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Case Reports
. 2018 Jul 3;18(1):162.
doi: 10.1186/s12886-018-0819-x.

IgG4-related ophthalmic disease involving extraocular muscles: case series

Namju Kim  1 , Hee Kyung Yang  1 , Jae Hyoung Kim  2 , Jeong-Min Hwang  3
Affiliations
Case Reports

IgG4-related ophthalmic disease involving extraocular muscles: case series

Namju Kim et al. BMC Ophthalmol. .

Abstract

Background: To elucidate the clinical features of strabismus associated with IgG4-related ophthalmic disease (IgG4-ROD). All of the four patients with IgG4-ROD showed marked enlargement of the extraocular muscles, however, two patients showed orthotropia with full ductions and versions. One patient showed a small angle of exotropia and hypertropia of less than 5 prism diopters. One remaining patient showed orthotropia, full ductions and versions despite marked enlargement of the extraocular muscles, then developed hypertropia up to 35 prism diopters with activation of inflammation, which promptly improved after treatment with oral steroids.

Conclusions: IgG4-ROD usually shows normal ocular motility despite extraocular muscle enlargement, which is the key distinguishing feature from other orbital inflammatory diseases. Active flare-up with increased serum IgG4 levels may produce a large angle of eye deviation, but mostly respond well to steroid treatment.

Keywords: Case report; Extraocular muscle; IgG4-related ophthalmic disease; Imaging findings; Strabismus.

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Conflict of interest statement

Ethics approval and consent to participate

This study complied with the tenets of the Declaration of Helsinki. This study received ethical approval from the Institutional Review Board of the Seoul National University Bundang Hospital.

Consent for publication

Written informed consent was obtained from the patients for publication and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Case 1. a) Ocular versions demonstrating full versions in both eyes. b, c) Orbit CT images showed enlargement of right superior rectus, right medial rectus, left lateral rectus, and left inferior rectus muscles (arrows)
Fig. 2
Fig. 2
Case 2. a) She showed orthotropia at distance and at near in the primary position. b, c) Orbit CT images showed a 2.5 cm sized enhancing mass in the left lacrimal gland (arrow) and enlargement of the left lateral rectus muscle belly (asterisk) like a spindle shaped mass
Fig. 3
Fig. 3
Case 3. a) Ocular versions demonstrating full versions in both eyes. b, c) Orbit MR imaging showed enlargement of the left lacrimal gland (arrow), right medial rectus, right inferior rectus, right lateral rectus, and right inferior oblique (asterisk) with nodular components
Fig. 4
Fig. 4
Case 4. a) Ocular versions demonstrating full versions in both eyes. b, c) Orbit MR imaging showed diffuse infiltrative mass in both orbits and enlargement of both lacrimal glands, both lateral rectus and inferior rectus muscles, infraorbital groove and foramen (arrow), and nodules in the parotid gland (double arrow) d) Ocular versions demonstrating − 3 limitation of downgaze, − 2 limitation of adduction, and − 1 limitation of abduction in the right eye. e) Ocular versions were fully recovered after treatment
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