Pediatric Cataract

1. PEDIATRIC
CATARACTPresenter: Dr. Ajay Kumar Singh
Moderator: Dr. Wangchuk Doma
Venu Eye Institute & Research
Centre, New Delhi

2. INTRODUCTION
 Pediatric cataract
 Congenital cataract
 (present at birth)
 Developmental cataract
 (develops soon after birth)
 Traumatic cataract

3. INTRODUCTION
 Incidence - 6/10,000
 10% of childhood blindness
 Congenital or Acquired
 Visually significant or not
 Stable or Progressive
 Unilateral or Bilateral
 Partial or Complete
 Developmental opacity are usually partial & stationary

4. ANATOMY
 Lens - A transparent,biconvex,crystalline structure
between iris & vitreous in a saucer shaped
depression called patellar fossa.
 Components
 Anterior capsule
 Anterior capsular epithelium
 Cortex
 Nucleus
 Posterior capsule

5. CONGENITAL VERSUS DEVELOPMENTAL
 Congenital & developmental cataract not synonymous
 In widest sense, most forms of cataract (even senile
cataract) may be considered as developmental, for human
lens grows until late in life
 Criteria for congenital cataract
 present at birth
 situated within fetal nucleus
 diameter of opacity less than 5.75mm (diameter of newborn
lens)
 If size of opacity more than 5.75mm,cataract must be
postnatal

6.  Genetic Mutation
 Autosomal Dominant
 Metabolic
 Galactosaemia
 Lowe
 Hypoparathyroidism
 Fabry
 Infective
 TORCH organisms
 Congenital rubella
 Chromosomal
 Trisomy 21 (Down)
 Trisomy 18 (Edward)
 Trisomy 13 (Patau)
 Skeletal
 Hallerman-Streiff-Francois
syndrome
 Nance-Horan syndrome
 Ocular anomalies
 Aniridia
 Anterior segment dysgenesis
syndrome
 Idiopathic
 in 50%
CONGENITAL CATARACTS: BILATERAL

7. CONGENITAL CATARACTS: UNILATERAL
 Sporadic
 no family history/systemic disease
 Cause identified in only 10%
 Ocular anomalies
 Persistent fetal vasculature (most common)
 Affected infants usually full term & healthy

8. MORPHOLOGY
 TYPES
 Punctate (blue dot) cataract
 Anterior Capsular cataract
 Anterior polar cataract
 Pyramidal
 Reduplicated
 Posterior polar cataract
 Embronal (nuclear) cataract
 Coronary cataract/Fusiform cataract
 Zonular (Lamellar) cataract
 Total cataract
 Soft
 Membranous

9. MORPHOLOGICAL TYPES

10. PUNCTATE(BLUE DOT) CATARACT
 Most common type,universal
 Cataracta coerulea/blue dot cataract
 Multiple tiny blue spots scattered all
over lens esp in cortex
 Variant –cataracta centralis
pulverulenta
dominantly inherited,non
progressive
central spheroidal/biconvex opacity
consisting of powdery fine white dots
within embronic /fetal nucleus
Non progressive&visually insignificant

11. ZONULAR (LAMELLAR) CATARACT
 Accounts for 50% of visually significant cataract
 May be congenital or occurs at a later stage as development
interfered at a later stage
 Zone around embryonic nucleus (usually in area of fetal
nucleus) become opacified,extent depending on duration of
inhibiting factor
 Opacity sharply demarcated . Area of lens within & around
opaque zone is clear

12.  Linear opacities like spokes of a
wheel (called riders) may run
outwards
 Usually bilateral,formed just
before/shortly after birth
 Fill pupillary aperature when pupil
undilated, thus affecting vision
 Often hereditary (autosomal
dominant)
 Associated with hypovitaminosis D
(with evidence of ricket) or
hypocalcemia (with tetany or
ZONULAR (LAMELLAR) CATARACT

