Title : Transient vision loss secondary to carotid artery dissection: An atypical presentation of vascular eagle syndrome
Abstract:
Vascular Eagle Syndrome is a rare but clinically significant condition caused by an elongated styloid process or calcified stylohyoid ligament compressing the internal carotid artery (ICA), potentially resulting in vessel wall injury, dissection, and thromboembolic events. We report a rare case of vascular Eagle Syndrome in a 54-year-old female with Ehlers-Danlos Syndrome (EDS), who presented with progressive right eye vision loss over two days, culminating in near-complete monocular blindness described as “all grey.” Her vision spontaneously improved within 24 hours of hospitalization. She denied typical systemic or neurologic symptoms such as headache, fever, limb weakness, or jaw claudication. Examination showed partial right eye visual impairment with preserved pupillary reflexes and no focal (CRAO) and giant cell arteritis (GCA). CT angiography of the neck revealed a short segment dissecting pseudoaneurysm of the distal right ICA without significant luminal narrowing, adjacent to mildly elongated bilateral styloid processes and calcified stylohyoid ligaments. These findings, along with the absence of traditional atherosclerotic risk factors and her underlying connective tissue disorder, supported the diagnosis of vascular Eagle Syndrome. She was started on aspirin and clopidogrel and referred for neurosurgical evaluation for potential styloidetomy. This case emphasizes the need to consider vascular Eagle Syndrome in patients with atypical neurovascular symptoms and structural predispositions. Early imaging. Thorough clinical evaluation, and multidisciplinary collaboration are critical for accurate diagnosis and prevention of serious complications such as stroke or sudden death.
