Roll No.

: 131, 33

CONGENITAL CATARACT

Scenario
A 2 weeks old neonate was brought to eye

OPD by his parents with the complaint of

bilaterally white pupil.
a. What is your differential diagnosis?
b. Classify congenital cataract.
c. What are the different causes of
congenital cataract?
d. How will you manage him?

Definition
Congenital opacity of the crystalline lens.

Cloudiness in the lens of the eye that is

present at, or develops shortly after birth.

Differential Diagnosis
Congenital Cataract
Retinoblastoma
Coloboma of Choroid
Coats Disease
Retrolental Fibroplasia
Norries Disease
Persistant Hyperplastic

Primary Vitreous

Anatomy of the Lens

Types of Cataract
Anterior polar cataracts(Pyramidal)
Lamellar (Zonular) Cataracts Most

Common
Posterior polar cataracts
Nuclear cataracts
Cerulean cataracts (Blue Dot )
Coronary Cataract
Posterior Subcapsular Cataract
Total/ Complete Cataract

Posterior Polar

Anterior Polar

Cerulean / Blue-Dot Cataract

Etiology
BILATERAL
Idiopathic (60%)
Hereditary (30%) Trisomy e.g. Downs,
Edwards
Intrauterine Infections - TORCH
Associated with ocular disorders
Tumor
Metabolic Disorders Hypoglycemia,
Hypocalcemia, Galactosemia
Muscular Disorders -Myotonic Dystophy
Maternal drug ingestion.
Malnutrition.

UNILATERAL
Idiopathic (80%)
Intrauterine infection - Rubella
Ocular abnormalities (10%) Lenticonus,
Anterior Segment Dysgenesis
Trauma (9%)

Epidemiology
Incidence is about 1 in
2000, and approximately one-third of
congenital cataractsare familial, onethird are associated with asyndrome,
and one-third are isolated.

Diagnosis
History:
Duration
Family history of congenital cataracts
Visual status: Ambulation in familiar & unfamiliar
surroundings
Behavioural pattern & school performance.
Birth history:
History & Degree of consanguinity
History of maternal infection during 1st trimester
Gestational age & birth weight
Birth trauma
Supplemental O2 therapy in perinatal period.
Developmental milestone

Diagnosis
Ocular Examination:
Measurement of visual acuity
Pupil examination
External eye examination including lids and lashes
Measurement of intraocular pressure
Slit lamp examination:
associated congenital anomalies of iris & lens
-type of cataract - iridodenesis/ phacodonesis.
Dilated examination of the cataract and fundus.
Biometry if the child is old enough to cooperate. If
not this may need to bedone under anaesthesia.

Management
1.Medical Care
Medical therapy is directed at the prevention of
amblyopia.
2. Surgical Care
Aspiration of the Lens matter is the treatment goal.
Cataract surgery is the treatment of choice and should
be performed when patients are younger than 17
weeks.
Extracapsular cataract extraction with primary posterior
capsulectomy and anterior vitrectomy is the procedure
of choice (via limbal or pars plana approach).
The resulting Aphakic Eye can be then managed via 3
options.

Contact Lens- If no IOL is implanted,

contact lenses are given as early as

possible to prevent stimulus deprivation
amblyopia. Frequent retinoscopy should be
performed to decide the power of CL and
an overcorrection of +2 to +3D is
mandatory. Silicon lenses or soft hydrogels
are well tolerated.

Spectacles- In some children with bilateral

aphakia spectacles are better tolerated

than contact lenses. In addition a
secondary strabismus may be manipulated
by prismatic effect of spectacles. Bifocal
glasses should be prescribed when the
child is about to start school.

Intra-Occular-Lens (IOL)
Preffered option
Traditional IOLs are monofocal, meaning

they offer vision at one distance only.

NewMultifocalandaccommodating IOLs
suchs as Crysta Lens offer the possibility of
seeing well at more than one distance.