Masquerading Orbital Sarcoidosis with Isolated Extraocular Muscle Involvement

Jane S. Kim1, Richard L. Scawn1, Bradford W. Lee1, Jonathan H. Lin1, 2, Bobby S. Korn1, 3, Don O. Kikkawa1, 3, *
1 Division of Ophthalmic Plastic and Reconstructive Surgery, Department of Ophthalmology, Shiley Eye Institute, University of California, San Diego, La Jolla, CA
2 Ophthalmic Pathology Service, Department of Pathology, University of California, San Diego, La Jolla, CA
3 Division of Plastic Surgery, Department of Surgery, University of California, San Diego, La Jolla, CA

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© Kim et al.; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution-Non-Commercial 4.0 International Public License (CC BY-NC 4.0) (, which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Department of Ophthalmology, Shiley Eye Institute, University of California, San Diago, 9415 Campus Point Drive, La Jolla, CA 92093-0946, USA; E-mail:


Two patients, previously diagnosed and treated for euthyroid, autoantibody-negative thyroid eye disease, presented with active orbitopathy. An atypical disease course and presentation prompted orbital biopsy. Extraocular muscle histopathology demonstrated noncaseating granulomatous inflammation consistent with presumed orbital sarcoidosis involving multiple extraocular muscles, including the inferior oblique in one of the cases. These two cases emphasize the importance of a broad differential diagnosis and the utility of an orbital biopsy in the context of an unusual disease presentation or poor treatment response. The patients’ clinical course is discussed alongside important clinical signs, imaging findings, and biopsy results that support a diagnosis of isolated orbital sarcoidosis.

Keywords: Carotid-cavernous fistula, extraocular muscles, orbital sarcoidosis, orbital biopsy, thyroid eye disease.