Pigmented Paravenous Retinochoroidal Atrophy (PPRCA): a systematic review

Abstract Background Pigmented paravenous retinochoroidal atrophy (PPRCA) is an uncommon and under-researched eye condition. Although onset and development of the condition is often asymptomatic, PPRCA has very distinguishing features. These are predominantly retinochoroidal atrophy and pigment clumping along the retinal veins. There is no known cause to the condition and no conclusive treatment has been developed. This systematic review aims to provide a holistic account of symptomatology and treatments. Methods This systematic review was registered with PROSPERO (CRD42022346753). All epidemiology and observational studies published in English between the 1st of January 1980-1st of August 2022 will be included. The search criteria were developed based upon the research question using PubMed, ESBCOhost, ProQuest, Medline, TRIP database, ScienceDirect Embase, SciELO, The Cochrane Library, and PROSPERO databases. The Critical Appraisal Skills Programme Systematic Review Checklist was used to assess the risk of bias. Results The systematic review included 62 papers and a total of 150 participants (54% Female, aged 4 to 82). Only 29% of patients had their ethnicity recorded of which 16 patients were White. Most of the data extracted and analysed was qualitative. Three key themes were uncovered: Symptoms (2 subthemes), Clinical Pathways (4 sub-themes), and Symptom Measurement Tools (5 sub-themes). Conclusions PPRCA lacks standardised criteria for diagnosis and treatment, with clinical records and case study publications reporting presentations in an arbitrary manner. To improve patient care and develop a comprehensive understanding of the condition, a methodical and clear clinical pathway framework needs to be developed.