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JAMA Ophthalmology Clinical Challenge
November 21, 2018

Bilateral Sequential Acute Proptosis in a Woman With No History of Trauma

Author Affiliations
  • 1Department of Ophthalmology, Wilmer Eye Institute, Johns Hopkins Hospital, Baltimore, Maryland
  • 2Now with Department of Ophthalmology, Kellogg Eye Center, University of Michigan, Ann Arbor
JAMA Ophthalmol. Published online November 21, 2018. doi:10.1001/jamaophthalmol.2018.3741

A woman in her early 30s presented with acute onset of painful proptosis of the right eye and mild epistaxis with no inciting trauma. Her medical history was notable for hypertension, treated with clonidine hydrochloride, and opiate abuse, treated with methadone hydrochloride. Her blood pressure was 138/87 mm Hg. The patient was afebrile with no leukocytosis. Her visual acuity was 20/20 OU with no afferent pupillary defect and normal intraocular pressures. Examination revealed periorbital edema, proptosis, and limitation in supraduction, abduction, and adduction in the right eye. There was no periorbital soft-tissue erythema, warmth, or tenderness to palpation. Computed tomography showed hyperattenuating soft tissue in the superior, medial, and inferior right orbit (Figure 1A, arrowheads). Magnetic resonance imaging showed no internal flow voids to suggest vascular malformation. Results of a laboratory workup revealed mildly elevated erythrocyte sedimentation rate (49 mm/h; reference range, 0-20 mm/h) and antinuclear antibody titer (1:80; reference range, <1:40) as well as positive results for anti-Ro and anti-La antibodies. Results of further autoimmune and infectious workup were negative. Two weeks later, new proptosis and periocular edema of the left eye developed, with no inciting trauma. Her examination results were significant for proptosis, periorbital edema, and limitation in supraduction in the left eye. Computed tomography showed new hyperattenuating soft tissue in the left superior and lateral orbit (Figure 1B, arrowhead).