3. Definition
Congenital cataract :
lens opacity present at birth or within first year of life
• Infantile cataract and congenital cataract are used interchangeably
by many physician
• Different from cataract in adult in terms of :
-etiology
-medical and surgical management
-visual rehabilitation
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Introduction…
• Earliest recognition of congenital cataract- Beer(1817)
• After introduction of slit lamp biomicroscope –morphological
varieties identified
• Congenital cataracts differ in two major respects from adult cataract.
– Firstly, in why they develop and,
– Secondly, in how they are managed
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EPIDEMIOLOGY
• Estimated figure of Childhood blindness- approximately 1.5 million
• Up to 500,000 new cases every year
• Incidence of Congenital cataract=1-3/10,000
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• Causes of blindness in different parts of the world vary widely.
– Nutritional factors and infections more common in developing
countries
– Hereditary and developmental disease more frequent causes in
countries with better standards of living and health care services
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Nepal
• Childhood blindness was 0.63/1000 live birth ( WHO,1980)
• According to NBS (1981) cataract is third (16.3%) most common
cause of blindness in under 15 years of age
8. CLASSIFICATION
• According to LATERALITY
• According to MORPHOLOGY
• According to ETIOLOGY
• According to AGE OF ONSET
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Classification….
• According to laterality
– Unilateral
– Bilateral
• 1/3rd - idiopathic - may be unilateral or bilateral
• 1/3rd - inherited - usually bilateral
• 1/3rd - associated with systemic disease - usually
bilateral
15. Importance of recognizing morphology
• Recognizing specific etiology
• Provides clues regarding timing of surgery
• Predicts prognosis
• Clues regarding progressive/ non-progressive nature of
cataract
• Helps in planning an appropriate surgical technique
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16. TOTAL CATARACT
• General opacity of all lens
fibers
• Lamellar and nuclear cataracts
progress to total cataract
• Suspect posterior lenticonus if
minimal opacity progresses in
short time to total cataract, or
suspect persistent fetal
vasculature.
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18. CONGENITAL MORGAGNIAN
CATARACT
• Nucleus sinks due to
liquefaction of the outer zone
lens
• Rarely if present late, fluid get
absorbed leading to fusion of
anterior and posterior capsule
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19. MEMBRANOUS CATARACT
• Occurs spontaneously or
following trauma
• Cataract gets absorbed, and
the anterior and posterior
capsule fuses, sometimes
even to an extent that allows
clear view of fundus,
leaving only an aphakic
refractive error
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20. • Congenital rubella
• Hallermann-Streiff
syndrome (congenital
aphakia)
• Persistent fetal
vasculature
• Following rupture of
anterior lenticonus
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21. ANTERIOR POLAR CATARACT
• Hereditary (dominantly inherited)
• Involves central part of anterior capsule and adjoining superficial
most cortex
• Vision not affected by obscuring
• Mydriatics
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22. • Pattern:
Dot form
Plaque form
Reduplicated or double cataract
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29. CENTRAL PULVERULENT CATARACT
• Bilateral
• Involves embryonic nucleus
• Seen in:
Galactosemia
Hypocalcemia
Hypoglycemia
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30. NUCLEAR CATARACT
• Involve pupillary area
• bilateral
• AD
• Common morphological form in Congenital Rubella Syndrome
• Associated with microphthalmos
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33. SUTURAL CATARACT
• Common morphological form
• Usually does not affect vision
• Bilateral, familial (AD/ X-linked recessive) and non progressive
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40. POSTERIOR CAPSULAR
• Cataract involving the posterior capsule, also associated with some
amount of posterior sub capsular cataract
• Vision affected early
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41. Posterior polar cataract
• Profound decrease in vision as larger
and closer to nodal point of eye
• Capsular fragility
• Familial bilateral and AD
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42. Cerulean cataract
• Usually does no affect vision
unless associated with some other
form of central cataract
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Metabolic
• Lamellar cataracts develop in Hypoglycemic or
hypocalcaemic conditions
Hypoglycemia common in Low Birth Weight Babies
Hypocalcaemia - hypoparathyroidism or
pseudohypoprathyroidism
Hypoglycemia/Hypocalcemia
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Systemic…
• Associated with number of syndrome - systemic disorders as
well as other ocular anomalies
1. Chromosomal
– Tirsomy 21 ( Down’s syndrome)
– Trisomy 13-15
– Trisomy 16-18
– Turner’s Syndrome
– Cri du chat syndrome( Deletion of Chr. 5 p)
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• Down’s Syndrome
– Trisomy 21
– The frequency of early cataract estimated to be 1.4%.
– Cataracts requiring surgery during childhood is rare.
– In one third - bilateral cataract
– Commonest association is Brushfield spot in the iris.
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Syndromic
2. Skeletal disease
– Conradi – Hunermann syndrome
– Rhizomelic Chondrodysplasia Punctacta
– Stickler syndrome
– CAMFAK syndrome
3. Syndactyly, Polydactlyl or other digital anomalies
– Rubenstein – Taybi Syndrome
– Ellis van Creveld syndrome
4. Central Nervous System abnormalities
– Marinesco –Sjogren syndrome
– Norrie’s disease
– Martsolf syndrome
– Cerebro – oculo – facial – skeletal syndrome
– Smith – Lemli – Opitz syndrome
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Infections
• Congenital Rubella
– If occur 1st trimester of pregnancy.
– Pearly white nuclear cataract
– Sometimes entire lens opacified ( complete cataract) and the
cortex may liquefy.
– Live virus recovered from the lens as late as 3 years after the
birth.
– Other ocular manifestations – diffuse pigmentary retinopathy,
microphthalmos, glaucoma and transient or permanent corneal
clouding.
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Medication
• Systemic corticosteroid cause cataracts in up to 15% of children once
a cumulative dose of 1000mg of prednisolone or the equivalent has
been reached.
• Begin as central posterior subcapsular opacities, may involve entire
lens.
60. Radiation Induced Cataracts
• Radiation used to treat ocular and periocular tumors
• dose of 15 GY shown to be associated ,with 50% risk
• Posterior subcapsular cataracts - 1-2 years after therapy
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Laser Photo Coagulation
• Used to ablate avascular retina of infants with threshold
retinopathy of prematurity
• Can result in formation of cataracts
• Transient but may progress to total opacification of lens
63. • Congenital cataracts represent a diverse spectrum of
morphologies, etiologies, and clinical presentations
• Different from cataract in adult in terms of :
-etiology
-medical and surgical management
-visual rehabilitation
CONCLUSION…..
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65. BIBLIOGRAPHY
1. Clinical Ophthalmology-Myron Yanoff
2. American Academy of ophthalmolog (lens and
cataract,,Pediatic opthalmology and strabismus)
3. Albert and Jakobiec
4. Clinical Ophthalmology -Jack J. Kanski
5. Atlas of opthalmology(Richard k Panish )
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