edited by:R.N. Weinreb, A.L. Grajewski, M. Papadopoulos,
J. Grigg, S. Freedman
The goal of this consensus was to provide a foundation for
diagnosing and treating childhood glaucoma and how it can be best done in clinical
practice. Identification of those areas for which we have little evidence and, therefore,
the need for additional research also was a high priority. We hope that this consensus
report will serve as a benchmark of our understanding. However, this consensus report,
as with each of the others, is intended to be just a beginning. It is expected that
it will be revised and improved with the emergence of new evidence.
Table of contents
1. DEFINITION, CLASSIFICATION, DIFFERENTIAL DIAGNOSIS
Allen Beck, Ta chen Peter Chang, Sharon Freedman
2. ESTABLISHING THE DIAGNOSIS AND DETERMINING GLAUCOMA PROGRESSION
Maria Papadopoulos, James D. Brandt, Kazuhisa Sugiyama, Peng T. Khaw, Jocelyn
Chua, Simon Law, Alberto Betinjane, Joseph Abbott, Nick Strouthidis, Ta Chen Peter
Viney Gupta, Robyn Jamieson, Lisa Schimmenti,
John Grigg, David Mackey
Oscar Albis-Donado, Elena Bitrian,
Manju Anilkumar, Maria Cristina Brito, Tam Dang, Thomas Klink, Ming-Yueh Lee, Carmen
Mendez Hernandez, Ta Chen Peter Chang, Julian Garcia Feijoo, Ching Lin Ho, Sharon
5. GLAUCOMA SURGERY IN CHILDREN
Beth Edmunds, Mark Chiang, Anil Mandal, Alana L. Grajewski, Peng T. Khaw
6. PRIMARY CONGENITAL GLAUCOMA AND JUVENILE OPENANGLE GLAUCOMA
Ta Chen Peter Chang, John Brookes, Kara Cavuoto, Elena Bitrian, Alana L. Grajewski
7. GLAUCOMA ASSOCIATED WITH NON-ACQUIRED OCULAR ANOMALIES
Michael Banitt, Jocelyn Chua, Barbara Cvenkel, Pradeep Ramula, Hernan Iturriaga-Valenzuela,
Ahmed Abdelrahman, Arif Khan, Patrick Hamel, Ta Chen Peter Chang, Elizabeth Hodapp,
Oscar Albis-Donado, Maria Papadopoulos
8. GLAUCOMA ASSOCIATED WITH NON-ACQUIRED SYSTEMIC DISEASE
Caroline DeBenedictis, Alex Levin, Eugenio J. Maul, Alana
L. Grajewski, Ta Chen Peter Chang, Claudio I. Perez, Elena Bitrian
9. GLAUCOMA ASSOCIATED WITH ACQUIRED CONDITION
Karen Joos, Allen Beck, John Grigg, Ken Nischal, Alicia Serra-Castanera, Deborah
Vanderveen, Paolo Nucci, Matteo Sacchi, Sushmita Kaushik, Viney Gupta, Susmito Biswas,
Orna Geyer, Kimberley Miller, Ta Chen Peter Chang
10. GLAUCOMA FOLLOWING CATARACT SURGERY
Fenerty, Nicola Freeman, John Grigg
ADDENDUM - PATIENTS, PARENTS AND PROVIDERS AS PARTNERS IN
MANAGING CHILDHOOD GLAUCOMA
Tanuj Dada, Jugnoo Rahi, Shveta Jindal Bali,
Summary Consensus points
Index of authors
Summary Consensus Points
Section 1 – Definition, classification, differential diagnosis
- Childhood glaucoma is intraocular pressure (IOP) related damage to the eye.
Comment: In addition to the IOP, optic disc appearance and visual
fields, the definition of glaucoma also reflects the effect of IOP on other
ocular structures in infancy.
- The interpretation of IOP measurement in infants and young children, especially
during examination under anesthesia, can potentially be affected by many factors.
