PMC10187048_01
Examination at presentation. a Optos  Daytona ultra-widefield retinal image of the left eye shows nasal to inferior cystic retinal detachment. Scattered mass lesions are observed beneath the retina. b Ultrasound examination (sagittal) indicates downward hyperechoic retinal detachment and a hypoechoic elevated posterior lesion in the left eye. c Widefield fluorescein angiography of the left eye. In the early phase, hyperfluorescence due to dye leakage is observed from the nasal side to the inferior side. d Orbital contrast-enhanced magnetic resonance imaging. The arrowhead indicates a lesion with a major axis of 2 cm. The arrow indicates retinal detachment and fluid retention.
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F1
The fundus showed severe exudative retinal detachment in the nasal and inferior quadrants and scattered, white elevated subretinal lesions beneath the nasal retina (Fig. 1a). Left eye ultrasound examination revealed exudative retinal detachment and a hypoechoic posterior elevated lesion at the choroidal level (Fig. 1b). Fluorescein angiography demonstrated dye leakage from the retinal vessels in the nasal and inferior quadrants of the left eye (Fig. 1c). Orbital contrast-enhanced magnetic resonance imaging (MRI) revealed a lesion with a major axis of 2 cm, retinal detachment, and fluid retention in the left eyeball (Fig. 1d).
Examination after initial treatment. a Optical coherence tomography (vertical) of the left eye after the first steroid pulse therapy. Vitreous cells, exudative retinal detachment with hyper-reflective small deposits, wavy retinal pigment epithelium, and choroidal thickening are visualized. No image was taken at presentation. b Optos  Daytona ultra-widefield retinal image of the left eye after the second steroid pulse therapy shows the resolution of mass lesions and cystic retinal detachment.
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F2
The exudative retinal detachment resolved and visual acuity (20/100 in the left eye) improved shortly after the treatment was initiated (Fig. 2a). After the second steroid pulse therapy, the retinal detachment resolved further (Fig. 2b).
Examination 17 months after the first visit. a Optos  Daytona ultra-widefield retinal image of the left eye. The granulomatous lesion and retinal detachment have resolved, and choroidal atrophy is observed. b Orbital contrast magnetic resonance imaging. The arrowhead indicates the mass lesion, which has shrunk. c Optical coherence tomography of the left eye. Exudative retinal detachment has resolved. The waviness of the retinal pigment epithelium has improved.
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F3
After the fourth dose of rituximab, the scleritis and nasal choroidal detachment in the left eye resolved (shown in Fig. 3a), and the best-corrected visual acuity in the left eye improved to 20/16. MRI revealed shrinkage of the orbital granuloma (Fig. 3b), and optical coherence tomography indicated the resolution of the orbital granuloma and exudative retinal detachment (Fig. 3c).