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Presented By: Dr. Sahil Thakur
Consultant Incharge: Dr. Suresh Kumar
Terminology
Classification Systems
Epidemiology and Genetics
Pathogenesis
Clinical Presentation
Diagnostic Examination
EUA
Medical Management
Surgical Management
Visual Rehabilitation
Impact of Childhood Glaucoma
Conclusion
According to age of onset
 CONGENITAL GLAUCOMA: Glaucoma exists at birth and usually before birth
 INFANTILE GLAUCOMA: Birth till 3 years of age
 JUVENILE GLAUCOMA: After the age of 3 years
This terminology lacks clear cut demarcation so use is discouraged
 Rare vision threatening heterogeneous group of diseases
 According to Shield’s classification of Childhood Glaucoma
PRIMARY GLAUCOMA SECONDARY GLAUCOMA
Congenital OAG Traumatic glaucoma
Juvenile open angle glaucoma Secondary to intraocular neoplasm
Associated with ocular abnormalities Secondary to uveitis
Associated with systemic abnormalities Lens induced glaucoma
After surgery for congenital cataract
PRIMARY: Due to maldevelopment of aqueous outflow
system
SECONDARY: Damage to aqueous outflow system due to
maldevelopment of some other portion of eye
HOSKIN’S
CLASSIFICATION
ISOLATED
TRABECULODYSGENESI
S
IRIDODYSGENESIS CORNEODYSGENESIS
Peripheral
Mid peripheral
Central
Corneal size
Anterior stromal defects
Anomalous iris vessels
Structural anomalies
Flat iris insertion
Concave iris insertion
Does not include glaucoma in absence of developmental anomaly
HALLMARK OF PRIMARY CONGENITAL GLAUCOMA
ISOLATED TRABECULODYSGENESIS
It is associated with primary congenital or developmental
glaucoma
IRIDO TRABECULODYSGENESIS
Anterior stromal defect
Hypoplasia of iris stroma
Malformation of iris collarette
Absence or marked reduction of crypt layer
Common anomaly seen in Axenfield’s and Rieger’s anomaly
Hyperplasia of iris stroma
Diffuse thickening of iris
Anomalous iris vessels
Persistence of tunica vasculosa lentis
Anomalous superficial vessel
Structural anomalies
Holes, Aniridia and Colobomata
CORNEO IRIDO TRABECULODYSGENESIS
Peripheral corneal lesions extend no more than 2mm into clear
cornea e.g. Posterior embryotoxon ( Axenfeld’s anomaly )
 Mid peripheral or central opacities with thinning of stroma
Microcornea: Rubella , PHPV , Nanophthalmos , Rieger’s
anomaly
Megalocornea: Axenfeld’s syndrome
PRIMARY CHILDHOOD
GLAUCOMAS
SECONDARY CHILDHOOD GLAUCOMAS
IA Primary congenital
glaucoma
IB Juvenile open angle
glaucoma
IIA Glaucoma associated with non
acquired ocular anomalies
IIB Glaucoma associated with systemic
disease or syndrome
IIC Glaucoma associated with acquired
condition
IID Glaucoma following congenital
cataract surgery
Classification developed by members of CGRN and vetted by consensus committee of WGA in
July 2013
IA PRIMARY CONGENITAL GLAUCOMA Isolated angle anomalies
Meets glaucoma definition
Spontaneously arrested cases but with typical
signs of PCG
IB JUVENILE OPEN ANGLE GLAUCOMA No ocular enlargement
No associated ocular anomalies /syndromes
Open angle
Meets glaucoma definition
Axenfeld’s Reiger anomaly
Peter’s anomaly
Ectropion uveae
Congenital Iris Hypoplasia
Aniridia
Oculodermal melanocytosis
Posterior Polymorphous Dystrophy
Microphthalmos
Microcornea
Ectopia lentis
IIA Conditions with predominantly ocular anomalies present at birth may or
may not be associated with systemic signs
IIB Conditions predominantly with known syndromes systemic anomalies or
systemic disease present at birth which may be associated with ocular signs
 Chromosomal disorders
 Connective Tissue Disorders
Marfan’s Syndrome, Weil Marchesani’s Syndrome and Stickler’s Syndrome
 Metabolic disorders
Homocysteinuria , Lowe’s Syndrome and Mucopolysaccharidosis
 Phacomatosis
Neurofibromatosis, Sturge Weber and Klippel Trenuanay Weber Syndrome
 Congenital Rubella
IIC Glaucoma associated with acquired conditions which are
not inherited or present at birth but develop after birth
Uveitis
Trauma
Steroid induced
Tumors
Retinopathy of Prematurity
IOP> 21 mm of Hg plus at least one of the following:
Optic disc cupping
 Progressive increase in VCDR
 Cup disc asymmetry of 0.2 or more
 Focal rim thinning
Corneal findings
 Haab’s striae
 Diameter ≥11 mm in new born
 Diameter > 12 mm in < 1 yr old child
 Diameter > 13 mm any age
Ocular enlargement like Progressive myopia / increasing axial length
Visual field defect consistent with glaucomatous optic neuropathy with no
observable reason for defect
Eyes meeting the CGRN glaucoma definition criteria are subcategorized
into three groups on the basis of age:
Neonatal: 0-1 month
Infantile: 1 -24 months
Late onset or late recognized: > 2 years
DEFINITION OF GLAUCOMA SUSPECT
Any 1 of the following
 IOP> 21 mm of Hg on two separate occasions
 Suspicious optic disc appearance for glaucoma
 Increased CDR
 Increased corneal diameter or axial length in setting of normal IOP
 Supicious visual field defect for glaucoma
INCIDENCE
 Congenital glaucoma is responsible for between 4% and 18% of childhood blindness.
 In developed countries incidence is 1 in 10000 births
 1 in 1250 in Slovakia
Ophthalmologica. 1972;181:61–73
 1 in 3300 in Andhra Pradesh
Arch Ophthalmol. 1998;116:545–6
 In the Toronto experience, a review of 306 children diagnosed with childhood glaucoma,
congenital glaucoma 38% followed by aphakic glaucoma 20% and Sturge–Weber syndrome-
associated glaucoma 10%.
J AAPOS 1999; 3: 308-15.
 In Dallas study 23% of patients had primary glaucoma, 45% had secondary glaucoma, and 31%
were glaucoma suspects.
