Pigmented paravenous retinochoroidal atrophy with acute angle-closure glaucoma and posterior subcapsular cataract: a case report

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Abstract

Background

Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare fundus disease characterized by the presence of osteoblast-like pigment, atrophy of retinal pigment epithelium (RPE), and choroid deposition along the large retinal veins.

Case presentation

A 55-year-old Chinese female presented with right eye distention and bilateral vision loss. Osteocyte-like pigmentation and retinal choroidal atrophy distributed along the large retinal veins were seen in the fundus of bilateral eyes. The atrophy in the left eye was more severe compared to the right eye. The patient also presented with bilateral acute angle-closure glaucoma (AACG) and posterior subcapsular cataract (PSC) accompanied with anterior segmental manifestations, similar to the complications of retinitis pigmentosa (RP). The patient underwent ultrasound biomicroscopy (UBM), Humphrey field analyser (HFA), optical coherence tomography (OCT), fundus autofluorescence (FAF), fluorescein fundus angiography (FFA), electroretinogram (ERG), and electrooculography (EOG), all of which confirmed the aforementioned diagnose.

Conclusion

PPRCA is a rare disease of unknown etiology. The patient in this case presented with complications similar to those of RP, and the two conditions may share a genetic basis. Further studies are needed to confirm this relationship.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12886-022-02355-5.

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Most cited references 8

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Intraocular cytokine profile and autoimmune reactions in retinitis pigmentosa, age‐related macular degeneration, glaucoma and cataract

Josianne ten Berge, Zainab Fazil, Ingeborgh van den Born … (2018)

Abstract Purpose To analyse intraocular cytokine levels and prevalence of intraocular antiretinal antibodies (ARAs) in patients with retinitis pigmentosa (RP), age‐related macular degeneration (AMD), glaucoma and cataract, and correlate the results to clinical manifestations. Methods We collected intraocular fluid samples from patients with RP (n = 25), AMD (n = 12), glaucoma (n = 28) and cataract (n = 22), and serum samples paired with the intraocular fluids from patients with RP (N = 7) and cataract (n = 10). Interleukin (IL)‐1β, IL‐1ra, IL‐2, IL‐6, IL‐6rα, IL‐7, IL‐8, IL‐10, IL‐17A, IL‐23, thymus‐ and activation‐regulated chemokine (TARC), monocyte chemoattractant protein‐1 (MCP‐1), tumour necrosis factor‐alpha (TNF‐α), placental growth factor (PlGF) and vascular endothelial growth factor (VEGF) were measured using a multiplex assay. Antiretinal antibodies (ARA) detection was performed by indirect immunofluorescence. Results Increasing age was associated with increasing levels of IL‐6, IL‐8, TNF‐α and VEGF. All patient groups exhibited distinct profiles of intraocular cytokines. Intraocular levels of IL‐8 were highest in patients with AMD and glaucoma. Cataract patients exhibited high intraocular levels of IL‐23. Intraocular levels of IL‐2, IL‐6, MCP‐1 and PlGF in RP patients exceeded the levels of serum, indicating intraocular production. Intraocular ARAs were found in only one patient with AMD. Conclusion Increased levels of inflammatory cytokines in intraocular fluid of patients with originally noninflammatory ocular diseases show that intraocular inflammation is involved in their pathogenesis of these entities. Moreover, we show that increasing age is associated with increasing levels of intraocular cytokines and conclude that future studies on intraocular mediators should be corrected for age of patients.

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Neuro-ophthalmic disease and optical coherence tomography: glaucoma look-alikes.

