Syphilitic posterior placoid chorioretinitis as initial presentation of early neurosyphilis

Syphilitic posterior placoid chorioretinitis as initial presentation of early neurosyphilis

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Syphilitic posterior placoid chorioretinitis as initial presentation of early neurosyphilis夽夽,夽 F.E. Molina-Sócola ∗ , F. López-Herrero, A. Medina-Tapia, T. Rueda-Rueda, M. Contreras-Díaz, J.L. Sánchez-Vicente Servicio de Oftalmología, Hospital Universitario Virgen del Rocío, Sevilla, Spain

a r t i c l e

i n f o

a b s t r a c t

Article history:

Case report: A 36 year-old male with a recent HIV diagnosis, presented with loss of vision of

Received 29 July 2016

his left eye. Ophthalmoscopy revealed a unilateral yellowish placoid lesion in the macula.

Accepted 17 October 2016

After fluorescein angiography, optical coherence tomography, optical coherence tomogra-

Available online xxx

phy angiography, syphilis serology, and cerebrospinal fluid results, he was diagnosed with neurosyphilis and syphilitic posterior placoid chorioretinitis.


Discussion: Acute syphilitic posterior placoid chorioretinitis is a rare ocular manifestation of

Placoid chorioretinitis

syphilis. All patients with characteristic clinical and angiographic findings of acute syphilitic

Placoid maculopathy

posterior placoid chorioretinitis should be tested for a neurosyphilis and human immuno-


deficiency virus co-infection. Early treatment with intravenous penicillin is usually effective


with good visual results.

Human immunodeficiency virus

˜ ˜ S.L.U. All rights © 2016 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana, reserved.

Optical coherence tomographic angiography

Coriorretinitis placoide posterior sifilítica como presentación inicial de neurosífilis temprana r e s u m e n Palabras clave:

˜ Caso clínico: Varón de 36 anos con diagnóstico reciente de VIH con pérdida de visión de

Coriorretinitis placoide

ojo izquierdo. En fundoscopia se observó placa amarillenta en mácula. Tras angiografía

Maculopatía placoide

con fluoresceína, tomografía de coherencia óptica, angiotomografía de coherencia óptica,


pruebas serológicas y examen de LCR se llegó al diagnóstico de neurosífilis y coriorretinitis


placoide posterior sifilítica.

Virus de inmunodeficiencia humana Angiografía por tomografía de coherencia óptica

夽夽 Please cite this article as: Molina-Sócola FE, López-Herrero F, Medina-Tapia A, Rueda-Rueda T, Contreras-Díaz M, Sánchez-Vicente JL. Coriorretinitis placoide posterior sifilítica como presentación inicial de neurosífilis temprana. Arch Soc Esp Oftalmol. 2017. 夽 ˜ Presented partially at the II Congreso de la Sociedad Espanola de Inflamación Ocular (SEIO). ∗ Corresponding author. E-mail address: fredy [email protected] (F.E. Molina-Sócola). ˜ ˜ S.L.U. All rights reserved. 2173-5794/© 2016 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana,

OFTALE-1106; No. of Pages 5


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Discusión: La coriorretinitis placoide posterior es una manifestación clínica poco frecuente de sífilis ocular. En todos los pacientes con coriorretinitis placoide posterior sifilítica debe descartarse la posibilidad de neurosífilis y coinfección con el VIH. El tratamiento temprano con penicilina intravenosa suele ser efectivo, con un buen resultado visual. ˜ ˜ S.L.U. Todos de Oftalmolog´ıa. Publicado por Elsevier Espana, © 2016 Sociedad Espanola los derechos reservados.

Introduction The incidence of syphilis has increased in recent years, particularly in individuals with co-infection by the human immunodeficiency virus (HIV), in whom ocular and central nervous system involvement is quite frequent.1,2 Ocular compromise occurs in 5–8% of syphilis infections and can arise at any stage, although more frequently in the secondary and tertiary stage.3,4 Neurosyphilis is the most severe complication of syphilis. It appears more frequently in patients with HIV co-infection. Up to one third of patients with neurosyphilis exhibit intraocular inflammation which is unrelated to viral charge or CD4 count.

Some cases can exhibit ocular symptoms without neurological expressions, leading to delayed diagnostic and the possibility of severe visual loss.1,5

Clinic case report A patient with HIV infection exhibiting unilateral syphilitic posterior placoid chorioretinopathy (PPC) as debut of early neurosyphilis without neurological signs. Male, 36, bisexual, recently diagnosed with HIV, who consulted due to central black spot in left eye (LE) starting one week earlier. Visual acuity in the right eye (RE) was 1.0 and 0.4 in the LE. No pathological signs were found

Fig. 1 – Retinographs: (a) papillary edema without macular compromise; (b) papillary edema with yellowish plaque in the posterior pole involving the macula; (c) autofluorescence showing hyperautofluorescence areas.

Fig. 2 – OCT: (a) subfoveal neuroepithelium detachment with granular RPE thickening and disruption of the ellipsoid layer temporal to the fovea (arrow); (b) normalization of foveal profile with recovery of RPE and ellipsoid layers, as well as diminished choriocapillary thickness.

