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Clinicopathologic Reports, Case Reports, and Small Case Series
October 2007

Pseudoduplication of Fovea in a Human Eye

Arch Ophthalmol. 2007;125(10):1428-1430. doi:10.1001/archopht.125.10.1428

Duplication of the optic disc and fovea is known to occur in lower vertebrates.1,2 Although additional optic discs usually serve no purpose, bifoveate birds (eg, swallows) use the additional fovea for limited conjugate binocular movements.2 Doubling of the optic disc, true diastasis as well as coloboma, has been described in the human eye.1 We report the unusual clinical finding of 2 foveae in 1 eye of a patient.

Report of a Case

A 25-year-old man had experienced diminished vision in the right eye for several months. There was no history of ocular injury or inflammation. His perinatal and family histories were unremarkable. Best-corrected visual acuity was 20/80, N12 OD and 20/20, N6 OS, with low myopic correction. The anterior segment was unremarkable in each eye. Fundus examination of the right eye revealed macular pucker and peripheral telangiectasia with exudation, suggestive of Coats disease. Fundus examination of the left eye revealed 2 parafoveal halos with 2 foveal reflexes; the temporal reflex was slightly distorted (Figure, A). No other ocular structure exhibited duplication. Optical coherence tomography using a Stratus OCT 3 (Carl Zeiss Meditec, Dublin, California) supported the clinical appearance of foveal duplication: the temporal fovea demonstrated a similar slope of clivus and the recession of inner retinal layers as the central fovea. However, the recession was not complete; a thin layer of inner retinal neurons remained above the photoreceptor layer, the probable cause of blunted foveal reflex. Despite additional layers, the central fovea (thickness, 165 μm) and the accessory fovea (thickness, 160 μm) were similar in thickness, probably owing to less-elongated photoreceptors in the latter (Figure, B). Fundus fluorescein angiography demonstrated only 1 foveal avascular zone, corresponding to the central fovea. A nonspecific mottled hypofluorescence was observed in the area of the temporal fovea (Figure, C). Fundus fluorescein angiography of the right eye revealed leaking telangiectasia and avascular areas in the inferotemporal periphery. Orthoptic evaluation revealed no fixation abnormalities in the left eye; the patient fixed consistently with the central fovea. Automated perimetry using the central 10-2 threshold test protocol (model 720i, Humphrey Field Analyzer II; Carl Zeiss Meditec) showed a normal foveal threshold (31 dB) at the fixation point, with a typical gradient of reducing sensitivity toward the periphery; no anomalous increase in sensitivity was registered temporal to the fixation point (central fovea). The patient was advised, but declined, to undergo pars plana vitrectomy and endophotocoagulation in the right eye.

Figure.
Clinical, tomographic, and angiographic representation of pseudo-duplication of the fovea in a human eye. A, Fundus view of the left eye shows 2 parafoveal annular reflexes with 2 foveal reflexes; the temporal foveal reflex is distorted into a vertical streak. The satellite fovea is situated approximately 1 optic disc diameter temporal and slightly inferior to the horizontal plane of the central fovea. B, Horizontal (345°) optical coherence tomogram traversing through both the foveal depressions shows the similar topography of the central and satellite foveae. Immaturity of the temporal fovea is evidenced by a residual layer of inner retinal neurons and less-elongated photoreceptors. C, Midphase fundus fluorescein angiogram of the left eye shows a normal foveal avascular zone at the central fovea and nonspecific hypofluorescence in the area corresponding to the temporal fovea (arrowheads).

Clinical, tomographic, and angiographic representation of pseudo-duplication of the fovea in a human eye. A, Fundus view of the left eye shows 2 parafoveal annular reflexes with 2 foveal reflexes; the temporal foveal reflex is distorted into a vertical streak. The satellite fovea is situated approximately 1 optic disc diameter temporal and slightly inferior to the horizontal plane of the central fovea. B, Horizontal (345°) optical coherence tomogram traversing through both the foveal depressions shows the similar topography of the central and satellite foveae. Immaturity of the temporal fovea is evidenced by a residual layer of inner retinal neurons and less-elongated photoreceptors. C, Midphase fundus fluorescein angiogram of the left eye shows a normal foveal avascular zone at the central fovea and nonspecific hypofluorescence in the area corresponding to the temporal fovea (arrowheads).

Comment

It has recently been suggested that slow development of central retinal vasculature creates hypoxic stress in the inner retinal neurons, which adapt by thinning out into incipient foveal depressions at approximately the seventh month of gestation.3 Active centrifugal migration of neuronal cells further deepens the depression, and stretching of Muller cells condenses and elongates the foveal cones.4 The foveal avascular zone is created 3 to 4 weeks postnatally by the inhibition of centripetal migration of astrocytes, the templates for endothelial proliferation. Foveal maturation is complete by age 3 to 4 years.3 Although we cannot explain this foveal duplication, it is possible that a transient metabolic injury in the perinatal period resulted in an additional zone of hypoxic stress and that consequent focal thinning began to create an immature foveal architecture, aborted before vascular differentiation. Although the extra fovea in this patient was incidentally discovered and nonfunctional, we are unaware of any study documenting its presence in a human eye. The presence of Coats disease in the fellow eye was most likely a chance occurrence, unrelated to the foveal duplication.

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Article Information

Correspondence: Mr Shukla, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, 1 Anna Nagar, Madurai 625 020, Tamil Nadu, India (daksh66@gmail.com).

Author Contributions: Mr Shukla had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Financial Disclosure: None reported.

References
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Brown  GCTasman  WS Other anomalies of the optic disc. Congenital Anomalies of the Optic Disc. New York, NY Grune & Stratton1983;266- 269
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Tychsen  L Binocular vision. Hart  WM  Jred.Adler's Physiology of the Eye. 9th St Louis, MO Mosby-Year Book1992;841- 842
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Provis  JMPenfold  PLCornish  EESandercoe  TMMadigan  MC Anatomy and development of the macula: specialisation and the vulnerability to macular degeneration. Clin Exp Optom 2005;88 (5) 269- 281
PubMedArticle
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Hendrickson  AE Primate foveal development: a microcosm of current questions in neurobiology. Invest Ophthalmol Vis Sci 1994;35 (8) 3129- 3133
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