Unilateral, Idiopathic Leopard-Spot Lesion of the Retinal Pigment Epithelium FREE

Several conditions have been described as having a retinal leopard-spot pattern.^1- 6 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).

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.

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.

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 lesion.

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 recollection.

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 the center.

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: sycohen@club-internet.fr).