Acquired syphilis is a sexually transmitted infection caused by the spirochete Treponema pallidum and involves the eye in 5% to 8% of cases.1 Although syphilis became less common in the late 20th century, the incidence has been steadily rising, and syphilis remains a major public health concern.2 Acute syphilitic posterior placoid chorioretinitis (ASPPC) is a rare ocular finding initially described by Gass.3 Most patients diagnosed with ASPPC are promptly treated with systemic antibiotics and usually have good visual recovery with disease resolution. We present a patient with untreated ASPCC that progressed to widespread necrotizing chorioretinitis. The disease evolution was captured with multimodal retinal imaging including spectral-domain ocular coherence tomography (SD-OCT) and fluorescein angiography (FA). To our knowledge, this is the first report documenting the evolution of untreated ASPCC to significant retinal atrophy.
A 57-year-old man presented with headache, weight loss, and visual acuity of 20/30 in the right eye and counting fingers in the left eye. He was taking 60 mg of oral prednisone daily, which was prescribed by an outside emergency department physician for presumed giant cell arteritis because the patient had a headache with elevated erythrocyte sedimentation rate. The patient did not keep his scheduled follow-up appointment after this emergency visit. His medical history was otherwise nonsignificant. The right eye was healthy (Figure 1A–C). The left eye displayed a macular placoid lesion (Figure 1D); OCT of this lesion revealed ellipsoid zone disruption (Figure 1E), and FA demonstrated stippled hypofluorescence (Figure 1F). Rapid plasma reagin (RPR) and fluorescent treponemal antibody (FTA-Abs) testing was recommended, but the patient failed to follow up for any further evaluation.
Initial presentation. (A) Color fundus photograph of the right eye demonstrates a normal posterior pole. (B) SD-OCT of the right eye with normal foveal contour and retinal architecture. (C) Fluorescein angiography (FA) of the right eye (left) and late-phase indocyanine green angiography (ICGA) (right) demonstrating preserved vasculature. (D) Color fundus photograph of the left eye with macular placoid chorioretinal lesion consistent with acute syphilitic posterior placoid chorioretinitis. (E) SD-OCT of the left eye with disruption of photoreceptor ellipsoid signal. (F) FA of the left eye (left) and late-phase ICGA (right) with hyperfluorescence with a stippled pattern.
He returned 6 weeks later with visual acuity of 20/200 in the right eye and hand motion in the left eye. The right eye developed panuveitis with retinal hemorrhages and choroiditis (Figure 2A, page 608). SD-OCT revealed disruption of the retinal architecture (Figure 2B), and FA revealed areas of nonperfusion (Figure 2C). Although the left eye had 4+ vitreous haze, limited peripheral views suggested a multifocal chorioretinitis.
The right eye 5 weeks later. (A) Composite color fundus photograph of the right eye with superotemporal infiltrates, multifocal retinal hemorrhages, and disc edema. (B) SD-OCT of the right eye with gross disruption of macular architecture and the inner segment/outer segment junction. (C) Fluorescein angiography of the right eye (left) and early-phase indocyanine green angiography (right) with branch retinal vein occlusion. Note: Dense vitritis in the left eye inhibited imaging modalities at this stage of the disease process.
The patient underwent diagnostic vitreous tap in both eyes and intravitreal injection of ganciclovir and foscarnet in the unlikely event that he had developed herpetic retinitis. He was noted to have a truncal and palmar rash. Laboratory results returned RPR titer of 1:256 and positive FTA-Abs, confirming the diagnosis of syphilis, and he was started on intravenous penicillin G. HIV Ab/Ag screening results were positive, with a CD4 count of 224. His condition improved and he was discharged on intravenous penicillin, highly active antiretroviral therapy, and prednisolone acetate eye drops.
One month later, his vision improved to 20/50 in the right eye but remained hand motion in the left eye. Examination demonstrated resolution of active chorioretinitis in the right eye (Figure 3A, page 609), with retinal atrophy and optic nerve pallor in the left eye (Figure 3D). Disruption of the retinal pigment epithelium (RPE) and the ellipsoid zone persisted on OCT of the right eye (Figure 3B). OCT of the left eye demonstrated diffuse retinal atrophy (Figure 3E). FA and indocyanine green angiography demonstrated retinal and choroidal ischemia (Figure 3F).
Both eyes 8 weeks after presentation and following diagnosis and treatment. (A) Composite color fundus photograph of the right eye with optic nerve pallor and diffusely attenuated vasculature. Although there is resolution of multifocal chorioretinal lesions, persistent intraretinal hemorrhage remains. (B) SD-OCT of the right eye with normalization of foveal contour with persistent disruption of the RPE and inner segment/outer segment. (C) Fluorescein angiography of the right eye (left) and late-phase indocyanine green angiography (ICGA) (right) with blocking from hemorrhages. (D) Color fundus photograph of the left eye with severe vascular attenuation and nerve pallor. (E) SD-OCT in the left eye with gross disruption of retinal architecture. (F) Fluorescein angiography of the left eye (left) and ICGA (right) with hypofluorescence and abnormal retinal circulation.