Clinic Ophthalmol .2013; 7:1739-46
GENETICS
 Sporadic
 Familial patterns show recessive inheritance with variable degree of penetrance and
multi factorial inheritance
 Increased incidence with high consanguinity
Arch Ophthalmol 1998;116(4):545–546
LOCUS LOCATION INHERITANCE GENE
GLC3A 2p21 AR CYP1B1
GLC3B 1p36 AR ?
GLC3C 14q24.3 AR ?
Genomics 1995;30:170-177
IOVS 2002; 43:
3015
GENETIC LOCI FOR PRIMARY CONGENITAL GLAUCOMA
 Although spectrum of mutations in CYP1B1 have been implicated in
PCG
 Very few reported genotype phenotype correlations
 Frameshift and R 390C are associated with severe phenotypes and
poor prognosis
IOVS April 2004 ;45 :1149-56
PRIMARY
CONGENITAL
GLAUCOMA
Faulty atrophy
of mesodermal
tissue
Faulty cleavage of
mesodermal tissue
Excessive collagenous
beams preventing
posterior sliding of iris
and ciliary body leading
to anterior iris insertion
and TM obstruction
Abnormal
migration of
neural crest
cells
TM
Ciliary
muscle
5 months :Iris meets
endothelium
TM & ciliary muscle
overlapping
Allen et al :Arch Ophthalmol 53:783 1955
Cleavage of
mesodermal tissue
Normal development
of the angle
Uveal tract splitting
by cleavage or
atrophy ciliary
muscle is seen
extending into iris
Differential growth rate leading
to slippage of ciliary muscle and
ciliary body posteriorly
 Typically bilateral but significant IOP elevation in one eye can occur in 25- 30 % of
cases
 Commonly between 3-9 months
 Rarely at birth or up to 3 years of age
Site Signs
Cornea Megalocornea
Breaks in Descemet’s membrane
Haab’s Striae
Sclera Increased axial length
Myopia
Astigmatism
Optic Nerve Cupping
IOP Increased
Cornea
Megalocornea
Corneal clouding
Breaks in Descemet’s
membrane
Haab’s Striae
Horizontal or curvilinear striae
formed as endothelial cells lay
down new basement membrane
and hyaline ridges develop
Sclera
Scleral thinning
Blue sclera
Increased Axial
length
Myopia
Astigmatism
Lens
Stretching and rupture of
zonules can cause lens
subluxation
Optic nerve cupping
Rapid and early
Reversible with normalization of
pressure
 To confirm the diagnosis of glaucoma
 Determining the type of glaucoma if present
Initial Evaluation
• Adnexal source of irritation
• Lacrimal system obstruction
• Visual response
• Corneal edema
• Opacification
• Preliminary digital tonometry
IOP evaluation can be done in sleepy infant or during bottle feeding
using:
 Perkin’s Applanation Tonometer
 Tonopen
5-6 yr old child:
 GAT evaluation can be done
Refraction and Perimetry
Myopia
Astigmatism
Visual Fields yield valuable information in older children
Anterior Segment Evaluation
Using portable slit lamp
UBM: to detect any associated anterior segment anomalies
Posterior Segment Evaluation
With direct or indirect ophthalmoscope if there is no corneal
clouding or opacification
B scan USG
EVALUATION UNDER
ANAESTHEISA
Sequence For EUA
Corneal Diameter
Tonometry
Anterior Segment Evaluation
Gonioscopy
Fundus
Refraction
Pachymetry
Axial Length Measurement
To avoid unnecessary anesthesia ophthalmologist undertaking EUA should be able
to perform surgery if indicated
 Haab’s striae
 Corneal edema
 Corneal opacification: 80% of patients
 Posterior embryotoxon
 Megalocornea
 Measurement of Corneal Diameter:
Hold the calipers on first appearance of
white scleral fibers on one side and same
point on other side. Accurate measurement
using plastic gauge with calibrated holes.
Can J Ophthalmology 1985; 20:93–97.
CORNEAL FINDINGS
Age Normal Megalocornea
Term 9.5 -10.5 11.5
1 yr 10-11.5 12-12.5
2yr 11-12 >12.5
Older child <12 >13
Sherwin Isenberg Formula in preterm babies
Corneal Diameter=0.0014 * wt in gm + 6.3
Normal IOP varies with age:
 Mean IOP up to 12 year of age is12.2 +/-0.74 mm of Hg
 Mean IOP < 1 year of age is 8 +/- 2.5 mm of Hg
J Pediatr Ophthalmol & Strabismus 2006; 43: 14
Tonometers
 Perkin’s hand held Applanation Tonometer
 Tonopen
 Schiotz Tonometer should be avoided
Sources Of Error
 Effect of anesthesia
 Corneal surface distortion , edema
 Mackay Marg Tonometer is accurate
IOP MEASUREMENT
ANAESTHETIC AGENTS
AFFECT IOP
IOP
Increased
Succinylcholine
Ketamine
Endotracheal
Intubation
Decreased
Halothane
Oxygen
Nitrous oxide
Sevoflurane
Midazolam
Methohexitol
Only raised IOP in EUA should never be the basis of diagnosis and treatment
Portable Slit Lamp
Corneal findings
Anterior chamber depth
Anatomy of iris
Direct gonioscopy using Koeppe’s type direct
goniolens with hand held slit lamp or
microscope:
Anterior insertion of iris with altered
translucency of angle
Lister’s morning mist: fine fluffy tissue covering
peripheral iris
Lochness Monster Phenomenon: loop of
vessels of major arterial circle can be seen
above iris
Can visualize fundus through the same lens
(in undilated pupil and with corneal edema )
GONIOSCOPY
Type 1 Type II
The Glaucomas,
Sampolesi, Springer, 2013
Possible if corneal edema or opacity do not hinder evaluation
Avoid dilation if surgery is contemplated
Direct ophthalmoscope
Koeppe’s lens can be used with direct ophthalmoscope in
small pupil
Careful drawings and fundus photographs (using hand held
fundus camera )
OPTIC NERVE HEAD
EVALUATION
A scan measures axial length , anterior chamber depth , lens
thickness
Baseline value for comparative purpose
B scan for posterior segment if opaque media precludes the
view
Can be used to visualize retinal or choroidal detachment/
mass lesion / etc.
