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JAMA Clinical Challenge | Clinician's Corner

Ptosis, Erythema, and Rapidly Decreasing Vision

Adam Zanation, MD; David Fleischman, MD; Sai H. Chavala, MD
JAMA. 2013;309(22):2382-2383. doi:10.1001/jama.2013.5517.
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A previously healthy 63-year-old white man presents to the emergency department with a swollen right eye and complete ptosis. Three weeks prior, the patient was evaluated for sinusitis by a local otolaryngologist. Cultures were performed on sinonasal aspirates and empirical ciprofloxacin and oral prednisone were initiated. The patient's symptoms, however, worsened. Cultures revealed Enterobacter aerogenes. On the morning of presentation, a complete ptosis of the right eye had developed (Figure, A). Vital signs were stable, but visual acuity was 20/200 in the right eye and 20/25 in the left. Examination was remarkable for limited ocular motility (Figure, B; Video) and a right relative afferent pupillary defect (Figure, C). Within an hour, vision had deteriorated to light perception. Serum glucose level was measured at 690 mg/dL (38.3 mmol/L) without an anion gap, and hemoglobin A1c level was 8.9%. Maxillofacial computed tomography imaging revealed sinusitis and orbital stranding. The patient was transferred to our institution. On arrival he had lost light perception. An area of ocular adnexal tissue necrosis had developed.

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Figure. A, Complete ptosis of the right eye with necrotic tissue adjacent to the medial canthus. B, External ophthalmoplegia of the right eye demonstrated by having the patient attempt right gaze. The patient also exhibited complete internal ophthalmoplegia (see Video). C, Mydriatic pupil.
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