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26 ectopia lentis
Ectopia lentis refers to displacement of the lens from its normal position. It can be acquired through trauma or tumors, or inherited through isolated familial forms or associated with systemic syndromes like Marfan syndrome, Weill-Marchesani syndrome, and Homocystinuria. Treatment options depend on the cause and symptoms but may include spectacle correction, laser zonulysis to displace the lens, or surgical removal of the lens.
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26 ectopia lentis
- 1. ECTOPIA LENTIS 1. Acquired 2.Isolated familial ectopia lentis 3. Associated with systemic syndromes • Marfan syndrome • Weill-Marchesani syndrome • Homocystinuria 4. Treatment options
- 2. Acquired ectopia lentis Trauma •Buphthalmos • Megalocornea Anterior uveal tumours Degenerate eye Stretched zonules
- 3. Isolated familial ectopialentis Autosomal recessive Pupil may be normal Pupil may be displaced in opposite direction (ectopia lentis et pupillae)
- 4. Autosomal dominant Systemic featuresof Marfan syndrome • Limb-trunk disproportion • Arachnodactyly • Pectus excavatum • High-arched palate • Aortic dilatation, dissection and regurgitation • Mitral valve prolapse
- 5. Ocular features ofMarfan syndrome Lens • Upward subluxation • Zonule usually intact Retinal detachment • Axial myopia Blue scleraCornea planaAngle anomaly and glaucoma • Lattice degeneration
- 6. Autosomal recessive Weill-Marchesani syndrome Systemicfeatures • Short stature Ocular features • Short stubby fingers (brachydactyly) • Mental handicap • Microspherophakia • Angle anomaly and glaucoma • Usually anterior lens subluxation
- 7. 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
- 8. Treatment Options forEctopia 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