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Diagnostic Dilemmas With a Great Ocular Masquerader

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

A white man in his early 80s with history of autoimmune pancreatitis, recurrent colon polyps, and benign prostatic hyperplasia developed rapid, painless vision loss of both eyes over a 2-week period 6 months after cataract surgery. Vision was 20/250 OD and 20/60 OS. The anterior segment was unremarkable in either eye, while the posterior segment examination noted 1+ vitreous cell, asteroid hyalosis, and a multilobulated, serous retinal detachment (RD) in the right eye and trace vitreous cell and a multilobulated, serous RD in the left eye. Imaging included B-scan, optical coherence tomography, and fundus autofluorescence, which revealed diffuse choroidal and ciliary body thickening, multifocal pockets of subretinal fluid, and speckled hyperautofluorescence and hypoautofluorescence in both eyes (Figure 1). No poliosis or skin changes were noted. A review of systems was unremarkable, most notably a lack of any pulmonary symptoms. A focused laboratory evaluation identified a significantly elevated angiotensin-converting enzyme (ACE) level and antinuclear antibody titer, but serum lysozyme, syphilis, and tuberculosis testing had negative results. To better differentiate between Vogt-Koyanagi-Harada (VKH) syndrome, a lymphoproliferative disorder, or sarcoidosis as the underlying cause of the vitreous inflammation and multilobulated, serous RD in both eyes, a computed tomographic scan was performed. It showed marked lymphadenopathy throughout the mediastinum and diffuse scleral thickening in both eyes. The institute’s radiologists believed these computed tomography findings were nonspecific and could be indicative of either sarcoidosis or intraocular lymphoma.

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A white man in his early 80s with history of autoimmune pancreatitis, recurrent colon polyps, and benign prostatic hyperplasia developed rapid, painless vision loss of both eyes over a 2-week period 6 months after cataract surgery. Vision was 20/250 OD and 20/60 OS. The anterior segment was unremarkable in either eye, while the posterior segment examination noted 1+ vitreous cell, asteroid hyalosis, and a multilobulated, serous retinal detachment (RD) in the right eye and trace vitreous cell and a multilobulated, serous RD in the left eye. Imaging included B-scan, optical coherence tomography, and fundus autofluorescence, which revealed diffuse choroidal and ciliary body thickening, multifocal pockets of subretinal fluid, and speckled hyperautofluorescence and hypoautofluorescence in both eyes (Figure 1). No poliosis or skin changes were noted. A review of systems was unremarkable, most notably a lack of any pulmonary symptoms. A focused laboratory evaluation identified a significantly elevated angiotensin-converting enzyme (ACE) level and antinuclear antibody titer, but serum lysozyme, syphilis, and tuberculosis testing had negative results. To better differentiate between Vogt-Koyanagi-Harada (VKH) syndrome, a lymphoproliferative disorder, or sarcoidosis as the underlying cause of the vitreous inflammation and multilobulated, serous RD in both eyes, a computed tomographic scan was performed. It showed marked lymphadenopathy throughout the mediastinum and diffuse scleral thickening in both eyes. The institute’s radiologists believed these computed tomography findings were nonspecific and could be indicative of either sarcoidosis or intraocular lymphoma.

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Article Information

Corresponding Author: Christopher D. Conrady, MD, PhD, Department of Ophthalmology, Pathology/Microbiology, University of Nebraska Medical Center, 3902 Leavenworth St, Omaha, NE 68105 (cconrady@unmc.edu).

Published Online: February 17, 2022. doi:10.1001/jamaophthalmol.2021.5241

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We thank Hakan Demirci, MD, and Victor Elner, MD, University of Michigan, for their help with this difficult case.

References
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Sève  P , Jamilloux  Y , Tilikete  C , Gerfaud-Valentin  M , Kodjikian  L , El Jammal  T .  Ocular sarcoidosis.   Semin Respir Crit Care Med. 2020;41(5):673-688. doi:10.1055/s-0040-1710536PubMedGoogle ScholarCrossref
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Teo  HMT , Elner  SG , Sassalos  TMP , Elner  VM , Demirci  H .  Ciliary body mass as a feature of ocular sarcoidosis.   JAMA Ophthalmol. 2020;138(3):300-304. doi:10.1001/jamaophthalmol.2019.5704PubMedGoogle ScholarCrossref
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Dana  M-R , Merayo-Lloves  J , Schaumberg  DA , Foster  CS .  Prognosticators for visual outcome in sarcoid uveitis.   Ophthalmology. 1996;103(11):1846-1853. doi:10.1016/S0161-6420(96)30417-XPubMedGoogle ScholarCrossref
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Kinast  RM , Solomon  SD , Cubillan  LD , Hovakimyan  A , Acharya  N , Cunningham  ET .  Prevalence and causes of clinically detectable uveitic serous retinal detachment.   Eur J Ophthalmol. 2021;31(6):3093-3098. doi:10.1177/1120672121991391PubMedGoogle ScholarCrossref
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Lobo  A , Barton  K , Minassian  D , du Bois  RM , Lightman  S .  Visual loss in sarcoid-related uveitis.   Clin Exp Ophthalmol. 2003;31(4):310-316. doi:10.1046/j.1442-9071.2003.00666.xPubMedGoogle ScholarCrossref
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Takeuchi  M , Mizuki  N , Ohno  S .  Pathogenesis of non-infectious uveitis elucidated by recent genetic findings.   Front Immunol. 2021;12:640473. doi:10.3389/fimmu.2021.640473PubMedGoogle ScholarCrossref
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Sehara  Y , Otsuka  H , Sakamoto  S , Ando  Y , Sawada  M .  Neurosarcoidosis occurring 6 years after onset of Vogt-Koyanagi-Harada disease.   Case Rep Ophthalmol. 2019;10(1):32-40. doi:10.1159/000496384PubMedGoogle ScholarCrossref
AMA CME Accreditation Information

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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