CONGENITAL GLAUCOMA PROF.DR.ÖZCAN OCAKOĞLU

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

PRIMARY CONGENITAL GLAUCOMA EPIDEMIOLOGY AND GENETICS RARE 1:10.000 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

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

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

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

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)

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

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.

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

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.

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

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

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

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.

OTHER SURGICAL TREATMENTS AHMED GLAUCOMA VALVE DIOD LASER CYCLOPHOTOCOAGULATION

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