Congenital Cataract

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Introduction
Every minute one child goes blind somewhere in the

world. In 1992 WHO estimated that there are 1.5 million children with severe visual impairment (SVI) or blindness (BL) in the world WHO defines the visually impaired (VI) child as having a corrected visual acuity of <20/60 in the better eye, SVI as having a corrected visual acuity of <20/200, BL as having a corrected visual acuity of <20/400.

Gilbert C, Foster A, Negrel AD, Thylefors B. Childhood blindness: a new form for recording causes of visual loss in children. Bull WHO 1993;71:485-489

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 The prevalence of childhood cataracts has been

reported as 1 to15 per 10,000 children Cataract amounts to 10% (7-20%)of preventable childhood blindness

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Classification of Childhood Cataract

Age of onset-

Congenital Cataract(from birth) 2. Infantile Cataract(<2years) 3. Juvenile Cataract(first decade)

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Lambert SR, Drack AV. Infantile cataracts. Surv Ophthalmol 1996;40:427-458.

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Etiological1. Congenital Cataract Inherited(1/3) Associated with syndromes (1/3) Idiopathic(1/3 ) 2.Metabolic Cataract 3.Traumatic Cataract 4.Secondary Cataract(juvenile arthritis and uveitis) 5.Secondary to maternal infections 6.Iatrogenic Cataract
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Etiological Classification of Congenital Cataracts

Isolated Findings

Hereditary
Autosomal dominant Autosomal recessive X Linked Sporadic (one-third of all congenital cataracts)

Modified with permission from Arkin M, Azar D, Fraioli A. 10/26/2013 Infantile cataracts. Int Ophthalmol Clin 1992;32(1):110-111

 Part of Syndrome or Systemic Disease Hereditary With renal disease Lowe's oculocerbrorenal syndrome Alport syndrome (autosomal dominant) With central nervous system disease Laurence-Moon-Bardet-Biedel syndrome With skeletal disease Conradi's syndrome (presence of cataract

indicates worse prognosis) Marfan's syndrome Stippled epiphysis

Modified with permission from Arkin M, Azar D, Fraioli A. 10/26/2013 Infantile cataracts. Int Ophthalmol Clin 1992;32(1):110-111

 With abnormalities of head and face Pierre Robin syndrome Oxycephaly Crouzon's disease Acrocephalosyndactyly (Apert's syndrome) With polydactyly With skin disease Incontinential pigmenti Atopic dermatitis Cockayne's syndrome

Modified with permission from Arkin M, Azar D, Fraioli A. 10/26/2013 Infantile cataracts. Int Ophthalmol Clin 1992;32(1):110-111

 With chromosomal disorders Trisomy 13 (usually die within 1 year) Trisomy 18: Edward's syndrome Trisomy 21: Down's syndrome (often cataract formation delayed until approximately age 10) Turner's syndrome Patau's syndrome With metabolic disease Galactosemia (autosomal recessive) Galactokinase deficiency Congenital hemolytic jaundice Fabry's disease Refsum's disease

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Nonhereditary Prenatal causes

Rubella syndrome Toxoplasmosis Varicella

Cytomegalovirus
Herpes simplex virus Measles Mumps Vaccinia Intrauterine hypoxia or malnutrition

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Modified with permission from Arkin M, Azar D, Fraioli A. 10/26/2013 Infantile cataracts. Int Ophthalmol Clin 1992;32(1):110-111

 Postnatal causes Retinopathy of prematurity Hypoglycemia Hypocalcemia Radiation Trauma Chronic uveitis Diabetes mellitus Wilson's disease Renal insufficiency Drug induced High-voltage electric shock
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 Associated with another ocular abnormality PFV (persistence of fetal vasculature) Microphthalmos Aniridia Retinitis pigmentosa Norrie's disease Colobomas Lenticonus

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 Morphological Types Diffuse/total Anterior polar Lamellar Nuclear

Posterior polar
Posterior lentiglobus Posterior (and anterior) subcapsular

Persistent hyperplastic primary vitreous

Traumatic
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Hiles DA, Carter BT. Classification of cataracts in children. Int 10/26/2013 Ophthalmol Clin 1977;17:15-29.

