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BMC Ophthalmology

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01-12-2020 | Night-Blindness | Case report

Pigmented paravenous retinochoroidal atrophy associated with Vogt-Koyanagi-Harada disease: a case report

Authors: Prithvi Ramtohul, Alban Comet, Pierre Gascon, Danièle Denis

Published in: BMC Ophthalmology | Issue 1/2020

Abstract

Background

To describe a unique case of pigmented paravenous retinochoroidal atrophy that developed several years after Vogt-Koyanagi-Harada disease.

Case presentation

A 28-year-old woman presented with gradual vision loss in both eyes and nyctalopia for 2 years. Past medical history was relevant for Vogt-Koyanagi-Harada disease since the age of 19 and positive HLA-DR4. Funduscopic examination revealed perivascular pigmentary clumping and atrophic changes radiating from the optic disks. Spectral domain optical coherence tomography through the macula demonstrated perifoveal outer retinal layers loss with cystic degeneration. Fundus autofluorescence showed zonal areas of hypoautofluorescence corresponding to the areas of atrophy. Full-field electroretinogram identified mildly reduced scotopic and photopic responses. The patient was diagnosed with pigmented paravenous retinochoroidal atrophy.

Conclusions

Pigmented paravenous retinochoroidal atrophy may be acquired after Vogt-Koyanagi-Harada disease. Pathogenesis of pigmented paravenous retinochoroidal atrophy may involve inflammatory-related precursors on a background of genetic predisposition.

Brown TH. Retino-choroiditis radiata. Br J Ophthalmol. 1937;21(12):645–8. https://doi.org/10.1136/bjo.21.12.645.CrossRefPubMedPubMedCentral

Choi JY, Sandberg MA, Berson EL. Natural course of ocular function in pigmented paravenous retinochoroidal atrophy. Am J Ophthalmol. 2006;141(4):763–5. https://doi.org/10.1016/j.ajo.2005.11.009.CrossRefPubMed

McKay GJ, Clarke S, Davis JA, Simpson DAC, Silvestri G. Pigmented paravenous chorioretinal atrophy is associated with a mutation within the crumbs homolog 1 (CRB1) gene. Invest Ophthalmol Vis Sci. 2005;46(1):322–8. https://doi.org/10.1167/iovs.04-0734.CrossRefPubMed

Batioglu F, Atmaca LS, Atilla H, Arslanpençe A. Inflammatory pigmented paravenous retinochoroidal atrophy. Eye (Lond). 2002;16(1):81–4. https://doi.org/10.1038/sj.eye.6700021.CrossRef

Obata R, Yanagi Y, Iriyama A, Tamaki Y. A familial case of pigmented paravenous retinochoroidal atrophy with asymmetrical fundus manifestations. Graefe’s Arch Clin Exp Ophthalmo. 2006;244(7):874–7. https://doi.org/10.1007/s00417-005-0179-0.CrossRef

Shona OA, Islam F, Robson AG, Webster AR, Moore AT, Michaelides M. Pigmented paravenous chorioretinal atrophy: detailed clinical study of a large cohort. Retina. 2019;39(3):514–29. https://doi.org/10.1097/IAE.0000000000001950.CrossRefPubMed

Aoki S, Inoue T, Kusakabe M, et al. Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: a case report. Am J Ophthalmol Case Rep. 2017;8:14–7. https://doi.org/10.1016/j.ajoc.2017.08.003.CrossRefPubMedPubMedCentral

Strong S, Liew G, Michaelides M. Retinitis pigmentosa-associated cystoid macular oedema: pathogenesis and avenues of intervention. Br J Ophthalmol. 2017;101(1):31–7. https://doi.org/10.1136/bjophthalmol-2016-309376.CrossRefPubMed

Ahmad M, Leisy H, Carr RE, Smith RT. A rare case of unifocal, unilateral pigmented paravenous retinochoroidal atrophy (PPRCA). Am J Ophthalmol Case Rep. 2016;4:41–4. https://doi.org/10.1016/j.ajoc.2016.08.009.CrossRefPubMedPubMedCentral

10.

Shen Y, Xu X, Cao H. Pigmented paravenous retinochoroidal atrophy: a case report. BMC Ophthalmol. 2018;18(1):136. https://doi.org/10.1186/s12886-018-0809-z.CrossRefPubMedPubMedCentral

11.

Vasconcelos-Santos DV, Sohn EH, Sadda S, Rao NA. Retinal pigment epithelial changes in chronic Vogt-Koyanagi-Harada disease: fundus autofluorescence and spectral domain optical coherence tomography findings. Retina. 2010;30(1):33–41. https://doi.org/10.1097/IAE.0b013e3181c5970d.CrossRefPubMedPubMedCentral

12.

Huang H-B, Zhang Y-X. Pigmented paravenous retinochoroidal atrophy (review). Exp Ther Med. 2014;7(6):1439–45. https://doi.org/10.3892/etm.2014.1648.CrossRefPubMedPubMedCentral

13.

Murray AT, Kirkby GR. Pigmented paravenous retinochoroidal atrophy: a literature review supported by a unique case and insight. Eye. 2000;14:711–6. https://doi.org/10.1038/eye.2000.189.CrossRefPubMed

Title: Pigmented paravenous retinochoroidal atrophy associated with Vogt-Koyanagi-Harada disease: a case report
Authors: Prithvi Ramtohul
Alban Comet
Pierre Gascon
Danièle Denis
Publication date: 01-12-2020
Publisher: BioMed Central
Keywords: Night-Blindness
Early Prenatal Syphilis
Pigmentary Retinopathy
Published in: BMC Ophthalmology / Issue 1/2020
Electronic ISSN: 1471-2415
DOI: https://doi.org/10.1186/s12886-020-1318-4

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Pigmented paravenous retinochoroidal atrophy associated with Vogt-Koyanagi-Harada disease: a case report

Abstract

Background

Case presentation

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case presentation

Conclusions

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