congenital glaucoma and congenital cataract - Dr. Regina Lalramhluni

1. CONGENITAL GLAUCOMA
&
CONGENITAL CATARACT
DR REGINA LALRAMHLUNI
II YR RSO
UPGRADED DEPARTMENT OF OPHTHALMOLGY
MYH & MGMMC, INDORE

2. CONGENI TAL GLAUCOMA
INTRODUCTION
Occurs due to developmental
defect in the trabecular meshwork
& anterior chamber angle.
Noted by hippocrates
Recognsied by Von Muralt in
1869

3. Epidemiology
1 in 10,000 births
b/l 65-80%
M:F = 3:2
25% diagnosed as newborn, 60% by 6 months, 80% by 1
year

4. Genetics
Most are sporadic
10% familial
Maybe autosomal dominant, autosomal recessive or
multifactorial inheritance
three major loci (GLC3A, GLC3B, GLC3C) identified on
chromosome 2, 1, 14 respectively

5. TERMINOLOGY
Relating to age of onset:
1. Pediatric glaucoma: is a broad term referring to any form of
glaucoma that may occur from birth to 18 years of age.
2. Congenital glaucoma: the glaucoma exists at birth and usually before
birth..
3. Infantileglaucoma: occurs from birth until 3 years of life
4. Juvenileglaucoma: occurs after the age of 3 to teenage years
5. developmental glaucoma: if there is associated anomalies, either
ocular or systemic

6. Relating to structural maldevelopment
1. Goniodysgenesis: maldevelopment of irido-corneal angle.
2. Trabeculodysgenesis: maldevelopment of trabecular
meshwork.
3. Iridodysgenesis: maldevelopment of iris
4. Corneodysgenesis: maldevelopment of cornea

7. Figure1: The normal
chamber angle: on the left
is a histological cross-
section; on the right is a
drawing of the same
Figure 2: An underdeveloped
chamber angle

8. PATHOGENESIS
- Despite general agreement that abnormal development of
the anterior chamber angle obstructs aqueous outflow
(isolated trabeculodysgenesis), the exact nature of this
abnormality has yet to be understood .
- Barkan initially postulated an impervious membrane over
the trabecular meshwork k/as barkans membrane but it
was disproved histopathologically.

9. Maumenee demonstrated developmental arrest of the iris
and ciliary muscle(longitudinal and circular fibers) in the
seventh month of gestation causes the insertion of the
iris and ciliary body in an anterior location,over-lapping
the trabecular meshwork.
Anderson provided histopathological support for the high
insertion of the iris into TM
The trabecular meshwork has also been noted to have
thickened trabecular beams and uveal cords,with
narrowed trabecular spaces lead to impaired trabecular
outflow.

10. Shaffers-weiss classification

11. Hoskins classification

12. Causes of visual loss
- Corneal scarring / irregular astigmatism
- Cataracts
- Optic nerve damage
- Anisometropic amblyopia
- Strabismic amblyopia
- Retinal pigmentary changes,detachment (possible
complication after filtration ,seton,or cycloablative
surgery)

13. Clinical features
Symptoms:-
A clinical triad of
photophobia , epiphora , and
blepharospasm is commonly
associated with the
presentation of primary
congenital glaucoma
Others- cloudy cornea,
enlarged cornea or eye,
irritability, red eye, poor vison,
pain.

14. -Tear in the descemet’s membrane
(Habb’s striae result from new
basement membrane laid down by
the endo. cells) ,which are single or
multiple, oriented horizontally or
concentric to the limbus associated
with corneal edema in the early
phases& have a significantly lower
endothelial count.
- In severe cases Acute hydrops
may occur.
signs

15. -Corneal enlargement is a very specific sign of
PCG (Till 1st
3 yrs)
sclera also expands slowly(till ten yrs) under the
influence of increased intraocular pressure and
thinning brings about increased visibility of the
underlying uveal tissue in neonates and causes the
blue sclera

16. - In advanced cases ,the zonules may become stretched
and rarely the lens may subluxate .
- Enlargement of the eye occurs under the influence of
elevated intraocular pressure with major enlargement
occurring at the corneo-scleral junction .
- As the axial length of the globe increases ,myopia and
astigmatism occur.
- Cupping of the optic nerve proceeds more rapid and
more likely to be reversible if IOP is normalized . The
younger the child, the faster this reversibility

17. - symmetrical cupping has been attributed to stretching of
the optic canal and backward bowing of lamina cribrosa .
- In eyes of young glaucoma patients there is often
generalized enlargement of the optic cup with
preservation of an intact neuroretinal rim.
- Hyphema,RD and Pthisis bulbi are often the final outcome
of untreated or refractory glaucoma.

