Ординатура / Офтальмология / Английские материалы / Illustrated Tutorials in Ophthalmology Kanski, Bolton 2001
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Gyrate atrophy
Cause - deficiency of ornithine keto-acid aminotransferase Inheritance - autosomal recessive
Presents - first decade with axial myopia and nyctalopia Prognosis - usually good VA until late
ERG - severely reduced
Progression
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Mid-peripheral, circular patches |
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Central and peripheral spread |
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of chorioretinal atrophy |
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Late retinal vascular attenuation |
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Enlargement and confluence |
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Fovea spared until late |
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HEREDITARY VITREORETINAL
DEGENERATIONS
1.Stickler syndrome
2.Congenital retinoschisis
3.Favre-Goldmann syndrome
4.Familial exudative vitreoretinopathy
Stickler syndrome
Inheritance - dominant
Presents - firstst decade
Prognosis - RD in 30%
Vitreous |
Retina |
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Empty central cavity |
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Radial lattice-like degeneration |
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Membranes extending into cavity |
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RPE hyperplasia |
Congenital retinoschisis
Inheritance - X-linked
Presents - first decade with maculopathy
Prognosis - poor (maculopathy, vitreous haemorrhage) ERG - decreased b-wave
Maculopathy (100%) |
Retinoschisis (50%) |
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‘Bicycle-wheel’ striae |
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Extremely thin inner layer |
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Eventually atrophic |
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Round inner layer defects |
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