Several conditions have been described as having a retinal leopard-spot
We report a similar pattern, observed in 1 eye each of 4 young patients (Figure 1), and discuss how this condition
is distinct from previously described lesions; in 2 cases, these leopard-spot
lesions were associated with choroidal neovascularization (CNV).
Fundus photograph of the posterior
pole of cases 1 (A), 2 (B), 3 (C), and 4 (D). Round lesions with peripheral
dark dots result in a leopard-spot pattern. The lesions are located at the
level of the retinal pigment epithelium (RPE) and include fibrosis, hyperplastic
changes of the RPE at the periphery, and thinning and atrophy of the RPE at
A 34-year-old man was seen because of metamorphopsia. Visual acuity
was 20/20 OU. Medical history and slitlamp examination findings were unremarkable.
Findings from right fundus examination were normal. Left fundus examination
disclosed a round lesion that was located at the level of the retinal pigment
epithelium (RPE) in the posterior pole (Figure
1A) above the macula and a localized serous retinal detachment.
Fluorescein angiography showed CNV at the inferior border of the lesion. Krypton
laser photocoagulation was applied to the new vessel. Three weeks later an
additional angiogram was performed (Figure
2A), which was of better quality than the initial study. This study
showed a leopard-spot pattern at the periphery of the lesion, with hypofluorescent
dots surrounded by reticular staining with the dye. The photocoagulation scar
appeared dark. B-scan ultrasonography did not show any signs of calcification
within the lesion. The lesion appeared to remain stable during a 2-year follow-up.
Fluorescein angiography of cases
1 (A), 2 (B), 3 (C), and 4 (D). The leopard-spot pattern appears to be localized
at the periphery of the lesion in each case. Fluorescein angiography is consistent
with thinning and atrophy of the RPE at the center of the lesion (A and B).
In one case, it also showed anomalies of retinal vasculature: tortuosity and
kinking of arterioles and venules in a localized part of the retina (B).
A 27-year-old man consulted us because he needed an attestation to renew
his boxing license. Visual acuity was 20/25 OD and 20/20 OS. Medical history
and slitlamp examination findings were unremarkable. Results of left fundus
examination were within normal limits. Right fundus examination, however,
disclosed a deep, large, grayish lesion in the interpapillomacular area, with
a leopard-spot pattern at its periphery (Figure 1B). A tiny epiretinal membrane was observed at the inferonasal
aspect of the macula, which was associated with 2 small hemorrhages. The leopard-spot
pattern appeared more obvious on fluorescein angiography (Figure 2B). Fluorescein angiography also disclosed tortuosity and
kinking of arterioles and venules located nasally and inferiorly to the macula.
We interpreted these anomalies of the retinal vessels as a consequence of
the epiretinal membrane. B-scan ultrasonography did not show any signs of
calcification within the lesion. Unfortunately, the patient was lost to follow-up.
A 16-year-old girl was seen with a complaint of "visual fatigue." Visual
acuity was 20/20 OU with correction (+0.50 OD, 90° +0.25 OS). Medical
history and slitlamp examination findings were unremarkable. The left fundus
appeared normal. Right fundus examination disclosed an irregular lesion located
at the level of the RPE, in the papillomacular area, with dark dots surrounded
by a whitish reticular net (Figure 1C).
The leopard-spot pattern appeared more obvious on fluorescein angiography
(Figure 2C). Follow-up was limited
to 6 months, but there was no change during this time.
A 24-year-old man was seen for visual loss and metamorphopsia of the
right eye. Visual acuity was 20/128 OD and 20/20 OS. Medical history revealed
type 2 diabetes mellitus, which was diagnosed when the patient was 2 years
old and treated solely by diet. His mother also had type 2 diabetes mellitus.
Slitlamp examination findings were unremarkable. The left fundus appeared
normal, but the right fundus showed a deep, oval macular lesion with a whitish
reticular border. A central macular detachment was also observed (Figure 1D). Fluorescein angiography showed
a leopard-spot–patterned lesion associated with a juxtafoveal CNV (Figure 2D). A feeder vessel was observed
in the early frames with leakage of dye on late frames (Figure 3A-B). Indocyanine green angiography also showed the CNV
on early frames and a hypofluorescent lesion on late frames (Figure 3C-D). The reticular peripheral net that was hyperfluorescent
on fluorescein angiography appeared hypofluorescent on indocyanine green angiography.
B-scan ultrasonography did not reveal any signs of choroidal calcification.
The CNV was then treated with krypton laser photocoagulation. To date (6-month
follow-up), no recurrence has occurred. A previous ophthalmic examination
had been performed 10 years ago because of the patient's diabetes. The medical
report at that time described a visual acuity of 20/20 OU, pigmentary alterations
of the right fundus, but no suggestion of diabetic retinopathy. An angiogram
had also been performed (Figure 3E).
Comparison of previous and recent angiographies disclosed marked progression
of the leopard-spot lesion.