18. Management of Congenital Glaucoma
InitialEvaluation
Office examination – Following can be performed in
children more than 5 year of age-
• Slit lamp examination
• Applanation tonometery
• Gonioscopy
• Optic nerve evaluation
• Retinoscopy
• Kinetic Goldman visual field testing(with the assistance
of patient and encouraging perimeterist)

19. Examinationunderanaesthesia
• General anaesthesia is usually required for thorough
examination of children under the age of 5 years.
• Its sequential components consist of :
-IOP measurement
-Cornea assessment
-Gonioscopy
-Ophthalmoscopy
-Additionally axial length
measurement, UBM or cycloplegic Retinoscopy

20. IOP and tonometery
• Most reliable IOP are obtained under intramuscular ketamine.
• Perkins tonometer and Tonopen are reliably used to measure IOP in
children
• Infant and young children appear to have IOP lower than those
expected in adults
• Mean IOP of 9.59 mm of Hg is found in the newborn which rises to
13.95 by 7 or 8 year of age
• Infant with primary congenital glaucoma may present with IOP
exceeding 30 –40 mm of Hg if unanesthetised, but may be much
lower under the influence of inhalational anesthesia

21. Corneal diametermeasurement
•An effective measurement of the corneal diameter can be
obtained using calipers to measure the horizontal diameter
from the first appearance of the white scleral fiber at the
limbus on one side to the same point on the other side, from
the 9 o’clock to 3 o’clock position
•Measuring the horizontal corneal diameter is a fundamental
part of childhood glaucoma assessment
•A horizontal corneal diameter of 12 mm in the first year of life
associated with corneal oedema is pathognomic of glaucoma

22. Gonioscopy
Ideally a smooth domed Koeppe 14-16 mm lens with a Barkan light
and hand- held binocular microscope is utilized for the purpose
A Goldmann goniolens is also used for viewing the angle through
the operating microscope
If marked corneal clouding exists the view may be improved by
using topical anhydrous glycerin or70% alcohol or 10% cocaine on a
cotton tipped applicator
If necessary, edematous epithelium may also be removed using a
surgical blade

23. Gonioscopic anatomyof normalinfant eye
- Iris inserts posterior to the scleral
spur
- Flat iris insertion due to poor
development of the angle recess till 6
to 12 months
- Ciliary body band is distinct in most
cases
- TM appears thicker and more
translucent than in adults

24. Gonioscopyof theeyes ininfant withprimarycongenital
glaucomareveals –
- Anterior insertion of the iris directly into the trabecular
meshwork
- Stippled trabecular meshwork surface
- trabecular meshwork appear thicker than normal
- Peripheral iris shows thinning of the anterior stroma
- Angle is usually devoid of vessels although root of vessels
from the major arterial circle is seen above the iris surface
and has been referred to as Loch Ness Monster
phenomenon
- The peripheral iris inserting into the trabecular meshwork
may appear translucent and is referred to as the Lister’s
morning mist

25. Ophthalmoscopy
Optic nerve cupping occurs rapidly in
infant with elevated intraocular
pressure and unlike in adult eyes, is
also rapidly reversible with
normalization of intraocular pressure
Persistent IOP elevation, however,
causes glaucomatous optic atrophy
due to loss of ganglion cells
C/D ratio greater than 0.3 are rare in
healthy infant and should cause
suspicion of glaucoma

