Case Report electroretinog raphy; Retinitis pigmentosa; Unilateral retinitis pigmentosa

Unilateral retinitis
pigmentosa: Case report
and review of the literature
Denise Goodwin1*, Amanda M Olsen2 and Karl
Citek3
1
Professor, OD, FAAO, Pacifi c University College of Optometry, 2043
College Way, Forest Grove, Oregon, USA
2
OD, South East Eyecare in Estevan, Saskatchewan, Canada Introduction
3
Professor, OD, PhD, FAAO, Pacifi c University College of Optometry, 2043
Retinitis pigmentosa is typically a bilateral, progressive
College Way, Forest Grove, Oregon, USA
retinal degeneration that ultimately leads to death of both
rod and the cone photoreceptors. Retinitis pigmentosa is
Abstract generally symmetric, but can present asymmetrically [1,2].
A challenge presents if only one eye has the hallmark
Signifi cance: Due to the limited number of reported cases little is known characteristics of bone spicules in the mid and far
about the characteristics of unilateral retinitis pigmentosa. Information
periphery of the retina, attenuated retinal vessels, and a
from additional case reports can aid in learning more about the condition.
We report a case of retinitis pigmentosa that has remained unilateral for pale, waxy appearing optic nerve head. In order to be
28 years and review the available literature. considered unilateral retinitis pigmentosa, the patient must
Case Report: A 40-year-old Caucasian female presented for an opinion as be followed for a sufϐiciently long time, at least 5 years,
to the cause of her vision loss. Fundus autofl uorescence demonstrated with sensitive testing methods that rule out abnormalities
hypoautofl uorescence in the midperipheral retina and a hyperautofl
in the contralateral eye [3,4]. Differentiating between
uorescent ring surrounding the area of preserved photoreceptors in the
macula. Optical coherence tomography showed disruption of the ellipsoid bilateral retinitis pigmentosa and the much rarer unilateral
zone and the external limiting membrane. Electroretinography (ERG) retinitis pigmentosa is critical since it affects the prognostic
showed severely reduced rod and cone function monocularly.
and psychological aspects of management.
Discussion: Retinitis pigmentosa is typically bilateral and symmetric.
Unilateral retinitis pigmentosa is a rare condition that manifests with only There are few reports of unilateral retinitis pigmentosa
one eye having changes typical of retinitis pigmentosa. The unaffected in the literature, and some have complicating factors or
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 infl ammatory, represent extremely asymmetric retinitis pigmentosa
traumatic, toxic, and cancer associated retinopathy that can present with [1,5-11]. In retrospective reviews of those with retinitis
retinal pigmentary changes. Unilateral retinitis pigmentosa generally
pigmentosa, between 0.002 and 5% of the study
remains unilateral, but long-term follow up with ERG is important. There is
currently no treatment that can stop the process of retinitis pigmentosa, populations had unilateral retinitis pigmentosa [1,3,12].
but gene therapy shows promise. The higher values12 may be skewed since there was no long
More Information term follow up of most of the patients. The true frequency
*Address for Correspondence: Denise Goodwin, OD, FAAO, Pacifi c University of the disease is unknown, but with more case reports
College of Optometry, 2043 College Way, Forest Grove, OR 97116, USA,Tel: + (503)
illustrating this disease, additional information may be
352-3070; Fax: (503) 352-2929; Email: goodwin@pacifi cu.edu
obtained. Here, we present a case of a woman with
Submitted: 17 July 2019
Approved: 21 August 2019
unilateral retinitis pigmentosa, and we review the available
Published: 22 August 2019 literature.
How to cite this article: Goodwin D, Olsen AM, Citek K. Unilateral retinitis
pigmentosa: Case report and review of the literature. Int J Clin Exp Ophthalmol.
Case Report
2019; 3: 013-019.
A 40-year-old Caucasian female originally presented
doi:10.29328/journal.ijceo.1001021
requesting an opinion as to the cause of her vision loss.
Copyright: © 2019 de Goodwin D, et al. This is an open access article distributed
under the Creative Commons Attribution License, which permits unrestricted use,
After extensive review of past records, we found that she
distribution, and reproduction in any medium, provided the original work is properly had been given a previous diagnosis of unilateral retinitis
cited
pigmentosa
Keywords: Fundus autofl uorescence; Optical co herence tomography; Multifocal

