Pigmented Paravenous Retinochoroidal Atrophy (PPRCA): a systematic review

doi:10.21203/rs.3.rs-3334235/v1

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Pigmented Paravenous Retinochoroidal Atrophy (PPRCA): a systematic review

https://doi.org/10.21203/rs.3.rs-3334235/v1

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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.

Health sciences/Diseases/Eye diseases

Health sciences/Health care/Medical imaging/Tomography

Health sciences/Risk factors

Pigmented Paravenous Retinochoroidal Atrophy

PPRCA

Retinochoroidal atrophy

pseudoretinitis pigmentosa

pigmentation of the retina

Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare eye disorder, affecting the retina and choroid. PPRCA is characterised by retinochoroidal atrophy and clumps of pigmentation along the retinal veins which supply blood to the tissues of the eye [1–3]. The first case of PPRCA was of a 47-year-old man recorded in 1937 who presented with abnormal bilateral fundus and pigmentation along the retinal veins [4–5]. PPRCA is commonly bilateral and symmetrical however varied presentations can arise in the condition [1–2]. The disease is often asymptomatic with no or minimal progression, hence remains widely undetected [1–2]. Diagnosis is often made after a routine ophthalmic appointment [1–2, 5]. The cause of PPRCA remains unknown with a mixture of conclusions related to the hereditary, degenerative, or inflammatory cause of the condition [1–3]. There is, at present, no cure or standardised treatment plan for PPRCA, with any treatment aiming to manage symptoms and reduce progression. Various exploratory methods can be used to identify and diagnose PPRCA. Some examples of these are optical coherence tomography (OCT), fundus autofluorescence (FAF), and electroretinogram (ERG) [3]. A variety of terminology has been attributed to PPRCA such as pseudoretinitis pigmentosa, chorioretinitis striata, or congenital pigmentation of the retina as a few examples [1].

Since the first case of PPRCA in 1937, there have only been very limited case studies and case series published of patients with the condition [4]. PPRCA is very under-researched, with no conclusive understanding on the etiology, symptomatology, and treatment of the condition. This systematic review aims to synthesis all published literature on PPRCA patients to provide a comprehensive report on the various symptom presentations of the condition, including any casual factors and treatment. This will also identify gaps within the research around this condition.

A systematic methodology was used to develop a comprehensive understanding of PPRCA, including its prevalence, symptomology, and treatment pathways. A systematic protocol was designed, peer reviewed and published on PROSPERO (CRD42022346753).

The search strategy comprised of searching through nine databases: PubMed, ESBCOhost, ProQuest, Medline, TRIP database, ScienceDirect Embase, SciELO, The Cochrane Library, and PROSPERO. The following MeSH terms and keywords were used: ("Pigmented Paravenous Retinochoroidal Atrophy" OR "PPRCA" OR "retinochoroiditis radiata" OR "pseudoretinitis pigmentosa" OR "chorioretinitis striata" OR "congenital pigmentation of the retina" OR "melanosis of the retina" OR "paravenous retinal degeneration" OR "pigmented paravenous chorioretinal degeneration") AND (case study OR review OR "systematic review" OR "meta-analysis" OR commentary OR opinion).

Study Eligibility

All study types were included, such as case studies and randomised controlled trials. In addition, we included letters to the editors, commentaries and perspectives that reported clinical information. All peer-reviewed articles published in English, between the 1st of January 1980 to the 1st of August 2022, were included in the study. Animal studies were excluded.

Data Extraction

Two reviewers did the data extraction process independently, and the results were then reviewed for accuracy and completeness by a senior researcher to ensure consistency. Any disagreements were discussed and resolved by consensus between the authors. All extracted quantitative data, such as sample size, study design, intervention types, outcomes, and measures, were then tabulated into a standardized format in a CSV file to enable consistent and objective data organization.

For qualitative data, we used a reflexive thematic analysis approach to extract classifications and strengths of evidence about the reviewed data quality and findings. Classifications were usually focused on the subject matter of the article, while the strengths focused on how well the article answered the research question and the extent to which it addressed important information gaps.

