Takayasu Arteritis Presenting as Orbital Apex Syndrome With Irreversible Monocular Blindness and Long-Segment Right Carotid Stenosis: A Case Report With 58 Months of Follow-Up

Abstract

Background: Takayasu arteritis is a granulomatous large-vessel vasculitis that usually becomes apparent only after stenotic damage has occurred and can be difficult to recognise when classical peripheral vascular signs are absent.

Objective: To describe the clinical course, diagnostic challenges and long-term outcome of Takayasu arteritis presenting as orbital apex syndrome with irreversible monocular blindness and long-segment right carotid stenosis.

Case Presentation: A 36-year-old man presented in August 2020 with a chronic progressive right hemicranial headache that acutely worsened and was followed by complete loss of vision in the right eye. Examination showed right optic nerve dysfunction, ptosis, proptosis, absent pupillary reflexes and complete ophthalmoplegia involving the third, fourth and sixth cranial nerves, a constellation corresponding to orbital apex syndrome. There were no constitutional symptoms, no limb claudication and no significant inter-arm blood pressure difference. Contrast-enhanced computed tomography of the brain and orbits was unrevealing. Computed tomography angiography seven days later showed long-segment luminal irregularity and stenosis of the right common carotid artery extending continuously into the cervical, petrous, cavernous and supraclinoid internal carotid segments, with a preserved contralateral carotid and posterior circulation; the erythrocyte sedimentation rate was elevated. Takayasu arteritis was diagnosed and treated with high-dose glucocorticoid followed by azathioprine and antiplatelet therapy. The headache resolved but the visual loss did not. Applied retrospectively, the 2022 American College of Rheumatology/EULAR criteria yielded one point against a threshold of five, because three examination items were never recorded and aortic imaging was never done. At 58 months the patient was clinically stable but had grade 3 lymphopenia and grade 1 thrombocytopenia.

Conclusion: Takayasu arteritis should be considered in young adults with progressive headache and focal neuro-ophthalmic deficits even when pulses and blood pressures are symmetrical.