Open Access www.heighpubs.org 013

Unilateral retinitis pigmentosa: Case report and review of the literature
 30 degrees (Figure 4).

by one retinal specialist but was then told she did not have
this by another retinal specialist. She started having
symptoms at the age of 14 years when she saw a “black,
ϐloating oval” in her left eye. Previous exam records indicated
that the left eye
visual acuity was 20/80 (6/24) twelve years previous to our Figure 1: The fundus of the right eye (a) was unremarkable. The left eye
examination, and 20/200 (6/60) ten years previous to our image (b) shows bone spicules in the mid-peripheral retina, as well as
pallor of the optic nerve and attenuation of the retinal arteries. The left
examination. The vision loss continued to progress to 20/400 image was partially obstructed due to the posterior subcapsular cataract.
(6/120) about 6 years previous to our evaluation. Posterior
subcapsular cataract, vascular attenuation, bone spicule
pigmentary changes, and cystoid macular edema were noted
in the left eye at these visits.

Health history was remarkable for gall bladder surgery

and polycystic ovarian syndrome resulting in a hysterectomy.
She had no history of trauma or systemic inϐlammatory
conditions but was a former cigarette smoker. She reported
no family history of retinitis pigmentosa. She has two
children, aged 12 years and 7 years, both of whom had
unremarkable ocular health and no evidence of retinitis
pigmentosa.

Best corrected visual acuities were 20/15 (6/4.5) in the

right eye and light perception at one meter in the left eye. A
left constant exotropia of 25 prism diopters was present.
Extraocular muscle testing was full with no diplopia or pain
on eye movement. Pupils were round and reactive to light
with a left relative afferent pupillary defect.

Intraocular pressures were 16 mmHg in each eye. There

was grade 2+ posterior subcapsular cataract in the left eye.
The remainder of the anterior ocular health was
unremarkable in both eyes. Posterior segment in the right
eye showed a healthy looking optic nerve head with no pallor
and no retinal pigmentary changes. The left eye revealed a Figure 2: Visual fi eld of the right (a) and left (b) eye.
pallorous optic nerve head with peripapillary atrophy. The
retinal arteries were attenuated. Bone spicules were seen
throughout the mid-peripheral retina (Figure 1). A macular
lamellar hole was present slightly inferior to the macula.

Automated Humphrey 30-2 SITA-standard visual ϐield was

unremarkable in the right eye. The left eye showed severe
defects in all four quadrants, with relative sparing of the
central 5 degrees of ϐield (Figure 2).

Macular optical coherence tomography of the right eye

was unremarkable with normal macular contour and
thickness and no disruption of the photoreceptor inner
segment/outer segment junction. The left eye optical
coherence tomography revealed a lamellar hole, as well as
signiϐicant disruption of the inner segment/outer segment
junction and external limiting membrane (Figure 3).
Figure 3: Macular optical coherence tomography of the right eye (top)
shows normal foveal architecture and complete photoreceptor inner
A 61 Hex multi-focal electroretinography (Diagnosys LLC, segment/outer segment junction. Left eye (bottom) demonstrates a partial
Lowell, Massachusetts, USA) performed according to thickness lamellar hole, as well as signifi cant disruption of photoreceptor
International Society for Clinical Electrophysiology of Vision inner segment/outer segment junction, external limiting membrane, and
retinal pigmented epithelium.
(ISCEV) standards (http://iscev.org/standards/index.html)
with DTL electrodes showed good reliability with normal
amplitudes and implicit times in the right eye. The left eye
demonstrated decreased amplitudes throughout the central
 Published: August 22, 2019 014
Unilateral retinitis pigmentosa: Case report and review of the literature

