CONGENITAL CATARACTBY: DR NIKITA JAISWAL

PG 1ST YEAR IMS AND SUM HOSPITAL

THESE CATARACTS ARE PRESENT AT BIRTH OR THAT DEVELOP WITHIN THE FIRST YEAR OF LIFE ARE CALLED

CONGENITAL /INFANTILE CATARACT.

FAIRLY OCCURRING IN 1 OF EVERY 2000 LIVE BIRTHS -SOME LENS OPACITIES DO NOT PROGRESS AND ARE USUALLY INSIGNIFICANT

-OTHERS CAN PRODUCE PROFOUND VISUAL IMPAIRMENT

CONGENITAL CATARACT -- UNILATERAL -- BILATERAL

IN GENERAL THESE CONGENITAL CATARACT1/3RD EXTENSIVE SYNDROMES

1/3RD INHERITED TRAIT1/3RD UNDETERMINED CAUSE

BASED ON ETIOLOGY

• BILATERAL

• IDIOPATHIC• HEREDITARY-AUTOSOMAL DOMINANT -AUTOSOMAL RECESSIVE/X-LINKED• GENETIC/METABOLIC DISEASE-DOWN SYNDROME,MARFAN SYNDROME-HYPOGLYCAEMIA,HYPOPARATHYROIDISM-MYOTONIC DYSTROPHY• MATERNAL INFECTION-RUBELLA,CMV,VARICELLA,SYPHILIS,TOXOPLASMOSIS• OCULAR ANOAMLIES-ANIRIDIA• TOXIC –CORTICOSTEROIDS,RADIATION

• UNILATERAL• IDIOPATHIC• OCULAR ANOMALIES-POST.LENTICONUS-POST POLE TUMORS-PERSISTENT FETAL VASCULATURE• TRAUMATIC• RUBELLA • MASKED B/L CATARACT

CONGENITAL CATARACT IN A VARIETY OF MORPHOLOGIC CONFIGURATION

LAMELLARPOLAR

SUTURALCORONARY CERULEANCAPSULAR

COMPLETE & MEMBRANOUS

LAMELLAR: IT IS ALSO KNOWN AS ZONULAR CATARACT

-THESE ARE AUTOSOMAL DOMINANT TRAIT

-EFFECT ON VISUAL ACUITY WITH THE SIZE & DENSITY OF THE OPACITY

-THESE ARE OPACIFICATIONS OF SPECIFIC LAYERS/ZONES OF THE LENS

-VISIBLE AS AN OPACIFIED LAYER THAT SURROUNDS A CLEARER CENTER & IS ITSELF SURROUNDED BY A LAYER OF CLEAR CORTEX

-DISC SHAPED CONFIGURATION-RIDERS-THESE ARE HORSESHOE SHAPED OPACITIES.

POLAR CATARACT:LENS OPACITY INVOLVES SUBCAPSULAR CORTEX&CAPSULE OF ANTERIOR OR POSTERIOR

POLE OF THE LENS.

ANT POLAR CAT.-IT IS A.D SMALL,B/L SYMMETRIC,NON PROGRESSIVE OPACITIES THAT

DO NOT IMPAIR VISION.POST POLAR CAT.-IT PRODUCES MORE VISUAL IMPAIRMENT

BECAUSE IT TENDS TO BE LARGER IN SIZE

THEY MAY BE-FAMILIAL-USUALLY B/LSPORADIC-OFTEN UNILATERAL

SUTURAL:THE SUTURAL OR STELLATE CATARACT IS AN OPACIFICATION OF THE “Y” SUTURES OF THE FETAL NUCLEUS

-IT DOESNOT IMPAIR VISION-THESE OPACITIES OFTEN HAVE BRANCHES OR KNOBS PROJECTING FROM

THEM.

