Presenter: Joeri De Hoog The Netherlands

Patient presented with subacute onset of blurry vision and grey or black spots in the visual fields of both eyes, macular subretinal fluid, a bacillary layer detachment, subRPE-fluid, thickening of the RPE and swollen optic nerve heads.

Case Summary:

We present the case of a 58-year old Caucasian male who was diagnosed with metastatic clear cell renal cell carcinoma for which he was treated with intravenous Nivolumab immunotherapy with palliative intent. Patient presented with subacute onset of blurry vision and grey or black spots in the visual fields of both eyes, macular subretinal fluid, a bacillary layer detachment, subRPE-fluid, thickening of the RPE and swollen optic nerve heads.

He had best corrected VA of 0.5 – 0.8. Initial working diagnosis was nivolumab-induced Vogt-Koyanagi-Harada-disease-like syndrome, upon which Nivolumab was stopped, and patient was treated with oral steroids, which worsened his ocular condition. The subretinal fluid increased in both eyes and BCVA dropped to 0.2-0.3. Grey patches in the peripheral retina of both eyes and an almost pathognomonic were then found, which prompted reassessment and led to the diagnosis Bilateral diffuse uveal melanocytic proliferation (BDUMP).

Patient was successfully treated with plasmapheresis. The final best corrected visual acuity was 0.2 – 0.8, 8 months after initiating plasmapheresis. The right eye showed a persisting thickening of the RPE, which may be the cause of the poor visual outcome in that eye. Due to cerebral metastases he passed away shortly after.

Some pearls:

• BDUMP can mimic other ophthalmological conditions
• Early BDUMP can resemble VKH
• OCT is not enough!
• Plasmapheresis is therapy of choice
• Treatment of underlying malignancy is paramount