Spectral domain optical coherence tomography findings in acute syphilitic posterior placoid chorioretinitis
© Burkholder et al.; licensee Springer. 2014
Received: 2 November 2013
Accepted: 23 January 2014
Published: 27 January 2014
We describe the spectral domain optical coherence tomography (SD-OCT) findings in three patients with acute syphilitic posterior placoid chorioretinitis (ASPPC). The SD-OCT images demonstrate the pathologic changes in ASPPC with a high level of anatomic detail and may provide information about the pathophysiology of the disease.
We report a series of three consecutive patients seen at the Wilmer Eye Institute in 2012 and 2013 who presented with clinical and laboratory findings consistent with a diagnosis of unilateral ASPPC. Two of the three patients had HIV co-infection with good immune recovery. SD-OCT images from their initial (pre-treatment) presentation demonstrated thickening and hyperreflective nodularity of the choroid-retinal pigment epithelium (RPE) complex, with focal disruption of the overlying photoreceptor inner segment-outer segment junction in the areas corresponding to the retinal lesions seen on clinical examination. These changes improved with intravenous antibiotic treatment over a 3-month period of follow-up.
SD-OCT imaging in ASPPC demonstrates reversible, focal thickening, and nodularity of the RPE with disruption of the overlying photoreceptor inner segment-outer segment junction. We believe that these SD-OCT images support the concept that ASPPC involves an inflammatory process at the level of the choroid-RPE with resultant structural and functional changes in the retinal photoreceptors. Further study with OCT imaging may be helpful in better understanding this disease.
KeywordsSyphilis Chorioretinis Optical coherence tomography Retinal pigment epithelium Photoreceptor
Acute syphilitic posterior placoid chorioretinitis (ASPPC) is a distinct clinical presentation of ocular syphilis, characterized by large, yellow-white geographic lesions involving the macula at the level of the outer retina/retinal pigment epithelium (RPE)[1, 2]. The clinical, angiographic, and autofluorescence findings have been described[1–7]. High-resolution spectral domain optical coherence tomography (SD-OCT) imaging allows the evaluation of ASPPC with a new level of anatomic detail and may provide more information about the pathophysiology of this disease. We describe here the SD-OCT findings in three patients with ASPPC.
Three patients with ASPPC, based on clinical characteristics and positive serologic treponemal testing, were identified from the records from the Division of Ocular Immunology in the Wilmer Eye Institute. SD-OCT (Spectralis; Heidelberg Engineering, Heidelberg, Germany) macular images from these patients were reviewed. Institutional Review Board (IRB)/Ethics Committee approval was obtained.
CD4 count (cells/μL)
Pre-treatment visual acuity
Post-treatment visual acuity
In all three patients, SD-OCT images demonstrated common features of (1) hyperreflective nodularity and thickening of the RPE in the area corresponding to the fundus lesion and (2) disruption and loss of the photoreceptor inner segment-outer segment junction overlying these RPE changes. No subretinal or interstitial fluid was seen. Despite visual improvement, subtle OCT changes persisted in the affected eyes, relative to the unaffected fellow eyes, 3 months after antibiotic therapy. It is unclear whether these mild abnormalities will continue to resolve with time.
Time domain OCT (TD-OCT), a lower-resolution predecessor to SD-OCT, first demonstrated hyperreflectivity and thickening of the subfoveal RPE-choriocapillaris complex in ASPPC[3, 8, 9]. A recent case series of SD-OCT findings in this disease reports nodular RPE thickening and photoreceptor disruption, as well as punctate hyperfluorescence in the choroid. The authors also note transient subretinal fluid, which resolved before treatment, in about half of the eyes on initial presentation. We observed similar findings in the RPE and photoreceptor layers of our patients. The absence of subretinal fluid in our series may reflect lag time between initial onset of symptoms and presentation to our clinic. While patients 1 and 3 presented to our clinic about a week after symptom onset, patient 2 reported visual changes for about 2 months prior to his referral to our clinic. In the series by Pichi and colleagues, subretinal fluid was noted only in patients who had imaging done within the first 2 days of presentation; images from 1 week after presentation and later failed to demonstrate any subretinal fluid. The images from our series also support the concept that subretinal fluid is a very early feature of ASPPC.
We also observed that, while two of the three patients in our series had HIV co-infection, there were no clear differences in clinical presentation, OCT findings, or response to treatment between these patients and the patient without HIV. Of note, both patients had been diagnosed with HIV more than 10 years ago and were on antiretroviral therapy with good immune recovery.
The pathophysiology of ASPPC is not completely understood. Gass et al. postulated that widespread dispersion of spirochetes may cause an inflammatory reaction at the level of the choroid-RPE-photoreceptor complex, resulting in the clinical appearance of the white placoid lesion and photoreceptor dysfunction. Deposition of soluble immune complexes in the RPE has also been invoked in the pathophysiology. Other imaging modalities may be helpful in understanding the pathogenesis of these fundus lesions. Indocyanine green angiography (ICGA) demonstrates hypofluorescence of the fundus lesions, suggestive of inflammation at the level of the inner choroid[3, 8]. The choriocapillaris, then, may be the primary focus of the inflammatory process within the eye, with contiguous spread to RPE and then to the photoreceptors. Some authors have postulated that ICGA hypofluoresence results from deposition of degraded material from the RPE and photoreceptor segments. Similarly, FAF imaging in the eyes with ASPPC demonstrates increased autofluorescence within the lesions, consistent with accumulation of lipofuscin or photoreceptor outer segment remnants in the RPE[3, 7]. The SD-OCT images in our series demonstrating RPE nodularity may be consistent with this theory. Further study is needed, both in the earliest phases of the disease, as well as in long-term follow-up.
In conclusion, SD-OCT imaging in patients with ASPPC demonstrate characteristic changes, including RPE deposits and disruption of the photoreceptor layers. These changes are consistent with an inflammatory process at the level of the choroid/RPE complex, with extension to the photoreceptors, and are largely reversible with appropriate antibiotic therapy. SD-OCT imaging of the chorioretinal lesions in the eyes with ASPPC may provide a better understanding of the pathophysiology of this particular type of syphilitic chorioretinitis.
All authors are affiliated with the Division of Ocular Immunology at the Wilmer Eye Institute. JET is a professor of Ophthalmology and Epidemiology. JPD is an associate professor of Ophthalmology. BMB, NJB, and TGL are assistant professors of Ophthalmology. TAO is a Uveitis Fellow.
acute syphilitic posterior placoid chorioretinitis
indocyanine green angiography
Institutional Review Board
retinal pigment epithelium
rapid plasma reagin
spectral domain optical coherence tomography
time domain optical coherence tomography.
We thank the Wilmer Photography Department for the images used in this paper.
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