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Bilateral Hemorrhagic Optic Disc Edema in a Middle-aged Man

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 man with a history of diabetes, hypertension, intravenous drug abuse, and multiple sexual partners presented to the emergency department with painless vision loss in both eyes for 10 days, left eye worse than right eye, and photophobia in both eyes. His visual acuity was count fingers OD and 20/70 OS. Intraocular pressure was 20 mm Hg OD and 16 mm Hg OS. There were 1+ (10-12 cells/high-power field) anterior chamber cells in both eyes with very hazy posterior pole details in the right eye and minimal vitreous haze in the left eye. There was a hazy view to the posterior pole in the right eye with disc edema and multiple flame hemorrhages surrounding the nerve. Examination of the posterior pole in the left eye revealed partial obstruction of all vessels and a complete halo on the disc with numerous flame hemorrhages emanating from and surrounding the optic nerve, along with a few dot blot hemorrhages within the macula (Figure). His blood pressure was 173/137 mm Hg, but the rest of his vital signs were normal.

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Syphilitic panuveitis and optic neuritis

A. Obtain HIV and syphilis serology results

In this patient with hemorrhagic optic neuritis and evidence of panuveitis, infection must first be ruled out before initiating any systemic corticosteroids (choice A). A patient with a history of drug use and many sexual partners further supports ruling out an infection, particularly syphilis and HIV. Idiopathic demyelinating optic neuritis is a common cause of optic nerve edema with abnormal visual function in patients younger than 50 years, although it typically does not present with panuveitis.1 Therefore, beginning methylprednisolone would not be appropriate without proper systemic workup (choice B). Cytomegalovirus optic neuritis should be on the differential diagnosis for any patient with substantial risk factors for HIV/AIDS, although there were no signs suggestive of cytomegalovirus retinitis on examination and therefore treatment with foscarnet is not appropriate (choice C). Intracranial imaging may be warranted in the evaluation of papilledema, but magnetic resonance imaging of the brain would be more appropriate than computed tomography of the head, as it better detects optic neuritis and cerebrospinal fluid outflow obstructions (choice D).1 Additionally, his subsequent blood pressure measurements lowered and his initial reading was felt to be transient in the setting of an emergency visit, and thus, hypertension was not felt to be the causative etiology.

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Article Information

Corresponding Author: James P. Dunn, MD, Mid Atlantic Retina, Retina Service of Wills Eye Hospital, 840 Walnut St, Ste 1020, Philadelphia, PA 19107 (jpdunn@willseye.org).

Published Online: January 13, 2022. doi:10.1001/jamaophthalmol.2021.3419

Conflict of Interest Disclosures: None reported.

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

References
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Margolin  E .  The swollen optic nerve: an approach to diagnosis and management.   Pract Neurol. 2019;19(4):302-309. doi:10.1136/practneurol-2018-002057PubMedGoogle ScholarCrossref
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Klein  A , Fischer  N , Goldstein  M , Shulman  S , Habot-Wilner  Z .  The great imitator on the rise: ocular and optic nerve manifestations in patients with newly diagnosed syphilis.   Acta Ophthalmol. 2019;97(4):e641-e647. doi:10.1111/aos.13963PubMedGoogle ScholarCrossref
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Oliver  GF , Stathis  RM , Furtado  JM ,  et al; International Ocular Syphilis Study Group.  Current ophthalmology practice patterns for syphilitic uveitis.   Br J Ophthalmol. 2019;103(11):1645-1649. doi:10.1136/bjophthalmol-2018-313207PubMedGoogle ScholarCrossref
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Ropper  AH .  Neurosyphilis.   N Engl J Med. 2019;381(14):1358-1363. doi:10.1056/NEJMra1906228PubMedGoogle ScholarCrossref
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Gu  X , Gao  Y , Yan  Y ,  et al.  The importance of proper and prompt treatment of ocular syphilis: a lesson from permanent vision loss in 52 eyes.   J Eur Acad Dermatol Venereol. 2020;34(7):1569-1578. doi:10.1111/jdv.16347PubMedGoogle ScholarCrossref
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Dutta Majumder  P , Chen  EJ , Shah  J ,  et al.  Ocular syphilis: an update.   Ocul Immunol Inflamm. 2019;27(1):117-125. doi:10.1080/09273948.2017.1371765PubMedGoogle ScholarCrossref
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Totten  YR , Hardy  BM , Bennett  B , Rowlinson  MC , Crowe  S .  Comparative performance of the reverse algorithm using architect syphilis TP versus the traditional algorithm using rapid plasma reagin in Florida’s public health testing population.   Ann Lab Med. 2019;39(4):396-399. doi:10.3343/alm.2019.39.4.396PubMedGoogle ScholarCrossref
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Dunseth  CD , Ford  BA , Krasowski  MD .  Traditional versus reverse syphilis algorithms: a comparison at a large academic medical center.   Pract Lab Med. 2017;8:52-59. doi:10.1016/j.plabm.2017.04.007PubMedGoogle ScholarCrossref
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Tyagi  M , Kaza  H , Pathengay  A ,  et al.  Clinical manifestations and outcomes of ocular syphilis in Asian Indian population: analysis of cases presenting to a tertiary referral center.   Indian J Ophthalmol. 2020;68(9):1881-1886. doi:10.4103/ijo.IJO_809_20PubMedGoogle Scholar
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Moradi  A , Salek  S , Daniel  E ,  et al.  Clinical features and incidence rates of ocular complications in patients with ocular syphilis.   Am J Ophthalmol. 2015;159(2):334-43.e1. doi:10.1016/j.ajo.2014.10.030PubMedGoogle 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 CME points in the American Board of Surgery’s (ABS) Continuing 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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