This document summarizes a symposium on paediatric cataracts presented by Dr. Ganesh Pillay. Some key points: - Paediatric cataracts are a major cause of preventable childhood blindness worldwide. - Cataracts in children can be bilateral or unilateral, and have many potential causes including genetic conditions, infections, trauma and metabolic disorders. - A thorough history and examination is important to determine the type and severity of the cataract. Investigations may be needed depending on clinical findings. - The timing and type of cataract surgery in children presents various challenges and debates regarding issues like IOL selection and post-operative management. - Studies like the Inf

1. SYMPOSIUM
PAEDIATRIC CATARACT
DR.GANESH PILLAY MD. DNB.
SENIOR RESIDENT
R.P CENTRE, AIIMS, NEW DELHI
DATE:20/07/2017

2. INTRODUCTION
• ONE OF THE MAJOR CAUSES OF
PREVENTABLE CHILDHOOD
BLINDNESS,
• AFFECTS APPROXIMATELY 200,000
CHILDREN WORLDWIDE
• PREVALENCE = THREE TO SIX PER
10,000 LIVE BIRTHS (1,2)
1) Foster A, Gilbert C, Rahi J. Epidemiology of cataract in childhood: a global perspective. J Cataract Refract Surg.
1997
2) Stayte M, Reeves B, Wortham C. Ocular and vision defects in preschool children. Br J Ophthalmol. 1993

3. FORMATION OF LENS VESICLE AND OPTIC CUP

4. Bilateral cataracts Unilateral cataracts
• Idiopathic
• Hereditary cataracts (autosomal dominant)
• Genetic and metabolic diseases
1. Down syndrome
2. Lowe syndrome
3. Galactosemia
4. Marfan syndrome
5. Trisomy 13-15
6. Hypoglycemia
7. AIport syndrome
8. Myotonic dystrophy
9. Fabry disease
10.Hypoparathyroidism
• Maternal infection
1. Rubella
2. Cytomegalovirus
3. Varicella
4. Syphilis
5. Toxoplasmosis
• Ocular anomalies
1. Aniridia
2. Anterior segment dysgenesis syndrome
• Toxic
• Corticosteroids
• Idiopathic
• Ocular anomalies
1. Persistent fetal vasculature (PFV)
2. Anterior segment dysgenesis
3. Posterior lenticonus
4. Posterior pole tumors
• Traumatic (rule out child abuse)
• Rubella
• Masked bilateral cataract

5. MORPHOLOGY
Whole lens Central Anterior Posterior Miscellaneous
1. Total
2. Congenital
Morgagnian
3. Membranous
1. Lamellar
2. Central
pulverulent
3. Ant egg
4. Nuclear
5. Oil droplet
6. Cortical
7. Coronary
1. Anterior
polar
a) Dot like
b) Plaque
like
c) Anterior
pyramid
al
2. Anterior
subcapsular
3. Anterior
lenticonus
1. Mittendorf
dots
2. Posterior
cortical
3. Posterior
subcapsular
4. Posterior
lenticonus
1. Punctate lens
opacities
2. Sutural
3. Coralliform
4. Wedge
shaped
5. Persistant
hyperplastic
primary
vitreous

6. MORPHOLOGY: SPECIFIC DIAGNOSTIC
Total
cataract
-Sporadic or hereditary
-Seen in Downs or congenital Rubella
Early intervention
Anterior
polar
- Commonly seen in aniridia
- Anterior pyramidal is described in RB and Ehler Danlos syndrome
Anterior
Subcapsular
associated with uveitis, trauma, irradiation, and atopic dermatitis
Anterior
lenticonus
-bilateral condition seen in Alport syndrome
-rarely in Waardenburg syndrome

