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Bilateral Refractory Neurosensory Retinal and Pigment Epithelial Detachments

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

An 88-year-old woman presented with persistent subretinal fluid (SRF) and enlarging pigment epithelial detachments (PEDs) in each eye following 32 near monthly injections of anti–vascular endothelial growth factor (VEGF). Ocular history included presumed exudative age-related macular degeneration. Central serous chorioretinopathy was also suspected secondary to the presence of yellow subretinal deposits located within an inferior macular detachment in the right eye.

Visual acuity was 20/40 OD and 20/25 OS. She had pseudophakia with an otherwise normal anterior segment examination in each eye. Fundus examination was remarkable for macular PEDs and SRF in each eye. There was an epiretinal membrane in the left eye.

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Immunogammopathy-associated maculopathy

D. Obtain serum protein electrophoresis and immunofixation test

Refractory SRF with vitelliform material and absence of leakage on the angiogram (angiographic silence) prompted an investigation for an immunogammopathy (choice D). Continuing intravitreal anti-VEGF injections (choice A) is unlikely to be beneficial because the SRF was refractory to 32 anti-VEGF treatments, and lack of drusen suggest a diagnosis other than exudative age-related macular degeneration. Photodynamic therapy (choice B) is not recommended as the next step because choroidal atrophy and lack of leakage on the angiogram are not consistent with central serous chorioretinopathy. An electrooculogram and molecular genetic testing (choice C) is expected to be negative because a diagnosis of Best disease is improbable given the patient’s age at diagnosis.

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Article Information

Corresponding Author: Odette M. Houghton, MD, Department of Ophthalmology, Mayo Clinic, 13400 E Shea Blvd, Scottsdale, AZ 85259 (houghton.odette@mayo.edu).

Published Online: May 19, 2022. doi:10.1001/jamaophthalmol.2022.0275

Conflict of Interest Disclosures: None reported.

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

References
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Orellana  J , Friedman  AH .  Ocular manifestations of multiple myeloma, Waldenström’s macroglobulinemia and benign monoclonal gammopathy.   Surv Ophthalmol. 1981;26(3):157-169. doi:10.1016/0039-6257(81)90065-5PubMedGoogle ScholarCrossref
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Smith  SJ , Johnson  MW , Ober  MD , Comer  GM , Smith  BD .  Maculopathy in patients with monoclonal gammopathy of undetermined significance.   Ophthalmol Retina. 2020;4(3):300-309. doi:10.1016/j.oret.2019.09.018PubMedGoogle ScholarCrossref
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Cohen  SM , Kokame  GT , Gass  JD .  Paraproteinemias associated with serous detachments of the retinal pigment epithelium and neurosensory retina.   Retina. 1996;16(6):467-473. doi:10.1097/00006982-199616060-00001PubMedGoogle ScholarCrossref
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Ho  AC , Benson  WE , Wong  J .  Unusual immunogammopathy maculopathy.   Ophthalmology. 2000;107(6):1099-1103. doi:10.1016/S0161-6420(00)00118-4PubMedGoogle ScholarCrossref
6.
Rusu  IM , Mrejen  S , Engelbert  M ,  et al.  Immunogammopathies and acquired vitelliform detachments: a report of four cases.   Am J Ophthalmol. 2014;157(3):648-57.e1. doi:10.1016/j.ajo.2013.11.020PubMedGoogle ScholarCrossref
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Grannis  CH , Dewan  VN , Wang  RC .  Resolution of bilateral cystoid macular edema and subfoveal serous retinal detachments after treatment with bortezomib in a patient with “smoldering” multiple myeloma.   Retin Cases Brief Rep. 2014;8(4):348-351. doi:10.1097/ICB.0000000000000067PubMedGoogle ScholarCrossref
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