Acute syphilitic posterior placoid chorioretinitis: when the great mimicker cannot pretend any more; new insight of an old acquaintance

Abstract

Purpose: To review the multimodal imaging patterns of Acute Syphilitic Posterior Placoid Chorioretinitis (ASPPC).

Methods: A systematic review.

Results: Syphilis has started to attract the attention of researchers once again due to recent surges, with The World Health Organization (WHO) reporting around 12 million new cases per year. When left untreated, syphilis has a mortality rate of 8-58%, with a higher death rate in males. Eye manifestations occur both in secondary and tertiary stages of syphilis, although ocular involvement may occur at any stage of the disease. Syphilis has been always recognized as "the great mimicker" since it can have multiple clinical patterns of presentation. However, Acute Syphilitic Posterior Placoid Chorioretinitis (ASPPC) represents the typical pattern of the disease and can be easily distinguished. In addition, the advent of modern technologies and the progress made in multimodal imaging have provided more details on its identikit: the pattern of pre-retinal, retinal, retinochoroidal and optic nerve involvement can be identified before going through the laboratory work-up for a correct and appropriate investigation of the disease.

Conclusion: This review highlights the peculiar pattern of ASPPC, by reporting the diagnostic process made by all the imaging techniques used for a correct multimodal imaging assessment.

Keywords: Multifocal retinitis; Posterior uveitis; Pre-retinal precipitates; Retinochoroiditis; Syphilis.

Fig. 1

Fundus color picture of tertiary syphilis: note the optic nerve swelling (white arrow)

Fig. 2

Fundus color picture showing pre-retinal lesions overlying active retinitis (black arrows) associated with vitritis

Fig. 3

Fundus fluorescein angiography in a case of tertiary syphilis, revealing segmental leakage typical of retinal vasculitis (black arrows)

Fig. 4

Fundus color picture showing a typical large, roundish, yellowish, placoid lesion affecting the RPE within the macular are, hallmark of ASPPC (A). At fundus fluorescein angiography, the macular lesion corresponds to an hyper-fluorescent area (B), while indocyanine green angiography presents an obvious hypo-fluorescence matching the FFA findings (C)

Fig. 5

Fundus autofluorescence showing a broad hyper-autofluorescence at the macular area (white arrows)

Fig. 6

Spectral domain optical coherence tomography of a tertiary syphilis showing intact external limiting membrane, disrupted ellipsoid zone, thickened and granular hyperreflective RPE, with nodular elevations (white arrows)

Fig. 7

Spectral domain optical coherence tomography of a secondary syphilis showing the presence of sub retinal fluid (white arrow) associated with thickened neurosensory retina and RPE–choriocapillaris complex