1. Panit Cherdchu, M.D.
Ophthalmology Department
Phramongkutklao Hospital
Childhood Cataracts

2. CLASSIFICATION
EVALUATION
TREATMENT
TAKE HOME MESSAGE

3. Pediatric
cataracts
Isolated or Associated with a systemic condition
Congenital or Acquired
Inherited or Sporadic
Unilateral or Bilateral
Partial or Complete
Stable or progress
CLASSIFICATION

5. • Pediatric cataracts can be congenital or
acquired
• The earlier the onset, the more amblyogenic
the cataract will be
• Lens opacities that are visually significant
before 2-3 months of age are the most likely
to be detrimental to vision

6. Morphology and etiology (manifest in pattern)
Cataract Etiology Other possible
finding
Spoke like Fabry syndrome
Mannosidosis
Corneal whorls
hepatosplenomegaly
Vacuolar Diabetes mellitus Increased blood glucose level
Multicolored flecks Hypoparathyroidism
Myotonic dystrophy
Low serum calcium level
Characteristic facial features,
tonic “grip”
Green sunflower Wilson disease Kayser-Fleisher corneal ring
Thin disciform Lowe syndrome Hypotonia, glaucoma

7. Anterior polar cataracts (APCs)
• Less than 3 mm in diameter
• Small white dots in the center of Anterior
lens capsule
• Congenital
• Sporadic opacities
• Unilateral or bilateral
• Nonprogressive and visually insignificant
• Unilateral APCs are associated with
anisometropia Amblyopiarefraction
and F/U is important
• Anterior pyramidal cataracts = pyramidal
shape and project into anerior
chambermust be distinct from original
APCs due to more progressive and
amblyogenic factors
APCs

8. Nuclear Cataracts
• Opacities involve the center
or nucleus
• 3 mm in diameter
• Irregularity of the lens fibers
can extend more
peripherally
• Stable in most cases
• bilateral>unilateral
• Inherited or sporadic
• Eyes with nuclear cataracts
might be small and are at
risk for glaucoma in
childhood

9. Lamellar cataracts
• Discrete, round, lamellar
affect 1 or more layers of
cortex
• Larger than nuclear cataracts
• 5 mm or larger in diameter
• Bilateral>Unilateral
• Because onset is usually after
the fixation reflex has been
established, patients with
lamellar cataracts have a
better visual prognosis then
those with earlier cataracts
onset

10. Anterior Lenticonus
• This refers to a thinned-
out central anterior
capsule with or without
anterior cortical
opacities.
• Anterior lenticonus is
said to be characteristic
of Alport syndrome.
• Spontaneous rupture of
the lens can occur,
resulting in a hydrated
total cataract.

11. Posterior lenticonus (lentiglobus)
• Progressive thinning of the central
posterior capsule
• Thinning causes the deformation to
have an oil droplet appearance on
red reflex examination
• Outpouching of the lens
• Capsule can develops tearrapid
total opacification will come along
• Almost always unilateral***
• Normal eye size
• Posterior capsule weakness is
congenital but cataracts will
develop later
• Good visual prognosis after surgery

12. Posterior subcapsular cataracts (PSCs)
• Less common in
children than adults
• Usually acquired and
bilateral
• Progressive
• Corticosteroid, uveitis,
retinal abnormalities,
and radiation exposure
are risk factors
• Association with NF
type 2

13. Sectoral cataracts
• Wedge-shaped cortical
cataracts are
occasionally seen in
children
• Idiopathic or
associated with occult
posterior segment
tumor, previous blunt
trauma, or retinal
coloboma with fibrous
bands***

14. Peripheral vacuolar cataract
• Premature infants
• Encounter during
ROP examination
• Resolve over time

15. Persistent fetal vasculature (PFV)
• Last knowns as persistent hyperplastic primary vitreous
• Most common cause of unilateral cataract
• Isolated, sporadic malformation
• Bilateral cases may be associated with systemic or neurologic
abnormalities
• Affected eye are smaller than the other
• PFV has a spectrum of severity
– Mild PFV are prominent hyaloid vessel remnants, a large mitendorf
dot, and a bergmeister pailla
– End of the spectrum are microphthalmic eyes with dense retrolental
plaques; a thick fibrous persistent hyaloid artery; elongated ciliary
processes (classic for PFV); and a prominent radial iris vessels
– Traction on the optic disc may cause distortion of the posterior retina
– Retrolental plaque is densest centrally

16. Courtesy of Nasreen Syed, MD.
• Photomicrograph of persistent fetal vasculature. Note the prominent anterior
fibrovascular plaque (asterisk). The posterior remnant of the persistent hyaloid
artery is evident at the optic nerve head (arrow).