13. CORONARY CATARACT
 Around puberty
 Situated in deeper layers of
cortex &superficial layers of
adolescent nucleus
 Corona or club shaped
opacities near periphery of
lens ,usually hidden by iris
while axial region &extreme
periphery remain clear
 Non progressive & does not
interfere with vision

14. NUCLEAR CATARACT
 Associated with rubella
 Incidence more if infection
contracted in 2nd month
 Development of lens inhibited
at very early stage
 Embyonal nuclear cataract
 Progressive ,becomes total
cataract
 Associated microphthalmos,
nystagmus,strabismus,glauco
ma,iris hypoplasia &
pigmentary retinopathy

15. ANTERIOR CAPSULAR CATARACT
 May be due to
1. Delayed formation of anterior chamber (Developmental)
2. Acquired (commonly)-follows contact of capsule with cornea,
usually after perforation of ulcer in ophthalmia neonatorum
 Three types-
 Anterior polar (flat type)
 Anterior pyramidal
 Reduplicated

16.  Flat type – white plaque forms
in central pupillary area less than
3mm in diameter
 visually insignificant
 Pyramidal type – sometimes
central plaque projects forwards
into anterior chamber like a
pyramid
 Frequently surrounded by area of
cortical opacity
 may affect vision
ANTERIOR CAPSULAR CATARACT

17.  Subcapsular epithelium may grow in between
capsule & cortical opacity
 Clear lens fibre subsequently growing from there lay
down a transparent zone between two opacities
 Buried opacity is called imprint & two together
constitute Reduplicated cataract
 Association–
 Persistant pupillary membrane
 Aniridia
 Peters anomaly
 Anterior lenticonus
ANTERIOR CAPSULAR CATARACT

18. POSTERIOR CAPSULAR ( POLAR) CATARACT
 Associated with persistent hyaloid remnants(mittendorf
dots),posterior lenticonus & persistent anterior fetal
vasculature
 Common in minimal degree & visually insignificant
 With persistent hyloid artery , lens deeply invaded by
fibrous tissue leading to total cataract

19. OTHER TYPES
 SUTURAL CATARACT
 Opacity follows anterior /posterior
 Y suture
 May occur in isolation or
association with other opacity
 MEMBRANOUS
CATARACT
 Rare
 Associated with Hallermann-Streiff-
Francois syndrome
 occurs when lenticular material
partially /completely reabsorbs
leaving behind residual chalky –
white lens matter sandwiched
between anterior & posterior
capsule

20. SYMPTOMS OF DEVELOPMENTAL CATARACT
Informant – usually parents
 History of white spot in pupillary area
 Child is usually brought with history of diminution of vision /
does not recognize objects and parents
 Unsteady eyes
 Deviation of eye
 Associated symptoms of systemic disease, if present

21. SIGNS
 Diminished vision (at times it is difficult to establish
in very young children)
 Lenticular opacity
 Nystagmus
 Deviation of eye
 There may be other ocular and systemic
abnormalities in cases of rubella nuclear cataract

22. MANAGEMENT OF DEVELOPMENTAL CATARACT
Investigations
I. Detailed history
II. Complete ocular examination-
I. UCVA, BCVA, pupillary reaction intra-ocular tension, fundus
examination
II. B scan ultrasonography to exclude posterior segment
abnormality like growth/ retinoblastoma
III. A scan to determine axial length of the eye
IV. Retinoscopy, to deremine the refractive status
V. cover test to exclude squint
VI. Early photographs to know onset of cataract

23.  Laboratory investigations:
 (For bilateral cases)
 Blood Test
 Full blood count,Blood glucose(FBS/RBS)
 Serum calcium and phosphorus
 RBC transferase and Galactokinase levels
 TORCH test
 Hepatitis B virus
 Urine analysis:
 For reducing substance for galactosaemia
 For amino acids (to exclude Lowe syndrome in suspected cases)
 U/L Pediatric cases are mostly idiopathic.
 No need of lab investigation
MANAGEMENT OF DEVELOPMENTAL CATARACT

24.  Cataract in childhood not only reduce vision but also interfere
with normal visual development
 Timing of surgery ,surgical technique,choice of aphakic
correction & amblyopia management are of utmost
importance to achieve good &long standing results
 The inflammatory response to surgical results are more
pronounced in children
 Post operative amblyopia management forms an integral part
of visual rehabilitation in children
MANAGEMENT OF DEVELOPMENTAL CATARACT

25. ??????
 To operate or not?
 If yes, when to operate?
 If no, how to manage?
 If operating, should one place the IOL?
 If not placing IOL, how to manage and follow the child?

