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 lentisTrauma

• Buphthalmos• Megalocornea

Anterior uveal tumours Degenerate eye

Stretched zonules

Isolated familial ectopia lentisAutosomal recessive

Pupil may be normal Pupil may be displaced in opposite direction (ectopia lentis et pupillae)

Autosomal dominantSystemic features of Marfan syndrome

• Limb-trunk disproportion • Arachnodactyly

• Pectus excavatum• High-arched palate

• Aortic dilatation, dissection and regurgitation• Mitral valve prolapse

Ocular features of Marfan syndromeLens

• Upward subluxation • Zonule usually intact

Retinal detachment

• Axial myopia

Blue scleraCornea planaAngle anomaly and glaucoma

• Lattice degeneration

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

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

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