JOCGP

Primary Congenital Glaucoma

REVIEW ARTICLE

Primary Congenital Glaucoma

Usha Yadava
Director, Professor of Ophthalmology, Maulana Azad Medical College and Allied Guru Nanak Eye Centre, New Delhi, India

Correspondence: Usha Yadava, Director, Professor, Glaucoma Services, Guru Nanak Eye Centre and Maulana Azad Medical
College, Bahadur Shah Zaffar Marg, New Delhi-110002, India, Phone: 91-1123234622 (O), e-mail: yadavau@yahoo.com

ABSTRACT
Glaucomas of the childhood are devastatinly deceptive in their presentations because of an insidious onset and late recognition. Primary
congenital glaucomas (PCG) which serve as a prototype for glaucomas in the young Subtleties of diagnosis may lie in simple symptoms
of watering and photophobia. This article is aimed to summarize all common manifestations and basics of surgical treatment, conventional
and contemporary. Parameters of diagnosis and success have been undergoing modifications from time to time, hence the need to take
stalk. How we look at childhood glaucomas in the light of conclusions drawn from glaucoma clinical trials in adults, evolving concepts of
risk factors like central corneal thickness at the first and subsequent visits are of practical concern.
Keywords: Childhood glaucoma, Pediatric glaucoma, Trabeculotomy, AGV.

INTRODUCTION

Other Study Groups have Reported as Under

With the availability of medical literature on the click of a

mouse, it is important that specific querries be answered.

What are the Common causes and Age of Onset?

Childhood glaucomas are of three etiological types (Table 1)
and the commonest age of onset is under the first year of life,1,2
with 80% of patients present in the first year of life, out of which,
20% present as newborn glaucoma while the remaining 80%
report as infantile glaucoma.3,4 Conventionally, the term infantile
glaucoma is extended up to 3 years, after which it is termed
juvenile glaucoma. This ambiguity in the nomenclature is due
to the typical clinical features attributed to the distensible eye up
to three years. Some study groups are less rigid and prefer to
club all these together as Primary infantile and Congenital
glaucomas or Infantile glaucomas.5 In any case, New born
glaucoma as a separate entity is important to recognize as this
is the most severe form with worst outcome.6

How Frequently do We see PCG?

There have been some additions to the older figures quoting an
incidence of 1 in 5 years in general ophthalmic practice and
1/10,000 live births (LB).6 Results of a survey of newly
diagnosed cases by the British infantile and congenital
glaucoma study Group (BIG study) in three population groups
were published in 2007:5,7
1/18,500 LB in British caucasians
9 times higher in British-Pakistani population
1/30,200 LB in irish population.

1/30,000 LB among Australians

Hungary: Higher incidence among gypsies
1/1,250 LB in Slovak nomads
,500 LB in Saudi Arabia
1 in 3300 LB in Andhra Pradesh
Brazil: 10.8% of 3,210 visually handicapped children
6.3% in China.

Higher incidence in inbred communities has got the

molecular biologists striving to find some correlations between
genetic mutations and phenotypes in the hope of predicting the
onset or the severity of disease. Hyderabad group is leading in
this global research.8

What is the Gender Distribution and How often

does it affect both Eyes?
An interesting facta pseudoautosomal dominant pattern may
emerge in families with consanguinity at multiple levels with
seemingly equal predilection for either sex (Table 2).9

What is the Social Impact, if any of this

Rare Disease?
It is true that this is a rare disease but we also know that glaucoma
causes progressive and complete blindness. With a long
anticipated life span, these children suffer economic blindness
and social discrimination and end up becoming a burden to the
society. There is also a higher chance of more than one just one
member of a family suffering. Hence, the importance of catching

Table 1: Shaffer-Weiss classification

Congenital

22.2%

Developmental Glaucomas with associations

46%

Secondary

31.8%

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Table 2: Gender ratio and bilaterality in PCG

Male:Female
Bilaterality

2:3
60 to 85%

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Usha Yadava

Which cases have the Worst Prognosis?

asthma. Additional benefit with oral acetazolamide may be

obtained in very high levels of IOP as it seems to have an
additive effect with topical CAI drops in pediatric patients
unlike in adults.15 Timolol instillation should be followed by
mechanical punctual occlusion for at least 3 to 4 minutes, to
minimize systemic absorption. In case, the surgical response is
incomplete postoperatively or late failure occurs, qualified
control with minimum medication may be achieved.16,17

Patients presenting with severe corneal clouding in the first

year have the worst outcome (Figs 1A and B).6,11,12

What is the Best Surgical Option for these Patients?

them young for treatment as well as parental counseling cannot

be over emphasized. Statistical estimations reveal that there
are 1.5 to 2 million glaucoma blind children globally, of which
2/3rd are in the developing nations. They constitute 15% of
blind school entries and 4.2% of all cases of pediatric
blindness.10

What is the Role of Medical Therapy?

Glaucoma medications have a limited and palliative value in
PCG because of poor response and compliance. Child should
be started on medical treatment only as a stopgap and surgery
should be performed without further delay.12,13

Which Drugs are not to be used in Children?

