Unilateral Retinal Pigment Epithelium Dysgenesis May Be a Bilateral

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Figure. Central serous chorioretinopathy in myopic eyes. A, Color photographs of the affected eyes of cases 1, 2, and 3 (from left to right). There are granular retinal pigment epithelial changes in the maculas of all 3 eyes and an absence of funduscopic features of myopic degeneration. B, Fundus autofluorescence imaging of the same eyes. Macular hyperautofluorescence and hypoautofluorescence changes are consistent with central serous chorioretinopathy. C, Enhanced-depth imaging spectral-domain optical coherence tomography of the same eyes demonstrates subretinal fluid. In each case, subfoveal choroidal thickness is high for the degree of myopia. Correspondence: Dr Freund, Vitreous Retina Macula (FAF) imaging.1 Associated epiretinal fibrosis, retinal Consultants of New York, 460 Park Ave, Fifth Floor, New detachment, or choroidal neovascularization can lead York, NY 10022 ([email protected]). to vision loss. We report a case of RPE dysgenesis in Financial Disclosure: None reported. which the patient had classic findings in only 1 eye but Funding/Support: This work was supported by The Macula distinct, stellate RPE abnormalities in both eyes indica- Foundation, Inc, and Stichting Wetenschappelijk Onder- tive of a bilateral, asymmetric disease. To our knowl- zoek Oogziekenhuis Rotterdam, Rotterdamse Blinden- edge, bilateral RPE dysgenesis has not been previously belangen, Stichting Blindenhulp, Gelderse Blinden Stich- described. ting, Landelijke Stichting voor Blinden (Dr Yzer).

1. Yannuzzi LA. Central serous chorioretinopathy. In: Yannuzzi LA, ed. Laser Report of a Case. A 35-year-old woman without signifi- Photocoagulation of the Macula. Philadelphia, PA: JB Lippincott Co; 1989:4. cant medical history had a 6-year history of photopsias 2. Imamura Y, Fujiwara T, Margolis R, Spaide RF. Enhanced depth imaging optical coherence tomography of the choroid in central serous chorioretinopathy. and dimmer vision in her right eye only. The patient’s Retina. 2009;29(10):1469-1473. visual acuity was 20/25 OD and 20/20 OS. Anterior seg- 3. Fujiwara T, Imamura Y, Margolis R, Slakter JS, Spaide RF. Enhanced depth ment examination findings were normal in both eyes. Fun- imaging optical coherence tomography of the choroid in highly myopic eyes. Am J Ophthalmol. 2009;148(3):445-450. dus examination of the right eye revealed a large, scal- 4. Margolis R, Spaide RF. A pilot study of enhanced depth imaging optical co- loped area of reticular RPE hyperplasia continuous with herence tomography of the choroid in normal eyes. Am J Ophthalmol. 2009; 147(5):811-815. the optic nerve. Within the lesion, there was mottled atrophy of the RPE (Figure 1A and B). Fundus examination findings of the left eye were normal. Humphrey visual field testing demonstrated an en- Unilateral Retinal Pigment Epithelium larged blind spot in the right eye and normal results in Dysgenesis May Be a Bilateral Disease the left eye. Fluorescein angiography revealed patchy hyperfluorescence and hypofluorescence with an irregular nilateral retinal pigment epithelium (RPE) dys- margin of hyperfluorescence (Figure 1C and D). Imaging genesis is a rare entity with a poorly docu- with FAF demonstrated a hypoautofluorescent lesion with U mented etiology, pathogenesis, and prognosis. a distinctive, reticular border that was inverted relative It has a unique scalloped appearance on fluorescein an- to fluorescein angiography. There was mild hyperauto- giography that is inverted on fundus autofluorescence fluorescence at the edge of the lesion. Interestingly, there

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Figure 1. Fundus photographs, fluorescein angiograms, and fundus autofluorescence images. A, Fundus examination of the right eye shows a lesion with an appearance typical of unilateral retinal pigment epithelium dysgenesis. B, The left eye appears grossly normal on fundus examination. C, Fluorescein angiography of the right eye demonstrates a lesion with a hyperfluorescent irregular margin (arrow). D, Fluorescein angiography findings of the left eye are normal. E, Fundus autofluorescence imaging of the right eye highlights the distinctive appearance of the lesion on the right, with scattered multifocal areas of retinal pigment epithelium loss. Arrow indicates inversion of fluorescence compared with fluorescein angiography. F, In the left eye, the multifocal stellate lesions not visible on clinical examination are now appreciated on fundus autofluorescence imaging (arrows).

