OSLI Retina

December 2016

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December 2016 · Vol. 47, No. 12 1143 ■ C A S E R E P O R T ■ Multimodal Imaging Features in Acute Exudative Paraneoplastic Polymorphous Vitelliform Maculopathy Daniel Q. Li, BSc; John Golding, BA; Carl Glittenberg, MD; Netan Choudhry, MD, FRCS(C) ABSTRACT: An 85-year-old woman with stage IV breast cancer was referred for gradually progres- sive blurred vision. Dilated fundus examination re- vealed unifocal, yellow, round vitelliform lesions in the macular region of both eyes. The diagnosis of acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM) was confirmed with swept-source optical coherence tomography (SS-OCT), fundus autofluorescence, and fluores- cein angiography. SS-OCT angiography revealed normal vascular findings in both eyes. Multimodal imaging is useful in the diagnosis and monitoring of AEPPVM and may further the understanding of its pathophysiology. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:1143-1146.] INTRODUCTION An 85-year-old woman was referred for gradually progressive, painless, blurred vision in both eyes dur- ing the course of 6 months. She was diagnosed with locally advanced breast cancer 41 years prior and was treated with a radical mastectomy. She had recurrent stage IV breast cancer with lung metastasis 2 years prior and was treated with radiotherapy and tamoxi- fen therapy. The patient had concurrent hypertension and osteoporosis and had successful cataract surgery in both eyes. There was no other relevant medical, ophthalmic, or family history. On clinical examination, her best-corrected visual acuity (BCVA) was 20/40 in both eyes with normal anterior segment examination findings. Dilated fun- dus examination revealed a unifocal, yellow, round amelanotic (vitelliform) lesions with a pseudohypo- pyon in the macular region bilaterally (Figures 1A and 1B). Green fundus autofluorescence (FAF) re- vealed hyperautofluorescence corresponding to the pseudohypopyon(s) (Figures 1C and 1D), whereas fluorescein angiography demonstrated hypofluores- cence of these regions without leakage (Figures 1E and 1F). Swept-source optical coherence tomogra- phy (SS-OCT) revealed bilateral, localized, well-de- marcated, hyperreflective, dome-shaped elevations of the neurosensory retina with subfoveal hyperreflec- tive material and a trace amount of subretinal fluid. (Figure 2). The choroid was also thickened in both eyes, but no evidence of choroidal metastases was identified. SS-OCT angiography (SS-OCTA) revealed normal vascular findings in the superficial capillary, deep capillary, and choriocapillaris layers (Figure 3). Although the patient denied electroretinography (ERG) testing, laboratory testing confirmed presence of serum antiretinal antibodies. As the patient had relatively preserved visual acuity, the decision was made to observe the macular lesions. On her most recent follow-up visit at 14 months after the initial diagnosis, the patient was stable with no clinically significant progression of her ocular and systemic conditions. The OCT devices used in this study have been approved by Health Canada, and this study con- formed to the tenets. DISCUSSION The differential diagnosis for vitelliform macular lesions includes: best vitelliform macular dystrophy (childhood onset), adult-onset vitelliform macular From the Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada (DQL); Vitreoretinal Surgery Service, Herzig Eye Institute, Toronto, Ontario, Canada (JG, NC); Topcon Europe Medical BV, Rotterdam, Neth- erlands (CG); and the Department of Ophthalmology & Vision Sciences, University of Toronto, Toronto, Ontario, Canada (NC). Originally submitted July 13, 2016. Revision received September 7, 2016. Accepted for publication September 16, 2016. Dr. Choudhry is a consultant for Topcon. Dr. Glittenberg is a senior clini- cal advisor for Topcon. The remaining authors report no relevant financial disclosures. Address correspondence to Netan Choudhry, MD, FRCS(C), Vitreoretinal Surgery Service, Herzig Eye Institute, 131 Bloor St. W., Suite 210, Toronto, ON Canada, M5S 1R1; email: netan.choudhry@gmail.com. doi: 10.3928/23258160-20161130-09

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