 Morphological Types Diffuse/total

Hiles DA, Carter BT. Classification of cataracts in children. Int Ophthalmol Clin 1977;17:15-29.
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 Anterior polar

Lamellar

Nuclear

Posterior polar

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 Posterior lentiglobus

Posterior (and anterior) subcapsular

PHPV

Traumatic

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Preoperative work up
Evaluation of Visually Significant Cataracts Informed consent IOL power calculation

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History Evaluation of Visual AcuityVision charts such as Lea Hyvarinen symbol charts and HOTV matching charts are very useful in assessment of visual status in a young child unable to read. Anterior Segment Evaluationportable handheld slit lamp is especially helpful for examining infants and young children. Glaucoma should be ruled out because cataracts and glaucoma are associated with congenital rubella and Lowe syndrome Fundoscopic Examination- EUA B scan- to rule out potential retinal and vitreous pathology
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Serologic screening for congenital infection (TORCH) titers, including testing for rubella and syphilis, is indicated for any infant with bilateral cataracts of uncertain origin. It is particularly important to identify the baby with rubella prior to surgery, because exposure of operating room personnel to lens material containing the virus may cause harm.

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When to operate?

As soon as possible Range from various studies 0 to 14 weeks Ideally with in 4 weeks(bilateral cataract). A study by Birch and Stager suggested that intervention before 6 weeks of age may minimize the effects of congenital unilateral deprivation on the developing visual system and provide for optimal rehabilitation of visual acuity.

Birch et al. (JAAPOS 2009;13(1):67-71) Lambert et al. (JAAPOS 2006;10(1):30-6) Watts et al. (JAAPOS 2003;7(2):81-5)
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Difference in management of adult and pediatric cataract

Adult Age Visual impairment Time of surgery Anaesthesia Surgeon Intra operatively >60 years Gradual Waiting period Local/Topical Ophthalmic day care procedure Capsule less tensile Superotemporal/temporal incision Phaco+IOL in bag Post operative recovery Early with presbyopic correction Pediatric Since birth Significant -amblyopia As soon as possible General anaesthesia Multi disciplinary institutional practice Difference in anatomy Elastic capsule Superior preferred Phaco+PCC+Anterior vitrectomy Refraction changes with near vision correction till stabilisation of eyeball growth
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Conservative treatment
DeVoe stated that it is better to have 20/50 vision

with accommodation than 20/20 vision without accommodation. Bilateral partial cataracts should not be extracted if the visual acuity is better than 20/50 to 20/70.5 When treating eyes with a conservative approach, it is important to prescribe appropriate amblyopia therapy.

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Indications for Treatment

Indications for cataract surgery with or without intraocular lens implantation as reported in the literature include central cataract >3 mm in diameter (visually significant); dense nuclear cataract; Cataract obstructing the examiner's view of the fundus or preventing refraction of the patient, if the contralateral cataract has been removed; and cataracts associated with strabismus and/or nystagmus.

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Birch EE, Stager DR. The critical period for surgical treatment of 10/26/2013 dense, congenital, unilateral cataracts. Invest Ophthalmol Vis Sci 1996;37:1532-1538.

Surgery
Phaco+Posterior Polar capsulorhexis+anterior

vitrectomy+IOL implatation
?PPC+Anterior Vitrectomy PCO formation in children is dense and plaque like difficult to treat with YAG capsulotomy Anterior hyaloid opacification is common

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Informed consent
An essential part of a surgical practice

Parents should be aware of long term follow up

for refractive correction and amblyopia management Complications of surgery;IOL implantation Complications of general anaesthesia

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Intraocular power calculation

Different from adult calculation

Increasing AXL

IOL formula

Target Refraction

K reading

Myopic shift
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Biometry
A-scan contact method or immersion method

(prefered)for calculating the Axial length K readings with hand held keratometer or PAKportable autokeratometer is reliable. Formula- SRKII/SRK-T/Holladay II/Hoffer-Q

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 Children less than 2 years old