26. Structuraldefect
• Isolated trabeculodysgenesis are highly responsive to
both goniotomy and trabeculotomy ab externo
• In iridotrabeculodysgenesis success rate for goniotomy
and trabeculotomy decreases, multiple surgeries
needed. Trabeculotomy is initial better procedure of
choice
• In iridocorneotrabeculodysgenesis prognosis for surgical
treatment is poor. Ab externo combined trabeculotomy
and trabeculectomy may be useful as initial procedure

27. Medical therapy
B- blocker
Timolol : is the most widely used beta blocker in children
It has been reported that plasma timolol level; after treatment with
0.25%timolol in children far exceeds adult plasma level, treated with
0.5%timolol
Reduction in heart rate, exacerbation of asthma and apnea has been
reported in 4-13%of children treated
Avoided in neonates and the premature due to risk of sleep apnea
When indicated, timolol gel forming solution preferred due to lesser
systemic absorption

28. Carbonic anhydraseinhibitors
- Temporary measure to reduce IOP and corneal edema prior to
surgery
- Doses 5-10 mg/kg/day in divided dose
- Serious side effects include growth suppression, metabolic acidosis,
drug idiosyncrasy, bone marrow suppression. Thus prolonged
therapy is avoided
- Currently topical dorzolamide is preferred and administered 2-3 times
daily
- Combination therapy of timolol and dorzolamide may be used in older
children with no contraindication

29. Alphareceptoragonists
Not recommended in children under 18 years
Cross immature blood brain barrier and causes adverse
CNS effect -slight drowsiness, respiratory depression,
failure of recovery from anaesthesia and death of
premature infant
Prostaglandinanalogues
- effective but may cause ocular hypotension

30. Cholinergic drug(Pilocarpine)
No role in congenital glaucoma
May be useful in children with glaucoma in aphakia and
pseudophakia with open iridocorneal angle

31. Surgical treatment
Goniotomy
Procedure of choice in eyes with
congenital glaucoma with corneal edema
and minimum ocular and corneal
enlargement
Initially practiced by Barkan
Aims to remove the obstructing tissue in
the angle causing resistance to aqueous
flow
Prerequisites – General anaesthesia,
operating microscope, contact lens (e.g..
Barkan lens), tapered goniotomy blade

32. Procedure:-
Preoperative pilocarpine instillation
help to open the angle
Inner portion of the nasal trabecular
meshwork over 90-120 degree is
incised
Mild hyphaema on withdrawal of
knife indicate correctly placed
incision

33. Mechanismof action
-Relieves the compressive traction of anterior uvea on the
meshwork
-Eliminate any resistance imposed by incompletely
developed inner meshwork
Advantages
Less traumatic
Safe
Rapid
Can be repeated
Spare the conjunctiva for possible later surgery

34. Disadvantages
- Procedure not possible if media hazy
- Require special instrument
- Need experienced surgeon
- Possibility of corneal endothelial, angle and lens trauma
- Moorefield experience showed 20% relapse rate over a 30 year
period with no peak age of relapse,life long follow up necessary
- Best prognosis for infant presenting between 2-8 months of age
- Worst prognosis with elevated pressure and cloudy cornea at birth

35. Trabeculotomy
Procedure of choice; when cornea is opaque
Ab externo procedure
Identify schlemm canal by external approach
As favorable as initial goniotomy procedure

36. Procedure
Limbal or fornix based conjunctival flap is
made
scleral flap is fashioned and schlemm’s
canal is located by slowly deepening a 2mm
radial incision placed at the corneoscleral
junction
Junction of the blue white sclera mark the
location of scleral spur; schlemm’s canal is a
mm anterior to the scleral spur
Trabeculotome is gently threaded into the
canal and swept into anterior chamber,
rupturing the internal wall of schelmm canal
and trabecular meshwork
It directly exposes it to aqueous humour

37. Procedure repeated to the other side of canal
Scleral flap is tightly closed
Accurate localization of the schlemm canal is the most important step
Mild to moderate hyphaema confirm accurate identification of the
schlemm canal
Appearance of aqueous is also evidence of entry into the schlemm
canal

