Objective: There are only limited epidemiological studies evaluating the association between vertebrobasilar dolichoectasia (VBD) and outcomes. This study was designed to elucidate the outcome and prognosis of adults diagnosed with VBD by magnetic resonance angiography (MRA) and to ascertain if these outcomes were independent of known vascular risk factors. Methods: A cohort study was designed to compare VBD cases identified retrospectively from a computerised database of MRA reports with age and sex matched controls evaluated after a 4–7 year period, and 1440 MRA reports were reviewed. The inclusion criteria were age ⩾18 years and a radiological diagnosis of VBD. Patients were excluded if there was haemodynamically significant stenosis or occlusion of the posterior circulation. Data were obtained by medical record review and telephone questionnaires. The primary outcome measure was transient or fixed posterior circulation dysfunction (PCD), with a secondary outcome measure of all cause mortality. Results: Sixty four VBD cases were obtained, and 19 cases (30%) were excluded due to refusal and/or insufficient follow up data. From the same computerised database, 45 controls were selected by consecutive sampling. The mean age at follow up was 73.4 years for VBD cases and 73.1 years for controls, with a median follow up period of 64 months. VBD was associated with fixed/transient PCD (p = 0.0001; estimated adjusted odds ratio (OR) of 20.6 and confidence interval (CI) of 4.4 to 95.3), and with all cause mortality (OR = 3.6 CI 1.3 to10.3); (p = 0.018). VBD cases had 36% mortality, with 50% occurring within 34 months of the initial diagnosis. The VBD cumulative survival curve was statistically different from the controls (p = 0.012 by Mantel-Cox log rank test). Conclusions: This study suggests that VBD may be an independent risk factor for stroke. VBD cases had an increased likelihood for PCD, all cause mortality, and reduced cumulative survival independent of other vascular risk factors in this cohort. Larger population based prospective studies are required to verify these results.

https://jnnp.bmj.com/content/jnnp/75/suppl_1/i22.full.pdf

Vertebrobasilar dolichoectasia diagnosed by magnetic resonance angiography and risk of stroke and death: a cohort study

Objective: The long-term prognosis of patients with vertebrobasilar dolichoectasia (VBD) is unknown. The purpose of this study was to explore the natural history of VBD, evaluate its progression, and examine factors that may influence the clinical course of this condition. Methods: We conducted a prospective clinical and imaging follow-up study of 156 consecutive patients with VDB followed for an average of 11.7 years. Predictors of events were evaluated by multivariate analysis. Survival analysis was used to evaluate rates of incidence. Results: During follow-up, 93 patients (60%) experienced at least one event: 75 patients had stroke (59 ischemic and 21 hemorrhagic), 31 patients had new compressive symptoms, and 2 patients had hydrocephalus. Events were significantly associated with the severity of VBD, i.e., diameter, height of bifurcation, and lateral displacement of the basilar artery. During follow-up VBD progressed in 43% of patients. Progression of VBD was associated with a higher morbidity and mortality. The cumulative proportion of survivors free of adverse health event was 54.1 at 5 years, 39.5 at 10 years, and 23.5 at 15 years. During follow-up, 62 patients died and stroke was the most common cause of death. Conclusions: The long-term prognosis of patients with vertebrobasilar dolichoectasia (VBD) depended mainly on the severity of the condition at diagnosis and on its evolutionary characteristics. Progression of VBD exposed patients to high risk of adverse events, especially stroke.

Natural history of vertebrobasilar dolichoectasia.

Objectives: The diameters of the vertebral arteries (VAs) are very often unequal. Therefore, this study investigated if unequal VA flow contributes to the development of basilar artery (BA) curvature and if it is a link to the laterality of pontine or cerebellar infarcts occurring around the vertebrobasilar junction. Methods: Radiological factors were analysed (infarct laterality, VA dominance, BA curvature and their directional relationships) in 91 patients with acute unilateral pontine or posterior inferior cerebellar artery (PICA) territory infarcts. The “dominant” VA side was defined as either that the VA was larger in diameter or the VA was connected with the BA in more of a straight line, if both VAs looked similar in diameter on CT angiography. Multiple regression analysis was performed to predict moderate to severe BA curvature. Results: The dominant VA was more frequent on the left side (p Conclusions: Unequal VA flow is an important haemodynamic contributor of BA curvature and development of peri-vertebrobasilar junctional infarcts.

