Pediatric (congenital, developmental) cataract and management - pediatric ophthalmology

Congenital
Cataract
Dr Shruti Laddha

Ophthalmology Department ,JJ group of hospitals

Embryonic
development

Embryonic development of human lens

• Day 25 – optic vesicle forms from forebrain
• Day 27-lens plate
• Day 29 –lens pit
• Day 33- lens vesicle
• Day 35-primary lens fibres(posterior epithelium)
• 7 weeks- secondary lens fibres(equator )
• 8 weeks- Y shaped sutures
• 3rd month- zonular fibres secreted by ciliary epithelium
(Pax6 and Sox2 genes, in the optic vesicle, induce the surface ectoderm to form the lens
placode, which invaginates and forms the lens vesicle)

Formation of lens fibres

Sutures are formed by
overlapping of secondary
lens fibres in each shell
Erect Y appear on the
anterior surface of fetal
nuclei

How the eyeball of
newborn differs from
adult
 length of eyeball 16.5
 volume 2.8ml
 ciliary body very small and cellular
 ciliary process touch the iris
 ora serrta develops by 7 yrs
 lens diameter at birth -6mm
 20 yrs –adult size 8 mm
 corneal diameter 10 mm
 AC depth 2mm
 dilator pupillae not so well developed

Pediatric cataract
5-20 percent blindness worldwide
Disrupt the development of immature visual pathyway
Timely removal and rehabilitation is of utmost importance

Pediatric cataract
Congenital Developmental Traumatic

Congenital cataract
Present at birth
Situated within the fetal nucleus
diametre of the opacity less than 5.75 mm (diameter of
newborn lens )

Congenital cataract : bilateral
Genetic
mutation
Autosomal
dominant
Metabolic
Galactosemia
Lowe
Hypoparathyro
idism
Fabry
Infective
TORCH
Chromosomal
Trisomy 21
Trisomy 18
Trisomy 13
Skeltal
Hallerman
streiff francois
syndrome
Ocular
syndromes
Aniridia
Anterior
segment
dysgenesis
Idiopathic
In 50
percent

Congenital cataract :unilateral
Ocular anomalies like persistent fetal vasculature (most common)
no family history
Cause identified only in 10
percent
Usually infants fullterm
and healthy

Syndromic cataract genes

Locus Gene OMIM Cataract inheritance Syndrome inheritance Syndrome Reference of only cataract
affection
4p16.1 WFS1 614296 AD AD Wolfram-like syndrome
(no cataract is associated
with this syndrome)
Berry et al., 2013
7q34 AGK 212350 AR AR Sengers syndrome Aldahmesh et al., 2012
8q13.3 EYA1 601653 AD AD brachio-oto-renal
syndrome-1, nystagmus
Azuma et al., 2000
Syndromic genes only with cataract

Only cataract gene

Cataract genes and anamolies

• In some cases, the description corresponds to the name of the author who first
described the cataract
• Marner cataract, a cataract with sutural opacity
• to the name of the affected family such as Coppock cataract, an embryonic nuclear
cataract
• the Volkman cataract, a central or zonular variety with opacities in the embryonic, fetal,
and juvenile nucleus
• to the affected community such as the Hutterite cataract.
• In other cases, anatomic localization is used to establish the cataract definition, i.e.,
subcapsular, nuclear, sutural, cortical, fetal, embryonic, or capsular cataract

Classification of congenital
cataract
Punctate (blue dot) cataract
Anterior capsular cataract
-anterior polar
-pyramidal
-reduplicated
 posterior polar cataract
Nuclear cataract
Coronary
fusiform cataract
Zonular /lamellar cataract
Total cataract – soft
- membranous

Punctate (blue dot)cataract/catracta coerulea
• Most common
• Autosomal dominant
• Multiple tiny blue dot spots all over the lens especially the cortex

• Variant – cataracta centralis purvulenta
opacity in embryonic /fetal nuclei
• Non progressive ,visually insignificant

Zonular /lamellar cataract
• 50 percent of visually significant congenital cataract
• may be congenital/developmental

Zonular cataract
• Zone around embryonic nucleus (surrounding fetal
nucleus) become opacified,extend depending on
duration of inhibiting factor
• Opacity sharpely demarcated
• Area surrounding opacity clear

Zonular cataract
• Linear opacities like spoke of a wheel (called riders)
• Bilateral
• Just before /after birth
• Fill pupillary aperture
• Often autosomal dominant
• Associated with hypovitaminosis D or hypocalcemia

Coronary cataract
• Puberty
• Deeper layer of cortex and superficial layer of
adolescent nucleus
• Corona or club shaped near periphery
• Non progressive
• Does not interfere with vision

Nuclear cataract
• Rubella
• Embryonal
• Progressive ,becomes total
• Associated –
microphthmos,nystagmus,strabismus,iris
hypoplasia,pigmentary retinopathy

Anterior capsular
cataract
Anterior polar (flat) Anterior pyramidal reduplicated
-delayed formation of
anterior chamber
-after
perforation(acquired)

Anterior capsular cataract
• Flat type
white plaque ,in central pupillar area less than
3mm diameter
visually insignificant

• Pyramidal type
central plaque like projection
frequently surrounded by cortical opacities
may affect vision

• Reduplicated cataract
subcapsular epithelium may grow between
capsule and corneal opacity
Buried opacity –imprint
Association-pupillary membrane
aniridia
Peters anamoly
anterior lenticonus

Posterior capsular (polar )cataract
• Associated –persistent hyloid remnant(mittendorf
dots)
posterior lenticonus
persistent anterior fetal vasculature
Generally Visually insignificant
With persistent hyloid artery,lens deeply invaded by
fibrous tissue leading to total cataract

Posterior polar cataract

Sutural cataract
• Opacity follows anterior /posterior Y suture
• Isolation/with other opacity

Membranous cataract
• Rare
• Hallermann streiff francois syndrome
• Lenticular material absorbs- residual chalky white
lens matter sandwiched between anterior and
posterior capsule

Morphological Classification
Characterization Etiology and Associations
Cortical cataract Cortical material opacification that does not involve the
lens nucleusThree morphologic subtypes: blue dot
opacities, pulverulent/cerulean and coronary
With punctate changes association with female carrier
of Lowe syndrome
Congenital nuclear cataract Lenticular opacity occurring between the anterior and
posterior sutures
Often associated with galactosemia
Anterior lenticonus Classically an oil-droplet reflex on retinoscopy; cataract
may develop later
Presents bilaterallySeen in association with Alport,
Fechtner, and Waardenburg syndromes
Anterior polar cataract A small, usually central opacity of the front part of the
lens capsule that generally does not grow during
childhood and is typically not visually significant; often
managed without surgery
Derives from incomplete or abnormal separation of the
lens from the surface ectoderm during embryonic
development.Often seen with persistent pupillary
membrane or iris strands connecting to the cataract
Anterior pyramidal cataract
2
A white conical cataract measuring 2.0-2.5 mm at the
base that protrudes into the anterior chamber like a
pyramid
Associated with aniridia

Anterior subcapsular cataract A area of opacity immediately beneath the anterior capsule in the anterior
lens cortex
Can be associated with galactosemia or Wilson diseaseAssociated with other
anterior lens abnormalities, trauma, or atopic dermatitis
Posterior polar cataract Focal opacity of the posterior capsule without other lenticular opacity Can be an early finding in Lowe syndrome
Lamellar cataract Opacity occurring between the nuclear and cortical layers of the lens Maternal hypoparathyroidism or rubella infection in the third trimester can
result in lamellar congenital cataractsSeen in the genetic disorders Bardet-
Biedl, Rothmund-Thomson, and McKusick-Kaufman syndromes
Total/complete cataract Presence of both nuclear and cortical opacity; many times appears as a
white cataract
Associated with trauma in childrenCan be a late finding in Lowe syndrome
Posterior lentiglobus Posterior bowing of the posterior capsule; can occur in the presence
or absence of a posterior capsule defect
-
Posterior lenticonus A localized, cone-shaped change in the posterior lens surface; more
common than anterior lenticonus
Unilateral and axial in locationCataract can develop rapidly with spontaneous
rupture of posterior capsule
Persistent fetal vasculature Presence of a retrolental membrane with or without posterior
involvement.
Not exactly a cataract, rather an opacity behind the posterior capsule of the
natural lens
Posterior subcapsular cataract A thin layer of opacity affecting the posterior lens cortex; the most
common form of secondary cataract
Can be associated with galactosemia, Bardet-Biedl syndrome, Fabry disease,
Alström syndrome, Refsum disease, and neurofibromatosis type II
Sutural/stellate cataract Opacities involving the Y-sutures of the lens Usually visually insignificant
Membranous Natural cortical material absorbed (eg, following a trauma) End-stage cataract; associated with trauma, intrauterine infection (TORCH),
PHPV

Symptoms
White reflex Does not
recognize
objects or
localise light
Unsteady
eyes
Deviation of
eyes
Associated
symptoms of
systemic
disease if
present

Signs
• Does not follows light
• Lenticular opacity
• Nystagmus
• Deviation of eyes
• Other ocular and systemic abnormalities in
cases of rubella nuclear cataract

Diagnosis
• History
duration
family history of congenital cataract
visual status
• Birth history
history and degree of consanguinity
history of maternal infection during first
trimester
gestational age and birth weight
birth trauma
supplemental O2 therapy in perinatal
period
Developmental milestones

Complete ocular examination
UCVA,BCVA
pupillary reaction
intraocular pressure
Direct ophthalmoscopy (Bruckners test)
Indirect ophthalmoscopy
BSCAN
ASCAN
Cover test
Retinoscopy
Early photographs to determine the time of onset of cataract

Laboratory
investigations
• For bilateral cataract
CBC ,RBS,BSL F PP
Serum Calcium and phosphorous
RBC transferase and galactokinase level
TORCH
Hepatitis B virus
Urine analysis for reducing substance for
galactosemia
for amino acids (to exclude Lowe
syndrome in suspected cases)
Unilateral pediatric cataract are generally idiopathic ,so
no need for other lab investigation

???
If not placing the intraocular lens,how to manage
If operating ,whether to implant the intraocular
lens
If yes,when to operate
to operate or not

Non surgical treatment
• Partial cataract < 3mm ,pericentral
• Pupillary dilatation with 2.5 percent phenylephrine and part time occlusion of
good eye
Prolonged cycloplegia with atropine can cause amblyopia
For 1-6 yrs children
If significant amblyopia persist ,cataract extraction should be performed

When to operate??
Bilateral dense
cataract – 4-6 weeks of
age
Bilateral partial
cataract- may not
require surgery or
require at later date
Unilateral dense
cataract –urgent
surgery,aggressive anti
amblyopia therapy
Partial unilateral
cataract –may be
treated non surgically

Surgical management of pediatric cataract
Anaesthesia –deep
sedation/general anaesthesia
children tend to rub eye after
surgery – so wound should be good

Wound construction- depends on type of surgery and which lens is to be put
lensectomy planned,patient to be kept aphakic –two corneal tunnel
if bag is to be preserved and foldable lens to be placed- 3mm corneal tunnel
If bag is to be preserved and rigid iol is to be put – 5.5 mm scleral tunnel dissected,
should be sutured

Viscoelastic substance
negates anterior chamber shallowing that occurs due to low scleral rigidity
and positive vitreous pressure
put adequate pressure in anterior chamber to facilates capsulorhexis
facilates in the bag implantation of lens
removed much easily by trabecular meshwork as compared to adults

Capsulorhexis
26 no needle –
capsulotome,forceps
anterior capsule
,thin,elastic ,tends to
run to periphery

• Aspirated with irrigation aspiration
cannula phaco probe or vitrector

• Primary posterior capsulorhexis and anterior vitrectomy
due to active lens epithelial cell in equatorial region,there is increase risk on posterior
capsular opacification
vitreous act as scaffold for proliferation of lens epithelial cells

• IOL implantation
upto 18 months left aphakic
implanted after 18 months of
age

Selection of IOL
depends on biometry and age of child
Age <2 yrs axial lenght and keratometric reading change rapidly
> 2 yrs changes are slower and moderate
Expected myopic shift ,so aim for undercorrection

Children <2 yrs -undercorrect by
20 percent
children 2-8 yrs old –
undercorrect by 10 percent

• Most surgeon follow Dahans et al simplified approach
based on axial length
• axial length IOL power
• 17 mm 28D
• 18mm 27D
• 19mm 26 D
• 20 mm 24D
• 21mm 24D
• 22mm 23D
• 23 mm 23D
• >24 mm axial length -1 D

• Smallest available size of IOL 5.25 mm(smallest available size)
• Implantation of big IOL in bag
difficulty in dialing in small bag
stretching of posterior capsule
stress on the zonules in the direction parallel to haptic
• <2 yrs sulcus implantation of IOL
• >2 yr in the bag IOL
• Choice of IOL PMMA

• Post operative routine
2 hrly steroid with antibiotic eyedrops prescribed

IOL implantation is contraindicated
active
juvenile
rheumatoid
arthritis
persistent
hyperplast
ic primary
vitreous
(PHPV)
microphth
almia

decenteration posterior synechiae
peripheral anterior
synechiae (PAS) formation
pigment precipitation
IOL optic capture, mostly
due to bag sulcus or sulcus
fixation

• The AcrySof IOL has been demonstrated to maintain good centeration with minimal
inflammation and fits well within small capsular bags*
•
*Nihalani BR, Vasavada AR. Single-piece AcrySof intraocular lens implantation in children with congenital and developmental cataract. J Cataract Refract Surg. 2006;32:1527–
1534. [PubMed]

Aphakic corrections in children
Spectacles Contact lens IOLs

Spectacle correction in
children
Satisfactory in cases with bilateral aphakia
Poor optical quality of high plus lens
Mostly develop good visual acuity

Contact lens
• Better optical correction than spectacles
• Diopteric power can be adjusted
throughout life
• Difficult to manage and costly
• Risk of infections
• Poor follow up
• Impractical

Intraocular lens
• More permanent correction
• Residual refractive error has to be
corrected by spectacles, which can be
adjusted throughout life
• Posterior chamber intraocular lens
implantation is the safe method

Complications
• Intraoperative complications
Anterior chamber tends to collapse
the iris can protrude through the surgical
wounds

Immediate post operative
Anterior post
operative uveitis
High intraocular
pressure
Incarceration of
iris in the wound
Endophthalmitis

Late complications
Glaucoma Chronic iritis
Decenteration
of IOL
Retinal
detachment

Ambylopia
management
• Ambylopia treatment starts soon after surgery ,after the
media is cleared
• Initial treatment must be aggressive
• Occlusion therapy
• Autorefractometres help to determine the residual
refractive error
• Spectacles are prescribed from the age of 4 months

• Bifocal lens should be given to pseudophakic eyes from the age of 3 yrs ,when the child
become verbal
• Unilateral pseudophakics should continue half days patching until 4-5 yrs of age
• After that ,patching time is gradually reduced but should not be abandoned until 10-12
yrs of age
• Cases of bilateral pseudophakia should be followed closely to detect and treat relative
ambylopia

Intraocular lens exchange and alternative options
• Intraocular lens should be considered when a great myopic shift has ocured
• When the pseudophakic becomes 7 D more myopic than the sound eye,the IOL
should be exchanged
• Refractive surgery in children is not yet acceptable options
• An alternative to IOL exchange is to implant an additional negative diopteric power
IOL in posterior chamber to correct the myopia
• The procedure is easily performed when the primary IOL was inserted in the bag

Prognosis
• Visual Outcomes depends on
types of cataract
timing of intervention
quality of life
amblyopia management
Aphakic and pseudophakic children should
be followed throughout childhood and
preferably throughout life

Thank you

Pediatric (congenital, developmental) cataract and management - pediatric ophthalmology

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