Cataract in

Children
Introduct
ion

Cataract in children : causes of

blindness /severe impairment of vision
the major preventable cause.

Congenital cataract = present at birth

Infantile cataract = present during

infancy

Incidence :
congenital cataract : 5% to 2% of
blindness in children !orld!ide.
infantile cataract : 2.2 and " cases per
#$.
Introduct
ion

#% and "%.%% of all blindness in

children is caused by infantile cataract

&revalence of infantile cataract '(.)* : " +

, visually signi-cant cataract per #$
live birth.

Cataract may be unilateral or bilateral

Cataract surgery in children is to create

the best conditions for the development
of binocular vision.

management of pediatric cataract : very

comple. 'intra ocular lens 'I/0* use$ I/0
po!er calculations$ technical problem
during surgery$ post operative
complications*
Cataract morphology

1he opacity may involve anatomical parts

of the lens 'nucleus$ corte.$
anterior/posterior capsules*.

1he opacities may very in si2e$ shape and

colour.

3natomical part of the lens involved

'nuclear$ cortical$ subcapsular*

1he morphology of congenital cataract

may often give the ophthalmologist a
clue as the possible etiology.

1able #. cataract morphology and 4no!n

etiologies
Cataract Known cause
Cataract involving the whole
lens
1otal cataract 5o!n$ metabolic$ rubella$ sporadic
syndromes
Congenital morgagnian cataract 6ot common in infancy
5is47li4e and membranous cataracts 1rauma$ rubella$ 8allermann7)trei9$
&8&:$ aniridia$ 0o!e$ ruptured
anterior$ lenticonus
Central cataract
0amellar or 2onular cataracts 3utosomal dominant$ galactosemia$
hypoglycemia
Central pulverulent cataracts 3utosomal dominant
;3nt egg< cataracts 7
6uclear cataracts 3utosomal dominant$ rubella$ =7
lin4ed$ galactosemia

1able #. cataract morphology and 4no!n

etiologies Cataract Known cause
Central cataract
/il drop cataracts 7
Cortical cataract 3utosomal dominant
5is47li4e and membranous cataracts 1rauma$ rubella$ 8allermann7)trei9$
&8&:$ aniridia$ 0o!e$ ruptured
anterior$ lenticonus
Cerulean$ >oriform$ or coronary
cataract
3utosomal dominant
Anterior cataracts
3nterior polar cataracts 7
5ot7li4e 3utosomal dominant$ aniridia$
retinoblastoma$ &ierre7?obin
3nterior pyramidal 7
3nterior pla@ue7li4e 7

1able #. cataract morphology and 4no!n

etiologies Cataract Known cause
Anterior cataracts
3nterior subcapsular cataracts (veitis$ trauma$ irradiation$ atopic
s4in$ 3lport
3nterior lenticonus )poradic$ =7lin4ed$hyperglycinuria$
microcornea$ 5uaneAs syndrome
Posterior cataract
BittendorfAs dot 7
&osterior lenticonus Cataract forms as capsule bo!s and
lens -bers are distorted$ =7lin4ed
and 3utosomal dominant
&osterior cortical cataracts 3utosomal dominant
&osterior subcapsular cataracts Byotonic dystrophies$ 1urnerAs$
CabryAs 6CB
&unctuate lens opacities 5o!n$ 0o!e carriers$ 6ance78oran
carrier$ CabryAs disease

1able #. cataract morphology and 4no!n

etiologies Cataract Known cause
Posterior cataract
)utural cataracts
Coralliform or crystalline cataracts 3utosomal dominant
Dedge7shaped cataracts Conradi$ )tic4ler$ 6C2$ Cabry$
1risomy #"
&ersistent hyperplastic primary
vitreous '&8&:*
Cibrovascular remnants of tunica
vasculosa lentis !hich fans out into
membrane to nourish the
embryonic lens
Etiology

&ossible to ascertain the etiology: good

history$ morphology of the cataract$
identifying associated eye problems$
systemic abnormalities.

1he majority of bilateral congenital or

infantile cataract not associated !ith a
syndrome have no identi-able cause.

Fenetic mutation is li4ely the most

common cause$ often autosomal
dominant although it can be =7
lin4ed or autosomal recessive.
Etiology

)ystemic associations include metabolic

disorders 'galactosemia$ Dilson
disease$ hypocalcemia and diabetes*

Cataract may be a part of the number of

syndrome$ the most common being
trisomy 2#.

Intra uterine infections 'rubella$ herpes

simple.$ to.oplasmosis$ varicella and
syphilis*

In contrast$ most unilateral cataracts are

not inherited/associated !ith a systemic
disease$ un4no!n etiology
Etiology

(nilateral cataract are usually the result

of local dysgenesis and may be
associated !ith other ocular dysgenesis
'&C:*$ posterior lenticonus/lentiglobus*.

