Charlotta Zetterström Stockholm Sweden
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Transcript of Charlotta Zetterström Stockholm Sweden
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Charlotta ZetterströmStockholmSweden
Surgery in ectopia lentis in children
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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%
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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
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Guitar player (Marfan?) of Picasso
- Defect in connective tissue
-Skeletal abnormalities
-Cardiac abnormalities
-Ocular abnormalities
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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
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Subluxation of the lens
Upwards (most common) Downwards
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Few zonulaes
Subluxation of the lens
Intact zonulaes
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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
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Ectopia of pupil and lens in different directions
Undilated pupil Dilated pupil
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Mios
Dilated after surgeryDilated before surgery
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Cataract
Sphaerophakia with cataract Lenscoloboma with cataract
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Different surgical techniques in former days
Ancient Egypt Incision with Graefe´s knife
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Marfan syndrome with Sphaerophakia
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Marfan Syndrom with dislocated lens
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After surgery
-Bifocal glasses
-Occlusion ?
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Visual acuity before and after surgery
0
0,2
0,4
0,6
0,8
1
1,2
0 0,1 0,2 0,3 0,4 0,5 0,6 0,7
PREOP. BCVA
PO
ST
OP
. BC
VA
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Complications after surgery
• Visual axis opacification
• Secondary suturing of the IOL (degradation of the Prolene suture)
• No serious complications
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VAO surgery
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Eight months old child with good fixation and no squint, surgery??
Right eye Left eye
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Six months child with no fixation, surgery??
Right eye Left eye
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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)