Pure arterial malformation

Last revised by Frank Gaillard on 9 Apr 2024

Citation, DOI, disclosures and article data

Citation:

Rasuli B, Gaillard F, Pure arterial malformation. Reference article, Radiopaedia.org (Accessed on 21 May 2024) https://doi.org/10.53347/rID-187028

DOI:

https://doi.org/10.53347/rID-187028

Permalink:

https://radiopaedia.org/articles/187028

rID:

187028

Article created:

1 Apr 2024, Bahman Rasuli ◉

Disclosures:

At the time the article was created Bahman Rasuli had no financial relationships to ineligible companies to disclose.

View Bahman Rasuli's current disclosures

Last revised:

9 Apr 2024, Frank Gaillard ◉ ◈

Disclosures:

At the time the article was last revised Frank Gaillard had no financial relationships to ineligible companies to disclose.

View Frank Gaillard's current disclosures

Revisions:

5 times, by 2 contributors - see full revision history and disclosures

Pure arterial malformations are very rare and characterized by mass-like dilated, overlapping, and tortuous arteries without arteriovenous shunting and no capillary or venous components. This distinguishes them from arteriovenous malformations and dural arteriovenous fistulas. Furthermore, the length and/or number of arterial loops do not conform to normal anatomy, as is the case with dolichoectasia.

Pure arterial malformations are uncommon, and only a few cases have been reported. Typically, these are found by chance during cranial cross-sectional imaging performed for other reasons. Due to the limited number of reported cases, strong epidemiological data is unavailable, however, the majority of cases have been diagnosed in younger individuals (adolescents and young adults) with a modest predilection for females ^1,2.

Clinical presentation

Most pure arterial malformations are asymptomatic and discovered incidentally. Nonetheless, a variety of symptoms have been reported, including headaches, seizures, dizziness and hemiplegia ^1,5,6.

Pathology

The key distinguishing feature of pure arterial malformations is that they are isolated to the proximal artery, without communication with veins and without arteriovenous shunting. ^1,4,8. They may, however, be associated with regional aneurysms ^1-3.

Their etiology is not understood and remains a source of debate ¹. Possibilities include congenital dysplasia and acquired injuries (e.g. viral infections, or healed arterial dissection) ^1,7.

Although pure arterial malformation can occur in any blood vessel within the brain, it most commonly affects the supraclinoid internal carotid artery and adjacent posterior communicating artery, M1 segment of the middle cerebral artery or A1 segment of the anterior cerebral artery ¹.

Radiographic features

CT

The abnormality appears as a blood-density serpignious mass, often in the interpeduncular or suprasellar cistern. There may be calcification in the walls.

Vascular imaging

CT angiography, MR angiography and catheter angiography reveal dilated tortuous vessels in the the subarachnoid space without shunting on nidus ^1,2. Associated aneursymal dilatation may be present.

Treatment and prognosis

The management of pure arterial malformation is still a topic of debate although the vast majority of reported cases have been treated conservatively and most remained asymptomatic ¹. However, if the malformation includes a local aneurysm, surgery or endovascular treatment may be necessary ¹.