Comment: Other signs of glaucoma in infants and young children, such
as ocular enlargement, Haab striae and increased cup-to-disc ratio, may be more
important than the IOP value in the assessment.
- Childhood glaucoma is classified as primary or secondary. Secondary childhood
glaucoma is further classified according to whether the condition is acquired
after birth or is present at birth (non-acquired). Non-acquired childhood glaucoma
is categorized according to whether the signs are mainly ocular or systemic.
Comment: Terms such as ‘developmental’, ‘congenital’ or ‘infantile’ glaucoma
lack clear definition and their use is to be discouraged.
- A child should not be labeled as having glaucoma or subjected to surgical
treatment unless one is reasonably sure of the diagnosis and has excluded other
conditions that may mimic glaucoma.
Section 2 – Establishing the diagnosis and determining glaucoma progression
- Prompt diagnosis of childhood glaucoma and appropriate prompt treatment
can minimize the degree of visual impairment.
Comment: Examination under anesthesia or sedation may be appropriate
to make the diagnosis, perform surgery or plan further treatment.
- A child should not be labeled as having glaucoma or subjected to surgical
treatment unless one is reasonably sure of the diagnosis and has excluded other
conditions that may mimic glaucoma.
Comment: If doubt exists about the diagnosis or evidence of progression
cannot be determined, then appropriately timed follow-up or examination under
anesthesia or sedation is advisable.
Comment: Children should be encouraged onto the slit lamp for more accurate
evaluation [intraocular pressure (IOP) measurement and optic disc assessment]
when it appears this may be possible.
- Glaucoma in children is characterized by the presence of elevated IOP and
characteristic optic disc cupping. In addition to these features, glaucoma in
infancy is associated with ocular enlargement, buphthalmos.
Comment: IOP measurement and optic disc appearance are fundamental features
of the examination throughout the life of a child with glaucoma. In an infant
whose eye is still vulnerable to other effects of elevated IOP, proxies of persistent
elevated IOP (enlarging corneal diameter, increasing axial length and progressive
myopia) also need to be taken into consideration and regularly assessed.
Comment: In children, the conclusion with regard to the diagnosis or
progression of glaucoma must be based on the overall clinical findings and investigation
- IOP measurement in infancy and early childhood can be influenced
by many factors so is often unreliable when used in diagnosis and management.
- IOP response to anesthetic agents is unpredictable. All inhaled agents lower
IOP, sometimes rapidly and profoundly.
Comment: Chloral hydrate, ketamine and midazolam appear not to lower
Comment: Use the same anesthetic for serial examinations.
- Increasing corneal diameter is the hallmark of all forms of glaucoma in
infancy and early childhood.
Comment: Corneal enlargement due to elevated IOP usually occurs before
three years of age. Serial corneal diameter measurements are useful in establishing
the diagnosis and in the monitoring of progression of glaucoma up to the age
of three years.
Comment: Central corneal thickness (CCT) should not be used to adjust
IOP measurements as its role in childhood tonometry remains to be determined.
- Gonioscopy is crucial in making the correct diagnosis and for planning surgical
treatment. It should be performed at least once when possible.
- Optic disc appearance is an important and sensitive parameter for both diagnosis
and determination of progression in childhood glaucoma.
Comment: Optic nerve size, the cup-disc ratio, focal areas of rim loss,
and nerve fiber layer defects should be documented, preferably through a large
Comment: A magnified binocular view is preferable, so attempt to examine
a child on the slit lamp as soon as they are cooperative.
Comment: Documenting the appearance of the disc at baseline and follow-up
is desirable in determining both diagnosis and response to treatment.
Comment: Cupping reversal is common in successfully-treated childhood
Comment: Automated optic nerve imaging (e.g., OCT) is limited by the
lack of normative data and portability of the devices.
- Rapid changes in refractive status and axial length (AL) determination are
helpful in both diagnosing the disease and determining response to treatment
while the sclera remains vulnerable to the effects of elevated IOP.
Comment: AL outside normal limits is strongly suggestive of glaucoma.