Joshua Pasol (2011)

The use of optical coherence tomography (OCT)-measured retinal nerve fiber layer (RNFL) thickness in neuro-ophthalmic disease has grown since its first use in glaucoma and retinal diseases. OCT-measured RNFL in nonglaucomatous optic neuropathies shows thinning, which may mimic those seen in glaucoma. This article aims to provide insight regarding the use of OCT in nonglaucomatous optic neuropathies and sheds light on common patterns of RNFL loss in different nonglaucomatous optic neuropathies. RNFL thinning is most likely to occur in the temporal peripapillary quadrant than in other quadrants in nonglaucomatous optic neuropathies. The pattern of RNFL thinning in ischemic optic neuropathy and optic nerve head drusen is more likely to mimic the pattern found in glaucoma due to the superior and inferior quadrant predilection. OCT-measured RNFL thickness in Alzheimer's disease reveals thinning superiorly and inferiorly, whereas superior and temporal thinning is seen in Parkinson's disease. The thinning observed in neurodegenerative diseases is believed to be multifactorial including causes such as axonal degeneration and retrograde degeneration. However, more studies are needed to further study these changes. OCT is a valuable tool in evaluating the peripapillary RNFL in both glaucomatous and nonglaucomatous optic neuropathies. This technology may be used for both research and clinical purposes to assess disease progression in optic neuropathies and diseases that affect the central nervous system. OCT-measured RNFL thickness remains complimentary to the clinical examination skills in the evaluation of nonglaucomatous optic neuropathies.

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Pigmented paravenous retinochoroidal atrophy (Review)

HOU-BIN HUANG, Yi-Xin Zhang (2014)

Pigmented paravenous retinochoroidal atrophy (PPRCA) is an uncommon disease characterized by perivenous aggregations of pigment clumps associated with peripapillary and radial zones of retinochoroidal atrophy that are distributed along the retinal veins. Patients are usually asymptomatic and the disease process is non-progressive or slow and subtly progressive. It is commonly bilateral and symmetric. The cause of the condition may be unknown or idiopathic, although a dysgenetic, degenerative, hereditary etiology or even an inflammatory cause has been hypothesized. A non-inflammatory cause is referred to as primary, while inflammation-associated PPRCA is referred to as secondary or pseudo PPRCA. The present study reviewed and summarized the features of PPRCA.

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Author and article information

Contributors

Yajuan Zheng:

ORCID: http://orcid.org/0000-0003-2327-3733

zhengyajuan124@126.com

Journal

Journal ID (nlm-ta): BMC Ophthalmol

Journal ID (iso-abbrev): BMC Ophthalmol

Title: BMC Ophthalmology

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2415

Publication date (Electronic): 22 April 2022

Publication date PMC-release: 22 April 2022

Publication date Collection: 2022

Volume: 22

Electronic Location Identifier: 184

Affiliations

GRID grid.452829.0, ISNI 0000000417660726, Department of Ophthalmology, , The Second Hospital of Jilin University, ; Changchun, China

Author information

Yajuan Zheng http://orcid.org/0000-0003-2327-3733

Article

Publisher ID: 2355

DOI: 10.1186/s12886-022-02355-5

PMC ID: 9026714

PubMed ID: 35459148

SO-VID: d30f4ee7-b91c-4408-b1ab-fed547d2cc9e

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

History

Date received : 26 July 2021

Date accepted : 15 March 2022

Funding

Funded by: FundRef http://dx.doi.org/10.13039/501100013061, Jilin Scientific and Technological Development Program;

Award ID: 20190303186SF

Award Recipient : Yajuan Zheng

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ScienceOpen disciplines: Ophthalmology & Optometry

Keywords: pigmented paravenous retinochoroidal atrophy,retinitis pigmentosa,acute angle-closure glaucoma,posterior subcapsular cataract

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ScienceOpen disciplines: Ophthalmology & Optometry

Keywords: pigmented paravenous retinochoroidal atrophy, retinitis pigmentosa, acute angle-closure glaucoma, posterior subcapsular cataract

Pigmented paravenous retinochoroidal atrophy with acute angle-closure glaucoma and posterior subcapsular cataract: a case report

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Abstract

Background

Case presentation

Conclusion

Supplementary Information

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Karger: Ophthalmology

Most cited references 8

Intraocular cytokine profile and autoimmune reactions in retinitis pigmentosa, age‐related macular degeneration, glaucoma and cataract

Neuro-ophthalmic disease and optical coherence tomography: glaucoma look-alikes.

Pigmented paravenous retinochoroidal atrophy (Review)

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