7. MORPHOLOGY: SPECIFIC DIAGNOSTIC
Oil-droplet
cataracts
infants with galactosemia
posterior subcapsular or small nuclear and cortical
opacification are also described
Sunflower
cataract
anterior subcapsular cataract almost only seen in Wilson’s disease
Posterior
subcapsular
cataract
- drug-induced, radiation, therapy for ocular and periocular tumors
-Turner’s syndrome, Fabry’s disease, Bardet–Biedl syndrome, and neurofibromatosis-2
Membranous
cataracts
-typically seen in Hallermann–
Streiff syndrome
congenital rubella syndrome, Lowe syndrome,
and PFV

8. MORPHOLOGY: SPECIFIC DIAGNOSTIC
Wedge-shape
cataracts
typically associated with
Stickler syndrome
Conradi–Hünermann syndrome,
neurofibromatosis type 2, and Fabry’s disease
Punctate
cortical
opacities
carriers of X-linked recessive Lowe syndrome and Down’s syndrome
Radiating spoke-like cortical
punctate opacities
Fabry’s disease
Sutural
cataracts
Incidental finding on routine examination
female carriers of Nance–
Horan syndrome

9. EVALUATION: HISTORY
• A DETAILED HISTORY INCLUDING FAMILY HISTORY
• A HISTORY OF THE ONSET OF THE LENTICULAR OPACITIES,
LATERALITY, AND PROGRESSION IS VERY IMPORTANT.
• A PRENATAL HISTORY INCLUDING MATERNAL DRUG USE AND
FEBRILE ILLNESSES WITH RASH
• BIRTH HISTORY, ESPECIALLY BIRTH WEIGHT
• DEVELOPMENTAL HISTORY SHOULD BE CAREFULLY ASSESSED

10. VISUAL ACUITY ASSESSMENT
• IN PREVERBAL CHILDREN, FIXATION BEHAVIOR, FIXATION PREFERENCE, AND OBJECTION TO
OCCLUSION SHOULD BE CHECKED.
• IN YOUNGER INFANTS WITH POORLY DEVELOPED FIXATION, A RED REFLEX TEST CAN BE
PERFORMED IN A DARKENED ROOM WITH A DIRECT OPHTHALMOSCOPE ALONG WITH
UNDILATED RETINOSCOPY TO ASSESS THE VISUAL SIGNIFICANCE OF THE LENS OPACITY.
• A CENTRAL CATARACT LARGER THAN 3 MM IN DIAMETER, UNILATERAL CATARACTASSOCIATED
WITH STRABISMUS, AND BILATERAL CATARACT WITH NYSTAGMUS ARE CONSIDERED VISUALLY
SIGNIFICANT.
• ASKING ABOUT THE VISUAL INTERACTION OF THE CHILD AT HOME WITH THE FAMILY
MEMBERS, HELPS IN DETERMINING THE SEVERITY OF VISUAL DYSFUNCTION.
• VISUALACUITY IN OLDER COOPERATIVE CHILDREN WITH PREFERENTIAL LOOKING CARDS
• PATTERN VER

11. Unilateral cataract Bilateral cataract
1. Not usually indicated
2. However, in any child with a positive
maternal antenatal history suggestive
of infection or the presence of
microcephaly, deafness, cardiac
abnormalities, and/or developmental
delay should be investigated for
TORCHS
1. Jaundiced infant with failure to thrive-
urine screening for reducing
substances and erythrocyte assays for
galactosemia.
2. Child with coexistent congenital
glaucoma, hypotonia, and
developmental delay,
oculocerebrorenal syndrome (Lowe
syndrome)- urine for amino acids
3. Neonatal tetany- calcium, phosphorus
and PTH
4. Sunflower cataract- Serum Cu,
ceruloplasmin, 24-hour urine Cu
INVESTIGATIONS

