This document discusses congenital and developmental cataracts. It describes the different types of cataracts including polar, nuclear, lamellar, and total cataracts. Causes are discussed such as hereditary factors, maternal infections like rubella, and metabolic disorders. Clinical features and management approaches are outlined including early surgery to prevent amblyopia, techniques like extracapsular cataract extraction, and correction of aphakia with lenses or contact lenses depending on age.

1. Congenital or Development
Cataract
By Priyanka ( MBBS Student )

2. When the disturbance occurs before
birth, the child is born with a congenital cataract.
Therefore, in congenital cataract the opacity is
limited to either embryonic or foetal nucleus.
Developmental cataract may occur from infancy to
adolescence. Therefore, such opacities may involve
infantile or adult nucleus, deeper parts of cortex or
capsule.
These are detected with the beam of slit-lamp under full
mydriasis.

3. About one-third cases are idiopathic, one-third
are hereditary, and the rest one-third due to
other causes.

4. II. Heredity
■■Inherited cases without systemic disorders,mode of
inheritance is usually autosomal dominant.
■■Inherited cases with systemic disorders include:
• Chromosomal disorders (e.g. trisomy 21),
• Skeletal disorders (e.g. Stickler syndrome),
• Central nervous system disorders (e.g. cerebrooculo-
facial syndrome),
• Renal system disorders (e.g. Lowe’s syndrome)

5. III. Maternal factors
1. Malnutrition during pregnancy
2. Infections. Maternal infections like rubella are
associated with cataract in 50% of cases. Other
maternal infections toxoplasmosis and cytomegalic
inclusion disease.
3. Drugs ingestion- mothers who
have taken certain drugs during pregnancy (e.g.,
thalidomide, corticosteroids).
4. Radiation.

6. IV. Foetal or infantile factors
1. Deficient oxygenation (anoxia) owing to placental haemorrhage.
2. Birth trauma, may cause cataract.
3. Metabolic disorders of the foetus or infant such as galactosemia,
galactokinase deficiency and neonatal hypoglycemia.
4. Cataracts associated with other congenital anomalies e.g., as seen in
Lowe’s syndrome, myotonia dystrophica and congenital icthyosis.
5. Ocular diseases associated with developmental cataract include
persistent hyperplastic primary vitreous (PHPV), aniridia, anterior
chamber cleavage syndrome ,retinopathy of prematurity, lenticonus
posterior and microopthalmos.
6. Malnutrition in early infancy may also cause developmental cataract.

7. Clinical Types

8. I. Congenital capsular cataracts
1. Anterior capsular cataracts are nonaxial, stationary and visually
insignificant.
2. Posterior capsular cataracts are rare and can be associated with
persistent hyaloid artery remnants.

9. II. Polar cataracts
1. Anterior polar cataract
It involves the central part of the anterior capsule and
the adjoining superficial-most cortex.
• Due to delayed development of anterior chamber. In
this case the opacity is congenital usually bilateral,
stationary and visually insignificant.
• Due to corneal perforation. Such cataracts may also
be acquired in infantile stage and follow contact of
the lens capsule with the back of cornea

10. • Thickened white plaque in the centre of anterior
capsule.
• Anterior pyramidal cataract. In this the thickened
capsular opacity is cone-shaped with its apex
towards cornea.
Morphological types

11. • Reduplicated cataract (double cataract).
Sometimes along with thickening of central
point of anterior capsule, lens fibres lying
immediately beneath it also become opaque and
are subsequently separated from the capsule by
laying of transparent fibres in between. The buried
opacity is called ‘imprint’ and the two together
constitute reduplicated cataract.

12. 2. Posterior polar cataract
It is a very common lens anomaly and consists of a small
circular circumscribed opacity involving the posterior pole.
Associations. Posterior polar cataract may be associated
with:
• Persistent hyaloid artery remnants (Mittendorf dot),
• Posterior lenticonus, and
• Persistent hyperplastic primary vitreous (PHPV).

13. Types. Posterior polar cataract occurs in two forms:
• Stationary form and
• Progressive form which progresses after birth. It
typically has an ‘onion whorl appearance’

15. III. Congenital nuclear cataracts

16. 1. Cataracta centralis pulverulenta
It has dominant genetic trait and occurs due to inhibition of
the lens development
at a very early stage and thus, involves the embryonic
nucleus.
The condition is bilateral and is characterised by a small
rounded opacity lying
exactly in the centre of the lens.
The opacity has a powdery appearance (pulverulenta) and
usually
does not affect the vision.

17. 2. Lamellar cataract
Lamellar or Zonular cataract refer to the
developmental cataract in which the opacity
occupies a discrete zone in the lens. It is the most
common type of congenital cataract presenting with
visual impairment. It accounts for about 50% of the
cases.

18. It may be either genetic or environmental
in origin.
■■Genetic pattern is usually of familial autosomal
dominant variety.
■■Environmental form is associated with:
• vitamin D deficiency,
• hypocalcemia
• sometimes maternal rubella infection contracted
between 7th and 8th week of gestation

19. ■■The main mass of the lens internal and external to
the zone of cataract is clear, except for small linear
opacities like spokes of a wheel (riders) which may
be seen towards the equator.
■■Occasionally two such rings of opacity are seen.
■■It is usually bilateral and frequently causes severe
visual defects.
Typically, this cataract occurs in a zone of foetal nucleus
surrounding the embryonic nucleus

20. Lamellar cataract : A & B, diagrammatic depiction
as seen by oblique illumination and in optical section with
the beam of the slit-lamp, respectively

21. clinical photograph

22. 3. Sutural and axial cataracts

23. Sutural cataracts are comparatively of common
occurrence and consist of a series of punctate opacities
scattered around the interior and posterior Y-sutures.
Such cataracts are usually static, bilateral and do not
have much effect on the vision.
The individual opacities vary in size and shape and have
different pattern

24. • Floriform cataract. Here the opacities are arranged like the
petals of a flower.
• Coralliform cataract also known as fusiform spindle shaped
axial cataract
• Spear-shaped cataract. The lenticular opacities are in the
form of scattered heaps of shining crystalline needles.
• Anterior axial embryonic cataract occurs as fine dot near
the anterior Y-suture of fetal nucleus
• Dendritic sutural cataract occurs as fine dots along the
dendritic sutures.

