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Multifocal Macular Lesions in a Middle-aged Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 49-year-old African American woman with no significant medical history came to the clinic because of abnormal retinal findings and mildly decreased visual acuity. She was told about these same findings 10 years ago but did not have a repeated eye examination until recently by another retina specialist. She reported no family history of retinal diseases. She initially received anti–vascular endothelial growth factor (VEGF) treatment, but was later referred because of a concern about either the idiopathic or paraneoplastic variant of acute exudative polymorphous vitelliform maculopathy (AEPVM). Initial workup by her primary care physician was unremarkable for malignancy. Her best-corrected visual acuity (BCVA) was 20/60 OD and 20/50 OS. An anterior segment examination was normal. Dilated fundus examination revealed multiple vitelliform lesions involving the macula and superior macula bilaterally (Figure 1). Fluorescein angiography revealed staining of these lesions with a pseudohypopyon appearance of the central vitelliform lesion in the left eye. Optical coherence tomography (OCT) showed hyperreflectance corresponding to the yellow pigment in the vitelliform lesions in the subretinal space. No evidence of a choroidal neovascular membrane was present on examination or imaging.

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A 49-year-old African American woman with no significant medical history came to the clinic because of abnormal retinal findings and mildly decreased visual acuity. She was told about these same findings 10 years ago but did not have a repeated eye examination until recently by another retina specialist. She reported no family history of retinal diseases. She initially received anti–vascular endothelial growth factor (VEGF) treatment, but was later referred because of a concern about either the idiopathic or paraneoplastic variant of acute exudative polymorphous vitelliform maculopathy (AEPVM). Initial workup by her primary care physician was unremarkable for malignancy. Her best-corrected visual acuity (BCVA) was 20/60 OD and 20/50 OS. An anterior segment examination was normal. Dilated fundus examination revealed multiple vitelliform lesions involving the macula and superior macula bilaterally (Figure 1). Fluorescein angiography revealed staining of these lesions with a pseudohypopyon appearance of the central vitelliform lesion in the left eye. Optical coherence tomography (OCT) showed hyperreflectance corresponding to the yellow pigment in the vitelliform lesions in the subretinal space. No evidence of a choroidal neovascular membrane was present on examination or imaging.

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Article Information

Corresponding Author: Albert L. Lin, MD, Department of Ophthalmology, University of Mississippi Medical Center, 2500 N State St, Jackson, MS 39216 (alin@umc.edu).

Published Online: February 3, 2022. doi:10.1001/jamaophthalmol.2021.4983

Conflict of Interest Disclosures: Dr Lin reported advisory board consulting fees of $1800 from Allergan about dexamethasone intravitreal implant (Ozurdex) outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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Agarwal  A.   Gass’s Atlas of Macular Diseases. 5th ed. Saunders; 2012 274-280.
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Parodi  MB , Iacono  P , Campa  C , Del Turco  C , Bandello  F .  Fundus autofluorescence patterns in Best vitelliform macular dystrophy.   Am J Ophthalmol. 2014;158(5):1086-1092. doi:10.1016/j.ajo.2014.07.026PubMedGoogle ScholarCrossref
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Boon  CJF , Klevering  BJ , den Hollander  AI ,  et al.  Clinical and genetic heterogeneity in multifocal vitelliform dystrophy.   Arch Ophthalmol. 2007;125(8):1100-1106. doi:10.1001/archopht.125.8.1100PubMedGoogle ScholarCrossref
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Giuffrè  C , Miserocchi  E , Modorati  G ,  et al.  Central serous chorioretinopathy-like mimicking multifocal vitelliform macular dystrophy: an ocular side effect of mitogen/extracellular signal-regulated kinase inhibitors.   Retin Cases Brief Rep. 2018;12(3):172-176. doi:10.1097/ICB.0000000000000491PubMedGoogle ScholarCrossref
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Sandhu  HS , Kolomeyer  AM , Lau  MK ,  et al.  Acute exudative paraneoplastic polymorphous vitelliform maculopathy during vemurafenib and pembrolizumab treatment for metastatic melanoma.   Retin Cases Brief Rep. 2019;13(2):103-107. doi:10.1097/ICB.0000000000000604PubMedGoogle ScholarCrossref
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Barbazetto  I , Dansingani  KK , Dolz-Marco  R ,  et al.  Idiopathic acute exudative polymorphous vitelliform maculopathy: clinical spectrum and multimodal imaging characteristics.   Ophthalmology. 2018;125(1):75-88. doi:10.1016/j.ophtha.2017.07.020PubMedGoogle ScholarCrossref
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Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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