PARAMETER NORMAL MILD MODERATE SEVERE
Corneal Diameter
(mm)
upto10.5 >10.5-12 >12-13 >13
IOP (mm of Hg) 11.5 11-20 >10-30 >30
Cup Disc ratio 0.3-0.4 >0.4-0.6 >0.6-0.8 >0.8
Last BCVA 20/20 <20/20-20/60 <20/60 – 20/200 <20/400- no PL
Panicker et al IOVS 2004;45 :1149-56
DIFFERENTIAL DIAGNOSIS BASED
ON SYMPTOMS
 Epiphora and red eye
 Conjunctivitis
 Nasolacrimal duct obstruction
Corneal epithelial defect
 Keratitis
 Anterior segment inflammation
 Conditions associated with corneal
edema or opacification
Birth trauma
Congenital malformation
Corneal dystrophy
Keratitis
Mucopolysaccharidosis
Idiopathic
 Conditions showing corneal enlargement
Myopia
Conditions showing actual or pseudo optic nerve
cupping
Physiologically large optic nerve cup
Coloboma
Atrophic optic nerve
Hypoplastic optic nerve
Malformation
Confirm The
Diagnosis Of PCG
Preoperative appropriate
Medical Treatment
Angle Surgery
Goniotomy
Trabeculotomy
Trabeculectomy and
Trabeculotomy
Cyclodestructive
procedures
Follow up
Lifetime
Amblyopia treatment once
IOP contolled
SUPPORTIVE ROLE
Preoperative :clear cornea for angle surgery
Adjunctive modality: in combination with surgery if IOP is
too high
Temporary measure if infant is unfit for surgery
ISSUE OF DRUG SAFETY IN CHILDREN
Smaller plasma volumes
Immature blood brain barrier
Increased receptor sensitivity
Increased systemic absorption
MEDICAL TREATMENT
MEDICATIONS INDICATION CONTRAINDICATION
B BLOCKERS
Non selective- (timolol -
0.1 ,0.25 ,0.5 %)
Selective (for reactive
airways )- betaxolol
1st line
2nd line for older children
Avoid in children with
reactive airways , cardiac
disease
Side effects:
bronchospasm
bradycardia
CAI
(Oral acetazolamide -5-
15mg/kg suspension )
Dorzolamide 2%
Brinzolamide 1%
1st line / 2nd line – young
children
Avoid in children with
corneal transplant
Prostaglandins
Latanoprost 0.005%
Bimatoprost 0.03%
Travoprost 0.004%
2nd / 3rd line Trichomegaly
Redness
Preoperative use – risk
of inflammation
Latanoprost use licensed in Europe since 2010 based on PANDA results
MEDICATION INDICATION CONTRAINDICATION
Miotics Diarrhoea
Interaction with
succinylcholine for GA
Headache ,
proinflammatory
Adrenergic agonist
Epinephrine
Apraclonidine
Brimonidine
Not used
Short term use in infants
and after corneal
transplant
Only in older children 3rd
line or last resort
Lack of efficacy
DO NOT USE IN
INFANTS / SMALL
CHILDREN
Lipophilic drugs –CNS
side effects fatigue ,
somnolence
Definitive therapy in most cases
Western world
 Early presentation with mild corneal edema at referral
 Goniotomy is the treatment of choice
In India / Middle east
 Late presentation
 Goniotomy is not possible
 Trabeculotomy or combined trabeculectomy and trabeculotomy
 OBJECTIVE : Remove the obstructing tissue that causes resistance to
aqueous outflow
 LENS: Swan Jacob lens
 KNIFE: Barraquer knife
Worst knife
Swan spade
Long needle
Reported results show success rate of 80% J AAPOS.2001;5:281–4
GONIOTOMY
Pre-op Preparation
 Adequate miosis
 Facilitates retraction of iris from angle
Steps
 After placing the goniotomy lens over cornea
 Anterior chamber is entered through a clear corneal incision (e.g. nasal
goniotomy – temporal incision is given )
 Knife is advanced towards the opposite angle parallel to iris and away from
pupil and the angle is engaged by the blade tip which is swept
circumferentially incising the nasal angle over 100-110 degree. Falling back of
peripheral iris is the end point of surgery
Failure
Failure of obtaining adequate incision during surgery
Presence of peripheral anterior synechiae
Fibrous proliferation
Success rate of 70 – 93 % in large series after multiple
goniotomies
Ophthalmology 2011;118:236-40
TRABECULOTOMY AB
EXTERNO
Independently described by two surgeons in 1960
Burian: Trabeculotomy ab externo
Smith: Nylon filament Trabeculotomy
Beck and Lynch: 360 degree trabeculotomy using 6-0
polypropylene
Recent: Illuminated Microcathether
Principle: To cannulate Schlemm’s canal from external
approach and then tear through TM into anterior chamber
Limbal or fornix based
Conjunctival flap is made
Hemostasis is achieved with
minimal cautery
Junction of Posterior border of
TM and Sclera is the external
landmark for scleral spur and
Schlemm’s canal – radial
incision is given at this site
Incision is deepened till inner
wall of Schlemm’s canal and
trabeculotome is introduced
Trabeculotome is rotated till
75% of probe is seen in AC
Rotation is reversed and
instrument is withdrawn
Trabeculotome is then
passed into Schlemm’s canal
on other side radial incision
GONIOTOMY VS
TRABECULOTOMYGoniotomy Trabeculotomy
Simple faster procedure Can be done in edematous or scarred cornea
Does not disturb conjunctiva Does not require introduction of sharp
instruments in anterior chamber
Can be repeated one or more times Does not require adaptation to gonioprism
Can be converted to trabeculectomy
COMBINED TRABECULOTOMY AND
TRABECULECTOMY WITH /WITHOUT
MMC
 Trabeculectomy combined with trabeculotomy to increase the long term
success
 Primary CTT : single operative procedure with better control of IOP in some
populations e.g. India
Trabeculotomy vs Trabeculectomy vs Combined procedure
 Favourable outcome with combined procedure but after 2 years advantage
was not statistically significant
Br J Ophthalmol 1999;83:317–322.