38. Advantages
Can be performed in opaque cornea
Higher success rate when combined with trabeculectomy
Disadvantages
Difficult visualization of angle structure; sometimes leading to serious
complication
Potential complications include DM stripping, iris prolapse, iridodialysis,
cyclodialysis with persistent hypotony, false passages, lens
subluxation, flat anterior chamber
Also damages conjunctiva decreasing success of further filtering
surgery

39. Trabeculectomy
indications:
- visual potential, unscarred conjunctiva, faithful follow up
- unlikely to respond to angle surgery
- very low target pressure required
- secondary glaucoma

40. Combinedtrabeculotomy+trabeculectomy
indications:
- Failure to cannulate Schlemm's canal
- Failed previous angle surgery (<=2 gonio or
trabeculectomy)
- Primary procedure
Procedure:
- Trabeculotomy creates a direct continuity between AC &
Schlemm's canal & trabeculectomy helps aqueous
humor bypass Schlemm's canal to be drained out of AC
to maintain normal IOP
- Superior in controlling IOP

41. MANAGEMENT OF REFRACTORY PEDIATRIC GLAUCOMAS
- filtration surgery with anti-fibrotic drugs
- glaucoma drainage implants
- cyclodestructive procedure

42. Role of anti-metabolites:
 success rate
Mitomycin C commonly used
Applied to area of bleb beneath conjunctiva
Thorough wash before entering AC

43. Drainageshunts
Indications:
- Failed trabeculectomy
- High risk of complication with filtration surgery( Sturge weber
syndrome)
- Scarring ( after multiple conjunctival surgeries)
non restrictive flow restrictive
-molteno implant - Ahmed valve
-Baerveldt implant - Krupin valve

44. Cyclodestructiveprocedures:
- cyclophotocoagulation( transscleral Nd:YAG , transscleral
diode, endoscopic diode)
- cyclocryotherapy
Success rate : 30%
1800
treated first to decrease risk of hypotony

45. Long term follow up and prognosis
Degree of relief from photophobia, tearing and
blepharospasm usually reflect the effectiveness of
surgery
Patient should be followed up between 3 and 6 months of
surgery
Should be examined periodically and for indefinite time

46. CONGENITAL
CATARACT

47. DEFINITION
CONGENITAL CATARACT:-
Disturbance in the normal growth
of the lens before birth
DEVELOPMENTAL CATARACT:-
Occur from infancy to
adolescence
Infantile-present at 1 year
Juvenile- developed later

48. Important facts
• 33% - idiopathic - may be unilateral or bilateral
• 33% - inherited - usually bilateral
• 33% - associated with systemic disease - usually bilateral
• Other ocular anomalies present in 50%

49. ETIOLOGY
1. Heredity: usually dominant. about 1/3
2. Maternal factors:
-malnutrition
-infections
-drugs ingestions
-radiation
3. Foetal or infantile factors
-deficient oxygenation
-metabolic disorders
-ass. with other congenital anomalies(lowes syndrome
myotonic dystrophica, congenital ichtyosis)
-birth trauma
-malnutrition
4. idiopathic

50. CLINICAL TYPES
- involves central part of anterior
capsule and adjoining superficial
most cortex
- due to delayed development of
anterior chamber
- due to corneal perforation
ANTERIOR POLAR CATARACT

51. 1. thickened white plaqued
2. Anterior pyramidal cataract: capsular opacity
is cone shaped with its apex towards cornea
3. Reduplicated cataract(double cataract):
along with central point of anterior capsule,
lens fibre lying immediately beneath is opaque
and subsquently separted from capsule, laying
transparent fibre in between

52. POSTERIOR POLAR CATARACT
Maybe associated with
- persistent hyaloid artery
remnants( Mittendorf dot)
- posterior lenticonus
- persistent hyperplastic primary
vitreous

53. NUCLEAR CATARACT
1. cataracta centralis pulverulenta
-invlolves embryonic nucleus
-small rounded opacity lying in the
centre of lens
-has a powdery appearance
2. total nuclear cataract
- involves embryonic and fetal
nucleus and somtimes infantile
- chalky white central opacity

54. LAMELLAR OR ZONULAR CATARACT
-Occurs in the zone of foetal
nucleus
- The main mass of the lens
internal and external to the zone
of cataract is clear, except for
small linear opacities like spokes
of wheel (riders)

