Is it melanoma-associated retinopathy or drug toxicity? Bilateral cystoid macular edema posing a diagnostic and therapeutic dilemma

Abstract

PurposeTo report the clinical presentation, multimodal imaging, and management of a patient with metastatic melanoma who presented with cystoid macular edema (CME).ObservationsWe report a case of a 71-year-old Caucasian male with metastatic melanoma who presented with bilateral cystoid macular edema after being on treatment with a programmed T cell death ligand 1 inhibitor, MPDL3280, for 1 year. Multimodal imaging techniques, including color fundus photographs, autofluorescence, spectral domain optical coherence tomography (OCT), fluorescein angiography (Spectralis, Heidelberg, Germany), and spectral-domain OCT angiography (Zeiss; California, USA) were performed to evaluate the etiology of his CME and to monitor his response to treatment. Clinical examination and multimodal imaging revealed 1 + chronic vitreous cells, an epiretinal membrane, and mild macular edema in both eyes. Fundus autofluorescence showed paravenous hypoautofluorescence in the right eye and scattered hypoautofluorescent spots in the left eye. Optical coherence tomography angiography (OCTA) revealed mild drop out of superficial vessels in the peri-foveal region bilaterally. These findings were concerning for melanoma-associated retinopathy, drug-related uveitis, or activation of a previous chronic autoimmune process. The patient was started on prednisone 30 mg oral daily and ketorolac tromethamine 0.5% 1 drop four times daily. He was then treated with bilateral sustained-release dexamethasone intravitreal implants (Ozurdex). He had complete resolution of CME, and was tapered off of oral steroids within 6 weeks.Conclusions and ImportanceMelanoma-associated retinopathy can be accompanied by CME, which presents a diagnostic and therapeutic dilemma in cases where a new drug has been recently initiated. By treating the condition locally, the ophthalmologist may be able to taper systemic immunosuppression more quickly.

Drug-Induced Pseudo-Central Serous Chorioretinopathy in Carcinoma Patients

“…In recent years, identification of tumor-specific genomic abnormalities has enabled dramatic improvement in the efficacy and tolerability of systemic palliative anticancer therapy. Nevertheless, retinal toxicity has been associated with the recent use of MEK and FRFR inhibitors developed for the treatment of metastatic lung cancer [3,8,9,12].…”

Autoimmune retinopathy: findings and limitations from optical coherence tomography angiography

“…A case of npAIR was reported by Kasongole et al showing OCTA void areas in the choroidal vasculature [12]. A case of MAR reported by Patel et al showed perifoveal vessel dropout on the OCTA, both the superficial and deep retinal layers, and perifoveal small vessel dropout but the FAZ was not measured [13]. Both studies were descriptive, did not have a control group and did not obtain objective measurements of the OCTA scans.…”

“…OCT findings that have been reported with AIR include the classic "flying saucer" sign with parafoveal attenuation of the outer nuclear layer, external limiting membrane and ellipsoid zone in the presence of subfoveal preservation of these outer retinal elements [3,16]. Other OCT findings include non-leaking intraretinal cystic spaces, retinal pigment epithelium and choriocapillaris atrophy and decreased macular retinal thickness [13], although up to 18% of AIR cases can have normal SD-OCT findings [3].…”

“…Specifically, OCTA has allowed visualization of the deep capillary plexus, which was previously not possible only with fluorescein angiography [11]. Very little has been written about the OCTA findings in AIR, with knowledge of the OCTA changes in this condition being limited to two published case reports [12,13]. The aim of this study is to report for the first time the OCTA findings of a small series of patients with AIR, to compare these findings to normal age matched controls and to discuss the challenges and limitations associated with the use of this technology when imaging AIR.…”

Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune Retinopathy

“…A number of other anti-retinal antibodies have been identified in patients with MAR, including 35-kDa Muller glial cell protein, 22 kDa neuronal antigen, 135-kDa, 46-kDa, 94-kDa, 30- kDa, 33-kDa, 35-kDa, 23-kDa, and 30-kDa. 43-64 Since the ERG finding of a preserved a-wave is non-specific and auto-antibodies other than anti-TRPM1 lack specificity, the minimal case-defining features of MAR remain unclear.…”