Cerebral Aneurysms with Internal Carotid Artery Agenesis: A Unique Case Similar to Moyamoya Disease and Literature Review

Abstract

Internal carotid artery (ICA) agenesis/aplasia is occasionally accompanied with cerebral aneurysms caused by hemodynamic stress. If the aneurysms are located around the circle of Willis, they are managed with clipping or coil embolization. Herein, we report a case of ICA agenesis with perforating artery aneurysms treated successfully with revascularization. Moreover, a literature review of ICA agenesis with cerebral aneurysms was performed to compare with the current case. We conducted a literature review using data from PubMed. A secondary search was also performed by reviewing the references of each article previously searched. In our case, the aneurysms shrank and disappeared after direct and indirect bypass surgeries, and indirect bypass developed as in moyamoya disease (MMD). The epidemiological and clinical features of aneurysms accompanied with ICA agenesis were identified via a literature review. Aneurysms with ICA agenesis categorized as type F based on the Lie classification system, or referred to as rete mirabile, are occasionally located in an untreatable site; hence, they cannot be treated with clipping or coil embolization. Moreover, results showed that previous studies did not use revascularization for the treatment of aneurysm. In conclusion, if an aneurysm with ICA agenesis is difficult to approach directly or via an endovascular procedure, revascularization can be a treatment option.

A Rare and Unusual Cause of Ischemic Stroke to Be Aware of

“…ICA agenesis can occur as an isolated vascular defect as seen in our case, but the literature points to a growing association with cerebral aneurysms, with an increased risk of 25-67% [ 16 ]. A study by Zink et al analyzed 165 cases with ICA aplasia or hypoplasia and found that nearly 28% of patients had associated cerebral aneurysms.…”

“…While the exact mechanism linking ICA agenesis with the increased risk of arterial aneurysms is not exactly known, one theory that has been proposed is that the lack of the ICA induces increased Johal et al J Med Cases. 2022;13 (11):536-540 hemodynamic stress due to increased flow through collateral arteries which can, in turn, cause hemorrhage [16].…”

“…Additionally, the fact there was a higher incidence of cerebral aneurysms in patients over 30 years old, suggests that these are not simply a concurrent structural anomaly, but rather an acquired etiology [ 17 ]. While the exact mechanism linking ICA agenesis with the increased risk of arterial aneurysms is not exactly known, one theory that has been proposed is that the lack of the ICA induces increased hemodynamic stress due to increased flow through collateral arteries which can, in turn, cause hemorrhage [ 16 ].…”

Bilateral Internal Carotid Artery Hypoplasia with Craniofacial Anomalies: A Case of Suspected Treacher Collins Syndrome

“… 2 ) Therefore, it is important to screen for intracranial aneurysms in patients with ICA aplasia or hypoplasia. 3 , 4 )…”

“…2) Therefore, it is important to screen for intracranial aneurysms in patients with ICA aplasia or hypoplasia. 3,4) Some abnormalities of the craniocervical arteries are as-sociated with neurocristopathy, a developmental abnormality of neural crest cells that can present with vascular abnormalities and craniofacial malformations. 5,6) The Goldenhar syndrome (GS), caused by abnormalities in the first and second branchial arches, is a type of neurocristopathy.…”