Alpha 2 agonist group of drugs like brimonidine are
contraindicated in children < 2 years of age because of the risk
of apnea.12,13 In general, prostaglandin analogues too are to be
avoided in unilateral glaucomas and prior to surgery.
Pilocarpine may paradoxically raise the intraocular pressure
(IOP) due to its action on the scleral spur, which may cause
further collapse of the trabecular meshwork due to the
abnormally high insertion of uveal tissue.14 Short-term use of
a topical carbonic anhydrase inhibitor (CAI) and beta-blocker
(0.25% or 0.50%) either alone or in combination is a safer
choice in these cases. Betaxolol may be used in children with

Fig. 1A: Severe bilateral corneal clouding at birth

An ideal surgical procedure aims to achieve the

following: 11,17-19
Ensure short-term and long-term control of IOP
Should have no vision threatening complications.

Should the Small Infant undergo such Major

Surgery? Does an Ideal Procedure Exist?
Undoubtedly, on the basis of documented clinical evidence,
the one fact we do know is that delay is disastrous for vision.
Concerns about general anesthesia (GA) and surgical outcome
in babies have been put to rest in recent years with advances in
safe anesthesia for newborns with significant reduction in
morbidity and mortality associated with GA than yesteryears.
A calculated risk needs to be taken because complications like
hypothermia, apnea spells and hemorrhage have been reported
in newborns making a case for minimizing the use and abuse
of GA in newborns.20,21 By far and large, modern anesthesia
and resuscitation methods have made surgical intervention quite
safe in newborns. No major complication were reported by
Mandal in 2003 in a study of 47 eyes of 25 patients of congenital
glaucoma operated under one month of age except one case of
hypothermia and one of uncontrolled bleeding from the trachea,
both resuscitated successfully. The above author prefers to
operate both eyes simultaneously as his way of maximizing
the care with the underlying need for urgency required to
manage such cases. Complete clearance of cornea was noted
in the same study in 66% of cases, IOP control at the end of 12
months of follow-up with the Kaplan-Meier survival rate of
89.4% and the mean glaucoma medication rate fell from 68.1%
to 14.9%. Excellent visual outcomes and reversal of cupping
with early intervention in these cases is known to occur
universally.16,22
There are several surgical options depending on the severity.
Let us first briefly summarize the relevant anatomy and
embryology of the anterior chamber (AC) angle for better
orientation and approach to the case at hand.

Anatomy and Embryology of the AC Angle

Fig. 1B: Bilateral ground glass edema of cornea due to bilateral

buphthalmos in a newborn

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The limbal transition zone between the cornea and sclera

measuring 1 to 1.5 mm is bounded anteriorly by the end of the
Descemets membrane (Schwalbes line) and posteriorly by
the scleral spur, which is the outer landmark for the trabecular
meshwork (TM). The TM fills up an indentation or depression

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Primary Congenital Glaucoma

Fig. 2: Normal anterior chamber angle structures

known as the scleral sulcus on the under surface of the sclera in

front of the scleral spur and stretches anteriorly upto the
Schwalbes line (Fig. 2). Schlemms canal courses
circumferentially beneath the TM tissue. The ciliary muscle,
on the other hand, takes attachment to the scleral spur, rendering
it taut so that the intertrabecular spaces do not collapse. The
same structures when viewed on gonioscopy are seen as lines
or bands peeping into the angle recess in the posteroanterior
order, i.e. iris, ciliary body, scleral spur, TM and Schwalbes
line.
Fig. 3: Origin of neural crest cells

Embryology of the AC Angle13,23

In general, ocular development goes through three stages:
Stage of embryogenesis (up to 3rd week): In the initial stages
after fertilization, sequential formation of three cell
massesmorula, blastula and lastly gastrula occurs. Cells
within the gastrula differentiate into three primary germ
layers: ectoderm, mesoderm and endoderm.
Stage of organogenesis (3rd to 7th week): Is the period of
organization of the segregated cells into rudimentary organs.
The lens separates from the surface ectoderm (6 weeks)
and the fetal fissure closes. The hitherto undifferentiated
mass of cells from the neural crest migrate to appear in the
vicinity of the anterior equator of the lens (Fig. 3) and give
origin to the corneal endothelium, stroma, iris, ciliary body,
trabecular meshwork and sclera. The limbus makes its
appearance and the AC appears as a potential space. The
three waves of neural migration now proceed as follows:
First wave forms the corneal and trabecular endothelium
Second wave forms corneal stroma
Third wave forms the iris stroma.
The anterior chamber boundaries are formed by the
cornea and tunica vasculosa lentis during the 7th week of
gestation.
Stage of differentiation (8th week onwards): With the
appearance of limbus, the rudimentary TM also appears.
The TM undergoes a process of internal reorganization so
that the highly specialized task of aqueous secretion and
excretion can begin. Following theories of meshwork
development have been proposed:

Atrophy and Absorption

Rarefaction
Cleavage
Theory of growth and differentiation: This new and the
most accepted process was demonstrated by
McMenamin in his study of 432 human fetal eyes.25
Trabecular tissue undergoes a process of differentiation
in which there appear gaps in the matrix with
reorganization of the cells so that the TM attains its
porous character with the creation of intertrabecular
spaces. The nonpigmented secretory epithelium of the
ciliary body gets thrown into folds by 12 weeks and the
longitudinal ciliary muscle development takes place in
the 4th month. Finally the primitive TM establishes
communication with the anterior chamber by 20 to 22
weeks of gestation. This is about the time the ciliary
body starts to function but the complete maturation of
the angle with regression of the hyaloid system occurs
by the 8th month. Further posterior movement of the
AC angle is a continuous process with full angle recess
formation achieved up to 6 to 12 months in the postnatal
period. Histopathology support was provided by
Anderson DR (1981) with demonstration of the high
insertion of the anterior uvea across the posterior TM
in the third trimester, persistence of which could be the
cause for compression of the trabecular beams leading
to congenital glaucoma. However, the possibility of
primary defects at various levels within the TM and

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Usha Yadava

Schlemms canal has not been ruled out. Absence of

the Schlemms canal, whenever found has been thought
to be a secondary change. No true membrane exists as
believed earlier.

Gonioscopic Anatomy of a Normal Infant Eye (Fig. 4)23,24

Iris inserts posterior to the scleral spur

Flat iris insertion due to poor development of the angle
recess till the age of 6 to 12 months
Ciliary body band is distinct in most cases
TM appears thicker and more translucent than in adults.

When should Glaucoma be Suspected in an Infant?

Whenever the classical triad of watering eye, photophobia and
cloudy cornea is seen, glaucoma should be suspected and an
initial examination followed by examination under anesthesia
(EUA) with prior consent for surgical intervention should be
done.

Initial Examination
General systemic and ophthalmic examination is done to screen
for possible systemic associations; adnexal sources of irritation
in infants like epiblepharon, entropion and foreign body. Visual
responses are judged and preliminary digital tonometry may be
done whenever possible.12 After the initial history and office
examination, EUA /surgery are planned (Fig. 5).

Suggested Sequence for EUA

Tonometry (as early as possible after induction and before

intubation)
External examination
Anterior segment examination
Corneal diameter measurement
Fundus examination
Gonioscopy
Refraction, pachymetry, axial length measurement.

Preanesthesia
Each clinical parameter is important in a unique way, either in
diagnosis, prognosis or management of these children. With a
view to maximizing the advantages of EUA in these small
children, it is imperative that the surgeon/assistant be familiar
with all possible variables associated with GA. The need to
make a team with the anesthesiologist cannot be over
emphasized in PCG patients.11 Informed consent for surgical
intervention must be taken prior to GA, prognosis and risk, if
any should always be explained beforehand. Parents must be
educated about (a) the type of glaucoma and (b) high demands
of care even after the operation and (c) the likely need for GA
in future.20

Do Anesthetic Agents alter IOP Levels?26,27

Anesthetic agents and dosage are known to alter the IOP
(Table 3). For this reason, high IOP in isolation should never
be the basis for diagnosis. Despite the inconvenience associated
with a GA event in terms of cost and time. A casual and
inconsistent reporting in EUA is not uncommon. This can lead
to an aimless venture towards controlling a pressure, which was
erroneously recorded high.

Are these IOP readings Comparable to Adult Values?

IOP is also known to increase with age, hyperopia and corneal
thickness. Adult values are normally reached only after the early
teens (Table 4).27 In a study of 405 Indian children up to the
age of 12 years Sihota et al found a mean overall value of IOP
as 12.02 .74 mmHg and 8 2.55 mmHg < 1year of age.
Adult values were achieved at 12 years of age.28
Fig. 4: Gonioscopic view of the anterior chamber angle

Photophobia, Watering, Redness, Pain

Severe photophobia causes the infant to bury the head into a
pillow. This symptom complex should be differentiated from
other causes of watering at this age. Symptoms of ocular
Table 3: Effect of anesthetic agents on intraocular pressure26
IOP increases
Succinyl choline
Ketamine
Endotracheal intubation

Fig. 5: The complete examination under anesthesia tray

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IOP decreases
Halothane
Oxygen
Nitrous oxide
Sevoflurane
Midazolam
Methohexitol

No change in IOP with oral chloral hydrate sedation.Tonometry with

< 1% halothane under the mask anesthesia is considered optimum.

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Primary Congenital Glaucoma
Table 4: Normal intraocular pressure by age27
Age in years

IOP in mmHg*

Birth
0-1
1-2
2-3
3-5
5-7
7-9
9-12
12-16

9-6
10.6
12.0
12.6
13.6
14.2
14.2
14.3
14.5

*These values were obtained using a hand held noncontact

tonometer Pulsair, Keeler.

irritation are also common in buphthalmic eyes due to steep

corneas and poor tear film stability, appearing sometimes like
dry eye.

Fig. 7: Severe stretching of the limbus

What are the Corneal Findings?

Under the effect of high IOP, the ocular globe distends resulting
in buphthalmos meaning Ox Eye. This expansion leads to
typical stretch marks in the cornea called Haabs Striae
(Figs 6A and B, Fig. 7) due to ruptures in the elastic Descemets
membrane typically in the circumferential direction. With
passage of time the overlying edematous cornea undergoes
opacification. 80% of PCG eyes in India have severe corneal
clouding secondary to increased IOP during intrauterine life or
later in infancy (Fig. 8). Posterior embryotoxon can also be
noted in some cases (Fig. 9).