Fundus autoϐluorescence showed normal retinal pigment

A full-ϐield electroretinogram (Diagnosys LLC, Lowell, ed epithelium in the right eye, but a ring of hyperautoϐluores
Massachusetts, USA) with DTL electrodes was performed cence surrounding the macula, as well as extensive hypoauto
according to ISCEV standards (https://iscev.wildapricot.org/ ϐluorescence throughout the vascular arcades in the left eye
standards). The average of two reliable readings for each (Figure 6).
protocol is shown in ϐigure 5. Following 10 minutes of light
We continued to follow the patient for an additional two
adaptation, the light-adapted 3.0 electroretinogram (30 cd*m
2 years with no signiϐicant changes. At the most recent visit,
background) showed normal a and b wave amplitude and
her son was tested and did not show any indication of
implicit time in the right eye. The left eye demonstrated an
retinitis pigmentosa on electroretinogram.
indistinguishable curve. This was followed by a light-adapted
3.0 ϐlicker electroretinogram (30 cd*m-2 background) which Discussion
showed a sinusoidal wave with normal amplitude. The left
eye demonstrated an indistinguishable curve. Following 20 Retinitis pigmentosa is used to describe a group of
minutes of dark adaptation, dark-adapted 0.01, dark hereditary retinal disorders that leads to progressive death of
adapted 3.0, and dark-adapted 10.0 electroretinograms were retinal photoreceptors, both rods and cones. The classic signs
performed. Each showed a well-formed curve with normal a include intraretinal pigmentation (bone spicules) in the mid
and b wave amplitudes and implicit times for the right eye and far periphery of the retina, attenuated retinal vessels,
and an indistinguishable curve in the left eye. and a pale, waxy appearing optic nerve head. Other
complications associated with retinitis pigmentosa include
posterior subcapsular cataracts and cystoid macular edema.

Unilateral retinitis pigmentosa was described as early as

1865 [13]. Since that time the existence of the diagnosis has
been questioned. It has been postulated that patients could
have had very asymmetric disease that would have been seen
if sufϐiciently sensitive testing was available [14].
Even today the existence of unilateral retinitis pigmentosa is
questioned. Some feel bilaterality is an essential diagnostic
criteria of retinitis pigmentosa [15]. With the availability of
electroretinography, autoϐluorescence, and optical coherence
tomography, conϐirmation of the diagnosis is now possible.

Epidemiology and etiology

Figure 4: Multifocal electroretinography trace array (a), standard deviation
map (b), and group averages (c). Normal trace array values are in red, and
Bilateral retinitis pigmentosa is estimated to affect 1 in
patient values are in black. Topography values in red are below average. 4,000 people and has been widely studied [2,16]. Forty-ϐive
Those in green are above average. separate gene loci have been identiϐied that account for 50%
of all bilateral retinitis pigmentosa cases [2]. In addition,
between 20%-30% of patients with bilateral retinitis
pigmentosa have an associated systemic disease, with more
than 30 syndromes identiϐied [2]. The most widely known
associated syndrome, Usher Syndrome, results in retinitis
pigmentosa and hearing impairment.

