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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
Acquired ectopia lentis
Trauma Stretched zonules

• Buphthalmos
• Megalocornea
Anterior uveal tumours Degenerate eye
Isolated familial ectopia lentis
Autosomal recessive

Pupil may be normal Pupil may be displaced in opposite


direction (ectopia lentis et pupillae)
Autosomal dominant
Systemic features of Marfan syndrome

• Limb-trunk disproportion • Arachnodactyly

• Pectus excavatum • Aortic dilatation, dissection


and regurgitation
• High-arched palate • Mitral valve prolapse
Ocular features of Marfan syndrome
Lens Retinal detachment

• Upward subluxation • Lattice degeneration • Axial myopia


• Zonule usually intact

Angle anomaly and Cornea plana Blue sclera


glaucoma
Autosomal recessive
Weill-Marchesani syndrome
Systemic features Ocular features

• Short stature • Microspherophakia


• Usually anterior lens subluxation
• Short stubby fingers (brachydactyly)
• Mental handicap • Angle anomaly and glaucoma
Homocystinuria
• Autosomal recessive
• Defect in cystathio beta-synthase
Systemic features Ocular features

• Malar flush and fine, fair hair • Downward lens subluxation


• Marfanoid habaitus • Disintegration of zonule
• Increased platelet stickiness
• Mental handicap
Treatment Options for Ectopia Lentis
1. Spectacle correction
• For induced astigmatism
• For aphakic portion

2. Nd:YAG laser zonulysis to displace lens out of visual axis

3. Surgical removal
• Associated cataract
• Lens-induced glaucoma
• Endothelial touch
• When other methods are inappropriate

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