Case of Amaurosis Fugax in the Setting of a Persistent Primitive Hypoglossal Artery Requiring Carotid Endarterectomy with Regional Anesthesia

Abstract

Congenital defects/diseases Background:A persistent primitive hypoglossal artery (PPHA) is a rare congenital anomaly leading to persistent carotid-basilar anastomosis. This is a report of an 83-year-old man with a PPHA presenting with amaurosis fugax of the left eye requiring carotid endarterectomy under regional anesthesia. Case Report:An 83-year-old man presented with 2 weeks of intermittent self-resolving visual disturbances, followed by an episode of left eye amaurosis fugax. The patient had been referred to the hospital for further investigation of symptoms 1 day following the amaurosis fugax event. Carotid Doppler ultrasound demonstrated a greater than 90% stenosis of the left internal carotid artery. Computed tomography carotid and Circle of Willis angiography confirmed a mixed, ulcerated plaque and revealed a persistent left hypoglossal artery originating from the left internal carotid artery and continuing as the basilar artery. On day 3 of admission, left carotid endarterectomy was performed under conscious sedation and regional anesthesia to permit continuous monitoring of neurological status and avoid the need for intraoperative shunting. "Permissive hypertension" by targeting a systolic blood pressure of 190 to 200 mmHg was sought for the duration of clamp time. There was no deterioration of neurological function during clamping of the carotid vessels. The patient recovered well and was discharged 2 days after surgery, with no residual neurology. Conclusions:This report has presented a rare case of PPHA to highlight awareness of this congenital vascular anomaly when undertaking carotid endarterectomy.