Figure 6: Fundus autofl uorescence of the right (a) and left (b) eye. Despite
Figure 5: Full fi eld electroretinography showed normal fi ndings in the right reduced quality due to poor fi xation and the cataract, a hyperfl uorescent
eye and signifi cantly reduced function in the left eye with the light-adapted ring is present around the macula, and hypopigmentation is present in the
3.0 electroretinography (a), light-adapted 3.0 fl icker electroretinography midperipheral retina.
(b), dark-adapted 0.01 electroretinography (c), dark-adapted 3.0
electroretinography (d), and dark-adapted 10.0 electroretinography.
 Published: August 22, 2019 015
Unilateral retinitis pigmentosa: Case report and review of the literature
time, the rods continue to die, leading to an enlarged scotoma
that eventually results in tunnel vision with a small central
island of vision. The rod photoreceptors die faster than the
Unilateral retinitis pigmentosa is reported to occur in cone photoreceptors, which allows relative sparing of the
0.002 and 5% of those with retinitis pigmentosa [1,3,12]. To central visual acuity [2]. Other common complaints include
be considered unilateral retinitis pigmentosa, one eye must decreased peripheral vision and photophobia. Up to 35%
show functional changes that are typical of retinitis experience photopsias [19].
pigmentosa while the other, unaffected eye, can have no
symptoms of retinitis pigmentosa and must have a normal Our patient had all of the classic retinal ϐindings of
electroretinogram [3]. The patient must be examined at retinitis pigmentosa, including intraretinal bone spicules,
sufϐiciently long intervals to ensure that it is not a highly attenuated retinal arterioles, and a pale, waxy optic disc.
asymmetric case of bilateral retinitis pigmentosa with Other ϐinding consistent with retinitis pigmentosa were
delayed onset in the other eye. Inϐlammatory and other present including a posterior subcapsular cataract and
causes of retinal pigmentary changes must also be excluded evidence of past macular edema (lamellar hole). Other
to satisfy the diagnosis of unilateral retinitis pigmentosa. technology helpful in the diag
nosis and continuing care of unilateral retinitis pigmentosa
The true etiology of unilateral retinitis pigmentosa is includes fundus autoϐluorescence, optical coherence tomog
difϐicult to determine due to the small number of cases. raphy, and electroretinography.
Sporadic appearance of the disease is more likely than
genetic manifestation in both unilateral and bilateral retinitis With fundus autoϐluorescence, retinal pigmented
pigmentosa. Many case studies suggested that those with epithelial cell death is represented by hypoautoϐluorescence.
unilateral retinitis pigmentosa have no family history of In retinitis pigmentosa, this hypoautoϐluorescence is seen
retinitis pigmentosa [1,3,6,11,17]. However, Farrell, et al. [12]. throughout midperipheral retina and corresponds with
Reported that while none of their 14 subjects with unilateral visual ϐield loss [20]. Additionally, more than half of retinitis
retinitis pigmentosa had a family history of unilateral retinitis pigmentosa patients display a hyperautoϐluorescent ring
pigmentosa, about one third of those with unilateral retinitis around the macula [21]. Hyperautoϐluorescence corresponds
pigmentosa had a family member with bilateral retinitis to increased levels of lipofuscin in the retinal pigmented
pigmentosa. Unfortunately, there was only long term follow epithelium or other ϐluorophores in the photoreceptor layer
up on two of these patients so it is possible that these were [22]. This increased intensity implies stressed retinal
asymmetric retinitis pigmentosa cases. Regardless, this pigmented epithelium and ongoing degeneration of
retrospective study found that of 256 bilateral cases, 34% photoreceptors. The inner border of the
had a genetic component, with the mode of inheritance being hyperautoϐluorescent ring has been shown to correspond
autosomal dominant in 59%, autosomal recessive in 34%, with the area of preserved visual function and relative
and X-linked in 7%. The 14 unilateral cases had a similar preservation of cone photoreceptors on optical coherence
inheritance pattern: 36% of those with unilateral retinitis tomography [22,23]. Outside the ring, visual function is
pigmentosa had a genetic component with 80% being severely affected [23,24]. The size of the ring may be useful in
autosomal dominant, 20% being autosomal recessive, and monitoring the progression of the disease [22,25]. Generally,
none being X-linked. Mukhopadhyay, et al. [18]. presented a those with abnormal autoϐluorescence within the
case of unilateral retinitis pigmentosa with a p.R677X hyperautoϐluorescent ring have an absent inner segment/
mutation, the most common cause of autosomal dominant outer segment junction in the foveal region [26]. Our patient
retinitis pigmentosa. did have hypoautoϐluorescence in the midperipheral area, as
