1. سلام
خوشحالم در جمع شما اساتید محترم حضور دارم

2. Pediatric cataracts , case selection before surgery
Dr.Sayed Ezatollah Memarzadeh
ophthalmologist
Esfahan 1391

3. EPIDEMIOLOGY 1 In every 250 newborn has lens opacity
most of them are not visually significant
Prevalence of visually significant cataract is
1.2 to 6 in live birth
10-20% of blindness in children worldwide
Bilateral cataract is more common than unilateral

4. MORPHOLOGY AND LOCATION
Three main types : zonular , polar , lenticonus
Zonular is the most common type
Zonular subtypes : nuclear , lamellar , sutural
Polar : anterior or posterior
Lenticonus : anterior or posterior

5. ANTERIOR POLAR Small , not progressive , visually not significant
2/3 unilateral , 90% sporadic
associated with corneal guttata and astigmatism
microphthalmos or persistent pupillary membrane
1/3 anisometropia , strabismus , amblyopia

6. Morphology : Examples

7. ANTERIOR PYRAMIDAL Central cone projecting into AC
Surrounding cortical cataract
May progress and need surgery
Bilateral , sporadic

8. Anterior pyramidal

9. ANTERIOR SUBCAPSULAR
Often idiopathic
Trauma
Alport’s syndrome

10. NUCLEAR Dense , congenital , visually significant Bilateral , often AD
Associated with microphthalmos

12. SUTURAL Involved Y sutures and fetal nucleus
Progressive , visually not significant
Bilateral , often AD

13. LAMELLAR Most common type
Cortical , outside the Y sutures , clear nucleus and subcapsular area
Progressive and eventually needs surgery
Bilateral , often AD
Galactosemia , hypoglycemia

14. Zonular lamellar cataracts

15. TOTAL All layers are involved
AD ,Down’s syndrome , Metabolic disease, Trauma
Progression of other types of cataract

16. PERSISTENT HYPERPLASTIC PRIMARY VITREOUS
Failure of regression of hyaloid system
Fibrovascular stalk and membrane over the posterior capsule
Elongated ciliary processes
Anterior displacement of lens lead to glaucoma
Microphthalmos , occasional intralenticular hemorrhage
Sporadic , unilateral(90%) , progressive
Early surgery with vitrectomy instruments
Visual prognosis depends on amount of microphthalmia and involvement of posterior pole
Bilateral PHPV : Norrie’s disease , Trisomy13

17. POSTERIOR SUBCAPSULAR
Developmental
Down’s syndrome ,steroid, blunt trauma ,idiopathic

18. BLUE DOT Small scattered blue white opacities Visually not significant
Down’s syndrome

19. POSTERIOR LENTICONUS Thinning and bowing of central posterior capsule
Unilateral
Myopia , irregular astigmatism , amblyopia
Progressive and eventually needs surgery
Difficult surgery due to bowing of posterior capsule

20. CHRISTMAS TREE Small flecks with various colors Myotonic dystrophy
,hypoparathyroidism

21. MEMBRANOUS End stage Absorbed lens material ,fused capsules
Hallerman strieff , Rubella , Lowe ,Trauma

22. IMPORTANT POINTS REGARDING MORPHOLOGY
Visually significant : nuclear , lamellar ,posterior , total ,membranous
Progressive : posterior lenticonus , PHPV ,lamellar , subcapsular
Most common : lamellar
Better visual prognosis : anterior ,sutural , posterior lenticonus

23. ETIOLOGY Hereditary isolated cataract Metabolic diseases
Intra uterine infections
Trauma
Idiopathic
Chromosomal abnormalities
Other causes

24. ETIOLOGY Bilateral : 45% idiopathic , 50% hereditary , 5% infectious
Unilateral : 85% idiopathic, 5% hereditary , 2% infectious , 8% trauma
Associated with systemic disease : 25% in bilateral ,5% in unilateral
Ocular abnormality : 15% in bilateral , 50% in unilateral

25. HEREDITARY ISOLATED CATARACT
AD : most common , 25% new mutation variable expression ,
examination of family members , associated with microphthalmos
AR, XR ,rare

26. INTRA UTERINE INFECTION
TORCHS : toxoplasma , rubella , CMV ,herpes , syphilis
Usually bilateral , dense , and central
IgM antibody titer is elevated
Rubella is the most common
Total cataract due to candida in premature infants

27. RUBELLA CATARACT 15% of patients with congenital rubella have cataract
80% bilateral
Retinopathy ,strabismus ,microphthalmos ,optic atrophy ,glaucoma
Systemic : congenital heart defects , hearing loss , mental retardation
Prone to sever inflammation , post operative high dose steroid and pupillary
dilation

