|Year : 2021 | Volume
| Issue : 4 | Page : 678-679
Commentary: Multimodal imaging of relentless placoid chorioretinitis
Prabu Baskaran, Eliza Anthony
Aravind Eye Hospital, Chennai, Tamil Nadu, India
|Date of Web Publication||09-Oct-2021|
Dr. Prabu Baskaran
Aravind Eye Hospital, Noombal, Poonamallee High Road, Chennai, Tamil Nadu - 600 077
Source of Support: None, Conflict of Interest: None
|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
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. 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. 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. RPC is usually bilateral and in the initial stages involves the mid periphery and peripheral retina with macular involvement in later stages. 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.,
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., Similar pattern of inner and outer nuclear layer hyperreflectivity has also been demonstrated in other chorioretinopathies such as APMPPE and serpiginous choroiditis., Interestingly, another case series reported no significant thickening of retina and choroid at the level of active lesions at the macula in RPC. Studies have also reported complete gain of foveal and retinal morphology with resolution of RPE hyperplasia on OCT after recovery in RPC., 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.
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., 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. 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. Recent reports also demonstrate the efficacy of adalimumab in controlling recurrences in RPC after the discontinuation of immunosuppression. 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.
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.
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