Comment: Continued enlargement of AL beyond the normal range suggests
Comment: Progressive myopia is additional evidence of glaucoma progression.
- Assessment of the visual field in children can be useful but is challenging.
Comment: It may be helpful to use the shortest possible test (e.g., program
24-2 Sita Fast).
Comment: Repeat visual fields to confirm deficits. If repeated testing
shows consistent findings, the measurements are probably valid.
Comment: Although there is no normative database for children, age correction
of the mean deviation for standard automated perimetry is small (0.7 db/decade).
Moreover, useful metrics, such as pattern standard deviation, glaucoma hemifield
test and glaucoma change probability, are largely unaffected by age.
Section 3 – Genetics
- Genetic evaluation of childhood glaucoma is especially important in those
types of glaucoma when genotype phenotype correlations are known to exist.
Comment: The results of these tests may be important in counseling, prognosis
- There is a known correlation between primary congenital glaucoma (PCG) and
mutations in the CYP1B1 gene.
Comment: Performing carrier testing for at risk relatives is possible
if both disease causing alleles of an affected family member have been identified.
- Families affected by autosomal dominant juvenile open angle glaucoma (JOAG)
have been found to have mutations in the MYOC gene.
Comment: Genetic screening and genetic counseling could be considered
in these patients to help diagnose pre-symptomatic cases among first and second
degree relatives of these patients.
- Axenfeld-Rieger anomaly and syndrome have been associated with PITX2 and
Comment: PITX2 mutations are more likely to be associated with systemic
findings, while the risk of glaucoma is increased with FOXC1 duplications and
Comment: Prospective parents may consider genetic counseling for risk
- Aniridia is usually inherited in an autosomal-dominant fashion with high
penetrance and variable expressivity, and it is almost exclusively caused by
Comment: A child with sporadic aniridia should have ultrasound surveillance
for Wilms tumor unless genetic testing rules out a microdeletion involving the
Wilms tumor gene.
- The LTBP2 mutations seem to be involved in complex ocular phenotypes including
ectopia lentis, megalocornea (unrelated to elevated intraocular pressure), microspherophakia
and associated secondary glaucoma.
- To optimize genetic counseling for families, accurate clinical ophthalmic
diagnosis is critical.
Comment: Marked variation in penetrance and expression in primary and
secondary childhood glaucomas exist, so parents and siblings of an affected
child should be examined to provide maximally accurate phenotypic diagnoses
for the clinical geneticist.
- General pediatric assessment forms an important part of the management for
children with glaucoma and can greatly assist in identifying systemic associations
and initiating early management.
- Genetics review plays a number of important roles including confirming or
identifying syndromic diagnoses, recurrence risk assessment, genetic diagnosis,
interpretation of molecular data, and reproductive counseling where this may
be requested by the family after appropriate genetic counseling.
Section 4 – Medications
- Medications alone rarely show sustained efficacy as primary treatment for
glaucoma in infants and young children, especially in primary congenital glaucoma
Comment: Medical therapy is frequently needed as temporizing intraocular
pressure (IOP) lowering treatment before surgery or as adjuvant therapy after
partially successful surgical procedures in childhood glaucoma.
Comment: IOP-reducing medication may help reduce corneal edema prior
to surgery for childhood glaucoma.
Comment: Medical therapy should be considered first-line for some cases
of childhood glaucoma (e.g., uveitis-related, glaucoma after cataract removal).
- Childhood glaucomas are heterogeneous in their causation as well as in their
responses to different glaucoma medications.
- Systemic pharmocokinetics for glaucoma medications are different in children
than in adults.
Comment: Systemic absorption can be significant and may be reduced by
advising parents to close the lids (if possible), remove excess periocular liquid
and perform naso-lacrimal occlusion.
Comment: Use minimum frequency and concentration to achieve target IOP.
- Potentially serious or fatal systemic adverse reactions which are rarely
seen in adults may occur in young children after exposure to glaucoma medications.
Comment: Adverse side effects may manifest atypically in children (e.g.,
nocturnal cough with beta blockers rather than wheeze with reactive airways).
