CONGENITAL CATARACT

1
CONGENITAL CATARACT
1. Important facts
2. Classification
3. Causes

• In healthy neonates

• In unwell neonates

2
Important facts

• 33 - idiopathic - may be unilateral or
  bilateral
• 33 - inherited - usually bilateral
• 33 - associated with systemic disease -
  usually bilateral
• Other ocular anomalies present in 50

3
Classification of congenital cataract
Anterior polar
Posterior polar
Coronary
Cortical spoke-like
Sutural
Focal dots
Lamellar
Central pulverulent
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Anterior polar cataract
May be dominant inheritance
Capsular
Pyramid
With persistent pupillary membrane
With Peters anomaly
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Posterior polar cataract

• Ocular associations
• Persistent hyaloid remnants
• Posterior lenticonus
• Persistent hyperplastic primary vitreous

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Coronary (supranuclear) cataract
Usually sporadic

• Round opacities in deep cortex
• Surround nucleus like a crown

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Cortical spoke-like cataract

• Systemic associations
• Fabry disease
• Mannosidosis

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Lamellar cataract
Usually dominant inheritance

• Systemic associations
• Galactosaemia
• Hypoglycaemia
• Hypocalcaemia

• Round central shell-like opacity
• surrounding clear nucleus
• May have riders

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Central pulverulent cataract
Dominant inheritance

• Spheroidal opacity within nucleus
• Relatively clear centre
• Non-progressive

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Sutural cataract
Usually X-linked inheritance
Opacity follows shape of Y suture
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Focal dot opacities

• Blue dot cortical opacities
• Common and innocuous
• May co-exist with other opacities

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Causes of cataract in healthy neonate

• Hereditary
• (usually dominant)
• Idiopathic
• With ocular anomalies
• . PHPV
• Aniridia
• Coloboma
• Microphthalmos
• Buphthalmos

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Causes of cataract in unwell neonate

• Intrauterine infections
• Rubella
• Toxoplasmosis
• Cytomegalovirus
• Varicella
• Metabolic disorders
• Galactosaemia
• Hypoglycaemia
• Hypocalcaemia
• Lowe syndrome