well as a hyperautoϐluorescent ring surrounding the macula
Unilateral retinitis pigmentosa has an age of onset of 31 in the left eye. In addition to the hyperautoϐluorescent ring,
years for non-familial cases and 38 years for familial cases our patient had other areas of hyperautoϐluorescence within
[12]. This is an older age compared with the age of onset with the perimacular ring. This may at least partially account for
bilateral retinitis pigmentosa of 11 to 24 years [12,19]. This the severe vision loss.
later age of onset is theorized to be due to the masking of
symptoms from the unaffected eye; however, sample sizes in Optical coherence tomography is useful for evaluating
studies of unilateral retinitis pigmentosa are small. macular edema, but it can also be used in evaluating progres
sion of photoreceptor damage associated with retinitis pig
Clinical manifestations/Evaluation
mentosa. The photoreceptor inner segment/outer segment
Patients with both bilateral and unilateral retinitis junction is seen on optical coherence tomography as a hyper
pigmentosa typically present with concerns of decreased reϐlective line above the retinal pigmented epithelium layer.
night vision or difϐiculty with dark adaptation. This can be The presence of the inner segment/outer segment junction
attributed to the death of rod photoreceptors, which in turn indicates functioning photoreceptors. Disruption of this line
causes release of pigment by the retinal pigment epithelial occurs with disturbance of the structure of the cells. In retini
cells creating a bone spicule formation in the mid and far tis pigmentosa, the presence and regularity of the inner seg
periphery of the retina. The death of these photoreceptors ment/outer segment junction is correlated with best-correct-
creates a ring-shaped scotoma in peripheral visual ϐield. With
 Published: August 22, 2019 016
Unilateral retinitis pigmentosa: Case report and review of the literature
particularly those caused by phenothiazine drugs,
chloroquine,
or hydroxychloroquine can cause maculopathy, peripheral
ed visual acuity and preserved visual ϐield, and continued pigmentary retinopathy, and decreased night vision [6].
loss of the inner segment/outer segment junction is Traumatic retinopathies, especially those from blunt trauma,
associated with retinitis pigmentosa progression [27-29]. In can also show retinal pigmentary changes similar to retinitis
addition, the absence the external limiting membrane pigmentosa. Finally, cancer associated retinopathy can
correlates with de creased visual acuity [27]. This is present with similar signs such as retinal artery attenuation
consistent with our patient where vision was reduced to light and pigmentary changes. However, the time course for vision
perception with absence of the inner segment/outer segment loss occurs over week or months for cancer associated
junction and external lim iting membrane in the perimacular retinopathy compared to years with retinitis pigmentosa. Our
area and irregular inner segment/outer segment junction in patient reported no history consistent with inϐlammation,
the central retina. use of known retinotoxic medications, or trauma; and the
vision loss occurred over many years.
Electroretinography is very helpful in the diagnosis and
monitoring of retinitis pigmentosa. Generally, rod function is Treatment/Management
greatly affected. Cone function, although less affected, is still
abnormal. A full ϐield electroretinogram can be extremely There are currently no studies involving the treatment of
helpful in the diagnosis of retinitis pigmentosa as it measures unilateral retinitis pigmentosa so management is based on
the function of the overall retina. Our patient showed bilateral retinitis pigmentosa studies. Unfortunately, there is
signiϐicantly decreased cone and rod function in the left eye. currently no treatment that can stop or reverse the process of
Despite it being more than 25 years since her ϐirst symptoms, retinitis pigmentosa. Gene therapy is being investigated as a
rod function in the right eye was completely normal. treatment, and in studies with Leber congenital amaurosis it
has been shown to be protective rather than restorative [30].
Multifocal electroretinography may be especially useful in Because of this, gene therapy is likely more effective in the
advanced retinitis pigmentosa where the remaining retinal early stages of the disease. Vitamin A therapy is
function is too small to be seen with a full ϐield controversial, and the minimal slowing of retinitis
electroretinogram. In mild retinitis pigmentosa, the outer pigmentosa progression must be balanced by the potential
ring average correlates well with the scotopic mixed cone/ adverse effects of long-term, high
rod amplitude performed with full ϐield electroretinogram dosage vitamin A usage [30,31]. Other antioxidants, including
[31]. Multifocal electroretinography shows a gradual loss in omega-3 fatty acid and lutein, may have some beneϐit, but