55. SUTURAL AND AXIAL CATARACTS
- Series of punctate opacities scaterred
around the anterior and posterior Y
sutures present in the foetal nucleus
1. Floriform cataract- arranged like
petals of flowers
2. Coralliforem cataract - arranged in
the form of coral
3. Spear shaped cataract- in the form
of scattered heaps of shining crystalline
needles
4. anterior axial embryonic cataract- as
fine dots near the anterior Y suture

56. BLUE DOT CORTICAL CATARACT
- cataracta punctata caerulea
- as rounded bluish dots situated
in the peripheral part of
adolescenct nucleus and deeper
layer of cortex.

57. CORONARY CATARACT
- involves either adolescent
nucleus or deeper layer of cortex
- regular radial distribution in the
periphery of lens encircling the
central axis.

58. MEMBRANOUS CATARACT
- total or partial absorption of
cataract leaving behind thin
membranous cataract

59. CONGENITAL CAPSULAR CATARACT
1. Anterior capsular cataract- nonaxial, stationary and
visually significant
2. posterior capsualr cataract- rare and can be ass with
persistent hyaloid artery remnants.

60. DIAGNOSIS
-Leukocoria
-Strabismus
-Nystagmus
-Photophobia
O THER CAUSES O F LEUKO CO RIA
-Retinoblastoma
-Toxocariasis
-Coats disease
-ROP
-PHPV
-Retinal detachment
-Coloboma
-Retinal dysplasia
-Norrie's disease

61. EXAMINATION PROTOCOL IN PAEDIATRIC CATARACTS
HISTORY:
1. Duration
2. F/H of congenital cataract
3. Visual status
4. Behavioural Pattern and School Performance
BIRTH HISTORY:
1. H/O of maternal infection in 1st trimester
2. Gestational age and birth weight
3. Birth Trauma
4. Supplemental oxygen therapy in perinatal period
5. Developmental Milestones

62. OCULAR EXAMINATION:
1. Visual acuity and fixation pattern
2. Refraction
3. Cover-uncover test (Hirschberg's)
4. Note nystagmus if any
5. Slit lamp examination
-associated congenital anomalies of iris,lens
-type of cataract
-iridodonesis/phacodonesis
6. Tension applanation if possible
7. Fundus examination if possible
8. BScan USG if there is no fundus view

63. LABORATORY INVESTIGATION
- intrauterine infection viz toxoplasmosis, rubella,
cytomegalovirus and herpes virus by TORCH test
- galactosemia by urine test, for reducing substance, red
blood cell transferase and galactokinase levels
- Lowe's syndrome by urine chromatography for amino
acids
- Hyperglycemia by blood sugar
- Hypocalcemia by serum calcium and phosphate levels
and x-ray skull.

64. IMPORTANT POINTS REGARDING MORPHOLOGY
Visually significant: nuclear, lamellar, posterior, total,
membranous
Progressive: posterior lenticonus, PHPV, lamellar,
subcapsular
Most common: lamellar
Best visual prognosis: anterior, sutural, posterior lenticonus

65. DECISION FOR SURGERY IS DEPENDENT IN THESE
FACTORS
Morphology and location of cataract
Size and density of the opacity
Laterality
Visual behavior of the infant
Presence of associated ocular abnormalities

66. MORPHOLOGY AND LOCATION OF CATARACT
-The more central and the more posterior the location of
the opacity, the more visualy significant the cataract will
be
- Nuclear cataracts degrade vision more than lamellar
cataracts

67. SIZE AND DENSITY OF CATARACT
- More than 3mm dense central opacity is significant and
need surgery
- In incomplete bilateral cataracts, density is more
important than the size of opacity
- If major retinal vessels can not be seen through the
cataract surgery is indicated
- Semi-transparent opacity should be treated conservatively

68. LATERALITY
- If a child with unilateral or bilateral cataract develops
strabismus, surgery must be done as soon as possible.
- In partial unilateral cataract, pupillary dilatation combines
with amblopic therapy is an alternative for surgery.
- If a child with bilateral cataract develops nystagmus,
surgery is indicated, although visual prognosis is
generally poor.

