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.
Keywords: bilateral; multimodal imaging; pigmented paravenous retinochoroidal atrophy.