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Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 675-678

Sequential imaging in a case of relentless placoid chorioretinitis

Department of Vitreoretina, M M Joshi Eye Institute, Hubballi, Karnataka, India

Date of Submission06-Feb-2021
Date of Acceptance05-May-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Apoorva Ayachit
Department of Vitreoretina, M M Joshi Eye Institute, Hubballi - 580 021, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_321_21

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Relentless placoid chorioretinopathy (RPC) is a relatively rare and more severe variant of acute posterior multiple placoid pigment epitheliopathy (APMPPE) and serpiginous choroidopathy (SC). The lesions can first involve the periphery and can recur in crops over months to years, causing atrophy in healed areas, thus the term relentless or ampiginous. Literature about RPC is limited to case reports and case series. The etiology and treatment of this condition are still unclear. In this illustrative essay, we demonstrate sequential clinical, autofluorescence (AF), and optical coherence tomography (OCT) imaging findings in a case of RPC that progressed over 8 weeks to cause bilateral severe vision loss.

Keywords: Ampiginous choroidopathy, autofluorescence, interdigitation zone, optical coherence tomography, relentless placoid chorioretinopathy

How to cite this article:
Ayachit A, Joshi M, Yadav N, Joshi S, Ayachit G. Sequential imaging in a case of relentless placoid chorioretinitis. Indian J Ophthalmol Case Rep 2021;1:675-8

How to cite this URL:
Ayachit A, Joshi M, Yadav N, Joshi S, Ayachit G. Sequential imaging in a case of relentless placoid chorioretinitis. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Nov 27];1:675-8. Available from: https://www.ijoreports.in/text.asp?2021/1/4/675/327734

Relentless placoid chorioretinopathy (RPC) is an inflammatory choriocapillaropathy that causes multiple yellowish placoid lesions in the early stages.[1] Lesions begin in the midperiphery and periphery and involve the macula in the later stages. Healed lesions are associated with severe atrophy of the outer retina, retinal pigment epithelium (RPE), and inner choroid.[2]

  Case Report Top

A 32-year-old Asian-Indian male presented with sudden, painless loss of vision in the right eye (RE). Visual acuity (VA) in the RE was 3/60 and left eye (LE) was 6/9. There were no anterior chamber cells, flare, or keratic precipitates. RE fundus showed healed lesions of chorioretinitis in the superior retina with active margins progressing toward the macula [Figure 1]a. Hyperautofluorescence (hyper-AF) was noted at the active margins [Figure 1]b. Left eye fundus showed healed retinal lesions inferiorly with clinically active margins that showed hyper-AF [Figure 1]c and [Figure 1]d. Optical coherence tomography (OCT) scans in both eyes (BE) showed outer nuclear layer (ONL) hyperreflectivity with hyporeflectivity in the interdigitation zone (IZ); thickened RPE with back shadowing and increased choroidal thickness in the region of the active lesions [Figure 1]e and [Figure 1]f. Complete hemogram, peripheral smear, contrast-enhanced computed tomography (CECT) of the chest, Mantoux test, venereal disease research laboratory (VDRL), and treponema plasma agglutination assay (TPHA) were normal. The patient was advised oral steroids (1 mg/kg of body weight) and close follow-up. The patient, however, returned after 2 weeks with confluent new lesions in BE [Figure 2]a and [Figure 2]b and macular involvement in the LE [Figure 2]c and [Figure 2]d. Vision RE was counting fingers close to face (CFCF) and 6/12 in LE. On OCT, active lesions revealed RPE thickening, ONL hyperreflectivity, and IZ hyporeflectivity, whereas healed areas showed outer retinal, RPE, and choroidal thinning [Figure 2]e and [Figure 2]f. The dose of oral steroids was increased to 1.5 mg/kg of body weight. The patient could return only after 6 weeks owing to the ongoing COVID pandemic. Another crop of new lesions was noted in BE. RE VA was CFCF and VA LE was now 3/60. Healed regions in both eyes showed significant atrophy that was confirmed on AF and OCT [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d, [Figure 3]e, [Figure 3]f. Fluorescein angiography (FA) in both eyes showed diffuse capillary leakage in areas of active lesions. Indocyanine green angiography (ICGA) showed a geographic area of hypofluorescence with no distinction between active and healed areas. [Figure 4].
Figure 1: (a) Healed areas of chorioretinitis in RE superior fundus (rectangle) with active borders (white arrows) involving the macula. (b) Hyper-AF edges of the chorioretinitis (white arrows). (c and d) LE color photo and AF, respectively, of healed chorioretinitis areas inferiorly (rectangle) with active edges (white arrows) not involving the fovea. (e) OCT RE hyporeflective IZ (pink arrow), hyperreflective ONL (cyan arrow), and disruption of EZ (green arrow). (f) RPE thickening (brown arrow) and ONL hyperreflectivity (cyan arrow)

