Pattern of inner retinal layers involvement in pigmented paravenous retinochoroidal atrophy as determined by SD-OCT: case report

Junqueira, Daniela L M; Lopes, Flavio S.; Biteli, Luis Gustavo; Prata, Tiago dos Santos

doi:10.1590/s0004-27492013000600014

Cited by 8 publications

(6 citation statements)

References 9 publications

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“…SD-OCT imaging in previous studies typically showed severe atrophy of the choroid and RPE, with relative sparing of the inner retinal layers, 9 although in one case the inner retinal layers appeared mildly thinned. 10 These findings are consistent with our patient with the exception of her thickened, cystic retinal nerve fiber layer (RNFL). To our knowledge, there are no other previous reports of thickening of the inner retinal layers, which would support a retinal vessel-based etiology for PPRCA rather than purely an RPE-based cause.…”

Section: Discussionsupporting

confidence: 90%

A rare case of unifocal, unilateral pigmented paravenous retinochoroidal atrophy (PPRCA)

Ahmad

Leisy

Carr

et al. 2016

American Journal of Ophthalmology Case Reports

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PurposeTo report an atypical case of unifocal, unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) characterized by thickening and cystic degeneration of the retinal nerve fiber layer (RNFL).ObservationsA 79-year old Asian woman presented with a large area of atrophic, pigmented change along the inferior arcade of her right eye. She denied nyctalopia and any other visual complaints. Visual acuity was 20/40 in both eyes and visual fields were significant for a large absolute peripheral scotoma superiorly in the affected eye corresponding to the atrophic area. Spectral domain optical coherence tomography through the lesion showed loss of choroid except for largest Haller's layer vessels, significant retinal pigment epithelium atrophy with migration and pigment clumping, outer retinal layer loss and RNFL thickening with cystic degeneration. Fundus autofluorescence imaging showed a large area of hypoautofluorescence corresponding to the area of atrophy. Full field electroretinogram demonstrated normal scotopic response and reduced photopic response in the right eye.Conclusions and importancePPRCA is typically bilateral and symmetric, affecting primarily the outer retina and choroid. However, in rare cases, this disease can present unilaterally and/or unifocally, with degeneration extending to the inner retinal layers.

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Section: Discussionsupporting

confidence: 90%

A rare case of unifocal, unilateral pigmented paravenous retinochoroidal atrophy (PPRCA)

Ahmad

Leisy

Carr

et al. 2016

American Journal of Ophthalmology Case Reports

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“…SD-OCT imaging over the PPCRA lesions have shown thinning of the choroidal, RPE, outer retinal 8 and inner retinal layers. 31 The RPE may be the primary layer affected given the aforementioned hypo-autofluorescence. However, Bartesilli demonstrated choroidal thinning in a 25 years old Japanese with early PPCRA, in areas where RPE was unaffected associated with visible overlying thinning of the entire outer retina 32 suggesting that in the early/mild stages of PPCRA choroidal thinning precedes development of definite RPE atrophy.…”

Section: Discussionmentioning

confidence: 99%

Pigmented Paravenous Chorioretinal Atrophy

Shona

Islam

Robson

et al. 2019

Retina

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Overall, most patients with pigmented paravenous chorioretinal atrophy maintained stable vision. The lack of other affected family members, slow or absent progression, and interocular asymmetry of the retinal features is suggestive of an acquired rather than inherited retinal disorder, generally nonprogressive disorder. We identify that patients commonly have marked interocular asymmetry both on structural and functional assessment.

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“…Classically, the most common changes in macular OCT imaging of PPRCA is attenuation of all outer retinal and choroidal layers 2. However, thinning of the inner layers along the region of paravenous retinochoroidal atrophy has also been described,6 which showed good correlation with functional loss.…”

Section: Discussionmentioning

confidence: 99%

Pigmented paravenous retinochoroidal atrophy associated with unilateral cystoid macular oedema

Figueiredo¹,

Morais-Sarmento

Garrido

et al. 2019

BMJ Case Rep

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A 65-year-old man was referred to our department with complaints of blurred vision in the left eye. Funduscopic examination revealed areas of retinochoroidal atrophy along the retinal veins bilaterally and bone spicule pigmentation along the nasal and superior temporal venous branches, as well as macular oedema in the left eye. Fluorescein angiography, visual field test, optical coherence tomography and electrophysiological examination were performed, and results were compatible with the diagnosis of pigmented paravenous retinochoroidal atrophy (PPRCA). Treatment with topical dorzolamide and intravitreal bevacizumab in the left eye resulted in poor anatomical and visual response. There is scarce documentation of macular involvement with non-inflammatory unilateral cystoid macular oedema in PPRCA in the literature. Further investigation is required to elucidate the pathogenesis of PPRCA and to properly manage these patients.

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Pattern of inner retinal layers involvement in pigmented paravenous retinochoroidal atrophy as determined by SD-OCT: case report

Cited by 8 publications

References 9 publications

A rare case of unifocal, unilateral pigmented paravenous retinochoroidal atrophy (PPRCA)

A rare case of unifocal, unilateral pigmented paravenous retinochoroidal atrophy (PPRCA)

Pigmented Paravenous Chorioretinal Atrophy

Pigmented paravenous retinochoroidal atrophy associated with unilateral cystoid macular oedema

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