Pediatric cataract

PEDIATRIC

CATARACTPresenter: Dr. Ajay Kumar Singh

Moderator: Dr. Wangchuk Doma

Venu Eye Institute & Research

Centre, New Delhi

INTRODUCTION

Pediatric cataract

Congenital cataract

(present at birth)

Developmental cataract

(develops soon after birth)

Traumatic cataract

INTRODUCTION

Incidence - 6/10,000

10% of childhood blindness

Congenital or Acquired

Visually significant or not

Stable or Progressive

Unilateral or Bilateral

Partial or Complete

Developmental opacity are usually partial & stationary

ANATOMY

Lens - A transparent,biconvex,crystalline structure

between iris & vitreous in a saucer shaped

depression called patellar fossa.

Components Anterior capsule

Anterior capsular epithelium

Cortex

Nucleus

Posterior capsule

CONGENITAL VERSUS DEVELOPMENTAL

Congenital & developmental cataract not synonymous

In widest sense, most forms of cataract (even senile cataract) may be considered as developmental, for human lens grows until late in life

Criteria for congenital cataract

present at birth

situated within fetal nucleus

diameter of opacity less than 5.75mm (diameter of newborn lens)

If size of opacity more than 5.75mm,cataract must be postnatal

Genetic Mutation

Autosomal Dominant

Metabolic

Galactosaemia

Lowe

Hypoparathyroidism

Fabry

Infective

TORCH organisms

Congenital rubella

Chromosomal

Trisomy 21 (Down)

Trisomy 18 (Edward)

Trisomy 13 (Patau)

Skeletal

Hallerman-Streiff-Francois syndrome

Nance-Horan syndrome

Ocular anomalies

Aniridia

Anterior segment dysgenesissyndrome

Idiopathic

in 50%

CONGENITAL CATARACTS: BILATERAL

CONGENITAL CATARACTS: UNILATERAL

Sporadic

no family history/systemic disease

Cause identified in only 10%

Ocular anomalies

Persistent fetal vasculature (most common)

Affected infants usually full term & healthy

MORPHOLOGY

TYPES

Punctate (blue dot) cataract

Anterior Capsular cataract

Anterior polar cataract

Pyramidal

Reduplicated

Posterior polar cataract

Embronal (nuclear) cataract

Coronary cataract/Fusiform cataract

Zonular (Lamellar) cataract

Total cataract

Soft

Membranous

MORPHOLOGICAL TYPES

PUNCTATE(BLUE DOT) CATARACT

Most common type,universal

Cataracta coerulea/blue dot cataract

Multiple tiny blue spots scattered all

over lens esp in cortex

Variant –cataracta centralis

pulverulenta

dominantly inherited,non

progressive

central spheroidal/biconvex opacity

consisting of powdery fine white dots

within embronic /fetal nucleus

Non progressive&visually insignificant

ZONULAR (LAMELLAR) CATARACT

Accounts for 50% of visually significant cataract

May be congenital or occurs at a later stage as development

interfered at a later stage

Zone around embryonic nucleus (usually in area of fetal

nucleus) become opacified,extent depending on duration of

inhibiting factor

Opacity sharply demarcated . Area of lens within & around

opaque zone is clear

Linear opacities like spokes of a wheel (called riders) may run outwards

Usually bilateral,formed just before/shortly after birth

Fill pupillary aperature when pupil undilated, thus affecting vision

Often hereditary (autosomaldominant)

Associated with hypovitaminosis D (with evidence of ricket) or hypocalcemia (with tetany or defective tooth enamel)&maternal

ZONULAR (LAMELLAR) CATARACT

CORONARY CATARACT

Around puberty

Situated in deeper layers of

cortex &superficial layers of

adolescent nucleus

Corona or club shaped

opacities near periphery of

lens ,usually hidden by iris

while axial region &extreme

periphery remain clear

Non progressive & does not

interfere with vision

NUCLEAR CATARACT

Associated with rubella

Incidence more if infection

contracted in 2nd month

Development of lens inhibited

at very early stage

Embyonal nuclear cataract

Progressive ,becomes total

cataract

Associated microphthalmos,

nystagmus,strabismus,glauco

ma,iris hypoplasia &

pigmentary retinopathy

ANTERIOR CAPSULAR CATARACT

May be due to

1. Delayed formation of anterior chamber (Developmental)

2. Acquired (commonly)-follows contact of capsule with cornea,

usually after perforation of ulcer in ophthalmia neonatorum

Three types-

Anterior polar (flat type)