17. • Courtesy of Jerry A. Shields, MD

18. Traumatic disruption of lens
• In children, traumatic anterior
lens capsule rupture quickly
results in a hydrated white
cataract.
• However, in children, lens cortex
in the anterior chamber may be
well tolerated without an
intraocular pressure (IOP) rise.
• Cataract surgery can often be
delayed for a few days or up to 3
or 4 weeks to allow the
traumatic iritis to subside before
the cataract and IOL surgery.

19. Role of vision screening
• vision screening is mandatory to detect cataracts as soon as possible.
• Late detection may result in poor visual outcomes.
• All newborns must have red reflex screening, ideally followed by another red reflex
examination at the 6-8 week neonatal checkup.
• Red reflex testing is done by using direct ophthalmoscope from a distance of 1-2
feet in a darkened room.
• Preschool vision screening (at 3 and 5 years) is often done in the community.
• Photo screeners are used in preverbal and verbal children. These may help the
pediatrician save time in screening. They work by a computer analyzing the red
reflex for inequality in color, intensity, or clarity.
• New screeners utilizing polarized laser light are more accurate at detecting
decreased vision.
• The presence of any opacities, an absent red reflex, or leukocoria should prompt an
urgent referral to a pediatric ophthalmologist.

20. Examination
• “Surgery is not definite”
• Anterior capsule opacities are not visually
significant unless they occlude the entire pupil
• Central or posterior lens opacities greater
than 3 mm in diameter are usually visually
significant
• Strabismus+unilateral cataract and bilateral
cataracts+nystagmus=visually significant

21. • Preverbal children older than 2 monthsfixation behavior,
fixation preference, and object occlusion
• Bilateral cataractsfixation behavior+family’s observation
• Older children (lamellar or PSCs), glare testing may be useful
for detecting decreased vision
• Monitor posterior segment, B-scan ultrasonography if blocked

23. Work up
• Unilateral cataracts are not usually associated with occult
systemic or metabolic disease
• Bilateral cataracts may be associated with many systemic and
metabolic disease
• Family history+no associated systemic disease  work up is
not necessary
• Geneticist is crucial in detecting associated disorders

24. Cataracts surgery in pediatric patients

25. Timing of the procedure
When?
IOL
implant?
Visually
significant? “SURGERY”

26. • “The younger the child, the greater the urgency to remove the
cataract”
• To reduce the risk of visual deprivation amblyopia
• For optimal visual outcome
• ***Visually significant unilateral cataract should be removed
before age 6 weeks
• ***Visually significant bilateral cataracts, before age 10 weeks

27. • For older children with bilateral cataracts, surgery should be
done when the level of visual function interferes with the
child’s visual needs
• Although BCVA with 20/70 is satisfied for kids but it is never
enough later in life surgery is performed when BCVA <
20/40
• Driver’s license**
• For older children with unilateral cataract, perform surgery
when optical treatment and amblyopia therapy cannot
improve VA beyond 20/40

28. Intraocular Lens Use in Children
• IOL implantation depends on age
• Children aged 1-2 years and older is widely accepted
• Younger infants is not the best candidate
• Compared with CL rehabilitation in aphakic patients, IOL implantation in
infants aged 1-6 months is associated with a significantly higher rate of
complication, but not to visual acuity outcome at age 1 year
• Surgical intervention with CL wear + patching of the uninvolved eye for
treatment of amblyopia
• Infants with aphakicsecondary IOL implantation can be performed after
1-2 years of age
• Infant aphakia treatment study showed that aphakic infants with mild PFV
treated with CL had a higher incidence of adverse evnts after lensectomy
compared with children with other forms of unilateral cataract  both
groups had similar visual outcomes 1 years after surgery