12. GENETIC ANALYSIS
• MORE THAN 40 DIFFERENT GENES AND VARIOUS LOCI HAVE BEEN IDENTIFIED WITH
CONGENITAL CATARACTS
• MUTATIONS IN THE GENES RESPONSIBLE FOR THE MAINTENANCE OF LENS CLARITY,
SUCH AS THE CRYSTALLIN AND CONNEXIN GENES, ARE THE MOST COMMONLY
DESCRIBED IN THE ETIOLOGY OF NONSYNDROMIC INHERITED CATARACTS
• MUTATIONS IN THE GENES CODING FOR TRANSCRIPTION FACTORS, AQUAPORIN (MAF),
BEADED FILAMENT STRUCTURAL PROTEIN, VIMENTIN, AND LENS INTRINSIC
MEMBRANE PROTEINS HAVE ALSO BEEN REPORTED
• GENES RESPONSIBLE FOR MAJOR SYNDROMIC CATARACTS INCLUDE
Gene Syndrome
OCRL Lowe syndrome
GALK117q Galactosemia
GLA Fabry’s disease
NHS Nance–Horan cataract–dental

13. INDIAN SCENARIO- PAEDIATRIC CATARACT
• Paediatric cataract is responsible for 7.4-15.3% of childhood blindness (1)(Rahi etal-12%)
• Cataract surgery in children is one of the most cost effective health care intervention in terms of
quality of life restored (DALY)(2)
• WHO has incorporated childhood blindness as major priority in its “vision 2020-the right to
sight” intiative.
• 25% are hereditary, 15% are syndrome, 10% infections and 50% are idiopathic.(3)
2. DALY-DISABILITY ADJUSTED LIFE YEARS
3. Wirth MG, Russell-Eggitt IM, Craig JE, Elder JE, Mackey DA.
Aetiology of congenital and paediatric cataract in an Australian population. Br J Ophthalmol. 2002;86(7):782–786.
1.Jun Yi et al, Epidemiology and molecular genetics of congenital cataracts, Int J of Ophthalmology, Aug 2011

14. PEDIATRIC CATARACT-AREAS OF DEBATE
• IOL selection and
power calculation
• Post operative target
refraction
• Biometry
measurement
accuracy
• Primary vs
Secondary
implantation
1 2
34

15. WHEN TO OPERATE
• VISUALLY SIGNIFICANT CATARACT
• IN CENTRAL VISUAL AXIS
• SIZE > 3MM
• POSTERIOR CATARACT
• RETINAL DETAILS NOT VISIBLE
• NYSTAGMUS OR STRABISMUS
• POOR CENTRAL FIXATION AFTER 8
WEEKS

16. TIME OF SURGERY

17. SURGICAL CHALLENGES
• Small eye and shallow anterior chamber
• Low scleral rigidity and Poor pupillary dilatation
• Thick and elastic anterior capsule
• Requirement for Posterior capsulorhexis and anterior vitrectomy

19. INFANT APHAKIA TREATEMENT STUDY (IATS)
• Infant Aphakia Treatment Study (IATS) is a multicenter, randomized, controlled clinical trial
sponsored by the National Eye Institute.
• Conducted in 12 centres across USA.
• The objective of the study is to compare the use of primary IOL implantation to surgery without
IOL implantation in infants with a unilateral congenital cataract removed between 1 and 6 months
of age.
The Infant Aphakia treatment study,Arch Ophthalmol. 2010;128(1):21-27. doi:10.1001/archopht

20. INFANT APHAKIA TREATEMENT STUDY (IATS)
• Inclusion criteria were a visually significant congenital cataract (≥3 mm central opacity) in one
eye and an age of 28 days to less than 210 days at the time of cataract surgery.
• Infants with a unilateral cataract resulting from persistent fetal vasculature were allowed in the
study as long as the persistent fetal vasculature was not associated with visible stretching of the
ciliary processes or involvement of the retina or optic nerve.
• Exclusion criteria were an acquired cataract, a corneal diameter of less than 9 mm, and
prematurity (36 gestational weeks).
The Infant Aphakia treatment study,Arch Ophthalmol. 2010;128(1):21-27. doi:10.1001/archopht

