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.

Department of Neurosurgery 2nd Medical Faculty Charles University and Motol University Hospital Praha Czechia

Department of Neurosurgery and Neurooncology 1st Medical Faculty Charles University and Military University Hospital Praha Czechia

Department of Radiology 48235Military University Hospital Praha Czechia

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