Dolichoectasia
Updates to Article Attributes
The term dolichoectasia means dilated and elongated. It is used to characterise arteries that have shown a significant deterioration of their tunica intima (and occasionally the tunica media), weakening the vessel walls and causing the artery to elongate and distend.
Epidemiology
Dolichoectasias are more common in males 2. With intracranial dolichoectasia, the incidence is estimated at ~3% (range 0.06-5.8%) 2.
Clinical presentation
Ischaemic effects on the brain stem and cerebellar hemispheres as well as symptoms related to hydrocephalus are common. Direct cranial nerve compression can lead to isolated cranial nerve dysfunction, usually associated with a normal-sized basilar artery that is tortuous and elongated (neurovascular compression syndrome (NVCS)). Cranial nerve dysfunction most commonly involves the VII cranial nerve and the V cranial nerve. Multiple cranial nerve dysfunction is far more likely to occur if there is dilation (ectasia) associated with a tortuous and elongated basilar artery. Cranial nerves affected in descending order of frequency include VII, V, III, VIII, and VI.
Pathology
As the arrangement of connective tissue is disturbed, the vessel wall is no longer able to hold its original conformation and begins to unravel due to the continued hypertension. High blood pressure moulds and force the artery to take now on an elongated, tortuous course to better withstand the higher pressures.
Subtypes
Can be divided into several subtypes 3:
- atherosclerotic dolichoectasia
- non-atheroscerotic dolichoectasia
- dolichoectatic appearance secondary to an arterial dissection
Aetiology
Hypertension most commonly causes the atherosclerotic type. Continued stress on the walls of the artery will degrade the vessel wall by damaging and loosening the collagen and elastin meshwork that comprises the intima. Similarly, hypercholesterolemia or hyperlipidemia can also provide sufficient trauma to the vessel wall resulting in dolichoectasia.
Location
- most commonly affected is the vertebrobasilar artery: vertebrobasilar dolichoectasia (VDBE)
- the internal carotid artery (ICA) is also at high risk to be affected
Associations
- patients with autosomal dominant polycystic kidney disease (ADPKD)
are, Marfan Syndrome, and Ehlers-Danlos Syndrome, are more likely to be subject to dolichoectasia(s)ref required9 - dolichoectasia is a possible cerebrovascular manifestation of Fabry disease ref required
Radiographic features
To be classified as dolichoectasia, in the vertebrobasilar system, the basilar arterial diameter should be >4.5 mm 1.Smoker’s criteria use three quantitative measures of basilar artery morphology: basilar artery diameter, laterality, and height of bifurcation.9
Internal carotid artery dolichoectasia is particularly interesting because the artery typically already contains one hairpin turn. Seen in an MRI as two individual arteries at this hairpin, a carotid artery dolichoectasia can progress so far as to produce a second hairpin turn and appear as three individual arteries on an MRI. The pathogenesis is primarily related to compression of the optic nerves at the optic chiasma.
Complications
-<li>patients with <a href="/articles/autosomal-dominant-polycystic-kidney-disease-1">autosomal dominant polycystic kidney disease (ADPKD)</a> are more likely to be subject to dolichoectasia(s) <sup>ref required</sup>- +<li>patients with <a href="/articles/autosomal-dominant-polycystic-kidney-disease-1">autosomal dominant polycystic kidney disease (ADPKD)</a>, <a href="/articles/marfan-syndrome">Marfan Syndrome</a>, and <a href="/articles/ehlers-danlos-syndrome-2">Ehlers-Danlos Syndrome</a>, are more likely to be subject to dolichoectasia(s) <sup>9</sup>
-</ul><h4>Radiographic features</h4><p>To be classified as dolichoectasia, in the vertebrobasilar system, the <a href="/articles/basilar-artery">basilar arterial</a> diameter should be >4.5 mm <sup>1</sup>.</p><p><a href="/articles/internal-carotid-artery-1">Internal carotid artery</a> dolichoectasia is particularly interesting because the artery typically already contains one hairpin turn. Seen in an MRI as two individual arteries at this hairpin, a carotid artery dolichoectasia can progress so far as to produce a second hairpin turn and appear as three individual arteries on an MRI. The pathogenesis is primarily related to compression of the <a href="/articles/optic-nerve">optic nerves</a> at the optic chiasma. </p><h4>Complications</h4><ul><li><a href="/articles/neurovascular-compression-syndromes">neurovascular compression syndrome (NVCS)</a></li></ul>- +</ul><h4>Radiographic features</h4><p>To be classified as dolichoectasia, in the vertebrobasilar system, the <a href="/articles/basilar-artery">basilar arterial</a> diameter should be >4.5 mm <sup>1</sup>. <a href="/articles/smoker-s-criteria">Smoker’s criteria</a> use three quantitative measures of basilar artery morphology: basilar artery diameter, laterality, and height of bifurcation.<sup>9</sup></p><p><a href="/articles/internal-carotid-artery-1">Internal carotid artery</a> dolichoectasia is particularly interesting because the artery typically already contains one hairpin turn. Seen in an MRI as two individual arteries at this hairpin, a carotid artery dolichoectasia can progress so far as to produce a second hairpin turn and appear as three individual arteries on an MRI. The pathogenesis is primarily related to compression of the <a href="/articles/optic-nerve">optic nerves</a> at the optic chiasma. </p><h4>Complications</h4><ul><li><a href="/articles/neurovascular-compression-syndromes">neurovascular compression syndrome (NVCS)</a></li></ul>
References changed:
- 9. Gutierrez J, Sacco R, Wright C. Dolichoectasia—an Evolving Arterial Disease. Nat Rev Neurol. 2011;7(1):41-50. <a href="https://doi.org/10.1038/nrneurol.2010.181">doi:10.1038/nrneurol.2010.181</a>
Unable to process the form. Check for errors and try again.