Comment: Brimonidine should be avoided in young children.
Comment: Children are more vulnerable to adverse effects of medications
as they may be unable to verbalize symptoms and parents may not readily recognize
Comment: Parents must be informed of the potential side effects.
- Compliance and adherence issues are greater and more complex in children
due to their dependence on caregivers or parents, possible lack of cooperation
in the administration of treatment, as well as concurrent medical conditions
that may complicate medical therapy.
- Target pressure must be chosen and reassessed with all available information
concerning whether glaucoma is adequately controlled.
Comment: Limitations on ability to perform structural and functional
testing of optic nerve make verification of glaucoma control more difficult
- Consider surgery when medical treatment fails to control glaucoma.
Comment: Glaucoma therapy in children has to be individualized, especially
in situations where the risk of surgery outweighs the benefits of continuing
Section 5 – Glaucoma surgery in children
- Surgery is a critical component of the management of childhood glaucoma.
Comment: It is important to prepare patients and parents or caregivers
for lifelong follow-up and possible future surgeries.
- Glaucoma surgery should preferably be performed by a trained surgeon in
centers where there is sufficient volume to ensure surgical experience and skill,
and safe anesthesia.
Comment: A long-term surgical strategy including choice of procedures
should be based on training, experience, logistics, and surgeon’s preference.
Comment: The first chance for surgery is often the best chance, and it
is important to choose the most appropriate operation.
- Glaucoma surgery in children is more challenging than in adults with a higher
failure and complication rate than in adults.
- Angle surgery (goniotomy and trabeculotomy – conventional or circumferential)
is the procedure of choice for primary congenital glaucoma with the exact choice
dictated by corneal clarity and the surgeon’s experience and preference.
Comment: Angle surgery success rates for secondary childhood glaucomas
are generally not as good as for primary congenital glaucoma (PCG) with certain
exceptions [e.g., glaucoma with acquired condition (uveitis) in juvenile idiopathic
- Trabeculectomy, when performed by experienced childhood glaucoma surgeons,
can be associated with good outcomes in appropriate cases.
Comment: Anti-scarring agents and other adjunctive techniques may be
- Glaucoma drainage devices (GDD) may offer the most effective long-term intraocular
pressure (IOP) control in many childhood glaucomas especially those that are
refractory to other surgical treatment.
Comment: There is no prospective evidence that anti-scarring agents influence
drainage device outcomes.
- Cyclophotocoagulation with the diode laser has limited long-term success
and often requires re-treatment and the continuation of medications.
- Other glaucoma procedures advocated in children for the treatment of glaucoma
have not been widely adopted either because of the technical challenges in buphthalmic
eyes or because they are yet to be proven efficacious or safe in children.
- Concurrent with glaucoma therapy, visual development needs to be evaluated
and optimized with ametropic correction and amblyopia therapy.
- With childhood glaucoma surgery, one needs carefully to consider the risks
and benefits of each intervention, especially in refractory cases when the fellow
eye is healthier, and in only eyes.
Comment: Whenever possible, the assent of the child should be sought
when making these difficult decisions.
Section 6 – Primary congenital glaucoma and juvenile open-angle glaucoma
- Primary congenital glaucoma (PCG) is the most common non-sydromic glaucoma
in infancy and is classified according to onset of signs. Its worldwide incidence
is variable and influenced by consanguinity.
- PCG is usually inherited in an autosomal recessive manner, with a family
history reported in 10-40% of cases and is more common in consanguineous populations.
Comment: Mutations in the CYP1B1 gene have been identified and
show variable expressivity and phenotypes.
Comment: Clinical screening of current and future siblings is essential
if there is parental consanguinity.
- The pathogenesis of PCG remains uncertain but the immature angle appearance
seen clinically is thought to result from the arrested maturation of tissues
derived from cranial neural crest cells.
- PCG is a surgical condition and the surgical procedure of choice is usually
angle surgery (goniotomy or trabeculotomy) with high rates of success reported
for both in favorable cases and after multiple procedures.
