From Complejo Asistencial de Palencia (AG-F, MRS), Palencia; Complejo Hospitalario de Burgos (EP-SG), Burgos; IOBA Universidad de Valladolid (RMC-M), Valladolid; Hospital Universitario Rio Hortega (JG-A), Valladolid; and Hospital Clínico Universitario (JP-E), Valladolid, Spain.
Presented in part as a poster at Sociedad Española de Oftalmología, September 25–28, 2008, Sevilla, Spain.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to M. Rosa Sanabria, MD, PhD, Edificio IOBA - Campus Miguel Delibes, Camino de Belen S/N, 47011, Valladolid, Spain. E-mail: firstname.lastname@example.org
In 1965, Krill and Folk1 classified the fleck retina diseases. These conditions are characterized by widespread discrete yellow-white lesions of variable shape and size at the level of the retinal pigment epithelium (RPE). Krill and Folk’s classification according to severity was: fundus albipunctatus, fundus flavimaculatus, familial drusen, and fleck retina of Kandori. All of these conditions bring on, to a smaller or greater degree, a functional defect.
In 1980, Sabel Aish and Dajani reported a family with a funduscopic picture of flecked retina but with normal visual findings.2 These authors rebuilt Krill’s and Folk’s classification and added a new condition, the benign fleck retina, which theoretically has no functional defect.
We illustrate a new isolated case of benign fleck retina and report multifocal electroretinogram  (mfERG), microperimetry, fundus autofluorescence imaging, and optical coherence tomography (OCT) findings.
A 45-year-old woman was referred by her family physician to a routine eye examination without any specific ocular complaint. Visual acuities were 6/6 in both eyes without correction. Slit-lamp examination was normal. Funduscopy revealed multiple discrete yellow-white flecks in the RPE, affecting both eyes in a symmetrical pattern, and choroidal background pattern was obscured (Fig. 1). The lesions were distributed around the fundus, sparing the area around the optic disc, papillomacular bundle, and macula. The shape of the flecks was round or ovoid, adopting a cerebriform appearance. The flecks were smaller and sparser in the nasal retina. Color D-15 panel Farnsworth tests were normal in both eyes.
Figure 1. Right (A) and Left (B) Fundus Images Showing Symmetrical Yellow-White Flecks in the Retinal Pigment Epithelium Distributed Around the Fundus but Sparing the Area Around the Optic Disc, Papillomacular Bundle, and Macula. The Flecks Were Smaller and Sparser in the Nasal Retina.
Fundus fluorescein angiography (Zeiss FF 450 Plus IR; Carl Zeiss Meditec, Dublin, CA) revealed patches of hyperfluorescence in the upper and temporal quadrant of the posterior pole. Although some flecks did match the hyperfluorescent areas, most of them did not produce hyperfluorescence (Figs. 2A and 2B). Fundus autofluorescence imaging (Topcon TRC-50IX; Topcon, Tokyo, Japan) revealed increased autofluorescence of the flecks. However, fundus autofluorescence also showed some hypoautofluorescent spots that correlated well with the areas of hyperfluorescence observed on fluorescein angiography in the superotemporal arcade of both eyes (Figs. 2C and 2D).
Figure 2. (A and B) Fundus Fluorescein Angiography Revealed Fine, Irregular Hyperfluorescent Patches in the Upper and Temporal Quadrant of the Posterior Pole. Only a Few Hyperfluorescent Areas Correspond to the Flecks ([A] 37 Seconds After Injection; [B] 29 Seconds After Injection). (C and D) Fundus Autofluorescence Images Showing Increased Autofluorescence Co-Localizing with the Flecks. Most of the Hyperfluorescent Points on Fundus Fluorescein Angiography Show Hypoautofluorescence Suggesting Focal Points of Retinal Pigment Epithelium Atrophy.
Central and peripheral visual fields performed with the Humphrey Visual Field Analyzer (Carl Zeiss Meditec) were normal in both eyes. Microperimetry (MP-1 Microperimeter; Nidek, Aichi, Japan) demonstrated a mild visual field defect in the vicinity of the vascular arcades that seemed to be deeper in the upper temporal area where the hypoautofluorescent spots were located (Fig. 3).3
Figure 3. Microperimetry Demonstrated Mild Defect in the Vicinity of the Arcades.
An OCT scan (Stratus OCT; Carl Zeiss Meditec) passing through the flecks revealed irregularities in the thickness of the outer high reflectivity band with an overlying normal retina (Fig. 4). An OCT scan passing through the fovea was normal.
Figure 4. Optical Coherence Tomography 5-mm Line as Shown in Figure 1A. An Irregularity in the Thickness of the Outer High Reflectivity Band with an Overlying Normal Retina Can Be Seen.
The full-field flash-evoked electroretinogram (ERG), performed according to the standards of the International Society for Clinical Electrophysiology of Vision and recorded under photopic and scotopic conditions, dark adaptation tests, and Pattern ERG 50 (PERG) were all normal in both eyes (Fig. 5); mfERG responses were affected, showing low P1 amplitude responses within the isopters 17° to 20° and widespread reduction with relative sparing of the foveal area (Fig. 6). On electrooculogram, Arden ratios were 2.58 and 2.42 in the right and left eye, respectively.
