Congenital absence of the bilateral internal carotid artery: a review of the associated (ab)normalities from a newborn status to the eighth decade of life.

Purpose: Due to the fact that the internal carotid artery (ICA) is responsible for nourishing two thirds of the brain volume, our aim was to inspect the morphofunctional consequences of the bilateral lack of this artery.

Methods: In order to examine this condition, we referred to both the library archive of our Faculty of Medicine and electronic databases of anatomical and clinical reports that included the following keywords: "absence," "aplasia," or "agenesis" in combination with "internal carotid artery," "common carotid artery," or only "carotid artery."

Result: We found 60 recorded cases of the bilateral ICA absence in the subjects of newborn status to the eighth decade of life, which had been discovered in 20 countries. The following ten parameters were described: the embryological base, terminology, history, incidence, general data, differential diagnosis, collateral circulation, the associated vascular aplasia and/or other variants, pathophysiology, and the importance in praxis.

Conclusion: This review noted all the cases of the bilateral ICA aplasia published for the past 104 years. Although there were 11.6% of cases of the associated cerebral aneurysms and 1-4 cases of 16 other diseases, approximately one quarter of the cases was without any pathology.