Data Analysis

Quantitative data

Descriptive statistics for study and patient characteristics will be reported (N, mean, SD, Median)

The systematically included studies had insufficient statistical data for a meta-analysis hence a narrative synthesis has been provided. This thematic analysis reports the symptomatology and presentations of PPRCA alongside any treatments or follow-ups which may have occurred. Data was extracted and synthesised in line with Thomas & Harden’s (2008) method for analysing qualitative research [6]. Thematic analysis provides further insight into perspectives and opinions, however, results in synthesising through a huge amount of data. The method of Thomas & Harden (2008) builds on Braun & Clarke’s (2006) methods further [6–7]. Thomas & Harden’s method has been used for numerous systematic reviews relating to healthcare services and health issues. Whilst Braun & Clarke’s method is descriptive, it can appear to lack guidance and clarity in the stages needed to complete a thematic analysis [7]. The three stages of this methodology are laid out to enable for the identification and development of sufficient themes. Stages one and two occur in conjunction with one another and relate to exploring the text to develop relevant themes. This is a dynamic process, evolving as the texts is further analysed. Codes and themes are routinely examined to ensure consistent with interpretations, allowing for adjustments if needed. In step three, inductive analysis occurs, whereby meanings are attributed to the codes generated to explore and answer the research question.

Risk of Bias (RoB) Assessment

An RoB assessment was completed to assess quality of the studies. Following the inclusion process, all final papers were either case studies or case series hence the Critical Appraisal Skills Programme (CASP) Qualitative Checklist was used to assess [8]. This is a 10-item tool with three quality parameters: ‘Are the results of the study valid?’, ‘What are the results?’, and ‘Will the results help locally?’. Each question is responded to by Yes (🗸), No (X), or Can’t tell (?). A scoring system is not recommended. The CASP checklists are regularly reviewed for relevancy and applicability and adjusted when required.

Prior to selection of the CASP, we considered the use of more popular RoB assessment tools (e.g., Newcastle Ottawa Scale, Cochrane, ROBIS) but these were inadequate for this type of systematic review. Whilst the ROBIS and AMSTAR-2 are common RoB tools used for systematic reviews, they are more suitable for reviews with predominantly quantitative analysis. As the studies used for this systematic review generated mainly thematic results, it was deemed fit for the CASP Qualitative scale to be utilised. Other alternatives where the JBI Checklist of Qualitative Research and the GRADE-CERQual scale. Research has supported the use of the CASP scale in conjunction with reviews utilising Thomas & Harden’s methodology, further justifying out use for this [6, 8]. The outcomes for this can be seen in Table 1.

The initial search identified 724 papers. After screening titles and abstracts for relevancy to PPRCA and its related characteristics, a final set of 62 papers were systematically included and thematically analysed. The PRIMA flow diagram outlines this process (Fig. 1).

Study Characteristics

Overall, 37 papers were case studies and 15 were case series. The geographic origin of the studies was split across by 33% in Asia and 67% across the West (Europe and America). The main study characteristics have been reported in Table 2.

Demographics

The participant sample size is 150. Ages of patients ranged from 4 to 82 years. Fifty-four percent of the sample being female (N = 81). The ethnicity of over 71% of patients was not disclosed. The largest reported ethnic group was White (N = 16). Table 2 reports demographic characteristics in further detail.

Best Corrected Visual Acuity:

Across all studies, the Best Corrected Visual Acuity (BCVA) scores were reported for 268 eyes. The breakdown of BVCA values has been provided in Table 3. Most eyes (29.1%) reported normal 20/20 vision. There was a huge variety of reported BCVA scores, with 33 different scoring categories noted (Table 3).

Intraocular Pressure:

The intraocular pressure for 28 patients was reported across all studies. Of these, 5 studies did not provide exact figures for the pressure readings, simply labelled as ‘Normal’. Twenty-three cases reported the quantitative statistics for intraocular pressure (See Fig. 2).

