CONGENITAL

GLAUCOMA
WAQAAS AKMAL

Objectives
1.

Definition

2.

Classification

3.

Epidemiology and genetics

4.

Etiology

5.

Clinical features

6.

Management

Definition of glaucoma
Glaucomais

a group of eye conditions

that damage the optic nerve. This
damage is mainly caused by an
abnormally high intraocular pressure.

Classification

Primary Congenital glaucoma

1.

Angle anomaly present at birth

2.

Elevated IOP during first year of life

3.

No associated anomalies

Secondary Glaucoma
i.

Ocular developmental anomalies

ii.

Ocular disease

iii.

Systemic disorders

Classification of Primary
Congenital Glaucoma
In which IOP becomes elevated during
intrauterine life.(40%)
True

Infantile

Manifests prior to the third birthday. (55%)

Least common. Pressure rise develops after

the third birthday but before the age of 16
Juvenile years.

Epidemiology and genetics

50% of cases of pediatric glaucoma

Incidence 1:10 000 at birth

10-12% are positive family histories

Bilateral in 70% of cases

2/3 of patients are males

IOP elevation

40% at birth

80% at 6 months

90% by 1 year of age

Epidemiology and genetics

GLC3 gene

Mutation of cytochrome P4501B1 gene i.e. CYP1B1

chromosome 2p21

Metabolism of a molecule needed for normal development

and function of anterior segment

GLC3B

Chromosome 1p36

Etiology

Presence of Barkans membrane in front of trabeculum

persistent mesodermal tissue

Etiology

Presence of Barkans membrane in front of trabeculum

persistent mesodermal tissue

Presence of cellular membrane in front of trabecular meshwork

Trabeculodysgenesis

Thickening of juxtacanalicular component

Maldevelopment of Schlemms canal

Anterior insertion of iris and ciliary body

Anterior insertion of iris and ciliary body

Clinical features
Lacrimation

Corneal
haze
Blepharospas
m

Photophobia

Buphthalmos

Examination

Increased IOP

Enlarged corneal diameter (normal = 10mm)

a.

12mm before 1 year is suspicious

b.

13mm after 1 year is diagnostic

c.

14mm is typical of advanced stage

Edema

Corneal edema

Examination

Increased IOP

Enlarged corneal diameter (normal = 10mm)

a.

12mm before 1 year is suspicious

b.

13mm after 1 year is diagnostic

c.

14mm is typical of advanced stage

Edema

Haabs striae

Healed breaks in the Descemets membrane that

appear as curvilinear lines

Examination

Increased IOP

Enlarged corneal diameter (normal = 10mm)

a.

12mm before 1 year is suspicious

b.

13mm after 1 year is diagnostic

c.

14mm is typical of advanced stage

Edema

Haabs striae

Lens subluxation

Axial myopia

Glaucomatous cupping

Reduced visual acuity

Optic atrophy, corneal edema, astigmatism, dislocation of lens, high
myopia and amblyopia

DDx

Increased IOP
Retinoblastoma (RB)
Retinopathy of prematurity (ROP)
Persistent hyperplastic primary vitreous

Excessive

tearing
Congenital nasolacrimal duct obstruction

Congenital

enlargement
Megalocornea
High myopia

Corneal

clouding
Birth trauma
Inflammatory corneal disease
Hereditary corneal disease
Keratomalacia
Corneal malformation

Diagnosis

Raised IOP

Enlargement of cornea

Gonioscopic findings

Optic disc cupping

Management
Medicinal Therapy

Beta-blockers

Carbonic anhydrase inhibitors e.g. Topical dorzolamide or

systemic acetazolamide

NOT MIOTICS

Surgery

Goniotomy

Goniotomy

Management
Medicinal Therapy

Beta-blockers

Carbonic anhydrase inhibitors e.g. Topical dorzolamide or

systemic acetazolamide

NOT MIOTICS

Surgery

Goniotomy

Trabeculotomy

Trabeculectomy

Trabeculectomy

Management
Medicinal Therapy

Beta-blockers

Carbonic anhydrase inhibitors e.g. Topical dorzolamide or

systemic acetazolamide

NOT MIOTICS

Surgery

Goniotomy

Trabeculotomy

Trabeculectomy

Artificial drainage shunt

Thank You.
REFERENCES:

CLINICAL OPHTHALMOLOGY: A SYSTEMATIC APPROACH,7TH EDITION. KANSKI

MEDSCAPE