Do biometry and under correct by 20%, or Use axial length measurements only Axial length IOL dioptic power 17 mm, 25 D 18 mm, 24 D 19 mm, 23 D 20 mm, 21 D 21 mm, 19 D Children between 2 and 8 years old Do biometry and under correct by 10% >8years Biometry as adults
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Dahan E, Drusedau MU. Choice of lens and dioptric power in pediatric pseudophakia. J Cataract Refract Surg

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 Enyedi et al. (Am J Ophthalmol. 1998;126:772-

81) recommended a postoperative goal of +6 D for a 1 year old, +5D for a 2 year old, +4 D for a 3 year old, +3 D for a 4 year old, +2 D for a 5 year old, +1 D for a 6 year old, plano for a 7 yearold and 1 to 2 D for an 8 year old or older children.
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 In normal children, the growth of the eye affects

both the AL and the cornea curvature. Most of the growth occurs in the first 2 years of life, but there is a smoothly changing curve, with some growth until adulthood. The AL increases from an average of 16.8 mm at birth to 23.6 mm in adulthood, while K decreases from an average power of 51.2 to 43.5 D.7

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 The aphakic refractions of normal eyes can be

calculated from Gordon and Donzis's biometric data.

J Pediatr Ophthalmol Strabismus 1997;34:88-95

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Prediction of change in refraction in pseudophakia

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Calculated Refractive Outcomes for Various IOLs, for a Hypothetical Child with Cataract Surgery at Age 9 Months and a Final Refraction at Age 20 Years

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Advantages and Disadvantages of Various IOL Power Choice Approaches

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Advantages and Disadvantages of Various IOL Power Choice Approaches

IOL Choice Approach
Initial hyperopia

Advantage(s)

Disadvantage(s)

Adult Refraction

Hyperopia will improve as the eye grows. Less myopic shift No spectacle or contact lens needed initially

Initial spectacle or contact lens correction is required

Low myopia or emmetropia, with a possibility of hyperopia

Initial emmetropia

Large myopic shift Myopia, moderate may occur with to high growth of the eye

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Initial myopia

At first, may not require contact lens or spectacle correction to prevent amblyopia

Large myopic shift can be expected with growth of the eye

Myopia, possibly very high

Standard adultpower IOL Unpredictable initial refraction, which can make clinical management much High myopia to more difficult hyperopia

No need to measure the eye

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Intraoperative Period
Risks of general anesthesia Smaller size of the eye Poor dilation of pupil more often associated in

pediatric eyes
Low scleral rigidity Relative size of the pars plana:

The pars plana region in the infant eye is incompletely developed, so the anterior retina lies just behind the pars plicata .
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 Incision and suturing:

As opposed to adult eyes, a superior tunnel is preferable in pediatric eyes (as it provides better protection and, in general, children do not have deep-seated eyes, which would require temporal incision). It is preferable to suture even a self-seal tunnel incision in children as opposed to adults .
Need for high-viscosity viscoelastic for capsular management Difficulty in performing an anterior capsulorhexis associated

with a highly elastic anterior capsule and increased intralenticular and intravitreal pressure
Dense formed vitreous and scleral collapse contributing to

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vitreous upthrust giving rise to raised intravitreal and lenticular pressure, making anterior and posterior capsular management difficult 10/26/2013

 Removal of lens substance rarely requires

phacoemulsification, but the cortex is stickier and adherent than in adults

Need for primary posterior capsule management Need for vitrectomy instrumentation Difficult IOL implantation

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Postoperative Period
Propensity for increased postoperative inflammation Compliance with the use of topical postoperative

medications difficult
Higher risk for opacification of the visual axis

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 Requirement for frequent correction of residual

refractive error, as it is constantly changing due to growth of the eye

Tendency to develop amblyopia and need for

patching
Long-term follow-up important but not always

easily achieved

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Primary IOL Implantation in Infantile Cataract Surgery

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IOL types
Rigid PMMA heparin surface modified

Silicone -3 piece IOL

Pavlovic S, Jacobi FK, Graef M, Jacobi KW. Silicone intraocular lens implantation in children: preliminary results. J Cataract Refract Surg 2000;26:88-95.

