Congenital or infantile cataracts that are present at birth or develop within the first year of life are called congenital cataracts. They occur in about 1 in 2,000 live births. Some lens opacities do not progress and are insignificant, while others can cause profound visual impairment. Congenital cataracts can be unilateral or bilateral. They are generally classified as extensive syndromes in 1/3 of cases, an inherited trait in 1/3, and of undetermined cause in 1/3. Treatment may involve medical management if vision is not significantly impaired, or early surgery within the first 2 months of life if dense cataracts are present to prevent amblyopia. Surgical techniques have

1. CONGENITAL CATARACT
BY: DR NIKITA JAISWAL
PG 1ST YEAR
IMS AND SUM HOSPITAL

2. 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

3. CONGENITAL CATARACT -- UNILATERAL
-- BILATERAL
IN GENERAL THESE CONGENITAL CATARACT
1/3RD EXTENSIVE SYNDROMES
1/3RD INHERITED TRAIT
1/3RD UNDETERMINED CAUSE

4. 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,TOXOPLASM
OSIS
• OCULAR ANOAMLIES-ANIRIDIA
• TOXIC –CORTICOSTEROIDS,RADIATION
• UNILATERAL
• IDIOPATHIC
• OCULAR ANOMALIES
-POST.LENTICONUS
-POST POLE TUMORS
-PERSISTENT FETAL VASCULATURE
• TRAUMATIC
• RUBELLA
• MASKED B/L CATARACT

5. CONGENITAL CATARACT IN A VARIETY OF MORPHOLOGIC
CONFIGURATION
LAMELLAR
POLAR
SUTURAL
CORONARY
CERULEAN
CAPSULAR
COMPLETE & MEMBRANOUS

6. LAMELLAR: IT IS ALSO KNOWN AS ZONULAR CATARACT
-THESE ARE AUTOSOMAL DOMINANT TRAIT
-EFFECTONVISUALACUITYWITHTHE SIZE & DENSITY OFTHE 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.

7. 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/L
SPORADIC-OFTEN UNILATERAL

8. 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.

9. CORONARY: A.D
GROUP 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

10. 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

11. CAPSULAR-THESE CATARACTS ARE SMALL
OPACIFICATIONOF THE LENS EPITHELIUM & ANTERIOR
LENS CAPSULE THAT SPARE THE CORTEX
COMPLETE- 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.

12. SPOKE LIKE FABRY’S DISEASE
VACUOLES MANNOSIDOSIS
DIABETES
MULTICOLOR FLECKS HYPOPARATHYRODISM
MYOTONIC DYSTROPHY
GREEN “SUNFLOWER” WILSON’S DISEASE
THIN DISCIFORM LOWE’S SYNDROME
LAMELLAR GALACTOSEMIA
HYPOGLYCEMIA

13. RUBELLA- CAUSED BY RUBELLA
VIRUS
CAN CAUSE FETAL DAMAGE
ESPECIALLY IF THE INFECTION
OCCURRS IN 1st TRIMESTER OF
PREGNANCY.
PEARLY WHITE
OPACIFICATIONS
ENTIRE 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

14. MANAGEMENT

15. -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

16. TIME OF SURGERY
SURGICAL TECHNIQUE
TYPE OF OPTICAL REHABILITATION
POST-OP MANAGEMENT OF AMBLYOPIA

17. CONGENITAL CATARACTS:LABORATORY EVALUATION
RESULT 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 DEFICIENCY
HYPER/HYPO GLYCEMIA
RUBELLA.TOXOPLASMOSIS,CMV,H
ERPES,SYPHILIS,HYPOPARATHYRO
IDISM
urine
Blood

18. 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

19. TREATMENT IS INDICATED ONLY IF
THE VISION IS CONSIDERABLY
IMPAIRED
--MEDICAL
--SURGICAL

20. 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.

21. 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

22. 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

23. HISTORICAL LANDMARKS

24. 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

25. 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

26. 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}

27. 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 PREDICTABILITY
VISUAL DEVELOPMENT
INCISION CLOSURE

28. Birth 34.4
0-1yr 28.7
1-2yr 26.4
2-3yr 23.0
3-4yr 22.1
4-5yr 20.9
5-6yr 19.5
INTRAOCULAR LENSES POWER TO ACHIEVE
EMMETROPIA

29. UNDERCORRECTING BIOMETRY BY 10% IN 2-8
YRS
FOR CHILDREN YOUNGER THAN 2 YRS UNDER
CORRECT BY 20%
1 year +6D
2 year +5 D
3 year +4 D
4 year +3 D
5 year +2 D
6 year +1 D
7year PLANO
8 year -1 TO -2 D
21MM 22.00D
20MM 24.00D
19MM 26.00D
18MM 27.00D
17MM 28.00D
AXIAL
LENGTH
POWER

30. VISUAL REHABILITATION
1. GLASSES {APHAKIC SPECTACLES}
2. CONTACT LENS
3. EPIKERATOPHAKIA
4. INTRAOCULAR LENS

31. 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
+26D
Which 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

32. 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 D
Gas permeable lens can also be used

33. EPIKERATOPHAKI
A
IN 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

34. THANK YOU