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JAMA Ophthalmology Clinical Challenge
January 14, 2021

Bilateral Enlargement of All Extraocular Muscles

Author Affiliations
  • 1Department of Ophthalmology, Université de Montréal, Montreal, Quebec, Canada
  • 2Department of Pathology and Cellular Biology, Université de Montréal, Montreal, Quebec, Canada
JAMA Ophthalmol. 2021;139(3):359-360. doi:10.1001/jamaophthalmol.2020.4679

A 64-year-old woman presented with a 6-month history of progressive bilateral proptosis and conjunctival erythema. Her medical history was significant for hypothyroidism, treated with levothyroxine, as well as 2 forms of hematologic malignancy: retroperitoneal extranodal marginal zone lymphoma and indolent-phase T-cell prolymphocytic leukemia. She had received no treatment for her hematologic malignancies, as she had been asymptomatic since her diagnoses 6 years prior.

Ophthalmological examination revealed 27 mm of bilateral proptosis, conjunctival injection, chemosis, and bilateral lower eyelid retraction (Figure 1A).1 Visual acuity, color vision, and pupillary responses were normal. Extraocular motility was full in all positions of gaze, and there was no associated pain. Upper eyelid retraction was minimal. Von Graefe sign (delayed descent of the upper eyelid with initiation of downgaze) and lagophthalmos were absent. Results of slitlamp examination and dilated fundus examination were normal, including the appearance of both optic nerves.

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