1rauma is a 4no!n cause of pediatric

cataracts. If there is no 4no!n history
of trauma$ investigation must be
considered in children !ho present !ith
other signs suggestive of child abuse.
Etiology
Bilateral
cataracts
Idiopathic (60%)
8ereditary '"%* 3utosomal dominant$
3utosomal recessive
=7lin4ed
Betabolic disorders Falactosemia$ Falacto4inase
de-ciency
8ypocalcemia$
8ypoglycemia
Bannosidosis$ Canconi7Gic4el
syndrome
Intra uterine infection ?ubella$ :aricella$
1o.oplasmosis
8erpes simple.$ )yphilis
3ssociated ocular
abnormalities
Bicrophthalmia$ 3niridia
&ersistent hyperplastic
primary vitreous '&8&:*
?etinopathy of prematurity
&eterAs anomaly$ Corneal
guttata
Etiology
Bilateral
cataracts
Chromosomal
anormalities
!risomy "# ($own
syndrome)
!risomy #% (Patau&s
syndrome)
!risomy #' ((dward
syndrome)
) chromosome monosomy
(!urner&s syndrome)
!ranslocations (Cri du chat
syndrome* !ranslocation
"+#,* !ranslocation %+,)
1rauma ?adiation$ 0aser
photocoagulation
6on7accidental$ 3ccidental
5rug induced Corticosteroids$ 3ntibiotics
/ther abnormalities
and syndromes
?enal disease 0o!e syndrome$ 3lportAs
syndrome
6ephrotic syndrome
Cardiac disease 8ypertropic cardiomyopathies
Central nervous system
abnormalities
Hell!eger syndrome
Barinesco7)jogren syndrome
Infantile neuronal ceroid7
lipofuscinosis
Etiology
Bilateral
cataracts
-ther anormalities
and syndromes
)4eletal diseases Bico syndrome$ Conradi
syndrome
Deill7Barchesani syndrome
)yndactyly$ polydactyly
or digital abnormalities
Gardet7Giedl syndrome
?ubenstein71aybi syndrome
5ermatological Coc4ayne syndrome$
Ectodermal dysplasia
Craniofacial syndromes Congenital cataract facial
dysmorphism neuropathy
syndrome or Cerebro7oculo7
facio7s4eletal 'C/C)*
5ental anomalies 6ance78oran syndrome
/culo7Cacio7Cardio75ental
syndrome '/CC5*
Bitochondrial
abnormalities
Comple. I de-ciency
Etiology
.nilateral
cataracts
Bas4ed bilateral
cataract
Idiopathic 'I%%*
3ssociated ocular
abnormalities '#%*
&8&:$ 3nterior segment
dysgenesis
&osterior lenticonus$ 1umor
'?etinoblastoma*
?etinopathy of prematurity
1rauma 6on7accidental$ 3ccidental
0aser photocoagulation
Infectious ?ubella '#%*
5iagnosis

8istory : family$ prenatal and pregnancy

history

)igns :
7 opacity in the lens 'morphological and

location77777ma4ing the diagnosis*.
7 may be a part of another disease
or syndrome.
7 may be accompanied by additional
noticeable
ocular abnormalities 'coloboma of
the iris
microcornea$ megalocornea$
aniridia$ and
2onular dehiscence*.
5iagnosis

)ymptoms :
7 often asymptomatic.
7 lac4 of reaction to light.
7 strabismus.
7 photophobia only in bright light.
7 sensory nystagmus.

0aboratory test:
7 1/?C8 titers$ :5?0$ serum calcium
and
phosphorus levels.
5i9erential 5iagnosis

leu4ocoria or !hite pupil :

7 ?etinoblastoma.
7 &8&: '&C:*.
7 ?etinopathy of &rematurity '?/&*.
7 Chorioretinal colobomas.
7 1o.ocariasis.
7 Coats disease.
7 :itreus hemorrhage.
7 /ther retinal tumors.
Banageme
nt

Feneral treatment:
7 6ot all pediatric cataract re@uire
surgery.
7 small$ partial or paracentral
'observation*.

)urgery:
7 Celt to be visually signi-cant.
7 Critical for visual development.
7 !ithin the -rs t!o months of live.
7 before one month of age 'ris4 of
apha4ic
glaucoma is increased*
7 bilateral cataracts 'surgery on both
eyes*
Complications

)econdary /paci-cationJ

)econdary FlaucomaJ

EndophthalmitisJ

?etinal 5etachment

/ther '!ound lea4$ iris/vitreous to the

!ound$ retinal hemorrhages can occur$
intraocular pressure lo!$ iris capture of
the I/0 optic$ cystoids macular edema*

3mblyopia$ strabismus and nystagmus.

&rognosis

highly variable and very diKcult to

predict.

permanent visual impairment777777

irreversible amblyopia and permanent
nystagmus.

(nilateral cataract less favorable than

bilateral cataract.

3 child !ith a unilateral cataract is also at

greater ris4 for anisometropia.