Anterior pyramidal

Reduplicated

Flat type – white plaque forms

in central pupillary area less than

3mm in diameter

visually insignificant

Pyramidal type – sometimes

central plaque projects forwards

into anterior chamber like a

pyramid

Frequently surrounded by area of

cortical opacity

may affect vision


Subcapsular epithelium may grow in between

capsule & cortical opacity

Clear lens fibre subsequently growing from there lay

down a transparent zone between two opacities

Buried opacity is called imprint & two together

constitute Reduplicated cataract

Association–

Persistant pupillary membrane

Aniridia

Peters anomaly

Anterior lenticonus


POSTERIOR CAPSULAR ( POLAR) CATARACT

Associated with persistent hyaloid remnants(mittendorf

dots),posterior lenticonus & persistent anterior fetal

vasculature

Common in minimal degree & visually insignificant

With persistent hyloid artery , lens deeply invaded by

fibrous tissue leading to total cataract

OTHER TYPES

SUTURAL CATARACT Opacity follows anterior /posterior

Y suture

May occur in isolation or

association with other opacity

MEMBRANOUS

CATARACT Rare

Associated with Hallermann-Streiff-

Francois syndrome

occurs when lenticular material

partially /completely reabsorbs

leaving behind residual chalky –

white lens matter sandwiched

between anterior & posterior

capsule

SYMPTOMS OF DEVELOPMENTAL CATARACT

Informant – usually parents

History of white spot in pupillary area

Child is usually brought with history of diminution of vision / does not recognize objects and parents

Unsteady eyes

Deviation of eye

Associated symptoms of systemic disease, if present

SIGNS

Diminished vision (at times it is difficult to establish in very young children)

Lenticular opacity

Nystagmus

Deviation of eye

There may be other ocular and systemic abnormalities in cases of rubella nuclear cataract

MANAGEMENT OF DEVELOPMENTAL CATARACT

Investigations

I. Detailed history

II. Complete ocular examination-I. UCVA, BCVA, pupillary reaction intra-ocular tension, fundus

examination

II. B scan ultrasonography to exclude posterior segment

abnormality like growth/ retinoblastoma

III. A scan to determine axial length of the eye

IV. Retinoscopy, to deremine the refractive status

V. cover test to exclude squint

VI. Early photographs to know onset of cataract

Laboratory investigations:

(For bilateral cases)

Blood Test

Full blood count,Blood glucose(FBS/RBS)

Serum calcium and phosphorus

RBC transferase and Galactokinase levels

TORCH test

Hepatitis B virus

Urine analysis:

For reducing substance for galactosaemia

For amino acids (to exclude Lowe syndrome in suspected cases)

U/L Pediatric cases are mostly idiopathic.

No need of lab investigation


Cataract in childhood not only reduce vision but also interfere

with normal visual development

Timing of surgery ,surgical technique,choice of aphakic

correction & amblyopia management are of utmost

importance to achieve good &long standing results

The inflammatory response to surgical results are more

pronounced in children

Post operative amblyopia management forms an integral part

of visual rehabilitation in children


??????

To operate or not?

If yes, when to operate?

If no, how to manage?

If operating, should one place the IOL?

If not placing IOL, how to manage and follow the child?

NON-SURGICAL TREATMENT

Used for partial cataracts

Partial cataract less than 3 mm and pericentral cataracts

respond

Pupillary dilatation with 2.5% phenylephrine and part time

occlusion of good eye

Cyclopentolate can be added once or twice a day if

required

Prolonged cyclopegia (daily atropine) can induce amblyopia

Pupillary dilatation is reserved for preverbal (1-6 years) with

partial cataracts and borderline amblyopia

If significant amblyopia persists, cataract extraction should

be performed

WHEN TO OPERATE??

1. Bilateral dense cataracts :

- require early surgery at 4-6 weeks of age to prevent

development of stimulus deprivation amblyopia

2. Bilateral partial cataracts

-may not require surgery or require only at a later date

3.Unilateral dense cataract

-urgent surgery is advised within days

-aggressive anti-amblyopia therapy should follow surgery

-results are often poor

-if detected after 16 weeks of age then surgery is

inadvisable because amblyopia is refractory

4.Partial unilateral cataract may be treated non surgically

GENERAL TECHNIQUES

Deep general anaesthesia is required

Pediatric cataracts are soft – lens material can be aspirated

through incisions that are 1-1.5mm long at the limbus ;

can be subjected to lensectomy through pars plana

A larger wound is needed to introduce IOL

Tunnel should be securely sutured to prevent dehiscence of

wound with iris incarceration

SPECIFIC TECHNIQUES

There are two approaches

1. Pars plana approach

2. Limbal approach

Pars plana approach is being abandoned gradually in

favour of limbal approach as limbal approach allows better

preservation of the capsular bag for in-the-bag IOL

placement.

PARS PLANA APPROACH

Indications :

neonates and infants <2years of age

with B/L cataracts

for whom IOL implantation is not intended immediately

ADVANTAGES

Rapid and allows a

permanently clear visual axis

Less complicated post-

operative course

Iris and corneal endothelium

suffer less iatrogenic damage

Two eyes can be operated at

the same time reducing the

risk of relative amblyopia and

second exposure to general

anaesthesia

DISADVANTAGES

Incarceration of the vitreous in

the scleral incisions

Subsequent vitreal tractions

and retinal breaks or

detachments

Insufficiently dilated pupil

hinders lensectomy leading to

too little capsular support for

future IOL implantation

LIMBAL APPROACH

Most versatile technique

Advantages:

visualization of the instruments

the ability to preserve the posterior capsule when

desired, and

the lack of disruption of the vitreous unless planned.

