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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Agenesis of Internal Carotid Artery

  • Background:
    • Internal carotid artery agenesis, hypoplasia, and aplasia are rare congenital anomialies occuring in only 0.01% of the general population.
    • Agenesis of internal carotid artery increases the risk of developing aneurysms due to increased flow through the collateral arteries.
    • Major collateral circulation may be recruited from the circle of Willis, embryonic vessels, or transcranial collaterals from the external carotid artery (ECA).
  • Clinical Presentation:
    • Patients may be asymptomatic or present with decreased visual acuity, unilateral weakness and numbness, slurred speech, headache, and drowsiness.
    • Agenesis of internal carotid artery may occur as a component of PHACE syndrome.
  • Key Diagnostic Features:
    • Lack of ICA enhancement associated with absence of the petrous carotid canal within the skull base
    • Abnormal ECA or collateral arteries are demonstrated on common carotid artery angiography.
  • Differential Diagnosis:
    • Internal carotid artery thrombosis
    • ICA stenosis
    • Moyamoya disease
    • ICA vasospasm
February 2017

A 41-year-old man with blurry vision and headaches

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Print ISSN: 0195-6108 Online ISSN: 1936-959X

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