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A 57-year-old woman with periocular pain
Digital Journal of Ophthalmology 2021
Volume 27, Number 3
September 3, 2021
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Bryan Strelow, MD, MA | Department of Ophthalmology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
Michelle Nguyen, BS | School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
Meredith R. Klifto, MD | Department of Ophthalmology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
Diagnosis and Discussion
Eagle syndrome should be considered in the differential diagnosis for nonophthalmic periocular pain. Although management of this disease is beyond the typical scope of practice for ophthalmology, knowledge of the nonophthalmic differential for angle closure symptoms is important for ophthalmologists.

Eagle syndrome, also known as stylohyoid syndrome, was first described by Eagle in 1937 and has since been associated with multiple complications, including carotid compression (presyncope and syncope) and cervicofacial pain.(2-3) The syndrome is characterized by calcification of the stylohyoid ligament or elongation of the styloid process, either by congenital abnormality or acquired (ie, following trauma). The incidence of stylohyoid abnormalities has been reported to be 22%-84%; however, the incidence of associated pain is only 4%-10%. In such cases, anatomic abnormalities are often present bilaterally, yet pain typically presents unilaterally.(4) Eagle syndrome typically presents with vague cervicofacial pain complaints and, as in our case, has been described as mimicking other diseases.(5,6)

Intermittent periocular headache is a common and nonspecific clinical symptom. However, in the setting of other ophthalmic findings, such as anatomically narrow angles, gonioscopic synechiae, elevated IOP, or glaucomatous optic neuropathy, this symptom is suggestive of intermittent angle closure. In patients who present with normal IOP during their routine office visits, persistent pain suggestive of angle closure presents a clinical challenge that requires prophylactic treatment or continued workup of alternative etiologies. The ultimate diagnosis of Eagle syndrome in our patient helped to clarify the pathology the patient’s pain and also freed the clinical team from anchoring on angle closure as the primary etiology of the patient’s symptoms. Additionally, our patient did ultimately present with glaucomatous optic neuropathy, and the history of this angle pathology confounded the work-up of her nonspecific pain symptoms, which were at least in part secondary to nonophthalmic disease. To our knowledge, this case is the first report of Eagle syndrome masquerading as intermittent angle closure.

Literature Search
PubMed was searched on June 22, 2020, for English-language results, using the following terms: Eagle syndrome and glaucoma.