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An Unusual Case of Severe Bilateral Extraocular Muscle Enlargement

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 47-year-old woman with a history of metastatic neuroendocrine carcinoid tumor (NET) status in remission after liver transplant was hospitalized for workup of a new pancreatic mass concerning for recurrence. Several days into her hospital course, she developed acute-onset severe bilateral periorbital swelling, chemosis, and ophthalmoplegia as well as occasional flushing and palpitations. Her best-corrected visual acuity was 20/70 in both eyes. Pupils, intraocular pressure, and color vision were normal. She had bilateral proptosis to 20/21 mm by Hertel exophthalmometer, −3 restriction of extraocular movements in all directions of gaze, diffuse punctate epithelial erosions, and mechanical lower lid entropion secondary to severe chemosis in both eyes (Figure 1A). Anterior segment examination and dilated ophthalmoscopy results were otherwise unremarkable.

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A 47-year-old woman with a history of metastatic neuroendocrine carcinoid tumor (NET) status in remission after liver transplant was hospitalized for workup of a new pancreatic mass concerning for recurrence. Several days into her hospital course, she developed acute-onset severe bilateral periorbital swelling, chemosis, and ophthalmoplegia as well as occasional flushing and palpitations. Her best-corrected visual acuity was 20/70 in both eyes. Pupils, intraocular pressure, and color vision were normal. She had bilateral proptosis to 20/21 mm by Hertel exophthalmometer, −3 restriction of extraocular movements in all directions of gaze, diffuse punctate epithelial erosions, and mechanical lower lid entropion secondary to severe chemosis in both eyes (Figure 1A). Anterior segment examination and dilated ophthalmoscopy results were otherwise unremarkable.

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Article Information

Corresponding Author: Katherine M. Lucarelli, MD, Department of Ophthalmology, University of Illinois at Chicago, 1855 W Taylor St, Chicago, IL 60612 (klucar2@uic.edu).

Published Online: May 5, 2022. doi:10.1001/jamaophthalmol.2022.0265

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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