developmental cat
TRANSCRIPT
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Congenital / DevelopmentalCataract
Copy of power point presentation of lecture
taken for junior final year by
Dr Sanjay ShrivastavaProfessor of Ophthalmology
Gandhi medical College, Bhopal (M.P.)
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Classification of Cataract
1. Developmental
2. Age related (senile)
3. Cataract associated with ocular diseases4. Cataract associated with systemic
diseases (pre-senile)
5. Traumatic Cataract6. 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 itsminute 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 fetalnucleus.
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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 astrong 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 Ddeficiency. Patient may have Rickets and
defective enamel of permanent teeth.
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Zonular Cataract
Zone around embryonic nucleus, usually area offetal nucleus is affected and opacified. Its extentdepends on the duration of the inhibiting factor.Sharply demarked opacity, area within andaround opacity is clear; with linear opacitiesresembling spokes of a wheel (riders) runningtowards equator.
Such cataracts are usually bilateral formed justbefore or shortly after birth, may be sufficient indiameter to fill the pupillary area and vision isaffected.
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Developmental Cataract- types
3. Fusiform CataractSpindle shaped,
axial or coralliform. There is antero-
posterior spindle shaped opacity
sometimes with offshoots giving anappearance of coral.
Discoid Cataract is disc shaped opacity in
posterior cortex behind nucleus.
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Nuclear Cataract
Other congenital deformities like,
congenital heart disease (PDA),
microphthalmos, micrencephaly, mental
retardation, deafness and dentalanomalies may be associated.
If the lens matter remains in eye during
surgery, it is followed by a chronicinflammatory 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 2ndor 3rdmonth of pregnancy
to prevent birth of a child with multiple congenitalanomalies.
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Developmental Cataract - Types
5. Coronary Cataract: It is a developmentalcataract, similar to zonular cataract,occurring around puberty. Situated in deep
layer of the cortex and the most superficiallayers of adolescent nucleus. Appears ascorona of club shaped opacities atperiphery, hidden by iris. Axial area and
periphery of lens remains clear. Vision isusually not affected. These opacities arenon-progressive.
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Developmental Cataract - Types
6. Anterior Capsular (polar) Cataract: may be dueto delayed development of anterior chamber(congenital cataract) or due to contact of anteriorlens capsule and posterior surface of corneadue to perforation of ulcer, as in case ofophthalmia neonatorum.
Types Anterior Polar, Pyramidal Cataract andReduplication cataract
Usually these opacities are non-progressive andrarely 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 isinsignificant.
Sometimes in cases of persistent hyaloid
artery, the lens is deeply invaded byfibrous 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 ofdiminution of vision / does not recognizeobjects 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 toestablish in very young children)
Lenticular opacity
Nystagmus
Deviation of eye, usually convergentsquint
There may be other ocular and systemicabnormalities in cases of rubella nuclearcataract
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Management of Developmental
CataractI. Investigations:
1. Detailed history
2. Detailed clinical examination- visual status,
intra-ocular tension, fundus examination, Bscan 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
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 6weeks
b. Bilateral partial if vision is not
significantly affected, surgery may bedelayed 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
Cataract2. Treatment options
* No treatment if vision is not affectedsignificantly
* Mydriatics- if opacity is central and visionimproves with mydriatics
* In cases in Rubella Cataract operation maybe 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 sixweeks of birth with immediate fitting of contactlens.
* Fixation develops between 2-4 months ofage, therefore any cataract interfering with visionshould be dealt before this age, and the earliestpossible time is preferred
* Medical/ Paediatric fitness for anaesthesiashould be obtained.
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Management of Developmental
Cataract
3. Operative procedure
a. Aspiration and irrigation (ECCE)
b. Lensectomy (Pars plana or anteriorroute)
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 secondaryprocedure 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