Journal List > J Korean Ophthalmol Soc > v.56(4) > 1010245

J Korean Ophthalmol Soc. 2015 Apr;56(4):559-566. Korean.
Published online April 15, 2015.
©2015 The Korean Ophthalmological Society
Unilateral Retinitis Pigmentosa: A Case Series and Literature Review
Seung Hoon Yoo, MD,1 Hae Jung Sun, MD,1 Sung Jin Lee, MD, PhD,1 and Young Hoon Ohn, MD, PhD2
1Department of Ophthalmology, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Korea.
2Department of Ophthalmology, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea.

Address reprint requests to Sung Jin Lee, MD, PhD. Department of Ophthalmology, Soonchunhyang University Seoul Hospital, #59 Daesagwan-ro, Yongsan-gu, Seoul 140-743, Korea. Tel: 82-2-709-9354, Fax: 82-2-710-3196, Email:
Received November 08, 2014; Revised December 31, 2014; Accepted March 07, 2015.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.



We report 4 cases of unilateral retinitis pigmentosa (URP) with a literature review.


A retrospective, observational case series of 4 URP patients with no signs of bilateral involvement during the follow-up period of up to 5 years.


The subjects were 4 female patients with an average age of 37 years. The mean follow-up period was 7 years. The subjects' blood analysis excluded any infective diseases. History of trauma or any family history of ocular diseases including retinitis pigmentosa was absent. The full-field electroretinograms (ERGs) were normal in the unaffected eyes and the affected eyes showed markedly low or undetectable responses. Computerized visual field examinations were normal in the unaffected eyes, while 3 of the affected eyes had total visual field loss and 1 had concentric narrowing within 20 degrees.


A long term follow-up of at least 5 years is required for diagnosis of URP to exclude a delayed onset in the unaffected eye. ERG is a reliable test to monitor the course of the disease and to confirm the diagnosis when uncertain. The etiology of URP is unknown although several studies regarding genetic causes that resulted in URP have been reported in recent years.

Keywords: Electroretinogram; Retinitis pigmentosa; Unilateral


Figure 1
Color fundus photographs of each patient showing typical retinitis pigmentosa abnormalities, including attenuated retinal arterioles, intraretinal clumps of black pigment, and loss of retinal pigment epithelium in the affected eye of each patient while the unaffected eyes are normal (the unaffected eye of patient 2 shows myopic fundus changes including localized chorioretinal atrophy and peripapillary atrophy).
Click for larger image

Figure 2
Full-field electroretinograms of each patient showing all 5 standard responses (rod response, maximal combined response, oscillatory potentials, cone response, 30-Hz flicker response). The responses are nearly non-recorded in the affected eyes while the unaffected eyes show nearly normal ERG responses. ERG = electroretinogram.
Click for larger image

Figure 3
Visual field change of affected eye in case 4. Concentric narrowing of visual field within 20 degrees was noticed at first visit (A). Six years later, the visual field was more narrowed about 5 degrees (B). Visual field using the Humphrey visual field analyzer (Carl-Zeiss Meditec, Dublin, CA, USA; C30-2 program).
Click for larger image


Table 1
Demographic characteristics of the patients in unilateral RP
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Table 2
ERG values in unilateral RP at initial visit
Click for larger image

1. Carr RE, Noble KG. Retinitis pigmentosa. Ophthalmology 1981;88:169–172.
2. Grover S, Fishman GA, Anderson RJ, et al. Visual acuity impairment in patients with retinitis pigmentosa at age 45 years or older. Ophthalmology 1999;106:1780–1785.
3. Bunker CH, Berson EL, Bromley WC, et al. Prevalence of retinitis pigmentosa in Maine. Am J Ophthalmol 1984;97:357–365.
4. Frank MH, Sayegh MH. Immunomodulatory functions of mesenchymal stem cells. Lancet 2004;363:1411–1412.
5. Noble KG, Carr RE. Peripapillary pigmentary retinal degeneration. Am J Ophthalmol 1978;86:65–75.
6. Francois J, Verriest G. Retinitis pigmentosa unilateral. Ophthalmologica 1952;124:65–88.
7. Pedraglia C. Retinitis pigmentosa. Klin Monbl Augenheilkd 1865;3:113–117.
8. Brill TF. Marcus Gunn pupil in a possible case of unilateral retinitis pigmentosa. Am J Optom Physiol Opt 1979;56:252–258.
9. Kim KH, Park YH, Hahn DK. A case of unilateral retinitis pigmentosa. J Korean Ophthalmol Soc 1996;37:1090–1094.
10. Spadea L, Magni R, Rinaldi G, et al. Unilateral retinitis pigmentosa: clinical and electrophysiological report of four cases. Ophthalmologica 1998;212:350–354.
11. Chen H, Wu D, Huang S, Jiang F. Unilateral retinitis pigmentosa with amblyopia in the fellow eye. Graefes Arch Clin Exp Ophthalmol 2006;244:1701–1704.
12. Thakur A, Puri L. Unilateral retinitis pigmentosa. Clin Exp Optom 2010;93:102–104.
13. Potsidis E, Berson EL, Sandberg MA. Disease course of patients with unilateral pigmentary retinopathy. Invest Ophthalmol Vis Sci 2011;52:9244–9249.
14. Dreisler KK. Unilateral retinitis pigmentosa; two cases. Acta Ophthalmol (Copenh) 1948;26:385–393.
15. Kato K, Miyake Y, Matsubara H, Uji Y. Long-term follow-up of a case of unilateral retinitis pigmentosa. Nihon Ganka Gakkai Zasshi 2012;116:1086–1093.
16. Marsiglia M, Duncker T, Peiretti E, et al. Unilateral retinitis pigmentosa: a proposal of genetic pathogenic mechanisms. Eur J Ophthalmol 2012;22:654–660.
17. Weller JM, Michelson G, Juenemann AG. Unilateral retinitis pigmentosa: 30 years follow-up. BMJ Case Rep 2014;2014:pii: bcr2013202236.
18. Ryan SJ. In: Retina. 5th ed. Vol. 2. Philadelphia: Saunders; 2013. pp. 761-762.
19. Weiss JF, Nicholl RJ. Nonsyphilitic unilateral retinitis pigmentosa. Am J Ophthalmol 1968;65:573–574.
20. Carr RE, Siegel IM. Unilateral retinitis pigmentosa. Arch Ophthalmol 1973;90:21–26.
21. Svěrák J, Peregrin J, Velický J. Unilateral pigmentary degeneration. A further case controlled by EOG and ERG. Acta Ophthalmol (Copenh) 1968;46:1256–1262.
22. Kandori F, Tamai A, Watanabe T, Kurimoto S. Unilateral pigmentary degeneration of the retina. Report of two cases. Am J Ophthalmol 1968;66:1091–1101.
23. Farrell DF. Unilateral retinitis pigmentosa and cone-rod dystrophy. Clin Ophthalmol 2009;3:263–270.
24. 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.
25. Berson EL, Sandberg MA, Rosner B, et al. Natural course of retinitis pigmentosa over a three-year interval. Am J Ophthalmol 1985;99:240–251.