Thematic analysis

As the studies included within these studies were reports of symptoms presentations for patients with PPRCA, most of the data has been qualitatively reported. The themes, and corresponding subthemes, have been stated in order of commonality and prevalence. These three themes are: Symptoms, Clinical Pathways, and Symptom Measurement Tools (See Table 4).

Symptoms

This theme was the predominant feature across all patient reports. It explores the various symptom presentations of PPRCA patients, highlighting common manifestations of the conditions as well as more singular occurrences. Within the theme of Symptoms, four prominent subthemes relating to highly prevalent symptom groups emerged: Retinochoroidal atrophy, Changes in fluorescence, Degeneration of retinal pigment epithelium, and Disruptions to retinal functioning.

1. Retinochoroidal Atrophy. Retinochoroidal atrophy is a defining feature of PPRCA. It is when the retina begins to degenerate followed by an accumulation of pigment in the retina [2, 9–45]. Retinochoroidal atrophy was reported in around 45% of studies (N = 27). Some reported varying degrees of severity, include “dense” and “severe” atrophy” [31, 37]. It was also spread across different locations in the eyes, with some patients reporting atrophy “observed throughout” and others with more constricted presentations, such as “in the peripheral paravenous distribution” [26, 37]. More specific reports of pigment clumping corresponding to retinochoroidal atrophy were made in 35% of studies (N = 22), with “pigment clumps” or “lesions”, along various parts of the eye, including “retinal veins” or “retinal vascular arcade” [2, 9–12]. Another reported characteristic of retinochoroidal atrophy was “shadowing corresponding to pigment clumps” [2, 33, 46]. Bony spicule is when pigment clumps migrate to the interstitial spaces of the retina after breaking away from pigmentation along the retinal vessel. This is another less common feature of retinochoroidal atrophy reported in only 15% of studies (N = 9). It was described that “bone spicule pigmentation” occurred due to the “choroidal atrophic changes” within the retina [9, 26–27, 33], present in certain areas such as “the nasal and superior temporal venous branches” [33].

2. Fluorescence. Changes or disruptions to the levels of fluorescence, detected by a fluorescein angiogram, is another prominent symptom of PPRCA. Hyperfluorescence is the increased level of fluorescence reported in 24 studies. It is often found to be “consistent with retinal pigment epithelium degeneration”, present along the retinal veins and choroidal vessels [2, 9, 18, 33–34, 47–49]. Some studies also reported “hyperfluorescence rings”, with one case series of four patients reporting demarcation lines too [47, 51–54]. A window defect is an area of hyperfluorescence, arising when the retinal pigment epithelium (RPE) is damaged and there is an absence of pigmentation. This was present in 13 studies and is often seen “along the choriocapillary vessels” and in areas of “RPE atrophy” [2, 53, 55–56]. Hypofluorescence is the reduction of fluorescence levels reported in around 30% of studies. Similar to hyperfluorescence, hypofluorescence is also present “along the retinal veins” [11, 15, 27, 30, 58]. It is predominantly noted in “atrophic patches”, “corresponding to the areas of pigment clumping”, and detected near “bony spicule” [3, 15, 18–19, 29–30, 38, 54, 59–60–62]. Blocked fluorescence was also detected around areas of pigment clumping and retinal veins [2, 9, 42, 53].

3. Retinal Pigment Epithelium. The retinal pigment epithelium (RPE) is a monolayer of cells situated on the outermost layer of the retina and was reported in 11 studies. It functions to ensure normal vision and degeneration of this can lead to various complications including retinopathy and has been reported in 11 studies. “Significant RPE degeneration” was described, often “in both eyes” [2, 9–11, 17, 33, 36, 42, 46,]. The innermost layer of the choroid is the choriocapillaris. It is responsible for nourishing the RPE layer and the photoreceptors. In PPRCA patients, extensive “atrophy of the choriocapillaris” [2], visible “along the choroidal vessels” [24, 59] has been reported.

