Retinitis Pigmentosa
Conditions
Keywords
Retinitis Pigmentosa, Retinal degeneration, Multimodal imaging, electroretinography, retinal pigment epithelium and outer retinal atrophy (RORA)
Brief summary
The objective of this study is to discover biomarkers that demonstrate a correlation between the severity of retinitis pigmentosa (RP) and the thickness of the retinal pigment epithelium (RPE). These biomarkers will serve as prognostic indicators for various kinds of retinitis pigmentosa. The objective of this study is to find biomarkers that establish a correlation between the severity of retinitis pigmentosa and the thickness of the retinal pigment epithelium (RPE), which can serve as a prognostic indicator for Retinitis Pigmentosa.
Detailed description
After a genetic confirmation of RP and classification, the patients will undergo a comprehensive ophthalmological examination that includes the following tests: slit-lamp anterior segment, visual acuity direct and indirect ophthalmoscopy, intraocular pressure, and family history.In order to evaluate the potential role of RPE in the advancement of RP, HD-OCT and OCT angiography images of the outer retina using OCT devices will be performed. Analysis of high-resolution images captured with an ultrawidefield system using a Zeiss Clarus device in order to determine the condition of the peripheral retina.Finally, Flicker Electroretinogram (fERG) performed on the central retina (macula), to assess the central macular function within an 18° field of view. This assessment involved measuring the response of the macula to a flickering stimulus with a frequency of 41 Hz, which is commonly done in routine clinical practice.
Interventions
DIAGNOSTIC_TESTOCT, OCT angiography, flicker ERG and Ultra Wide Field retinography and autofluorescence.
the best visual acuity will be evaluated using decimal tables and then converted to logMar. Ocular fundus examination will be evaluated following pharmacological mydriasis with Tropicamide 1%. OCT will be essential to quantify the central macular thickness and the thickness of the RPE. OCT angiography is a non-invasive method to assess the presence or absence of neovascular membrane and macular flow density. Color and ultra wide field autofluorescence images of the retina will be performed with Zeiss Clarus retinography to search for signs that identify different types of RP and predict their activity and evolution. Electroretinogram flicker (ERG) is a common measure of cone pathway function that is used to study the normal visual system and that of patients with inherited and acquired retinal dysfunction Measurements will be performed using an electrophysiological recording system (CSO). Intermittent stimuli are presented using a Ganzfeld dome (CSO).
Sponsors
Fondazione Policlinico Universitario Agostino Gemelli IRCCS
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patients who are able to read and sign informed consent * Patients with Retinitis pigmentosa confirmed by genetic test. * Patients older than or equal to 18 years of age
Exclusion criteria
* Other retinal diseases such as macular hole, retinal detachment, macular neovascularization. * Corneal surgery in the last 12 months * Glaucoma with pressure above 25 mmHg in the last three months. * Best Correct Visual Acuity below 1/10 in at least one eye.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Retinal pigment epithelium changes in Retinitis Pigmentosa. | 14 months | Retinal pigment epithelium extent measured with OCT calliper (micrometers). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Retinitis Pigmentosa biomarkers | 14 months | Measurement of retinal pigment epithelium extension by oct calliper (micrometers) in different forms of retinitis pigmentosa. Measurement of superficial and deep retinal vascularisation in the different forms of retinitis pigmentosa. |
Countries
Italy
Contacts
Primary ContactStanislao Rizzo, MD, Prof
Backup ContactValentina Cestrone
Outcome results
None listed