: 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.

Pigmented paravenous chorioretinal atrophy: Updated scenario / Antropoli, Alessio; Arrigo, Alessandro; Pili, Lorenzo; Bianco, Lorenzo; Berni, Alessandro; Saladino, Andrea; Bandello, Francesco; Battaglia Parodi, Maurizio. - In: EUROPEAN JOURNAL OF OPHTHALMOLOGY. - ISSN 1120-6721. - 34:4(2024), pp. 941-951. [10.1177/11206721231199118]

Pigmented paravenous chorioretinal atrophy: Updated scenario

Antropoli, Alessio
Primo
;
Arrigo, Alessandro
Secondo
;
Pili, Lorenzo;Bianco, Lorenzo;Berni, Alessandro;Saladino, Andrea;Bandello, Francesco
Penultimo
;
Battaglia Parodi, Maurizio
Ultimo
2024-01-01

Abstract

: 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.
2024
Pigmented paravenous chorioretinal atrophy
inherited retinal disease
multimodal imaging
retina
ultra-widefield
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11768/150717
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