Congenital & Developmental Cataract

CONGENITAL & DEVELOPMENTAL CATARACT


CONGENITAL & DEVELOPMENTAL CATARACT

1. Congenital cataract:

  • Present at birth.
  • Opacity is limited to either embryonic or fetal nucleus.

2. Developmental cataract:

  • Occurs from infancy to adolescence.

Opacities involve:

  1. infantile or adult nucleus
  2. deeper parts of cortex or capsule.

TYPES OF CONGENITAL CATARACT

1. PUNCTATE CATARACT

  • M/C manifestation.
  • Almost universal in occurence.
  • When the small opaque spots are multiple & scattered all over the lens, appearing as tiny blue dots.
  • They are k/a cataracta coerulea or blue-dot cataract.
  • When cowded in Y- sutures, the terms sutural cataract & anterior axial embryonic cataract has been used.

2. ZONULAR CATARACT (Lamellar cataract)

  • This accounts for approx 50% of all visually significant congenital cataracts.
  • A zone around the embryonic nucleus (foetal nucleus) becomes opacified.
  • The area around the lens within & around the opaque zone is clear.
  • Linear opacities like spokes of a wheel (riders) run outwards towards the equator.
  • Lack of vitamin D is apparently a potent factor.
  • Evidence of rickets may be found in affected children.
  • Hypoparathyroidism during pregnancy may also cause.

3. FUSIFORM CATARACT (Spindle-shaped/axial/coralliform)

  • Anterioposterior spindle shaped opacity with offsets
  • Giving an appearance resembling a coral.

4. NUCLEAR CATARACT

  • Due to disturbance in development at a very early stage.
  • Central nucleus remains → Embryonic nuclear cataract.
  • Associated with rubella (German measles) in mother.

5. CORONARY CATARACT

  • Occurs around puberty.
  • Appears as a corona of club shaped opacities in the periphery of the lens.

6. ANTERIOR CAPSULAR (POLAR) CATARCT

  • Involves the central part of the anterior capsule & the adjoining superficial most cortex.

May be either due to :

  • Developmental delay in formation of anterior chamber
  • Acquired due to corneal perforation (as in penetrating injury or ophthalmia neonatorum)It may be:
  1. Thickened white plaque
  2. Anterior pyramidal cataract
  3. Reduplicate (double) cataract
  • The burried opacity is called imprint & the two together constitute reduplicate cataract.

7. POSTERIOR CAPSULAR (POLAR) CATARACT

  • Due to persistence of the posterior part of the vascular sheath of the lens.

MANAGEMENT OF CONGENITAL/ DEVELOPMENTAL CATARACT

  • Treatment is not indicated unless vision is considerably impaired.
  • If the opacity is large or dense, the treatment of choice is extracapsular extraction of the cataractous lens.
  • Intracapsular cataract extraction in children is contraindicated because of vitreous traction.

TIMING OF SURGERY

  • Very crucial.
  • The critical period for developing the fixation reflex in both unilateral & bilateral visual deprivation disorders is b/w 2-4 months of age.
  • Any cataract dense enough to impair vision must be dealt with before this age & the earliest possible time is preferred.
  • The timing of surgery depends whether cataract is unilateral or bilateral & whether opacity is dense or partial:

1. Bilateral cataract

  1. Dense: Early surgery by 4-6 weeks of age.
  2. Partial: May not required surgery

2. Unilateral cataract

  1. Dense: Urgent surgery within days
  2. Partial: observed & treated non-surgically by mydriasis & contralateral part-time occlusion to prevent amblyopia.

SURGERY FOR CONGENITAL OR DEVELOPMENTAL CATARACT

  • Cataract surgery is the treatment of choice.
  • Extracapsular cataract extraction (extracapsular removal of the cataractous lens) is the surgery of choice. 
  • This is done by phacoemulsification.
  • Intracapsular cataract extraction (ICCE) in children is contraindicated because of vitreous traction.

Method for extracapsular cataract extraction are:

  1. Posterior casulectomy & anterior vitrectomy (Procedure of choice) – done via limbal or pars plana approach.
  2. Lens aspiration
  3. Lensectony
  •  The traditional treatment of needling or discussion of cataract are obsolete now.

After removal of cataractous lens, the resultant aphakia in children is treated by:

  1. If child is < 2years→ Contact lens
  2. If child is > 2 years → Intraocular lens implantation

Exam Important

  • Commonest type of concussion cataract is Punctate cataract.
  • Most common type of congenital cataract is Punctate cataract.
  • Most common type of congenital cataract is Blue dot.
  • Minimum vision loss with Blue dot cataract.
  • Zonular cataract is the commonest congenital cataract which presents as visual impairment.
  • Second sight phenomenon is seen in Nuclear cataract.
  • Most visually handicapping cataract is Posterior subcapsular cataract.
  • Surgery of choice for congenital cataract is ECCE.

Method for extracapsular cataract extraction (ECCE) are:

  1. Posterior casulectomy & anterior vitrectomy (Procedure of choice)
  2. Lens aspiration
  3. Lensectony
  • Treatment of congenital cataract is Needing and aspiration.
  • Visual prognosis is poor in Unilateral congenital cataract.
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