Primary congenital glaucoma is a rare genetic congenital ocular disorder that affects children at birth. It is characterized by abnormally high intraocular pressures. This activity describes the etiology, risk factors, varied presentations, investigations, management guidelines, differential diagnosis, and prognosis for primary congenital glaucoma.

5.  The lens has
- anterior surface
- posterior surface
- meet at the equator which is circular and has a rippled or
undulated appearance.
 The centre of surfaces are the poles
 The anterior pole is about 3mm from back of the cornea.

6.  Lens can be divided into
1.lens capsule
2.anterior lens
epithelium
3.lens fibres
-nucleus
-cortex

8. LENS CAPSULE:
- It is an acellular ,thin, transparent membrane
-composition: type IV collagen
10% glycosaminoglycans
-it varies in its thickness both in its extent and with age
-it is thicker anteriorly than posteriorly and at equator than
at poles
-outer layer gives attachment to zonules and help in
moulding the shape of lens

9. It is a single layer of cuboidal nucleated epithelial
cells deep to anterior capsule.
It is the major site of metabolism in lens
At the equator these cells actively divide and
elongate throughout the life to form new lens
fibres
There is no posterior epithelium as they are used
up in filling the lens vesicle during development

10. Zones of lens epithelium:
1. central zone:
-cuboidal cells which are polygonal on flat
section
-nuclei are round and apical
-they divide in response to variety of
injurious stimuli
-during repair of injury they can pile up to
10 layers under the capsule

11.  They are stable cells in
normal conditions
 Metaplasia into spindle
shaped myofibroblast cells
-Glaucomflecken (acute
congestive glaucoma)
-Atopic dermatitis(sheild
shaped cataract)

12. 2.Intermediate zone:
-comparatively smaller and more
cylindrical cells located peripheral to
the central zone
-nuclei are round and central
-they mitose occasionally
3.Germinative layer:
-columnar cells located in most
peripheral area pre-equatorially
-they actively divide and migrate
posteriorly to form the lens fibres

13. Applied aspects
Dysplasia of cells of germinative layer leads to
posterior subcapsular cataracts
- radiation induced cataracts
-myotonic dystrophies
- NF-2

14. 3.Lens fibres:
-initially posterior epithelium fills the lens vesicle from
posterior to anterior
- later on, the lens fibres are filled by anterior epithelium
-these cells elongate and differentiate to produce long and thin
regularly arranged lens fibres
-the new lens fibres are laid on the older deeper fibres

15. -The superficial fibres are
elongated and nucleated with
the nuclei in relatively
anterior position
-As new fibres are laid down
these anteriorly placed nuclei
form a line convex forward at
the equator known as the LENS
BOW or NUCLEAR BOW

16.  There are interlocking processes between cells (ball &
socket and tongue & groove interdigitations) with zonula
occludens
 The interdigitations are less in superficial zone of lens
which may permit moulding of shape of lens during
accommodation

17.  The fibres forming the
foetal nucleus are
arranged as anterior
upright Y suture and a
posterior inverted Y-
suture
 Later they grow irregularly
in a complicated dendritic
pattern

19. For surgical purposes lens
can be divided into:
A central hard nucleus
An epinuclear plate
(EN)of varying thickness
A layer of cortex
A capsule

20.  Hydrodissection:
Definition:
Seperation of lens nucleus from outer cortex and capsule
Plane of injection of fluid:
In the cortical layers under the lens capsule

21. Hydrodelineation:
Definition:it is the separation of outer epinuclear shell or
multiple shells from central compact endonucleus
Plane of injection:
Into the mass of nucleus

22.  Grading should be done to
set the parameters of
phacoemulsification
machine
 The hardness depending on
the colour can be graded
as:
grade colour
I whitish/g
reyish
II Yellow
III Amber
IV Brown
V black

23.  Suspensory ligaments/ciliary zonules
 Series of fibres from ciliary process
 Holds the lens in position
 Assist action of ciliary muscle
 Attached to lens capsule at zonular
lamella
23

24. - Transparent , stiff and non elastic
- Diameter of 0.35 to 1.0 microns
-Composition:
glycoproteins
mucopolysaccharides
 Applied aspect:
Their susceptibility to hydrolysis by alpha –chymotrypsin has
been used to advantage in intracapsular cataract surgery

25.  Fibres can be divided into
1. Main fibres of ciliary
zonules:
 Orbiculo- posterior capsular
fibres
 Orbiculo- anterior capsular
fibres
 Cilio-posterior capsular
fibres
 Cilio-equatorial fibres
2. Auxillary fibres

26. 2. Auxiliary fibres:
They provide strength to the main fibres by anchoring
the individual portions of zonules
These also help to hold the various portions of the
ciliary body together

27. 1.Main zonular fibres
-pars orbicularis
-zonular plexus
-zonular fork
-zonular limbs
2.Hyaloid zonule
3.Hyalocapsular zonule
4.Cicumferential zonular girdles

28. Hyaloid zonule:
Single layer of zonules binding anterior hyaloid
with pars plana and plicata
Hyalocapsular zonules:
Circular band present where anterior hyaloid
membrane is attached to patellar fossa
Circumferrential girdles:circular band of fibres
-Anterior:ciliary processes to anterior hyaloid
-posterior:pars plana with anterior hyaloid

