Abstract

Case Report

Unilateral retinitis pigmentosa: Case report and review of the literature

Denise Goodwin*, Amanda M Olsen and Karl Citek

Published: 22 August, 2019 | Volume 3 - Issue 2 | Pages: 013-19

Significance: Due to the limited number of reported cases little is known about the characteristics of unilateral retinitis pigmentosa. Information from additional case reports can aid in learning more about the condition. We report a case of retinitis pigmentosa that has remained unilateral for 28 years and review the available literature.

Case Report: A 40-year-old Caucasian female presented for an opinion as to the cause of her vision loss. Fundus autofluorescence demonstrated hypoautofluorescence in the midperipheral retina and a hyperautofluorescent ring surrounding the area of preserved photoreceptors in the macula. Optical coherence tomography showed disruption of the ellipsoid zone and the external limiting membrane. Electroretinography (ERG) showed severely reduced rod and cone function monocularly.

Discussion: Retinitis pigmentosa is typically bilateral and symmetric. Unilateral retinitis pigmentosa is a rare condition that manifests with only one eye having changes typical of retinitis pigmentosa. The unaffected eye can have no signs of retinitis pigmentosa and must have a normal ERG after long-term follow up. It is critical to rule out inflammatory, traumatic, toxic, and cancer associated retinopathy that can present with retinal pigmentary changes. Unilateral retinitis pigmentosa generally remains unilateral, but long-term follow up with ERG is important. There is currently no treatment that can stop the process of retinitis pigmentosa, but gene therapy shows promise.

Read Full Article HTML DOI: 10.29328/journal.ijceo.1001021 Cite this Article Read Full Article PDF

Keywords:

Fundus autofluorescence; Optical coherence tomography; Multifocal electroretinography; Retinitis pigmentosa; Unilateral retinitis pigmentosa

