Presenters: Dr. CHAUVIN Aurore, Dr TOUHAMI Sara, Pr. BODAGHI Bahram

This is a case with a typical vitreous involvement but a rare retinal manifestation.

Case Summary:

A 52-year-old man presenting with a brain lesion, evolving for more than a year, with a negative stereotactic brain biopsy was referred for ophthalmological evaluation. Visual acuity was normal and slit lamp examination of both eyes showed no anterior segment involvement, but a dense vitritis, characterized by white cells of varied sizes organized in clumps and sheets along vitreous fibrils. Examination of the posterior segment noted a denser vitritis in the periphery and white sub-retinal infiltrates in the nasal and temporal areas in both eyes. Macular OCT was normal. Autofluorescence (AF) imaging showed discrete hyperautofluorescent dots in the temporal zone of the left eye.

Fluorescein angiography (FA) revealed no signs of inflammation (vasculitis or papillitis) but hypofluorescent dots on the same location with a mild leakage around them, in the late frame. Indocyanine green angiography (ICGA) was normal in the early frame but showed an hypofluorescent area with an hyperfluorescent front in the late frame with the previous dots appearing hyperfluorescent.

A vitreous biopsy was quickly performed and the analysis revealed a primary vitreoretinal lymphoma (PVRL) of B clonality. A few days after vitrectomy, we noted an interruption of the IS/OS line progressing from the temporal to the nasal and finally reaching the entire posterior pole with only a small decrease of the visual acuity (25/20). Unfortunately, the patient contracted COVID-19 and did not perform the visual field and ERG. Two months after vitrectomy and the beginning of chemotherapy, he presented with clinical remission in both eyes (without vitritis and with an IL-10 level inferior to 15 picog/ml). Visual acuity improved, the IS/OS line reappeared on macular OCT and retinal thickness increased without residual retinal atrophy. We present a case of primary vitreoretinal (PVRL) and central nervous system (CNS) lymphoma with typical vitreous involvement leading to diagnostic confirmation, associated with a unilateral progressive and reversible retinopathy. Several types of retinopathy have been described (1) in the context of PVRL but loss of outer retinal laminations/disruption of the ellipsoid layer is probably the less reported. Barile et al (2) reported on unilateral retinopathy secondary to occult unilateral intraocular lymphoma which seems close to our case but the visual acuity was very impaired and the evolution of OCT features after treatment was not mentioned. The visual prognosis after treatment remains uncertain in case of retinal involvement during PVRL. In this case the retinopathy was reversible but it can lead to definitive atrophy (1).

Sobolewska B, Chee SP, Zaguia F, Goldstein DA, Smith JR, Fend F, Mochizuki M, Zierhut M. Vitreoretinal Lymphoma. Cancers (Basel). August 2021;13(16):3921.
Barile GR, Garg A, Hood DC, Marr B, Hussein S, Tsang SH. Unilateral retinopathy secondary to occult primary intraocular lymphoma. Doc Ophthalmol. December 2013;127(3):261-9. Oct 1. 2013