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Charlotta Zetterström Stockholm Sweden

Charlotta Zetterström Stockholm Sweden. Surgery in ectopia lentis in children. Ectopia lentis - epidemology. Etiological classification possible in 70% Marfan syndrome 68% Ectopia lentis et pupillae 21% Simple ectopia lentis 8% Homocysteinuria 1%

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Charlotta Zetterström Stockholm Sweden

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  1. Charlotta Zetterström Stockholm Sweden Surgery in ectopia lentis in children

  2. Ectopia lentis - epidemology Etiological classification possible in 70% Marfan syndrome 68% Ectopia lentis et pupillae 21% Simple ectopia lentis 8% Homocysteinuria 1% Sulfite oxidase deficiency 1% Weill-Marchesani 1%

  3. Marfan syndrome-genetics • Autosomally dominant heritage • 25% de-novo mutations • Mutation in chromosome 15 • Defect in fibrillin-1 (FBN1) • Loose connective tissue • Sclera, cornea, zonulae etc. affected • Incidence: 1-3 / 10 000 • Not all carry the mutation • Clinical diagnostic criteria (Ghent), including all organ systems

  4. Guitar player (Marfan?) of Picasso - Defect in connective tissue -Skeletal abnormalities -Cardiac abnormalities -Ocular abnormalities

  5. Ocular manifestation in Marfan syndrom Major criterion: • Subluxation of the lens (60%) Minor criteria: • Increased axial length • Abnormally flat cornea • Hypoplastic iris or ciliary muscle • Decreased miosis Major criterion or 2 minor criteria required for ocular involvement

  6. Subluxation of the lens Upwards (most common) Downwards

  7. Subluxation of the lens Few zonulaes Intact zonulaes

  8. Subluxation of the lens • ↓ Visual acuity (amblyopia) • Myopia (lens) • Astigmatism (lens) • Flat and thin cornea • Iris and pupil changes • Secondary glaucoma • Luxation of the lens to anterior chamber (children) or vitreous (old patient) • Cataract

  9. Ectopia of pupil and lens in different directions Undilated pupil Dilated pupil

  10. Mios Dilated after surgery Dilated before surgery

  11. Cataract Sphaerophakia with cataract Lenscoloboma with cataract

  12. Different surgical techniques in former days Ancient Egypt Incision with Graefe´s knife

  13. Marfan syndrome with Sphaerophakia

  14. Marfan Syndrom with dislocated lens

  15. After surgery -Bifocal glasses Occlusion ?

  16. Visual acuity before and after surgery

  17. Complications after surgery • Visual axis opacification • Secondary suturing of the IOL (degradation of the Prolene suture) • No serious complications

  18. VAO surgery

  19. Eight months old child with good fixation and no squint, surgery?? Right eye Left eye

  20. Six months child with no fixation, surgery?? Right eye Left eye

  21. Five years old child, surgery?? Right eye VA 0.4 (-5.0 -7.0 cyl 90) Left eye VA 0.5 (-4.5 -6.0 cyl 90)

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