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Internal carotid absence

ORPHA:981· ICD-10 Q28.1

Definition

A rare vascular anomaly characterized by agenesis (complete absence of the carotid canal), aplasia (a remnant of the artery remains) or hypoplastic (incomplete embryonic development) of the internal carotid artery, which can be unilateral or bilateral. Children with the condition are mostly asymptomatic due to the presence of collateral circulation. With increasing age, the risk of cerebrovascular events increases. Patients may present with headache, seizures, or transient ischemic attacks. Cerebral aneurysms may form in the context of the condition.

Prevalence
<1 / 1 000 000
Inheritance
Not applicable
Age of onset
All ages