July 2018
Volume 59, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2018
Spatial Characteristics in Retinal Dysfunction of ABCA4-associated Retinal Disorder: East Asia Stargardt Disease West China Studies
Author Affiliations & Notes
    Southweat Hospital, Chongqing, China
    National Hospital Organization, Laboratory of Visual Physiology, Division of Vision Research, National Institute of Sensory Organs, Tokyo, Japan
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Investigative Ophthalmology & Visual Science July 2018, Vol.59, 3144. doi:
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      XIAO LIU; Spatial Characteristics in Retinal Dysfunction of ABCA4-associated Retinal Disorder: East Asia Stargardt Disease West China Studies. Invest. Ophthalmol. Vis. Sci. 2018;59(9):3144.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose : Prognostic values of generalized functional phenotypes are well-established in ABCA4-associated retinal disorder. However, the spatial characteristics of retinal dysfunction are not fully known. The purpose of this study is to determine spatial characteristics of retinal dysfunction in West Chinese patients with ABCA4-associated retinal disorder in comparison with the generalized functional phenotypes.

Methods : Twenty-three patients with ABCA4 mutations who underwent full ophthalmic examinations were recruited. Full-field electroretinogram (ffERG) and multifocal electroretinogram (mfERG) were recorded incorporating the ISCEV standards. Patients were classified into 3 ffERG groups based on the previously established grouping: Group 1 - dysfunction confined to the macula; Group 2 - macular and generalized cone system dysfunction; and Group 3 - macular and both generalized cone and rod system dysfunction. Decline rate for P1 amplitude of mfERG was calculated via comparing to the normal control group. In order to investigate the spatial characteristics, decline rate in the total recoded area and in the eccentric ring groups (1-2 rings, 3-4 rings, and 5-6 rings) was compared between ffERG groups.

Results : There were 5 patients in Group 1, 6 in Group 2, and 12 in Group 3. The total averaged amplitude decline rate of mfERG P1 responses were 57.59% in Group 1, 73.61% in Group 2 and 86.83% in Group 3, respectively. Mean decline rate in 1-2 rings, 3-4 rings and 5-6 rings were 80.03%, 60.08%, 32.66% in Group 1, 73.27%, 77.58%, 69.98% in Group 2 and 85.98%, 87.53%, 86.99% in Group 3. There were significant differences between ffERG groups in terms of spatial trends of eccentric decline rate.

Conclusions : The spatial range of retinal dysfunction differed based on the ffERG groups. The mutual relations between groups of generalized dysfunction and spatial range of retinal dysfunction was confirmed, which indicates the predominant dysfunction in the central cone expands to the peripheral rod photoreceptors in ABCA4-associaed retinal disorder.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.


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