[Pathological tortuosity of intracranial arteries (pure arterial malformation) - diagnosis and management tactics].

Background: Tortuosity of intracranial arteries is rare and usually mistaken for arteriovenous malformations. In the world literature, the term «pure arterial malformations» is used to refer to this disease.

Objective: To summarize the experience of the Burdenko Neurosurgery Center on diagnosis of intracranial artery tortuosity, management and treatment of these patients, as well as to review appropriate literature data.

Material and methods: Tortuosity of intracranial arteries was detected in 11 patients (8 women and 3 men) aged 7-48 years who underwent outpatient or inpatient examination and treatment at the Burdenko Neurosurgery Center between 2009 and 2022. We analyzed angiographic, clinical and follow-up data of these patients, as well as appropriate literature data.

Results: According to angiography data, all patients had moderate dilatation, elongation and tortuosity of intracranial arteries without signs of arteriovenous shunting. The most common finding was tortuosity of several segments of internal carotid artery (5 cases). Lesion of PCA, PComA, MCA and ACA was less common. In 7 cases, the walls of the deformed vessels had calcified zones. In two cases, there were saccular aneurysms in the walls of the tortuous vessels. In one case, tortuosity was combined with kinking of the left subclavian artery, in another one - tortuosity of C1 segment of the right ICA. No patient had specific clinical manifestations. The follow-up period was 1-10 years in 7 patients. There were no changes in structure of tortuosity or appearance of new aneurysms.

Conclusion: Tortuosity of intracranial arteries is an extremely rare disease with the highest incidence in young women. This abnormality has no specific clinical manifestations and does not require surgical or conservative treatment. Tortuosity of intracranial arteries should be differentiated from arterial dolichoectasia, fusiform aneurysms and AVMs.