Soft drusen or not?

Case

A 55-year-old Caucasian female noted floaters and gradual onset of blurred vision in the right eye (OD) for 5 months. She was found to have retinitis and uveitis, classified as autoimmune retinitis and treated with oral and periocular corticosteroids. After little improvement over 2 months, the patient was referred to rule out the underlying malignancy. On examination, visual acuity was 20/80 OD and 20/30 in the left eye (OS). Intraocular pressures were normal and vitreous was clear in both eyes (OU). There were multiple, large, coalescent soft drusen (retinal pigment epithelium detachment [RPED]) in OU. There was no history of age-related macular degeneration.

What Is Your Next Step?

Intravitreal antivascular endothelial growth factor injection

Biopsy the material underlying the largest RPED

Photodynamic therapy to the PEDs

Perform indocyanine green angiography to identify choroidal neovascularization.

Findings

There were numerous yellow coalescent RPEDs in OD (A) and smaller RPEDs OS (B). On optical coherence tomography (OCT) the multifocal RPEDs were large OD (C) and more subtle OS (D), each with homogeneous sub-RPE debris and mild outer retinal edema. Due to the lack of vitreous cellularity on clinical examination and OCT, trans-scleral fine needle aspiration biopsy of the largest RPED OD was performed and represented necrotic large cell lymphoma, consistent with vitreoretinal lymphoma. Intravitreal methotrexate was advised for globe control. Magnetic resonance imaging of the brain revealed a solitary lesion, scheduled for biopsy and management.

Diagnosis: Vitreoretinal large-cell lymphoma OU.

Correct Answer: b.

Discussion

Vitreoretinal large-cell lymphoma is a subtype of primary central nervous system lymphoma that can display OU and brain involvement. This high-grade malignancy often has a stuttering onset with intermittent blurred vision and mild vitreous inflammatory cells, sometimes with “cloudy” paraneoplastic subretinal infiltration, best seen on OCT, as in our case.[12] Ocular management typically involves intravitreal chemotherapy or radiotherapy. Despite ocular control, systemic prognosis is guarded depending on brain involvement. One collaborative study found 5-year cumulative survival of 35% in those with related brain lymphoma versus 68% in those without brain involvement.[3]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.