The following case is featured in the "You Make The Diagnosis" section of the June 2024 Optometric Retina Society quarterly e-newsletter, which can be found here.

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A 60-year-old Caucasian male was referred in for ophthalmic evaluation from a local emergency department (ED) for a suspected retinal artery occlusion. He had presented to the local ED one week prior due to sudden, painless central vision loss in the left eye. A full stroke work-up had been performed with unremarkable CT imaging and vasculopathic serology. He was started on apixaban 5 mg PO BID, 0.5% timolol BID OS, and referred to the ophthalmology department for further ophthalmic examination and testing. His ocular history was otherwise unremarkable. Medical history included coronary artery disease, hypertension, iron deficiency anemia, and non-ST-elevation myocardial infarction. Review of systems was positive for a maculopapular rash on his abdomen and both arms. Best corrected visual acuities were 20/20 OD and 20/150 OS (PHNI). Entrance testing was normal in the right eye and left eye revealed 1+ APD, reduced color vision, and central scotoma on confrontation field testing. Intraocular pressure was 13 mmHg in each eye. Gonioscopy findings were open to scleral spur without NVA or PAS OU. Anterior segment evaluation was unremarkable OU. Dilated examination of the vitreous revealed 1-2+ cell OD and 1+ cell OS without hemorrhage. Retinal evaluation showed bilateral yellow, hypopigmented areas throughout the macula and posterior pole more prominent in the left eye. Retinal vasculature was normal without any signs of vasculitis, periphlebitis, or hemorrhages OU.
 
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Fundus autofluorescence showed hyper-autofluorescent multifocal lesions OD and hyper-autofluorescent placoid-like lesions OS corresponding to the lesions noted on fundoscopy. OCT imaging revealed hyperreflective retinal pigment epithelium (RPE) nodules and ellipsoid zone (EZ) disruption OS >> OD. Given the presence of bilateral chorioretinitis and the maculopapular rash on his abdomen and arms, treatment was withheld until infectious titer results were available. He was instructed to stop timolol. Serology returned positive for serum T. pallidum antibodies at 44.70 Chemiluminescence Units (CU) (normal: <0.90) and T. pallidum reflexed-RPR at 1:1024 (normal: not present) with an automated message sent to the Maryland Department of Health. He was admitted same-day to the hospital ED and received IV penicillin G 4 million units continuously for 14 days. Additional STI testing was negative for HIV, hepatitis C, chlamydia, and gonorrhea. Follow up was scheduled at 6- and 12-month visits with infectious disease at hospital discharge. One-month ophthalmic follow up revealed significant functional and structural improvement in the left eye with IV penicillin treatment including BCVA 20/30-2 OS (PH 20/25+1).

Systemic and ophthalmic manifestations of syphilitic infection are caused by the spirochete bacterium Treponema pallidum. Transmission of the disease occurs primarily via sexual contact, while congenital syphilis may occur through transplacental passage from mother to fetus during pregnancy.1-3 Syphilitic infection most commonly affects males but spans patients of all decades of life. Recent statistics from the Center for Disease Control (CDC) suggest an alarming increase in prevalence for all stages of syphilis in the United States in the last one year (17.0%) and five years (78.9%) between 2018 and 2022.4 Among groups most affected by this epidemic outbreak include gay, bisexual, and other men who have sex with men (MSM). Syphilitic infection is categorized by four stages: primary, secondary, latent (early and late), and tertiary. Ocular complications of syphilitic infection can occur at any stage of the disease but present most commonly in secondary, late latent or tertiary syphilis.1,5-7 Uveitis is the most common ophthalmic presentation with posterior uveitis and panuveitis frequently encountered posterior segment findings.1,3,6,7 Acute syphilitic posterior placoid chorioretinitis (ASPPC), as diagnosed here, is a rare posterior manifestation of ocular syphilis.5 Clinical findings include a large, yellow, placoid lesion located within the posterior pole at the level of the RPE.3,7 SD-OCT is the gold standard for ASPPC diagnosis given its characteristic findings of EZ disruption and hyperreflective nodular elevations of the RPE.7

Diagnosis of syphilis is made with a combination of nontreponemal and treponemal tests. For patients exhibiting signs of neurosyphilis such as meningitis, cranial nerve involvement, stroke, or altered mental status, it is recommended to conduct a CSF analysis to confirm neurosyphilis diagnosis.6 All patients diagnosed with ocular syphilis should also have HIV testing performed given the elevated risk of comorbid infection.6 The preferred treatment for syphilis at all stages of disease is parenteral penicillin G as recommended by the CDC.6 Treatment for ocular syphilis recommends aqueous crystalline IV penicillin G with 18-24 million units per day for 10-14 days.6 After completion of therapy, patients should have clinical and serological testing performed at 6- and 12-months.6 All sexual partners should also be evaluated and, in specific cases, treated presumptively.6 Healthcare providers must report all syphilis cases by stage to their local or state health department.8

 

1. Kiss S, Damico FM, Young LH. Ocular manifestations and treatment of syphilis. Semin Ophthalmol. Jul-Sep 2005;20(3):161-7. doi:10.1080/08820530500232092
2. Singh AE, Romanowski B. Syphilis: review with emphasis on clinical, epidemiologic, and some biologic features. Clin Microbiol Rev. Apr 1999;12(2):187-209. doi:10.1128/CMR.12.2.187
3. Furtado JM, Simoes M, Vasconcelos-Santos D, et al. Ocular syphilis. Surv Ophthalmol. Mar-Apr 2022;67(2):440-462. doi:10.1016/j.survophthal.2021.06.003
4. Sexually Transmitted Infections Surveillance, 2022. Centers for Disease Control and Prevention. Updated January 30, 2024. Accessed March 19, 2024, https://www.cdc.gov/std/statistics/2022/default.htm
5. Dutta Majumder P, Chen EJ, Shah J, et al. Ocular Syphilis: An Update. Ocul Immunol Inflamm. 2019;27(1):117-125. doi:10.1080/09273948.2017.1371765
6. Workowski KA, Bachmann LH, Chan PA, et al. Sexually Transmitted Infections Treatment Guidelines, 2021. MMWR Recomm Rep. Jul 23 2021;70(4):1-187. doi:10.15585/mmwr.rr7004a1
7. Neri P, Pichi F. Acute syphilitic posterior placoid chorioretinitis: when the great mimicker cannot pretend any more; new insight of an old acquaintance. J Ophthalmic Inflamm Infect. Feb 22 2022;12(1):9. doi:10.1186/s12348-022-00286-2
8. What Healthcare Providers Can Do. Centers for Disease Control and Prevention. Updated January 30, 2024. Accessed March 26, 2024, https://www.cdc.gov/std/syphilis/CTAproviders.htm