University of Gondar College of Medicine and Health Sciences

Department of Optometry
Advanced pediatric optometry

Childhood glaucoma

By : Merkineh M. & Tarekegn C. ( 1st year MSc in clinical Optometry)

Moderator :Ayanaw Tsega ( MSc, Assistant professor of clinical
optometry )
09/30/2021 childhood glaucoma 1
 Outline
 Introduction

 pathogenesis of childhood glaucoma

 clinical manifestation of childhood glaucoma
 Ocular or systemic conditions associated with childhood glaucoma
 Differential diagnosis for congenital glaucoma
 How to diagnosis childhood glaucoma
 Management modalities
 summary
 References
09/30/2021 childhood glaucoma 2
 Objective
At the end of this seminar you are expected to:
• Define glaucoma
• Explain the pathogenesis of congenital glaucoma
• Summarize the clinical manifestations of congenital glaucoma

• Explain ocular and systemic conditions associated with CG

• List differential diagnosis for CG
• Internalize how to diagnose CG
• Explain management modalities for CG
09/30/2021 childhood glaucoma 3
 Introduction
Glaucoma : refers to a group of diseases with Characteristic optic nerve
head damage and Visual field defect for which high intraocular
pressure is the major risk factors.
Classification
 primary open angle glaucoma
Secondary glaucoma
 Angle closure glaucoma
 Childhood Glaucoma
 primary congenital glaucoma (exist at birth)
 Infantile glaucoma ( birth to 3 Yrs)
 Juvenile Glaucoma (> 3 to teenage)
09/30/2021 childhood glaucoma 4
 Cont…
Pediatric glaucoma : constitute a heterogeneous group of diseases
that may result
 From an intrinsic disease or structural abnormality of the
aqueous outflow pathways (primary glaucoma)
 From abnormalities affecting other regions of the eye
(secondary glaucoma).
 Can be also associated with variety of systemic abnormalities

09/30/2021 childhood glaucoma 5

 Cont…
Primary congenital glaucoma(without associated anomalies)
 True congenital glaucoma (IUL- birth)
 Infantile congenital glaucoma (up to 3 yrs)
 Juvenile glaucoma (>3yrs)

09/30/2021 childhood glaucoma 6

 Pathogenesis of PCG
 Remains controversial.
 Primary congenital glaucoma is due to failure or abnormal
development of the trabecular meshwork or Mal development of
trabeculum including the iridotrabecular junction (trabeculo
dysgenesis) is responsible for impaired aqueous outflow resulting in
raised IOP.
 Barkan proposed that PCG is due to a thin, imperforate membrane
that covered the anterior chamber angle and blocked aqueous outflow

09/30/2021 childhood glaucoma 7

 Primary Congenital Glaucoma (PCG)
 usually occurs sporadically.

 Pattern of inheritance is an autosomal recessive trait.

 The incidence of PCG varies with different populations
 Increased incidence with high consanguinity

 Results in blindness in 2%- 15% of cases.

 Visual acuity is worse than 20/50 in at least 50% of cases.

09/30/2021 childhood glaucoma 8

 Cont…
 Is bilateral in about two-thirds of patients

 Occurs more frequently in males (65%) than in females

(35%).
 75% present during first year of life

09/30/2021 childhood glaucoma 9

 Cont…
 Diagnosis is made in only 25% of affected infants at birth.

 does not appear to be associated with adult primary open-

angle glaucoma.
 Juvenile-onset glaucoma is inherited as an AD trait and has
been linked to the myocilin gene

09/30/2021 childhood glaucoma 10

 Clinical manifestation of CG
Classic clinical triads:
 Epiphoria
 Photophobia
 Bleprarospasm

Other signs
 Corneal clouding
 Buphthalmos ( corneal diameter >13mmHg)
 Breaks in descement membrane (Haab straie)
 Pseudoproptosis
09/30/2021
Scleral thinning childhood glaucoma 11
 Cont…
 Deep anterior chamber
 Lens may be Sublaxated/ dislocated (due to stretching)
 Axial myopia may be due to increase in axial length

 Raised IOP
Lens dislocation
 Optic nerve cupping

Optic nerve cupping

09/30/2021 childhood glaucoma 12

 Ocular conditions associated with childhood
glaucoma
 Microphthalmos
 Corneal anomalies (microcornea, megalocornea, cornea plana,
sclerocornea, corneal staphyloma)
 Anterior segment dysgenesis (Axenfield-Rieger syndrome, Peters
anomaly, sclerocornea)
 Aniridia
 PHPV

 Lens anomalies (congenital cataracts, lens dislocation, micro-

spherophakia)
09/30/2021 childhood glaucoma 13
 Systemic conditions associated CG
 Sturge-Weber syndrome
 Neurofibromatosis
 Marfan syndrome
 Homocystinuria
 Lowe’s syndrome