Case 4. Fluorescein angiography
at 24 seconds (A) and 6 minutes (B). Indocyanine green angiography at 36 seconds
(C) and 25 minutes (D). Previous fluorescein angiography performed 10 years
ago (E). Feeder vessel was present on early frames (A), with marked leakage
on late frames (B). The reticular peripheral net, which was hyperfluorescent
on fluorescein angiography, appeared hypofluorescent on indocyanine green
angiography (C and D). Comparison with a previous angiogram (E) showed marked
extension of the peripheral dots.
Herein, we have described 4 young patients, 3 men and 1 girl, who presented
with a round lesion of one posterior pole. The lesion had a leopard-spot pattern
observed on color fundus photographs and, more obviously, with fluorescein
angiography. B-scan ultrasonography, performed in 3 cases, did not reveal
any calcification. Choroidal neovascularization was observed in 2 patients,
in one case at the border of the lesion and in one case within the leopard-spot
Several systemic conditions may be associated with a leopard-spot pattern
of the fundus, including leukemia, systemic form of large-cell non-Hodgkin
lymphoma, systemic carcinoma with bilateral diffuse uveal melanocytic proliferation,
and idiopathic uveal effusion syndrome.1- 5
However, all of these acute conditions appear differently from the asymptomatic
pattern observed in our patients, whose troubles were confined to a localized
part of the retina, without associated systemic or ocular conditions.
Patients with chronic idiopathic central serous chorioretinopathy may
have a bone corpuscular pattern of migration of pigment in areas of recurrent
and/or chronic serous detachment.6 This
pattern is more frequently observed in patients receiving high doses of corticosteroids.
None of our patients had previous episodes consistent with typical chronic
idiopathic central serous chorioretinopathy, and none received corticosteroids.
Furthermore, chronic idiopathic central serous chorioretinopathy is unlikely
to develop in a 16-year-old girl (case 3).
Trauma should be taken seriously as a possible cause of the pattern
observed in the present cases. Traumatic choroidopathy can result from acute
contusion necrosis of the RPE. Rupture in the inner choroid and RPE may be
absent, but hemorrhagic detachment of the retina is frequently observed. Resolution
of the detachment and hemorrhages may then reveal varying degrees of RPE atrophy.7 Case 2 acknowledged many instances of trauma to
the face during his boxing activities. However, none of the 4 patients remembered
severe ocular trauma. Moreover, a traumatic origin appeared particularly unlikely
in case 4, for whom comparison of recent and previous angiograms showed marked
changes (Figure 3). In this case,
one could assume that secondary hyperplastic changes followed initial sequelae
of trauma; however, a lack of recollection of severe trauma in all patients
and demonstration of evolution of the lesions in one case led us to hypothesize
that a traumatic cause is unlikely. However, ocular trauma cannot be ruled
out because it could have occurred during infancy or childhood without any
Choroidal or subretinal neovascularization is an acquired abnormality
observed in many congenital, degenerative, infectious, inflammatory, tumoral,
and traumatic processes, some of which are more commonly observed in young
patients.8 Among these conditions, choroidal
osteomas may show a pattern similar to that seen in our patients. Choroidal
osteomas, in summary, are unique, unilateral tumors that arise in the juxtapapillary
and macular region of young adults.9 They
typically have an orange hue but may show some mottling of gray pigment on
the surface. The osteoblastic activity of osteomas may encapsulate some osteoclastic
change. Feeder vessels may be observed exiting from the holes in the anterior
surface of the cancellous bone. This process can evolve to true choroidal
neovascular membranes, which may reveal the osteoma.9
Case 4 also developed CNV with a typical feeder vessel. However, marked attenuation
of sound by the tumor ultrasonographically, which is typical of choroidal
osteomas, could not be demonstrated in our study in the 3 patients so tested.
In our cases, RPE changes may have facilitated the occurrence of CNV.
In summary, to our knowledge, the peculiar leopard-spot pattern of the
retinal lesion observed in our 4 patients has not been previously reported.
Because the cause of these lesions remains unclear, we have designated them
as unilateral, idiopathic leopard-spot lesions of the RPE. Lack of histologic
analysis precludes more discussion on the possible etiologic nature of the
disorder, which seems to associate fibrosis and hyperplastic changes of the
RPE at the periphery of the lesion and thinning and atrophy of the RPE at
However, we could not rule out trauma as a possible cause of these lesions,
and they may also represent a similar end stage of different causes. The condition
is probably very rare, and its prognosis likely depends on whether CNV develops,
which was observed in 2 of our 4 patients.
Presented in part at the European Fluorescein Club Meeting, Creteil,
France, December 4, 1994, and at the Annual Meeting of the Macula Society,
Scottsdale, Ariz, February 28, 2001.
Corresponding author and reprints: Salomon Y. Cohen, MD, Centre Ophtalmologique
d'Imagerie et de Laser, 11 rue Antoine Bourdelle, 75015 Paris, France (e-mail: email@example.com).
Cohen SY, Massin P, Quentel G. Unilateral, Idiopathic Leopard-Spot Lesion of the Retinal Pigment Epithelium. Arch Ophthalmol. 2002;120(4):512-516. doi: