophthalmic

None

2

2

No

N/A

2

5 mm periophthalmic, 3 mm cavernous

Stent-assisted coiling of periophthalmic aneurysm

3

1

No

N/A

1

4 mm MCA

Aneurysmectomy

4

2

No

N/A

2

3 mm basilar, 5 mm left cavernous

None

5

1

Yes

9 mm basilar tip

0

N/A

6

1

No

N/A

1

3 mm basilar tip

Stent-assisted coiling with 3 mm residual filling at the base of aneurysm None

Stable aneurysm in 6-year MRA F/U 6-year DSA and 8-year MRA F/U, stable cavernous aneurysm, no recanalization of coiled periophthalmic aneurysm No new aneurysm in 5-year MRA F/U 7-year MRA F/U, stable basilar aneurysm, enlargement of 3 mm cavernous aneurysm from 3 to 5 mm, new 4 mm supraclinoid aneurysm 7-year MRA showed stable 3 mm residual filling at the base of coiled aneurysm

7

1

No

N/A

1

4 mm petrous ICA

None

8 9

1 4

No Yes

N/A 3 mm left PCA

1 3

3 mm periophthalmic 4 mm right PCA, 2 mm bilateral terminal ICA

None Stent-assisted coiling for left PCA aneurysm

10

6

Yes

3.5 mm supraclinoid ICA

5

Clipping

11

4

No

N/A

4

12

1

Yes

3 mm Acomm

0

Five 3–6 mm supraclinoid ICA aneurysms 3 mm right cavernous, 3 mm right supraclinoid, 4 mm right Pcomm, 3 mm left Pcomm N/A

13

3

No

N/A

3

14

6

Yes

Multi-compartmental right supraclinoid ICA, largest component 8 mm

5

15 16

1 2

No No

N/A N/A

1 2

17

3

Yes

8 mm PICA

2

18

1

No

N/A

1

19

2

No

N/A

2

3 mm left cavernous, periophthalmic and supraclinoid 6 mm right MCA, two (6 mm and 2 mm) right ACA, two (4 mm and 3 mm) left ACA 3 mm cavernous 3 mm PCA, 3 mm superior cerebellar 3 mm basilar tip, 3 mm cavernous 4 mm supraclinoid 3 mm right periophthalmic, 2 mm left supraclinoid

None

Coiling

None

Clipping of 5 aneurysms, with residual 4 mm filling of right supraclinoid ICA clipped aneurysm None None None in our institution None None

Multiple negative studies before diagnosis of aneurysm, No new aneurysm in 4-year MRA F/U Multiple negative studies before the diagnosis of aneurysm No F/U 1 year DSA F/U showed stable coiled aneurysm and right PCA, bilateral terminal ICA No new aneurysm in 5-year DSA F/U, no recanalization of clipped aneurysm Unchanged 4 aneurysms in 4-year MRA F/U

2 mm residual filling of coiled aneurysm in 1.5-year F/U, which was recoiled, no filling in 1-year DSA F/U Unchanged 3 aneurysms in 6-month MRA F/U No F/U

No F/U Stable aneurysms in 7-month MRA F/U No F/U Stable 4 mm aneurysm in 5-year CTA F/U Stable aneurysms in 4-year MRA F/U

Patients 1–7 are pediatric and patients 8–19 are adult. ACA, anterior cerebral artery; CTA, computed tomography angiography; F/U, follow-up; ICA, internal carotid artery; MCA, middle cerebral artery; MRA, magnetic resonance angiography; PCA, posterior cerebral artery; Pcomm, posterior communicating artery; PICA, posterior inferior cerebellar artery.

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Nabavizadeh SA, et al. J NeuroIntervent Surg 2015;0:1–7. doi:10.1136/neurintsurg-2014-011572

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Neuroimaging MRA data, multiplanar reformats, maximum intensity projections, and volume rendered images, were reviewed in various planes. DSA images were reviewed in multiple angiographic planes, and where available, 3D rendered images. Brain CT and MR images were also reviewed for pertinent findings by a board-certified diagnostic neuroradiologist and a second year neuroradiology fellow. For each patient, the location, number, and size of the cerebral aneurysms were recorded. Subarachnoid hemorrhage was diagnosed based on non-contrast head CT. In patients with multiple aneurysms, the ruptured aneurysm was determined based on the location of subarachnoid hemorrhage and aneurysm wall irregularity/nipple on cerebral angiogram. Occlusive vasculopathy was also diagnosed based on the finding

of arterial stenosis and Moyamoya-type collaterals in each patient by visual qualitative assessments.

Statistical analysis Patient demographics and aneurysm size, location, and type of treatment were evaluated using descriptive statistics. Mann– Whitney U test was used to compare the number of aneurysms in pediatric and adult populations, and also between male and female subjects. Fisher’s exact test was performed to compare the distribution of posterior circulation aneurysms and also to compare the frequency of multiple aneurysm formation in pediatric and adult populations. Spearman’s rank correlation

Figure 1 Representative cerebral angiogram from a patient with stent-assisted coiling. (A) Unenhanced head CT shows a subarachnoid hemorrhage adjacent to the basilar tip extending into the left Sylvian fissure and left ambient cistern (arrow). (B) Anteroposterior view of left vertebral artery injection shows bilateral P1 aneurysms (arrows). (C) Three-dimensional vertebral artery angiogram shows bilateral P1 aneurysms with nipple on left aneurysm (arrows). (D) Three-dimensional post-coiling vertebral artery angiogram showing right P1 aneurysm (arrow) and coiled left P1 aneurysm (arrowhead). Nabavizadeh SA, et al. J NeuroIntervent Surg 2015;0:1–7. doi:10.1136/neurintsurg-2014-011572

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Neuroimaging coefficient was obtained to investigate the correlation between age and number of aneurysms.

RESULTS Demographics, characteristics, clinical features A total of 709 patients with sickle cell disease had vascular imaging, with 19 patients demonstrating aneurysms ( prevalence 2.7%). The mean age of patients (11 female, 8 male) at the time of initial diagnosis of an aneurysm was 29.4±17 years (range 12.9–53 years, median age 21 years). Fifteen (78.9%) patients had HgbSS, two (10.5%) had HgbSC, and two (10.5%) had HgbS-β-0 thalassemia. Of the 19 patients with aneurysms, seven of 598 were pediatric (

Intracranial aneurysms in sickle cell anemia: clinical and imaging findings.

Cerebral aneurysm formation is one of the cerebrovascular complications of sickle cell disease...
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