21. INFANT APHAKIA TREATEMENT STUDY (IATS)
• Randomisation to have either an IOL placed at the time of the initial surgery or to be left aphakic.
• Follow-up examinations were performed by an IATS certified investigator at 1 day, 1 week, 1
month, and 3 months after cataract surgery. Subsequent follow-up examinations were obtained at
3-month intervals.
• Infants randomized to the contact lens (CL) group underwent a lensectomy and anterior
vitrectomy. Infants randomized to the IOL group had the lens contents aspirated followed by the
implantation of an AcrySof SN60AT IOL into the capsular bag.
The Infant Aphakia treatment study,Arch Ophthalmol. 2010;128(1):21-27. doi:10.1001/archopht

22. INFANT APHAKIA TREATEMENT STUDY (IATS)
• In the event that both haptics could not be implanted into the capsular bag, an AcrySof MA60AC
IOL was implanted into the ciliary sulcus.
• The IOL power was calculated based on the Holladay 1 formula targeting an 8-diopter (D)
undercorrection for infants 4 to 6 weeks of age and a 6 D undercorrection for infants older than 6
weeks.
• After IOL placement, a posterior capsulectomy and an anterior vitrectomy were performed
through the pars plana and plicata.
The Infant Aphakia treatment study,Arch Ophthalmol. 2010;128(1):21-27. doi:10.1001/archopht

23. DEFINITIONS FOR INTRAOPERATIVE
COMPLICATIONS (ICS)
• (1) iris prolapse, or extrusion of the iris through the operative wound during surgery;
• (2) hyphema, or intraoperative bleeding that necessitated intraocular cautery or washout;
• (3) iris damage, or permanent structural change to the iris occurring during surgery;
• (4) retained cortex, or cortical material remaining in the eye after cataract surgery;
• (5) corneal clouding, or loss of corneal clarity occurring during the surgical procedure;
• (6) iris sphincterotomy, or intentional permanent enlargement of the pupil;
• (7) lens fragment in vitreous, or known loss of lens fragment into vitreous that was not retrieved; and
• (8) ruptured posterior capsule, or inadvertent rupture of the posterior capsule during the procedure.
The Infant Aphakia treatment study,Arch Ophthalmol. 2010;128(1):21-27. doi:10.1001/archopht

24. DEFINITIONS FOR ADVERSE EVENTS (AES)
• Glaucoma was defined as an IOP of more than 21 mmHg with one or more of the following
anatomic changes:
• (1) corneal enlargement;
• (2) asymmetrical progressive myopic shift coupled with enlargement of the corneal diameter,
axial length, or both;
• (3) increased optic nerve cupping defined as an increase of 0.2 or more in the cup-to-disc ratio;
or
• (4) the use of a surgical procedure for IOP control.
The Infant Aphakia treatment study,Arch Ophthalmol. 2010;128(1):21-27. doi:10.1001/archopht

25. DEFINITIONS FOR ADVERSE EVENTS (AES)
• Patient was designated a glaucoma suspect if there was either:
• (1) two consecutive IOP measurements of more than 21 mmHg on different dates after topical
corticosteroids had been discontinued without any of the anatomic changes listed above, or
• (2) glaucoma medications were used to control IOP without any of the anatomic changes listed above.
• Pupillary membrane was defined as fibrous tissue extending across the pupil.
• Lens reproliferation into the visual axis was defined as lens material regrowth extending into the
pupillary space and interfering with vision.
• Children who had strabismus surgery were not classified as orthotropic, even if they later were shown
to be orthotropic on motility testing.
The Infant Aphakia treatment study,Arch Ophthalmol. 2010;128(1):21-27. doi:10.1001/archopht

26. DEFINITION FOR ADDITIONAL INTRAOCULAR
SURGERY (AIS)
• AIS WAS DEFINED AS ANY RETURN TO THE OPERATING ROOM FOR AN
INTRAOCULAR SURGERY DURING THE FIRST POSTOPERATIVE YEAR BECAUSE OF
AN AE OR COMPLICATION.

