Congenital / Developmental

Cataract

Dr Sanjay Shrivastava
Professor of Ophthalmology
Gandhi medical College, Bhopal (M.P.)
 Classification of Cataract
1. Developmental
2. Age related (senile)
3. Cataract associated with ocular diseases
4. Cataract associated with systemic
diseases (pre-senile)
5. Traumatic Cataract
6. Drug induced cataract

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 Developmental Cataract
• When cataract is present at birth it is called
congenital.
• Types
1. Punctate: most common manifestation, in its
minute form it is of universal occurrence.
Multiple small opaque spots are seen scattered
all over the lens. Types:
a. Blue dot – appearing as tiny blue dots in
oblique illumination.

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 Punctate Cataract
b. Sutural – when opaque spots are
crowded in the Y sutures
c. Central Pulverulent Cataract –
Autosomal dominant inherited, non-
progressive, central spheroidal or
biconvex opacity consisting of fine white
powdery dots within embryonic or fetal
nucleus.

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 Developmental Cataract.. Types
2. Zonular Cataract: accounts for approximately
50% of all visually significant congenital
cataracts.
Etiology – May have a genetic origin with a
strong hereditary tendency of autosomal
dominant type. They may have an environmental
origin, due to malnutrition at late intra-uterine or
early infantile life and Vitamin D deficiency.
Patient may have Rickets and defective enamel
of permanent teeth.
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 Zonular Cataract
• Zone around embryonic nucleus, usually area of
fetal nucleus is affected and opacified. Its extent
depends on the duration of the inhibiting factor.
Sharply demarked opacity, area within and
around opacity is clear; with linear opacities
resembling spokes of a wheel (riders) running
towards equator.
• Such cataracts are usually bilateral formed just
before or shortly after birth, may be sufficient in
diameter to fill the pupillary area and vision is
affected.
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 Developmental Cataract- types
3. Fusiform Cataract – Spindle shaped,
axial or coralliform. There is antero-
posterior spindle shaped opacity
sometimes with offshoots giving an
appearance of coral.
Discoid Cataract is disc shaped opacity in
posterior cortex behind nucleus.

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 Developmental Cataract - Types
4. Nuclear Cataract: Due to inhibition at an early
stage, the central nucleus (embryonic nucleus)
remains opaque.
A progressive type of nuclear cataract is
observed in child when mother suffers from
rubella (German measles) during 2nd or 3rd
month of pregnancy.
Nucleus is necrotic, the whole lens becomes
opaque, with accompanying retinitis (salt and
pepper retinopathy) at the posterior pole.

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 Nuclear Cataract
• Other congenital deformities like,
congenital heart disease (PDA),
microphthalmos, micrencephaly, mental
retardation, deafness and dental
anomalies may be associated.
• If the lens matter remains in eye during
surgery, it is followed by a chronic
inflammatory endophthalmitis.

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 Nuclear Cataract
• Prevention of rubella by administration of MMR
vaccine or rubella vaccine to pre-pubertal girls or
women planning to have child may be
considered to prevent this condition.
• Medical termination of pregnancy may be
considered if evidence of rubella infection is
confirmed during 2nd or 3rd month of pregnancy
to prevent birth of a child with multiple congenital
anomalies.

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 Developmental Cataract - Types
5. Coronary Cataract: It is a developmental
cataract, similar to zonular cataract,
occurring around puberty. Situated in deep
layer of the cortex and the most superficial
layers of adolescent nucleus. Appears as
corona of club shaped opacities at
periphery, hidden by iris. Axial area and
periphery of lens remains clear. Vision is
usually not affected. These opacities are
non-progressive.
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 Developmental Cataract - Types
6. Anterior Capsular (polar) Cataract: may be due
to delayed development of anterior chamber
(congenital cataract) or due to contact of anterior
lens capsule and posterior surface of cornea due
to perforation of ulcer, as in case of ophthalmia
neonatorum.
Types – Anterior Polar, Pyramidal Cataract and
Reduplication cataract
Usually these opacities are non-progressive and
rarely interfere with vision.

