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The ‘pitchfork sign’ on optical coherence tomography in a case of acute syphilitic posterior placoid chorioretinitis

2019; Wiley; Volume: 97; Issue: 6 Linguagem: Inglês

10.1111/aos.14094

1755-3768

Christos Christakopoulos, Inger Christine Munch,

Ocular Diseases and Behçet’s Syndrome

Acute syphilitic posterior placoid chorioretinitis (ASPPC) is a feature of secondary syphilis characterized by placoid, yellowish outer retinal lesions surrounded by a curvilinear inflammatory edge (De Souza et al. 1988; Gass et al. 1990; Eandi et al. 2012). We present a case of ASPPC depicting fully reversible retinal abnormalities on spectral domain optical coherence tomography (SD-OCT), including the ‘pitchfork sign’ that has previously been described in cases with inflammatory choroidal neovascularization (Hoang et al. 2013). A 56-year-old male patient was referred for blurred vision in the right eye (RE) through 1 week. His best-corrected visual acuity (BCVA) was hand movements in RE and 1.0 (20/20) in LE. Ocular biomicrosopy showed cells (1+) in the anterior chamber and mild vitritis in both eyes. Fundus examination revealed bilateral, circular, placoid, yellowish macular lesions with bright margins (Fig. 1A). SD-OCT manifested disruption of the ellipsoid zone and of the external limiting membrane (Fig. 1C) in both eyes with multiple, hyper-reflective, vertical finger-like densities extending from the outer to the inner retina producing the ‘pitchfork sign’ in RE (Fig. 1D). In LE, there was macular serous detachment and highly reflective outer linear deformity attached to the retinal pigment epithelium (Fig. 1E). The patient admitted having unprotected sexual intercourse with male partners. He presented a truncal maculopapular eruption (Fig. 1B) and was diagnosed with secondary syphilis with positive titres of treponema pallidum flagellum antibody (FTA-ABS) IgM (1:2) and IgG (1:16), and positive rapid plasma reagin (RPR) (1:64) and Wassermann reaction (1:6). Cerebrospinal fluid tested negative for Wassermann reaction and FTA-ABS IgM but positive for FTA-ABS IgG. Human immunodeficiency virus testing was negative. The patient was started on intravenous penicillin G, 6 million IU t.i.d. and methylprednisolone at a starting dose of 80 mg q.d. and subsequently 40 mg q.d. through 1 week followed by intravenous ceftriaxone 2 g p.q. for 10 days. During follow-up there was steady improvement in BCVA reaching 1.0 (20/20) in both eyes at month seven. On SD-OCT, the ‘pitchfork sign’ in the right macula and the serous detachment in left macula disappeared after 2 months, and after 7 months, the outer retinal layer segregation was complete with reappearence of the ellipsoid zone in the right fovea (Fig. 1 F). Serological testing normalized during follow-up. Acute syphilitic posterior placoid chorioretinitis was first described by De Souza et al. (1988) and named by Gass et al. (1990). It can lead to permanent retinal damage if untreated. However, prompt antibiotic therapy results in restoration of visual acuity (Eandi et al. 2012). Different theories exist on the pathogenesis of the macular lesions. De Souza et al. theorized on deposition of soluble immune complexes in the outer retina as the cause of the pathological lesion, whereas Gass et al. postulated that spirochetes are present in and around the inflammatory lesion in the retinal pigment epithelium. The SD–OCT findings in the present case support the localization of the pathological lesions in the outer retina (Eandi et al. 2012). This is in agreement with the development of the ‘pitchfork sign’, which may represent intercalation of outer retinal components during the active inflammatory stage. The ‘pitchfork sign’ has been reported in cases of punctate inner choroidopathy (Hoang et al. 2013) but not in ASPPC. In conclusion, this case of ASPPC demonstrates SD-OCT findings in the outer retinal segments similar to those seen in inflammatory outer retinopathies. The development of the ‘pitchfork sign’ further supports that notion. Eventually, prompt targeted antibiotic therapy resulted in reconstitution of the outer retinal anatomic landmarks on SD-OCT.