Transcript ECTOPIA LENTIS 1. Acquired 2. Isolated familial ectopia lentis 3. Associated with systemic syndromes • Marfan syndrome • Weill-Marchesani syndrome • Homocystinuria 4.

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
• Pectus excavatum
• High-arched palate
• Arachnodactyly
• Aortic dilatation, dissection
and regurgitation
• Mitral valve prolapse
Ocular features of Marfan syndrome
Lens
•
Upward subluxation
•
Zonule usually intact
Angle anomaly and
glaucoma
Retinal detachment
•
Lattice degeneration
Cornea plana
•
Axial myopia
Blue sclera
Autosomal recessive
Weill-Marchesani syndrome
Systemic features
Ocular features
• Short stature
• Short stubby fingers (brachydactyly)
• Mental handicap
• Microspherophakia
• Usually anterior lens subluxation
• Angle anomaly and glaucoma
Homocystinuria
• Autosomal recessive
• Defect in cystathio beta-synthase
Systemic features
Ocular features
•
•
•
•
Malar flush and fine, fair hair
Marfanoid habaitus
Increased platelet stickiness
Mental handicap
•
Downward lens subluxation
•
Disintegration of zonule
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