Ophthalmology · MU Sofia

49. Congenital lens anomalies

Covers hereditary and congenital cataract, beginning with a general classification by aetiology (congenital, developmental and acquired) and by morphology, with capsular, subcapsular, cortical, supranuclear, nuclear and polar forms. It then describes congenital and developmental cataract in more detail, including causes such as heredity, maternal infection and drugs, and the morphological types including lamellar, coronary, blue dot and total forms. The note closes with examination, laboratory work-up and timing and methods of surgical treatment.

Inside: 2 sections · 2 labelled pictures · 15 flashcards · 3 min

Topic 49 of 91 in the MU Sofia syllabus for Ophthalmology (Medicine, Year 4). The free AceMed note covers it in 2 sections, about 488 words (3 min of reading), with 15 flashcards.

In this note

  1. Cataract hereditary and Congenital Topic 35
  2. Congenital & developmental cataract

Cataract hereditary and Congenital Topic 35

= development of opacity In the normally transparent crystalline lens

There are different classification

A) According to aetiology (cause)

  1. 1. Congenital & developmental
  2. 2. Acquired –Senile, complicated, metabolic, electric, radiational, toxic ,
  • Association with skin diseases ,osseous diseases & syndromes

B) Morphology

  1. 1. Capsular cataract ; either Anterior or posterior capsule
  2. 2. Subcapsular→ superficial part of the cortex (just below the capsule)
  • Also anterior or posterior subscapular cataract
  1. 3. Cortical: involves major part of cortex
  2. 4. Supranuclear cataract ( just deeper part of cortex)
  3. 5. Nuclear cataract (involves the nucleus of lens)
  4. 6. Polar cataract ( anterior or posterior polar )

Congenital & developmental cataract

=very common in population but usually without visual disturbances

=due to disturbances of growth of the lens before birth or from after birth to adolescence

  • If congenital only the embryonic/foetal nucleus is affected but
  • Developmental may involve infantile /adult nucleus
  • It usually affects the layer that is being formed at that time thus previous or subsequent fibres usually are normal and clear

Aetiology: unknown but some factors

  • Heredity : 1/3 of all congenital due to chromosomal dominant patters
  • Maternal: malnutrition, infections (rubella) ,drug (corticosteroids, thalidomide) radiation
  • Infantile : anoxia, metabolic (Galactosemia, hypoglycaemia) , trauma &malnutrition
  • Idiopathic : 50 % unknown

Classified according to morphology

  1. 1. Capsular ;anterior or posterior pole
  2. a) Anterior : central part of the anterior capsule &superficial most cortex

Caused by : delayed anterior chamber or corneal perforation (contact between lens &cornea) Types: thickened white plaque in centre of capsule , pyramidal (with apex towards cornea) Reduplicated : thickening of central point and involvement of lens fibres immediately beneath

  1. b) Posterior :very common ,small circular circumcised opacity in posterior
  • Cause: hyaloid artery remnants , lenticonus, hyperplastic primary vitreous
  • Types : stationary or progressive (progression after birth)
  1. 2. Nuclear: cataracta centralis puverulenta → bilateral nuclear powdery opacity in embryonic
  • Total nuclear : embryonic,fetal +infantile nuc.→ dense chalky with serious impairment
  1. 3.Lamellar /zonular :most common type of congenital (40%)
  • Congenital or vitamin D deficiency or rubella
  • Features : foetal nucleus surrounding embryonic layer
  • Bilateral , main mass internal &external to mass is clear

But small linear opacities towards the equator sometimes

  1. 4.Generalised
  2. a) Coronary : extremely common of developmental (in puberty)
  • Affect adolescent nucleus , many hundreds of opacities in A regular radial distribution in periphery (hence unaffected vision)
  1. b) Blue dot :present as blue in periphery of adolescent nucleus (2nd decade)

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