29. IOL formula
• Method 1: emmetropia
– pros: no glasses needed after surgery and low risk
of amblyopia in poor compliance patients
– Cons: myopia when grow up
• Method 2: undercorrection
– Pros: emmetropia when grow up
– Cons: marked hyperopia and glass needed after
surgery+ risk of amblyopia in poor compliance
patients

30. Intraocular Lens Use in Children
Age Aim
undercorrection
อ.อาภัทรสา add
< 3 mo 40% + 5%
3-6 mo 35% + 5%
6-12 mo 30% + 5%
12-24 mo 25% + 5%
24-60 mo 20% + 5%
60-72 mo 10-15% + 5%
7-8 yr 2 D
8-10 yr 1 D
>10 yr Emmetropia
Rupal H Trivedi et al. Ophthalmic pearls: Selecting intraocular
lens power in children. www.aao.org

31. Rupal H Trivedi et al. Ophthalmic pearls: Selecting intraocular
lens power in children. www.aao.org
Intraocular Lens Use in Children
Age Residual refraction
<1.9 mo +10 D
2-3.9 mo +9 D
4-5.9 mo +8 D
6-11.9 mo +7 D
1-1.9 yr +6 D
2-3.9 yr +5 D
4-4.9 yr +4 D
5-5.9 yr +3 D
6-6.9 yr +2 D
7-7.9 yr +1.5 D
8-8.9 yr +1 D
10-13.9 yr +0.5 D
>14 yr plano

32. BJO 2015
Intraocular Lens Use in Children
Age Residual refraction (D)
<6 mo +6 to +10
6-12 mo +4 to +6
1-3 yr +4
3-4 yr +3
4-6 yr +2 to +3
6-8 yr +1 to +2
>8 yr Plano to +1

33. Intraocular Lens Use in Children
Dahan (JCRS 1997) <2 yr: 20% undercorrection
2-8 yr: 10% undercorrection
Enyedi (AJO 1998) Rule of 7
Age + undercorrection = 7
Infant aphakia treatment study
(IATS)
4-6 wk: 8 D undercorrection
7-26 wk: 6 D undercorrection
IOL: age based undercorrection

34. Management of the Anterior Capsule
• Different
technique with
adult capsulorhexis
• Elasticity of the
capsule is greatest
in younger patients
• 2-incision push-
pull technique

35. Lensectomy without IOL implantation
• Lensectomy is performed through a small limbal or pars plana
incision with a vitreous-cutting instrument (vitrector)
• Irrigation can be provided by an integrated infusion sleeve or by a
separate cannula
• Phacoemulsification is not required ( lens are soft in children)
• Remove all cortical material because of the propensity for
reproliferation of pediatric lens epithelial cells
• Posterior capsule opacification occurs rapidly I nyoung
childrencontrolled posterior capsulectomy and anterior
vitrectomy should be performed at the time of surgery in children
who are unlikely candidates for awake Nd:YAG capsulotomy
• Clear view leads to better screening, refraction and follow up

36. Lensectomy with IOL implantation
• Single-piece acrylic foldable IOLs is usually used
• Single-piece PMMA lenses are also still used
• Silicone lenses have not been well studied in
children
• Primary IOL implantation
– Leave the posterior capsule intact then NdYAG
capsulotomy later (mostly 5 yrsold or older)
– Primary capsulectomy is usually prefered for younger
children due to opacification of lens capsule after
surgery might occur in 18-24 months

37. Technique with posterior capsule intact
• Cortex is aspirated
• Clear corneal or scleral tunnel incision is enlarged
to allow placement of the IOL
• Place in capsular bag or ciliary sulcus fixation is an
acceptable choice
• Remove all viscoelastic material to prevent post
operative rising of IOP
• Closure of 3-mm incision with absorbable suture
has been shown to be safe and does not induce
astigmatigsm

38. Techniques for primary posterior capsulectomy
• Posterior capsulectomy/vitrectomy before IOL placement
– After lensectomy, the vitrector (low suction/high-cutting rate) is performed to
remove posterior capsule promtly with anterior vitrectomy
– The anterior capsule is enlarged
– Lens is implanted in the capsular bag +/- ciliary sulcus
– The capsulotomy must not extend/ IOL haptics do not pass the posterior
opening
– Vitreous must not entangle with IOL
• Posterior capsulectomy/vitrectomy after IOL placement
– Place the IOL in the capsular bag
– Close the anterior incision
– Approach the posterior capsule through the pars plana
– Small conjunctival opening over the pars plana
– Sclerotomy by microvitreoretinal blade 2.5-3.0 mm posterior to the limbus
– Wide anterior vitrectomy can be performed

