Constitutes 10-40% of childhood blindness Dandona et al (India)-15% Rahi et al (India)-12% Tailor et al (USA)-15%
Study by Rahi et al (India, blind school) Unoperated cataract-40% Uncorrected aphakia & amblyopia -40% Unsuccessful surgery / post operative complications-20 %
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
Only 60% of Bilateral cataract and 40% of Unilateral cataract can be established with specific etiology Others are undetermined
Congenital cataract Hereditary, genetic Metabolic Secondary Embryodisgenesis Etiology
Hereditary factors Non hereditary factors
Isolated hereditary congenital cataracts Cataracts associated with ocular disorder Cataract associated with autosomal syndrome Cataract associated with metabolic disorder
Autosomal dominant Most common Variable expressivity with high penetrance Different morphology in families and in individuals Autosomal recessive Less common responsible for metabolic disorders
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)
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
Pulverulent Anterior polar Posterior polar Nuclear Lamellar Sutural Blue dot cataract Total cataract
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
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
Conical opacity with apex of the cone projecting into the anterior chamber Measures 2-2.5mm in diameter Usually bilateral Occurs sporaidically
Thinning and protrusion of center of the posterior capsule posteriorly Associated with posterior lenticular opacity Characterised by late onset
Small opacity at posterior capsule Inherited as autosomal dominant and recessive form Can cause gross visual impairment Can be associated with Mittindorf’s dot
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
Inherited as autosomal dominant Lamella of lenticular opacification sandwiched between clear nucleus and cortex Usually bilateral with variable density Gene located at chromosome 2q
Inherited as X-linked trait Opacities of lens sutures Seldom impairs vision Gene located at chromosome 17q
Autosomal dominant Multi coloured dot like opacities Genes located at 17q and 22q
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
Anterior segment disorders Aniridia Anterior segment dysgenesis Peter’s anomaly Microcornea Microphthalmia Coloboma Posterior lenticonus
Posterior segment disorder Mittindorf’s dot PHPV Retinitis pigmentosa Lebers congenital amaurosis Contd..
Chondrodysplasia punctata (AD, AR or x-linked) Hallerman -shrief syndrome (AD or AR) Myotonic dystrophy (AD) Neurofibromatosis type II (AD)
Stickler syndrome (AD) Bardt- Biedl syndromes (AR) Cockayane syndrome (AR) Usher disease (AR) X - linked - Alport’s syndrome Marfan’s syndrome Contd..
Galactosemia (AR) G6PD deficiency (AR) Hypocalcemia (X-linked) Lowe syndrome (X-linked) Fabry disease (X-linked)
Down syndrome (Trisomy 21) Trisomy 10q, 13, 18 & 20p Turner syndrome (XO) Chromosome translocation 3:4, 2:14, 2:16
Maternal illness Maternal drugs Maternal nutrition Prematurity Radiation Photocoagulation Steroid intake Trauma Acquired
Intrauterine infections caused by Rubella virus, Toxoplasmosis, Cytomegalo virus, Herpes Zoster and Simplex
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
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
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
History Family history Antenatal and perinatal Birth weight Mile stones Onset- noticed by whom? What?
Ophthalmic examination Laboratory investigation Paediatrician evaluation
Visual acuity Fixation pattern Nystagmus Strabismus
Anterior segment Corneal diameter Size, location & density of lens opacities Diffuse light examination Red reflex test Slit lamp and Fundus Examination EUA if necessary
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
Healthy child - No investigations Sick child TORCH for IgM Reducing sugars Other Investigations -SOS Routine investigations for general anesthesia
In all Syndromes In all Sick Children In all Mentally retarded children Ectopic and Spheroaphakic lenses Assessment for general anesthesia Genetic analysis if possible
General Considerations Small eye 100% PCO Increased inflammatory response
General considerations Low Scleral rigidity Elastic anterior capsule Post operative visual rehabilitation Long term follow up
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
Lensectomy with Anterior vitrectomy(AV) ECCE + PCCC + AV ECCE + PCCC + AV + IOL ECCE + IOL ECCE
Choice of surgical procedure depends upon age, type of cataract, other ocular & systemic disabilities & socio- economic background of the parents
90% of the growth of eye ball is complete during the first 18 months after birth (Gordon Donzis)
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
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
2 – 5 years ECCE with or without central posterior capsulectomy +/- Primary IOL Above 5 years ECCE with Primary IOL
Children < 2yrs Do biometry and undercorrect by 20% Or use axial length only Axial length(mm) Power (D) Children between 2-8 yrs Do biometry and undercorrect by 10%
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
Unilateral Cataract Prognosis not good Options are minimal Influencing factors on decision making manifold
Up to 2 years once in three months 2-5 years once in 6 months After 5 years 6 months / yearly
Visual acuity,Visual axis IOP, Fundus examination Strabismus and amblyopia
Depends upon Individualized approach Suitable technique Implanted lens power Careful postoperative monitoring Recognition of PCO & its management Overall co-operation from patient & parents
Usage of Intraocular lenses in infants Newer Viscoelastics Better intraocular lenses Dyes for visualisation of capsule Contact lenses Surgical techniques
Array Multifocal IOL
Normal PMMASquareedge IOL
AT-45 CrystaLens
To achieve emmetropia in extremely short eyes To avoid explantation of primary IOL’s in children
Morcher Iris diaphragm lens
Foldable acrylic IOL for implantation into the capsular bag through a microincision Optic diameter:5.5mm Length:11mm AcriSmart 48S Thin OptX