4. Retinal functioning. Retinal functioning is often tested through various electroretinograms (ERGs) which were reported across 18 studies. The pattern electroretinogram (PERG) tests central retinal function through responses to pattern reversing stimuli. PERG has shown normal retinal functioning in some PPRCA patients [2, 9, 55] but cases of reduced amplitudes and photoreceptor dysfunction have been reported [13, 34]. The multifocal electroretinogram (mfERG) enables the assessment of various retinal functions concurrently. For patients with PPRCA, it has been useful in identifying issues with response amplitudes as well as photoreceptor dysfunction [2, 9, 33–34, 40]. The full-field electroretinogram (ffERG) is an electrophysiologic test, assessing retinal functioning through exposure to a full-field light-stimulus. Eleven studies reported decreased scotopic and photopic waves [3, 11, 18, 20, 23, 25, 32, 46, 52, 60], whilst 7 studies reported decreased a- and b-wave amplitudes [3, 11, 18, 20, 23, 46, 52]. Damaged or dysfunctional photoreceptors of rods and cones were found in a few PPRCA patients [16, 34, 58].

Clinical Pathways

This theme explores the various elements of care received by PPRCA patients, ranging from assessments and referrals to treatment and follow-ups. This theme is further broken down into five subthemes: Duration of Symptoms, Genetic and Family History, Treatments, and Follow-ups.

1.Duration of Symptoms.

Out of all 150 patients, the duration of symptoms for 51.3% of patients were not recorded (Fig. 3). Of those whose duration was recorded, 14% (N = 21) reported symptoms for under a year, 19.3% (N = 29) reported symptoms for 1–5 years, 8.6% (N = 13) reported symptoms for 5–10 years and 7.3% (11) reported symptoms for 10 + years.

2.Genetic or Family History

Out of all 150 patients, 62% reported no familial or genetic history related to their PPRCA diagnosis and 32% patients had no information reported in relation to this. Nine patients reported a family history relevant to their PPRCA diagnosis. Three reported similar symptoms with their brother and 3 with their mother, 2 reported similarities with their sisters and 2 with their maternal female cousin.

3.Treatment. Out of all 150 patients, there were only 6 documented cases where patients were provided with treatment. These individual cases are described in Table 5. There were no significant similarities across these cases. Five of these 5 cases were in Europe with a symptom duration of under 2 years.

For the remaining 146 patients, there was no recorded treatment offered or implemented. The sheer lack of treatment details and the varying offered interventions made it obvious that there was no standardised treatment plan for patients with PPRCA.

4. Follow-up. In the first instance, all patients presented to an ophthalmology department. Around 45% of patients (N = 70) were offered a follow-up appointment. Most studies that conducted follow-up were from Western nations (i.e., USA, UK, and Europe). Ethnicity was not reported in 20 studies, with the most common ethnic groups being Korean (N = 20) and White (N = 14). In relation to genetic or familial factors, 50% reported no family history related to PPRCA. Duration of symptoms and follow-up period varied across all patients, with 66% not attending their follow-up appointments. Seventeen patients were stable, 6 had deteriorated and 1 had improved symptoms reported at follow-up. Table 6 highlights the characteristics of these follow-up studies.

Following treatment, Patient 1 returned for “a 3-year follow-up with fundus examination” and showed slight improvements in the BCVA [33]. Patient 2 returned for follow-up after 5 years, and showed “no deterioration of the condition, with a slight improvement in best corrected visual acuity” [54]. For the no-treatment patients, two patients reported “slight improvement” in BVCA [33;54].

At Patients 3’s 6-month follow-up, “vision in the left eye had reduced to ‘Counting Fingers’” [17]. For some no-treatment patients, there was a deterioration of symptoms including “progressive vision loss” and “progressive retinal thinning”, “increased blind spots” and “deteriorated night vision” [17, 24, 31, 44, 64–66, 67]

Patient 5 “exhibited no change at the 12-month follow-up” [42]. Patients 4 and 6 did not attend the follow-up [39, 64]. Out of the patients who did not receive treatment, 11 patients had no change to their symptom presentations and 21 did not attend [2, 9, 13, 19–20, 36, 41–42, 54, 56, 58].