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 Developmental Cataract – Types
7. Posterior Capsular (Polar) Cataract: Is
due to persistence of the posterior part of
vascular sheath of the lens. In minimal
degree it is common and usually is
insignificant.
Sometimes in cases of persistent hyaloid
artery, the lens is deeply invaded by
fibrous tissue and a total cataract forms.

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Etiology of Developmental Cataract
• Usually unknown
• Maternal (and infantile) malnutrition
• Maternal Viral (rubella) infection
• Placental Haemorrhage causing deficient
oxygenation
• Hypocalcaemia
• Chromosomal abnormality (Down syndrome)
• Metabolic disorders (galactosaemia)

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 Symptoms of Developmental
Cataract
Informant – usually parents
 History of white spot in pupillary area
 Child is usually brought with history of
diminution of vision / does not recognize
objects and parents
 Unsteady eyes
 Deviation of eye
 Associated symptoms of systemic
disease, if present
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 Signs
• Diminished vision (at times it is difficult to
establish in very young children)
• Lenticular opacity
• Nystagmus
• Deviation of eye, usually convergent
squint
• There may be other ocular and systemic
abnormalities in cases of rubella nuclear
cataract
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 Management of Developmental
Cataract
I. Investigations:
1. Detailed history
2. Detailed clinical examination- visual status,
intra-ocular tension, fundus examination, B
scan ultrasonography to exclude posterior
segment abnormality like growth/
retinoblastoma, A scan to determine axial
length of the eye, retinoscopy and cover test
to exclude squint.

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 Management of Developmental
Cataract
3. Laboratory investigations:
A. Blood Test
Blood glucose, calcium and phosphorus
RBC transferase and Galactokinase levels
TORCH test
Hepatitis B virus

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 Management of Developmental
Cataract
B. Urine analysis:
For reducing substance for
galactosaemia
For amino acids (to exclude Lowe
syndrome in suspected cases)

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 Management of Developmental
Cataract
B. Treatment
1. Timing of surgery
a. Bilateral Dense cataract – by 6
weeks
b. Bilateral partial – if vision is not
significantly affected, surgery may be
delayed up to the age of 2 years or up to
puberty
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 Management of Developmental
Cataract
c. Uniocular dense cataract- urgent
surgery within days
d. Partial Uniocular cataract- if vision is
not significantly affected, surgery may be
delayed up to the age of 2 years or up to
puberty

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 Management of Developmental
Cataract
2. Treatment options –
* No treatment if vision is not affected
significantly
* Mydriatics- if opacity is central and vision
improves with mydriatics
* In cases in Rubella Cataract operation may
be delayed till 1-2 years of age. But early
surgery may be indicated if cataract is total,
squint and nystagmus is developing.

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 Management of Developmental
Cataract
* Uniocular cataract – if vision is affected
then early surgery, preferably within first six
weeks of birth with immediate fitting of contact
lens.
* Fixation develops between 2-4 months of
age, therefore any cataract interfering with vision
should be dealt before this age, and the earliest
possible time is preferred
* Medical/ Paediatric fitness for anaesthesia
should be obtained.

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 Management of Developmental
Cataract
3. Operative procedure
a. Aspiration and irrigation (ECCE)
b. Lensectomy (Pars plana or anterior
route)
c. Aspiration and irrigation (ECCE) with
primary posterior capsulotomy with partial
anterior vitrectomy

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 Management of Developmental
Cataract
4. Post-operative visual rehabilitation:
a. Posterior Chamber IOL (PMMA or
acrylic polymer foldable lens) in patients
who are more than two years in age,
Uniocular cataract where contact lens
fitting is not possible/ practical.
b. Contact lens – after surgery for
uniocular cataract at very young age.
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 Management of Developmental
Cataract
c. Aphakic Spect- In bilateral cataract
operated cases below the age of two
years. These cases are implanted
posterior chamber IOL as secondary
procedure at later age.
d. Occlusion therapy for treatment of
amblyopia / prevention of amblyopia.

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 Post-operative complications
• Posterior capsular opacification
• Secondary membrane formation
• Proliferation of lens epithelium
(Sommerring ring)
• Glaucoma
• Retinal detachment

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