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Brian Do, Jasmine Francis, Brian Marr, David Abramson, Scott Brodie; ERG as Indicator of Retinal Function in Persistent Fetal Vasculature. Invest. Ophthalmol. Vis. Sci. 2013;54(15):5127.
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Persistent fetal vasculature is a well-known cause of leukocoria in young children. Although much less worrisome than other causes of leukocoria with which it may be confused, PFV can be difficult to diagnose definitively. As there are no data delineating visual function in eyes with PFV, this study examines correlation between ERG results (as a proxy for visual function), and retinal involvement in PFV.
A retrospective chart review included 26 eyes of 13 patients who had undergone examination under anesthesia including ERG testing of both eyes after referral to the Memorial Sloan-Kettering Cancer Center. Inclusion criteria included a diagnosis of PFV in at least one eye, as well as ERG testing. Standard measures of ERG responses were determined using the Espion V6 software package from Diagnosys LLC; values included a and b-wave amplitudes under photopic single flash, 30 Hz Flicker, dark adapted rod-specific, dark-adapted red flash, and dark-adapted maximal flash conditions. Each eye was assigned a “retinal integrity score (RIS)” on a scale from 0 to 4. A retinal integrity score of zero represented total retinal detachment, while a score of four represented a fully attached retina, with minimal disruption due to PFV. Scores of 1, 2, and 3 indicated varying degrees of retinal detachment, dysplasia, and laser treatment, evident upon ophthalmoscopy and/or B-scan ultrasonography. Calculations were then performed to determine correlation between RIS and 30 Hz flicker waveform amplitudes.
Of 28 eyes, 6 were assigned a RIS of zero, 5 assigned RIS between 1 and 3 for varying retinal pathology, and 15 eyes assigned an RIS of 4 for normal retinal examination. ERG was without exception extinguished in all eyes assigned an RIS of zero. Mean 30-Hz flicker average amplitude in eyes assigned an RIS greater than zero was 108.65 μV. Correlation between RIS and averaged 30 Hz flicker amplitudes in all eyes was 0.62; in eyes with an RIS greater than zero, the correlation was 0.498. It should be noted that ERG amplitudes varied widely among eyes assigned an RIS of four, ranging anywhere from 42.98 to 184.65 μV in eyes with PFV.
We demonstrated that there is a significant correlation between visual function and overall retinal condition in eyes with PFV. Further, the wide range of ERG amplitudes in eyes with PFV and essentially intact retinas suggests significant retinal disruption in many of these eyes.
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