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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