Focal Retinitis as the primary manifestation of ocular syphilis Ashley R.P. Luke, O.D. Marisa M. Kim, O.D. Zuni Comprehensive Community Health Center

Abstract: We present a case of focal retinitis. Laboratory testing confirms the diagnosis of ocular syphilis. This case report reviews the diagnosis and management of focal retinitis, and the outcome of a patient with ocular syphilis.

I. Case History ● Demographics: 82yo Native American female ● Chief complaint: gradually worsening blurry vision OS with and without glasses ● Ocular history ○ Age related ○ Tear film insufficiency ○ ○ Posterior capsular opacification ● Medical history ○ Diabetes mellitus II with macroalbuminuric diabetic nephropathy, diabetic peripheral neuropathy ○ Hyperlipidemia ○ Osteoporosis ○ Onychomycosis of toenails ● Medications ○ Amlodipine ○ AREDS 2 ○ Aspirin ○ Hydrochlorothiazide ○ Losartan ● Allergies ○ Penicillin ● Last eye exam: July 2015 at Zuni IHS II. Pertinent findings ● Clinical ○ Refraction OD: +0.75-2.75x078 DVA: 20/70++ OS: +0.25-2.25x078 DVA: 20/100-- Add +2.75 ○ SLE ■ ● OS: Large guttata ● OU: round stromal opacity adjacent to visual axis, clear corneal incisions, peripheral corneal degeneration ■ Aqueous OU: clear ■ OU: PC IOL with PCO ○ Tonometry by applanation ■ OD: 17mmHg, OS: 18mmHg ○ Posterior segment

■ Vitreous OU: clear, (-)cells, (-)haze ■ Macula OU: scattered drusen, (-)FLR (-)CSME ■ ONH OU: 0.25rd, with 360 PPA, (-)NVD, (-)edema ■ Periphery ● OD: 7-9:00 RPE hypertrophy surrounding an area of elevated fibrotic tissue ~1.5DD vs. resolving retinitis within the pigmented area. Bordered with heavy RPE hypertrophy (not noted at previous examinations) ● OS: poor views due to corneal scarring ● Physical: oriented to person, place, and time ● Laboratory studies: ○ Reactive Rapid Plasma Reagin (RPR) and fluorescent treponemal antibody absorbed (FTA-ABS) ○ Negative: HIV, ANA, TB ○ Unremarkable CBC ● Others: unsuccessful lumbar puncture at Zuni IHS ○ Referred for lumbar puncture in Albuquerque, scheduled for 09/07/2016 III. Differential Diagnoses ○ ○ retinitis ○ Lyme disease ○ Lymphoma ○ Progressive outer retinal necrosis ○ Syphilis ○ Systemic lupus erythematosus ○ IV. Diagnosis and Discussion  Primary Diagnosis: Ocular syphilis ● Syphilis is a sexually transmitted, chronic infection caused by the spirochete, Treponema pallidum ○ Increasing incidence of syphilis in the United States (CDC) ○ Ocular involvement in 10% of cases (Kiss et al, 2005) ○ May affect the neurological system many years after being contracted ● Clinical signs ○ Ocular symptoms may present during any of the four stages of the disease, but most often occurs in the latent or tertiary stages (Kiss et al, 2005) ■ Leading cause of inactive interstitial ; 3rd leading cause of active interstitial keratitis ○ May affect any part of the eye causing a plethora of complications ■ Argyll-Robertson , interstitial keratitis, , salt and pepper fundus, , and chancres of the (Sahin & Ziaei, 2016) ○ Most common manifestation reported differs between studies, likely , chorioretinitis ● Confirming the diagnosis: Treponema pallidum is unable to be cultured ○ Need both a non-treponemal (RPR or VDLR) and treponemal (FTA-ABS or MHA-TP) test ○ Fundus Fluorescein Angiography (FFA) ■ Leopard spots: acute syphilitic posterior placoid chorioretinitis ■ Vascular staining with severe ocular inflammation ○ Indocyanine Green Angiography (ICGA) ■ Dark dots indicate active inflammation ■ Hot spots indicate longer disease duration

V. Treatment and Management ● Refer to ophthalmologist for treatment of active retinitis ● Refer for further testing: CBC, RPR and FTA-ABS, HIV, ANA, TB, Herpes, Lyme testing ○ Treat underlying cause ● If syphilis is confirmed, lumbar puncture is indicated for testing ○ Latent ocular syphilis or neurosyphilis treatment: intravenous penicillin for 10-14 days ○ Alternative treatment for penicillin allergy: doxycycline or ceftriaxone (Puech et al, 2010; Shen et al, 2015); desensitization (CDC) ○ Consider adding methotrexate post-treatment with penicillin (Sahin & Ziaei, 2016) VI. Conclusion ● Syphilis is the “great masquerader”; test all patients with retinitis for syphilis ● Syphilis is more likely to cause ocular complications at the later stages of the disease ● Treatment includes penicillin; with an allergy to the medication, doctors have found success with doxycycline and ceftriaxone VI. Bibliography ● Available at: http://www.cdc.gov/std/stats14/syphilis.htm. Accessed August 29, 2016. ● Kiss S, Damico FM, Young LH. Ocular manifestations and treatment of syphilis. Semin Ophthalmol. 2005;20(3):161-7. ● 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. ● Puech C, Gennai S, Pavese P, et al. Ocular manifestations of syphilis: recent cases over a 2.5- year period. Graefes Arch Clin Exp Ophthalmol. 2010;248(11):1623-9. ● Sahin O, Ziaei A. Clinical and laboratory characteristics of ocular syphilis, co-infection, and therapy response. Clin Ophthalmol. 2016;10:13-28. ● Shen J, Feng L, Li Y. Ocular syphilis: an alarming infectious . Int J Clin Exp Med. 2015;8(5):7770-7.