ASPPC presents as a characteristic pale-yellow, placoid, subretinal lesion with central fading in patients with syphilis.3 FA findings of ASPPC include early hypofluorescence in a stippled “leopard spot” pattern, with staining in the late frames. Histopathology is unknown, but ophthalmoscopic and angiographic findings suggest that there is active inflammation within the retinal vasculature, RPE, and choriocapillaris that may contain spirochetes similar to syphilitic mucocutaneous lesions.3 To date, only 60 cases with this ASPPC have been described in the literature. In a recent cohort of 16 patients with ASPPC, 60% were co-infected with HIV, as was our patient.4
SD-OCT has emerged as a new imaging modality for ASPCC. A recent case series describes OCT findings before and after treatment.5 At presentation (1 to 9 days after symptom onset), OCT revealed disruption of the photoreceptor ellipsoid signal, subretinal fluid, RPE thickening, and hyperreflective choroidal lesions. The standard treatment for neurosyphilis, intravenous penicillin G, was initiated in all patients within 10 to 14 days of presentation. Post-treatment OCT displayed restoration of the ellipsoid and RPE layers in nearly all patients. Because most patients had resolution of OCT findings, the authors suggest that retinal findings are secondary to temporary immunologic impairment.5 At presentation, our patient displayed clinical and angiographic evidence of ASPCC, and his initial OCT findings were consistent with previous reports.3,5 However, because he did not return to the clinic, there was a 6-week delay between ASPCC onset and initiation of treatment. His post-treatment OCT demonstrates extensive atrophy with persistent scattered retinal and choroidal hyperreflectivity (Figure 3E). This suggests that untreated ASPCC can progress and cause significant structural damage.
Our patient was treated with oral corticosteroids prior to presentation. Several reports describe the onset of ocular syphilis after oral steroids without concurrent antibiotic coverage, suggesting that immunosuppression leads to treponemal propagation and new lesion formation.6 Thus, steroids may have contributed to the ASPCC onset and progression observed in our patient. In addition to ASPPC, a punctate retinitis with inner retinal and preretinal white dots has been reported rarely in association with ocular syphilis, and this was present in the superotemporal retina of our patient (Figure 2A).7 Punctate retinitis may represent a phenotypical variation of the involved T. pallidum serotype; serotyping was not performed in our patient.7
Our patient presented to us after a course of un-monitored, high-dose steroid therapy. Lack of follow-up and treatment delay resulted in progression to a bilateral necrotizing chorioretinitis. This evolution was captured with multimodal retinal imaging, and diffuse outer retinal atrophy on OCT correlated with his poor visual outcome. Because syphilis is increasing in incidence, it should be considered in the differential diagnosis in all patients with unexplained ocular inflammation prior to initiation of steroid therapy.
- Tucker JD, Li JZ, Robbins GK, et al. Ocular syphilis among HIV-infected patients: a systematic analysis of the literature. Sex Transm Infect. 2011;87(1):4–8. doi:10.1136/sti.2010.043042 [CrossRef]
- 2012 STD Surveillance Syphilis. CDC. http://www.cdc.gov/std/syphilis/stats.htm. Accessed February 1, 2014.
- Gass JD, Braunstein RA, Chenoweth RG. Acute syphilitic posterior placoid chorioretinitis. Ophthalmology. 1990;97(10):1288–1297. doi:10.1016/S0161-6420(90)32418-1 [CrossRef]
- Eandi CM, Neri P, Adelman RA, Yannuzzi LA, Cunningham ET. International Syphilis Study Group. Acute syphilitic posterior placoid chorioretinitis: report of a case series and comprehensive review of the literature. Retina. 2012;32(9):1915–1941.
- Pichi F, Ciardella AP, Cunningham ET, et al. Spectral domain optical coherence tomography findings in patients with acute syphilitic posterior placoid chorioretinopathy. Retina. 2014;34(2):373–384. doi:10.1097/IAE.0b013e3182993f11 [CrossRef]
- Zamani M, Garfinkel RA. Corticosteroid-induced modulation of acute syphilitic posterior placoid chorioretinitis. Am J Ophthalmol. 2003;135(6):891–894. doi:10.1016/S0002-9394(02)02160-8 [CrossRef]
- Wickremasinghe S, Ling C, Stawell R, Yeoh J, Hall A, Zamir E. Syphilitic punctate inner retinitis in immunocompetent gay men. Ophthalmology. 2009;116(6):1195–1200. doi:10.1016/j.ophtha.2008.12.055 [CrossRef]