Pigmented Paravenous Retinochoroidal Atrophy: A Case Report Supported by Multimodal Imaging Studies

Abstract

Background and Objectives: Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare disease with bilateral retinal pigment epithelium and choroidal atrophy. We present a case of PPRCA using multimodal imaging studies. Case summary: A 61-year-old female was referred to our department for floaters. Funduscopic examination revealed pigment clumps and grayish lesions along the retinal vein and the peripheral area, bilaterally. She did not have nyctalopia or any other visual symptoms including visual loss. She was diagnosed with pigmented paravenous retinochoroidal atrophy based on the typical findings of fundus. The findings of wide fluorescein angiography (FA), wide indocyanine green angiography (ICGA), fundus autofluorescence (FAF), spectral domain-optical coherence tomography (SD-OCT), optical coherence tomography angiography (OCTA), the visual field (VF) and an electroretinogram (ERG) could help us to confirm the diagnosis. The patient did not have any specific treatment for PPRCA in our study and there was no change in visual acuity and multimodal imaging of both eyes over one year. Conclusions: We report a case of PPRCA and the multimodal imaging of this patient. PPRCA is very rare disease and sometimes it is easy to get confused with other diseases such as retinitis pigmentosa and vasculitis when it comes to diagnosis. Multimodal imaging features of PPRCA will improve our understanding, diagnosis and prediction of the prognosis of this disease.

Posterior vitreous cortex hyalocytes visualization in asymmetric pigmented paravenous chorioretinal atrophy (PPCRA) using en face OCT

“… 12 Optical coherence tomography angiography (OCT-A) has been recently utilized to investigate pathological alterations in vessel density and choriocapillaris porosity in patients with PPCRA. 12 , 13 , 14 , 15 , 16 These reports provide evidence of choriocapillaris hypoperfusion and reduced vessel density in the deep capillary plexus of the retina in this condition. 13 , 16 …”

“… 29 , 30 In 2021, Jung et al published a case report detailing wide fluorescein angiography (FA), wide indocyanine green angiography (ICGA), optical coherence tomography angiography (OCT-A), and electroretinogram (ERG) findings in order to help clinicians distinguish PPCRA from other pigmentary retinopathies, such as retinitis pigmentosa, hydroxychloroquine retinopathy, or serpiginous choroidopathy. 15 More recent studies using OCT-A in patients with PPCRA have demonstrated alterations in vessel perfusion and density. Lee et al describe the OCT-A imaging characteristics of eyes affected by PPCRA.…”

Étude de cas : présentation inhabituelle d’une atrophie rétinochoroïdienne

“…L'atrophie rétinochoroïdienne paraveineuse pigmentée (ARPP) est un trouble rare de la rétine et de la choroïde. Bien que l'étiologie soit inconnue à ce jour, on a émis l'hypothèse de pathologies inflammatoires et héréditaires [1][2][3] . L'affection se caractérise par une atrophie de l'épithélium pigmentaire rétinien et une dégénérescence des photorécepteurs, une atrophie de la choriocapillaire et une accumulation de pigments autour des veines rétiniennes rayonnant à partir du nerf optique.…”

“…L'autofluorescence du fond d'oeil est considérée comme la meilleure modalité d'imagerie, car elle permet de capturer l'étendue de l'atrophie rétinochoroïdienne associée aux lésions de l'ARPP. Cette atrophie se manifeste par des lignes de séparation entre les zones actuellement touchées (hyperautofluorescence) et les zones précédemment touchées/ endommagées (hypoautofluorescence) 2,3,13 . Les zones spécifiques d'hypoautofluorescence doivent correspondre aux zones de lésions de la rétine externe mises en évidence par l'angiographie à la fluorescéine et la tomographie par cohérence optique 2 .…”

Presumed Autoimmune Pigmented Paravenous Retinochoroidal Atrophy in a Female Patient Aged 69 Years

“…Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare disease of unknown etiology, although dysgenetic, degenerative, hereditary, and inflammatory causes have been suggested. It is usually slowly progressive, and visual acuity remains good unless the macula is involved . Given the personal history of autoimmune disease and a negative genetic test result, a diagnosis of presumed autoimmune PPRCA was suggested.…”