NCT02023086

Clinical Investigation on the Blood Oxygenation at the Optic Nerve Head in Fabry Patients

Clinical Investigation on the Blood Oxygenation at the Optic Nerve Head in Relation With Visual Field Loss in Fabry Patients

• Status: Completed
• Phase: —
• Study type: Observational
• Enrollment: 8 (actual)
• Sponsor: Université de Montréal · Academic / Other
• Sex: All
• Age: 18 Years
• Healthy volunteers: Accepted

Summary

This study aimed to evaluate blood oxygenation at the optic nerve head in relation with visual field losses observed in many Fabry patients. Data collected allowed to evaluate if there is a link between these two entities. Study lasted 2 years during which a limited number of Fabry patients had been compared to a control group to confirm any relationship between blood flow and field losses, and to see if these results varied over time. HYPOTHESIS 1\. Fabry patients will present significant differences in visual fields compared to control 2 There will be variability of the visual field defects on the long term but not on the short term 3 Blood oxygenation will be higher for Fabry patients 4 Blood volume at the optic nerve head will be the same for both groups.

Detailed description

The first subjects who meet the following criteria will be enrolled in the study. Inclusion criteria (Fabry group ): * Aged over 18 years old * Being diagnosed with Fabry disease * 3 subjects will be under enzyme replacement treatment for the treatment of Fabry disease. * 3 subjects will not receive enzyme replacement treatment * Is fit to give legal consent. * Is available for a period of 2 years Inclusion criteria (CONTROL group ): * Matched for age and sex with group A - 6 participants * Being healthy, with no known chronic systemic disease nor acute disease at the moment of the recruitment * Is fit to give legal consent. * Is available for a period of 2 years Exclusion criteria (Both groups): * Presents with an active pathological ocular condition * Presence of an abnormal optic nerve (congenital or acquired) * Usage of topical ocular drug(s) at the time of selection * To have known allergy to topical diagnostic drugs used in this study * Usage of systemic medication with known effect on the visual field

Conditions

• Fabry Disease

Interventions

Type	Name	Description
PROCEDURE	Contrast sensitivity measurement	Contrast sensitivity test is made with targets alternating black and white lines. The larger the lines are, easier it is to see them. Targets are distributed in 5 rows, each oriented at a different angle. Contrast sensitivity level is determined by the smallest target seen by the participant, line by line. This test is done under dim lightning with the habitual visual correction worn.
PROCEDURE	Slit Lamp assessment and intra-ocular pressure measurement	Slit lamp is used to assess the presence of significant lens opacities known as Fabry cataracts. In fact, these opacities can induced a bias on the contrast sensitivity measurement and can increase the symptoms felt by the patient. A grade 2 (CFDI grading chart) of lens opacities is considered clinically significant and this will become an exclusion criteria for the following of the study. Intra-ocular pressure will be measured with a non contact tonometer (air pulsed) (Ocular Response Analyser, Reichert Instrumentation.)
PROCEDURE	Visual field testing	Visual field will be assessed with an automated perimeter (Humphrey, Texas) using a threshold strategy. This implies that the subject has to identify not only if he perceives the visual stimulus but the minimal level of this stimulus (in decibels), to be seen, is also recorded. Visual field will be tested up to 30 degrees from the central point of fixation. In order to measure immediate variability of the visual testing among Fabry subjects, this test will be done at the beginning of the testing session (in the morning) and another time 1h00 later.
PROCEDURE	Oxygen flow at the optic nerve head measurement	Pupils will be dilated with 1 drop of 1% tropicamide. The procedure is similar to the one used for taking a photo of the retina. Each measurement session represents a continuous recording of 20 simultaneous functions of reflectometry from the optic nerve area during 10 seconds. During all recording sessions, the systemic arterial oxygen saturation will be also monitored at the right index finger using a pulse oximeter (Escort M10, Invivo, Orlando, USA). Arterial blood pressure will be also monitored before and after the testing using a manual sphygmomanometer.
DRUG	Tropicamide	Used to dilate patient's pupil during testing
DEVICE	OSOME	The OSOME system is the only technology able to perform on line and real time capillaries blood oxygenation measurement in the eye. The system operates with 1200 discreet wavelengths between 400 nm to 700 nm with 500 millisecond integration times. It is not invasive, functioning just like a fundus camera.

Timeline

• Start date: 2014-08-01
• Primary completion: 2016-09-01
• Completion: 2016-09-01
• First posted: 2013-12-30
• Last updated: 2024-09-19
• Results posted: 2024-09-19

Locations

1 site across 1 country: Canada