statdx/docs_md/articles/dolichoectasia_31d50b93-b057-4da3-86b5-4cc8fb0bc806.md

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Dolichoectasia	31d50b93-b057-4da3-86b5-4cc8fb0bc806	Brain Differential Diagnosis Arteries Anatomically Based Differentials Fusiform Arterial Enlargement

ESSENTIAL INFORMATION

• Key Differential Diagnosis Issues

  • Ectasia = elongated/tortuous artery
  • Fusiform aneurysm - Long-segment fusiform arterial dilatation - Can be acute (dissecting) or chronic (atherosclerosis, nonatherosclerotic vasculopathy)

• Helpful Clues for Common Diagnoses

  • Dolichoectasia - Dilated/elongated arteries ± slow flow - Vessel layers intact - Older patients, chronic hypertension - Vertebrobasilar > internal carotid artery (ICA) - Ectasia often extends into branches
  • Atherosclerotic Fusiform Aneurysm - Thick wall ± organized thrombus - Variable slow flow
  • Dissecting Aneurysm/Pseudoaneurysm - Focal arterial dilatation - Trauma = most common etiology - Next to hard/fixed structures (bone, dura)
  • Nonaneurysmal Dissection - Vertebral > basilar > ICA - Lacks changes of atherosclerosis in other vessels - Can be spontaneous or traumatic

• Helpful Clues for Less Common Diagnoses

  • CNS Vasculitis - Involves multiple vessels - Alternating stenoses and fusiform dilatations
  • Inherited Connective Tissue Disorders - Abnormal connective tissue involves vessel walls - Aneurysm and dissections - Thoracic aorta most common location
  • HIV Infection - Vasculopathy may be present in children or adults - Multifocal fusiform enlargement involving supraclinoid ICA and basilar artery - No consistent infectious agent isolated

• Helpful Clues for Rare Diagnoses

  • Giant Serpentine Aneurysm - Large, partially thrombosed mass - Distal branches arise from aneurysm dome - Lacks definable neck - ICA/middle cerebral artery > vertebrobasilar artery
  • Atypical Saccular Aneurysm - Arises from vessel bifurcations - Long "aspect ratio" → fusiform appearance - Often multilobulated, bizarre

Images

Selected Images

Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.

Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.

Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.

Coronal CTA shows fusiform dilatation of the right supraclinoid internal carotid artery (ICA). Irregularity from atherosclerotic disease can be seen of the M1 segment of the middle cerebral artery . No significant mural thrombus was noted in this fusiform aneurysm.

Dissecting pseudoaneurysm in the V4 segment of the right vertebral artery seen on 3D TOF MRA , T2 , and T1 pre- and post DANTE VWI sequences shows peripheral enhancement, suggestive of instability.

3D MIP MRA of the vertebrobasilar arteries in a teenage female with a history of type 4 Ehlers-Danlos shows fusiform dilatation of the vertebral artery . The affected gene is COL3A1, and this specific type of Ehlers-Danlos has a higher risk of aneurysm and vascular rupture.

Axial MIP from CT arteriography shows fusiform dilatation of the left middle cerebral artery bifurcation in this child with a history of Marfan syndrome.

Coronal MIP reformat from CT arteriography shows bilateral fusiform aneurysms of supraclinoid ICAs . This patient also had thoracic aortic aneurysm, which is consistent with familial thoracic aortic aneurysm and dissection and is associated with a mutation of ACTA2. This gene is responsible for a component of vascular smooth muscle.

Axial T2WI MR shows strikingly enlarged middle cerebral arteries in this child with congenital HIV/AIDS (an uncommon but well-recognized cause of pediatric fusiform arteriopathy). The stroke-like presentations of HIV infection may relate to vasculopathies, including large-vessel aneurysmal vasculopathy.

Axial NECT demonstrates a giant serpentine aneurysm in the basilar artery with associated mural thrombus seen on sagittal CTA.

Additional Images

Sagittal T1WI MR shows an elongated basilar artery with a slow-flow, thickened wall . The apex of the tortuous basilar artery indents the hypothalamus, 3rd ventricle .

Axial T2WI MR shows an elongated, tortuous basilar artery with a thickened arterial wall , typical for atherosclerosis-associated fusiform ectasia.

Lateral angiography shows a large fusiform middle cerebral artery aneurysm that extends into smaller, more distal branches . This is an unusual example because of the location (ICA, middle cerebral artery).

Axial T1WI MR shows an enlarged right vertebral artery with high signal intensity as well as an absent flow void of the left vertebral artery .

Anteroposterior oblique view of the left vertebral angiogram shows focal elongations and widening of the basilar artery in a 6-year-old child with Ehlers-Danlos type 4.

Axial MRA submentovertex view shows an unusual nonatherosclerotic giant serpentine fusiform aneurysm. The patent channel lies within the clot in the partially thrombosed lumen.

Lateral angiography in 30-year-old man with a subarachnoid hemorrhage shows an elongated, bizarre-appearing, multilobulated aneurysm with long aspect ratio, tit-like projections.