27. RESULT
• VISUALACUITY
THE MEDIAN LOGMAR VISUALACUITY WAS NOT SIGNIFICANTLY DIFFERENT
BETWEEN THE TREATED EYES IN THE TWO GROUPS (CONTACT LENS GROUP, 0.80;
IOL GROUP, 0.97; P = .19).
THERE WERE MORE PATIENTS WITH ICS REPORTED IN THE IOL GROUP, 16 (21%),
THAN THE CL GROUP, 6 (11%; P = 0.031).
IN THE FIRST POSTOPERATIVE YEAR, THERE WERE MORE PATIENTS WITH AES IN
THE IOL GROUP, 44 (77%), THAN THE CL GROUP,

28. RESULT
• COMPLICATIONS ASSOCIATED WITH PERMANENT VISUAL LOSS—RETINAL
DETACHMENT, ENDOPHTHALMITIS, AND PHTHISIS— WERE CONFINED TO THE CL
GROUP.
• GLAUCOMA WAS DIAGNOSED MORE OFTEN IN THE IOL GROUP THAN THE CL
GROUP—7 CASES VERSUS 3 CASES—ALTHOUGH THIS DID NOT REACH
STATISTICAL SIGNIFICANCE.

29. RESULT
• IOL POWER AND PLACEMENT
• THE MEAN POWER (±SD) OF THE IMPLANTED IOL WAS 29.9 (5.7) D OVERALL (31.5
[5.0] D FOR THE YOUNGER AGE GROUP AND 28.7 [6.0] D FOR THE OLDER AGE
GROUP); IOL POWER RANGE WAS 11.5 TO 40.0 D.
• FOLLOW-UP REFRACTION AND PREDICTION ERROR (PE) THE OVERALL MEAN
(±SD) REFRACTION AT 1 MONTH WAS +6.1 (2.0) D. THE MEAN REFRACTION WAS
+6.6 (1.9) D IN THE YOUNGER AGE GROUP AND +5.7 (1.9) D IN THE OLDER AGE
GROUP.

30. RESULT
• TWENTY-TWO EYES (45%) ACHIEVED A POSTOPERATIVE REFRACTION WITHIN 1.0
D OF THE TARGET REFRACTION OF +8.0 D OR +6.0 D OUTLINED BY THE IATS
PROTOCOL.

31. CONCLUSION
• AT 1 YEAR AFTER SURGERY, THERE HAVE BEEN MORE ICS, AES, AND NEED FOR
AISS IN THE IOL GROUP COMPARED WITH THE CL GROUP. THESE DIFFERENCES
BETWEEN THE 2 GROUPS HAVE NOT BEEN ASSOCIATED WITH A WORSE VISUAL
OUTCOME TO DATE. THERE IS REASON TO BELIEVE THAT, OVER THE LONG TERM,
THE NUMBER OF AISS BETWEEN THE 2 GROUPS WILL BECOME MORE EVEN.

32. PRIMARY VS SECONDARY IMPLANTATION
PRIMARY IMPLANTATION
• Challenge is IOL power calculation
• Unpredictable post operative refraction
• More reaction in anterior chamber and
membrane formation.
SECONDARY IMPLANTATION
• Risk of amblyopia
• Compliance and handling of contact lens.
• Not cost effective.
• Risk of infection

36. Refractive Error Correction
• Prescription of glasses
• < 3 years- Correction only for near vision
• > 3 years- Bifocal glasses with +3 D near add
• Executive type glasses
• Bilateral aphakia – High refractive index glasses

38. IOL SELECTION
Emmetropia
IOL Exchange/
Refractive Surgery
Myopic Shift
Advantage. Disadvantage
No spectacles No
Contact Lenses
Prevention of
Amblyopia
Under-Correction
Unpredictable growth
of pediatric eye
Increased chances of
Emmetropia
in adult age
Spectacles Or
Contact Lenses
Risk of Amblyopia

