TY - JOUR
T1 - Electroretinogram recording for infants and children under anesthesia to achieve optimal dark adaptation and international standards
AU - Lam, Byron L.
AU - Mendoza-Santiestaban, Carlos
AU - Gonzalez, Alex
AU - Rowaan, Cornelis
AU - Liu, Mu
AU - Martin, Joanne
AU - Gayer, Steven
AU - Figueredo, Osmany Gil
AU - Parel, Jean Marie
N1 - Funding Information:
This paper is supported in part by the James V. Bastek, M.D. Hereditary Retinal Disease Research Program, Bascom Palmer Eye Institute, University of Miami, FL, USA; NIH Center Core Grant P30EY014801; Research to Prevent Blindness Unrestricted Award and Career Development Awards; Florida Lions Eye Bank and the Beauty of Sight Foundation, Miami, FL, USA; and Henri and Flore Lesieur Foundation.
Publisher Copyright:
© 2020 JoVE Journal of Visualized Experiments.
PY - 2020/9
Y1 - 2020/9
N2 - Electroretinogram (ERG) is the only clinical objective test available to assess retinal function. Full-field ERG (ffERG) measures the panretinal rod and cone photoreceptor function as well as inner retinal function and is an important measure in the diagnosis and management of inherited retinal diseases as well as inflammatory, toxic, and nutritional retinopathies. Adhering to international standards and maintaining retinal dark adaptation are critical to acquire valid and reliable dark-adapted (scotopic) and light-adapted (photopic) ffERG responses. Performing ffERG in infants and children is challenging and often requires general anesthesia in the operating room. However, maintaining retinal dark adaptation in the operating room is becoming increasingly difficult given the numerous light sources from anesthesiology monitoring systems and other equipment. A practical and widely applicable method for ffERG testing is described in the operating room that optimizes retinal dark adaptation. The method reduces operating room time by dark-adapting the patient before general anesthesiology is instituted. The operating room is modified for dark adaptation and any remaining light source in the darkened operating room is minimized with the use of a modified portable foldable darkroom that encloses the patient’s head and the ERG examiner during ffERG scotopic recordings. The simple method adheres to ffERG international standards and provides valid reliable scotopic and photopic ffERG recordings that are critical to assess objective retinal function in this young age group where subjective assessment of visual function such as visual acuity and visual fields are not possible. Furthermore, the ffERG is the gold standard clinical test in detecting early onset inherited retinal diseases including Leber congenital amaurosis where approved gene therapy has become available. In sedated conditions, very low amplitude ffERG signals can be detected due to minimal orbicularis muscle activity.
AB - Electroretinogram (ERG) is the only clinical objective test available to assess retinal function. Full-field ERG (ffERG) measures the panretinal rod and cone photoreceptor function as well as inner retinal function and is an important measure in the diagnosis and management of inherited retinal diseases as well as inflammatory, toxic, and nutritional retinopathies. Adhering to international standards and maintaining retinal dark adaptation are critical to acquire valid and reliable dark-adapted (scotopic) and light-adapted (photopic) ffERG responses. Performing ffERG in infants and children is challenging and often requires general anesthesia in the operating room. However, maintaining retinal dark adaptation in the operating room is becoming increasingly difficult given the numerous light sources from anesthesiology monitoring systems and other equipment. A practical and widely applicable method for ffERG testing is described in the operating room that optimizes retinal dark adaptation. The method reduces operating room time by dark-adapting the patient before general anesthesiology is instituted. The operating room is modified for dark adaptation and any remaining light source in the darkened operating room is minimized with the use of a modified portable foldable darkroom that encloses the patient’s head and the ERG examiner during ffERG scotopic recordings. The simple method adheres to ffERG international standards and provides valid reliable scotopic and photopic ffERG recordings that are critical to assess objective retinal function in this young age group where subjective assessment of visual function such as visual acuity and visual fields are not possible. Furthermore, the ffERG is the gold standard clinical test in detecting early onset inherited retinal diseases including Leber congenital amaurosis where approved gene therapy has become available. In sedated conditions, very low amplitude ffERG signals can be detected due to minimal orbicularis muscle activity.
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U2 - 10.3791/61734
DO - 10.3791/61734
M3 - Article
C2 - 32955497
AN - SCOPUS:85090637168
VL - 2020
SP - 1
EP - 19
JO - Journal of visualized experiments : JoVE
JF - Journal of visualized experiments : JoVE
SN - 1940-087X
IS - 163
M1 - e61734
ER -