https://jnnp.bmj.com/content/jnnp/80/10/1087.full.pdf

Vertebral artery dominance contributes to basilar artery curvature and peri-vertebrobasilar junctional infarcts

Dolichoectasia (dilatative arteriopathy) describes marked elongation, widening, and tortuosity of arteries. The intracranial vertebral and basilar arteries are preferentially involved. Dolichoectatic arteries usually have an abnormally large external diameter and a thin arterial wall, with degeneration of the internal elastic lamina, multiple gaps in the internal elastica, thinning of the media secondary to reticular fiber deficiency, and smooth muscle atrophy. The most important clinical presentations of dilatative arteriopathy include acute brain ischemia; a progressive course related to compression of cranial nerves, the brain stem, or the third ventricle; and catastrophic outcome caused by vascular rupture. Flow in dilated arteries can become bidirectional, resulting in reduced antegrade flow and thrombus formation. Elongation and angulation of arteries can stretch and distort the orifices of arterial branches, leading to decreased blood flow, especially in penetrating branches.

Vertebrobasilar dilatative arteriopathy (dolichoectasia)

Dolichoectasia is an arterial disease that causes dilatation and/or tortuosity of the affected vessel. The prevalence of dolichoectasia increases with age, and this disease is also associated with other traditional cardiovascular risk factors. Multiple pathophysiological processes might lead to the development of dolichoectatic vessels, and activation of metalloproteinases and irregular turbulent blood flow seem to cause irreversible disruption of the internal elastic lamina. Intracranial dolichoectasia commonly presents with ischemic or hemorrhagic stroke, and/or cranial neuropathies. The posterior circulation is more frequently affected by the dolichoectatic process than the anterior circulation. A positive diagnosis of dolichoectasia requires visual assessment of vessel shape and, if the posterior circulation is affected, application of Smoker's criteria. Reproducible criteria that aid diagnosis of dolichoectasia in the anterior circulation are lacking. No specific treatment for dolichoectasia exists, and the surgical and medical therapies that have been used to treat this condition have not been systematically evaluated. More evidence is needed to better understand the underlying dilatatory artheriopathy that causes this disease, and to determine whether patients with dolichoectasia might benefit from early diagnosis. In this article, we provide a comprehensive review of current knowledge regarding dolichoectasia, and highlight gaps in our knowledge to aid future research.

Dolichoectasia—an evolving arterial disease

Background and Purpose—Vertebrobasilar dolichoectasia (VBD) may produce symptoms by direct compression of cranial nerves or the brain stem, by obstructive hydrocephalus, or by ischemia in the vertebrobasilar arterial territory. This study was undertaken to examine and characterize clinical and imaging findings in patients with stroke associated with VBD and compare these data with those for patients with VBD who did not have a stroke. Methods—We studied 40 consecutive stroke patients with associated VBD. All were evaluated by CT scan (n=9), MRI (n=6), or both (n=25). The diameter of the basilar artery (BA), height of bifurcation, and transverse position were evaluated. Clinical and imaging findings were compared with those found in a group of 40 VBD patients without stroke. Results—More than half of the patients (24 of 40) had infratentorial infarcts, located mainly in the midpons. Sixteen patients had supratentorial lesions localized in the thalamus (n=8) or in the superficial arterial territory of the p...

Posterior Circulation Infarcts in Patients With Vertebrobasilar Dolichoectasia

The authors describe a case of solitary eosinophilic granuloma of the sphenoid bone in a ten-year-old boy, which led to exophthalmos, diplopia, pain and a reduction in visual acuity. Contrast enhanced computed tomography demonstrated osteolytic areas associated with a soft tissue. A fine needle aspiration biopsy allowed the diagnosis of eosinophilic granuloma.With the local infiltration of triamcinolone and radiation therapy (1000 rads in all), a clinical and radiological regression was noted after a few months, while pain had notably subsided one week after the infiltration. After 15 months, CT demonstrated the complete structural restoration of the bone.In our study, as well as in those of others, CT has proved to be an extremely valid and precise medium for documenting lesions associated with histiocytosis, because of its definition of cancellous and solid structures and soft pathologic tissue.The therapeutic problems of this benign and self-limiting disease are discussed. Though many cases of spontane...

Giuseppe Filosomi

Papers

Posterior Circulation Infarcts in Patients With Vertebrobasilar Dolichoectasia

Evolving aspects of solitary granuloma of the sphenoid bone after radiation and cortisone therapy observed with computed tomography