26. NON-SURGICAL TREATMENT
 Used for partial cataracts
 Partial cataract less than 3 mm and pericentral cataracts
respond
 Pupillary dilatation with 2.5% phenylephrine and part time
occlusion of good eye
 Cyclopentolate can be added once or twice a day if
required
 Prolonged cyclopegia (daily atropine) can induce amblyopia
 Pupillary dilatation is reserved for preverbal (1-6 years) with
partial cataracts and borderline amblyopia
 If significant amblyopia persists, cataract extraction should
be performed

27. WHEN TO OPERATE??
1. Bilateral dense cataracts :
- require early surgery at 4-6 weeks of age to prevent
development of stimulus deprivation amblyopia
2. Bilateral partial cataracts
-may not require surgery or require only at a later date

28. 3.Unilateral dense cataract
-urgent surgery is advised within days
-aggressive anti-amblyopia therapy should follow surgery
-results are often poor
-if detected after 16 weeks of age then surgery is
inadvisable because amblyopia is refractory
4.Partial unilateral cataract may be treated non surgically

29. GENERAL TECHNIQUES
 Deep general anaesthesia is required
 Pediatric cataracts are soft – lens material can be aspirated
through incisions that are 1-1.5mm long at the limbus ;
 can be subjected to lensectomy through pars plana
 A larger wound is needed to introduce IOL
 Tunnel should be securely sutured to prevent dehiscence of
wound with iris incarceration

30. SPECIFIC TECHNIQUES
There are two approaches
1. Pars plana approach
2. Limbal approach
Pars plana approach is being abandoned gradually in
favour of limbal approach as limbal approach allows better
preservation of the capsular bag for in-the-bag IOL
placement.

31. PARS PLANA APPROACH
 Indications :
 neonates and infants <2years of age
 with B/L cataracts
 for whom IOL implantation is not intended immediately

32.  ADVANTAGES
 Rapid and allows a
permanently clear visual axis
 Less complicated post-
operative course
 Iris and corneal endothelium
suffer less iatrogenic damage
 Two eyes can be operated at
the same time reducing the
risk of relative amblyopia and
second exposure to general
anaesthesia
 DISADVANTAGES
 Incarceration of the vitreous in
the scleral incisions
 Subsequent vitreal tractions
and retinal breaks or
detachments
 Insufficiently dilated pupil
hinders lensectomy leading to
too little capsular support for
future IOL implantation

33. LIMBAL APPROACH
 Most versatile technique
 Advantages:
 visualization of the instruments
 the ability to preserve the posterior capsule when
desired, and
 the lack of disruption of the vitreous unless planned.

35. APHAKIC CORRECTION IN CHILDREN
 Spectacles
 Contact lenses
 IOLs

36. SPECTACLES
 Satisfactory only in cases of B/L aphakia
 Most develop good visual acuity
 Cosmetically not acceptable
 Poor optical quality of high –plus lenses

37. CONTACT LENSES
 Better optical correction than spectacles
 Dioptric power can be adjusted throughout the life
 Difficult to manage and costly
 Loss of lenses
 Frequent infections
 Poor follow up
 Thus most impractical

38. INTRAOCULAR LENSES
 The IOL facilitates amblyopia management by providing
a more permanent correction
 Implanting an IOL in a growing eye is not an ideal
solution, but it is currently the most practical one.
 The aim in the IOL option is to correct most, but not all,
of the aphakia
 the residual refractive error has to be corrected using
spectacles, which can be adjusted throughout life.
 Posterior chamber IOL implantation is the safe method