References

  1. Auerbach E, Rowe H. The “good” eye in unilateral retinitis pigmentosa. Ophthalmologica. 1968; 155: 98-116. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/5636434
  2. Hartong DT, Berson EL, Dryja TP. Retinitis pigmentosa. Lancet. 2006; 368: 1795-1809. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/17113430
  3. Potsidis E, Berson EL, Sandberg MA. Disease course of patients with unilateral pigmentary retinopathy. Invest Opthalmology Vis Sci. 2011; 52: 9244-9249. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/21989720
  4. Francois J, Verriest G. Retinitis pigmentosa unilateral. Ophthalmologica. 1952; 124: 65-88. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/12993447
  5. Weller JM, Michelson G, Juenemann AG. Unilateral retinitis pigmentosa: 30 years follow-up. BMJ Case Rep. 2014; bcr2013202236-bcr2013202236. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/24515232
  6. Brill TF. Marcus Gunn pupil in a possible case of unilateral retinitis pigmentosa. Am J Optom Physiol Opt. 1979; 56: 252-258. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/517626
  7. Marsiglia M, Duncker T, Peiretti E, Brodie SE, Tsang SH. Unilateral retinitis pigmentosa: a proposal of genetic pathogenic mechanisms. Eur J Ophthalmol. 2012; 22: 654-660. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/22139616
  8. Pearlman JT, Saxton J, Hoffman G. Unilateral retinitis pigmentosa sine pigmento. Br J Ophthalmol. 1976; 60: 354-360. PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1042728/
  9. Alina-Cristina S, Marian B, Mihail Z. Unilateral pigmentary retinopathy – a review of literature and case presentation. Rom J Ophthalmol. 2016; 60: 47-52. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/27220234
  10. Thakur A, Puri L. Unilateral retinitis pigmentosa. Clin Exp Optom. 2010; 93: 102-104. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/20406260
  11. Kolb H, Galloway NR. Three cases of unilateral pigmentary degeneration. Br J Ophthalmol. 1964; 48: 471-479. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/14218207
  12. Farrell DF. Unilateral retinitis pigmentosa and cone-rod dystrophy. Clin Ophthalmol. 2009; 3: 263-270. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/19668577
  13. Padraglia C. Klinische beobachtungen. Retinitis pigmentosa. Klin Mbl Augenheilk. 1865; 3: 114-117. PubMed: https://www.ncbi.nlm.nih.gov/ pubmed/3259404
  14. Skalka HW. Asymmetric retinitis pigmentosa, luetic retinopathy and the question of unilateral retinitis pigmentosa. Acta Ophthalmol (Copenh). 1979; 57: 351-357. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/474081
  15. Seeliger M, Kretschmann U, Apfelstedt-Sylla E, Rüther K, Zrenner E. Multifocal electroretinography in retinitis pigmentosa. Am J Ophthalmol. 1998; 125: 214-226. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/9467449
  16. Bunker CH, Berson EL, Bromley WC, Hayes RP, Roderick TH. Prevalence of retinitis pigmentosa in Maine. Am J Ophthalmol. 1984; 97: 357-365. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/6702974
  17. Spadea L, Magni R, Rinaldi G, Dragani T, Bianco G. Unilateral retinitis pigmentosa: clinical and electrophysiological report of four cases. Ophthalmologica. 1998; 212: 350-354. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/9693297
  18. Mukhopadhyay R. Holder GE, Moore AT, Webster AR. Unilateral retinitis pigmentosa occurring in an individual with a germline mutation in the rp1 gene. Arch Ophthalmol. 2011; 129: 954-956. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/21746989
  19. Heckenlively JR, Yoser SL, Friedman LH, Oversier JJ. Clinical findings and common symptoms in retinitis pigmentosa. Am J Ophthalmol. 1988; 105: 504-511. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/3259404
  20. Ogura S, Yasukawa T, Kato A, Usui H, Hirano Y, et al. Wide-field fundus autofluorescence imaging to evaluate retinal function in patients with retinitis pigmentosa. Am J Ophthalmol. 2014; 158: 1093-1098. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/25062603
  21. Mitamura Y, Mitamura-Aizawa S, Nagasawa T, Katome T, Eguchi H, et al. Diagnostic imaging in patients with retinitis pigmentosa. J Med Invest. 2012; 59: 1-11. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/22449988
  22. Greenstein VC, Duncker T, Holopigian K, Carr RE, Greenberg JP, et al. Structural and functional changes associated with normal and abnormal fundus autofluorescence in patients with retinitis pigmentosa. Retina. 2012; 32: 349-357. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/21909055
  23. Escher P, Tran HV, Vaclavik V, et al. Double concentric autofluorescence ring in NR2E3-p.G56R-linked autosomal dominant retinitis pigmentosa. Investig Opthalmology Vis Sci. 2012; 53: 4754-4764. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/22661467
  24. Duncker T, Tabacaru MR, Lee W, Tsang SH, Sparrow JR. Comparison of near-infrared and short-wavelength autofluorescence in retinitis pigmentosa. Investig Opthalmology Vis Sci. 2013; 54: 585-591. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/23287793
  25. Lima LH, Burke T, Greenstein VC, Chai LC, Wener C, et al. Progressive constriction of the hyperautofluorescent ring in retinitis pigmentosa. Am J Ophthalmol. 2012; 153: 718-727. PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3830946/
  26. Iriyama A, Yanagi Y. Fundus autofluorescence and retinal structure as determined by spectral domain optical coherence tomography, and retinal runction in retinitis pigmentosa. Graefes Arch Clin Exp Ophthalmol. 2012; 250: 333-339. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/21947266
  27. Battaglia Parodi M, La Spina C, Triolo G, Riccieri F, Pierro L, et al. Correlation of SD-OCT findings and visual function in patients with retinitis pigmentosa. Graefes Arch Clin Exp Ophthalmol. 2016; 254: 1275-1279. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/26472300
  28. Hagiwara A, Mitamura Y, Kumagai K, et al. Photoreceptor impairment on optical coherence tomographic images in patients with retinitis pigmentosa. Br J Ophthalmol. 2013; 97: 237-238. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/23172877
  29. Yoon CK, Yu HG. The structure-function relationship between macular morphology and visual function analyzed by optical coherence tomography in retinitis pigmentosa. J Ophthalmol. 2013; 2013: 1-7. PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3866715/
  30. Guadagni V, Novelli E, Piano I, Gargini C, Strettoi E. Pharmacological approaches to retinitis pigmentosa: a laboratory perspective. Prog Retin Eye Res. 2015; 48: 62-81. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/26113212
  31. Rayapudi S, Schwartz SG, Wang X, Chavis P. Vitamin A and fish oils for retinitis pigmentosa. Cochrane Database Syst Rev. 2013; 12: CD008428. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/24357340
  32. Yoshida N, Ikeda Y, Murakami Y, Nakatake S, Fujiwara K. Factors affecting visual acuity after cataract surgery in patients with retinitis pigmentosa. Ophthalmology. 2015; 122: 903-908. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/25601536
  33. Gauvin M, Chakor H, Koenekoop RK, Little JM, Lina JM, et al. Witnessing the first sign of retinitis pigmentosa onset in the allegedly normal eye of a case of unilateral rp: a 30-year follow-up. Doc Ophthalmol. 2016; 132: 213-229. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/27041556

Figures:

Figure 1

Figure 1

Figure 1

Figure 2

Figure 1

Figure 3

Figure 1

Figure 4

Figure 1

Figure 5

Figure 1

Figure 6

Similar Articles

Recently Viewed

Read More

Most Viewed

Read More

Help ?