Intracranial Dolichoectasias
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The term dolichoectasia means elongation and distension. It is used to characterize arteries throughout the human body which have shown significant deterioration of their
tunica intima The tunica intima (Neo-Latin "inner coat"), or intima for short, is the innermost tunica (biology), tunica (layer) of an artery or vein. It is made up of one layer of endothelium, endothelial cells (and macrophages in areas of disturbed blood flo ...
(and occasionally the tunica media), weakening the vessel walls and causing the artery to elongate and distend.


Signs and symptoms

Vertebrobasillar dolichoectasia *
Hemifacial spasm Hemifacial spasm (HFS) is a rare neuromuscular disease characterized by irregular, involuntary muscle contractions (spasms) on one side (hemi-) of the face (-facial). The facial muscles are controlled by the facial nerve (seventh cranial nerve), ...
*
Paresis In medicine, paresis (), compound word from Greek , (πᾰρᾰ- “beside” + ἵημι “let go, release”), is a condition typified by a weakness of voluntary movement, or by partial loss of voluntary movement or by impaired movement. Whe ...
Internal carotid dolichoectasia * Progressive visual field defect


Cause

Most commonly caused by
hypertension Hypertension, also known as high blood pressure, is a Chronic condition, long-term Disease, medical condition in which the blood pressure in the artery, arteries is persistently elevated. High blood pressure usually does not cause symptoms i ...
, continued stress on the walls of the artery will degrade the vessel wall by damaging and loosening the collagen and elastin meshwork which comprises the intima. Similarly, hypercholesterolemia or hyperlipidemia can also provide sufficient trauma to the vessel wall resulting in dolichoectasia. 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 mold and force the artery to now take on an elongated, tortuous course to better withstand the higher pressures.


Pathophysiology

Most commonly affected are the
vertebral Each vertebra (: vertebrae) is an irregular bone with a complex structure composed of bone and some hyaline cartilage, that make up the vertebral column or spine, of vertebrates. The proportions of the vertebrae differ according to their spina ...
and basilar arteries (Vertebral Basilar Dolichoectasia or Vertebrobasillar Dolichoectasia). The
internal carotid artery The internal carotid artery is an artery in the neck which supplies the anterior cerebral artery, anterior and middle cerebral artery, middle cerebral circulation. In human anatomy, the internal and external carotid artery, external carotid ari ...
may also be affected. Patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD) are more likely to be subject to dolichoectasias. Dolichoectasias are most common in elderly males. In cases involving the basilar artery (VBD), pathology can occur due to direct compression of cranial nerves, by ischemia related to the dolichoectatic vessel, or by the development of hydrocephalus. Rupture of the dolichoectatic vessel can lead to catastrophic
intracerebral hemorrhage Intracerebral hemorrhage (ICH), also known as hemorrhagic stroke, is a sudden bleeding into Intraparenchymal hemorrhage, the tissues of the brain (i.e. the parenchyma), into its Intraventricular hemorrhage, ventricles, or into both. An ICH is ...
. Internal carotid artery dolichoectasia is particularly interesting because the artery normally 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. In the case of a dolichoectasia of the Internal Carotid Artery (ICD), the pathogenesis is primarily related to compression of the optic nerves at the
optic chiasma In neuroanatomy, the optic chiasm, or optic chiasma (; , ), is the part of the brain where the optic nerves cross. It is located at the bottom of the brain immediately inferior to the hypothalamus. The optic chiasm is found in all vertebrates, ...
(see Fig. 1 and 2).


Diagnosis


Treatment

There are no standard treatments for existing dolichoectasias. Prevention is most important, usually related to control of high blood pressure. Interventions can include medications or lifestyle changes.


References


External links


Relation between ADPKD and Dolichoectasia
{{commons category Central nervous system disorders Diseases of arteries, arterioles and capillaries