CORONARY: A.DGROUP OF CLUB SHAPED CORTICAL OPACITIES THAT ARE

ARRANGED AROUND THE EQUATOR OF LENS LIKE A CROWN

--THEY CANT BE SEEN UNTILL THE PUPILS ARE DILATED--USUALLY DO NOT AFFECT THE VISUAL ACUITY

CERULEAN:SMALL BLUISH OPACITIES LOCATED IN THE LENS CORTEX

--HENCE THEY ARE ALSO K/AS BLUE DOT CATARACT

--NON-PROGRESSIVE USUALLY DO NOT CAUSE VISUAL SYMPTOMS

CAPSULAR-THESE CATARACTS ARE SMALL OPACIFICATIONOF THE LENS EPITHELIUM & ANTERIOR

LENS CAPSULE THAT SPARE THE CORTEXCOMPLETE- ALSO K/AS TOTAL CATARACT

ALL THE LENS FIBRES ARE OPACIFIED.THE RED REFLEX IS TOTALLY OBSCURED

RETINA CANT BE SEEN BY DIRECT /INDIRECT OPH.

SPOKE LIKE FABRY’S DISEASE

VACUOLES MANNOSIDOSISDIABETES

MULTICOLOR FLECKS HYPOPARATHYRODISMMYOTONIC DYSTROPHY

GREEN “SUNFLOWER” WILSON’S DISEASE

THIN DISCIFORM LOWE’S SYNDROME

LAMELLAR GALACTOSEMIAHYPOGLYCEMIA

RUBELLA- CAUSED BY RUBELLA VIRUS

CAN CAUSE FETAL DAMAGE ESPECIALLY IF THE INFECTION OCCURRS IN 1st TRIMESTER OF

PREGNANCY.

PEARLY WHITE OPACIFICATIONSENTIRE LENS IS

OPACIFIED & CORTEX MAY LIQUEFY

LIVE VIRUS PARTICLES MAY BE RECOVERED AS LATE AS 3 YRS AFTER

BIRTH CATARACT REMOVAL MAY BE

COMPLICATED BY EXCESSIVE POST-OP INFLAMMATION RELEASE BY

THESE LIVE VIRUS

MANAGEMENT

-DETAILED HISTORY -CAREFUL CLINICAL EVALUATION

-BASIC ASSESSMENT OF CHILD’S VISION-IOP

-FUNDUS EXAMINATION UNDER DILATATION -B-SCAN FOR POSTERIOR SEGMENT

A-SCAN TO MEASURE AXIAL LENGTH OF BOTH THE EYES

TIME OF SURGERYSURGICAL TECHNIQUE

TYPE OF OPTICAL REHABILITATIONPOST-OP MANAGEMENT OF AMBLYOPIA

CONGENITAL CATARACTS:LABORATORY EVALUATIONRESULT POSSIBLE DIAGNOSIS

+ REDUCING SUBSTANCE AMINOACIDURIA HEMATURIA , PROTEINURIA ‘’MALTESE CROSS” FIGURES

GALACTOKINASE DEFICIENCY LOWE’S SYNDROME FABRY’S DISEASE

ERYTHROCYTE ENZYMES GLUCOSE TORCH titres,VDRL test CALCIUM, PHOSPHORUS

GALACTOKINASE DEFICIENCYHYPER/HYPO GLYCEMIARUBELLA.TOXOPLASMOSIS,CMV,HERPES,SYPHILIS,HYPOPARATHYROIDISM

urine

Blood

CONGENITAL CATARACT:DIAGNOSTIC EVALUATION CONDITION LABORATORY TEST

GALACTOSEMIA URINE REDUCING SUBSTANCE

RUBELLA ANTIBODY TITERS

SYPHILIS VDRL TEST

HYPOPARATHYROIDISM SERUM CALCIUM,PHOSPHORUS,ALKALINE PHOSPHATASE

WILSON’S DISEASE SERUM CERULOPLASMIN

HYPERGLYCEMIA/HYPOGLYCEMIA BLOOD GLUCOSE

FABRY’S DISEASE URINE”MALTESE CROSS”(POLARIZED IIGHT)

LOWE’S SYNDROME URINE AMINO ACIDS

TREATMENT IS INDICATED ONLY IF THE VISION IS CONSIDERABLY

IMPAIRED

--MEDICAL--SURGICAL

MEDICAL• IF THE PATIENT HAS SMALL

OPACITIES IN WHOM THE RED REFLEX IS NOT CONSIDERED SIGNIFICANTLY IMPAIRED

• IN SOME PATIENTS WITH SMALL CENTRAL OPACITY{3 MM OR LESS}

• PATCHING• DILATATION WITH TROPICAMIDE

0.5%OR CYCLOPENTOLATE 0.5%

• IF VISION IMPROVES 6/18 THEN NO SURGERY REQUIRED

• WHO REQUIRES CHRONIC CYCLOPLEGIC AGENTS TO MAINTAIN DILATION & IN VISUAL ACUITY HAS IMPROVED –SURGICAL OPTICAL IRIDECTOMY SHOULD BE CONSIDERED.

