case_images: PMC11302219_01
case_images: Case 1 magnetic resonance imaging with contrast scan in December 2018. (a) A red arrow is pointing to tissue (isointense to muscles in T1, hyperintense in T2-short-tau inversion recovery (STIR), and with post-contrast enhancement) molds and does not appear dissociable from the upper complex, obliterating both extraconal and intraconal fat. (b) A red arrow is pointing to imprint of the proximal portion of the optic nerves. (c) A red arrow is showing that all extraocular eye muscles appear swollen and hyperintense in STIR, indicative of edema.
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case_images: F1
case_images: A December 2018 flare led to an orbital MRI in January 2019, showing optic nerve dislocation and muscle swelling [Figure 1].
case_images: Case 1 biopsy of the right orbital tissues, performed in March 2019. (a) Lobules of lacrimal gland with >two foci of periductal and perivascular lymphocytic infiltration and minor interstitial infiltrate, consisting of CD3+ LAT+ T lymphocytes (b & d), without a significant increase in immunoglobulin G4-positive plasma cells (c). (Hematoxylin and Eosin (H&E) stain, x20 magnification). (CD3: Cluster of Differentiation 3, IGg4: Immunoglobulin G4, LAT: Linker for activation of T cells.)
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case_images: F2
case_images: March 2019 orbital tissue biopsy revealed two periductal and perivascular lymphocytic infiltrates of CD3+ LAT+ T cells, increased connective tissue, without a significant increase of IgG4 positive plasma cells [Figure 2], leading to an idiopathic orbital inflammation (IOI) diagnosis. Methotrexate (17.5 mg/week) initiated in March 2019 resulted in clinical improvement, and prednisone was tapered to 25 mg/day.
case_images: Case 1 magnetic resonance imaging with contrast scan in November 2021. (a) A red arrow is pointing to volumetric reduction of tumefactive alterations in the extrinsic muscles with modest contrast enhancement of the examined muscle structures. (b) A red arrow is pointing to a slight decrease in the size of the solid tissue that was previously inseparable from the complex of the upper right rectus. (c) A red arrow is showing that both optic nerves and intra-orbital adipose tissue exhibit normal signal, morphology, and course.
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case_images: F3
case_images: Orbital MRI demonstrated reduced inflammatory tissue, regular optical nerve course, and normal orbital adipose tissue [Figure 3].
case_images: PMC11302219_02
case_images: Case 2 computed tomography scan in February 2019. A red arrow is pointing to a volumetric increase of the inferior rectus muscle of the right orbit (measuring 12 mm) compared to the left side (9 mm).
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case_images: F4
case_images: February 2019 cranial CT revealed lymphadenopathies, parotid swelling, and right inferior rectus muscle enlargement [Figure 4].
case_images: PMC11302219_03
case_images: Clinical presentation of Case 3 in June 2018.
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case_images: F5
case_images: Marked left exophthalmos persisted [Figure 5], with orbital magnetic resonance (MR) showing optical nerve thickening, retrobulbar fat tissue hyperintensity, and left ethmoidal, sphenoid, and frontal sinus inflammation, compatible with granulomatosis with polyangiitis [Figure 6].
case_images: Case 3 magnetic resonance imaging with contrast scan in June 2018. (a) A red arrow is pointing to a marked condition of left exophthalmos, accompanied by a significant volumetric increase in the extrinsic ocular musculature, the optic nerve in all assessable segments with liquid distension of the peri-optic sheath. There is pronounced hyperintensity in signal within the retrobulbar adipose tissue, along with soft-tissue edema. (b) A red arrow is pointing to evidence of inflammatory tissue involving the ethmoidal cells on the left side, the sphenoidal sinus, and the frontal sinuses, particularly on the left. (c) A red arrow is pointing to a notable volumetric increase visible following intravenous gadolinium administration in all previously mentioned structures consistent with the clinical inquiry, suggesting an ocular manifestation of Wegener's granulomatosis.
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case_images: F6
case_images: Marked left exophthalmos persisted [Figure 5], with orbital magnetic resonance (MR) showing optical nerve thickening, retrobulbar fat tissue hyperintensity, and left ethmoidal, sphenoid, and frontal sinus inflammation, compatible with granulomatosis with polyangiitis [Figure 6].
case_images: PMC11302219_04
case_images: Case 4 magnetic resonance imaging with contrast scan in August 2020. (a) A red arrow is pointing to a fusiform thickening of the right medial rectus muscle (10 mm). (b) A red arrow is pointing to a slight thickening in the right lateral rectus muscle (4 mm compared to 2.3 mm at the left side) and the right superior oblique muscle (4.2 mm on the right compared to 2.3 mm at the left side). (c) A red arrow is pointing to evidence of a slightly sinuous course in the intraorbital segment of the optic nerve at the left side. Both optic nerves exhibit a regular signal.
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case_images: F7
case_images: MRI findings revealed severe thickening of the medial rectus muscle, along with mild thickening of the lateral rectus and superior oblique muscles of the eye [Figure 7].
case_images: Case 4 biopsy of the lateral rectus muscle of the left eye, performed in May 2021. (a-d) are illustrating different parts of soft tissues and bundles of striated muscle that show degenerative-regressive phenomena of muscle fibrocells, collagen fibrotic dissociation, and significant T-cell lymphocytic infiltration. (Hematoxylin and eosin (H/E) stained, x20 magnification).
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case_images: F8
case_images: A biopsy of the left eye's lateral rectus muscle showed degenerative processes, fibrotic involution, and massive T cell infiltrate [Figure 8].