Proposed new classification for internal carotid artery segmental agenesis based on embryologic and angiographic correlation

Background and purpose: Internal carotid artery (ICA) agenesis is a rare anatomical variant that can involve different segments of the ICA. Although many authors proposed their own classifications of this variant basing on different criteria, none of these allows to include all the cases described in the literature. The aim of the authors is to propose a new classification that allows to include all cases of ICA agenesis described until now. Materials and methods: The study is based on the review of all the cases of ICA agenesis described in the literature and of the classifications already proposed. After the analysis of these cases, we looked for the limits of each classification to elaborate a new more complete one. Results: We found 228 cases of ICA agenesis. Among them 40 were not includible in the existing classifications. For this reason, we proposed a new classification based on the type of flow compensation and on the embryological events that determine the different variants. The flow is compensated in Type I by the Circle of Willis; in Type II by the non-regression of an embryonic artery; in Type III by the presence of an arterio-arterial anastomosis and in Type IV by external-internal carotid arteries anastomoses. Conclusion: After the literature review, we proposed a new comprehensive classification based on the detailed analysis of arterial embryology. Even if all the embryological details that determine this complex variant are not completely understood yet, this classification allows to include all the cases described in literature.