Symptom Measurement Tools

There are a variety of standardised measurement tools which were used to assess various symptoms and presentations of PPRCA. These have been explored as subthemes: Ultra-Wide Field Fundus Photography, Fluorescein Angiography, Indocyanine Green Angiography, Optical Coherence Tomography and Spectral Domain Optical Coherence Tomography, and Electroretinograms. Overall, there is no standardised criteria for assessing and measuring PPRCA. Whilst these 5 measurement tools are the most common, they are not consistent across all patient cases (Table 7).

1. Ultra-Wide Field Fundus Photography. Over 75% of studies used Ultra-Wide Field Fundus Photography (UFPP). This method can capture the eye up to 200 degrees, providing images of around 80% of the retinal surface. It was used to identify retinochoroidal atrophy, pigment clumps, and bony spicule in various parts of the eye.

2. Fluorescein Angiography. Fluorescein Angiography (FA) records blood flow within the retina, allowing for the diagnoses of various eye conditions and was used in almost 68% of studies. It is mainly used to detect disturbances in fluorescence (i.e., hypo-, hyper-, or blocked fluorescence. FA can also be used to identify atrophy of choriocapillaris atrophy within the RPE.

3. Indocyanine Green Angiography. Indocyanine Green Angiography (IGC) was used in only 9 studies and is a less common diagnostic technique used to identify the presence of retinochoroidal diseases. In the context of this review, it highlighted window defects in the RPE as part of hyperfluorescence

4. Optical Coherence Tomography and Spectral Domain Optical Coherence Tomography. Optical Coherence Tomography (OCT) uses a low-level red light to generate a scan of the back of the eye. Spectral Domain Optical Coherence Tomography (SD-OCT) provides high-resolution, cross-sectional images and analyses of the eye. It is used to assess and quantify optic atrophy. Standard OCT was used in only 13% of studies to identify RPE atrophy, choroidal thinning and intraretinal cysts. SD-OCT was used in 29% of papers for the identification of symptoms such as retinal and choroidal thinning, shadowing and RPE atrophy but with much more detailed.

5. Electroretinograms. Electroretinograms (ERGs) measures the electrical activity within the retina in response to a stimulus. There are many variations of this diagnostic tool used to assess different elements and functions of the retina. They are useful in clarifying retinal diseases, enabling the identification of PPRCA symptoms however, these are not commonly used. In this review, four main ERGs were identified: pattern electroretinogram (PERG), multifocal electroretinogram (mfERG), photopic electroretinogram (PhERG), and full field electroretinogram (ffERG). The PERG was used in 5 studies and is used to test central retinal function through responses to pattern reversing stimuli. PERG results showed normal retinal functioning in some PPRCA patients, but some cases of reduced amplitudes and photoreceptor dysfunction were reported. mfERG was also used in 5 studies and it assesses various retinal functions concurrently. For patients with PPRCA, it has been useful in identifying issues with response amplitudes as well as photoreceptor dysfunction. Similarly, only 5 studies used PhERG and found that decreased b-waves, scotopic waves, and photopic waves in PPRCA patients. Nineteen studies used ffERG which assesses retinal functioning through exposure to a full-field light-stimulus. For patients with PPRCA, it is common for decreased scotopic and photopic waves to be found as well as decreases a- and b-wave amplitudes.

Pigmented paravenous retinochoroidal atrophy is a rare eye disorder characterised by retinochoroidal atrophy and pigment clumping along the retinal veins. The aetiology for this condition remains unknown. This systematic review synthesised current published literature on PPRCA with the aim provide a comprehensive report on symptomatology, causation, treatments, and any other related factors that arose whilst simultaneously identifying gaps within the research. There was no comprehensive review which explored PPRCA hence there was a huge gap within research which needed to be explored. Most published literature was individua case studies or case series. There was no standardised definition, diagnostic criteria, or presentation of PPRCA, but this could be due to the complexity and rarity of the condition.

