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ECTOPIA LENTIS

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. Megalocorne a.

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ECTOPIA LENTIS

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  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 Stretched zonules • Buphthalmos • Megalocornea Anterior uveal tumours Degenerate eye

  3. Isolated familial ectopia lentis Autosomal recessive Pupil may be normal Pupil may be displaced in opposite direction (ectopia lentis et pupillae)

  4. Autosomal dominant Systemic features of Marfan syndrome • Arachnodactyly • Limb-trunk disproportion • Aortic dilatation, dissection • and regurgitation • Pectus excavatum • High-arched palate • Mitral valve prolapse

  5. Ocular features of Marfan syndrome Lens Retinal detachment • Lattice degeneration • Axial myopia • Upward subluxation • Zonule usually intact Angle anomaly and glaucoma Cornea plana Blue sclera

  6. 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

  7. 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

  8. Treatment Options for Ectopia Lentis 1. Spectacle correction • For induced astigmatism • For aphakic portion 2. Nd:YAG laser zonulysisto 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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