 Mandal et al Primary CTT without MMC in299 eyes of 157 patients reported a
success rate of 63.1 % by the end of 1 year
Ophthalmology 2004; 111:283–290
Intraoperative use of mitomycin C has somewhat enhanced the
success of procedure but with significant risk of complications
 Complications with MMC use in children
Thin walled avascular bleb : risk of endophthalmitis
Bleb leaks
Wound rupture
One reported series of retrospective comparison of
trabeculectomy with or without MMC noted no significant
difference in outcome
J Glaucoma 2004;13:228–232
REGULAR FOLLOW UP
For IOP monitoring
Retinoscopy
Amblyopia Therapy
Optical Keratoplasty
SURGICAL SUCCESS
Corneal clarity
Decreased IOP
Pale blebs
Reversal or non progression of disc cupping
Reversal or non progression of myopia
Good visual recovery
Surgical success has been defined as IOP <16 under GA or IOP <
21 mm of Hg with no progression of cupping or corneal diameter
Qualified success: Maintenance of above pressures with single drug
 Total of 148 eyes (85 patients)
 Trabeculotomy, trabeculectomy, or combined trabeculotomy-trabeculectomy (CTT)
were compared: equal success rate
 Overall success rate was 80.4%
 One surgery 105 (70.9%), 34 eyes (23.0%) 2 surgeries, and 7 eyes (4.7%) had 3
surgeries
 A progressive decline in success rate over time was evident, as success rate dropped
from 96.6% at 5 months to less than 50% after 11 years of follow-up
 Surgical outcome of PCG better in infants presenting before 6 months
 Adjuvant topical anti glaucoma medications augment success rate
MANAGEMENT OF
REFRACTORY GLAUCOMA /
FAILED SURGERY
UNFAVOURABLE FACTORS IN PEDIATRIC GLAUCOMA
Low scleral rigidity
Rapid healing process
Exuberant scarring process
Enlargement of glaucomatous eyes with thinning and
distortion of intraocular anatomy
TRABECULECTOMY WITH OR
WITHOUT MMC
Indication
Failed Angle Surgery
Secondary Glaucomas
Success Rate
Variable results in literature
Non Valved
Molteno
Baerveldt
Valved
AGV
Indications
Failed filtration surgery
Failure to control IOP with
medication post surgery
Aphakic glaucoma
Neovascular glaucoma
GLAUCOMA DRAINAGE
DEVICES
AGV vs. Baerveldt vs MMC trabeculectomy in children <2 yrs:
 Retrospective study; Better IOP control with the GDDs than the MMC trabeculectomy
group
 Cumulative success rates of 87% versus 36%, respectively, at 1year and even a
larger difference of 53% versus 19% at 6 years.
Am J Ophthalmol 2004;137 (6):1163–1164
AGV vs MMC trabeculectomy in pediatric aphakic glaucoma:
 Prospective, randomized study
 Higher qualified success in the AGV(67%) vs the MMC trabeculectomy group (40%)
 Complication rates higher in the MMC trabeculectomy (40%) than
AGV(26.7%)(differences were not statistically significant).
Saudi Journal of Ophthalmology, 2011; 25:317–27
CYCLODESTRUCTIVE
PROCEDURES
Indications
 Failed angle surgery with
minimal visual potential
 Failed trabeculectomy /
GDD with poor central
vision
 Anatomy precludes
intraocular glaucoma
surgery
Options
 Trans-scleral cycloablation
(Diode laser )
 Endoscopic diode laser
cycloablation
 Cyclocryotherapy
21 year follow up
53 eyes included
Mean logMAR VA was 0.61 ± 0.57.
Good VA in 51%, Moderate VA in 30%, and Poor VA in 19%
Deprivation Amblyopia (64%) was main cause of visual
impairment with VA <20/50
VISUAL
REHABILITATION
Appropriate spectacle correction
If problem persists for distance
vision even telescopes can be
prescribed
Appropriate training for
recommended device is must
VISUAL
REHABILITATIONRetrospective study of B/L congenital glaucoma
 100 children, mean age 6.3 yrs (1 month-16 years)
 Success rate of early treatment is 70 to 80%
 Only 35% of all patients will have visual acuity better than 20/50, and 2% to
15% will remain blind.
 Regarding the optical prescriptions, 80% were corrected for myopia and
20% were for hyperopia and astigmatism.
 Optical devices for distance were prescribed for 34% of the patients. All of
the optical aids for distance were telescopic manual systems for one eye
only
 Optical aids for near vision were necessary for 6% of the patients, and the
most commonly prescribed were illuminated stand magnifiers of + 38
aspheric diopters
Clinics 2009;64(8):725-30
Consider repeat trab,
Drainage implant,
Cyclodestruction.
Goniotomy/Trabeculectomy/Combined Trab-Trab
Surgical Outcome?
EUA after 3 weeks
IOP controlled
Evaluation after 3 months
Normal IOP
Evaluation after 3 months
FU every 3 months
Record IOP, CDR, VA
Axial length, VF (if possible)
IOP not controlled
Add medical therapy
If IOP not controlled
repeat Trab-Trab
Controlled
VISUAL
REHABILITATION
Uncontrolled
Very poor prognosis
BROAD MANAGEMENT OUTLINE
IMPACT OF CHILDHOOD
GLAUCOMA119 children in the study, all grades of glaucoma
Older children reported less impairment on CVAQC1, IVI-C2, and PedsQL
3than younger children.
Parents reported greater impact on their child's HR QoL than children
reported themselves.
Children with glaucoma report HR QoL scores similar to those described
by children with severe congenital cardiac defects, who have undergone
liver transplants, or who have acute lymphoblastic leukemia.
(1) functional visual ability (FVA) with the Cardiff Visual Ability Questionnaire for Children (CVAQC), (2) VR QoL
with the Impact of Vision Impairment for Children (IVI-C), and (3) HR QoL with the Paediatric Quality of Life
Inventory (PedsQL) version 4.0.
PROGNOSIS OF
CHILDHOOD GLAUCOMA230 patients with 10 year follow up
79 having ocular hypertension with open angles or primary angle closure
(PAC), 35 primary open angle glaucoma (POAG), 50 PAC glaucoma (PACG),
20 primary congenital glaucoma (PCG), 46 secondary glaucoma patients.
15% of primary congenital glaucomas (PCGs) showed a glaucomatous VF
defect after 10 years.
This study provides evidence that routine delivery of care can provide well
controlled IOP in glaucomas, both primary and secondary, and the VF
stabilized in about 90% of patients over a period of 10 years, with the
currently available glaucoma medications and trabeculectomy.
INDIAN PRACTICE
PATTERNS
CONCLUSION
Congenital Glaucoma is a potentially treatable cause of irreversible
childhood blindness provided it is picked up early.
CGRN classification should be done for each case.
