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PRIMARY CONGENITAL

GLAUCOMA
SGD B4
FACULTY OF MEDICINE UDAYANA UNIVERSITY
DENPASAR
2015

Introduction
Ocular disorders that result in optic nerve damage, often
associated with increase intraocular pressure
By the 2020, the prevalence 58,6 million worldwide
3,4 million in United State.
Congenital
Primary

Glaucoma
Secondary
Absolute

relatively rare.
Primary congenital
glaucoma is
estimated to affect
less than 0,05% of
opthalmic
patients and
0,05% of children

Defination
characterized by:
- elevated intraocular pressure (IOP)
- enlargement of the globe (buphthalmos),
- edema,
- opacification of the cornea with rupture of
Descemet's membrane (Haabsstriae),
- thinning of the anterior sclera and iris atrophy,
anomalously deep anterior chamber
- and structurally normal posterior segment except
for progressive glaucomatous optic atrophy

Etiology
several theories
- membrane covering the anterior chamber angle
(Barkan membrane),
- trabecular meshwork obstruction,
- developmental arrest of anterior chamber tissue
in utero

Cilinical Features
excessive tearing
light sensitivity
a large loudy cornea ( the normally clear
front surface of the eye) which can cause the
iris (colored part of the eye) to appear dull

Diagnosis
The exam is almost always done in operating
room under general anaesthesia because it can
be difficult to examine the eyes of baby or
small child. Doctor need : measure the
pressure inside the eye, thoroughly examine all
parts of the eye.

While under anesthesia the ophthalmologist


evaluates :
the intraocular pressure (for elevation)
cornea diameter (for increased size)
cornea clarity (for cloudiness and Haabstriae
which are breaks in the back surface of the
cornea)
axial length (for elongation of the eye- caused by
stretching from increased pressure)
refractive error (for myopia- also caused by
stretching)
and the optic nerve (for abnormal cupping which
infers optic nerve damage)

Such children may also be able to participate in


a diagnostic visual field exam evaluate
peripheral vision any significant optic
nerve damage.

Ultrasonography (USG) B-scan to rule out


any posterior segment pathology.
For more prolonged examination and surgery,
an endotracheal tube is necessary.
Laboratory :
Hybridization analysis using hybridization of a
mutant nucleic acid probe to the CYP1B1 gene
Direct mutation analysis by restriction digest
Sequencing of the CYP1B1 gene
Hybridization of an allele-specific oligonucleotide
with amplified genomic DNA
Identification of the presence of mutant proteins
encoded by the CYP1B1 gene

Differential Diagnosis
Depends on the major presenting symptom, the
differential diagnosis include :
a.Cloudy cornea at birth and edema Obstetric
trauma, Intrauterine rubella, Metabolic
disorders
(mucopolysaccharidoses),
Congenital
hereditary
endothelial
dystrophy, Congenital ocular anomalies like
sclerocornea or Peters anomaly
b.Corneal enlargement Megalocornea
c.Epiphora Congenital obstruction of the
nasolacrimal duct
d.Secondary infantile glaucoma : trauma,
ectopia lentis, uveitis, tumors, retinopathy
of prematurity and persistent hyperplastic
primary vitreous, corticosteroid related
glaucoma

megalocornea

Treatment
Surgery treatment such as :
a. Goniotomy
designed to create a route for aqueous
drainage through Schlemms canal by
incision of the trabecular meshwork under
direct visualization
b. Trabeculotomy
involves disrupting the tissue between
Schlemms canal and the anterior chamber
using an ab externo approach to create direct
communication

c. Trabeculectomy
section of trabecular meshwork
and Schlemms canal is removed
under a partial thickness sclera flap
to create a wound fistula

Medication To avoid severe metabolic


acidosis, topical drops are preferred to
systemic suspensions. Medication used such as
:
Beta-blockers (timolol)
extensively and show improvement in
intraocular pressure, but may lead to respiratory
complications in predisposed young children
Parasympathomimetics (pilocarpine)
Sympathomimetics :
adrenergic agonist
alpha-2 adrenergic receptor agonist severe
side effects and must be used with caution in
infants and children
Carbonis anhydrase inhibitors generally
safe and well tolerated
Prostaglandin agonist showing mostly
nonresponders
in
the
Primary
Congenital
Glaucoma group.

SUMMARY
Glaucoma is a group of ocular disorders that
result in optic nerve damage, often associated
with increase intraocular pressure
Primarycongenitalglaucoma
(PCG)
is
characterized by
elevated intraocular pressure (IOP)
enlargement of the globe (buphthalmos)
edema
opacification of the cornea with rupture of
Descemet's membrane (Haabs striae)
thinning of the anterior sclera and iris
atrophy
anomalously deep anterior chamber
and structurally normal posterior segment
except for progressive glaucomatous optic
atrophy

This disease usually is diagnosed at birth or


shorthly thereafter, most cases are diagnosed
in the first year of life.
The etiology of primary congenital glaucoma is
unknown but here are several theories. Eye
examination is needed to accurately diagnose
PCG and determine the proper treatment.
The treatment of congenital glaucoma remains
a challenge as the surgical options are limited
by the short window of intervention available
before the disease results in devastating
complications.

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