ECTOPIA 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 lentis
Trauma
Stretched zonules

• Buphthalmos

• Megalocornea

Anterior uveal tumours
Degenerate eye
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Isolated familial ectopia lentis
Autosomal recessive
Pupil may be normal
Pupil may be displaced in opposite direction
(ectopia lentis et pupillae)
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Autosomal dominant
Systemic features of Marfan syndrome

• Arachnodactyly

• Limb-trunk disproportion

• Aortic dilatation, dissection
• and regurgitation

• Pectus excavatum

• High-arched palate

• Mitral valve prolapse

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Ocular features of Marfan syndrome
Lens
Retinal detachment

• Axial myopia

• Lattice degeneration

• Upward subluxation

• Zonule usually intact

Angle anomaly and glaucoma
Blue sclera
Cornea plana
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Autosomal recessive
Weill-Marchesani syndrome
Systemic features
Ocular features

• Microspherophakia

• Short stature

• Usually anterior lens subluxation

• Short stubby fingers (brachydactyly)

• Angle anomaly and glaucoma

• Mental handicap

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Homocystinuria

• Autosomal recessive

• Defect in cystathio beta-synthase

Systemic features
Ocular features

• Downward lens subluxation

• Malar flush and fine, fair hair

• Marfanoid habaitus

• Disintegration of zonule

• Increased platelet stickiness

• Mental handicap

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Treatment Options for Ectopia Lentis
1. Spectacle correction

• For induced astigmatism

• For aphakic portion

2. NdYAG laser zonulysis to displace lens out
of visual axis
3. Surgical removal

• Associated cataract

• Lens-induced glaucoma

• Endothelial touch

• When other methods are inappropriate