It has become apparent that there is no standardised way of assessing, reporting, or treating PPRCA. Symptoms and presentations were reported in inconsistent manner. Whilst certain symptoms were often reported, there was no commonality across all studies. This highlights a huge issue in healthcare provision, possibly fuelled by the lack of knowledge and understanding of this condition. Any findings that were reported were often descriptive, with very little quantitative data available aside from BVCA and intra-ocular pressure. These finding highlight the importance of early diagnosis, treatment, and prevention strategies to mitigate the impact of PPRCA on this vulnerable population. Due to the disparities in identification and treatment, our analysis showed that personalized treatment plans are necessary for PPRCA management, as the disease progression varies between individuals. This finding highlights the essential role of patient-centred approaches to disease management in improving patient outcomes.

Our final dataset only consisted of case studies and case series; there were no systematic reviews, meta-analyses, or randomised controlled trails on PPRCA despite the availability of published case studies. Individual patient cases were reported in all studies, unique to their population and presentation which makes it difficult to generalise and compare across different cases. A variety of MeSh terms were used to identify papers for this review due to the different terminology often used to describe PPRCA. This broad range of terms further highlights the issue of ambiguity surrounding PPRCA; there is no standardisation of terms or clarity of criteria.

Standardisation for diagnoses and assessments, reports and reviews, treatments, and clinical pathways are needed. This is especially important for rare conditions, about which limited information is already know. The heterogeneity within the outcomes of this review means it is difficult to understand the prevalence and incidence rates of PPRCA alongside various other symptom presentations and treatment pathways. If healthcare professionals create an in-depth case review on each patient, it can allow for better exploration of causation and etiology, enabling the development of treatment or interventions to help with this. In order for research to analyse the cases and provide a comprehensive analysis of the available data, it is important for healthcare professionals to provide comprehensive case reports with all the data required to do this. In turn, this will be most beneficial for the patients. It will improve our understanding, diagnosis, and prediction of the prognosis of PPRCA, enabling for better tailored treatment plans to help stop the disease progression.

Limitations

In the initial stages of screening, we excluded 30 papers as they were in languages other than English. Due to the lack of comprehensive research studies conducted, it was not possible to complete a meta-analysis. Existing case reports and case-series studies provided limited information that enabled us to report a narrative synthesis and analyses. Our results are limited by the sample size, and various studies included in the review had low sample sizes. Consequently, the outcome may not be representative of the PPRCA population in practice. Furthermore, the duration of follow-up in the studies varied substantially. Thus, long-term studies are necessary to establish any potential advancement in the condition and treatment interventions.

PPRCA is a complex degenerate condition. The identified studies provide valuable insights into the clinical presentation, diagnosis, aetiology, and pathogenesis, improving clinicians' understanding of PPRCA. More high-quality, long-term research studies are required to produce more reliable knowledge to provide sufficient guidance for PPRCA management. These findings can guide future research, inform clinical decision-making, and enhance prevention and management strategies for PPRCA.

Funding

None

Conflicts of Interest

PP has received research grant from Novo Nordisk, and other, educational from Queen Mary University of London, other from John Wiley & Sons, other from Otsuka, outside the submitted work. All other authors report no relevant conflicts of interest for this article.

All views expressed in this manuscript are those of the authors. They do not reflect the views and values of the NHS or NIHR or any other affiliated organization.

Author contributions

PP and GD developed the concept and idea of the manuscript. SS, PP and GD developed the first draft. SS completed literature search and data extraction. SS, PP, GD, and JQS carried out the analysis. All authors reviewed and approved the final manuscript.

Acknowledgements

Registration

This systematic review was registered with PROSPERO (CRD42022346753) under the title ‘Pigmented Paravenous Retinochoroidal Atrophy (PPRCA): a systematic review on case studies and relevant commentaries. This can be accessed via the PROSPERO website (PROSPERO (york.ac.uk)).

Peter Phiri: 0000-0001-9950-3254

Sana Sajid 0000-0001-5737-7552

Gayathri Delanerolle: 0000-0002-9628-9245

Jian Qing Shi: 0000-0002-2924-1137

Gabriella De Salvo 0000-0002-1185-6942

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Tables 1 to 7 are available in the Supplementary Files section.

There is conflict of interest

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Pigmented Paravenous Retinochoroidal Atrophy (PPRCA): a systematic review

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