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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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