Fig. 8: Buphthalmos in two patients

Fig. 9: Posterior embryotoxon in the anterior chamber (garland sign)

Differential Diagnosis of Corneal Clouding

(STUMPED)29

Figs 6A and B: Haabs striae

Sclerocornea
Traumatic rupture
Metabolic
Peters anomaly
Posterior polymorphous dystrophy of cornea
Endothelial dystrophy
Dermoid.

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Corneal Diameter
What about corneal diameter? What is the correct way to
measure cornea in a stretched limbus (Fig. 7)?
Corneal diameter is measured by using callipers to measure the
horizontal and vertical diameters (Fig. 10). Under good ambient
illumination, callipers are held at the point of the first appearance
of the white scleral fibers on one side to the same point on the
other. This allows a measurement accuracy of approximately
0.5 mm. Normally, horizontal diameter measures marginally
more than vertical. Considering 9 to 10.5 mm as normal at birth,
taking > 12 mm in either meridian as abnormal at 1 year, one
should look for other evidence of glaucoma.23,29 A value of
13 mm or more is definitely abnormal for all age groups. The
following formula has been worked out by Sherwin Isenberg
for interpretation in preterm babies:30
0.0014 Weight (in grams) + 6.3 = Corneal diameter
Developmental glaucoma may have anomalies like Microspherophakia with or without aniridia (Fig. 11), persistent
hyperplastic primary vitreous and congenital ectropion uvea
syndrome (Table 5).

Fig. 11: Microspherophakia with aniridia

Differential Diagnosis of Buphthalmos23,29,31

Congenital myopia
Megalocornea
Anterior megalophthalmos
Keratoglobus
Secondary glaucomas.

Gonioscopy
Why is Gonioscopy done and which Gonioscope is
Preferable?

Fig. 10: Measuring the horizontal corneal diameter in

bilateral buphthalmos

Gonioscopy is done with Koeppes type direct goniolens

available in a set of different sizes for use in supine position.31
Direct goniolens gives an erect and panoramic view of the angle
of the AC at a lower magnification than the commonly used
indirect gonioprism of the Goldman type in adults. It is also

Table 5: Other development glaucomas with associated anomalies

1 Iridocorneal endothelial syndrome

2 Posterior polymorphous dystrophy

3 Peters anomaly
4 Aniridia

5 Iridogoniodysgenesis
6 Oculodentodigital dysplasia

7 Ectopia lentis et pupillae

8 Congenital ectropion uveae

9 Congenital microcoria and Myopia

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Presence of corneal endothelial abnormalities

Unilaterality
Absence of family history
Onset in young adult hood
Corneal endothelial abnormality
Central portion of cornea, iris and lens involved
Rudimentary iris
Peripheral corneal opacity and pannus
Localized congenital opacities in the lens, subluxation of lens or its complete absence
Foveal hypoplasia
Glaucoma develops in late childhood or adolescence
Mutation in PITX2 gene
Congenital hypoplasia of the iris without the anterior chamber angle defects
Dental anomalies
Mild stromal hypoplasia
Anterior chamber angle defects
Microphthalmia
Glaucoma
Mutation in connexin-43 gene
Bilateral displacement of lens and pupil in opposite directions
Presence of pigment epithelium on the stroma of the iris
High incidence of glaucoma
Systemic anomalies may be present
Results from underdevelopment of dilator pupillae muscle of iris
Associated with goniodysgenesis and glaucoma

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Surgical Treatment of PCG

For all the practical reasons, buphthalmic eyes are a special
surgical challenge:33-36
Opaque cornea
Distended globe with high IOP
Posterior shift of limbus
Thin sclera and limbus
Thick and active Tenons capsule, rapid wound healing
Lower scleral rigidity
Friable iris
Life long lasting results anticipated
Visual rehabilitation is difficult.

Choice of Surgery
Fig. 12: Central cupping with a pink neuroretinal rim in PCG

possible to see the fundus with this 50 diopter concave lens

despite significant corneal edema through an undilated pupil.
This is a nongonioscopic use of Koeppes lens.

Gonioscopic Features of PCG23,31

Morning mist appearance.

Disc and Fundus Evaluation

Central, concentric steep walled cup (Fig. 12).

What Type of Refractive Errors are Common in

PCG Patients?

Induced myopia
Astigmatism due to irregular scarring.

Trabeculotomy with Trabeculectomy (CTT)

Pachymetry

Due to aggressive fibrosis and rapid healing in younger age

groups, conventional filtering surgery enjoys limited long-term
success. Best surgical results are obtained, when surgery is done
as early as possible before the onset of irreversible glaucoma
and secondary changes in the angle and cornea occur.21,35-38
The following procedures are listed for their application in
childhood glaucoma.39,40-46
Trabeculectomy with antimetabolites46
Trabeculotomy ab externo47
Combined trabeculotomy with trabeculectomy (CTT) with
or without antimetabolites
Nonpenetrating Schlemms canal resection44,45
Goniotomy50
Glaucoma drainage devices (GDD)
Cyclodestructive procedures.

