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Retinoschisis
Retinoschisis is a splitting of the neurosensory retina into inner and outer layers, causing visual field defects. Senile retinoschisis occurs in 5% of people over 20 and presents as bilateral, peripheral retinal elevations that may progress circumferentially. Juvenile retinoschisis is an X-linked condition causing macular schisis and peripheral retinoschisis from birth, leading to progressive vision loss over decades. Both types are generally asymptomatic aside from visual field defects but may develop complications like retinal detachment, vitreous hemorrhage, or neovascularization. Diagnosis involves OCT, FA, and ERG and management focuses on complications or calcium channel blockers for macular schisis
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Retinoschisis
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- 2. Definition • Separation orsplitting of the NSR into inner and outer layers with severing of neurones and complete loss of visual function in the affected area.
- 9. Senile • Prevalence – 5%of the population >20 years – Particularly in hypermetropics • Typical retinoschisis – Split occurs in the outer plexiform layer • Reticular retinoschisis – Split occurs at the level of RNFL
- 10. Symptoms • NO PhotopsiaNOR floaters • Visual field defect but rarely noticed • If any, result from VH or a progressive RD
- 11. Signs • Bilateral in50-80% • Early Extreme Inferotemporal periphery – exaggeration of microcystoid degeneration • Elevation is convex, smooth, thin and relatively immobile • May progress circumferentially until it has involved the entire periphery – Typical usually remains anterior to the equator – Reticular is more likely to spread posteriorly
- 14. • The surfaceof the inner layer may show sclerosis of blood vessels & ‘snowflakes’ (whitish remnants of Müller cell footplates) • Breaks may be present in one or both layers – Inner layer breaks are small and round – outer layer breaks are usually larger, with rolled edges & located behind the equator
- 15. • Distinction betweenthe typical and reticular types is difficult clinically, though the inner layer is thinner and tends to be more elevated in the latter; differentiation is based principally on behaviour, with complications much more common in the reticular form
- 16. Complications • RD israre – even in an eye with breaks in both layers the incidence is only around 1% – The detachment is almost always asymptomatic, infrequently progressive and rarely requires surgery • Posterior extension to involve the fovea – very rare • Vitreous haemorrhage is rare
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- 19. Juvenile • Bilateral maculopathy,with associated peripheral retinoschisis in 50% • Splitting occurs at RNFL • Inheritance is XLR • Poor prognosis • Due to progressive maculopathy; visual acuity deteriorates during the first two decades, but may remain reasonably stable until the fifth or sixth decades before further deterioration
- 22. Symptoms • Presentation – Betweenthe ages of 5-10 years • Reading difficulties • squint or nystagmus occurs in infancy – associated with advanced peripheral retinoschisis, often with VH • Carrier females are generally asymptomatic
- 23. • Foveal schisis –Spoke-like striae radiating from the foveola, associated with cystoid changes
- 24. • Whitish drusen-likedots and pigment variation may be seen • Peripheral schisis predominantly involves the inferotemporal quadrant – Does not extend – Inner layer may develop oval defects – Defects may coalesce, leaving only retinal blood vessels floating in the vitreous (‘vitreous veils’)
- 26. Complications • Vitreous andintra-schisis haemorrhage • Neovascularization • Subretinal exudation • Rhegmatogenous or tractional RD • Traumatic rupture of the foveal schisis
- 27. Investigations • OCT – Documentingmaculopathy progression • FAF – spoke-like patterns – central hypoautofluorescence with surrounding hyperautofluorescence
- 28. • FA – Mildwindow defects but NO leakage • ERG – Normal in eyes with isolated maculopathy – Selective decrease in amplitude of the b-wave on scotopic and photopic testing
- 30. Management • Small lesionsfound incidentally need annual follow-up • Progression towards the fovea – Retinopexy or surgical repair • Recurrent VH – PPV • In cases of Maculopathy – Topical or oral CAI – Reduce foveal thickness and improve visual acuity in some patients
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Editor's Notes
- #30 a-wave cones & rods B-wave bipolar & muller C-wave RPE