Marked Enlargement of Bilateral Infraorbital Nerve in a Known Case of IgG4-related Disease : Ophthalmic Plastic & Reconstructive Surgery

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Marked Enlargement of Bilateral Infraorbital Nerve in a Known Case of IgG4-related Disease

Rajabi, Mohammad Taher; Mohammadi, Abbas M.D.

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Ophthalmic Plastic and Reconstructive Surgery: November/December 2022 - Volume 38 - Issue 6 - p e188
doi: 10.1097/IOP.0000000000002206
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A 44-year-old man reported bilateral proptosis for 8 years and decrease of vision in the OU for 3 months. Ten years ago, he developed parotid gland enlargement and severe headaches and underwent imaging and pathology evaluations. He was diagnosed with IgG4-related disease and immunosuppression therapy was started. The patient’s visual acuity was 20/40 in the OU. Intraocular pressures were normal in OU, anterior and posterior segment examination were unremarkable. The patient had proptosis, and Hertel exophthalmometry was 20 in the OS and 23 in the OD. He had full range of eye movements in OU. There was no previous significant ocular history or family history. Also, the patient had bilateral parotid gland enlargement (Fig. 1). CT imaging demonstrated a high-density mass in both maxillary sinuses (Fig. 2A). Orbital CT revealed bilateral proptosis, bilateral lacrimal nerve enlargement, extraocular muscles enlargement, and tenting of the both globes (Fig. 2B) due to huge bilateral infraorbital nerve enlargement and expansion of the infraorbital canals and spread to the orbit and paranasal sinus (Fig. 2C, D). MRI (Fig. 2E, F) revealed bilateral infraorbital nerve enlargement and enhancement with intraorbital and maxillary sinus extension. Due to massive extension of the infraorbital nerve, the patient was offered tumor debulking and immunosuppressive treatment after multidisciplinary consultation, but unfortunately, he refused all of them and he missed follow up. This case demonstrates the aggressive and unbelievable nature of IgG4-related disease.

F1
Fig. 1.:
External photographs demonstrating bilateral parotid gland enlargement (A) and bilateral proptosis (B).
F2
Fig. 2.:
CT maxillofacial demonstrating mass in both maxillary sinuses (A). CT orbits demonstrated bilateral proptosis and intra orbital expansion of infraorbital nerve (B). B, Bilateral infraorbital nerve enlargement that expanding of the infraorbital canal and spread to orbit and paranasal sinus, EOMs) enlargement and bilateral lacrimal nerve enlargement (C, D). MRI (E, F) MRI showed bilateral infraorbital nerve enlargement with intraorbital and maxillary sinus extension and bilateral lacrimal nerve enlargement (arrow).
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