EUA is the best method to clinically evaluate the patient.
IOP should be taken immediately after induction with Tonopen or
Perkin’s Tonometer to counter effect of anesthetic drugs.
Management is mostly surgical.
Goniotomy in West and Trab with augumented Trab forms the
mainstay in our setup.
Post operative life long follow up is vital.
Amblyopia should always be treated to improve the QOL of the
patient.

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Understanding Childhood Glaucoma

  • 1. Presented By: Dr. Sahil Thakur Consultant Incharge: Dr. Suresh Kumar
  • 2. Terminology Classification Systems Epidemiology and Genetics Pathogenesis Clinical Presentation Diagnostic Examination EUA Medical Management Surgical Management Visual Rehabilitation Impact of Childhood Glaucoma Conclusion
  • 3. According to age of onset  CONGENITAL GLAUCOMA: Glaucoma exists at birth and usually before birth  INFANTILE GLAUCOMA: Birth till 3 years of age  JUVENILE GLAUCOMA: After the age of 3 years This terminology lacks clear cut demarcation so use is discouraged
  • 4.  Rare vision threatening heterogeneous group of diseases  According to Shield’s classification of Childhood Glaucoma PRIMARY GLAUCOMA SECONDARY GLAUCOMA Congenital OAG Traumatic glaucoma Juvenile open angle glaucoma Secondary to intraocular neoplasm Associated with ocular abnormalities Secondary to uveitis Associated with systemic abnormalities Lens induced glaucoma After surgery for congenital cataract
  • 5. PRIMARY: Due to maldevelopment of aqueous outflow system SECONDARY: Damage to aqueous outflow system due to maldevelopment of some other portion of eye
  • 6. HOSKIN’S CLASSIFICATION ISOLATED TRABECULODYSGENESI S IRIDODYSGENESIS CORNEODYSGENESIS Peripheral Mid peripheral Central Corneal size Anterior stromal defects Anomalous iris vessels Structural anomalies Flat iris insertion Concave iris insertion Does not include glaucoma in absence of developmental anomaly HALLMARK OF PRIMARY CONGENITAL GLAUCOMA
  • 7. ISOLATED TRABECULODYSGENESIS It is associated with primary congenital or developmental glaucoma IRIDO TRABECULODYSGENESIS Anterior stromal defect Hypoplasia of iris stroma Malformation of iris collarette Absence or marked reduction of crypt layer Common anomaly seen in Axenfield’s and Rieger’s anomaly Hyperplasia of iris stroma Diffuse thickening of iris
  • 8. Anomalous iris vessels Persistence of tunica vasculosa lentis Anomalous superficial vessel Structural anomalies Holes, Aniridia and Colobomata CORNEO IRIDO TRABECULODYSGENESIS Peripheral corneal lesions extend no more than 2mm into clear cornea e.g. Posterior embryotoxon ( Axenfeld’s anomaly )  Mid peripheral or central opacities with thinning of stroma Microcornea: Rubella , PHPV , Nanophthalmos , Rieger’s anomaly Megalocornea: Axenfeld’s syndrome
  • 9. PRIMARY CHILDHOOD GLAUCOMAS SECONDARY CHILDHOOD GLAUCOMAS IA Primary congenital glaucoma IB Juvenile open angle glaucoma IIA Glaucoma associated with non acquired ocular anomalies IIB Glaucoma associated with systemic disease or syndrome IIC Glaucoma associated with acquired condition IID Glaucoma following congenital cataract surgery Classification developed by members of CGRN and vetted by consensus committee of WGA in July 2013
  • 10. IA PRIMARY CONGENITAL GLAUCOMA Isolated angle anomalies Meets glaucoma definition Spontaneously arrested cases but with typical signs of PCG IB JUVENILE OPEN ANGLE GLAUCOMA No ocular enlargement No associated ocular anomalies /syndromes Open angle Meets glaucoma definition
  • 11. Axenfeld’s Reiger anomaly Peter’s anomaly Ectropion uveae Congenital Iris Hypoplasia Aniridia Oculodermal melanocytosis Posterior Polymorphous Dystrophy Microphthalmos Microcornea Ectopia lentis IIA Conditions with predominantly ocular anomalies present at birth may or may not be associated with systemic signs
  • 12. IIB Conditions predominantly with known syndromes systemic anomalies or systemic disease present at birth which may be associated with ocular signs  Chromosomal disorders  Connective Tissue Disorders Marfan’s Syndrome, Weil Marchesani’s Syndrome and Stickler’s Syndrome  Metabolic disorders Homocysteinuria , Lowe’s Syndrome and Mucopolysaccharidosis  Phacomatosis Neurofibromatosis, Sturge Weber and Klippel Trenuanay Weber Syndrome  Congenital Rubella
  • 13. IIC Glaucoma associated with acquired conditions which are not inherited or present at birth but develop after birth Uveitis Trauma Steroid induced Tumors Retinopathy of Prematurity
  • 14. IOP> 21 mm of Hg plus at least one of the following: Optic disc cupping  Progressive increase in VCDR  Cup disc asymmetry of 0.2 or more  Focal rim thinning Corneal findings  Haab’s striae  Diameter ≥11 mm in new born  Diameter > 12 mm in < 1 yr old child  Diameter > 13 mm any age Ocular enlargement like Progressive myopia / increasing axial length Visual field defect consistent with glaucomatous optic neuropathy with no observable reason for defect
  • 15. Eyes meeting the CGRN glaucoma definition criteria are subcategorized into three groups on the basis of age: Neonatal: 0-1 month Infantile: 1 -24 months Late onset or late recognized: > 2 years DEFINITION OF GLAUCOMA SUSPECT Any 1 of the following  IOP> 21 mm of Hg on two separate occasions  Suspicious optic disc appearance for glaucoma  Increased CDR  Increased corneal diameter or axial length in setting of normal IOP  Supicious visual field defect for glaucoma
  • 16.
