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Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 678-679

Commentary: Multimodal imaging of relentless placoid chorioretinitis

Aravind Eye Hospital, Chennai, Tamil Nadu, India

Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Prabu Baskaran
Aravind Eye Hospital, Noombal, Poonamallee High Road, Chennai, Tamil Nadu - 600 077
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2002_21

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How to cite this article:
Baskaran P, Anthony E. Commentary: Multimodal imaging of relentless placoid chorioretinitis. Indian J Ophthalmol Case Rep 2021;1:678-9

How to cite this URL:
Baskaran P, Anthony E. Commentary: Multimodal imaging of relentless placoid chorioretinitis. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 28];1:678-9. Available from: https://www.ijoreports.in/text.asp?2021/1/4/678/327651

Multimodal imaging has opened new insights into the pathogenesis of various retinal diseases and deepened our understanding of their progression, atypical behavior, and response to treatment. Ayachit et al.[1] have well demonstrated the characteristic findings in relentless placoid chorioretinitis (RPC) and its progression through sequential multimodal imaging. The case also demonstrates various nuances in multimodal imaging features of this rare entity. Multimodal imaging is an important tool in understanding various features of this relentless pathology. We would like to briefly discuss here the literature to further highlight various multimodal imaging features of this rare entity and their subtle variations.

Jones et al.[2] first described relentless placoid chorioretinitis as a rare clinical entity with clinical resemblance to both acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and serpiginous choroidopathy. APMPPE is a bilateral pathology characterized by creamy white placoid lesions at the level of retinal pigment epithelium (RPE) and has a self-limiting course. Serpiginous choroiditis usually causes active lesions in one eye at a time and lesions initially involve the macula or the peripapillary area. Unlike APMPPE, serpiginous choroiditis heals with significant scarring and atrophy and can lead to profound vision loss especially if macular is involved. RPC usually presents with numerous (>50) creamy white multifocal lesions that coalesce together and are scattered all over the retina from the posterior pole to periphery in different stages of activity with a prolonged course of disease, ranging from months to years. These lesions generally have good visual prognosis if treated appropriately.[2] RPC is usually bilateral and in the initial stages involves the mid periphery and peripheral retina with macular involvement in later stages.[3] Presence of healed choroiditis lesions with scarring in midperiphery and the characteristic adjacent placoid active lesions, progressively expanding and encroaching the posterior pole in this case definitely resembles RPC.[1],[4]

Optical coherence tomography (OCT) at the level of active lesion in RPC is characterized by subretinal fluid, pigment epithelium detachment, transretinal hyperreflectivity, hyperplasia, and hyperreflectivity of the ellipsoid zone with RPE hyperplasia.[5],[6] Similar pattern of inner and outer nuclear layer hyperreflectivity has also been demonstrated in other chorioretinopathies such as APMPPE and serpiginous choroiditis.[7],[8] Interestingly, another case series reported no significant thickening of retina and choroid at the level of active lesions at the macula in RPC.[9] Studies have also reported complete gain of foveal and retinal morphology with resolution of RPE hyperplasia on OCT after recovery in RPC.[5],[6] However, this case reveals thickening of the choroid at the level of active lesions of RPC with subsequently rarefied outer retina, RPE, and choroid following healing, demonstrating the nuances in multimodal imaging in RPC.[1]

This case also shows the characteristic cockade autofluorescence pattern of active RPC lesions, described as central hypofluorescence, with an outer ring of hyperautofluorescence surrounded by another zone of faint hypoautofluorescence.[1],[10] Angiographically, RPC resembles APMPPE and serpiginous choroiditis with the pattern of early hypo fluorescence and late hyper fluorescence on fluoresceine angiogram and early and late hypocyanascence on indocyanine angiography.[2] In view of various overlapping and similar clinical and multimodal imaging features of these chorioretinopathies, it is very important to correlate precisely the ocular manifestations with imaging for prompt diagnosis of RPC.

Considering the relentless nature of RPC, systemic corticosteroids are insufficient and early immunosuppression is very crucial to control the inflammation and recurrences. Cyclosporin and mycophenolate mofetil both have shown good efficacy in controlling the recurrences in RPC, in combination with systemic corticosteroids.[11] Recent reports also demonstrate the efficacy of adalimumab in controlling recurrences in RPC after the discontinuation of immunosuppression.[5] However, in this case, late follow-up of the patient due to the COVID-19 pandemic restriction and delay in initiation of immunosuppression could be the reason for relentless progression of inflammation and poor visual recovery.[1]

Therefore, early diagnosis and initiation of immunosuppression are very important for good visual outcome in RPC. Identifying the characteristics of clinical presentations along with multimodal imaging are very valuable in assessing the protean morphological features of various mimicking retinal pathologies and establishing correct diagnosis and management. However, further studies are needed to unveil the pathogenesis of this rare relentless entity and establish the role of biologicals in its management.

  References Top

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.  Back to cited text no. 1
  [Full text]  
Jones BE, Jampol LM, Yannuzzi LA, Tittl M, Johnson MW, Han DP, et al. Relentless placoid chorioretinitis: A new entity or an unusual variant of serpiginous chorioretinitis. Arch Ophthalmol 2000;118:931-8.  Back to cited text no. 2
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. 3
Cimino L, Mantovani A, Herbort CP. Primary inflammatory choriocapillaropathies. In: Uveitis and Immunological Disorders. Berlin, Heidelberg: Springer; 2005. p. 209-31.  Back to cited text no. 4
Asano S, Tanaka R, Kawashima H, Kaburaki T. Relentless placoid chorioretinitis: A case series of successful tapering of systemic immunosuppressants achieved with adalimumab. Case Rep Ophthalmol 2019;10:145-52.  Back to cited text no. 5
Amer R, Florescu T. Optical coherence tomography in relentless placoid chorioretinitis. Clin Exp Ophthalmol 2008;36:388-90.  Back to cited text no. 6
Gallagher MJ, Yilmaz T, Cervantes-Castañeda RA, Foster CS. The characteristic features of optical coherence tomography in posterior uveitis. Br J Ophthalmol 2007;91:1680-5.  Back to cited text no. 7
Souka AA, Hillenkamp J, Gora F, Gabel VP, Framme C. Correlation between optical coherence tomography and autofluorescence in acute posterior multifocal placoid pigment epitheliopathy. Graefes Arch Clin Exp Ophthalmol 2006;244:1219-23.  Back to cited text no. 8
Dolz-Marco R, Rodríguez-Ratín 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. 9
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. 10
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. 11


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