CONGENITAL GLAUCOMA PROF DR ZCAN OCAKOLU DEFINITION OF

CONGENITAL GLAUCOMA PROF. DR. ÖZCAN OCAKOĞLU

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

PRIMARY CONGENITAL GLAUCOMA l EPIDEMIOLOGY AND GENETICS l RARE l 1: 10. 000 LIVE BIRTHS l 50 -70% OF CONGENITAL GLAUCOMA l 60% DIAGNOSED BY 6 MONTHS l 80%- 1 ST YEAR OF LIFE l M>F (65%) l BILATERAL > UNILATERAL (70%) A HEREDITARY FACTOR IS OCCASIONALLY PRESENT

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

PATHOGENESIS OF PCG l THE IOP ELEVATION IS CAUSED BY MALDEVELOPMENT OF THE ANTERIOR CHAMBER ANGLE DURING INTRAUTERINE LIFE FIgure 1: The normal chamber angle l IN THESE INFANTS, THE AQUEOUS HUMOR DOES NOT PROPERLY DRAIN TO OUTFLOW PATHWAYS BECAUSE OF MALDEVELOPMENT OF ANTERIOR X CHAMBER ANGLE l HIGH IRIS INSERTION l THICKNESS OF UVEAL MESHWORK FIgure 2: An underdeveloped chamber angle INTRAOCULAR PRESSURE IS HIGH

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

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

l 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 str. Iae

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

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

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

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

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

GONIOTOMY l THE GONIOTOMY INVOLVES l ENTERING THE ANTERIOR CHAMBER WITH A SHARP GONIOTOMY KNIFE l MAKING AN OPENING INCISION THROUGH THE ABNORMALLY DEVELOPED UVEAL MESHWORK l 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

TRABECULOTOMY l TRABECULOTOMY INVOLVES l MAKING AN EXTERNAL INCISION l IDENTIFYING THE SCHLEMM’S CANAL FROM THE OUTSIDE l INSERTING A FINE INSTRUMENT INTO THE SCHLEMM’S CANAL l 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.

OTHER SURGICAL TREATMENTS AHMED GLAUCOMA VALVE DIOD LASER CYCLOPHOTOCOAGULATION

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