PROF.DR.ÖZCAN OCAKOĞLU

Congenital GlaucomaCongenital 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.

The CauseA 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

Consequences of an Increased IOP during InfancyDepending on the IOP level,

glaucomatous damage is inevitable after weeks, months or even years.

This basically occurs via the same 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. 

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

Clinical appearance

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 striaeHaabs 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 Congenital GlaucomaClinical clues

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

HAAB’S STRIAE

CORNEAL EDEMA

HIGH IRIS INSERTION

ON GONIOSCOPY

Infantile GlaucomaInfantile 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 GlaucomaJuvenile 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)

TreatmentThe treatment is primarily surgical.Different surgical procedures

(according to the degree of the maldevelopment and the clarity of the cornea)GoniotomyTrabeculotomyTrabeculotomy + trabeculectomy

Supplemental treatment options areMedical therapy Implant surgeryCyclodestructive procedures

TRABECULOTOMY

GONIOTOMY