  • 17. INCIDENCE  Congenital glaucoma is responsible for between 4% and 18% of childhood blindness.  In developed countries incidence is 1 in 10000 births  1 in 1250 in Slovakia Ophthalmologica. 1972;181:61–73  1 in 3300 in Andhra Pradesh Arch Ophthalmol. 1998;116:545–6  In the Toronto experience, a review of 306 children diagnosed with childhood glaucoma, congenital glaucoma 38% followed by aphakic glaucoma 20% and Sturge–Weber syndrome- associated glaucoma 10%. J AAPOS 1999; 3: 308-15.  In Dallas study 23% of patients had primary glaucoma, 45% had secondary glaucoma, and 31% were glaucoma suspects. Clinic Ophthalmol .2013; 7:1739-46
  • 18. GENETICS  Sporadic  Familial patterns show recessive inheritance with variable degree of penetrance and multi factorial inheritance  Increased incidence with high consanguinity Arch Ophthalmol 1998;116(4):545–546 LOCUS LOCATION INHERITANCE GENE GLC3A 2p21 AR CYP1B1 GLC3B 1p36 AR ? GLC3C 14q24.3 AR ? Genomics 1995;30:170-177 IOVS 2002; 43: 3015 GENETIC LOCI FOR PRIMARY CONGENITAL GLAUCOMA
  • 19.  Although spectrum of mutations in CYP1B1 have been implicated in PCG  Very few reported genotype phenotype correlations  Frameshift and R 390C are associated with severe phenotypes and poor prognosis IOVS April 2004 ;45 :1149-56
  • 20. PRIMARY CONGENITAL GLAUCOMA Faulty atrophy of mesodermal tissue Faulty cleavage of mesodermal tissue Excessive collagenous beams preventing posterior sliding of iris and ciliary body leading to anterior iris insertion and TM obstruction Abnormal migration of neural crest cells
  • 21. TM Ciliary muscle 5 months :Iris meets endothelium TM & ciliary muscle overlapping Allen et al :Arch Ophthalmol 53:783 1955 Cleavage of mesodermal tissue Normal development of the angle
  • 22. Uveal tract splitting by cleavage or atrophy ciliary muscle is seen extending into iris Differential growth rate leading to slippage of ciliary muscle and ciliary body posteriorly
  • 23.  Typically bilateral but significant IOP elevation in one eye can occur in 25- 30 % of cases  Commonly between 3-9 months  Rarely at birth or up to 3 years of age
  • 24. Site Signs Cornea Megalocornea Breaks in Descemet’s membrane Haab’s Striae Sclera Increased axial length Myopia Astigmatism Optic Nerve Cupping IOP Increased
  • 25. Cornea Megalocornea Corneal clouding Breaks in Descemet’s membrane Haab’s Striae Horizontal or curvilinear striae formed as endothelial cells lay down new basement membrane and hyaline ridges develop
  • 26. Sclera Scleral thinning Blue sclera Increased Axial length Myopia Astigmatism
  • 27. Lens Stretching and rupture of zonules can cause lens subluxation Optic nerve cupping Rapid and early Reversible with normalization of pressure
  • 28.  To confirm the diagnosis of glaucoma  Determining the type of glaucoma if present Initial Evaluation • Adnexal source of irritation • Lacrimal system obstruction • Visual response • Corneal edema • Opacification • Preliminary digital tonometry
  • 29. IOP evaluation can be done in sleepy infant or during bottle feeding using:  Perkin’s Applanation Tonometer  Tonopen 5-6 yr old child:  GAT evaluation can be done
  • 30. Refraction and Perimetry Myopia Astigmatism Visual Fields yield valuable information in older children Anterior Segment Evaluation Using portable slit lamp UBM: to detect any associated anterior segment anomalies Posterior Segment Evaluation With direct or indirect ophthalmoscope if there is no corneal clouding or opacification B scan USG
  • 31. EVALUATION UNDER ANAESTHEISA Sequence For EUA Corneal Diameter Tonometry Anterior Segment Evaluation Gonioscopy Fundus Refraction Pachymetry Axial Length Measurement To avoid unnecessary anesthesia ophthalmologist undertaking EUA should be able to perform surgery if indicated
  • 32.  Haab’s striae  Corneal edema  Corneal opacification: 80% of patients  Posterior embryotoxon  Megalocornea  Measurement of Corneal Diameter: Hold the calipers on first appearance of white scleral fibers on one side and same point on other side. Accurate measurement using plastic gauge with calibrated holes. Can J Ophthalmology 1985; 20:93–97. CORNEAL FINDINGS
  • 33. Age Normal Megalocornea Term 9.5 -10.5 11.5 1 yr 10-11.5 12-12.5 2yr 11-12 >12.5 Older child <12 >13 Sherwin Isenberg Formula in preterm babies Corneal Diameter=0.0014 * wt in gm + 6.3
  • 34. Normal IOP varies with age:  Mean IOP up to 12 year of age is12.2 +/-0.74 mm of Hg  Mean IOP < 1 year of age is 8 +/- 2.5 mm of Hg J Pediatr Ophthalmol & Strabismus 2006; 43: 14 Tonometers  Perkin’s hand held Applanation Tonometer  Tonopen  Schiotz Tonometer should be avoided Sources Of Error  Effect of anesthesia  Corneal surface distortion , edema  Mackay Marg Tonometer is accurate IOP MEASUREMENT
  • 35. ANAESTHETIC AGENTS AFFECT IOP IOP Increased Succinylcholine Ketamine Endotracheal Intubation Decreased Halothane Oxygen Nitrous oxide Sevoflurane Midazolam Methohexitol Only raised IOP in EUA should never be the basis of diagnosis and treatment
  • 36. Portable Slit Lamp Corneal findings Anterior chamber depth Anatomy of iris
  • 37. Direct gonioscopy using Koeppe’s type direct goniolens with hand held slit lamp or microscope: Anterior insertion of iris with altered translucency of angle Lister’s morning mist: fine fluffy tissue covering peripheral iris Lochness Monster Phenomenon: loop of vessels of major arterial circle can be seen above iris Can visualize fundus through the same lens (in undilated pupil and with corneal edema ) GONIOSCOPY
  • 38. Type 1 Type II The Glaucomas, Sampolesi, Springer, 2013
  • 39. Possible if corneal edema or opacity do not hinder evaluation Avoid dilation if surgery is contemplated Direct ophthalmoscope Koeppe’s lens can be used with direct ophthalmoscope in small pupil Careful drawings and fundus photographs (using hand held fundus camera ) OPTIC NERVE HEAD EVALUATION
  • 40. A scan measures axial length , anterior chamber depth , lens thickness Baseline value for comparative purpose B scan for posterior segment if opaque media precludes the view Can be used to visualize retinal or choroidal detachment/ mass lesion / etc.
