Pigmented paravenous retinochoroidal atrophy

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Pigmented paravenous retinochoroidal atrophy (PPRCA) is a non-progressive or slowly progressive bilateral disease.[1] This condition is usually symmetrical and is characterized by pigmentation along the retinal veins, choriocapillary atrophy, and retinal pigment epithelium (RPE) degeneration. It is usually associated with a good visual prognosis.[2] However, the exact cause of this condition is unknown. Case A 39-year-old male complained of a gradual, painless diminution of vision for the past year. He was born out of a third-degree consanguineous marriage with no similar complaints from family members. His best corrected visual acuity was 6/7.5 and 6/6 in the right and left eyes, respectively, with an intra-ocular pressure of 12 mmHg in both eyes. Color vision was normal, and the anterior segment was unremarkable. Posterior segment examination showed both eyes had normal optic discs with peripapillary atrophy. There were multiple radial chorioretinal atrophic patches and pigment clump aggregates progressing centrifugally along the retinal veins [Figs. 1 and 2]. The electroretinogram (ERG) showed low photopic and scotopic responses [Figs. 3 and 4]. Based on the clinical findings and ancillary investigations, which were suggestive of PPRCA, the patient was advised to have a regular ophthalmological evaluation.Figure 1: Montage color fundus image of the right eye depicting multiple radial chorioretinal atrophic patches and pigment clump aggregates progressing centrifugally along the retinal veinsFigure 2: Montage color fundus image of the left eye depicting multiple radial chorioretinal atrophic patches and pigment clump aggregates progressing centrifugally along the retinal veinsFigure 3: Full field ERG showing reduced photopic responsesFigure 4: Full field ERG showing reduced scotopic responsesDiscussion Patients with PPRCA usually remain asymptomatic and are diagnosed on routine examination. The primary involvement in PPRCA is mainly the retinal pigment epithelium, further leading to the involvement of the choroidal vasculature.[3] ERGs disclose abnormal or low-normal rod and cone responses (scotopic and photopic ERG), maximal combined responses, flicker responses, and oscillatory potentials. Reduction in the B-wave amplitude is the most common finding, followed by delayed latency and amplitude reduction in the A-wave.[4] To conclude, PPRCA is a slowly progressive condition that requires no specific treatment. Statement of ethics Written assent for publication (including clinical information and the images) from the patient and consent from the parent have been obtained. All procedures carried out were in accordance with the tenets of the Declaration of Helsinki. Institute Ethics Committee approval is not required for a case report according to Indian Council of Medical Research guidelines. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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