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I I Figure 2. Optical coherence tomography. A, Optical coherence tomography through the right eye demonstrates outer retinal thinning with associated attenuation of the inner segment–outer segment junction with a corrugated appearance to the superficial aspect of the inner retina. B, Optical coherence tomography through the left eye shows normal findings in comparison. I indicates inferior; N, nasal; S, superior; and T, temporal.

were also bilateral, multifocal, stellate areas of hypoau- edge illustrating this for RPE dysgenesis, which perhaps tofluorescence that were not appreciated clinically in either should no longer be termed unilateral. eye (Figure 1E and F). Optical coherence tomography through the lesion in Jennifer Renz, MD the right eye demonstrated outer retinal thinning with Jordana G. Fein, MD, MS associated attenuation of the inner segment–outer seg- Robin Vora, MD ment junction (Figure 2). Full-field electroretinogra- Harold Woodcome Jr, MD phy findings were normal in both eyes. Elias Reichel, MD Jay Duker, MD Comment. The first report of unilateral RPE dysgenesis Author Affiliations: Department of Ophthalmology, New was published in 2002.2 In this report, 3 young men and England Eye Center, Tufts Medical Center, Boston, Mas- 1 woman aged 16 to 34 years were noted to have a round sachusetts (Drs Renz, Fein, Vora, Reichel, and Duker); leopard-spot lesion contiguous with the optic nerve. In and Department of Ophthalmology, Brown University, 2009, the typical characteristics of unilateral RPE dys- Providence, Rhode Island (Dr Woodcome). genesis were described in a set of 9 affected patients, 6 Correspondence: Dr Reichel, Department of Ophthal- males and 3 females aged 14 to 42 years.1 The margin of mology, Tufts University School of Medicine, 800 Wash- the lesion is pathognomonic, with a scalloped reticular ington St, PO Box 450, Boston, MA 02111 (ereichel fringe of mild fibrosis and atrophy with inverted FAF @tuftsmedicalcenter.org). imaging relative to fluorescein angiography, and is iden- Financial Disclosure: None reported. tical to the lesion in our patient. The differential diagnosis of this lesion includes 1. Cohen SY, Fung AE, Tadayoni R, et al. Unilateral retinal pigment epithelium acute zonal occult outer retinopathy. However, acute dysgenesis. Am J Ophthalmol. 2009;148(6):914-919, e2. 2. Cohen SY, Massin P, Quentel G. Unilateral, idiopathic leopard-spot lesion of zonal occult outer retinopathy typically has a smooth the retinal pigment epithelium. Arch Ophthalmol. 2002;120(4):512-516. curvilinear border on autofluorescence3 and the electro- 3. Fujiwara T, Imamura Y, Giovinazzo VJ, Spaide RF. Fundus autofluorescence and optical coherence tomographic findings in acute zonal occult outer retinographic findings are abnormal. Alternative differ- retinopathy. Retina. 2010;30(8):1206-1216. ential diagnoses include traumatic retinal pigment epi- 4. Shields CL, Thangappan A, Hartzell K, Valente P, Pirondini C, Shields JA. theliopathy and combined hamartoma of the retina and Combined hamartoma of the retina and retinal pigment epithelium in 77 con- 4 secutive patients: visual outcome based on macular vs extramacular tumor RPE. location. Ophthalmology. 2008;115(12):2246-2252, e3. This case is unique in that it shows bilateral loss of the RPE in a multifocal, scattered, stellate fashion, best appreciated with FAF imaging. To our knowledge, this Levofloxacin-Associated Panuveitis is the first reported case of unilateral RPE dysgenesis with With Chorioretinal Lesions abnormal findings on FAF imaging in the fellow eye. In the previously reported series of 9 patients by Cohen et rug-induced uveitis is a rare complication of al,1 only 3 cases were evaluated with FAF imaging and many commonly prescribed medications, in- no evidence of bilateral disease was seen. However, as cluding bisphosphonates, sulfonamides, mul- D ␤ 1 most patients in the case series did not have FAF imaging tiple vaccines, and topical -blockers. Recently, a uveitis- performed, some of these unilateral cases may have had like syndrome with iris transillumination defects and subclinical bilateral disease. pupillary mydriasis associated with oral moxifloxacin use The etiology of this condition remains elusive. It may has been described.2 Fluoroquinolones are an increas- be an RPE dysgenesis or dystrophy, or it could reflect pre- ingly recognized cause of bilateral uveitis,3 although the vious inflammatory, infectious, or autoimmune insult to visual significance is often minimal. Herein, we de- the RPE. It is intuitive that a dysgenesis should ulti- scribe a visually disabling but reversible manifestation mately be bilateral, and this is the first report to our knowl- of levofloxacin-associated panuveitis.

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