1. CONGENITAL GLAUCOMAS
PROF.DR.ÖZCAN OCAKOĞLU

2. 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.

3. The Cause 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

4. 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

5. Consequences of an Increased IOP during Infancy
Depending 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. 

6. 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

7. 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

8. Corneal epithelial edema caused by elevated intraocular pressure and failure of the corneal endothelial pump mechanism.
Epifora, photofobia and blepharospazm (clinical triad)

9. 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.

10. Diagnosis of Congenital Glaucoma
Clinical 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.

11. EPIPHORA
CORNEAL EDEMA
HIGH IRIS INSERTION
ON GONIOSCOPY
HAAB’S STRIAE

13. 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.

14. 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)

15. Treatment The treatment is primarily surgical.
Different surgical procedures
(according to the degree of the maldevelopment and the clarity of the cornea)
Goniotomy
Trabeculotomy
Trabeculotomy + trabeculectomy
Supplemental treatment options are
Medical therapy
Implant surgery
Cyclodestructive procedures
GONIOTOMY
TRABECULOTOMY

16. Goniotomy

17. Trabekülotomi