1.  Constitutes 10-40% of childhood blindness  Dandona et al (India)-15%  Rahi et al (India)-12%  Tailor et al (USA)-15%

2. Study by Rahi et al (India, blind school) Unoperated cataract-40% Uncorrected aphakia & amblyopia -40% Unsuccessful surgery / post operative complications-20 %

3.  Lens structures include embryonal, fetal nuclei, cortex, lens epithelium & lens capsule  Because of the layered development of the lens the timing of intrauterine insult can be judged by the location of the opacity  Since lens and other anterior segment structure interrelated during development the abnormalities many times coexist

4.  Only 60% of Bilateral cataract and 40% of Unilateral cataract can be established with specific etiology  Others are undetermined

5. Congenital cataract Hereditary, genetic Metabolic Secondary Embryodisgenesis Etiology

6.  Hereditary factors  Non hereditary factors

7.  Isolated hereditary congenital cataracts  Cataracts associated with ocular disorder  Cataract associated with autosomal syndrome  Cataract associated with metabolic disorder

8.  Autosomal dominant  Most common  Variable expressivity with high penetrance  Different morphology in families and in individuals  Autosomal recessive  Less common responsible for metabolic disorders

9.  X-linked inheritance - 3 forms  Dense cataract in affected male Sutural cataract in carrier female  Associated with microcornea and microphthalmos  Cataract & dental anamolies ( Nancy Horan syndrome)

10.  New mutation (50%)  Familial (8-23%) Dominant & recessive  Chromosomal trisomy 21,13,31,18,32, turners  Systemic disease- lowes, Hallerman shreif, conradis, potters, sticklers,cockayne

11.  Pulverulent  Anterior polar  Posterior polar  Nuclear  Lamellar  Sutural  Blue dot cataract  Total cataract

12.  Inherited as autosomal dominant - 2 types  Zonular pulverulent cataract (Coppock cataract)  Central pulverulent cataract  Typically bilateral & symmetrical  Genes located at Chromosome 1q, 2q & 13q  Mutated genes - connexin 50 & crystalins

13.  Inherited as autosomal dominant  Opacity situated at the anterior pole of the lens  Minimal effect on visual acuity  Usually unilateral & stationary  Gene located at chromosome 17p

14.  Conical opacity with apex of the cone projecting into the anterior chamber  Measures 2-2.5mm in diameter  Usually bilateral  Occurs sporaidically

15.  Thinning and protrusion of center of the posterior capsule posteriorly  Associated with posterior lenticular opacity  Characterised by late onset

16.  Small opacity at posterior capsule  Inherited as autosomal dominant and recessive form  Can cause gross visual impairment  Can be associated with Mittindorf’s dot

17.  Inherited as AD, AR or X-linked  Opacification of central zone of lens specifically the region between the anterior & posterior sutures  Usually bilateral with variable density  gene located at chromosome 21 q  Mutated gene -  crystelins

18.  Inherited as autosomal dominant  Lamella of lenticular opacification sandwiched between clear nucleus and cortex  Usually bilateral with variable density  Gene located at chromosome 2q

19.  Inherited as X-linked trait  Opacities of lens sutures  Seldom impairs vision  Gene located at chromosome 17q

20.  Autosomal dominant  Multi coloured dot like opacities  Genes located at 17q and 22q

21.  Autosomal dominant  Complete opacification of lens  Usually bilateral and often begins as lamellar or nuclear cataract  Gene located at chromosome 10q  Mutated gene Pitx 3

22. Anterior segment disorders  Aniridia  Anterior segment dysgenesis  Peter’s anomaly  Microcornea  Microphthalmia  Coloboma  Posterior lenticonus

24. Posterior segment disorder  Mittindorf’s dot  PHPV  Retinitis pigmentosa  Lebers congenital amaurosis Contd..

25.  Chondrodysplasia punctata (AD, AR or x-linked)  Hallerman -shrief syndrome (AD or AR)  Myotonic dystrophy (AD)  Neurofibromatosis type II (AD)

26.  Stickler syndrome (AD)  Bardt- Biedl syndromes (AR)  Cockayane syndrome (AR)  Usher disease (AR)  X - linked - Alport’s syndrome  Marfan’s syndrome Contd..