amplitude that is greatest in the perimacular area, while more studies are necessary to determine the effectiveness.
more advanced cases also affect the macular response [32]. With bilateral retinitis pigmentosa, low vision services are
Implicit time of the central ring remains at a normal level but often utilized, but this may not be necessary in unilateral
increases with increased eccentricity [15, 31]. There is a retinitis pigmentosa depending on the quality of vision in the
correlation between best-corrected acuity and multifocal unaffected eye. Genetic counseling is recommended,
electroretinogram amplitude of the central hexagon [31,33]. especially if there is a family history of retinitis pigmentosa.
The relationship between visual acuity and implicit time is Cataract extraction may be warranted to decrease glare and
more controversial. Moschos, et al. [33], found an inverse improve vision of the affected eye, although prognosis is
relationship between acuity and implicit time. Others have guarded in those with a disruption of the inner
not found this relationship between acuity and implicit time segment/outer segment junction [32].
[31]. Our patient showed signiϐicantly reduced amplitude in
all concentric rings, including the central hexagon. Due to the Prognosis
reduced amplitude not forming a deϐinite peak, implicit time
is difϐicult to determine. The central retinal function of this There are very few studies on long-term follow-up of
patient may be affected in part due to the presence of the unilateral retinitis pigmentosa, but it has been previously
lamellar hole, but given the severity of abnormal central understood that unilateral retinitis pigmentosa stays
multifocal electroretinogram function, degeneration due to unilateral. Farrell, et al. [12], had two patients who were
retinitis pigmentosa is more likely to be the cause of our followed for 8 years and 14 years, respectively, and in both
patient’s visual loss. cases the unaffected eye had no signs of RP. Weller, et al. [5],
had one patient who showed no changes in the unaffected
Differential diagnosis eye over a time span of 30 years. However, Gauvin and
colleagues [33], had a patient present with unilateral retinitis
It is important to rule out other causes of treatable retinal pigmentosa who was seen eight separate times over thirty
pigmentary degeneration, vessel attenuation, and optic nerve years during which bilateral retinitis pigmentosa developed.
pallor. The main differentials can be excluded with a thorough The electroretinogram was the ϐirst assessment to detect the
case history. Infectious or inϐlammatory conditions, such as development of signs of retinitis pigmentosa in the
syphilis or toxoplasmosis, can result in pigment irregularities contralateral eye. There should be caution in explaining
similar to retinitis pigmentosa [14,34]. Toxic retinopathies, long-term prognosis to patients with unilateral retinitis
 Published: August 22, 2019 017
Unilateral retinitis pigmentosa: Case report and review of the literature
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.
pigmentosa. Follow-up visits, especially electroretinography, PubMed: https://www.ncbi.nlm.nih.gov/pubmed/22139616
should be conducted routinely with these patients.
8. Pearlman JT, Saxton J, Hoffman G. Unilateral retinitis pigmentosa sine
Most of what is known about the progression of unilateral pigmento. Br J Ophthalmol. 1976; 60: 354-360.
PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1042728/
retinitis pigmentosa is inferred from studies on bilateral
retinitis pigmentosa. However, Potsidis et al. [3], 9. Alina-Cristina S, Marian B, Mihail Z. Unilateral pigmentary retinopathy –
retrospectively investigated 15 cases of unilateral retinitis a review of literature and case presentation. Rom J Ophthalmol. 2016;
60: 47-52.
pigmentosa to determine the rate of visual ϐield and PubMed: https://www.ncbi.nlm.nih.gov/pubmed/27220234
electroretinogram loss compared to the normal, age-related
changes of the opposite eye. The visual ϐield had an annual 10. Thakur A, Puri L. Unilateral retinitis pigmentosa. Clin Exp Optom. 2010;
93: 102-104.
decrease of 4.9% compared to no change in a normal, aging PubMed: https://www.ncbi.nlm.nih.gov/ pubmed/ 20406260
eye. The electroretinogram amplitude with 0.5 Hz ϐlashes
decreased by 4.7% each year, whereas senile changes resulted 11. Kolb H, Galloway NR. Three cases of unilateral pigmentary
degeneration. Br J Ophthalmol. 1964; 48: 471-479.
in a decrease of 1.4%. The electroretinogram amplitude with PubMed: https://www.ncbi.nlm.nih.gov/pubmed/14218207
30.0 Hz ϐlashes showed a similar decrease of 4.6% each year
in the retinitis pigmentosa eye versus 1.2% decrease in the 12. Farrell DF. Unilateral retinitis pigmentosa and cone-rod dystrophy. Clin
Ophthalmol. 2009; 3: 263-270.
normal, aging eye. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/19668577

