Aim: To describe the multimodal imaging findings of pigmented paravenous retinochoroidal atrophy.
Methods: A 23-year-old female presented to us for a routine ocular examination. She had a best-corrected visual acuity of 6/6 in both eyes. Anterior segment examination was unremarkable. Fundus examination showed pigmentary changes along the retinal vasculature extending from mid periphery to post-equatorial retina suggesting a diagnosis of pigmented paravenous retinochoroidal atrophy. Swept-source optical coherence tomography of the macula showed choriocapillaris thinning at the mid periphery whereas coherence tomography angiography at the mid periphery showed a relatively normal choriocapillaris vasculature in the early stage of the disease.
Conclusion: A relatively normal choriocapillaris structure was seen on ocular coherence tomography angiography which could have been due to a milder form of the disease in a young patient.
Keywords: PPCRA; PPRCA; Pigmented paravenous retinochoroidal atrophy; ocular coherence tomography angiography; pigmented paravenous chorioretinal atrophy.