APHAKIC CORRECTION IN CHILDREN

Spectacles

Contact lenses

IOLs

SPECTACLES

Satisfactory only in cases of B/L aphakia

Most develop good visual acuity

Cosmetically not acceptable

Poor optical quality of high –plus lenses

CONTACT LENSES

Better optical correction than spectacles

Dioptric power can be adjusted throughout the life

Difficult to manage and costly

Loss of lenses

Frequent infections

Poor follow up

Thus most impractical

INTRAOCULAR LENSES

The IOL facilitates amblyopia management by providing

a more permanent correction

Implanting an IOL in a growing eye is not an ideal

solution, but it is currently the most practical one.

The aim in the IOL option is to correct most, but not all,

of the aphakia

the residual refractive error has to be corrected using

spectacles, which can be adjusted throughout life.

Posterior chamber IOL implantation is the safe method

Both the biometry and the age of the child determine the

choice of the IOL dioptric power.

Age <2 years- axial length and the keratometric (K)

readings change rapidly

Age 2-8 years- changes are slower and more moderate.

Expected large Myopic Shift

AIM FOR UNDERCORRECTION

SELECTION OF IOL

GUIDELINES FOR THE CHOICE OF

INTRAOCULAR LENS DIOPTRIC POWER

CHILDREN LESS THAN 2 YEARS OLD

• Do biometry and undercorrect by 20%

• Use axial length measurements only:

- 17mm, 25 D

-18 mm, 24 D

-19 mm, 23 D

-20mm, 21 D

-21 mm, 19 D

CHILDREN BETWEEN 2 AND 8 YEARS OLD

• Do biometry and undercorrect by 10%

IOL Implantation in children

After the cataract has been aspirated, an elective posterior capsulectomy-anterior vitrectomy is performed.

Sulcus implantation is easier and also allows an easier explantation- may be done in neonates and infants less than 1 year of age. But with the newer foldable IOLs, in the bag implantation is the preferred technique.

An in-the-bag IOL is more difficult to explant, this option should be chosen for infants above 1 year of age because they are less likely to need an IOL exchange, provided they are undercorrected by 20%.

IOL Implantation in children

In the bag technique requires both anterior and posterior

capsulorrhexises

The IOL haptics are placed in the bag fornices, while the

optic is protruded through both capsulorrhexises to be

captured beneath the posterior capsule remnants.

Tassignon has developed a special IOL called bag-in-the-

lens.

The technique consists of creating an anterior and posterior

capsulorrhexis. The specially designed IOL has, at its

periphery, a groove that contains both anterior and posterior

capsule rims

COMPLICATIONS

Intraoperative complications

-The anterior chamber tends to collapse

- the iris can protrude through the surgical wounds

- the pupil constricts on injury to the iris

Immediate postoperative complications

-anterior plastic uveitis

- high intraocular pressure,

-incarceration of iris tissue in the wound

- endophthalmitis

Late complications

-dislocation of the IOL

- chronic iritis

- glaucoma

-retinal detachment

AMBLYOPIA MANAGEMENT

The unilateral cases are the most difficult to manage

Amblyopia treatment starts soon after surgery, after clarification

of the media.

The initial treatment must be aggressive

Full-time occlusion of the sound eye is carried out for a few

days - 1 day per month of age

Thereafter, occlusion is reduced to half the waking hours.

Autorefractometers help to determine the residual refractive

error

Spectacles are prescribed from the age of 4 months onward.

A bifocal lens with an add of +3.00 is prescribed in the

pseudophakic eye from the age of 3 years, when the

child becomes verbal.

Unilateral pseudophakes should continue with half-day

patches until 4-5 years of age.

Thereafter, the patch time can be reduced gradually, but

should not be abandoned until 10–12 years of age.

After that age, amblyopia management is practically

superfluous.

Cases of bilateral pseudophakia should be followed

closely to detect and treat relative amblyopia.

Intraocular Lens Exchange and Alternative

Options

Exchange of IOLs should be considered when a great

myopic shift has occurred

When the pseudophakic eye becomes 7 D more myopic

than the sound eye, the IOL should be exchanged

Refractive surgery in children is not yet an acceptable

option

An alternative to IOL exchange is to implant an additional

negative dioptric power IOL in posterior chamber to correct

the myopia.

This procedure is easily performed when the primary IOL

was inserted in the bag.

PROGNOSIS Visual outcome depends on

- Type of cataract

- Timing of intervention

- Quality of life

- Amblyopia management

Near normal vision can be achieved in U/L congenital

cataract, provided amblyopia management is aggressive

Binocularity is usually poor

Aphakic & pseudophakic children should be followed

throughout childhood &preferably throughout life

THANK YOU

Pediatric cataract

Health & Medicine

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