A rare presentation of pigmented paravenous retinochoroidal atrophy

Ahmed, Arshee S; Rishi, Pukhraj

doi:10.4103/0974-620x.149867

Cited by 7 publications

(5 citation statements)

References 5 publications

(11 reference statements)

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“…In contrast, Ahmed et al, showed a thickened cystic retinal nerve fibre layer on SD-OCT in a 79 years old woman with uniocular PPCRA which they believed supported a vascular aetiology rather than an RPE based cause. 33 We found no such changes in our series.…”

Section: Discussioncontrasting

confidence: 50%

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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Section: Discussioncontrasting

confidence: 50%

Pigmented Paravenous Chorioretinal Atrophy

Shona

Islam

Robson

et al. 2019

Retina

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“…Pigmented paravenous retinochoroidal atrophy (PPRCA) is an unusual retinal degeneration characterized by perivenous aggregations of pigment clumps associated with peripapillary and radial zones of retinochoroidal atrophy that are distributed along the retinal veins [ 1 ]. Patients are often asymptomatic and the diagnosis is based on a characteristic fundus appearance [ 2 ]. The etiology remains unknown or idiopathic, although many report of familial cases make hereditary nature of this condition quite apparent [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Pigmented paravenous retinochoroidal atrophy: a case report

Shen

Cao

2018

BMC Ophthalmol

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BackgroundPigmented paravenous retinochoroidal atrophy (PPRCA) is an unusual retinal degeneration, and its performance on optical coherence tomography angiography (OCTA) is unclear. We report a Chinese female case of PPRCA and her OCTA features.Case presentationA 66-year-old female patient was referred to the author’s center for gradual progressive loss of vision in both eyes and photophobia of 2 years duration. She reported having no family history of inherited ocular diseases. The funduscopic examination revealed bone-spicule pigmentation and retinochoroidal atrophy along the retinal veins. This patient was diagnosed with PPRCA which is a rare disease, uncommon in females, more commonly affecting the paravascular fundus. Noninvasive imaging techniques features of this patient was described, including ultra-wide field fundus autofluorescence, spectral domain optical coherence tomography (SD-OCT), OCTA (SSADA), etc. The en face OCTA images demonstrated areas of flow void beneath the retinal pigment epithelium-Bruch membrane layer suggestive of choriocapillaris hypoperfusion that corresponded with indocyanine green angiography (ICGA). Further studies should be conducted to clarify the relationship between choriocapillaris hypoperfusion and the development of PPRCA.ConclusionsThe OCTA features in patients with PPRCA has not been described previously in the literature. This case might provide preliminary information regarding the pathophysiology of PPRCA and improve our understanding of the nature of this disease.

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“…3 The involvement of the macula is an important factor that may aggravate the disease, leading to severe visual impairment. 1,28 In a prior study, chorioretinal atrophy affecting the fovea was found to be present in only a small proportion of patients. 1,29 On optical coherence tomography (OCT) images, macular involvement is usually seen as extensive damage of the outer retina and RPE.…”

Section: Associated Ocular Findingsmentioning

confidence: 95%

“…1 With this technique, other macular comorbidities have been reported, such as epiretinal membrane, cystoid macular edema, lamellar and full-thickness macular hole, RPE atrophy and macular coloboma. 5,28,30,36,57 OCT of the affected paravenous regions show variable degree of outer retinal layers thinning, focal or generalized choroidal thinning and RPE atrophic changes, together with areas of pigment clumping visible as intraretinal hyperreflective plaques with posterior hypotransmission. 1,8 In particular, areas characterized by a reduction of the outer retinal thickness without RPE atrophy correspond to the junctional zones in FAF.…”

Section: Optical Coherence Tomographymentioning

confidence: 99%

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Pigmented paravenous chorioretinal atrophy: Updated scenario

Antropoli,

Arrigo,

Pili

et al. 2023

European Journal of Ophthalmology

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Pigmented paravenous chorioretinal atrophy (PPCRA) is an uncommon form of chorioretinal atrophy characterized by perivenous aggregations of pigment clumps associated with peripapillary and radial zones of retinal pigment epithelial atrophy that are distributed along the retinal veins. Most patients are asymptomatic, and evidence suggest that PPCRA is slowly progressing. Unless macular involvement is present, the majority of patients usually retain a normal visual function. Our ability to diagnose PPCRA has recently improved thanks to multimodal imaging, especially with the advent of ultra-widefield (UWF) imaging. Blood tests and functional and genetic testing can help with the correct differential diagnosis of pseudo-PPCRA or other disorders with similar characteristics. Although the cause of PPCRA is unknown, it is possible that it has a genetic basis. In this review we provide a summary of the multimodal imaging characteristics of PPCRA, and discuss its possible pathogenesis, based on the genes that have been associated with this disease.

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A rare presentation of pigmented paravenous retinochoroidal atrophy

Cited by 7 publications

References 5 publications

Pigmented Paravenous Chorioretinal Atrophy

Pigmented Paravenous Chorioretinal Atrophy

Pigmented paravenous retinochoroidal atrophy: a case report

Pigmented paravenous chorioretinal atrophy: Updated scenario

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