25. 4. Total nuclear cataract
Total nuclear cataract. It usually involves the
embryonic and fetal nucleus and sometimes infantile
nucleus as well. It is characterized by a dense chalky
white central opacity seriously impairing vision. The
opacities are usually bilateral and non-progressive.

26. Generalized Cataract

27. 1. Coronary cataract
Coronary cataract is an extremely common form of developmental
cataract occurring about puberty; thus involving either the adolescent
nucleus or deeper layer of the cortex.
The opacities are often many hundreds in number and have a regular
radial distribution in the periphery of lens (corona of clubshaped
opacities) encircling the central axis.
Since,the opacities are situated peripherally, vision is usually
unaffected.

28. Coronary cataract: A & B as seen by oblique
illumination and in optical section with the beam of the slit-
lamp, respectively,

29. clinical photograph

30. 2. Blue dot cataract
It is also called cataracta-punctata-caerulea.
most common type of congenital cataract.
It usually forms in the first two decades of life.
The characteristic punctate opacities are in the form of rounded bluish
dots situated in the peripheral part of adolescent nucleus and deeper
layer of the cortex.
Opacities are usually stationary and do not affect vision.
However, large punctate opacities associated with coronary cataract may
marginally reduce thevision.

31. 3. Total congenital cataract
It is a common variety and may be unilateral or bilateral
In many cases there may be hereditary character.
Its other important cause is maternal rubella.

33. Rubella Cataract
• Maternal Rubella infection acquired during first
trimester (second or third month) may cause
rubella cataract.
• Rubella cataract-typically, the child is born with a ‘pearly
white’ nuclear cataract. It is a progressive type of cataract.
• Lens matter may remain soft or may even liquify (congenital
Morgagnian cataract).

34. 4. Congenital membranous cataract
Sometimes there may occur total or partial
absorption of congenital cataract, leaving behind
thin membranous cataract.
Such a patient may be misdiagnosed as having congenital
aphakia. This is associated with Hallermann-Streiff-
Francois Syndrome.

35. Management

36. 1. Ocular examination should be carried out with
special reference to:
• Density and morphology of cataract.
Density is indicated by quality of red reflex seen on
distant direct ophthalmoscopy before and after
dilation of pupil.
Cataract morphology may suggest underlying cause.

37. Assessment of visual function is difficult in infants
and small children.
An idea may be made from
the density and morphology of the cataract by
oblique illumination examination and fundus
examination.
Special tests like fixation reflex, forced choice
preferential looking test, visually evoked potential
(VEP), optic-kinetic nystagmus (OKN) etc. also
provide useful information.

38. • Associated ocular defects which need to be noted
include microphthalmos, glaucoma, PHPV, foveal
hypoplasia, optic nerve hypoplasia, and rubella
retinopathy etc.

39. 2. Laboratory investigations should be carried out
■■Intrauterine infections viz. toxoplasmosis, rubella,
cytomegalo virus and herpes virus by TORCH test.
■■Galactosemia by urine test for reducing substances,
red blood cell transferase and galacto kinase levels.
■■Lowe’s syndrome by urine chromatography for
amino acids.
■■Hyperglycemia by blood sugar level.
■■Hypocalcemia by serum calcium and phosphate
levels and X-ray skull.

40. Indications and timing of paediatric cataract surgery

41. 1. Partial cataracts and small central cataracts which
are visually insignificant can safely be ignored and
observed or may need non-surgical treatment
with pupillary dilatation

42. 2. Bilateral dense cataracts should be removed early
(within 6 weeks of birth) to prevent stimulus
deprivation amblyopia. Second eye should be
operated within a few days of the first eye.

43. 3. Unilateral dense cataract should preferably
be removed as early as possible (within days)
after birth with optical correction in the first
few weeks.

44. Childhood cataracts (congenital, developmental as
well as acquired) can be dealt with extra capsular
cataract extraction technique involving anterior
capsulorrhexis and irrigation aspiration of the lens
matter (lens aspiration) or lensectomy.

45. Lens aspiration
can be performed either by manual SICS technique
or by phacoemucification technique. Lens aspiration
should be combined with primary posterior
capsulotomy in children below 6 years of age and also
with anterior vitrectomy in all children below 2 years of
age.

46. E. Correction of paediatric aphakia
• Children above the age of 2 years can be corrected
by implantation of posterior chamber intraocular
lens during surgery.
• Children below the age of 2 years should preferably
be treated by extended wear contact lens. Spectacles
can be prescribed in bilateral cases. Later on
secondary IOL implantation may be considered.

47. • Size of IOL above the age of 2 years may be
standard 12 to 12.75 mm diameter for in the bag
implantation.
• Design of IOL of choice at present is foldable IOLs
made of hydrophobic acrylic material

48. • Power of IOL. Most surgeons target emmetropia
in older children (>8 years). In children between
2–8 years of age 10% undercorrection and below 2
years an undercorrection by 20% is recommended
from the calculated biometric power to counter
the myopic shift.
• Correction of amblyopia

49. Thank You!
Acknowledgement – A K Khurana
Comprehensive Ophthalmology