39. IOL implantation issues
• Complicated IOL calculation
• Variable growth of the eye
• Inaccurate keratometry
• Inaccurate axial length measurement
• Formulas is mostly for an adult
• Studies have shown RF error of aphakic pediatric eyes
undergoes a variable myopic shift of approximately
7.00-8.00 D from age 1-10 with a wide SD
• RF at age 10 years is up to -8.00 D or greater
• RF change below age 1 year is even more
unpredictable

40. • 2 factors should be considered for IOL
implantation in children
– Age
– Target refraction at the time of surgery
• IOL power for adulthood but mostly
undercorrected and requires hyperopic
spectacles or CL of decreasing powers until the
teenaged years
• Aim for emmetropia (widely selected in unilateral
cases) to reduce the risk of amblyopia and
encourage binocular function

41. Postoperative care
• Medical therapy
– Topical ATB
– Topical corticosteroids
– Topical cycloplegics
– Intracameral corticosteroids
– Intracameral ATB

42. • Amblyopia management
– Amblyopia therapy start as soon as possible after surgery
– For aphakic patients, corrective lenses—in general, contact
lenses for unilateral or bilatreal aphakia, spectacles for
bilateral aphakia start within 1 week of surgery
– For infants with bilateral aphakia, spectacles are the safest
and simplest
– For infants with unilateral aphakia, cnotact lenses are the
most popular method
– After optical correction of aphakiapatching better eye
(unilateral cataract/asymmetrical VA in bilateral cataract)

43. complication
• Incidence of postoperative infections and
bleeding is similar to adults
• Strabismus is very commonly associated with
cataracts
• Risk of glaucoma is increased in children who
have surgery in infancy
• Glaucoma often develops many years after
lens extraction

44. Visual outcome after cataract extraction
• Depends on many factors
– Age of onset
– Type of cataract
– Timing of surgery
– Choice of optical correction
– Treatment of amblyopia

45. TAKE HOME MESSAGE
• Central or posterior lens opacities greater than 3
mm in diameter are usually visually significant
• Strabismus+unilateral cataract and bilateral
cataracts+nystagmus=visually significant
• ***Visually significant unilateral cataract should
be removed before age 6 weeks
• ***Visually significant bilateral cataracts, before
age 10 weeks
• Although BCVA with 20/70 is satisfied for kids but
it is never enough later in life surgery is
performed when BCVA < 20/40

47. REFERENCE
• Gilbert C. Worldwide causes of blindness in children. In: Wilson ME, Saunders RA, Trivedi RH,
eds. Pediatric Ophthalmology: Current Thought and a Practical Guide. Heidelberg, Germany:
Springer; 2009: 47-60.
• Haargaard B, Wohlfahrt J, Fledelius HC, Rosenberg T, Melbye M. Incidence and cumulative
risk of childhood cataract in a cohort of 2.6 million Danish children. Invest Ophthalmol Vis Sci.
2004;45(5):1316-1320.
• Xu LT, Traboulsi EI. Genetics of congenital cataracts. In: Wilson ME, Trivedi RH,
editors. Pediatric Cataract Surgery: Techniques, Complications and Management.
Philadelphia: Lippincott Williams & Wilkins; 2014: 1-8.
• Gillespie RL, O'Sullivan J, Ashworth J, Bhaskar S, Williams S, Biswas S, et al. Personalized
diagnosis and management of congenital cataract by next-generation
sequencing. Ophthalmology. 2014;121(11):2124-2137 e1-2.
• Serafino M, Trivedi RH, Levin AV, Wilson ME, Nucci P, Lambert SR, et al. Use of the Delphi
process in paediatric cataract management. Br J Ophthalmol. 2015. doi:
10.1136/bjophthalmol-2015-307287. [Epub ahead of print].

48. THANK YOU FOR YOUR ATTENTION