Acrylic lenses
Rowe NA, Biswas S, Lloyd IC. Primary IOL implantation in children: a risk analysis of foldable acrylic v PMMA lenses. Br J Ophthalmol 2004;88:481-485.

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 Clinical trials of capsular IOLs, downsized to

approximately 10.0-mm diameter, for children <2 years of age.

Capsular IOLs were defined as flexible open-loop,

one-piece, all-PMMA, modified C-loop designs made especially for in-the-bag placement.
Since this report was published, the AcrySof single-

piece IOL has been introduced and the most common material for pediatric implantation has changed from PMMA to hydrophobic acrylic.
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Wilson ME, Bluestein EC, Wang XH. Current trends in the use 10/26/2013 of intraocular lenses in children. J Cataract Refract Surg 1994;20:579-583.

Recent Trends
Jacobi PC, Dietlein TS, Konen W.

Multifocal intraocular lens implantation in pediatric cataract surgery. Ophthalmology 2001;108:1375-1380. Hunter DG. Multifocal intraocular lenses in children. Ophthalmology 2001;108:1373-1374.

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Trans scleral fixation of IOL

Implantation of a scleral-fixated PCIOL offers an

alternative to placement of an angle-supported ACIOL or an iris-claw implant for visual rehabilitation of aphakic children who are spectacle- and contact lens-intolerant and who lack capsular support. Further studies on these patients will help determine the long-term safety of this technique.

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Biglan AW, Cheng KP, Davis JS, Gerontis CC. Secondary intraocular lens implantation after cataract surgery in children. Am J Ophthalmol 1997;123:224-234.

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Optic Capture
The role of optic capture in preventing VAO in the

absence of vitrectomy is controversial. However, optic capture helps to achieve a well-centered IOL.

Raina UK, Gupta V, Arora R, Mehta DK. Posterior continuous curvilinear capsulorhexis with and without optic capture of the posterior chamber intraocular lens in the absence of vitrectomy. J Pediatr Ophthalmol Strabismus 2002;39:278-287.

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Simultaneous Bilateral Cataract Surgery

Pros Reduction in mortality and morbidity associated with risk of two sessions of general anesthesia Immediate improved visual acuity and early binocular vision Only one admission for surgery and fewer hospital visits Less surgical stress Cons Concern with the risk of bilateral postoperative complications, especially endophthalmitis Difficult to defend medicolegally, especially if serious complications arise Loss of ability to adjust surgical plans for second eye that are based on results from first eye surgery
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Arshinoff SA, Strube YN, Yagev R. Simultaneous bilateral 10/26/2013 cataract surgery. J Cataract Refract Surg 2003;29:1281-

Precautions for Simultaneous Bilateral Cataract Surgery

Consider treating the second eye as a separate

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case (scrub in between the surgeries, use a separate instrument set and separate intraocular medications) but recognize that doing so have not been proven to reduce the risk of bilateral endophthalmitis. Use of intraocular substances such as viscoelastic materials and BSS (balanced salt solution) made by different manufacturers, or from different batches if from the same manufacturer, for each of the two eyes is recommended. Defer the second eye if any intraoperative Arshinoff SA, Strube YN, Yagev R. Simultaneous bilateral 10/26/2013 complication occurs with the first eye. cataract surgery. J Cataract Refract Surg 2003;29:1281-

Amblyopia management
Started within 2 weeks postoperatively with

refraction for distance and near add of +2.5D to +3 D In children <9 to 11 years of age after refraction/retinoscopic examination.
Occlusion therapy of the normal eye in cases of

unilateral congenital, developmental, or traumatic cataract may be needed to reverse or prevent amblyopia in visually immature children.

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Occlusion therapy
Age in years Better eye-occlusion Amblyopic eyeocclusion

0-1 2-3
4-6 >6

3 days 4
5 6

1 day 1
1 1

Occlusion is done during waking hours -full time or part time

References
Pediatric Cataract Surgery-1st edition 2005

M Edward wilson,Rupal Trivedi,Suresh Pandey

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