Rationale and design of the extracranial Carotid artery Aneurysm Registry (CAR).

BACKGROUND: Aneurysms of the extracranial carotid artery (ECAA) are rare. Although most ECAA are identified in asymptomatic patients, serious neurological complications may occur. Current literature on treatment outcome contains mainly case reports and small case series with incomplete data and lack of long-term follow-up. There is clear lack on natural follow-up data, and there is no clear treatment algorithm. An international web-based registry to collect data on patients with ECAA is designed to provide clinical guidance on this scarce pathology. METHODS: The Carotid Aneurysm Registry (CAR) is open for inclusion of all patients with a fusiform or saccular ECAA. Patients with primary or secondary ECAA can be enrolled in CAR independent of the type of treatment (conservative or invasive). CAR participation does not interfere with the local physician's treatment policy. Follow-up and imaging can also be scheduled according to local clinical practice. The primary endpoint of the CAR in conservative patients is occurrence of symptoms related to the aneurysm at 30 days, one, three, and five years. The primary endpoint in invasively treated patients is freedom from symptoms of the aneurysm at 30 days, one, three, and five years. RESULTS: Analyses will relate outcome to etiology, imaging characteristics, ECAA growth patterns, and (if applicable) revascularization technique applied. The aim of the registry is to prospectively collect follow-up data on patients with an ECAA, being either treated conservatively or by invasive aneurysm exclusion strategies. The CAR database will be used to address diagnostic and therapeutic research questions. CONCLUSIONS: Collecting and analyzing the data gained from the registry could be the first step towards development of treatment guidelines and expert consensus for the management of ECAA.

Epidemiology, pathophysiology, diagnosis, and management of intracranial artery dissection.

Spontaneous intracranial artery dissection is an uncommon and probably underdiagnosed cause of stroke that is defined by the occurrence of a haematoma in the wall of an intracranial artery. Patients can present with headache, ischaemic stroke, subarachnoid haemorrhage, or symptoms associated with mass effect, mostly on the brainstem. Although intracranial artery dissection is less common than cervical artery dissection in adults of European ethnic origin, intracranial artery dissection is reportedly more common in children and in Asian populations. Risk factors and mechanisms are poorly understood, and diagnosis is challenging because characteristic imaging features can be difficult to detect in view of the small size of intracranial arteries. Therefore, multimodal follow-up imaging is often needed to confirm the diagnosis. Treatment of intracranial artery dissections is empirical in the absence of data from randomised controlled trials. Most patients with subarachnoid haemorrhage undergo surgical or endovascular treatment to prevent rebleeding, whereas patients with intracranial artery dissection and cerebral ischaemia are treated with antithrombotics. Prognosis seems worse in patients with subarachnoid haemorrhage than in those without.

Bare metal stents for treatment of extracranial internal carotid artery aneurysms: long-term results.

PURPOSE: To examine the long-term outcomes of bare metal stent placement for exclusion of extracranial internal carotid artery (ICA) aneurysms. METHODS: From 2006 to 2011, 7 consecutive symptomatic patients (4 men; mean age 52 years) with surgically inaccessible extracranial ICA aneurysms were treated with a bare stent at a single center. Patients received clopidogrel for 3 months after the procedure and aspirin for life. Clinical follow-up with duplex ultrasound and/or computed tomographic angiography was performed at 3, 6, and 12 months and yearly thereafter. RESULTS: All procedures were technically successful; no neurological complications occurred. After 6 months, there was complete thrombosis of the aneurysm in all except one case. In this asymptomatic patient, the residual active flow was successfully obliterated by additional coil embolization. Over a mean follow-up of 57 ± 22 months, all patients were alive and free of local or central neurological symptoms. All stents were patent, and thrombosis of the aneurysms was complete. CONCLUSION: In this small series, treatment of extracranial ICA aneurysms with a bare stent seems technically feasible and safe. All treated extracranial ICA aneurysms were excluded by primary intervention or secondary coil embolization.

Histological analysis of extracranial carotid artery aneurysms.

INTRODUCTION: Extracranial carotid artery aneurysms (ECAA) are rare but may be accompanied with significant morbidity. Previous studies mostly focused on diagnostic imaging and treatment. In contrast, the pathophysiological mechanisms and natural course of ECAA are largely unknown. Understanding the pathophysiological background may add to prediction of risk for adverse outcome and need for surgical exclusion. The aim of this study was to investigate the histopathological characteristics of ECAA in patients who underwent complete surgical ECAA resection. MATERIAL AND METHODS: From March 2004 till June 2013, 13 patients were treated with open ECAA repair. During surgery the aneurysm sac was resected and processed for standardized histological analysis. Sections were stained with routine hematoxylin and eosin and special stains to detect elastin, collagen, different types of inflammatory cells, vascular smooth muscle cells and endothelial cells. RESULTS: Histopathological characterization revealed two distinct categories: dissection (abrupt interruption of the media; n = 3) and degeneration (general loss of elastin fibers in the media; n = 10). In the degenerative samples the elastin fibers in the media were fragmented and were partly absent. Inflammatory cells were observed in the vessel wall of the aneurysms. CONCLUSION: Histological analysis in this small sample size revealed dissection and degeneration as the two distinct underlying mechanisms in ECAA formation.

Technical options for the treatment of extracranial carotid aneurysms.

Extracranial carotid artery aneurysm (ECAA) is an uncommon but serious condition. The morbidity and mortality of ECAA are assumed to be high when untreated. ECAA treatment presents a challenge because of accessibility of the carotid artery and lack of evidence-based guidelines. When exclusion of the aneurysm is considered, surgical resection of the aneurysm with reconstruction of blood flow is still considered the gold standard. Several alternative and endovascular approaches are discussed.