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Surgery in ectopia lentis in children

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Presentation on theme: "Surgery in ectopia lentis in children"— Presentation transcript:

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)

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