Conclusion 13. Padraglia C. Klinische beobachtungen. Retinitis pigmentosa. Klin Mbl

Augenheilk. 1865; 3: 114-117.
Here we present a case of unilateral retinitis pigmentosa. PubMed: https://www.ncbi.nlm.nih.gov/ pubmed/3259404
Twenty-eight years following the initial symptoms, a 14. Skalka HW. Asymmetric retinitis pigmentosa, luetic retinopathy and
complete workup showed no signs of retinitis pigmentosa in the question of unilateral retinitis pigmentosa. Acta Ophthalmol
the contralateral eye. Unilateral retinitis pigmentosa is a rare (Copenh). 1979; 57: 351-357.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/474081
condition in which only one eye manifests the symptoms and
clinical hallmarks of retinitis pigmentosa. The unaffected eye 15. Seeliger M, Kretschmann U, Apfelstedt-Sylla E, Rüther K, Zrenner E.
has no signs or symptoms of retinitis pigmentosa, including a Multifocal electroretinography in retinitis pigmentosa. Am J
Ophthalmol. 1998; 125: 214-226.
normal electroretinogram. Once the differential diagnoses PubMed: https://www.ncbi.nlm.nih.gov/pubmed/9467449
are ruled out, electrodiagnostic data can aid in conϐirming the
diagnosis of unilateral retinitis pigmentosa. More information 16. Bunker CH, Berson EL, Bromley WC, Hayes RP, Roderick TH.
Prevalence of retinitis pigmentosa in Maine. Am J Ophthalmol. 1984;
about the progression of this disease is required and patients 97: 357-365. PubMed:
with unilateral retinitis pigmentosa should be closely https://www.ncbi.nlm.nih.gov/pubmed/6702974
monitored for changes in the unaffected eye. Immediate
17. Spadea L, Magni R, Rinaldi G, Dragani T, Bianco G. Unilateral retinitis
family members, especially children, should be assessed as pigmentosa: clinical and electrophysiological report of four cases.
well in order to establish or rule out inheritance patterns. Ophthalmologica. 1998; 212: 350-354.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/9693297
References 18. Mukhopadhyay R. Holder GE, Moore AT, Webster AR. Unilateral retinitis
1. Auerbach E, Rowe H. The “good” eye in unilateral retinitis pigmentosa. pigmentosa occurring in an individual with a germline mutation in the
Ophthalmologica. 1968; 155: 98-116. rp1 gene. Arch Ophthalmol. 2011; 129: 954-956.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/5636434 PubMed: https://www.ncbi.nlm.nih.gov/pubmed/21746989