IOP in children shows an increasing trend with age,

hyperopia and central corneal thickness (CCT), reaching
adult IOP values by 12 years in a study of the Indian pediatric
population by Sihota.28 PCG patients have revealed a trend
towards thinner corneas on ultrasound pachymetry.31 Studies
on CCT in PCG however are of preliminary nature and have
not been confirmed.
Determining accuracy of measurement of CCT in edematous
corneas anyhow remains a challenge.

This is the most commonly performed surgery in India. It has

the following clear advantages over goniotomy:39-47
Can be performed in opaque cornea
Familiarity with ab externo approach
Can be combined with trabeculectomy
Satisfactory IOP control.

Principle

As a guide to the severity of glaucoma and for follow-up.

To cannulate Schlemms canal from external approach and then

tear through the TM into the anterior chamber, creating a direct
communication between the AC and Schlemms canal.
Additional trabeculectomy is done to enhance long-term
success.

Severity Index

History

A severity assessment on first presentation has been proposed

by Mandal et al in their review of cases (Table 6).32

Burian and Smith independently reported it in 1960 as an

alternative to goniotomy. Harms and Dannhiem modified the

Axial Length (AL)

Table 6: Severity index in PCG*18

Clinical parameter

Normal

Up to 10.5
11.5
0.3-0.4
20/20

Cornea diameter in mm
IOP mmHg
C:D ratio
Last BCVA

Mild
> 10.5-12
> 11-70
> 0.4-0.6
< 20/60-20/60

Moderate

Severe/Very severe

> 12-13
> 10-30
> 0.6-0.8
< 20/60-20/200

> 13
> 30
> 0.8
< 20/400-no PL

This comprehensive chart may help to provide a realistic approach in management of PCG.

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procedure.47 Reported success rates vary from 73 to 100%.

Indian studies reported a cumulative success rate of 60% over
a long-term follow-up.33,37

Steps of Combined Trabeculotomy with

Trabeculectomy using Intraoperative MMC
Conjunctiva is sutured appropriately, fornix based flap with
two wing sutures (purse string type) and multiple (usually 2-3)
bleb forming sutures with 10-0 nylon. Limbus based flap
requires double layer suturing of the Tenons layer and
conjunctiva. Care is taken to hydrate the side port and titrate
the bleb to ensure water tight closure (Figs 13 to 16).

Chronic hypotony
Shallow anterior chamber
Hyphema
Choroidal detachment
Cataract
Retinal detachment
Inadequate IOP control
Blebitis
Endophthalmitis
Long-term effects of antifibrotic agents is not known.
Enhanced astigmatism.

Postoperative Protocol

Intraoperative Complications51,52

Postoperative Complications47,51-58

Perforation of sclera/false passage

Descemets stripping
Nonlocalization of TMs canal
Severe hypotony after first canulation, so noncanulation on
second side
Loss of anterior chamber and lens injury
Wrap around iris
Iridodialysis
Cyclodialysis
Hyphema.

Topical steroid and cycloplegic drugs are given for a maximum

of six weeks. Cushings syndrome has been reported with
unsupervised topical use of glucocorticoids in infants.48,49

Regular Follow-up
To assess control of IOP
Refraction
Amblyopia therapy
Success may be complete or qualified (on one topical drug)
on the basis of IOP lowering effect54
Optical keratoplasty.

Fig. 13: Globe fixation with two bridle sutures through the episclera on either side of superotemporal quadrant

Fig. 14: Fornix based conjunctival flap dissection

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Fig. 15: Gentle sponge application of MMC 0.02% on the surface for 1-2 minutes and washed copiously

Fig. 16: A 5 5 mm rectangular partial thickness scleral flap in the superotemporal quadrant dissected up to the clear cornea

Alternate Procedures55

360 suture trabeculotomy.

6-0 Prolene suture is used to thread the entire Schlemms canal
and rupture it to communicate with the anterior chamber.

Nonpenetrating Schlemms Canal Resection

Procedure

In this procedure, a block of tissue is excised beneath the scleral

flap to exteriorize the Schlemms canal without penetrating
the AC. It has been reported to be fairly successful.

A round, dome shaped direct gonioscope is placed on the cornea

with suturing or with a handle (Swan Jacob type). A nontapered
knife or needle enters the AC just inside the limbus and is
withdrawn. Injection of viscoelastic is done to maintain the AC
during the procedure. Re-entry is done with the knife, which
courses in the same plane as the iris. The TM is engaged just
below the Schwalbes line in the opposite quadrant and a
circumferential incision is made across the visible meshwork.
It should be a superficial incision with no grating or scraping
sensation. Prior treatment with pilocarpine eye drops is
recommended. An immediate widening of the angle under view
and posterior movement of the iris, if visible is a definite
indication of success. The knife is withdrawn in a similar fashion
without collapsing the AC. An AC maintainer has also been
used for the same.

Goniotomy50,59
Aim is to transect the Schlemms canal by ab-interno approach.

Historical Aspects
This operation was first done by Carlo de Vincentiis in 1893
without angle visualization. Barkan, later in 1938, under
gonioscopic visualization aimed to cut the Barkans
membrane. However, the concept of Barkans membrane has
not been substantiated. The aim of modern goniotomy is to cut
open the Sclemms canal by slicing through the TM.