30.  Natural crystalline lens is 9.6 mm
in diameter
 Zonules extend 2 mm onto anterior
surface of natural lens
 Capsulorrhexis is done with a
maximum diameter of 6-7 mm as it
has to be confined to the zonule
free zone

31. CONGENITAL CATARACT

32. THESE CATARACTS ARE PRESENT AT BIRTH ORTHAT
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 AREUSUALLY
INSIGNIFICANT
-OTHERS CAN PRODUCE PROFOUND VISUAL IMPAIRMENT

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

34. 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

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

36. LAMELLAR: IT IS ALSO KNOWN AS ZONULARCATARACT
-THESE ARE AUTOSOMAL DOMINANT TRAIT
-EFFECT ONVISUAL ACUITYWITHTHE SIZE& DENSITY OFTHE OPACITY
-THESE ARE OPACIFICATIONS OF SPECIFIC LAYERS/ZONES OF THE LENS
-DISC SHAPEDCONFIGURA
TION
-RIDERS-THESE A
R
EH
O
R
S
E S
H
O
E SHAPED OP
ACITIES.

37. POLAR CATARACT-Lens opacity involves
subcapsular,cortex,capsule of anterior or posterior
pole of lens
Aant
Ant polar cataract-it is AD, Small B/L symmetric non
progressive opacities that do not impair vision
Post polar cataract-produsen more visual impairment
because it tends to be larger in size

38. SUTURAL:The sutural or stellate cataract is an opacification of the Y sutures of
the fetal nucleus
-IT DOES NOT IMP
AIR VISION
-THESE OP
ACITIES OFTENHA
VE BRANCHES ORKNOBS PROJECTING FROM
THEM.

39. CORONARY: A.D
GROUP OF CLUB SHAPED CORTICAL OPACITIES THAT
ARE ARRANGED AROUND THE EQUATOR OF LENS LIKEA
CROWN
--THEY CANT BE SEEN UNTILL THE PUPILS AREDILATED
--USUALLY DO NOT AFFECT THE VISUALACUITY

40. CERULEAN:SMALL BLUISH OPACITIES
LOCATED IN THE LENS CORTEX
--HENCE THEY ARE ALSO K/AS BLUEDOT
CATARACT
--NON-PROGRESSIVE
USUALLY DO NOT CAUSE VISUALSYMPTOMS

41. 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.

42. 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

43. 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 RECOVEREDAS
LATE AS 3 YRS AFTER
BIRTH
CATARACT REMOVAL MAY BE
COMPLICATED BY EXCESSIVE POST-
OP INFLAMMATION RELEASE BY
THESE LIVE VIRUS

44. MANAGEMENT

45. -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

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

47. 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,VDRLtest
CALCIUM,
PHOSPHORUS
GALACTOKINASE DEFICIENCY
HYPER/HYPO GLYCEMIA
RUBELLA.TOXOPLASMOSIS,CMV,H
ERPES,SYPHILIS,HYPOPARATHYRO
IDISM
urine
Blood

48. 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

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

50. 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 TOMAINTAIN
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.

51. 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

52. HISTORICAL LANDMARKS

53. 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

54. CURRENT SURGICAL TECHNIQUE
• INCISION_ USUALLY THE INCISIONS WE TAKE ARE SELF HEALING BUT IN
CHILDREN THE CORNEAL TISSUE IS LESS LIKELY TO HEAL THUSSUTURE
CLOSURE OF TUNNEL WOUNDS READVISED.
• 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 PERFORMINGANT 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

55. 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 & ANYCHILDREN WITH NYSTAGMUS WHERE
FUTURE YAG MAY BE DIFFICULT
IT IS DONE TO PREVENT THE PCO AS IT IS AMBLYOGENICAND 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 CHOICEAS
IOL & UVEAL TISSUE CONTACT IS LESSER& CENTRATION IS ACHIEVED{AIOS
ADVICE IT TO BE DONE BY PAEDIATRIC OPHTHALMOLOGISTS}

56. IOL SELECTION: PMMA IOLS WERE THE ONLY CHOICE
THE SINGLE PIECE HYDROPHOBIC ACRYLIC IOLS ARE IDEAL FORIMPLANTATION
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

57. 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

58. 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
AXIAL
LENGTH
POWER
21MM 22.00D
20MM 24.00D
19MM 26.00D
18MM 27.00D
17MM 28.00D

59. VISUALREHABILITATION
1. GLASSES {APHAKIC SPECTACLES}
2. CONTACT LENS
3. EPIKERATOPHAKIA
4. INTRAOCULAR LENS

60. APHAKICSPECTACLES
DISADVANTAGES:LENS THIKNESS & WEIGHT
AS WELL AS OPTICALDISTORTIONS
ADVANTAGES: THEY CAN EASILY BE
UPDATED TO MATCH THE RAPIDLY
CHANGING REFRACTIONS IN YOUNG
CHILDREN
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

61. CONTACT
LENS
MOST COMMON METHOD
FOR BOTH BILATERALAND
UNILATERALAPHAKIA.
ADVANTAGES:OPTICAL QUALITYIS
GOOD *SOME CL CAN BE WORN
THROUGHOUT 24 HOURS ADAY
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

62. EPIKERATOPHAKI
A
IN 1980’S FIRST PERFORMED
BECAUSE OF PROBLEM IN
SPECS & C.L’S
PROCEDURE:- REMOVING ACENTRAL
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

63. THANK YOU