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

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 /
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
Intact zonulaes
Few 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 before surgery
Dilated after 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 ( cyl 90)
Left eye VA 0.5 ( cyl 90)