This is a Prospective Observational study. The aim of the study is to understand the underlying photoreceptor, retinal pigment epithelium or retinal vascular aberrations in inherited and acquired retinal disorders. The study would use adaptive optics (AO) technology to assist in-vivo visualization of these retinal structures and ascertain changes from normal. Further, by using the AO imaging in patients before and after treatments, this study aims to better understand the effect of various interventions and develop AO as an outcome measure in various retinal disorders.
The investigators plan to recruit approximately 200 participants (approximately 175 study subjects and 25 Control group) having an inherited or acquired retinal disease. The study participants will be screened based on Inclusion and exclusion criteria. Eligible study and control participants will be consented prior to conducting any study related procedures. For the Data Collection, patient information including previous eye examination, past medical history and family history will be obtained from hospital charts. Patients will be asked to return for a follow-up visit at 6-months and at 1 year. The Objectives of the study include: 1. To test AO imaging in patients with well characterized genetic and non-genetic diseases in an attempt to - To better understand the underlying photoreceptor, retinal pigment epithelium or retinal vascular aberrations - To utilize the AO imaging in patients before and after treatments to better understand the effect of the intervention and develop AO as an outcome measure in various retinal disorders 2. To test AO in healthy controls to serve as internal controls Patients with inherited or acquired retinal disease who satisfy the inclusion criteria and those who consent for the study will be screened for the study. Study participants and participants from the cohort group will then have a 6 month or annual follow-up visit as per the study protocol. The primary outcome measures will be the quantify cone photoreceptors (density and spacing), retinal pigment epithelium density and retinal blood vascular flow in retinal disorders and compare it with controls. This will be calculated using software algorithms incorporated within AO machine (rtx1). Patient data will be compared with age-matched control data. The secondary outcome measure would be to ascertain if using rtx1, the rate of progression of retinal disease or treatment effect can be quantified. To accomplish this, areas that were imaged at baseline will be reimaged at the follow up visits. Since the rtx1 machine has the capability to identify exact retinal location between the visits, these follow up images will be compared to baseline images to determine rate of disease progression or effectiveness of treatment.
Study Type
OBSERVATIONAL
Enrollment
200
The rtx1 is a non-invasive device functions without making contact with the eye. The fundus of the patient's eye is illuminated with the IR light emitted from the illumination optical system. The device is comprised of an optoelectronic sensor (OES) that measures the optical defects, software that calculates the necessary corrections and a deformable mirror (DM) that constantly adapts its shape to restore the image's clarity. The digital camera, which is built into the instrument, receives the images and then the images are recorded in the computer hard disk. The AO image software registers and averages the captured image series in order to reduce noise and produce a final enhanced image. The rtx1 integrates AO technology in a flood illumination imaging system and enables visualizing the retina with a high transverse optical resolution of 250 line-pairs per millimeter.
The Hospital for Sick Children
Toronto, Ontario, Canada
RECRUITINGTo measure the change in Quantification of cone receptors
The primary outcome measures will be to measure the change in the quantification of cone photoreceptors (density and spacing).This will be calculated using software algorithms incorporated within AO machine (rtx1).
Time frame: Primary outcome will be measured at Baseline, 6-months ,1 year. The patient data will be compared to the control data.
To measure the change in Quantification of the retinal pigment epithelium density
The primary outcome measure will be to measure the change in quantification of the retinal pigment epithelium density. This will be calculated using software algorithms incorporated within AO machine (rtx1).
Time frame: Primary outcomes will be measured at Baseline, 6-months ,1 year. The patient data will be compared to the control data.
To measure the change in Quantification of retinal blood vascular flow in retinal disorders
The primary outcome measures the wall-to-lumen ratio (WLR) and the vascular wall cross-sectional area (WSCA) of retinal arterioles.
Time frame: Primary outcomes will be measured at Baseline, 6-months ,1 year. The patient data will be compared to the control data.
To measure the change and rate of progression of retinal disease
The secondary outcome measure would be to ascertain if using rtx1, the rate of progression of retinal disease or treatment effect can be quantified. To accomplish this, areas that were imaged at baseline will be reimaged at the follow up visits. Since the rtx1 machine has the capability to identify exact retinal location between the visits, these follow up images will be compared to baseline images to determine rate of disease progression or effectiveness of treatment.
Time frame: Secondary outcomes will be measured at follow-up visits. They will be compared to Baseline visits and measured at 6-months interval and a year
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