Charlotta ZetterströmStockholmSweden

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%Sulfite oxidase deficiency 1%Weill-Marchesani 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

Few zonulaes

Subluxation of the lens

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 after surgeryDilated before 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

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

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)