Disease Directory X-Linked Retinoschisis
Ophthalmological

X-Linked Retinoschisis

Also known as: XLRS, juvenile retinoschisis, RS1 deficiency

Prevalence

1 per 5,000–25,000 males

Onset

Early childhood (usually detected by school age)

Type

X-linked recessive

Gene

RS1

About X-Linked Retinoschisis

X-Linked Retinoschisis is a vitreoretinal dystrophy caused by mutations in the RS1 gene encoding retinoschisin, a protein secreted by photoreceptors that maintains the structural integrity of the retina through cell-to-cell adhesion. Loss of retinoschisin leads to splitting (schisis) of the retinal layers, most prominently in the fovea, resulting in reduced visual acuity that is typically identified in affected boys between three and ten years of age. The disease is stationary or slowly progressive in many patients, though complications such as vitreous haemorrhage and retinal detachment can cause acute vision loss.

Common Clinical Features

Reduced central visual acuity (typically 20/60 to 20/120) Foveal schisis visible on OCT as cystic spaces Spoke-wheel or stellate pattern at the fovea on fundus examination Peripheral schisis in approximately 50% of patients Electronegative ERG (markedly reduced b-wave relative to a-wave) Strabismus or nystagmus in some children Risk of vitreous haemorrhage Risk of retinal detachment

Clinical Trial Eligibility Tips

What to know before applying to X-Linked Retinoschisis trials.

Molecular confirmation of a pathogenic RS1 variant is required for gene therapy trials; testing of at-risk male relatives is recommended to identify additional eligible patients.

Foveal cyst architecture on OCT is used as both a diagnostic and monitoring biomarker; baseline and recent OCT scans should be available for screening visits.

Some trials exclude patients with prior retinal detachment surgery or vitrectomy; disclose full surgical history during screening.

Patient Resources

Patient Organization

Foundation Fighting Blindness

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Natural History Registry

My Retina Tracker

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Orphanet

European reference resource for rare diseases (ORPHA:792)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting X-Linked Retinoschisis trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for X-Linked Retinoschisis. Updated daily.

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