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 (New Latin "inner coat"), or intima for short, is the innermost tunica (layer) of an artery or vein. It is made up of one layer of endothelial cells and is supported by an internal elastic lamina. The endothelial cells are i ...

(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), whi ...

Paresis In medicine, paresis () is a condition typified by a weakness of voluntary movement, or by partial loss of voluntary movement or by impaired movement. When used without qualifiers, it usually refers to the limbs, but it can also be used to desc ...

Trigeminal neuralgia Trigeminal neuralgia (TN or TGN), also called Fothergill disease, tic douloureux, or trifacial neuralgia is a long-term pain disorder that affects the trigeminal nerve, the nerve responsible for sensation in the face and motor functions such as ...

Internal carotid dolichoectasia * Progressive visual field defect

Cause

Most commonly caused by hypertension, 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 The vertebral column, also known as the backbone or spine, is part of the axial skeleton. The vertebral column is the defining characteristic of a vertebrate in which the notochord (a flexible rod of uniform composition) found in all chordates ...

and basilar arteries (Vertebral Basilar Dolichoectasia or Vertebrobasillar Dolichoectasia). The internal carotid artery 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. 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 vertebrate ...

(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