Congenital cataract

Dr Ghada ELNady
Congenital cataract
 Opacification of the lens or its capsule .
 Occurs in 3- 10000 livebirths.
 2/3 of cases are bilatral.
 Unilateral cataracts are usually sporadic,

without a family history or systemic disease

and affected infants are usually otherwise
healthy.
types

Since birth (congenital).

Shortly after birth (infantile).
In early years of life up to 9y (juvenile).
Start intrauterine and increasing with age
called (developmental cataract).

Trumatic cataract.
Complicated cataract.
Etiology

1- hereditary :(AD) inheritance is the most

common etiological factor.
 TORCH INFECTION
2-maternal infection during pregnancy :
Rubella 40-60%
Mumps 10-22%
Hebatitis 16%
Toxoplasmosis 5%
Others as CMV,HSV,HIV and syphilis.
Rubella infection
 Usually total cataract,bilatral.
 Presented with leukocoria and choroiditis .

 The infection occurs during the 5th to 8th

weekof pregnancy,no protective lens capsule.

 Associated with other developmental

anomalies (cataract,open ductus arteiosus
and hearing loss) Gregg syndrome.
Toxoplasmosis

 Ophthalmic features of congenital

toxoplasmosis include:
cataract,chorioretinitis, microphthalmos and
optic atrophy.
CMV
Systemic features :
jaundice, hepatosplenomegaly, microcephaly
and intracranial calcification.

Ocular features : cataract,chorioretinitis,

microphthalmos, keratitis and optic atrophy.
 3-maternal drug ingestion during pregnancy
like steroides,alchol,smoking and drugs.
 4- maternal malnutrition:vit D ,ca deficieny.
 5- fetal hypoxia in cases of placental hge.
 6- metabolic disorders like galactosemia,DM,
lowe syndrome and wilsons disease
Galactosaemia

 Galactosaemia is (AR) condition characterized by:

impairment of galactose utilization caused by

absence of the enzyme galactose-1phosphate
uridyl transferase (GPUT).

 Unless galactose (milk and milk products) is

withheld from the diet, severe systemic
complications up to death may occur.
oil droplet opacity in galactoseamia
LOWE SYNDROME (oculocerebrorenal)
syndrome
 inborn error of amino acid metabolism
 Cataract,microphakia and congnital galucoma
 Cerebral and renal manifestations
 7- congenital cataract with genetic syndromes
eg: Down (Trisomy 21) .
 Systemic features

stunted growth,facial features,thyroid

dysfunction and cardiorespiratory disease
 Ocular features

Cataract (75%)
myopia
KCS
Squint
Edwards syndrome (trisomy 18)
Systemic features
Small head,low set ears
Cleft lip and palate
Fingers may be fused
defness
Cardiac and renal malformation
Ocular features
Cataract, ptosis, microphthalmos,corneal
opacity, uveal and disc coloboma and
vitreoretinal dysplasia
 8- congenital cataract associated with
idiopathic hypoparathyroid (tetany)
Morphological types of congenital
cataract :
 1- anterior polar
 2-posterior polar
 3-zonular (lamellar)
 4-cronary
 5-blue dots
 6- Sutural
 7- total
1- ant polar cataract
Ant sub cap epith proliferation
Rarely affects vision
2- post polar cataract
Markedly affect vision (near to the nodal point)
3- lamellar cataract(zonular)
The opacity affects zone or lamella of fibers.
4-cronary cataract
Club shaped opacities in the lens periphery
leaving central part clear.
5- blue dots cataract
Multiple small opaque dots scattered allover
the lens.
 6- sutural cataract :opacification of fetal
nucleus ,rarely affects vision.
Diagnosis

 family history and maternal history during

pregnancy
 Poor fixation
 Leukocoria
 Nystagmus
 squint
EX and IX:
 For unilataral cataract in healthy child an
extensive work up is not necessary.

 Slit lamp ex
 IOP
 Fundus ex or U/S
 In bilaltral cataract :
 Family history and ex

if there is a clear AD and the child is healthy

further evaluation is not necessary.

In cases without clear family history

Ped ex and lab ix is necessary (TORCH titers,
VDRL,serum calcium and phosporus levels).
 Urine analysis for reducing substance after
drinking milk (galactosaemia) and
chromatography for amino acids (Lowe
syndrome).
Mangement

 Surgery or leave
 Time of surgery
 Type of surgery (IOL)or not.
Surgery will depends on:

 V/A
 Density of cataract
 Morphology and location of cataract (the

more posterior and central the opacity the

more it affect vision).
 Associated ocular pathology.
surgery
 In unilateral cataract : at 4-6 weeks.
 In bilateral cataract :before the age of 3

months.
 Not before 1 month for fear of galucoma.

 Usu start with the worest eye followed by the

other eye after 1 week.
 1- irrigation/aspiration with posterior
capsulotomy and anterior vitrectomy

 2- parsplana lensectomy using vitrectomy

machine.
Correction of aphakia
 Cotact lense
 Glasses in bilatral casses.
 IOL by the age of 2 years

better inside the capsular bag to minimize

reaction and get best stability.
best type in children is acrylic type (least
reaction and least PCO).
COMPLICATIONS
 UVITIS
 Pco
 IOL decentration and optic capture
 endophthalamitis
DD of leukocoria
DD
 1-Congenital cataract
 2-retinoblastoma
 3-retinopathy of prematurity (ROP)
 4-Persistent fetal vasculature (PFV)
 5- coat’s disease
Retinoblastoma
ROP
PFV
COAT’S DISEASE