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Fig. 3 – FAG: (a) early papillary hyperfluorescence; (b) hyperfluorescence persisting in late times; (c) early hypofluorescence and hyperfluorescence in posterior pole; (d) late papillary hyperfluorescence with hyperfluorescence dots in posterior pole.

in the anterior pole. The RE ocular fundus showed papilla edema. In the LE the papilla was also edematous with central yellowish chorioretinal exudate involving the posterior pole. No associated vitritis was found. Autofluorescence revealed granular hyperautofluorescence (Fig. 1). LE spectral domain optic coherence tomography (DRI OCT Triton; Topcon, Tokyo, Japan) showed subfoveal neuroepithelium detachment with irregular thickening of the retinal pigment epithelium (RPE), loss of the ellipsoid at the paramacular level and choriocapillary thickening (Fig. 2a). In addition, the peripapillary thickness of the nerve fiber layer was increased in both eyes, slightly more in the LE. Fluorescein angiography (FAG) showed papillary hyperfluorescence in both eyes that remained up to late times and early hypofluorescence and late hyperfluorescence areas in the posterior pole with temporal predominance (Fig. 3). Angiographic optical coherence tomography (angio-OCT) (DRI OCT Triton; Topcon, Tokyo, Japan) confirmed dilatation of choroidal and choriocapillary vessels as well as of the deep retinal plexus temporal to the fovea (Fig. 4a). Lumbar puncture produced pathological LCR with positive VDRL. Serology (RPR, FTA-ABS, VDRL) was also positive.

A diagnostic of neurosyphilis was established and treatment was initiated with 24 million IU of sodicum G penicillin during 14 days. At day 10 the patient exhibited VA recovery and normalization of the macula in the LE as well as diminished choroidal thickness in OCT (Fig. 2b); FAG (Fig. 5) and angioOCT showed diminished diameter of the choriocapillary and choroidal vessels as well as normalization of the deep plexus (Fig. 4b). One month after beginning the treatment, LE VA was 1.

Discussion The most frequent expression of ocular syphilis is uveitis. It could be anterior, intermediate, posterior or panuveitis, the latter being the most frequent form.6 Expressions at the posterior level could include vitritis, chorioretinitis, retinal vasculitis, venous occlusion, exudative detachment and rarely necrotizing retinitis. The optic disc can exhibit edema, neuroretinitis, perineuritis, retrobulbar neuritis and optical atrophy.5


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Fig. 4 – angio-OCT comparison: (a) dilatation of choroidal and choriocapillary vessels as well as dilatation of deep retinal plexus temporal to the fovea; (b) post-treatment, showing normalization of the deep plexus and choriocapillary.

PPC is considered to be a distinctive clinic form of ocular syphilis. It was first described by Gass as an inflammatory condition compromising the external retina and the internal choroids, appearing as one or more yellowish plaques at the macular level. It can express uni- or bilaterally.7 There is no consensus on the physiopathology of PPC. According to Gass, an inflammatory reaction occurs at the level of the choroids-RPE-photoreceptor complex that causes the clinical expression of the placoid lesion and photoreceptor dysfunction. It is also been proposed that the immunocomplex deposit in the RPE would be the cause of the granular RPE thickening observed in the present patient.8 The choriocapillary could be the main target of the inflammatory process with subsequent compromise of

RPE and photoreceptors. Initially, the choroids would become thicker followed by reduction after treatment, which are the findings observed in the angio-OCT of the present case. The clinic findings of PPC are quite characteristic, which facilitates its diagnostic. Due to the increased incidence of syphilis-HIV co-infection and of neurosyphilis that can debut without associated neurological expressions, it is important to be aware of the ocular presentation described above as early treatment with penicillin avoids permanent visual loss and diminishes the frequency of more severe forms of neurosyphilis. Accordingly, lumbar puncture must be performed in syphilitic PPC cases to discard early neurosyphilis, even without neurological symptomatology.

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Fig. 5 – At one month evolution: (a) diminished papilla edema; (b) diminished papilla edema with marked posterior plaque reduction and persistence of some yellowish areas in the posterior pole; (c) diminished papilla edema without late hyperfluorescence; (d) slight late hyperfluorescence with some hyperfluorescence areas in posterior pole below the upper temporal arcuate.

Conflict of interests No conflict of interests was declared by the authors.


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4. Moradi A, Salek S, Daniel E, Gangaputra S, Ostheimer TA, Burkholder BM, et al. Clinical features and incidence rates of ocular complications in patients with ocular syphilis. Am J Ophthalmol. 2015;159:334–43. 5. Lee SY, Cheng V, Rodger D, Rao N. Clinical and laboratory characteristics of ocular syphilis: a new face in the era of HIV co-infection. J Ophthalmic Inflamm Infect. 2015;5:56. 6. Davis JL. Ocular syphilis. Curr Opin Ophthalmol. 2014;25:513–8. 7. Eandi CM, Neri P, Adelman RA, Yannuzzi LA, Cunningham ET Jr, International Syphillis Study Group. Acute syphilitic posterior placoid chorioretinitis: report of a case series and comprehensive review of the literature. Retina. 2012;32:1915–41. 8. Burkholder BM, Leung TG, Ostheimer TA, Butler NJ, Thorne JE, Dunn JP. Spectral domain optical coherence tomography findings in acute syphilitic posterior placoid chorioretinitis. J Ophthalmic Inflamm Infect. 2014;4:2.