Syphilitic Outer Retinopathy: A Case Report and Review of the Literature

Abstract

Purpose: This work presents a case of syphilitic outer retinopathy with findings similar to those of acute zonal occult outer retinopathy (AZOOR). We also discuss the clinical characteristics, treatment, and prognosis of this entity.

Methods: A case report and systematic literature review are presented.

Results: A 56-year-old woman presented with acute vision loss, localized photopsia, a central scotoma, and retinal findings that were all consistent with AZOOR. A further workup led to a diagnosis of syphilis. Oral prednisone and intravenous penicillin resulted in the resolution of the posterior uveitis and the restoration of visual acuity. However, the central scotoma remained at the 3-year follow-up visit.

Conclusions: Syphilitic outer retinopathy is a distinct entity characterized by the disruption of the ellipsoid zone visible on optical coherence tomography and a corresponding increase in fundus hyperautofluorescence in the affected areas. Although some patients may present with a demarcation line, as is seen with AZOOR, the fundus is oftentimes unremarkable or may show only subtle retinal pigment epithelium changes. Uveitis resolution and visual acuity restoration may be expected following treatment; however, visual field disturbances may persist.

Keywords: acute zonal occult outer retinopathy; case report; central scotoma; syphilitic outer retinopathy; uveitis.

Figure 1.

Ultra-widefield fundus imaging and optical coherence tomography of the left eye on presentation. (A) Color photographs show a white area of discoloration with a demarcation line that is superior to the disc and the macula. Further macular retinal pigment epithelial changes are also noted. (B) Fundus autofluorescence is markedly increased within the posterior pole, up to the level of the demarcation line, superiorly, and extending temporally up to the midperiphery. (C) A late-phase intravenous fluorescein angiogram revealed late-phase disc hyperfluorescence, macular and perivenular leakage within the posterior pole and on the temporal periphery, and marked hyperfluorescence with associated leakage of the demarcation line. (D) Late-phase indocyanine green angiography revealed a geographic area of hypocyanescence. (E) Spectral-domain optical coherence tomography revealed posterior hyaloid separation, vitreous cells, and ellipsoid zone disruption as well as subretinal drusenoid deposits.

Figure 2.

Color photograph taken at presentation shows the presence of symmetric brownish macules, papules, and plaques with collarettes of scales on the soles of both feet.

Figure 3.

Left-eye visual field progression (Humphrey, central 30-2 threshold test, stimulus III, white, SITA Fast). (A) On presentation, there was a profound central scotoma with a temporal extension. (B) Six months and (C) 3 years after the conclusion of therapy, the scotoma improved markedly but nevertheless persisted. ASB indicates apostilbs; DC, double cylinder; DS, double sphere; GHT, glaucoma hemifield test; MD, mean deviation; NEG, negative; POS, positive; PSD, pattern standard deviation; VFI, visual field index.

Figure 4.

Ultra-widefield fundus imaging and optical coherence tomography of the left eye 3 months following the completion of therapy. (A) Color photograph reveals the resolution of the retinitis and the disappearance of the demarcation line. (B) Fundus autofluorescence shows a marked improvement of the macular hyperautofluorescence, with only slight hyperfluorescence remaining superior to the disc. (C) A late-phase fluorescein angiogram shows the resolution of the disc, macular, and perivascular leakage, and the absence of a hyperfluorescent demarcation line. (D) Late-phase indocyanine green angiography reveals a residual area of late hypocyanescence encompassing the fovea and nasal macula and extending superior to the disc. (E) Spectral-domain optical coherence tomography reveals the restoration of the inner segment/outer segment junction area, the resolution of the drusenoid deposits, and a paucity of vitreous cells.