Cataract in Children

Dr. Rusdianto, SpM(K)

Department of Ophthalmology
Sriwijaya University
Moehammad Hoesin General Hospital
Palembang 2014

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 20% of
blindness in children worldwide.
infantile cataract : 2.2 and 3 cases per
10,000.

Introduct
ion
10% and 38.8% of all blindness in
children is caused by infantile cataract
Prevalence of infantile cataract (U.S) : 3
4 visually significant cataract per 10,000
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
complex (intra ocular lens (IOL) use, IOL
power calculations, technical problem

Cataract morphology

The opacity may involve anatomical parts

of the lens (nucleus, cortex,
anterior/posterior capsules).
The opacities may very in size, shape and
colour.
Anatomical part of the lens involved
(nuclear, cortical, subcapsular)
The morphology of congenital cataract
may often give the ophthalmologist a
clue as the possible etiology.

 Table 1. cataract morphology and known

etiologies
Cataract
Known cause
Cataract involving the whole
lens
Total cataract

Down, metabolic, rubella, sporadic

syndromes

Congenital morgagnian cataract

Not common in infancy

Disk-like and membranous cataracts Trauma, rubella, Hallermann-Streiff,

PHPV, aniridia, Lowe, ruptured
anterior, lenticonus
Central cataract
Lamellar or zonular cataracts

Autosomal dominant, galactosemia,

hypoglycemia

Central pulverulent cataracts

Autosomal dominant

Ant egg cataracts

Nuclear cataracts

Autosomal dominant, rubella, Xlinked, galactosemia

 Table 1. cataract morphology and known

etiologies
Cataract
Known cause
Central cataract
Oil drop cataracts

Cortical cataract

Autosomal dominant

Disk-like and membranous cataracts Trauma, rubella, Hallermann-Streiff,

PHPV, aniridia, Lowe, ruptured
anterior, lenticonus
Cerulean, floriform, or coronary
cataract

Autosomal dominant

Anterior cataracts
Anterior polar cataracts

Dot-like

Autosomal dominant, aniridia,

retinoblastoma, Pierre-Robin

Anterior pyramidal

Anterior plaque-like

 Table 1. cataract morphology and known

etiologies
Cataract
Known cause
Anterior cataracts
Anterior subcapsular cataracts

Uveitis, trauma, irradiation, atopic

skin, Alport

Anterior lenticonus

Sporadic, X-linked,hyperglycinuria,
microcornea, Duanes syndrome

Posterior cataract
Mittendorfs dot

Posterior lenticonus

Cataract forms as capsule bows and

lens fibers are distorted, X-linked
and Autosomal dominant

Posterior cortical cataracts

Autosomal dominant

Posterior subcapsular cataracts

Myotonic dystrophies, Turners,

Fabrys NFM

Punctuate lens opacities

Down, Lowe carriers, Nance-Horan

carrier, Fabrys disease

 Table 1. cataract morphology and known

etiologies
Cataract
Known cause
Posterior cataract
Sutural cataracts
Coralliform or crystalline cataracts

Autosomal dominant

Wedge-shaped cataracts

Conradi, Stickler, NF2, Fabry,

Trisomy 13

Persistent hyperplastic primary

vitreous (PHPV)

Fibrovascular remnants of tunica

vasculosa lentis which fans out into
membrane to nourish the
embryonic lens

Etiology

Possible to ascertain the etiology: good

history, morphology of the cataract,
identifying associated eye problems,
systemic abnormalities.
The majority of bilateral congenital or
infantile cataract not associated with a
syndrome have no identifiable cause.
Genetic mutation is likely the most
common cause, often autosomal
dominant although it can be
Xlinked or autosomal recessive.

Etiology

Systemic associations include metabolic

disorders (galactosemia, Wilson
disease, hypocalcemia and diabetes)
Cataract may be a part of the number of
syndrome, the most common being
trisomy 21.
Intra uterine infections (rubella, herpes
simplex, toxoplasmosis, varicella and
syphilis)
In contrast, most unilateral cataracts are
not inherited/associated with a systemic
disease, unknown etiology

Etiology

Unilateral cataract are usually the result

of local dysgenesis and may be
associated with other ocular dysgenesis
(PFV), posterior lenticonus/lentiglobus).
Trauma is a known cause of pediatric
cataracts.
If there is no known history
of trauma, investigation must be
considered in children who present with
other signs suggestive of child abuse.