Good visibility of angle structures with clear identification

of meshwork, which is difficult
Technical expertise required
Corneal diameter not >15 mm.

Prerequisites

Advantages

Clear cornea
Age 3 to 12 months preferably

Good success rate up to 90% between 3 to 12 month

Special role in aniridia to prevent glaucoma.

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Disadvantages

Not possible in opaque cornea

Minimal opacity like Haabs striae can jeopardize
visualization of the angle
Expertise development required
Peripheral anterior synechiae form easily leading to failure.

All the above listed procedures have been modified from time
to time. Some important ones are mentioned here:
Goniotomy with goniopunctureaim is to raise a bleb
Trabeculopunctureaim is direct laser opening of the
Schlemms canal under gonioscopic visualization
Trabeculodialysisaim is surgical scraping of the TM from
the scleral spur .

How is the Surgical Success determined after Any

of these Operations?
The following parameters are to be assessed:21,35-38,41-58
Corneal clarity
A shallow AC is not common in PCG. Transient shallowing
may occur in the first 3 to 4 days after the surgery
Relief of photophobia
Lowering of IOP: An IOP in the low teens is preferable
Bleb: diffuse, pale blebs are seen after using MMC
Reversal or nonprogression of disk cupping
Reversal or nonprogression of myopia
Visual outcome: Good visual recovery, which may be
difficult to document in children.
*Tonometry may be possible under topical anesthesia while
the infant is being fed with the bottle.
*Use of topical or systemic steroids must not be prolonged
beyond 6 weeks.

What is the Status of these Surgeries?

Comparison of CTT with goniotomyCTT is the first choice
because of:
Easy adaptability 57,58
Safe and successful
Suitable even in compromised corneas
More predictable than goniotomy
Goniotomy not suitable for > 15 mm cornea
Higher success rates of primary trabeculotomy than
goniotomy, 83% vs 33% in a comparative study.57

Results of Primary CTT53-58

Surgical success has been defined as IOP < 16 mm under GA
or < 21 mm without GA with no progression of cupping or
corneal diameter. Qualified success is defined as maintenance
of this pressure with a single drug (timolol). Extreme hypotony
(< 5 mm) leading to maculopathy has been defined as failure.
Macular hole has been reported following severe hypotony:56
CTT is the preferred choice in Indian patients as 80% present
with severe corneal clouding and are anyway not suited for
goniotomy. Encouraging reports from Indian authors in large
series of PCG cases add to the credibility of the procedure.
Mandal AK et al have published several reports on the high
success and safety profile of combined trabeculotomy and

66

trabeculectomy in PCG even in very small infants. The

authors were confident to perform this procedure
simultaneously in newborn patients with bilateral glaucoma
under one month of age with no complication, reporting a
success rate of 94.4% by Kaplein Mayor analysis in 182
eyes of patients.59 Bilateral simultaneous surgery is, however
not a universally accepted practice. Good visual recovery
was reported in another study on advanced glaucoma
children with corneal diameter of 14 mm or more.18 No
sight threatening intra- or postoperative complications were
noted in any of the patients.
In a series of 61 eyes with PCG, Dietlein36 however reported
the need for resurgery in 1/3rd of their cases in 36 months
of median follow-up with no decrease in the number of
glaucoma medications.
British infantile glaucoma (BIG) I study in a prospective
analysis of 99 recently diagnosed cases of pediatric
glaucoma patients reported < 21 mm IOP in 94% with
medication, 60% without any glaucoma medication in the
primary congenital group as compared to 86% with and
28% without glaucoma medication in the secondary
glaucoma group.5
The success criteria chosen by different authors do not
match, variable IOP levels at 21 mm, 18 mm and 16 mm
have been taken as indicators of successful control.54,55

Results of Re-surgery
Trabeculectomy with mitomycin C (MMCT) has a moderate
probability of success ranging from 36 to 95%, with lower
success seen in younger children. Though this pharmacologic
modulation does control IOP in refractory pediatric glaucoma,
the procedure seems to have a higher risk profile with no
additional IOP lowering effect in a retrospective study of 61
patients in 19 years of follow-up by Rodriques.67 Mandal in
1997 reported complete success in 94.74% of complicated
glaucoma inclusive of failed secondary glaucomas. One patient
had qualified success and one patient had retinal detachment
which is a rare association for which cause could not be
ascertained.20 Fluorouracil (5FU) as a single intraoperative
application has shown uniformly poor efficacy in children for
controlling glaucoma.41

Management of Failures, Residual and

Refractory Glaucoma
Medical Therapy
It is an established fact that best outcome is after first successful
surgery.54 Each time surgery is repeated, success rate is likely
to drop. Following guidelines are recommended:
Stop steroids: oral/topical
Start on topical beta-blockers and CAI

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JOCGP
Primary Congenital Glaucoma

Prostaglandin analogues have to be avoided in unilateral

cases due to hyperpigmentation and hypertrichosis of lashes.
Alpha 2 agonists are not recommended in children below
two years of age.
Oral CAI have been reported to produce additive effect with
topical drugs same in children but still long-term use is not
advisable.

