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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