28. METABOLIC DISORDERS Cataract usually appears after birth
Galactosemia , Hypoglycemia , DM
Fabry’s disease
Hypoparathyroidism : multicolor cataract
Wilson’s disease :AD , 20% develop posterior subcapsular sunflower cataract

30. GALACTOSEMIA
Oil droplet cataract progress to lamellar and then total cataract
AR , three types
Transferase deficiency : early cataract , sever systemic disease vomiting ,
diarrhea , hepatomegaly , jaundice
Galactokinase deficiency : cataract later in infancy , few systemic signs ,
milk restriction reverse cataract
Epimerase deficiency : no cataract
Diagnosis : urine for reducing substance 2 hours after milk feeding

31. HYPOGLYCEMIA Complicated pregnancy Small for gestational age
Mental retardation
Bilateral lamellar cataract
More in boys
Reversible in most cases

32. FABRY’S DISEASE Alpha galactosidase deficiency XR
Posterior spoke like cataract in 50%
Not visually significant
Whorl like sub epithelial corneal opacity
Tortuosity of ocular vessels

33. RENAL DISORDERS
Lowe’s syndrome : XR , rare , congenital cataract in all , glaucoma ,
miotic pupil, mental retardation , death in 2nd decade , amino acid in urine
Alport : XD , anterior lenticonus , hematuria , proteinuria , deafness , myopia ,
cataract is not visually significant

34. OCULAR DISEASES ASSOCIATED WITH PEDIATREIC CATARACT
Microphthalmia: the most common
Aniridia
PHPV
ROP

35. STREOID INDUCED CATARACT
Posterior sub capsular cataract
Reversible in initial stage
Slow progression in children

36. IATROGENIC PEDIATREIC CATARACT
Laser photoablation for ROP or tumor
External beam radiation
steroid therapy
Damage to posterior capsule due to posterior vitrectomy

37. IMPORTANT POINTS
Reversible in early stages : galactosemia , steroid induced , hypoglycemia
cataract and glaucoma : Lowe, Rubella, Aniridia, Anterior segment dysgenesis

38. DIAGNOSIS
LEUKOCORIA
White reflex in anterior or diffuse opacities of lens
STRABISMUS
NYSTAGMUS
PHOTOPHOBIA

39. SIZE AND DENSITY OF CATARACT
Evaluation with direct ophthalmoscope or retinoscope
More than 3mm dense central opacity is significant and need surgery
In incomplete bilateral cataracts , density is more important than the size of opacity
If major retinal vessels can not be seen through the cataract , surgery is indicated
Semi transparent opacities should be treated conservatively

41. DIAGNOSIS
Early detection and treatment of cataract in all infants is the aim
This aim is difficult to achieve
Screening is mandatory in, nursery ,
at 6 weeks , and in 6 months of age for all infants
by assessment of red reflex with direct ophthalmoscope
Photophobia more in zonular type

42. Causes of Leukocoria DIFFERENTIAL DIAGNOSIS OF LEUKOCORIA Cataract
Retinoblastoma
Toxocariasis
Coat´s disease
ROP
PHPV
Retinal detachment
Coloboma
Retinal dysplasia
Norrie´s disease

43. STRABISMUS Common May develop even after surgery
Up to 50% of children with cataract will develop strabismus
More in unilateral cataract
ET is more common in congenital cataract
XT is more common in later onset and traumatic cataracts

44. NYSTAGMUS Develop in 2 to 3 months of life
Sign of early visual deprivation
Searching nystagmus
Poor visual prognosis , VA<20/200
Presents in 50% of children with dense bilateral cataract
Rarely seen in unilateral cataract
Cataract surgery may dampen the nystagmus

45. EVALUATION OF A CHILD WITH BILATERAL CATARACT
Family history : critical , exam of parents and siblings
History : age of onset
General
Ocular
Laboratory tests : IgM for TORCH , VDRL , Urine for reducing agent after milk
Optional laboratory tests : Urine amino acid (Lowe’s syndrome) and protein( Alport’s syndrome)
( , RBC galactokinase) , Blood sugar Ca and Ph , Copper

46. The visually significant cataract
In central visual axis, bigger than 3mm
Posterior cataract
No clear zones in between
Retinal details not visible with direct ophthalmoscope
Nystagmus or strabismus present
Poor central fixation after 8 weeks

47. DECISION FOR SURGERY IS DEPENDENT IN THESE FACTORS
Laterality
Visual behavior of the infant (FIXATION)
Presence of associated ocular and systemic abnormalities

