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Background and purposeAneurysms in various arterial beds have common risk- and genetic factors. Data on the correlation of extracranial carotid artery aneurysms (ECAA) with aneurysms in other vascular territories are lacking. We aimed to investigate the prevalence of ECAA in patients with an intracranial aneurysm (IA).MethodsWe used prospectively collected databases of consecutive patients registered at the University Medical Center Utrecht with an unruptured intracranial aneurysm (UIA) or aneurysmal Subarachnoid hemorrhage (SAH). The medical files of patients included in both databases were screened for availability of radiological reports, imaging of the brain and of the cervical carotid arteries. All available radiological images were then reviewed primarily for the presence of an ECAA and secondarily for an extradural/cavernous carotid or vertebral artery aneurysm. An ECAA was defined as a fusiform dilation ≥150% of the normal internal or common carotid artery or a saccular distention of any size.ResultsWe screened 4465 patient records (SAH database n = 3416, UIA database n = 1049), of which 2931 had radiological images of the carotid arteries available. An ECAA was identified in 12/638 patients (1.9%; 95% CI 1.1–3.3) with completely imaged carotid arteries and in 15/2293 patients (0.7%; 95% CI 0.4–1.1) with partially depicted carotid arteries. Seven out of 27 patients had an additional extradural (cavernous or vertebral artery) aneurysm.ConclusionsThis comprehensive study suggests a prevalence for ECAA of approximately 2% of patients with an IA. The rarity of the disease makes screening unnecessary so far. Future registry studies should study the factors associated with IA and ECAA to estimate the prevalence of ECAA in these young patients more accurately.

PurposeIncreased arterial tortuosity has been suggested as a predisposing factor for carotid artery dissection, which is an important risk factor for development of extracranial carotid artery aneurysms (ECAA). Prior to comparison with non-ECAA controls, the optimal measurement technique should be defined. This study describes the difference between software packages in terms of reproducibility and absolute outcome of arterial tortuosity measurements in ECAA patients.MethodsCT-angiography analysis was performed on 12 ECAA patients selected from our registry, using four software packages: 3mensio Vascular, TeraRecon, Vital Images, and Aycan OsiriX PRO. The tortuosity index (TI) was calculated from the skull base until the carotid bifurcation and aortic arch, and was defined as the centerline’s true length divided by the straight line distance. Intraclass correlation coefficients (ICC) with 95% confidence intervals were calculated to quantify inter- and intra-observer variability within one software package, and differences in measured TI between packages.ResultsInter-observer agreement was nearly perfect for 3mensio, excellent for Vital Images and OsiriX, and substantial for TeraRecon, with ICC 0.99 (0.96–1.0), 0.90 (0.69–0.97), 0.84 (0.53–0.95), and 0.72 (0.28–0.91), respectively. Intra-observer agreement ranged from ICC 1.0 for 3mensio to 0.91 for TeraRecon. Agreements in TI ranged from ICC 0.99 (0.98–1.0) for 3mensio vs. OsiriX, to 0.95 (0.82–0.98) for 3mensio vs. TeraRecon. Median time needed to complete one round of measurements was highest for OsiriX (p = 0.013).ConclusionsCarotid artery tortuosity measurements are reproducible and comparable between current commercially available software packages, with high intra-observer agreement. Although the reproducibility differed per software packages, all packages scored an acceptable inter-observer agreement.