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Bilateral Enlargement of All Extraocular Muscles

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

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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T-cell prolymphocytic leukemia infiltration of the extraocular muscles

C. Perform extraocular muscle biopsy

Thyroid-associated orbitopathy (TAO) is the most prevalent orbital disease.2 Patients typically present in their fourth or sixth decade of life and women are affected 5 times more than men.2 TAO is associated with autoimmune thyroid dysfunction: 90% of individuals with TAO have Graves hyperthyroidism, 1% have primary hypothyroidism, 3% have Hashimoto thyroiditis, and 5% are euthyroid.2 The most common signs are upper eyelid retraction (38% to 90%) and proptosis (65%), followed by von Graefe sign, lower eyelid retraction, and lagophthalmos.3,4 Active TAO may manifest with orbital pain and eyelid, conjunctival, or caruncular inflammation.4

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Article Information

Corresponding Author: Evan Kalin-Hajdu, MD, Department of Ophthalmology, Université de Montréal, CUO Maisonneuve-Rosemont, 5415 Blvd de l’Assomption, Montréal, QC H1T 2M4, Canada (evan.kalin-hajdu@umontreal.ca).

Published Online: January 14, 2021. doi:10.1001/jamaophthalmol.2020.4679

Conflict of Interest Statement: None reported.

Additional Contributions: We acknowledge Jérémie Berdugo, MD, Department of Pathology and Cellular Biology, Université de Montréal, Montreal, Quebec, Canada, and Marie-Christine Carette, MD, Department of Hemato-oncology, Hôpital de Saint-Eustache, Saint-Eustache, Quebec, Canada, for their contributions to patient care and the writing of this article. They were not compensated. We thank the patient for granting permission to publish this information.

References
1.
Dagi  LR , Zoumalan  CI , Konrad  H , Trokel  SL , Kazim  M .  Correlation between extraocular muscle size and motility restriction in thyroid eye disease.   Ophthalmic Plast Reconstr Surg. 2011;27(2):102-110. doi:10.1097/IOP.0b013e3181e9a063PubMedGoogle ScholarCrossref
2.
Bartley  GB .  The epidemiologic characteristics and clinical course of ophthalmopathy associated with autoimmune thyroid disease in Olmsted County, Minnesota.   Trans Am Ophthalmol Soc. 1994;92:477-588.PubMedGoogle Scholar
3.
Gaddipati  RV , Meyer  DR .  Eyelid retraction, lid lag, lagophthalmos, and von Graefe’s sign quantifying the eyelid features of Graves’ ophthalmopathy.   Ophthalmology. 2008;115(6):1083-1088. doi:10.1016/j.ophtha.2007.07.027PubMedGoogle ScholarCrossref
4.
Bartley  GB , Fatourechi  V , Kadrmas  EF ,  et al.  Clinical features of Graves’ ophthalmopathy in an incidence cohort.   Am J Ophthalmol. 1996;121(3):284-290. doi:10.1016/S0002-9394(14)70276-4PubMedGoogle ScholarCrossref
5.
Nugent  RA , Belkin  RI , Neigel  JM ,  et al.  Graves orbitopathy: correlation of CT and clinical findings.   Radiology. 1990;177(3):675-682. doi:10.1148/radiology.177.3.2243967PubMedGoogle ScholarCrossref
6.
McNab  AA .  Orbital myositis: a comprehensive review and reclassification.   Ophthalmic Plast Reconstr Surg. 2020;36(2):109-117. doi:10.1097/IOP.0000000000001429PubMedGoogle ScholarCrossref
7.
Kahaly  GJ . Management of moderately severe Graves' orbitopathy. In: Wiersinga  WM , Kahaly  GJ , eds.  Graves’ Orbitopathy: A Multidisciplinary Approach—Questions and Answers. 3rd ed. Karger; 2017:140-176. doi:10.1159/000475956
8.
Mombaerts  I , Rose  GE , Garrity  JA .  Orbital inflammation: biopsy first.   Surv Ophthalmol. 2016;61(5):664-669. doi:10.1016/j.survophthal.2016.03.002PubMedGoogle ScholarCrossref
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