Symptomatic internal carotid artery stenosis in the presence of a persistent primary hypoglossal artery

Objectives Anatomic variations of the extracranial carotid artery are rare. Persistent primitive hypoglossal artery appears with a reported incidence between 0.03% and 0.2%. We report a case of recurrent transient ischemic attacks originating from proximal internal carotid artery stenosis associated with ipsilateral persistent primitive hypoglossal artery and give a review of the existing literature. Methods A 78-year-old patient with a medical history of two previous transient ischemic attacks consulted our emergency department with an acute left hemispheric stroke. Intravenous thrombolysis permitted complete resolution of symptoms. Concurrent Computed Tomography (CT) and Magnetic Resonance (MR) angiography revealed an unstable plaque causing 50% stenosis of the left internal carotid artery with a persistent primitive hypoglossal artery dominantly perfusing the posterior circulation, and bilateral hypoplastic vertebral arteries. Results Uneventful carotid artery stenting using a proximal protection device was performed, and the patient was discharged after 12 days. Six months follow-up was uneventful with a patent stent in the internal carotid artery. Conclusions Treatment of symptomatic carotid artery stenosis in the presence of persistent primitive hypoglossal artery is challenging. Management should be driven by patients’ co-morbidities, the anatomical localization of the lesions and local expertise. In the case of a high origin of the persistent primary hypoglossal artery, carotid artery stenting with the use of a proximal cerebral protection device is probably the preferred and simplest approach.