27.  Galactosemia (AR)  G6PD deficiency (AR)  Hypocalcemia (X-linked)  Lowe syndrome (X-linked)  Fabry disease (X-linked)

28.  Down syndrome (Trisomy 21)  Trisomy 10q, 13, 18 & 20p  Turner syndrome (XO)  Chromosome translocation 3:4, 2:14, 2:16

29.  Maternal illness  Maternal drugs  Maternal nutrition  Prematurity  Radiation  Photocoagulation  Steroid intake  Trauma Acquired

30.  Intrauterine infections caused by Rubella virus, Toxoplasmosis, Cytomegalo virus, Herpes Zoster and Simplex

31.  Caused by the virus getting into the developing lens  Characterised by central nuclear cataract usually bilateral  Associated ocular findings are microcornea, glaucoma, keratitis and retinopathy  Systemic associations are deafness and mental retardation

32. 70% of cases were bilateral  Mean age at 1st presentation 5.5 yrs  60% of children had a manifest squint  44% of children had nystagmus

33.  More complex than in adults  Amblyopia is imminent in infants  Aphakic correction and amblyopia therapy more difficult than cataract itself  Parents are often unable to attend for follow up

34.  History  Family history  Antenatal and perinatal  Birth weight  Mile stones  Onset- noticed by whom? What?

35.  Ophthalmic examination  Laboratory investigation  Paediatrician evaluation

36.  Visual acuity  Fixation pattern  Nystagmus  Strabismus

37.  Anterior segment  Corneal diameter  Size, location & density of lens opacities  Diffuse light examination  Red reflex test  Slit lamp and Fundus Examination  EUA if necessary

38.  Try to ascertain the time of onset  Rule out PHPV,retinoblastoma and injury  Evaluate the motility  USG for post segment pathology  Good prognosis if there is good central fixation, no deviation, good convergence movement

39.  Healthy child - No investigations  Sick child  TORCH for IgM  Reducing sugars  Other Investigations -SOS  Routine investigations for general anesthesia

40.  In all Syndromes  In all Sick Children  In all Mentally retarded children  Ectopic and Spheroaphakic lenses  Assessment for general anesthesia  Genetic analysis if possible

41. General Considerations  Small eye  100% PCO  Increased inflammatory response

42. General considerations  Low Scleral rigidity  Elastic anterior capsule  Post operative visual rehabilitation  Long term follow up

43. Basic Principles  Nucleus is soft in nature  Can be aspirated with Simcoe cannula, I & A tip of phaco machine or by Vitrectomy instrument  No phacoemulsification or nucleus expression is necessary  ICCE is contraindicated

44.  Lensectomy with Anterior vitrectomy(AV)  ECCE + PCCC + AV  ECCE + PCCC + AV + IOL  ECCE + IOL  ECCE

45.  Choice of surgical procedure depends upon age, type of cataract, other ocular & systemic disabilities & socio- economic background of the parents

46.  90% of the growth of eye ball is complete during the first 18 months after birth (Gordon Donzis)

47. Less than 6 Months  Lensectomy + vitrectomy with posterior capsulectomy either central or total especially in sick infants,cat with other ocular anomalies, Membranous or in calcified type  Other cases – ECCE with PCC  Aphakic correction with spectacles or contact lenses as early as possible

48. 6 months – 2 years  ECCE with PCC /or Lensectomy with 4mm posterior capsulectomy depending upon the type of cataract  Aphakic correction with spectacles or contact lenses  Unilateral cataract; ECCE PCC with IOL

49.  2 – 5 years  ECCE with or without central posterior capsulectomy +/- Primary IOL  Above 5 years  ECCE with Primary IOL

50.  Children < 2yrs  Do biometry and undercorrect by 20% Or use axial length only Axial length(mm) Power (D) 1728.00 1827.00 1926.00 2024.00 2122.00  Children between 2-8 yrs  Do biometry and undercorrect by 10%

51.  Infant & toddler: within a week in equally dense cataracts  Older child: If IOL is decided atleast 2 months after  To watch the performance in both eyes and decide in unequally dense cataracts

52. Unilateral Cataract  Prognosis not good  Options are minimal  Influencing factors on decision making manifold

53.  Up to 2 years once in three months  2-5 years once in 6 months  After 5 years 6 months / yearly

54.  Visual acuity,Visual axis  IOP, Fundus examination  Strabismus and amblyopia

55.  Depends upon  Individualized approach  Suitable technique  Implanted lens power  Careful postoperative monitoring  Recognition of PCO & its management  Overall co-operation from patient & parents

58.  Usage of Intraocular lenses in infants  Newer Viscoelastics  Better intraocular lenses  Dyes for visualisation of capsule  Contact lenses  Surgical techniques

60. Array Multifocal IOL

61. Normal PMMASquareedge IOL

62. AT-45 CrystaLens

63.  To achieve emmetropia in extremely short eyes  To avoid explantation of primary IOL’s in children

64. Morcher Iris diaphragm lens

65.  Foldable acrylic IOL for implantation into the capsular bag through a microincision  Optic diameter:5.5mm  Length:11mm AcriSmart 48S Thin OptX