PROF.DR.

ZCAN OCAKOLU

Congenital
Glaucoma

Congenital glaucoma is a rare form of glaucoma

Affected infants may be born with a high

intraocular pressure or may develop an increased

IOP within the first weeks of life".
Both eyes are usually involved, but to varying

severity
Boys are affected slightly more frequently than girls.

A hereditary factor is occasionally present.

The IOP elevation is caused by the failure of

the anterior chamber angle and the

trabecular meshwork to develop
appropriately during intrauterine
development.
In these infants, the aqueous humor does not
properly drain, but since the production of
aqueous humor is nevertheless normal,
The intraocular pressure is high

Figure1: The normal chamber angle: on the left is a

histological cross-section; on the right is a drawing of the
same

Figure 2: An underdeveloped chamber angle

Depending on the IOP level, glaucomatous

Consequences
of
an
damage is inevitable after weeks, months or
even years.
Increased
IOP during
This basically occurs via the same
Infancy
mechanisms as in the adult.

In addition to optic nerve damage, the globe

(eyeball) enlarges because the sclera in the

eye of a baby is distensible.

Clinical
appearance

Enlargement of the globe (buphthalmos) is a

result of elevated intraocular pressure.

The anatomic landmarks are displaced.

The anterior chamber is deep
All segments of the outer eye, but especially the

cornea and sclera, expand.

Principally at the corneoscleral junction

However, certain layers of the cornea are not

very elastic, and stretching may result in small

tears(Haabs striae) that cause a certain
degree of corneal opacification.

Haabs striae

Haabs striae

Corneal epithelial edema caused by

elevated intraocular pressure and failure of

the corneal endothelial pump mechanism.
Epifora, photofobia and blepharospazm (clinical

triad)

If the IOP is lowered, this opacity is partially

reversible.
As a result of the optic nerve damage and/or

corneal opacity, children with congenital

glaucoma may be permanently visually
impaired.

Diagnosis of
Clinical clues
Congenital
Glaucoma
Enlarged eyes; tearing,
and photophobia
(avoidance of light).
Often, babies also rub their eyes.

If CG is suggested, a thorough examination

under general anesthesia is necessary.

to avoid blepharospasm (spasmodic closure of the

eyes). to prevent a transient rise in the IOP.

Besides measuring the IOP, anesthesia allows a
thorough investigation of all segments of the eye
and, in particular, the optic disc.

EPIPHORA

CORNEAL EDEMA

HIGH IRIS INSERTION

HAABS STRIAE

ON GONIOSCOPY

Infantile Glaucoma
Infantile glaucoma is also congenital

glaucoma
However, intraocular pressure starts to rise at
some time during the first years of life.
The cause for this IOP increase is basically the
same as in congenital glaucoma, but it occurs
later since the anterior chamber angle is more
mature than when glaucoma is present at birth.
The IOP may be normal during the first years of
childhood and then gradually increase.

Juvenile Glaucoma

Juvenile glaucoma is an IOP increase that

occurs in an older child or young adult and is

often inherited.
During a thorough examination, the
ophthalmologist may find discreet evidence of
an incomplete maturation of the chamber angle,
The clinical features as well as treatment of
juvenile glaucoma are quite similar to adult
Primary Chronic Open-Angle Glaucoma
(POAG)

Treatment
The treatment is primarily surgical.
Different surgical procedures

(according to the degree of the maldevelopment

and the clarity of the cornea)

GONIOTOMY

Goniotomy
Trabeculotomy
Trabeculotomy + trabeculectomy

Supplemental treatment options are

Medical therapy
Implant surgery
Cyclodestructive procedures

TRABECULOTOMY