Etiology
Idiopathic (60%)
Hereditary (30%)

Autosomal dominant,
Autosomal recessive
X-linked

Metabolic disorders

Galactosemia, Galactokinase
deficiency
Hypocalcemia,
Hypoglycemia
Mannosidosis, Fanconi-Bickel
syndrome

Intra uterine infection

Rubella, Varicella,
Toxoplasmosis
Herpes simplex, Syphilis

Associated ocular
abnormalities

Microphthalmia, Aniridia
Persistent hyperplastic
primary vitreous (PHPV)
Retinopathy of prematurity
Peters anomaly, Corneal
guttata

Bilateral
cataracts

Etiology

Bilateral
cataracts

Chromosomal
abnormalities

Trisomy 21 (Down
syndrome)
Trisomy 13 (Pataus
syndrome)
Trisomy 18 (Edward
syndrome)
X chromosome monosomy
(Turners syndrome)
Translocations (Cri du chat
syndrome, Translocation
2;14, Translocation 3;4)

Trauma

Radiation, Laser
photocoagulation
Non-accidental, Accidental

Drug induced

Corticosteroids, Antibiotics

Other abnormalities
and syndromes
Renal disease

Lowe syndrome, Alports

syndrome
Nephrotic syndrome

Cardiac disease

Hypertropic cardiomyopathies

Etiology
Other abnormalities
and syndromes
Skeletal diseases

Mico syndrome, Conradi

syndrome
Weill-Marchesani syndrome

Syndactyly, polydactyly Bardet-Biedl syndrome

or digital abnormalities Rubenstein-Taybi syndrome
Bilateral
cataracts

Dermatological

Cockayne syndrome,
Ectodermal dysplasia

Craniofacial syndromes

Congenital cataract facial

dysmorphism neuropathy
syndrome or Cerebro-oculofacio-skeletal (COFS)

Dental anomalies

Nance-Horan syndrome
Oculo-Facio-Cardio-Dental
syndrome (OFCD)

Mitochondrial
abnormalities

Complex I deficiency

Etiology
Masked bilateral
cataract
Idiopathic (98%)
Unilateral
cataracts

Associated ocular
abnormalities (10%)

PHPV, Anterior segment

dysgenesis
Posterior lenticonus, Tumor
(Retinoblastoma)
Retinopathy of prematurity

Trauma

Non-accidental, Accidental
Laser photocoagulation

Infectious

Rubella (10%)

Diagnosis

History : family, prenatal and pregnancy

history
Signs :
- opacity in the lens (morphological and
location-----making the diagnosis).
- may be a part of another disease
or syndrome.
- may be accompanied by additional
noticeable
ocular abnormalities (coloboma of

Diagnosis

Symptoms :
- often asymptomatic.
- lack of reaction to light.
- strabismus.
- photophobia only in bright light.
- sensory nystagmus.
Laboratory test:
- TORCH titers, VDRL, serum calcium
and
phosphorus levels.

Differential Diagnosis

leukocoria or white pupil :

- Retinoblastoma.
- PHPV (PFV).
- Retinopathy of Prematurity (ROP).
- Chorioretinal colobomas.
- Toxocariasis.
- Coats disease.
- Vitreus hemorrhage.
- Other retinal tumors.

Manageme
nt
General treatment:
- Not all pediatric cataract require
surgery.
- small, partial or paracentral
(observation).
Surgery:
- Felt to be visually significant.
- Critical for visual development.
- within the firs two months of live.
- before one month of age (risk of
aphakic

Complications

Secondary Opacification;
Secondary Glaucoma;
Endophthalmitis;
Retinal Detachment
Other (wound leak, iris/vitreous to the
wound, retinal hemorrhages can occur,
intraocular pressure low, iris capture of
the IOL optic, cystoids macular edema)
Amblyopia, strabismus and nystagmus.

Prognosis

highly variable and very difficult to

predict.
permanent visual impairment-----irreversible amblyopia and permanent
nystagmus.
Unilateral cataract less favorable than
bilateral cataract.
A child with a unilateral cataract is also at
greater risk for anisometropia.