48. LATERALITY
If a child with unilateral or bilateral cataract develops strabismus,
surgery must be done as soon as possible
In partial unilateral cataract , pupillary dilation combined with amblyopic therapy is an alternative for surgery
If a child with bilateral cataract develops nystagmus ,
surgery is indicated although visual prognosis is generally poor

49. VISUAL BEHAVIOR Visual attention
Pupillary reflex : RAPD is poor prognostic sign
Ability to pick up small objects

50. TIMING OF SURGERY
dense cataract , surgery must be done before age of 6 weeks in unilateral cases
dense cataract , surgery must be done before age of 10 weeks in bilateral cases
Interval between the surgery of the two eyes with should be minimized
Surgery before 4 weeks of life will increases the risk of glaucoma and pupillary membrane
Some authors advocate surgery on both eyes simultaneously in selected cases

51. BILATERAL PATCHING
Total bilateral patch for unilateral or bilateral cataract
Temporary , less than 2 weeks
It may extend the critical period of visual development
Contraindicated in unilateral cataract with poor prognosis like optic nerve or macular pathology

52. Evaluation Screen newborns with red reflex test
History : Family Maternal infections
Examination: systemic diseases or syndromes
Workup: Bilateral cases without known hereditary basis
TORCH screen
s-glucose
s-calcium, phosphate
Urine: reducing substances (galactosaemia)
amino acids ( Lowe syndrome)
haematuria (Alport syndrome)

53. CATARACT IN 4-6 MONTHS BABY
Is it truly congenita?
Previous photographs to see red reflex
Best prognosis if there is no strabismus and nystagmus
Surgery must be done even in presence of strabismus or nystagmus
VA>20/60 in 40% of unilateral and 60% of bilateral cases

54. PARENTS CONSULTATION
Parents should understand that treatment of the child starts only after surgery
Need to regular follow up visits
Need for wearing glasses or contact lenses even despite IOL implantation
Need for occlusion therapy

55. POST SURGICAL TREATMENT
Evaluation of fixation behavior
Refraction in each visit
Periodically IOP measurement under GA
In unilateral cases , occlusion of the fellow eye 50 to 70% of waking hours

56. NONSURGICAL TREATMENTNS
For peri central or small semi transparent central cataracts
Pupil dilation with 2.5% phenylephrine and sometimes tropicamide
Part time occlusion of good eye

57. FACTORS INFLUENCING VISUAL OUTCOME
Age of the onset of cataract
Age of the surgery
Associated ocular and systemic conditions
Compliance with optical an occlusion therapy

58. PROGNOSTIC FACTORS
The most posterior and the most central the cataract, the greater the visual impact
Surgery before 6-8 weeks of life is the most important prognostic factor
Surgery before 4 weeks of life is associated with increased risk of glaucoma and pupillary membrane
IOL implantation will increase risk of reoperation
Nystagmus is a poor prognostic factor
Amblyopia is more advanced in unilateral cataracts

59. VISUAL OUTCOME Surgery before 2months’’
Unilateral : VA 20/60 ( 20/800 to 20/30 )
Bilateral : 80% VA more than 20/50
gross stereopsis may develop on rare occasions
Surgery after 2 months or with nystagmusm’’
Unilateral : VA HM to 20/160
Bilateral : VA less than 20/100

60. Considerations regarding surgery
Intraocular lens : Power of lens – Myopic shift of the
growing eye
Surgical technique
Postoperative intraocular inflammation (uveitis)
Glaucoma and retinal detachment may develop

61. Ideal” IOL for the implantation in children
No anterior chamber IOLs
IOL haptic size - 10,0 – 11 mm
Haptics angulation - 0 .
Foldable optic sized 5,75 – 6,0 mm (rigid - 5x6 mm)
Biconvex
Material - hydrophilic acryl HYDROPHOBIC
IOL design
a. Single piece
b. Possibility of haptics adaptation without IOL decentration in
response to capsular growth or its contraction (no posterior
vaulting) - probably complex, closed loop haptic
c. Very good torsional and rotational stability of IOL in the capsul
d. Square edges
8. Blue filter ?
9. Possibility of implantation with the use of injector with the
nozzle diameter smaller than 2,0 mm.

62. IOL POWER 1 - 2 YEARS = IOL FORMULA - 20%
UNDERCORRECT IF RESULTS
QUESTIONABLE

63. Postoperative considerations
Clear vision for distance and near
Intraocular lens: regular refraction
Spectacles
Contact lenses
Treatment of amblyopia : Occlusion therapy

64. Conclusion: Congenital cataracts
Correct management essential to prevent permanent
visual loss
Team effort ophthalmologist, paediatrician,
geneticist,family
Early detection within the first month of life is very
important
Knowledge of systemic conditions associated with cataract
Glaucoma may develop

65. با تشکر از توجه شما