Comment: Combined trabeculotomy with trabeculectomy as an initial procedure
is suggested by some to be more successful than either procedure performed alone
in certain populations. There are no supporting prospective comparisons in the
- Once angle surgery fails, the next procedure of choice is either trabeculectomy
or a glaucoma drainage device.
- Juvenile open-angle glaucoma (JOAG) is a relatively rare form of childhood
glaucoma usually presenting after the age of four years, with a normal angle
appearance and no signs of other ocular anomalies or systemic disease.
- Depending on age, medical therapy is the first-line treatment for JOAG,
although surgery is often required.
- Evidence remains weak for the optimum first-line surgical intervention for
Section 7 – Glaucoma associated with non-acquired ocular anomalies
- Children who have non-acquired ocular anomalies often have systemic conditions
that require pediatric evaluation and/or treatment.
- Many non-acquired ocular anomalies are genetic in nature.
Comment: Screening of family members in such cases and genetic counseling
- Glaucoma related to non-acquired ocular anomalies may be present at birth
or may develop over time, so regular lifelong monitoring is necessary.
- Before glaucoma develops, one should consider treating elevated intraocular
pressure (IOP) associated with non-acquired ocular anomalies (secondary ocular
- Infantile onset of glaucoma related to non-acquired ocular anomalies is
associated with buphthalmos and the risk of Descemet membrane breaks.
- Medical treatment is usually first-line, but surgery is often required early
for congenital/infantile presentations and should not be delayed.
Comment: Angle surgery in infants may be effective although the results
are usually not as good as for primary congenital glaucoma (PCG).
Comment: Often trabeculectomy with anti-scarring agents or primary tube
surgery is necessary for IOP control.
Comment: Cyclodestruction may be considered after faiIed trabeculectomy
or tube surgery.
- There is considerable phenotypic variability associated with genetic mutations
recognized in children with non-acquired ocular anomalies.
- Axenfeld-Rieger (AR) anomaly is recognized now to represent a spectrum of
disease previously referred to as Axenfeld anomaly and Rieger anomaly. Axenfeld-Rieger
syndrome includes the ocular fi ndings of Axenfeld-Rieger anomaly with the addition
of systemic abnormalities.
Comment: Examination of family members including gonioscopy is important
to determine whether the patient is part of a larger pedigree or a new case.
- Peters anomaly is usually seen as an isolated ocular disorder but can be
associated with systemic abnormalities of neural crest origin and is referred
to as Peters plus syndrome.
Comment: It is important to exclude the presence of systemic involvement
and when it is present, to co-manage it with a pediatrician.
Comment: Assessing for glaucoma can be challenging as typical IOP measurement
over the central cornea may be inaccurate and the optic discs may not be visible.
Measure the IOP in a clear area of cornea if possible.
- Aniridia is commonly associated with glaucoma and is due to both open- and
Comment: Children with sporadic aniridia should be screened for Wilms
- Management of persistent fetal vasculature can be challenging because of
the heterogeneity of clinical presentation.
Comment: Surgical treatment is aimed towards obtaining useful vision
and preventing or treating secondary complications such as glaucoma.
Section 8 – Glaucoma associated with non-acquired systemic disease or syndrome
- Syndromes with system anomalies or systemic diseases that are present at
birth can be associated with ocular signs that include glaucoma.
Comment: Patients should be regularly monitored for glaucoma throughout
life and elevated intraocular pressure (IOP) treated, should it occur.
Comment: Patients also should be assessed for systemic manifestations
of their disease.
- Sturge-Weber syndrome (SWS) is commonly associated with glaucoma.
Comment: Periocular port-wine marks are associated with ipsilateral glaucoma.
Lid involvement and/or episcleral capillary vascular malformation appear to
further increase the risk of glaucoma.
Comment: Choroidal hemangioma increases the risk of serous choroidal
effusion and suprachoroidal hemorrhage with surgery, especially if the IOP drops
precipitously or hypotony develops. Modifications to the surgical technique
must be employed to minimize risk of hypotony.
