Congenital internal carotid artery hypoplasia: A systematic review

Abstract

This review evaluates the current evidence for the clinical management of congenital internal carotid artery hypoplasia (CICAH). We summarise clinical presentations diagnostic standards, imaging recommendations, treatment and follow-up. The review was prompted by a case of CICAH in a 50-year-old female who presented to our neurosurgery clinic with an acute episode of vertigo. The patient underwent CT angiogram, which showed an unusually low right carotid bifurcation. The right internal carotid artery (ICA) was hypoplastic, and the A1 segment of the anterior cerebral artery (ACA) was absent. Skull base CT showed an ipsilateral hypoplastic carotid canal. To summarise current evidence for clinical management of CICAH we followed PRISMA guidelines to identify papers meeting our predefined inclusion criteria. We searched three databases using the terms ‘ICA’ and ‘Hypoplasia’. We reviewed 41 papers meeting our criteria. 34 were clinical reports. We performed a data extraction and quality appraisal on these reports. We found that CICAH may be less rare than previously described. Blood pressure control in CICAH is crucial due to the increased risk of stroke and aneurysm formation. Follow-up imaging is strongly recommended. Carotid doppler sonography is a powerful and underutilised diagnostic tool, and carotid canal hypoplasia is not a pathognomic sign. In conclusion, clinicians should be alert to anatomic variations such as CICAH because these produce haemodynamic changes that may have serious clinical consequences. We recommend a central registry of patients with CICAH in order to understand the longer-term natural history of the condition.

A Case Report of Migraine With Hypoplasia of the Internal Carotid Artery

“…The internal carotid artery rarely exhibits developmental mutations. Recognition of such aberrations becomes paramount, especially when considering potential endovascular interventions for cerebrovascular events necessitating revascularization [ 4 , 5 ]. While cases of the hypoplastic internal carotid artery are occasionally encountered due to collateral circulation compensation into the anterior and middle cerebral arteries, clinical symptoms are typically absent.…”

“…The vascular consequences of hypoplastic internal carotid artery encompass a spectrum of intracranial events [ 5 ]. Aneurysm formation within the posterior circulation, driven by hemodynamic stress through collateral circulation, is a prevalent outcome.…”