26ectopia lentis
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ECTOPIA LENTIS
1. Acquired
2. Isolated familial ectopia lentis
3. Associated with systemic syndromes• Marfan syndrome
• Weill-Marchesani syndrome
• Homocystinuria
4. Treatment options
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Acquired ectopia lentisTrauma
• Buphthalmos• Megalocornea
Anterior uveal tumours Degenerate eye
Stretched zonules
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Isolated familial ectopia lentisAutosomal recessive
Pupil may be normal Pupil may be displaced in opposite direction (ectopia lentis et pupillae)
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Autosomal dominantSystemic features of Marfan syndrome
• Limb-trunk disproportion • Arachnodactyly
• Pectus excavatum
• High-arched palate
• Aortic dilatation, dissection and regurgitation• Mitral valve prolapse
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Ocular features of Marfan syndromeLens
• Upward subluxation • Zonule usually intact
Retinal detachment
• Axial myopia
Blue scleraCornea planaAngle anomaly and glaucoma
• Lattice degeneration
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Autosomal recessiveWeill-Marchesani syndrome
Systemic features
• Short stature
Ocular features
• Short stubby fingers (brachydactyly)
• Mental handicap
• Microspherophakia
• Angle anomaly and glaucoma
• Usually anterior lens subluxation
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Homocystinuria• Autosomal recessive• Defect in cystathio beta-synthase
Systemic features
• Malar flush and fine, fair hair• Marfanoid habaitus• Increased platelet stickiness• Mental handicap
Ocular features
• Downward lens subluxation
• Disintegration of zonule
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Treatment Options for Ectopia Lentis
• For induced astigmatism
1. Spectacle correction
• For aphakic portion
• Associated cataract
3. Surgical removal
• Lens-induced glaucoma
2. Nd:YAG laser zonulysis to displace lens out of visual axis
• Endothelial touch
• When other methods are inappropriate