Classic eg.-peter anomaly –central cataract + corneal opacity but has a clear peripheral lens & cornea optical iridectomy better than corneal transplant & cataract extraction.

SURGICAL

• IF DENSE UNILATERAL OR BILATERAL CRITICAL PERIOD APPEARS TO BE WITHIN THE FIRST 2 MONTHS.• FIRST 6 WKS –PRECORTICAL STAGE 6-8 WKS-CORTICAL STAGE• UNILATERAL CAT.--OPERATED ON BY AGE 6 WKS• BILATERAL CAT.—SLIGHTLY LARGER WINDOW 8--10

WKS

BILATERAL PARTIAL-MAY NOT REQUIRE SURGERY

MONITOR LENS OPACITIES AND VISUAL FUNCTION &

INTERVENE LATER IF VISION DETERIOTES

UNILATERAL DENSE- URGENT Sx FOLLOWED BY

AGGRESSIVE anti AMBLYOPIA therapy

IF DETECTED AFTER 16 WKS OF AGE THEN PROGNOSIS IS

VERY POOR

PARTIAL UNILATERAL-CAN USUALLY BE OBSERVED OR TREATED NON SURGICALLY

WITH PUPILLARY DILATATION AND CONTRALATERAL

OCCLUSION

HISTORICAL LANDMARKS

BEFORE 1960 – MOST CONGENITAL CATARACTS WERE REMOVED BY AN EXTRACAPSULAR TECHNIQUE.

IN 1960- SCHEIE INTRODUCED DISCISSION & ASPIRATION TECHNIQUE

IN 1972-MACHEMAR ET AL DEVELOPED A NEW INSTRUMENT {VISC} VITREOS INFUSION SUCTION CUTTER

CURRENT SURGICAL TECHNIQUE: VITRECTOMY CUTTING INSTRUMENTS, IRRIGATION/ASPIRATION,PHACO OR SOME

COMBINATION OF THIS TECHNIQUE

CURRENT SURGICAL TECHNIQUE• INCISION_ USUALLY THE INCISIONS WE TAKE ARE SELF HEALING BUT IN CHILDREN

THE CORNEAL TISSUE IS LESS LIKELY TO HEAL THUS SUTURE CLOSURE OF TUNNEL WOUNDS RE ADVISED.

• ANTERIOR CAPSULORHEXIS:A 1.4% SODIUM HYLURONATE IS RECOMMENDED FOR PAEDIATRIC SURGERY TO MAINTAIN THE A.C STABILITY ABD INCREASED VITREOUS UPTHRUST.THE ANT. CAPSULOTOMY SHAPE,SIZE AND INTEGRITY ARE IMPORTANT TO LONG TERM CENTRATION OF IOL.{THE FUGO PLASMA BLADE IS A NEW TOOL FOR PERFORMING ANT CAPSULOTOMY IN CHILDREN.

• HYDRODISSECTION:TO ENSURE MAXIMUM REMOVAL OF LENS CORTEX AND LENS EPITHELIAL CELLS, MAY BE A SINGLE OR MULTIPLE SITE ---------PRERFORMED BY INJECTING RL OR BALANCED SALT SOLUTION INN 2 ML DISPOSABLE SYRINGE AVOIDED IN CATARACT WITH POST. LENTICONUS OR POST POLAR CATARACT

CATARACT REMOVAL-LENS MATERIAL MAY BE REMOVED WITH PHACOASPIRATIONOR IRRIGATION AND ASPIRATION.

