Don't Forget What You Can't See: A Case of Ocular Syphilis

Monica I. Lee, MD; Annie W.C. Lee, MD; Sean M. Sumsion, MD; Julie A. Gorchynski, MD


Western J Emerg Med. 2016;17(4):473-476. 

In This Article

Case Report

A 24-year-old male presented to the emergency department (ED) with five days of acute painless progressive bilateral loss of vision without photophobia, discharge, trauma, or contact lens use. Review of systems was negative except for recent alopecia[18,19] The patient was a sexually active homosexual male with a negative HIV test three months prior. Social history included marijuana, cocaine and methamphetamine use.

ED vital signs reported a temperature of 98.7 degrees F, respiratory rate of 16 breaths per minute, heart rate of 90 beats per minute, blood pressure of 113/70 mmHg, 98% O2 saturation on room air, visual acuity of 20/600 bilaterally and intraocular pressures of 8 mmHg in the left eye and 10 mmHg in the right eye. Pupils were equal, round, reactive to light and accommodation and extra ocular movement intact, without Argyll Robertson (AR) pupils. Anterior chamber was clear, without conjunctival injection, foreign body, abrasion or ulceration. No afferent pupillary defect was appreciated. Ophthalmology was urgently consulted to evaluate the patient and perform a dilated fundus exam. Fundoscopy revealed bilateral vitritis (Figure 1) with scattered white tufts and globular white opacities inferiorly suggestive of posterior uveitis (Figure 2). These findings on the fundus exam are consistent with syphilis. The neurological exam was otherwise normal.

Figure 1.

Vitritis Bilateral fundus photos. Vitreous haze, ½ +, retina is flat 360 degrees with arrows pointing to periphlebitis and diffuse homogenous retinal pallor.

Figure 2.

Uveitis Bilateral fundus photos: Inferior vitreous with arrows pointing to vitreous " snowballs," which represent aggregates of inflammatory cells at the level of the pre-retinal vitreous and peripheral retina.

Given the patient's social history and risk for sexually transmitted infections, specifically syphilis that can cause vision loss, and confirmation with ED rapid plasmin reagin (RPR) and HIV tests, ocular syphilis was high on the differential. Syphilitic posterior uveitis was the presumed diagnosis after the ED ophthalmology fundus exam and the first dose of a 14-day course of IV penicillin G was initiated in the ED with a medicine admission. Inpatient cerebral spinal fluid (CSF) results were a white blood cell (WBC) count of 33 cells/μL, red blood cell (RBC) count of 26 cells/μL, a protein level of 44 mg/dL, glucose 53 mg/dL, and a Venereal Disease Research Laboratory (VDRL) titer of 1:2. CSF ink stain, quantiferon gold, Lyme Ig, Bartonella Henselae, HBsAg, HCVAb were negative, as were bacterial and fungal cultures. The patient had an absolute CD4 count of 1347 cells/μL and a viral load of 52,900 c/mL HIV RNA. Magnetic resonance imaging of the brain and orbits were normal.

Current CDC guidelines recommend that any ocular manifestation of syphilis (irits, uveitis and choroidoretinitis) now be treated as neurosyphilis, regardless of the outcome of the LP.[3–5] This is a departure from the past, where the clinical stage and CSF results (+VDRL, WBC cell count > 10 cells/μL, protein > 50 mg/dL) were the deciding factor for the treatment regimen for ocular syphilis. Since ocular manifestations can occur in secondary or tertiary syphilis the CSF results had previously been used to determine the presence of neurosyphilis. Current CDC guidelines recommend that all ocular manifestations of syphilis regardless of the stage of presentation or CSF results be treated as neurosyphilis with a 14-day course of IV penicillin G. An LP is still recommended since analysis provides additional evidence of other central nervous system (CNS) infections especially if the patient has a co-infection with HIV.[1–5]