1. CONGENITAL GLAUCOMA
PROF.DR.ÖZCAN OCAKOĞLU

2. DEFINITION OF TERMS PRIMARY CONGENITAL / INFANTIL GLAUCOMA
PRESENT AT BIRTH OR 1ST FEW YEARS OF LIFE (< 3 YEARS OLD)
ANTERIOR CHAMBER ANGLE ABNORMALITIES
NO SYSTEMIC ANOMALIES
JUVENIL GLAUCOMA >3 Y/O
SECONDARY INFANTIL GLAUCOMA
ASSOCIATED WITH INFLAMATORY,NEOPLASTIC, HAMARTOMATOUS , METABOLIC OR OTHER CONGENITAL ABNORMALITIES
APHAKIC OR PSEUDOPHAKIC CHILDREN

3. PRIMARY CONGENITAL GLAUCOMA
EPIDEMIOLOGY AND GENETICS
RARE
1: LIVE BIRTHS
50-70% OF CONGENITAL GLAUCOMA
60% DIAGNOSED BY 6 MONTHS
80%- 1 ST YEAR OF LIFE
M>F (65%)
BILATERAL > UNILATERAL (70%)
A HEREDITARY FACTOR IS OCCASIONALLY PRESENT

4. DIFFERENT TYPES OF PCG ACCORDING TO PATHOLOGY
ISOLE TRABECULODYSGENESIS (MOST COMMONLY)
IRIDOTRABECULODYSGENESIS
CORNEOTRABECULODYSGENESIS
MORE SERIOUS

5. INTRAOCULAR PRESSURE IS HIGH
PATHOGENESIS OF PCG
THE IOP ELEVATION IS CAUSED BY MALDEVELOPMENT OF THE ANTERIOR CHAMBER ANGLE DURING INTRAUTERINE LIFE
IN THESE INFANTS, THE AQUEOUS HUMOR DOES NOT PROPERLY DRAIN TO OUTFLOW PATHWAYS BECAUSE OF MALDEVELOPMENT OF ANTERIOR CHAMBER ANGLE
HIGH IRIS INSERTION
THICKNESS OF UVEAL MESHWORK
FIgure1: The normal chamber angle X
FIgure 2: An underdeveloped chamber angle
INTRAOCULAR PRESSURE IS HIGH

6. SYMPTOMS OF PCG THERE ARE 3 COMMON SYMPTOMS
EXCESSIVE TEARING (EPIPHORA)
LIGHT SENSITIVITY (PHOTOPHOBIA)
DUE TO CORNEAL OEDEMA
FIRMLY CLOSURE OF THE EYELIDS (BLEPHAROSPASM)
DUE TO EXCESSIVE SENSITIVITY TO LIGHT

7. CLOUDY, HAZY CORNEA (CORNEAL EDEMA)
SIGNS OF PCG
ELEVATED IOP > 15 mmHg
ENLARGED LENGTH OF THE EYE (BUPHTHALMUS)
THE GLOBE (EYEBALL) ENLARGE BECAUSE THE SCLERA IN THE EYE OF A BABY UNTIL FIRST THREE YEARS IS DISTENSIBLE
ENLARGED CORNEA >12 mm
CLOUDY, HAZY CORNEA (CORNEAL EDEMA)

8. 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

9. DIAGNOSIS OF PCG CLINICAL CLUES OF PCG
ENLARGED EYES; TEARING, AND PHOTOPHOBIA
OFTEN, BABIES ALSO RUB THEIR EYES.
IF PCG IS SUGGESTED, GENERAL ANESTHESIA IS NECESSARY.

10. GONIOSCOPY NO MEMBRAN AT ANTERIOR CHAMBER ANGLE OPEN ANGLE
ABSENCE OF ANGLE RECESS
HİGH AND FLAT IRIS INSERSION
THICKENED UVEAL TM
ANTERIOR INSERSION OF IRIS
CONCAVE IRIS POSITION

11. EVENTUALLY, IF PCG DO NOT TREAT PROPERLY, THE OPTIC NERVE WILL BECOME DAMAGED (AS LIKE ADULT GLAUCOMA)
HOWEVER, UNLIKE ADULT GLAUCOMA, THE OPTIC NERVE DAMAGE IN CONGENITAL GLAUCOMA MAY BE REVERSIBLE IN THE EARLY STAGES OF DISEASE
IF THE GLAUCOMA IS TREATED PROMPTLY AND EFFECTIVELY.

12. DIFFERENTIAL DIAGNOSIS
EXCESSIVE TEARING
NASOLACRIMAL DUCT OBSCTRUCTION
CORNEAL/CONJUNCTIVAL ABRASIONS
CONJUNCTIVITIS
CORNEAL ENLARGEMENT
X-LİNKED MEGALOCORNEA
EXOFTHALMUS
CORNEAL CLOUDING/TEARS
BIRTH TRAUMA
CHED
METABOLIC DISORDERS
OPTIC DISC EXCAVATION
PHYSIOLOGICAL LARGE EXCAVATION
CONGENITAL ANOMALIES OF OD

13. TREATMENT OF PCG THE TREATMENT OF PCG IS PRIMARILY SURGICAL
DIFFERENT SURGICAL PROCEDURES (ACCORDING TO THE DEGREE OF THE MALDEVELOPMENT AND THE CLARITY OF THE CORNEA)
GONIOTOMY
TRABECULOTOMY
THE OTHER SURGICAL PROCEDURES
IMPLANT SURGERY
CYCLODESTRUCTIVE PROCEDURES
MEDICAL THERAPY IS ONLY SUPPLEMENTAL TREATMENT OPTION
GONIOTOMY
TRABECULOTOMY

14. GONIOTOMY THE GONIOTOMY INVOLVES
ENTERING THE ANTERIOR CHAMBER WITH A SHARP GONIOTOMY KNIFE
MAKING AN OPENING INCISION THROUGH THE ABNORMALLY DEVELOPED UVEAL MESHWORK
TO ALLOW TO REACH AQUEOUS FLUID INTO SCHLEMM’S CHANNEL
OFTEN 120 DEGREES (OUT OF 360 DEGREES TOTAL) OF THE TRABECULAR MESHWORK CAN BE TREATED WITH GONIOTOMY IN A SINGLE SETTING

15. TRABECULOTOMY TRABECULOTOMY INVOLVES
MAKING AN EXTERNAL INCISION
IDENTIFYING THE SCHLEMM’S CANAL FROM THE OUTSIDE
INSERTING A FINE INSTRUMENT INTO THE SCHLEMM’S CANAL
BREAKING THROUGH THE TRABECULAR MESHWORK TO INCREASE THE AQUEOUS OUTFLOW
ONE ADVANTAGE OF TRABECULOTOMY OVER GONIOTOMY IS THAT A CLEAR CORNEA IS NOT NECESSARY TO PERFORM THE PROCEDURE, WHILE A REASONABLY CLEAR CORNEA IS NECESSARY FOR GONIOTOMY.

16. OTHER SURGICAL TREATMENTS
AHMED GLAUCOMA VALVE
DIOD LASER CYCLOPHOTOCOAGULATION

17. DECREASED VISUAL ACUITY
OPTIC ATROPHY
CORNEAL CLOUDING
ASTIGMATISM
AMBLYOPIA
CATARACT
LENS DISLOCATION
RETINAL DETACHMENT