Research Article

Unilateral retinitis pigmentosa: Case report and review of the literature

Denise Goodwin*, Amanda M Olsen and Karl Citek

Published: 08/22/2019 | Volume 3 - Issue 2 | Pages: 013-019


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


  1. Auerbach E, Rowe H. The “good” eye in unilateral retinitis pigmentosa. Ophthalmologica. 1968; 155: 98-116. PubMed:
  2. Hartong DT, Berson EL, Dryja TP. Retinitis pigmentosa. Lancet. 2006; 368: 1795-1809. PubMed:
  3. Potsidis E, Berson EL, Sandberg MA. Disease course of patients with unilateral pigmentary retinopathy. Invest Opthalmology Vis Sci. 2011; 52: 9244-9249. PubMed:
  4. Francois J, Verriest G. Retinitis pigmentosa unilateral. Ophthalmologica. 1952; 124: 65-88. PubMed:
  5. Weller JM, Michelson G, Juenemann AG. Unilateral retinitis pigmentosa: 30 years follow-up. BMJ Case Rep. 2014; bcr2013202236-bcr2013202236. PubMed:
  6. Brill TF. Marcus Gunn pupil in a possible case of unilateral retinitis pigmentosa. Am J Optom Physiol Opt. 1979; 56: 252-258. PubMed:
  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:
  8. Pearlman JT, Saxton J, Hoffman G. Unilateral retinitis pigmentosa sine pigmento. Br J Ophthalmol. 1976; 60: 354-360. PubMed:
  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:
  10. Thakur A, Puri L. Unilateral retinitis pigmentosa. Clin Exp Optom. 2010; 93: 102-104. PubMed:
  11. Kolb H, Galloway NR. Three cases of unilateral pigmentary degeneration. Br J Ophthalmol. 1964; 48: 471-479. PubMed:
  12. Farrell DF. Unilateral retinitis pigmentosa and cone-rod dystrophy. Clin Ophthalmol. 2009; 3: 263-270. PubMed:
  13. Padraglia C. Klinische beobachtungen. Retinitis pigmentosa. Klin Mbl Augenheilk. 1865; 3: 114-117. PubMed: 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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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:
  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: