Download paediatric cataract management

PAEDIATRIC CATARACT AND
MANAGEMENT
Presenter
Maj Kunal Dhole
Moderator
Col Vijay K Sharma
Reference
• Parsons’ Diseases of the Eye: 23rd Edn
• Atlas of Pediatric Cataract: S K Khokhar
• Pediatric Cataract Management: AIOS CME Series 26
INCIDENCE
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Pediatric cataract is an treatable leading cause of childhood blindness.
It accounts for 7.4%–15.3% of pediatric blindness
The incidence ranges from 1.8 to 3.6/10,000 per year
The prevalence of childhood cataract has been reported as 1 to 15 cases in
10,000 children in the developing countries .
• It is estimated that globally, there are 200,000 children blind from bilateral
cataract.
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Congenital cataract is associated with ocular abnormalities in 27% of cases
and with systemic abnormalities in 22% of cases.
ETIOLOGY
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The main causes of infantile cataract are genetic, metabolic disorders, prematurity
and intrauterine infections.
Other causes of childhood cataract in older children include trauma, drug-induced,
radiation and laser therapy for ROP.
Trauma is one of the commonest causes of unilateral cataract in the developing
countries.
Bilateral cataracts occur commonly due to the long-term use of topical or systemic
steroid.
Inherited cataracts contribute significantly in the aetiology of childhood cataracts.
Approximately, half of the families have mutations in crystallins and a quarter have
mutations in connexins.
Zonular cataract is the commonest type of congenital cataract
PEDIATRIC CATARACT
• Intrauterine infections • Metabolic disorders
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Systemic Association
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Lowe syndrome
Alport’s syndrome
Apert’s syndrome
Cockayne syndrome
Incontinentia pigmenti
Aniridia
Coloboma
Persistent hyperplastic
primary vitreous
Myotonic dystrophy
Potter syndrome
Weil-Marchesani syndrome
Rubella
Rubeola
Varicella
Poliomyelitis
Cytomegalovirus
Toxoplasma
Herpes simplex
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Galactosemia
Fabry’s syndrome
Wilson’s disease
Diabetes mellitus
Refsum’s disease
Multiple sulfatase
deficiency
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Morphological classification of pediatric cataract based on
location of opacity
ETIOLOGY
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Idiopathic
Gene mutation: α crystalline
Trauma
Prematurity
Secondary Cause
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Inflammatory: Uveitis
Infection: Rubella
Genetic: Trisomy 21, 13 & 18
Metabolic: Galactosemia
• Iatrogenic
 Radiation
 Systemic steroides
 Laser therapy
TOTAL CATARACT
Sporadic or Hereditary
Downs or Congenital Rubella
Early
Intervention
NUCLEAR CATARACT
• At early stage of life
• Progressive
• Visualy significant
Maternal
infection
CENTRAL PULVERULENT
CATARACT
Autosomal dominant
Central biconvex opacity within
embryonic nucleus
Mostly non progressive
No major visual significance
ZONULAR CATARACT
Embryonic or foetal nucleus
Aproxx 50% visual significant
cataract
PUNCTATE CATARACT
Most common manifestation
When crowded in Y suture
a/k/a bluecataract
dot cataract
Cerulean
Anterior axial embryonic cataract
SUNFLOWER
CATARACT
ANTERIOR SUBCAPSULAR
CATARACT
SEEN IN WILSONS DISEASE
OIL DROPLET CATARACT
Infant with
GALACTOSEMIA
Posterior subcapsular, small
nuclear, cortical opacification
SNOWFLAKE CATARACT
seen in a case with
diabetes mellitus
ANTERIOR POLAR
ANTERIOR SUBCAPSULAR
ANTERIOR LENTICONUS
Seen in Aniridia
RB, EDS
Associated with Uveitis, trauma, irridation,
atopic dermatitis
Bilateral seen in Alport Syndrome &
Waardenburg Syndrome
Seen around puberty
CORONARY CATARACT
In deep layer of cortex hidden by iris
CORONA, CLUBED SHAPED OPACITY
Vision usually not
affected
WORKUP
• first symptom is a white or partially white reflex noted by the
parents.
• Strabismus may be the initial manifestation, especially in unilateral
cases, or poor visual fixation
• A history from the parents is useful to understand whether the
cataract is congenital, developmental or traumatic in origin.
• It is a must to screen parents and siblings to rule out familial causes.
• Ascertain any history of maternal drug use, infection or exposure
during pregnancy.
• A thorough ocular and systemic examination is must in every child.
WORKUP
• Ocular examination: visual acuity assessment, pupillary response and
ocular motility.
• The slit lamp biomicroscopic examination: to evaluate the size,
density, and location of cataract to plan the surgical procedure
• Fundus examination
• A-scan: to measure the axial length for calculating IOL power and
monitoring the globe elongation postoperatively.
• B scan: to rule out any posterior segment pathology (retinoblastoma, PPHV, RD)
• Each child should be examined by a pediatrician for thorough
systemic work up
VISUL ACUITY ASSESSMENT
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Preverbal children
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1–2 years of age
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visual-evoked response (VER),
Catford drum
optokinetic nystagmus
Teller’s acuity Cards
Worth’s ivory ball test
Boeck’s candy test
Screening Test for Young Children and
Retards (STYCAR)
Cardiff’s acuity test
2–3 years age
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miniature toy test
Coin test
LEA symbols® test
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3–5 years
 Allen’s picture card
 Lippman’s HOTV test
 letter test
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more than 5 years
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Tumbling E
Landolt’s broken ring
Snellen’s chart
LogMAR chart
LAB-WORKUP
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Fasting blood sugar
urine for reducing substance for galactosemia
urine amino acids for Lowe’s syndrome.
Plasma phosphorus
red blood cells transferase, galactokinase levels
Sr calcium for hypothyroidism
The titres for toxoplasma, rubella, cytomagalovirus and herpes
simplex (TORCH titres)
TIMING OF SURGERY AND AMBLYOPIA
• Once visually significant cataract is detected → early as possible.
• In symmetrical bilateral cases, the second eye should be operated on
within one to two week of the first.
• When there is significant asymmetry, the denser cataract is generally
removed first; surgery on the second eye may then be deferred until after
the first eye receives optical correction
• Unilateral cataracts should be operated within first 6 weeks of life to
prevent development of deprivation amblyopia
TIMING OF SURGERY AND AMBLYOPIA
• Most polar opacities (particularly those involving only the
anterior capsule), smaller nuclear cataracts, and lamellar
cortical opacities that transmit light centrally usually can be
left alone at least until the child reaches a developmental
stage that permits reliable assessment of visual potential &
outcome
UNILATERAL
LESS THAN 6 WEEKS
BILATERAL
WITHIN 10-12 WEEKS
LATERALITY
PAEDIATRIC
CATARACT
INSIGNIFICANT
WAIT & WATCH
VISUAL
SIGNIFICANNCE
SIGNIFICANT
EARLY SURGERY
WHEN TO INTERVENE
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Dense cataract
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Strabismus or Nystagmus
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Central(3mm or more)
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posterior cataract covering visual axis
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Unilateral Dense cataract : at 4 – 8 weeks
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Bilateral dense cataract: 2-3 months
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Poor fixatation after 08 weeks
IOL or NO IOL
• Less than 02 yrs
– Unilateral: CONTACT LENS : RGP lenses
: Silicon Elastomer lenses
: Hydrogel
– Bilateral: SPECTACLES → less than 03yrs : only near correction
→ more than 03yrs : bifocal, add +3.0D for near correction
• More than 02 yrs
– IOL insertion
In infants, implantation of IOL still remains controversial and several surgeons prefer to leave
the infants aphakic after cataract surgery.
The findings of this cohort study :- intraocular lens implantation does not confer
better vision or protection against postoperative glaucoma
- conversely increases the risk of requiring early reoperation in children younger
than 2 years with bilateral or unilateral cataract.
- the routine use of intraocular lens implantation in this age group cannot be
recommended
Compliance
& hygiene
Spectacle
Contact Lens
Difficult to
fit contact
lens
Dirty living
condition
Avaibality
of CL /
glasses
Dry eye
Cost
• IOL MEASURMENT
• FORMULAS
• BIOMETRY IN INFANTS
AXIAL LENGTH
The eye of an adult is 40 to 50% larger than that of a child
16.6-17mm
18.23mm
23.6mm
(at birth)
(at 3 months)
(at 15 yrs)
AL increased @ 0.18mm/week until 40 weeks followed by 0.15mm/week until 3 months
Parameters
Axial length
At Birth
17mm
Adult
23-24mm
Lens diameter
Capsular bag size
6mm
7mm
9.3mm (at 16yrs)
9mm (at 2 yrs)
AXIAL LENGTH
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Obtaining Readings
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EUA
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Conventional A Scan U/S biometry
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Applanation
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Immersion
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Optical biometry
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Partial Coherence Interferometry
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Very accurate
• Requirs pt cooperation
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Not viable option in Infants & young child
AXIAL LENGTH
MORE TIME REQUIRED
CONVENIENT
ACCURATE WITH
TRAINED
TECHNICIANS
LOW RISK OF
ERROR
CORNEAL COMPRESSION
MORE
ACCURATE
0.32 MM ERROR RATE
APPLANATION
IMMERSION
AXIAL LENGTH
• Axial length error
• 1.0 mm error: average 3.0 D
– In long eyes:
1.75 D
– In shot eyes:
3.75 D
KERATOMETRY
• Keratometry steeply reduced in first
6months
• - 0.4 D/month
• - 0.14 D/month for next 6
months
• - 0.08D/month in 2nd year
• Birth 53-56 D → Adult 42- 44 D
• reaching the adult range at
about 3 years of age
• Values are obtained with hand
held auto keratometer
• General anaesthesia
IOL POWER CALCULATION-AXIAL LENGTH, ACUITY AND
REFRACTIVE AIMS
• In adults, modern theoretical formulas are accurate within
approximately 0.5 diopters (D); in children various studies
have found mean absolute errors of between 1.08 and 1.4D
• Holladay 1, Holladay 2, SRK and SRK/T
• More than 8 yrs : emmetropia
• 2-8 yrs: 10 % under correction
• Les than 2yrs: 20 % under correction
WHAT IOL POWER TO USE
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On the basis of axial length (Dohan et al, J cataract refract Surg 1997)
Axial length
IOL Power
17mm
28.0 D
18mm
27.0 D
19mm
26.0 D
20mm
24.0 D
21mm
22.0 D
CALCULATED POST OP REFRACTIVE GOAL
AGE
POWER
1
+6
2
+5
3
+4
4
+3
5
+2
6
+1
7
PLANO
14
0
Enyedi et al
IOL POWER CALCULATION FORMULAE
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All formulas presently avbl are developed for adult eyes
In children which formula to be used
Vanderveen et al: evaluated (Infant Aphakia Treatment Study;2013)
 Hoffer Q, Holladay 1, Holladay 2, SRK, and SRK/T formulae in infants that received IOL
implantation at age 7 months or younger
 Holladay 1 formula showed the lowest median absolute prediction error
 Holladay I and SRK T formula gave good comparable results
Andreo and coworkers found
 All formulas were less accurate for shorter axial length
 HOFFER Q lowest error 1.4 D
 SRK II highest error of 1.8 D
Trivedi et al.
 children who underwent IOL implantation at a mean age of 3.56 years
 In this study there was a low mean absolute error of 0.68–0.84 D with the Holladay 2
•SURGERY
CHALANGES
• Low scleral rigidity,
• Increased elasticity of the anterior capsule
• High vitreous pressure
HOW DOES PEDIATRIC CATARACT SURGERY DIFFER
FROM ADULT
• Intra-operative:
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scleral collapse,
vitreous pressure
highly elastic anterior and posterior capsule for rhexis
Miosis
fibrin release
• Post-operative:
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Uveitis
visual axis opacification (VAO)
secondary membrane formation
Amblyopia
myopic shift.
Surgical Technique for removal of Paediatric Cataract
TECHNIQUE
METHOD
ADVANTAGE
Lens
Aspiration
≥ 06 yrs
Limbal route, single or
two port bimanual
technique, 5mm anterior
rexis
Versatile,
manoeuvre in
poorly dilated pupil,
can implant IOL in
bag
Theoritical risk
of astigmatism
Preffered method
when IOL to be
implanted
Reduce chance of
PCO
Risk of CME
Prefered in infant
less than 2 yrs
No PCO
Incarceration of
vitreous in
scleral incision,
risk of RD
Prefered in
neonates
Limbal route, 5mm
Lens Aspiration
anterior rexis, 4mm post
with anterior
rexis with anterior
vitrectomy
vitrectomy
DISADVANTAGE
INDICATION
2-6 yrs
Lensectomy
≤ 02 yrs
Limbal/pars plana route,
lens is compleely eaten
away with vitrectomy
cutter
CURRENT SURGICAL TECHNIQUES
• Incisions
 because of elastic sclera even the corneal tissue is less likely to selfseal in children.
 Recommended to suture closure of tunnel wounds and paracentesis
openings
• Anterior capsulorhexis
 A cohesive viscoelastic like sodium hyluronate 1.4% is recommended
for pediatric cataract surgery to facilitate anterior capsulorrhexis as
they maintain anterior chamber stability
CURRENT SURGICAL TECHNIQUES
• Cataract removal:
 The lens material may be removed using phaco aspiration, or automated
irrigation and aspiration
 membranous or calcified cataract may need phacoemulsification
• Posterior continuous curvilinear capsulorhexis (PCCC):
 Visual axis opacification (VAO) is the most common complication after a
successful cataract surgery in children
 Manual PCCC with the help of cystitome and forceps is preferable over other
methods.
 Vitrector assisted posterior capsulotomy is also done in selected situation
 Size should be 4 – 4.5mm aprox 1mm smaller than anterior capsulorexis
Posterior capsulorhexis.
(a) Anterior chamber is formed with
viscoelastic devices
(b) Nick is given to posterior capsule
(c) Capsulorhexis started using
microincision forceps
(d) Capsulorhexis is completed
CURRENT SURGICAL TECHNIQUES
• Anterior vitrectomy
 Anterior vitreous acts as a scaffold and helps in lens epithelial cell
migration and proliferation.
 The vitrectomy may be performed using limbal or pars plana route
• IOL insertion
 IOL can be implanted in eyes with AL >17 mm and white to white
distance >10 mm
 Insertion of IOL in smaller eyes is associated with more complications
POSTOPERATIVE MANAGEMENT
• Post op a child’s eye tends to show more tissue reaction.
• The inflammatory response can be managed with the use of
intensive topical steroid (as frequently as six to eight times a day).
• The steroids are tapered over a period of 6 to 8 weeks.
• Topical antibiotics are instilled three times a day for 10 to 14 days.
• Homatropine eye drops (2%) twice a day or atropine eye ointment
once a day should be used for about four weeks to prevent
posterior synechiae formation.
POSTOPERATIVE AMBLYOPIA MANAGEMENT
Age in
Months
Patching Required
Age in
Months
0-1 month
No patching required
1-2 yrs
1:1
1-2 months 1-2 hrs /day
3 yrs
1:2
2-4 months 2-3 hrs /day
4 yrs
1:3
4-6 months Upto 50% waking hrs
5 yrs
1:4
6-12
months
Upto 80% waking hrs
Patching Required
(Affected : Normal)
POSTOPERATIVE COMPLICATIONS IN PEDIATRIC CATARACT
SURGERY
• Uveitis
 Postoperative uveitis (fibrinous or exudative) is a common
complication due to increased tissue reactivity in children
• Posterior Capsular Opacification
 Posterior capsular opacification is the most common complication
after cataract surgery with or without IOL surgery in children
Postoperative Complications in Pediatric Cataract
Surgery
• Glaucoma
 The incidence of glaucoma following pediatric cataract surgery varies
from 3% to 32%
 Glaucoma occurring soon after surgery is usually due to pupillary block
or peripheral anterior synechie Formation
POSTOPERATIVE COMPLICATIONS IN PEDIATRIC CATARACT
SURGERY
• Secondary Membrane Formation
 Secondary membranes are common after infantile cataract
surgery and traumatic cataract.
 Nd: YAG laser capsulotomy is sufficient to open them in
the early stage
THANK YOU