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

Yoo, Sun, Lee, and Ohn: Unilateral Retinitis Pigmentosa: A Case Series and Literature Review

초록

Purpose:

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

Methods:

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.

Results:

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.

Conclusions:

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.

References

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Figure 1.
Color fundus photographs of each patient showing typical retinitis pigmentosa abnormalities, including attenu-ated 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).
jkos-56-559f1.tif
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.
jkos-56-559f2.tif
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).
jkos-56-559f3.tif
Table 1.
Demographic characteristics of the patients in unilateral RP
Patient no. Sex/Age (years) Follow-up period (years) ) Affected eye   BCVA at first visit BCVA at last visit
1 F/24 8.5 OD AE FC/50 cm HM
        UE 1.0 1.0
2 F/36 5.5 OD AE HM NLP
        UE 0.04 0.5
3 F/61 8 OD AE HM HM
        UE 1.0 1.0
4 F/28 6 OD AE 0.9 0.8
        UE 1.0 1.0

RP = retinitis pigmentosa; BCVA = best corrected visual acuity; AE = affected eye; UE = unaffected eye; FC = finger count; HM = hand motion; NLP = no light perception.

Table 2.
ERG values in unilateral RP at initial visit
Patient no. Scotopic (μV) Mesopic (μV) Oscillatory potentials (μ V) Photopic (μV) 30 Hz flicker (μV)
1 AE Undetectable Undetectable Undetectable Undetectable 1.254
  UE 75.4 341.8 80.0 59.8 36.1
2 AE Undetectable Undetectable Undetectable Undetectable 6.1
  UE 73.2 202.0 146.9 46.4 21.6
3 AE Undetectable Undetectable Undetectable Undetectable 1.849
  UE 69.6 232.5 119.0 57.6 42.5
4 AE Undetectable 38.9 Undetectable 26.0 10.7
  UE 111.6 257.6 183.6 149.2 107.1

ERG = electroretinogram; RP = retinitis pigmentosa; AE = affected eye; UE = unaffected eye.

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