Takayasu arteritis is a large-vessel vasculitis that may cause oculo-cerebral ischemia. We report a patient with visual loss as initial manifestation, and discuss transcranial Doppler (TCD) findings before and after surgical revascularization.
A 19-year-old female developed episodes of transient positional right vision loss, progressing to permanent right vision loss and bright light-induced left amaurosis. Examination demonstrated subclavian bruits, palpable epigastrium aortic pulsation, faint radial pulses, right retinal pallor, arteriolar narrowing, and bilateral boxcarring. Head and neck computed tomography angiogram demonstrated left subclavian origin and right common carotid artery occlusion, and severe innominate and left common carotid artery stenosis. TCD demonstrated right ophthalmic artery flow reversal and nonpulsatile waveforms with dampened spectra in the right anterior circulation. Corticosteroids, methotrexate, infliximab, and dual-antiplatelet therapy were initiated. Eleven weeks later, the patient underwent ascending aortic aneurysm repair and bilateral carotid artery bypass with an aortic graft. Pathology was consistent with chronic active Takayasu arteritis. Two weeks postoperatively, left eye visual symptoms resolved; right visual loss persisted. Postoperative TCD showed marked improvement in cerebral perfusion.
Retinal ischemia in young women should prompt emergent inflammatory and systemic vascular evaluation. In our subject, prolonged right retinal ischemia had dismal prognosis despite carotid-aortic revascularization, whereas left retinal boxcarring reversed. Surgical revascularization is recommended for severely symptomatic oculo-cerebrovascular disease, once inflammation is better controlled with immunosuppressive therapy. TCD documented the presence and monitored the subsequent resolution of blood flow changes causing retinal and brain hypoxia.
Departments of *Neurology
†Laboratory Medicine and Pathology, Mayo Clinic Arizona, Scottsdale, AZ
‡Department of Neurology, Cedars-Sinai Medical Center, Los Angeles, CA
The authors declare no conflict of interest.
Correspondence to: Michael E. Christiansen, MD, Department of Neurology, Mayo Clinic Arizona, 13400 E. Shea Blvd., Scottsdale, AZ 85259. E-mail: firstname.lastname@example.org.