This document provides an overview of childhood glaucoma, including terminology, classification systems, epidemiology, pathogenesis, clinical presentation, diagnosis, and management. It discusses primary and secondary childhood glaucomas. Primary childhood glaucomas are further classified based on the presence of ocular anomalies, systemic diseases/syndromes, or acquired conditions. The document outlines evaluation procedures such as examination under anesthesia and diagnostic testing. It also reviews medical and surgical treatment options for childhood glaucoma.
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
Congenital OAG
Juvenile open angle glaucoma
Associated with ocular abnormalities
Associated with systemic abnormalities
SECONDARY GLAUCOMA
Traumatic glaucoma
Secondary to intraocular neoplasm
Secondary to uveitis
Lens induced glaucoma
After surgery for congenital cataract
5. PRIMARY: Due to maldevelopment of aqueous outflow
system
SECONDARY: Damage to aqueous outflow system
maldevelopment of some other portion of eye
due to
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 SECONDARY CHILDHOOD GLAUCOMA
GLAUCOMAS
IAPrimary congenital
glaucoma
IB Juvenile open angle
glaucoma
IIAGlaucoma 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 WGAin
July 2013
10. IA PRIMARY CONGENITAL GLAUCOMA Isolated angle anomalies
Meets glaucoma definition
Spontaneously arrested cases but with typical
signs of PCG
IB JUVENILE OPENANGLE GLAUCOMA No ocular enlargement
No associated ocular anomalies /syndromes
Open angle
Meets glaucoma definition
11. IIA Conditions with predominantly ocular anomalies present at birth may or
may not be associated with systemic signs
Axenfeld’s Reiger anomaly
Peter’s anomaly
Ectropion uveae
Congenital Iris Hypoplasia
Aniridia
Oculodermal melanocytosis
Posterior Polymorphous Dystrophy
Microphthalmos
Microcornea
Ectopia lentis
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
GENETIC LOCI FOR PRIMARY CONGENITAL GLAUCOMA
LOCUS LOCATION INHERITANCE GENE
GLC3A 2p21 AR CYP1B1
GLC3B 1p36 AR ?
GLC3C 14q24.3 AR ?
Genomics 1995;30:170-177
IOVS 2002; 43:
3015
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
21. TM
Ciliary
muscle
5 months :Iris meets
endothelium
TM & ciliary muscle
overlapping
Cleavage of
mesodermal tissue
Allen et al :Arch Ophthalmol 53:783 1955
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
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
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
edema or opacification
Birth trauma
Congenital malformation
Corneal dystrophy
Keratitis
Mucopolysaccharidosis
Idiopathic
with corneal
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 1st line Avoid in children with
Non selective- (timolol - 2nd line for older children reactive airways , cardiac
0.1 ,0.25 ,0.5 %) disease
Selective (for reactive Side effects:
airways )- betaxolol bronchospasm
bradycardia
CAI 1st line / 2nd line – young Avoid in children with
(Oral acetazolamide -5- children corneal transplant
15mg/kg suspension )
Dorzolamide 2%
Brinzolamide 1%
Prostaglandins 2nd / 3rd line Trichomegaly
Latanoprost 0.005% Redness
Bimatoprost 0.03% Preoperative use – risk
Travoprost 0.004% 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:
KNIFE:
Swan Jacob lens
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
TRABECULOTOMY
Goniotomy 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
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
FailedAngle 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 theAGV(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 VAin 51%, Moderate VAin 30%, and Poor VAin 19%
Deprivation Amblyopia (64%) was main cause of
impairment with VA <20/50
visual
69. VISUAL
REHABILITATION
Retrospective 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. 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
Consider repeat trab,
Drainage implant,
Cyclodestruction.
BROAD MANAGEMENT OUTLINE
71. IMPACT OF CHILDHOOD
G
119
L
cA
hildU
renC
in th
O
e sM
tudy,
A
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 VisualAbility 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
C
23
H
0 p
Ia
L
tie
D
ntsH
witO
h 10
O
yeD
ar foG
llowL
up
AUCOMA
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 T
onopen 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.