Surgery in ectopia lentis in
children
Charlotta Zetterström
Stockholm
Sweden
Ectopia lentis - epidemology
Etiological classification possible in 70%
Marfan syndrome
Ectopia lentis et pupillae
Simple ectopia lentis
Homocysteinuria
Sulfite oxidase deficiency
Weill-Marchesani
68%
21%
8%
1%
1%
1%
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
Guitar player
(Marfan?) of Picasso
- Defect in
connective tissue
-Skeletal
abnormalities
-Cardiac
abnormalities
-Ocular
abnormalities
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
Subluxation of the lens
Upwards (most common)
Downwards
Subluxation of the lens
Few zonulaes
Intact zonulaes
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
Ectopia of pupil and lens in
different directions
Undilated pupil
Dilated pupil
Mios
Dilated before surgery Dilated after surgery
Cataract
Sphaerophakia with cataract
Lenscoloboma with cataract
Different surgical techniques in former days
Ancient Egypt
Incision with Graefe´s knife
Marfan syndrome with Sphaerophakia
Marfan Syndrom with dislocated lens
After surgery
-Bifocal glasses
-Occlusion ?
Visual acuity before and after surgery
1,2
POSTOP. BCVA
1
0,8
0,6
0,4
0,2
0
0
0,1
0,2
0,3
0,4
PREOP. BCVA
0,5
0,6
0,7
Complications after surgery
• Visual axis opacification
• Secondary suturing of the
IOL (degradation of the
Prolene suture)
• No serious complications
VAO surgery
Eight months old child with good fixation and
no squint, surgery??
Right eye
Left eye
Six months child with no fixation, surgery??
Right eye
Left eye
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)