Ultra-wide field imaging of pigmented para-venous retino-choroidal atrophy

Objective: To describe the ultra-wide field imaging features of pigmented para-venous retino-choroidal atrophy. Design: Retrospective review at a tertiary care centre. Participants: Eight eyes of five patients with pigmented para-venous retino-choroidal atrophy who presented to our retina clinic over last 2 years. Methods: Retrospective review of ultra-wide field pseudo-colour and short wave autofluorescence imaging was performed. In vivo histology of the macula and areas of retino-choroidal atrophy was studied with swept source optical coherence tomography (SS-OCT). Results: The median age was 40 years (range: 22–67 years). Best corrected visual acuity ranged from perception of light to 20/20. The para-venous retino-choroidal atrophy and pigment clumping not only involved the major arcade vessels but also extended into the peripapillary area and retinal periphery. The affected areas demonstrated hypoautofluorescence with sharp hyperautofluorescent borders. Macular atrophy, epiretinal membrane and optic disc pallor were noted in two eyes each. In all cases, the affected pigmentary area had disorganization of inner retinal layers, disruption of outer retinal layers and retinal pigment epithelium and markedly thinned out choroid on swept source optical coherence tomography. Concurrent involvement with retinitis pigmentosa in the fellow eye was noted in two patients. Conclusion: Ultra-wide field imaging of pigmented para-venous retino-choroidal atrophy sheds light onto the widespread retino-choroidal abnormalities. Concurrent disc and macular involvement may jeopardize the visual function. Pigmented para-venous retino-choroidal atrophy may be considered as a self-limited form of retinitis pigmentosa.