  • 41. PARAMETER NORMAL MILD MODERATE SEVERE Corneal Diameter (mm) upto10.5 >10.5-12 >12-13 >13 IOP (mm of Hg) 11.5 11-20 >10-30 >30 Cup Disc ratio 0.3-0.4 >0.4-0.6 >0.6-0.8 >0.8 Last BCVA 20/20 <20/20-20/60 <20/60 – 20/200 <20/400- no PL Panicker et al IOVS 2004;45 :1149-56
  • 42. DIFFERENTIAL DIAGNOSIS BASED ON SYMPTOMS  Epiphora and red eye  Conjunctivitis  Nasolacrimal duct obstruction Corneal epithelial defect  Keratitis  Anterior segment inflammation  Conditions associated with corneal edema or opacification Birth trauma Congenital malformation Corneal dystrophy Keratitis Mucopolysaccharidosis Idiopathic
  • 43.  Conditions showing corneal enlargement Myopia Conditions showing actual or pseudo optic nerve cupping Physiologically large optic nerve cup Coloboma Atrophic optic nerve Hypoplastic optic nerve Malformation
  • 44. Confirm The Diagnosis Of PCG Preoperative appropriate Medical Treatment Angle Surgery Goniotomy Trabeculotomy Trabeculectomy and Trabeculotomy Cyclodestructive procedures Follow up Lifetime Amblyopia treatment once IOP contolled
  • 45. SUPPORTIVE ROLE Preoperative :clear cornea for angle surgery Adjunctive modality: in combination with surgery if IOP is too high Temporary measure if infant is unfit for surgery ISSUE OF DRUG SAFETY IN CHILDREN Smaller plasma volumes Immature blood brain barrier Increased receptor sensitivity Increased systemic absorption MEDICAL TREATMENT
  • 46. MEDICATIONS INDICATION CONTRAINDICATION B BLOCKERS Non selective- (timolol - 0.1 ,0.25 ,0.5 %) Selective (for reactive airways )- betaxolol 1st line 2nd line for older children Avoid in children with reactive airways , cardiac disease Side effects: bronchospasm bradycardia CAI (Oral acetazolamide -5- 15mg/kg suspension ) Dorzolamide 2% Brinzolamide 1% 1st line / 2nd line – young children Avoid in children with corneal transplant Prostaglandins Latanoprost 0.005% Bimatoprost 0.03% Travoprost 0.004% 2nd / 3rd line Trichomegaly Redness Preoperative use – risk of inflammation Latanoprost use licensed in Europe since 2010 based on PANDA results
  • 47. MEDICATION INDICATION CONTRAINDICATION Miotics Diarrhoea Interaction with succinylcholine for GA Headache , proinflammatory Adrenergic agonist Epinephrine Apraclonidine Brimonidine Not used Short term use in infants and after corneal transplant Only in older children 3rd line or last resort Lack of efficacy DO NOT USE IN INFANTS / SMALL CHILDREN Lipophilic drugs –CNS side effects fatigue , somnolence
  • 48. Definitive therapy in most cases Western world  Early presentation with mild corneal edema at referral  Goniotomy is the treatment of choice In India / Middle east  Late presentation  Goniotomy is not possible  Trabeculotomy or combined trabeculectomy and trabeculotomy
  • 49.  OBJECTIVE : Remove the obstructing tissue that causes resistance to aqueous outflow  LENS: Swan Jacob lens  KNIFE: Barraquer knife Worst knife Swan spade Long needle Reported results show success rate of 80% J AAPOS.2001;5:281–4 GONIOTOMY
  • 50. Pre-op Preparation  Adequate miosis  Facilitates retraction of iris from angle Steps  After placing the goniotomy lens over cornea  Anterior chamber is entered through a clear corneal incision (e.g. nasal goniotomy – temporal incision is given )  Knife is advanced towards the opposite angle parallel to iris and away from pupil and the angle is engaged by the blade tip which is swept circumferentially incising the nasal angle over 100-110 degree. Falling back of peripheral iris is the end point of surgery
  • 51.
  • 52. Failure Failure of obtaining adequate incision during surgery Presence of peripheral anterior synechiae Fibrous proliferation Success rate of 70 – 93 % in large series after multiple goniotomies Ophthalmology 2011;118:236-40
  • 53. TRABECULOTOMY AB EXTERNO Independently described by two surgeons in 1960 Burian: Trabeculotomy ab externo Smith: Nylon filament Trabeculotomy Beck and Lynch: 360 degree trabeculotomy using 6-0 polypropylene Recent: Illuminated Microcathether Principle: To cannulate Schlemm’s canal from external approach and then tear through TM into anterior chamber
  • 54. Limbal or fornix based Conjunctival flap is made Hemostasis is achieved with minimal cautery Junction of Posterior border of TM and Sclera is the external landmark for scleral spur and Schlemm’s canal – radial incision is given at this site Incision is deepened till inner wall of Schlemm’s canal and trabeculotome is introduced
  • 55. Trabeculotome is rotated till 75% of probe is seen in AC Rotation is reversed and instrument is withdrawn Trabeculotome is then passed into Schlemm’s canal on other side radial incision
  • 56. GONIOTOMY VS TRABECULOTOMYGoniotomy Trabeculotomy Simple faster procedure Can be done in edematous or scarred cornea Does not disturb conjunctiva Does not require introduction of sharp instruments in anterior chamber Can be repeated one or more times Does not require adaptation to gonioprism Can be converted to trabeculectomy
  • 57. COMBINED TRABECULOTOMY AND TRABECULECTOMY WITH /WITHOUT MMC  Trabeculectomy combined with trabeculotomy to increase the long term success  Primary CTT : single operative procedure with better control of IOP in some populations e.g. India Trabeculotomy vs Trabeculectomy vs Combined procedure  Favourable outcome with combined procedure but after 2 years advantage was not statistically significant Br J Ophthalmol 1999;83:317–322.  Mandal et al Primary CTT without MMC in299 eyes of 157 patients reported a success rate of 63.1 % by the end of 1 year Ophthalmology 2004; 111:283–290
  • 58. Intraoperative use of mitomycin C has somewhat enhanced the success of procedure but with significant risk of complications  Complications with MMC use in children Thin walled avascular bleb : risk of endophthalmitis Bleb leaks Wound rupture One reported series of retrospective comparison of trabeculectomy with or without MMC noted no significant difference in outcome J Glaucoma 2004;13:228–232
  • 59. REGULAR FOLLOW UP For IOP monitoring Retinoscopy Amblyopia Therapy Optical Keratoplasty