POSTERIOR CONT.. CURVILINEAR CAPSULOREXHIS{PCCC}: WE PERFORM THIS AT THE AGE LESS THAN 6-8 YEARS & ANY CHILDREN WITH NYSTAGMUS WHERE

FUTURE YAG MAY BE DIFFICULT IT IS DONE TO PREVENT THE PCO AS IT IS AMBLYOGENIC AND THE SURGEON IS

DEFEATED IN ACHIEVING THE TARGET USE OF HIGH VISCOSITY VISCOELASTIC HELPS TO ACHIEVE PCCC.THE

DESIRABLE SIZE OF POST RHEXIS IS 3-3.5 MM.ANT.VITRECTOMY.

IOL LENS IMPLANTATION: CAPSULAR BAG IMPLANTATION IS THE BEST CHOICE AS IOL & UVEAL TISSUE CONTACT IS LESSER& CENTRATION IS ACHIEVED{AIOS

ADVICE IT TO BE DONE BY PAEDIATRIC OPHTHALMOLOGISTS}

IOL SELECTION: PMMA IOLS WERE THE ONLY CHOICE THE SINGLE PIECE HYDROPHOBIC ACRYLIC IOLS ARE IDEAL FOR IMPLANTATION

NOW MULTIFOCAL IOL ARE GAINING GROUNDS AS IT GIVES THE GOOD COMPATIBILITY WITH NEAR AND FAR VISION OF CHILD

LIMITATIONS :IOL POWER PREDICTABILITYVISUAL DEVELOPMENT

INCISION CLOSURE

Birth 34.40-1yr 28.71-2yr 26.42-3yr 23.03-4yr 22.14-5yr 20.95-6yr 19.5

INTRAOCULAR LENSES POWER TO ACHIEVE EMMETROPIA

UNDERCORRECTING BIOMETRY BY 10% IN 2-8 YRS

FOR CHILDREN YOUNGER THAN 2 YRS UNDER CORRECT BY 20%

1 year +6D2 year +5 D3 year +4 D4 year +3 D5 year +2 D6 year +1 D7year PLANO8 year -1 TO -2 D

21MM 22.00D20MM 24.00D19MM 26.00D18MM 27.00D17MM 28.00D

AXIAL LENGTH

POWER

VISUAL REHABILITATION

1. GLASSES {APHAKIC SPECTACLES}

2. CONTACT LENS

3. EPIKERATOPHAKIA

4. INTRAOCULAR LENS

APHAKIC SPECTACLES

ADVANTAGES: THEY CAN EASILY BE UPDATED TO MATCH THE RAPIDLY CHANGING REFRACTIONS IN YOUNG CHILDREN

DISADVANTAGES:LENS THIKNESS & WEIGHT AS WELL AS OPTICAL DISTORTIONS

IN NEW BORNS LENS POWER OF +24 TO +26DWhich can be accomplished with very thick bubble shaped lens in older children the thinner high ensity aphakic specs can be used .

Patching of normal eye is necessary when the child is using aphakic specs

CONTACT LENS

MOST COMMON METHOD FOR BOTH BILATERAL AND UNILATERAL APHAKIA.

ADVANTAGES:OPTICAL QUALITY IS GOOD *SOME CL CAN BE WORN THROUGHOUT 24 HOURS A DAY

DISADVANTAGES--RELATIVELY THICK -CAN BE WASHED OR RUBBED OUT EASILY-TIDIOUS FOR PARENTS-ASSOCIATED WITH CORNEAL COMPLICATIONS AS INFECTIONS & ULCERS

LENS : SILICONE – HIGH O2 PERMEABILITY CHILDREN YOUNGER THAN 6 MONTHS-36 DGas permeable lens can also be used

EPIKERATOPHAKIAIN 1980’S FIRST PERFORMED

BECAUSE OF PROBLEM IN SPECS & C.L’S

PROCEDURE:- REMOVING A CENTRAL HALF THICKNESS OF THE

CORNEA & THEN SUTURING PREDETERMINED CORNEAL DONOR

TISSUE.

• DISADVANTAGES:PERSISTENT HAZINESSESPECIALLY AT THE INTERFACE BETWEEN HOST & THE GRAFT THAT COULD TAKE UP AN YEAR TO CLEAR.

• LATE MYOPIA & ASTIGMATISM IN MANY EYES

THANK YOU