Figure 5. Full-Field Electroretinogram (ERG) With: (1) Dark-Adapted 0.01 ERG (rod Response); (2) Dark-Adapted 3.0 ERG (combined Rod-Cone Response); (3) Dark-Adapted 3.0 Oscillatory Potentials; (4) Light-Adapted 3.0 ERG (cone Response); (5) Light-Adapted 3.0 Flicker (30 Hz Flicker); and (6) Pattern ERG. All of These Tested Responses Were Normal.
Figure 6. (A) Right Eye Multifocal Electroretinogram . (B) Left Eye Multifocal Electroretinogram  Showing Low P1 Amplitude Responses Within the Isopters 17° to 20° and Widespread Reduction with Relative Sparing of the Foveal Area.
There was no history of ocular illnesses in her family. Complete ocular examinations of her parents, brothers, sisters, and only daughter were normal.
We present a sporadic, rare case of benign fleck retina in a middle-aged woman. The OCT illustrated irregularities in the thickness of the outer high reflectivity band, which may correspond to the flecks and an overlying normal retina in exactly the same place where the abnormal deposits were observed.
After Sabel Aish and Dajani described a family with benign fleck retina,2 only two sporadic cases of benign fleck retina have been published: Isaacs et al.4 reported a case and Audo et al.5 recently described the fundus autofluorescence imaging of another case. Miyake and Harada6 described three patients in two families with similar fundus appearance but, because these patients had congenital stationary night blindness, Gass considered these cases as variants of albipunctate dystrophy.7 In the same way, the case described by Tsuchiya et al.8 could be better defined as albipunctate dystrophy rather than benign fleck retina, because the patient showed abnormal dark adaptation.
Benign fleck retina has been considered benign because no functional defect has been described in previous reports of the disease in comparison to other similar fleck retina diseases.7,9,10
In the current case, RPE did not appear to be functionally affected in general because Arden ratios were normal. Nevertheless, choroidal background pattern is obscured in the fundus photography and fluorescein angiography demonstrated hyperfluorescent zones in superior areas without correlation to the flecks. Moreover, fundus autofluorescence showed hypoautofluorescence in this same area, indicating local zones of absent RPE function.
The mfERG is useful in identifying functional deficits limited to the macula. The mfERG of the current case showed low P1 amplitude responses in the isopters 17° to 20°, although the standard full-field flash-evoked ERG was normal. That could be explained because the standard ERG requires approximately 30% of the retina to be dysfunctional before abnormalities can be detected.11 The size and shape of the abnormal P1 amplitude on mfERG show a high positive correlation with the delineated areas of mildly impaired retinal sensitivity on microperimetry. All of these observations would suggest local functional abnormalities of the retina near the superior arcade where fluorescein angiography and fundus autofluorescence abnormalities of the RPE are more obvious.5
The mfERG and microperimetry tests were able to detect a functional deficit not observed on full-field ERG, pattern ERG, or automated perimetry, demonstrating for the first time that benign fleck retina can bring on a functional defect.
- Krill AE, Folk MR. Retinitis punctata albescens: a functional evaluation of an unusual case. Am J Ophthalmol. 1962;53:450–455.
- Sabel Aish SF, Dajani B. Benign familial fleck retina. Br J Ophthalmol. 1980;64:652–659. doi:10.1136/bjo.64.9.652 [CrossRef]
- Midena E, Vujosevic S, Cavarzeran FMicroperimetry Study Group. Normal values for fundus perimetry with the microperimeter MP1. Ophthalmology. 2010;117:1571–1576. doi:10.1016/j.ophtha.2009.12.044 [CrossRef]
- Isaacs TW, McAllister IL, Wade MS. Benign fleck retina. Br J Ophthalmol. 1996;80:267–268. doi:10.1136/bjo.80.3.267 [CrossRef]
- Audo I, Tsang SH, Fu AD, Barnes JA, Holder GE, Moore AT. Autofluorescence imaging in a case of benign familial fleck retina. Arch Ophthalmol. 2007;125:714–715. doi:10.1001/archopht.125.5.714 [CrossRef]
- Miyake Y, Harada K. Familial fleck retina with night blindness. Ann Ophthalmol. 1982;14:836–841.
- Gass JDM. Stereoscopic Atlas of Macular Disease: Diagnosis and Treatment, 4th ed. St. Louis: Mosby; 1997:346–347, 350.
- Tsuchiya T, Kato M, Tomita N, et al. A case of sectorial benign flecked retina. Jpn J Ophthalmol. 2004;48:72–74. doi:10.1007/s10384-003-0002-6 [CrossRef]
- De Laey JJ. Flecked retina disorders. Bull Soc Belge Ophthalmol. 1993:249;11–22.
- Flynn MF, Bohnert D. Fundus albipunctatus and other flecked retina syndromes. J Am Optom Assoc. 1999;70:571–580.
- Jacobi PC, Miliczek K, Zrenner E. Experiences with the international standard for clinical electroretinography: normative values for clinical practice, interindividual and intraindividual variations and possible extensions. Doc Ophthalmol. 1993;85:95–114. doi:10.1007/BF01371126 [CrossRef]