Eagle syndrome an overlooked cause of internal carotid artery dissection, Case series

Background

Eagle syndrome is a rare condition with a prevalence estimated at 4%, with 0.16% exhibiting symptoms of Eagle’s syndrome resulting from an elongated styloid process or a calcified stylohyoid ligament, compressing over nearby vessels and nerves. Symptoms range from simple headache, neck pain, and odynophagia to cerebral ischemia. Since it can be an incidental finding, management is controversial and ranges from conservative treatment to surgical intervention, including internal carotid stenting and styloidectomy.

Clinical presentation

We report two cases of Eagle’s syndrome. Case one (Vascular variant): 44 years old presented with left-sided weakness with Magnetic Resonance Imaging and an angiogram of the brain showing a right middle cerebral artery hemispheric infarct with a right internal carotid artery (ICA) dissection and elongated styloid processes more on the left. The patient’s condition improved with supportive care. He was treated with aspirin, atorvastatin, and an intensive 16-week rehabilitation program. Case two (Classic variant): 41 years old presented with refractory occipital headache and neck pain without vascular insult. Magnetic resonance of both patients showed a long styloid process.

Conclusions

Eagle’s Syndrome is a rare entity and perhaps overlooked as an anatomical variant that ought to be considered a possible etiology in atraumatic ICA dissection in patients with no discernable risk factors. As observed in case one, with atraumatic ICA dissection and unremarkable connective tissue disease work-up, highlighting anatomical variant, as an etiology to his ischemic stroke. Styloidectomy should be considered to avert risk of recurrent IC dissections in such patients with atraumatic IC dissection and classic eagle’s syndrome with refractory orofacial pain syndromes exacerbated by head movement or yawning.