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Abstract

A 71 year-old female with an unremarkable medical history was referred for evaluation of progressive painless vision loss in her left eye. An outside corneal specialist had diagnosed Fuch's corneal dystrophy. She had undergone cataract extraction OS, DSEK OS, and then repeat DSEK OS after the initial graft failed. However, the patient's vision had not improved following the surgery. Rather, her vision had worsened. On examination, visual acuity was 20/30 OD and HM OS. External examination showed 4 mm proptosis OS and ptosis OS. Motility was full OU. There was a 2.8 log unit RAPD OS. Anterior segment examination showed changes consistent with Fuch's corneal dystrophy, an endothelial graft OS, and a partially dislocated posterior chamber intraocular lens OS. Dilated funduscopic examination showed mild temporal optic disc pallor OD and moderate optic disc pallor OS. Goldmann visual field testing showed an inferior arcuate defect with generalized depression OD and a small nasal island of vision OS. MRI orbits with contrast showed bilateral. On enhancing inferior orbital soft tissue masses involving both inferior recti and infraorbital nerves, extending into both cavernous sinuses. Although there was a broad differential diagnosis, the MRI findings were felt to be most consistent with orbital inflammatory disease, possibly IgG4-related disease. A laboratory work-up was obtained, including CBC, basic metabolic panel, ESR, CRP, ANCA, and syphilis serology. Most of the studies were unrevealing, but the ESR was elevated at 38 mm/hr. A diagnostic test was then performed

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