Glaucoma Drainage devices in Children

Glaucoma drainage devices (GDD) offer a promising

alternative in such conditions. Quoting Albert W Biglan
(Costenbader Lecture, 2006) Success in treatment of
primary infantile glaucoma will be determined by the age
of onset and the ability to deliver a safe and effective
procedure.11 Several glaucoma procedures and devices
have been attempted to fulfil this adage.
In an initial clinical experience with Ahmed glaucoma valve
(AGV) in 21 pediatric patients, Coleman has reported
similar success as other GDD up to 77.9% at 12 months
and 60.6% at 24 month.65 In another comparative study, 38
eyes of complicated pediatric glaucoma were studied in two
groups, 20 had MMCT and 18 had AGV implantation.52
Comparisons drawn in the complication rates between
MMCT and AGV are given in Table 7.

Glaucoma Drainage Devices

These are broadly divided into two types:
Nonvalved/nonrestrictive
Molteno
Baerveldt
Schocket.
These rely on the formation of a fibrous bleb that is formed
on the endplate. This fibrous capsulation provides resistance to
outflow. Amount of outflow depends on the surface area of the
implant encapsulation and the capsular thickness. The nonvalved implants have a higher incidence of postoperative
hypotony. Either two stage surgery to implant the plate first
followed by 2nd stage tube implantation or a flow restrictive
suture in the initial postoperative period is recommended to
prevent hypotony.

Valved/flow restrictive
Ahmed
Krupin
Joseph
Optimed.

Valved implants have an internal mechanism to control the

outflow of the aqueous. Therefore, the fluid is not able to drain
unless a minimum IOP is reached. Once the threshold IOP is
reached, the device allows aqueous humor to flow. The valved
devices prevent hypotony.

Implants used in Pediatric Cases

Non-valved
Molteno: This was the 1st aqueous shunt to gain widespread
acceptance, with the initial model being released in 1976. It
can be single or double plate.
Baerveldt: This flexible elliptically shaped silicone rubber
device is available in three plate sizes 250/350/500 mm. The
current version has been modified with the introduction of
fenestrations to encourage tissue ingrowth.
Valved implants: Ahmed glaucoma valve (AGV) has been in
use for adults and children. The newer all silicon models have
shown more encouraging and improved results. The authors
experience in GDD is confined to AGV and the same will be
presented here.

Ahmed Glaucoma Valve (AGV)

Specifications of Pediatric Model of AGV

Surface area of plate96 mm2

Tube outer diameter0.635 mm
Tube inner diameter0.305 mm

When the IOP rises (8-12 mmHg), the valve opens, thus
letting fluid flow out of the eye through the drainage tube. The
valve automatically closes when the pressure is normal again.
Aqueous humor from AC of the eye flows through the tube into
the trapezoidal chamber within the plate element. This chamber

Table 7: Comparisons in complication rates between Ahmed glaucoma valve (AGV) and trabeculectomy with mitomycin C (MMCT)
AGV(%) eyes

MMCT(%) yes

Tube extrusion

Reposition of tube

0
0

Shallow AC

Transient: 10

Reformation: 14

Choroidal hemmorhage

Limited: 10

Massive: 5
10

Corneal blood staining

Scleral graft thinning

Choroidal detachment: drainage

Aqueous misdirection

Corneal decompensation

10

Lenticular opacity

29

Total no. of patients = 38, MMCT = 20, AGV = 18.

Journal of Current Glaucoma Practice, May-August 2010;4(2):57-71

67

Usha Yadava

is formed by a folded-over silicone elastomer membrane with

its free edges forming a one way valve.

Accessories

Tube extender and tube holding forceps.

In the rare event of tube retraction with the growth of the
eye, tube extension is possible using the extender. Adjunctive
tissue may be needed for better tube stability and to prevent its
exposure. Use of the following materials has been reported in
literature: pericardium, sclera, fascia lata and amnion.

Indications in Pediatric Glaucoma63,65,70,71

Failed glaucoma surgery

Failed medical control after surgery
Pseudophakic/aphakic glaucoma
Secondary glaucoma
Complicated PCG
Neovascular glaucoma.

Steps of AGV Implantation

Postoperative Treatment
Topical steroids and cycloplegia as in any other intraocular
surgery.

Advantages of GDD (Valved)67-71

Avoids the risk of a thin avascular filtering bleb.

Ciliary body function is preserved.
Safer surgery in large buphthalmic eyes.

How to Maximize Results of AGV

Conjunctiva should be adequate

Two episcleral bridle sutures; on either side of proposed
site
Must prime the valve
Subscleral tube insertion
Use only 23G needle for entry into AC
Do not hold implant at the trapezoid chamber
Use atraumatic forceps for holding tube
Introduce tube bevel up and in iris plane
Water tight conjunctival closure
Patch graft over the tube prevents late exposure.

Complications

68

Tube migration/retraction with

Diplopia
Tube exposure/extrusion
Posterior Tenons cyst
Tube blockage
Endothelial decompensation
Hypotony
Infection
Failure to control IOP

Corneal blood staining

Shallow AC
Scleral flap thinning
Choroidal detachment
Aqueous misdirection.