69. VISUAL BEHAVIOR
- Ability to fixate the light or follow motions
- Visual attention
- Pupillary reflex: RAPD is poor prognostic sign
- Ability to pick up small objects

70. TIMING OF SURGERY
- Dense cataract, surgery must be done before age of 6 weeks in
unilateral cases
- Dense cataract, surgery must be done before age of 10 week in
bilateral cases
- Interval between surgery of the two eyes should be minimised
- Surgerybefore 4 weeks of life will increase risk of glaucoma and
pupillary membrane
- Some authors advocate surgery on both eyes simultaenously in
selected cases

71. MANAGEMENT
-Mangement of both anterior and posterior capsules had
markedly improved visual results.
-CCC technique provides additional safety and facilities in-
the-bag fixation
-Posterior capsulotomy with anterior vitrectomy or posterior
CCC is helpful in decreasing the incidence of PCO in the
central visual axis

72. SURGICAL TECHNIQUE
WoundConstruction:
- a self-sealing corneal tunnel incision with a relatively long internal
entry preferable.
- this technique helps to decrease iris prolapse.
- most children rub their eyes postoperatively; therefore it is prudent to
use a suture in SICS.
Increasedintravitreal pressure:
- high intravitreal pressure may be produced as a result of scleral
collapse due to the the typical low scleral rigidity in children.
- intravitreal pressure is more significantly elevated in eyes with large
incision than small incision

73. Anteriorcapsulotomy:
- to achieve a smooth continuous capsular tear is to perform a skilled
manual CCC
- one alternative to the manual CCC is the creation of a vitrector
mediated anterior capsulotomy.
Removal of lens substance:
- Hydrodissection helps to ensure max removal of lens cortex and cells
from the equatorial region.
- It is highly recommended in paediatric cataract to aspirate the lens
matter with the use of two port irrigation and aspiration to remove
cortex completely.

74. Posteriorcapsulemanagement:
- Since the intact post. capsule opacifies
rapidly in children, post capsulorrhexis is
preferred by most surgeons.
- post. capsule is thinner and inelastic than
ant. capsule capsulorrhexis smaller than
ant. CCC is done.
Anteriorvitrectomy:
- to reduce PCO post CCC with ant.
vitrectomy is preferred.
- However, one should recognise the
possibility of an increased risk of RD or
CME

75. IOLimplantation:
- IOL should be placed in the bag rather than the ciliary
sulcus to prevent pupillary capture and IOL decentration.
- Foldable IOL are now being used for paediatric eyes
because of reduced incidence of PCO and ability to be
inserted through a small incision.

76. GUIDELINES FOR CHOICE OF IOL POWER
Childre n le ss than 2 ye ars o ld:
do biometry and undercorrect by 20% 0r
use axial length measurements only
if axial length 17mm - 25D
18mm - 24D
19mm - 23D
20mm - 21D
21mm - 19D
Childre n be twe e n 2 and 8 ye ars o ld
do biometry and undercorrect by 10%

77. COMPLICATIONS
The propensity for post operative non specific inflammation
is the reason that the risk of postoperative complication
is higher in children
- post. capsule opacification
- uveitis
- pupillary capture
- glaucoma

78. CORRECTION OF APHAKIA
IOLImplantation:
- it is perfectly safe and acceptable in to perform primary implantation in child
older than 1 year
- in children younger than 1year IOL Implantation is controversial
- a foldable IOL is the most biocompatible IOL as of today
Contact lens:
- If IOl is not implanted, contact lenses are given as early as possible to prevent
stimulus derprivation amblopia
- overcorrection of +2 to +3D is mandatory.
- Silicon lenses or soft hydrogels are well tolerated
Spectacles:
- in bilateral cataract, spectacles are better tolerated
- a secondary strabismus may be manipulated by prismatic effect of spectacles.
- bifocal should be prescribed when the child starts school.

79. POST SURGICAL TREATMENT
-Evaluation of fixation behaviour
-Refraction in each visit
-Periodically IOP measurement under GA
-In unilateral cataract, occlusion of the fellow eye 50 to 70%
of working hours

80. thank you