  • 60. SURGICAL SUCCESS Corneal clarity Decreased IOP Pale blebs Reversal or non progression of disc cupping Reversal or non progression of myopia Good visual recovery Surgical success has been defined as IOP <16 under GA or IOP < 21 mm of Hg with no progression of cupping or corneal diameter Qualified success: Maintenance of above pressures with single drug
  • 61.  Total of 148 eyes (85 patients)  Trabeculotomy, trabeculectomy, or combined trabeculotomy-trabeculectomy (CTT) were compared: equal success rate  Overall success rate was 80.4%  One surgery 105 (70.9%), 34 eyes (23.0%) 2 surgeries, and 7 eyes (4.7%) had 3 surgeries  A progressive decline in success rate over time was evident, as success rate dropped from 96.6% at 5 months to less than 50% after 11 years of follow-up  Surgical outcome of PCG better in infants presenting before 6 months  Adjuvant topical anti glaucoma medications augment success rate
  • 62. MANAGEMENT OF REFRACTORY GLAUCOMA / FAILED SURGERY UNFAVOURABLE FACTORS IN PEDIATRIC GLAUCOMA Low scleral rigidity Rapid healing process Exuberant scarring process Enlargement of glaucomatous eyes with thinning and distortion of intraocular anatomy
  • 63. TRABECULECTOMY WITH OR WITHOUT MMC Indication Failed Angle Surgery Secondary Glaucomas Success Rate Variable results in literature
  • 64. Non Valved Molteno Baerveldt Valved AGV Indications Failed filtration surgery Failure to control IOP with medication post surgery Aphakic glaucoma Neovascular glaucoma GLAUCOMA DRAINAGE DEVICES
  • 65. AGV vs. Baerveldt vs MMC trabeculectomy in children <2 yrs:  Retrospective study; Better IOP control with the GDDs than the MMC trabeculectomy group  Cumulative success rates of 87% versus 36%, respectively, at 1year and even a larger difference of 53% versus 19% at 6 years. Am J Ophthalmol 2004;137 (6):1163–1164 AGV vs MMC trabeculectomy in pediatric aphakic glaucoma:  Prospective, randomized study  Higher qualified success in the AGV(67%) vs the MMC trabeculectomy group (40%)  Complication rates higher in the MMC trabeculectomy (40%) than AGV(26.7%)(differences were not statistically significant). Saudi Journal of Ophthalmology, 2011; 25:317–27
  • 66. CYCLODESTRUCTIVE PROCEDURES Indications  Failed angle surgery with minimal visual potential  Failed trabeculectomy / GDD with poor central vision  Anatomy precludes intraocular glaucoma surgery Options  Trans-scleral cycloablation (Diode laser )  Endoscopic diode laser cycloablation  Cyclocryotherapy
  • 67. 21 year follow up 53 eyes included Mean logMAR VA was 0.61 ± 0.57. Good VA in 51%, Moderate VA in 30%, and Poor VA in 19% Deprivation Amblyopia (64%) was main cause of visual impairment with VA <20/50
  • 68. VISUAL REHABILITATION Appropriate spectacle correction If problem persists for distance vision even telescopes can be prescribed Appropriate training for recommended device is must
  • 69. VISUAL REHABILITATIONRetrospective study of B/L congenital glaucoma  100 children, mean age 6.3 yrs (1 month-16 years)  Success rate of early treatment is 70 to 80%  Only 35% of all patients will have visual acuity better than 20/50, and 2% to 15% will remain blind.  Regarding the optical prescriptions, 80% were corrected for myopia and 20% were for hyperopia and astigmatism.  Optical devices for distance were prescribed for 34% of the patients. All of the optical aids for distance were telescopic manual systems for one eye only  Optical aids for near vision were necessary for 6% of the patients, and the most commonly prescribed were illuminated stand magnifiers of + 38 aspheric diopters Clinics 2009;64(8):725-30
  • 70. Consider repeat trab, Drainage implant, Cyclodestruction. Goniotomy/Trabeculectomy/Combined Trab-Trab Surgical Outcome? EUA after 3 weeks IOP controlled Evaluation after 3 months Normal IOP Evaluation after 3 months FU every 3 months Record IOP, CDR, VA Axial length, VF (if possible) IOP not controlled Add medical therapy If IOP not controlled repeat Trab-Trab Controlled VISUAL REHABILITATION Uncontrolled Very poor prognosis BROAD MANAGEMENT OUTLINE
  • 71. IMPACT OF CHILDHOOD GLAUCOMA119 children in the study, all grades of glaucoma Older children reported less impairment on CVAQC1, IVI-C2, and PedsQL 3than younger children. Parents reported greater impact on their child's HR QoL than children reported themselves. Children with glaucoma report HR QoL scores similar to those described by children with severe congenital cardiac defects, who have undergone liver transplants, or who have acute lymphoblastic leukemia. (1) functional visual ability (FVA) with the Cardiff Visual Ability Questionnaire for Children (CVAQC), (2) VR QoL with the Impact of Vision Impairment for Children (IVI-C), and (3) HR QoL with the Paediatric Quality of Life Inventory (PedsQL) version 4.0.
  • 72. PROGNOSIS OF CHILDHOOD GLAUCOMA230 patients with 10 year follow up 79 having ocular hypertension with open angles or primary angle closure (PAC), 35 primary open angle glaucoma (POAG), 50 PAC glaucoma (PACG), 20 primary congenital glaucoma (PCG), 46 secondary glaucoma patients. 15% of primary congenital glaucomas (PCGs) showed a glaucomatous VF defect after 10 years. This study provides evidence that routine delivery of care can provide well controlled IOP in glaucomas, both primary and secondary, and the VF stabilized in about 90% of patients over a period of 10 years, with the currently available glaucoma medications and trabeculectomy.
  • 74. CONCLUSION Congenital Glaucoma is a potentially treatable cause of irreversible childhood blindness provided it is picked up early. CGRN classification should be done for each case. EUA is the best method to clinically evaluate the patient. IOP should be taken immediately after induction with Tonopen or Perkin’s Tonometer to counter effect of anesthetic drugs. Management is mostly surgical. Goniotomy in West and Trab with augumented Trab forms the mainstay in our setup. Post operative life long follow up is vital. Amblyopia should always be treated to improve the QOL of the patient.