39. INTRAOCULAR LENS POWER CALCULATION
AGE DAHAN et al Enyedi et al
(Diopters)
Flitecroft et al
(Diopters)
RPC
<1 year 20% undercorrection -- +6 10-15%
1-2 years 20% undercorrection +6 TO +5 +3 5-10%
3-4 years 10% undercorrection +4 TO +3 +3 5%
5-6 years 10% undercorrection +2 TO+1 +1 2-3%
7-8 years 10% undercorrection Plano +1 NO

40. COMPLICATIONS
IMMEDIATE POST-OPERATIVE
COMPLICATIONS
• SEVERE INFLAMMATION AND
INFLAMMATORY MEMBRANE
• PUPILLARY BLOCK
• WOUND LEAK AND LOSS OF
ANTERIOR CHAMBER
• SYNECHIAE FORMATION
VISUALAXIS OPACIFICATION
• AGE AT SURGERY < 6 MONTHS- 21.4 -30 % 1,2
• AGE AT SURGERY > 6 MONTHS – 2%1,2
• PRE-LENTICULAR AND RETRO-
LENTICULAR MEMBRANE
• MEMBRANECTOMY INDICATED
1.Vasavada AR, Trivedi RH, Nath VC. Visual axis opacification after AcrySof intraocular lens implantation in children. J Cataract Refract Surg. 2004;30(5):1073-
1081.
2. Stager DR Jr, Weakley DR Jr, Hunter JS. Long-term rates of PCO following small incision acrylic intraocular lens implantation in children. J Pediatr Ophthalmol
Strabismus. 2002;39(2):73-76.

42. COMPLICATIONS
• GLAUCOMA-
• AGE < 9 MONTHS -31.9%- 40% 1,2
• AGE> 9 MONTHS - 2.9-4.1%1,2
• AGE OF PRESENTATION- 3.1- 6 YEARS AFTER SURGERY
• RETINAL DETACHMENT-
• IN 1%-3.1% OF PATIENTS
• MEAN AGE- 6.8 YEARS AFTER SURGERY1
• ENDOPHTHALMITIS
• IN 0.071 -0.1 % OF PATIENTS
1.Plager DA, Yang S, Neely D, Sprunger D, Sondhi N. Complications in the first year following cataract surgery with and without IOL in infants and children. J
AAPOS. 2002;6(1):9-14.
2. Risk of Glaucoma after Pediatric Cataract Surgery Birgitte Haargaard,,Christian Ritz,,Anna Oudin,,wan Wohlfahrt,1 John Thygesen,,Thomas Olsen,4 and Mads
Melbye

43. SURGICAL ADVANCES IN USE
• Manual capsulorhexsis is the gold standard with most extensible capsulotomy (185%)> fugo
plasma blade (171%)> vitreorhexsis 165%) under electron microscope.
• High speed vitrectomy cutter reduces the traction while doing anterior vitrectomy.
• Autosert for controlled injection of IOL in bag.
• Active fluidics

44. INTRA OPERATIVE CONSIDERATIONS
Child < 2 Year
Lens aspiration + PPCC + Anterior Vitrectomy
IOL not recommended in all cases
IOL implantation in unilateral cataract
Surgeon preference
Child- 2-6 Years
Lens aspiration + PPCC + Anterior vitrectomy + PCIOL
Child > 6 Years
Lens aspiration + PCIOL implantation

45. POST OP ADVICE-AMBLYOPIA MANAGEMENT
• Rigorous amblyopia therapy in unilateral aphakia and pseudophakia
• Surgical correction of squint when indicated

46. TAKE HOME MESSAGE
• EARLY IDENTIFICATION AND EARLY SURGICAL INTERVENTION
• IOL IMPLANTATION AS PRIMARY PROCEDURE
• PCCC AND ANTERIOR VITRECTOMY<6 YEARS CHILD
• SUTURE PLACEMENT
• REFRACTIVE CORRECTION
• AMBLYOPIA THERAPY
• REGULAR FOLLOW UP