A rare case of infiltrative optic neuropathy caused by vitreoretinal lymphoma with hyperreflective deposits in the intraretinal and subretinal pigment epithelial layers prior to the disease onset.

Background: To describe a rare case of infiltrative optic neuropathy (ION) caused by vitreoretinal lymphoma (VRL) with hyperreflective deposits in the intraretinal and subretinal pigment epithelial (RPE) layers before the disease onset.

Case presentation: An 87-year-old Japanese female, previously treated for unexplained uveitis in the right eye, was referred for further evaluation. Despite the absence of intraocular inflammation or vitreous opacification (VO), yellowish-white deposits were noted in the macula, and OCT revealed hyperreflective deposits in the intraretinal and sub-RPE layers. One month later, she returned with acute visual deterioration. Optic disc swelling and disc hemorrhage were observed without VO or intraocular inflammation. Magnetic resonance imaging (MRI) showed no abnormalities. One week later, VO developed and we performed a 25-gauge vitrectomy. Cytology, flow cytometry, and gene rearrangement confirmed malignant B-cell lymphoma. The diagnosis of ION secondary to VRL was made, and intravitreal methotrexate injections were administered a total of 6 times. Six months postoperatively, VO and optic disc swelling resolved, and visual acuity improved to 20/50, with no recurrence on MRI.

Conclusions: Although there were reports on VRL advanced from hyperreflective deposits in the intraretinal or sub-RPE layers, there have been no studies that these OCT findings has progressed to ION with VRL. When differentiating optic neuropathy, OCT may assist in the diagnosis by evaluating intraretinal and sub-RPE findings.