2. Hartong DT, Berson EL, Dryja TP. Retinitis pigmentosa. Lancet. 2006; 19. Heckenlively JR, Yoser SL, Friedman LH, Oversier JJ. Clinical fi ndings
368: 1795-1809. and common symptoms in retinitis pigmentosa. Am J Ophthalmol.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/17113430 1988; 105: 504-511.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/3259404
3. Potsidis E, Berson EL, Sandberg MA. Disease course of patients with
unilateral pigmentary retinopathy. Invest Opthalmology Vis Sci. 2011; 20. Ogura S, Yasukawa T, Kato A, Usui H, Hirano Y, et al. Wide-fi eld fundus
52: 9244-9249. autofl uorescence imaging to evaluate retinal function in patients with
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/21989720 retinitis pigmentosa. Am J Ophthalmol. 2014; 158: 1093-1098.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/25062603
4. Francois J, Verriest G. Retinitis pigmentosa unilateral. Ophthalmologica.
1952; 124: 65-88. 21. Mitamura Y, Mitamura-Aizawa S, Nagasawa T, Katome T, Eguchi H, et
al. Diagnostic imaging in patients with retinitis pigmentosa. J Med
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/ 12993447
Invest. 2012; 59: 1-11.
5. Weller JM, Michelson G, Juenemann AG. Unilateral retinitis PubMed: https://www.ncbi.nlm.nih.gov/pubmed/22449988
pigmentosa: 30 years follow-up. BMJ Case Rep. 2014;
22. Greenstein VC, Duncker T, Holopigian K, Carr RE, Greenberg JP, et al.
bcr2013202236- bcr2013202236.
Structural and functional changes associated with normal and
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/ 24515232
abnormal fundus autofl uorescence in patients with retinitis
6. Brill TF. Marcus Gunn pupil in a possible case of unilateral retinitis pigmentosa. Retina. 2012; 32: 349-357.
pigmentosa. Am J Optom Physiol Opt. 1979; 56: 252-258. PubMed: PubMed: https://www.ncbi.nlm.nih.gov/pubmed/21909055
 Published: August 22, 2019 018
Unilateral retinitis pigmentosa: Case report and review of the literature
ceptor impairment on optical coherence tomographic images in pa
tients with retinitis pigmentosa. Br J Ophthalmol. 2013; 97: 237-238.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/23172877
23. Escher P, Tran HV, Vaclavik V, et al. Double concentric autofl
29. Yoon CK, Yu HG. The structure-function relationship between macular
uorescence ring in NR2E3-p.G56R-linked autosomal dominant retinitis
morphology and visual function analyzed by optical coherence
pigmentosa. Investig Opthalmology Vis Sci. 2012; 53: 4754-4764.
tomography in retinitis pigmentosa. J Ophthalmol. 2013; 2013: 1-7.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/22661467
PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3866715/
24. Duncker T, Tabacaru MR, Lee W, Tsang SH, Sparrow JR. Comparison
of near-infrared and short-wavelength autofl uorescence in retinitis 30. Guadagni V, Novelli E, Piano I, Gargini C, Strettoi E. Pharmacological
pigmentosa. Investig Opthalmology Vis Sci. 2013; 54: 585-591. approaches to retinitis pigmentosa: a laboratory perspective. Prog
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/23287793 Retin Eye Res. 2015; 48: 62-81.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/26113212
25. Lima LH, Burke T, Greenstein VC, Chai LC, Wener C, et al. Progressive
constriction of the hyperautofl uorescent ring in retinitis pigmentosa. 31. Rayapudi S, Schwartz SG, Wang X, Chavis P. Vitamin A and fi sh oils for
Am J Ophthalmol. 2012; 153: 718-727. retinitis pigmentosa. Cochrane Database Syst Rev. 2013; 12:
PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3830946/ CD008428. PubMed:
https://www.ncbi.nlm.nih.gov/pubmed/24357340
26. Iriyama A, Yanagi Y. Fundus autofl uorescence and retinal structure as
determined by spectral domain optical coherence tomography, and 32. Yoshida N, Ikeda Y, Murakami Y, Nakatake S, Fujiwara K. Factors
retinal runction in retinitis pigmentosa. Graefes Arch Clin Exp affecting visual acuity after cataract surgery in patients with retinitis
Ophthalmol. 2012; 250: 333-339. pigmentosa. Ophthalmology. 2015; 122: 903-908.
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/21947266
PubMed: https://www.ncbi.nlm.nih.gov/pubmed/25601536
27. Battaglia Parodi M, La Spina C, Triolo G, Riccieri F, Pierro L, et al. Cor
33. Gauvin M, Chakor H, Koenekoop RK, Little JM, Lina JM, et al.
relation of SD-OCT fi ndings and visual function in patients with
retinitis pigmentosa. Graefes Arch Clin Exp Ophthalmol. 2016; 254: Witnessing the fi rst sign of retinitis pigmentosa onset in the allegedly
1275-1279. PubMed: normal eye of a case of unilateral rp: a 30-year follow-up. Doc
https://www.ncbi.nlm.nih.gov/pubmed/26472300 Ophthalmol. 2016; 132: 213-229. PubMed:
https://www.ncbi.nlm.nih.gov/pubmed/27041556
28. Hagiwara A, Mitamura Y, Kumagai K, Baba T, Yamamoto S. Photore
Published: August 22, 2019 019