Comment: Patients should be assessed, perhaps including neuroimaging,
for other manifestations of SWS.
- Neurofibromatosis (NF1) is uncommonly associated with glaucoma.
Comment: Optic pathway gliomas affect 12-15% of patients with neurofibromatosis
and can present with decreased vision distinct from glaucoma.
- Ectopia Lentis (EL) can present as an isolated ocular anomaly or be associated
with other ocular or systemic findings.
Comment: Patients with EL are at risk of acute pupillary block.
Comment: All patients without a proven or obvious cause of EL should
be tested for homocystinura by urine analysis and investigated for blood homocyteine
levels prior to any anesthesia or sedation because of life-threatening vascular
Comment: Patients with Marfan syndrome should have echocardiography and
cardiology consultation prior to surgery.
- Maintain close follow-up for infants with known or suspected congenital
rubella, since glaucoma signs might be less evident at birth in some cases.
Comment: Rubella keratitis should be differentiated from corneal findings
associated with IOP related corneal edema.
Section 9 – Glaucoma associated with acquired conditions
- Managing uveitic glaucoma in children is challenging. The control of intraocular
inflammation with adequate immunosuppression, topical and/or systemic agents,
is a crucial part of management.
Comment: Hypotony is a particular concern with surgery and modifications
to the surgical technique must be employed to minimize its risk.
- Traumatic glaucoma pathogenesis is multifactorial.
Comment: Patients with sickle cell disease (not trait) are at higher
risk for rebleed and are likely to develop glaucomatous optic nerve damage,
even with only moderately raised intraocular pressure (IOP).
Comment: Management is aimed at controlling IOP and minimizing damage
to the cornea and optic nerve. Consider surgical intervention with sustained
elevated IOP > 30 mmHg unresponsive to maximum medical therapy or if corneal
staining is present.
- Steroid-induced elevated IOP is not uncommon and may be severe in children
treated with ocular and systemic corticosteroids .
Comment: Consider discontinuing the corticosteroid if possible or switching
to a steroid sparing agent to ensure underlying disease control, which takes
Comment: IOP elevation may persist for months, years or even become permanent,
requiring medical or surgical intervention.
- Glaucoma secondary to intraocular tumors in children is a relatively rare
Comment: Patients can be symptomatic with acute glaucoma due to the fast
growth of the tumor, or symptom free in the case of a progressive, slow growing
Comment: In cases of unexplained glaucoma, the possibility of an intraocular
tumor should be considered, especially when a child presents with a severe chronic
uveitis associated with high IOP.
Comment: Incisional surgical intervention to lower IOP is contra-indicated
for glaucoma secondary to malignant ocular lesions.
- The causes of retinopathy of prematurity (ROP) induced glaucoma are multifactorial
in nature but largely due to secondary angle closure.
Comment: Glaucoma may develop years or decades later in patients with
treated or untreated Stage-4 or -5 ROP, so long-term surveillance is warranted.
Comment: IOP elevation may follow laser therapy for threshold ROP.
Section 10 – Glaucoma following cataract surgery
- Glaucoma following cataract surgery is that which occurs after pediatric
cataract removal of either congenital idiopathic cataract, cataract associated
with ocular or systemic syndromes and acquired cataract.
- Glaucoma can occur in aphakic and pseudophakic eyes.
- Young age at the time of cataract surgery and microcornea increase the risk
- Glaucoma usually occurs in eyes with open angles but angle closure can occur,
and it is important to elucidate the underlying mechanism when possible (e.g.,
- The risk of glaucoma following cataract surgery in children is lifelong,
so regular monitoring is necessary.
- Medical intraocular pressure (IOP) lowering therapy is usually first line
treatment for glaucoma following cataract surgery. When medical therapy fails,
surgical therapy is indicated but there is no consensus on the preferred approach.
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Robert N. Weinreb, MD
Consensus Initiative Chair
World Glaucoma Association