39.  Both the biometry and the age of the child determine the
choice of the IOL dioptric power.
 Age <2 years- axial length and the keratometric (K)
readings change rapidly
 Age 2-8 years- changes are slower and more moderate.
 Expected large Myopic Shift
 AIM FOR UNDERCORRECTION
SELECTION OF IOL

40. GUIDELINES FOR THE CHOICE OF
INTRAOCULAR LENS DIOPTRIC POWER
CHILDREN LESS THAN 2 YEARS OLD
• Do biometry and undercorrect by 20%
• Use axial length measurements only:
- 17mm, 25 D
-18 mm, 24 D
-19 mm, 23 D
-20mm, 21 D
-21 mm, 19 D
CHILDREN BETWEEN 2 AND 8 YEARS OLD
• Do biometry and undercorrect by 10%

41. IOL Implantation in children
 After the cataract has been aspirated, an elective
posterior capsulectomy-anterior vitrectomy is performed.
 Sulcus implantation is easier and also allows an easier
explantation- may be done in neonates and infants less
than 1 year of age. But with the newer foldable IOLs, in
the bag implantation is the preferred technique.
 An in-the-bag IOL is more difficult to explant, this option
should be chosen for infants above 1 year of age
because they are less likely to need an IOL exchange,
provided they are undercorrected by 20%.

42. IOL Implantation in children
 In the bag technique requires both anterior and posterior
capsulorrhexises
 The IOL haptics are placed in the bag fornices, while the
optic is protruded through both capsulorrhexises to be
captured beneath the posterior capsule remnants.
 Tassignon has developed a special IOL called bag-in-the-
lens.
 The technique consists of creating an anterior and posterior
capsulorrhexis. The specially designed IOL has, at its
periphery, a groove that contains both anterior and posterior
capsule rims

43. 

44. COMPLICATIONS
 Intraoperative complications
-The anterior chamber tends to collapse
- the iris can protrude through the surgical wounds
- the pupil constricts on injury to the iris
 Immediate postoperative complications
-anterior plastic uveitis
- high intraocular pressure,
-incarceration of iris tissue in the wound
- endophthalmitis
 Late complications
-dislocation of the IOL
- chronic iritis
- glaucoma
-retinal detachment

45. AMBLYOPIA MANAGEMENT
 The unilateral cases are the most difficult to manage
 Amblyopia treatment starts soon after surgery, after clarification
of the media.
 The initial treatment must be aggressive
 Full-time occlusion of the sound eye is carried out for a few
days - 1 day per month of age
 Thereafter, occlusion is reduced to half the waking hours.
 Autorefractometers help to determine the residual refractive
error
 Spectacles are prescribed from the age of 4 months onward.

46.  A bifocal lens with an add of +3.00 is prescribed in the
pseudophakic eye from the age of 3 years, when the
child becomes verbal.
 Unilateral pseudophakes should continue with half-day
patches until 4-5 years of age.
 Thereafter, the patch time can be reduced gradually, but
should not be abandoned until 10–12 years of age.
 After that age, amblyopia management is practically
superfluous.
 Cases of bilateral pseudophakia should be followed
closely to detect and treat relative amblyopia.

47. Intraocular Lens Exchange and Alternative
Options
 Exchange of IOLs should be considered when a great
myopic shift has occurred
 When the pseudophakic eye becomes 7 D more myopic
than the sound eye, the IOL should be exchanged
 Refractive surgery in children is not yet an acceptable
option
 An alternative to IOL exchange is to implant an additional
negative dioptric power IOL in posterior chamber to correct
the myopia.
 This procedure is easily performed when the primary IOL
was inserted in the bag.

48. PROGNOSIS
 Visual outcome depends on
- Type of cataract
- Timing of intervention
- Quality of life
- Amblyopia management
 Near normal vision can be achieved in U/L congenital
cataract, provided amblyopia management is aggressive
 Binocularity is usually poor
 Aphakic & pseudophakic children should be followed
throughout childhood &preferably throughout life

49. THANK YOU