09/30/2021 childhood glaucoma 14

 Differential diagnosis
 Megalo cornea
 Axial myopia
 Congenital NLDO
 Keratitis or uveitis
 Conjunctivitis
 Corneal epithelial abrasion
 Birth trauma
 Retinoblastoma
 Optic nerve pit, coloboma or physiological cupping
 Posterior polymorphous dystrophy
09/30/2021 childhood glaucoma 15
 Con….
 Intrauterine inflammations (congenital syphilis and rubella)

 Inborn metabolic errors (like mucopolysaachridoses)

 Sclerocornea

09/30/2021 childhood glaucoma 16

 Diagnosis
 Full history (maternal and birth)

 Visual acuity

 Anterior segment examination (with portable SLM, pen torch, loupe or

ophthalmoscope)
 UBM: to detect any associated anterior segment anomalies
 Corneal inspection (size, clarity, breaks, inter corneal diameter size difference )

 Tonometry (Tono pen, Perkin's and iCare best options) =>mean lop is between
10 and 15 mm Hg in newborns.
 CCT ( with portable pachymetry)

 Optic nerve examination

09/30/2021 childhood glaucoma 17
 Diagnosis
 Examination under anesthesia

 Corneal diameter ( using vernier calipers in infants)

 Measuring Axial length
 Refraction

 Gonioscopy

09/30/2021 childhood glaucoma 18

 Cornea diameter
Age Normal Suspicious for
possible glaucoma

Birth – 6 months
9.5 -11.5 mm > 12mm

1-2 years 10 -12 mm

> 12.5 mm

> 2 years < 12 > 13 mm

09/30/2021 childhood glaucoma 19

 Severity index for CG
parameter Normal mild Moderate Severe

Corneal Up to 10.5 > 10.5 -12 > 12 -13 >13

diameter (mm)
IOP ( mm Hg) 11.5 12-20 > 20 - 30 > 30
CDR 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 < 20/200-NLP

09/30/2021 childhood glaucoma 20

 Treatment
Medical
 Temporary measures to control IOP and to clear cloudy cornea
prior to surgery
Avoid use of :
 Brimonidine

 Apraclonidine

09/30/2021 childhood glaucoma 21

 Treatment
Surgical

 mainstay of treatment for PCG

 But it may not be effective for secondary pediatric glaucomas

 The treatment of most secondary glaucomas in childhood is similar to that of

open-angle or secondary glaucomas in adults.

1. Goniotomy : an incision is made, under direct gonioscopic visualization,

across the trabecular meshwork

Indications => clear corneas

09/30/2021
=> Its success rate ischildhood
aboutglaucoma
85% 22
 Cont…

2. Trabeculotomy :an external approach is used to identify,

cannulate, and then connect the Schlemm canal with the
anterior chamber through incision of the trabecular meshwork
from outside the anterior chamber.

Indication => hazy cornea

=> success rates similar to that of goniotomy

09/30/2021 childhood glaucoma 23

 Goniotomy Vs Trabeculotomy
Gonitomy
 Simple faster procedure

 Does not disturb conjunctiva

 Can be repeated one or more times

Trabeculotomy
 Can be done in edematous or scarred cornea

 Does not require introduction of sharp instruments in anterior chamber

 Does not require adaptation to gonioprism

 Can be converted to trabeculectomy

09/30/2021 childhood glaucoma 24

 Cont..
• Who are indicated for additional available options like
trabeculectomy, glaucoma implant procedures or cycloablative
procedures
=> children in who angle surgery is not successful.
=> those who are not indicated for angle surgery ( many of 2ndary
glucoma)
=> if medical therapy inadequate

09/30/2021 childhood glaucoma 25

Who are prone to treatment failure
 Children younger than 1 year

 Those who are aphakic

09/30/2021 childhood glaucoma 26

 Prognosis
 Good in asymptomatic patients diagnosed before 24
months.
 Guarded in symptomatic patients diagnosed after 24
months even if IOP controlled after surgery
 If PCG presents at birth at least half of the patients
becoming legally blind.
 With a corneal diameter greater than 14 mm at diagnosis
the visual prognosis is similarly poor.
– Up to 80%-90% of cases can be controlled with angle surgery.
– The remaining 10%-20% of these cases often present a lifelong
challenge.
09/30/2021 childhood glaucoma 27
 Summary
 Congenital Glaucoma is a potentially treatable cause of
irreversible childhood blindness
 EUA is the best method to clinically evaluate the pediatric
patient.
 Management is mostly surgical
 Post operative life long follow up is vital.

09/30/2021 childhood glaucoma 28

 Reference
• Congenital Glaucoma power point by: Dr. Sahil
Thakur
• Case presentation on congenital glaucoma by
Dr. Ajinkya Kulkarni
• Congenital glaucoma ppt by Shubham
Vasudeva
• BCSC : pediatric ophthalmology; chapter 20

09/30/2021 childhood glaucoma 29

 Th
an

09/30/2021
ky
ou
fo
r yo
ur
att
en
tio
n
!!!
! !!

childhood glaucoma
30