127 lines
13 KiB
Markdown
127 lines
13 KiB
Markdown
---
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title: "Dolichoectasia"
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docid: "31d50b93-b057-4da3-86b5-4cc8fb0bc806"
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breadcrumbs:
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- "Brain"
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- "Differential Diagnosis"
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- "Arteries"
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- "Anatomically Based Differentials"
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- "Fusiform Arterial Enlargement"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- Ectasia = elongated/tortuous artery
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- Fusiform aneurysm
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- Long-segment fusiform arterial dilatation
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- Can be acute (dissecting) or chronic (atherosclerosis, nonatherosclerotic vasculopathy)
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- ## Helpful Clues for Common Diagnoses
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- **Dolichoectasia**
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- Dilated/elongated arteries ± slow flow
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- Vessel layers intact
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- Older patients, chronic hypertension
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- Vertebrobasilar > internal carotid artery (ICA)
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- Ectasia often extends into branches
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- **Atherosclerotic Fusiform Aneurysm**
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- Thick wall ± organized thrombus
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- Variable slow flow
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- **Dissecting Aneurysm/Pseudoaneurysm**
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- Focal arterial dilatation
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- Trauma = most common etiology
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- Next to hard/fixed structures (bone, dura)
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- **Nonaneurysmal Dissection**
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- Vertebral > basilar > ICA
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- Lacks changes of atherosclerosis in other vessels
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- Can be spontaneous or traumatic
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- ## Helpful Clues for Less Common Diagnoses
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- **CNS Vasculitis**
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- Involves multiple vessels
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- Alternating stenoses and fusiform dilatations
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- **Inherited Connective Tissue Disorders**
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- Abnormal connective tissue involves vessel walls
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- Aneurysm and dissections
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- Thoracic aorta most common location
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- **HIV Infection**
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- Vasculopathy may be present in children or adults
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- Multifocal fusiform enlargement involving supraclinoid ICA and basilar artery
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- No consistent infectious agent isolated
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- ## Helpful Clues for Rare Diagnoses
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- **Giant Serpentine Aneurysm**
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- Large, partially thrombosed mass
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- Distal branches arise from aneurysm dome
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- Lacks definable neck
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- ICA/middle cerebral artery > vertebrobasilar artery
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- **Atypical Saccular Aneurysm**
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- Arises from vessel bifurcations
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- Long "aspect ratio" → fusiform appearance
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- Often multilobulated, bizarre
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## Images
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### Selected Images
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*Axial CT shows fusiform dilatation and tortuosity of the basilar artery <img src='/img/arrows/CS.png'/> in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.*
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*Axial CT shows fusiform dilatation and tortuosity of the basilar artery <img src='/img/arrows/CS.png'/> in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.*
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*Axial CT shows fusiform dilatation and tortuosity of the basilar artery <img src='/img/arrows/CS.png'/> in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.*
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*Coronal CTA shows fusiform dilatation <img src='/img/arrows/CS.png'/> of the right supraclinoid internal carotid artery (ICA). Irregularity from atherosclerotic disease can be seen of the M1 segment of the middle cerebral artery <img src='/img/arrows/CO.png'/>. No significant mural thrombus was noted in this fusiform aneurysm.*
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*Dissecting pseudoaneurysm in the V4 segment of the right vertebral artery seen on 3D TOF MRA <img src='/img/arrows/WS.png'/>, T2 <img src='/img/arrows/BS.png'/>, and T1 pre- <img src='/img/arrows/CS.png'/> and post <img src='/img/arrows/CO.png'/> DANTE VWI sequences shows peripheral enhancement, suggestive of instability.*
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*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 <img src='/img/arrows/CS.png'/>. The affected gene is COL3A1, and this specific type of Ehlers-Danlos has a higher risk of aneurysm and vascular rupture.*
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*Axial MIP from CT arteriography shows fusiform dilatation of the left middle cerebral artery bifurcation <img src='/img/arrows/CS.png'/> in this child with a history of Marfan syndrome.*
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*Coronal MIP reformat from CT arteriography shows bilateral fusiform aneurysms of supraclinoid ICAs <img src='/img/arrows/CS.png'/>. 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.*
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*Axial T2WI MR shows strikingly enlarged middle cerebral arteries <img src='/img/arrows/CS.png'/> 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.*
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*Axial NECT demonstrates a giant serpentine aneurysm in the basilar artery <img src='/img/arrows/WC.png'/> with associated mural thrombus <img src='/img/arrows/CC.png'/> seen on sagittal CTA.*
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### Additional Images
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*Sagittal T1WI MR shows an elongated basilar artery with a slow-flow, thickened wall <img src='/img/arrows/WS.png'/>. The apex of the tortuous basilar artery indents the hypothalamus, 3rd ventricle <img src='/img/arrows/WO.png'/>.*
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*Axial T2WI MR shows an elongated, tortuous basilar artery with a thickened arterial wall <img src='/img/arrows/BS.png'/>, typical for atherosclerosis-associated fusiform ectasia.*
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*Lateral angiography shows a large fusiform middle cerebral artery aneurysm <img src='/img/arrows/BS.png'/> that extends into smaller, more distal branches <img src='/img/arrows/BO.png'/>. This is an unusual example because of the location (ICA, middle cerebral artery).*
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*Axial T1WI MR shows an enlarged right vertebral artery with high signal intensity <img src='/img/arrows/WS.png'/> as well as an absent flow void of the left vertebral artery <img src='/img/arrows/WC.png'/>.*
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*Anteroposterior oblique view of the left vertebral angiogram shows focal elongations and widening of the basilar artery <img src='/img/arrows/BS.png'/> in a 6-year-old child with Ehlers-Danlos type 4.*
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*Axial MRA submentovertex view shows an unusual nonatherosclerotic giant serpentine fusiform aneurysm. The patent channel <img src='/img/arrows/WS.png'/> lies within the clot in the partially thrombosed <img src='/img/arrows/WO.png'/> lumen.*
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*Lateral angiography in 30-year-old man with a subarachnoid hemorrhage shows an elongated, bizarre-appearing, multilobulated aneurysm <img src='/img/arrows/BS.png'/> with long aspect ratio, tit-like projections.*
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