Brief Summary of Clinical Trials in

Pediatric Glaucoma
Earliest reported use of glaucoma drainage implants in pediatric
glaucomas dates back to 1970 by Molteno in eyes with
inadequately controlled IOP despite previous glaucoma
surgery.66-71
Dietlein et al performed different types of ab externo
procedures in 61 eyes of 35 consecutive patients of PCG.
20 of 61 eyes (1/3) needed a resurgery at 36 months of
median age.36
In 149 eyes of 89 patients of development glaucoma, Ikeda
et al reported complete success in 63.4% (71 eyes) and
qualified success in 25.9% (29 eyes). Best corrected visual
acuity of > 20/40 was observed in 59.5% (78 eyes). Age
< 2 years was found to be associated with poor prognosis
and needed more interventions.58
Englert et al found devastating complications in 20 eyes
treated by MMCT as against 5% in the GDD treated group.
Higher rate of complications like cataract and infections
has prompted some surgeons to look to GDD as a favorable
option especially in resurgery cases.70
Beck and associates reported a success rate ranging from
87% in one year to 53% in six years of follow-up in children
< 2 years of age. They also noted that drainage implantation
provides a greater successful control as compared to
MMCT. A relatively lower success rate of 36% was noted
in the MMCT.69
Armenian eye care project: 38 eyes of 34 patients underwent
either MMCT (20) or Ahmed valve (18) with a mean age
of 12.5 years for a variety of glaucomas, including secondary
glaucomas. The AGV group was twice as likely to use
postoperative antiglaucoma medication compared to
MMCT44% and 23% respectively. A remarkable drop
of 3 lines in visual acuity mostly due to onset of cataract in
the MMCT group was observed in 28% compared to 12%
in the AGV group (p value < 0.05%). Total success rate
was comparable in the two groups at 13.5 vs 14.8 mmHg
(p value < 0.05%).67
Overdam et al studied the role of Baerveldt implant in
55 eyes of 40 patients < 16 years of age out of which 35
patients had congenital glaucoma. Baerveldt (350 mm2)
implantation was performed. Surgical outcome was
evaluated by Kaplan-Meier table analysis. The overall
success rate was 80% at last follow-up, with a mean followup of 32 (range 2-78) months. Cumulative success was 94%
in 12 months and 24 months, 85% in 36 months, 78% in 48
months, and 44% in 60 months. 11 eyes (20%) failed
postoperatively because of an IOP >21 mmHg (eight eyes),
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JOCGP
Primary Congenital Glaucoma

persistent hypotony (two eyes), and choroidal hemorrhage

following cataract surgery (one eye). The most frequent
complication needing surgery was tube related (20%). A
new observation was mild to moderate dyscoria in 22% of
the eyes, all buphthalmic, caused by entrapment of a tuft of
peripheral iris in the tube track. The BGI is effective and
safe in the management of primary and secondary glaucoma.
When angle surgery has proved to be unsuccessful or
inappropriate in pediatric patients, a BGI is a good treatment
option. One must be prepared to deal with the tube related
problems.68
Another implant was introduced by Sussana in Brazil, which
combined the features of both Molteno and Baerveldt
implants. These implants were studied in 24 children of
primary congenital glaucoma with uncontrolled IOP despite
previous surgery. A success rate of 87.5% was noted at the
end of 1 year.71
In summary, it appears that valves do have a place in
pediatric glaucoma, though so far most reports are on patients
with failure of prior surgery. It may be speculative to presume
that results would be better in primary cases, where the tissues,
especially conjunctiva, are well preserved. Preliminary evidence
does support the need for a controlled trial of GDD as against
the prevalent CTT in this subset of glaucoma.

Role of Cyclodestructive Procedures

Trans-scleral cycloablation with Nd-YAG or diode has been

used safely in children and seems to have a palliative and
temporizing effect. Lower settings than adults are
recommended as these children have thin sclera.
Cyclocryotherapy has a significant risk of hypotony and
recommended in eyes with low vision potential and
cosmetically disfiguring eyes with high IOP only.

Role of Optical Keratoplasty105

Higher chances of graft failure

Suture infections in pediatric age
Arduous follow-up.

Prognosis of Childhood Glaucomas

These factors emerge decisive:
The severity of the disease
Age of onset and intervention
Number of surgeries
Timely and sustained control of IOP
Residual corneal scarring, anisometropia and astigmatism
Aggressive treatment of amblyopia.
Glaucoma in children presents a vexing problem. These,
patients are often brought in late, firstly due to the ignorance of
parents and secondly inadvertent delay caused by indecision
on the part of the attending physician. To make matters worse,
relative unfamiliarity with the condition extracts an arbitrary

approach at the level of primary care, which further adds to the

woes of the patients and soon all vision may be lost forever.
Undoubtedly, the issues of timely diagnosis, techniques in
children and parental motivation are some of the problems.
To sum up, it seems that surgical results are very case and
surgeon specific depending on their experience in case selection
and choosing the appropriate procedure. The time is now right
to let go of the old dogmas and letting in the new. Let us join
hands, organize newer multicentric control trials in childhood
glaucomas as the disease is a special concern of the ethnic
groups.

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