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Figure 2: Follow-up images (2 weeks). (a) RE color photo showing reactivation with advancing edges (white arrows) involving inferior macula. (b) RE AF showing hyper-AF active borders (white arrows). Cockade signs are seen inferior and nasal to the disc. (c) LE color photo with white arrows denoting active edges involving the macula. (d) LE AF image hazy due to vitritis. White arrows denoting active edges. (e) OCT RE [line scan through the same area as Fig. 1e] – brown arrows- RPE thickening with back shadowing. White circle denotes outer foveal, RPE, and choroid thinning denoting healing. (f) OCT LE [line scan corresponding to Fig. 1f] with ONL hyperreflectivity, IZ hyporeflectivity (pink arrow) denoting an active lesion

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Figure 3: (a) RE fundus at 8-weeks follow-up, previously active areas now healed, active edges advancing to inferior retina. (b) hypo-AF areas denoting healing and white arrows showing advancing edge. (c) LE color photo showing active margin encroaching superior retina. (d) LE- hazy AF due to increased vitreous haze, white arrows showing advancing edge. (e) RE OCT —white circle showing thinning. Brown arrow showing RPE clumping. (f) [line-scan corresponding to Figs. 1f and 2f] white circle showing an area of thinning. Violet arrows showing outer retinal loss

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Figure 4: 102-degree wide-field simultaneous FA and ICGA-superior healed lesions showed window defects on FA. The active lesions showed hyperfluorescence in late frames of FA with diffuse capillary leakage in far nasal and far temporal regions, and a geographic pattern of hypofluorescence with advancing edges on ICGA

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A diagnosis of relentless placoid chorioretinitis was made based on (i) clinical and imaging features, (ii) negative infectious and immunological work-up, and (iii) suboptimal response to steroid treatment.[3] Immunosuppressive therapy was advised in conjunction with the rheumatologist.

  Discussion Top

Our case illustrates sequential imaging in RPC with clinical, AF, and OCT findings in acute and healed lesions with a set of complementary FA and ICGA images. The sequential imaging adds to the limited literature available on the clinical course of RPC.

Gendy et al.[4] described the OCT features of persistent placoid maculopathy (PPM). In its acute stage, placoid lesions were described as having hyperreflective outer nuclear layers with disruption of External limiting membrane (ELM) and Ellipsoid zone (EZ) and associated hyporeflectivity of cone outer segments. At follow-up, some areas of restoration of the outer retina were noted in their series. Amer et al.[5] described OCT features of RPC in acute stages with subretinal fluid, PED, and hyperreflective outer retinal layers. However, at a 15-months follow-up, they describe the complete restoration of foveal contour and retinal thickness without any atrophy. In our case, however, the outer retina, RPE, and choroid were severely thinned in the healed areas. It was also noteworthy that choroidal thickness in the acute lesions increased and decreased in the healing stages in our case.

Veronese et al.[6] described serial AF findings in RPC comparing it with a cockade. Initial acute lesion has central hypo-AF surrounded by a sliver of hyper-AF and another outer ring of hypo-AF. The intermediary hyper-AF ring increases in the subacute phase and there is a centrifugal retraction of all three rings in the healed lesions. In the case illustrated here, although there were a few cockade lesions in the initial and second follow-up, for example, in [Figure 2]b; the fulminant course of the disease precluded the longitudinal study of individual cockade lesions.

  Conclusion Top

We present sequential imaging of acute-phase RPC that showed rapid progression and poor response to steroids. Early immunosuppression may be considered in such cases with acute progression. Multimodal imaging helps differentiate RPC from other placoid maculopathies such as serpiginous choroiditis, acute posterior multifocal placoid pigment epitheliopathy, and PPM.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Jyotirmay B, Jafferji SS, Sudharshan S, Kalpana B. Clinical profile, treatment, and visual outcome of ampiginous choroiditis. Ocul Immunol Inflamm 2010;18:46-51.  Back to cited text no. 1
Dolz-Marco R, Rodríguez-Ratón Á, Hernández-Martínez P, Pascual-Camps I, Andreu-Fenoll M, Gallego-Pinazo R. Macular retinal and choroidal thickness in unilateral relentless placoid chorioretinitis analyzed by swept-source optical coherence tomography. J Ophthalmic Inflamm Infect 2014;4:24.  Back to cited text no. 2
Uraki T, Namba K, Mizuuchi K, Iwata D, Ohno S, Kitaichi N, et al. Cyclosporine and prednisolone combination therapy as a potential therapeutic strategy for relentless placoid chorioretinitis. Am J Ophthalmol Case Rep 2019;14:87-91.  Back to cited text no. 3
Gendy MG, Fawzi AA, Wendel RT, Pieramici DJ, Miller JA, Jampol LM. Multimodal imaging in persistent placoid maculopathy. JAMA Ophthalmol 2014;132:38-49.  Back to cited text no. 4
Amer R, Florescu T. Optical coherence tomography in relentless placoid chorioretinitis. Clin Exp Ophthalmol 2008;36:388-90.  Back to cited text no. 5
Veronese C, Marcheggiani EB, Tassi F, Pichi F, Morara M, Ciardella AP. Early autofluorescence findings of relentless placoid chorioretinitis. Retina 2014;34:625-7.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
1 Commentary: Multimodal imaging of relentless placoid chorioretinitis
Prabu Baskaran,Eliza Anthony
Indian Journal of Ophthalmology - Case Reports. 2021; 1(4): 678
[Pubmed] | [DOI]


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