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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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Biopsy-proven, Harada-like ocular sarcoidosis in both eyes

C. Perform ciliary body–choroidal biopsy in the right eye

With the patient’s advanced age and inconclusive clinical picture, the diagnosis of a lymphoproliferative disorder vs sarcoidosis was unclear, and a biopsy was considered necessary for definitive diagnosis. With primarily choroidal and ciliary body involvement in both eyes, the sensitivity of a diagnostic vitrectomy for intraocular lymphoma was believed to be insufficient because of the relatively mild vitritis (choice A). Although some advocate a combined pars plana vitrectomy with choroidal biopsy in cases such as this one to increase diagnostic yields, we wanted to avoid, if possible, complications related to operating on an eye with active uveitis (eg, hypotony, inducing rhegmatogenous RD).1 The protracted course, unremarkable review of systems, and lack of pain or hypopyon made an underlying infectious etiology unlikely (choice B). Because of the diagnostic dilemma, conflicting treatment algorithms, and risk of future central nervous system involvement with intraocular lymphoma, an incisional flap-based ciliary body–choroidal biopsy was performed in the right eye (choice C). An initial approach with ocular biopsy was favored because of the difficult location of many of the enlarged lymph nodes within the patient’s chest. Systemic corticosteroids were not given until the biopsy could be performed because of the concern that their use may limit diagnostic yield (choice D).2

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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
1.
Lim  LL , Suhler  EB , Rosenbaum  JT , Wilson  DJ .  The role of choroidal and retinal biopsies in the diagnosis and management of atypical presentations of uveitis.   Trans Am Ophthalmol Soc. 2005;103:84-91.PubMedGoogle Scholar
2.
Borenstein  SH , Gerstle  T , Malkin  D , Thorner  P , Filler  RM .  The effects of prebiopsy corticosteroid treatment on the diagnosis of mediastinal lymphoma.   J Pediatr Surg. 2000;35(6):973-976. doi:10.1053/jpsu.2000.6945PubMedGoogle ScholarCrossref
3.
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
4.
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
5.
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
6.
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
7.
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
8.
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
9.
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
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