The document discusses congenital glaucoma, defining it as glaucoma present at birth associated with developmental anomalies of the eye. It covers the epidemiology, genetics, classification, pathogenesis, clinical features, evaluation, and management of congenital glaucoma. The main treatment involves surgical options like goniotomy, trabeculotomy, and trabeculectomy to lower intraocular pressure and clear the cornea.

1. CONGENITAL GLAUCOMA
DR SIVATEJA CHALLA

2. Definition
Terminology
Epidemiology
Genetics
Dev of angle
Classification
Pathogenesis
Clinical features
Evaluation
Management
Prognosis

3. DEFINITION
Glaucoma associated with developmental anomalies of eye present
at birth
Includes
1. Isolated congenital glaucoma
2. Glaucoma's asso with development anomalies either systemic or ocular

4. WHAT TO LABEL???
A. Isolated congenital glaucoma
B. Infantile glaucoma
C. Juvenile glaucoma

5. Isolated congenital glaucoma
1. Isolated mal development of the trabecular meshwork
2. No other developmental ocular anomalies or ocular diseases that can raise IOP.
3. Recognized with in first month
Infantile glaucoma
1. Synonymous with congenital glaucoma.
2. 1 month to 3 years
Juvenile glaucoma
1. Primary glaucoma occurring later in child hood or early adulthood
2. 3 years to 35 years

6. Three years of age is generally taken as the division between infantile and
juvenile glaucoma.
After this age the eye no longer expands in response to raised intraocular
pressure.
 Juvenile glaucoma doesn’t have corneal enlargement or Haab striae

7. EPIDEMIOLOGY
1. 1 in 10000 births
2. 50% isolated congenital
3. 60% diagnosed by 6 months and 80% by 1 year
4. 65% affected are males
5. 70% bilateral involvement

8. GENETICS
ISOLATED CONGENITAL GLAUCOMAS
1. Most are sporadic
2. 10% familial, usually AR pattern with incomplete or variable penetrance
3. Genes  CYP1B1, LTBP2
JUVENILE OPEN-ANGLE GLAUCOMA
1. Autosomal dominant inheritance
2. Genes TIGR and MYOC

9. DEVELOPMENT OF ANGLE
1. At 5 months gestation , continuous layer of endothelium (e) creates a closed cavity of the
anterior chamber and anterior surface of the iris inserts in front of the primordial TM
endothelium ( e )
Trab meshwork

10. 3rd trimester
1. Endothelial progressively disappears from the pupillary area and iris
2. Cavitates over the angle and gets incorporated in the TM
3. The peripheral uveal tissue begins to slide posteriorly to expose TM.
At birth
1. In newborn the iris and ciliary body have recessed to the
level of scleral spur.
2. Posterior sliding of the uveal tissue continues during the first
6-12 months of life thus forming the adult angle
configuration.

11. Anterior chamber angle in Congenital Glaucoma
1. Eyes with isolated congenital glaucoma have the appearance of an eye in the 7th or 8th month of gestation
rather than one at full term.
2. Iris and ciliary body have failed to recede posteriorly, and thus the iris insertion and anterior ciliary body
overlap the posterior portion of TM.
Anderson et al: Trans Am Ophthalmol Soc 79 : 458 1981

12. CLASSIFICATION
1. Shaffer-Weiss classification of congenital glaucoma.
2. Hoskins’s anatomic classification of Developmental glaucoma.
a) clinically identifiable anatomic defects of the eye were chosen as the basis.
b) identification of the anatomic defect helps in determining appropriate therapy and assessing
prognostic factors.

13. Shaffer-Weiss classification
1.Isolated congenital glaucoma (infantile glaucoma) 2.Glaucomas associated with congenital anomalies
1. Aniridia
2. Sturge-Weber syndrome
3. Neurofibromatosis
4. Marfan syndrome
5. Pierre Robin syndrome
6. Homocystinuria
7. Goniodysgenesis
8. Lowe’s syndrome
9. Microcornea
10. Microspherophakia , PHPV
3.Acquired glaucomas in infants
1. Retrolental fibroplasia
2. Tumors- retinoblastoma, juvenile xanthogranuloma
3. Inflammation
4. Trauma

14. Hoskin’s Anatomic classification
A. Isolated trabeculo dysgenesis
B. Iridotrabeculo dysgenesis
C. Corneotrabeculo dysgenesis

15. Isolated Trabeculo-dysgenesis
• Most common defect, occurs in two ways
Concave iris insertion
1. Plane of Iris insertion normal
2. Anterior iris stroma sweeps over TM
ending short of schwalbe’s line-
probably confused as Barkan’s
membrane?
Flat iris insertion
1. Iris inserts ant to scleral spur /on TM.
2. Obscures view of ciliary body.
3. Surface of TM - stippled / orange peel
appearance.
4. Peripheral anterior iris stroma –thinned.
Central stroma appears normal

17. Flat iris insertion
Concave iris
insertion

18. Irido-trabeculo-dysgenesis
Trabeculodysgenesis with iris maldevelopment
Anterior stromal defects
1. Hypoplasia
2. Hyperplasia
Anomalous iris vessels
1. Persistence of tunica vasculosa lentis
2. Anomalous superficial vessels
Structural anomalies
1. Holes
2. Colobomata
3. Aniridia

19. Corneo-trabeculo-dysgenesis
Corneal defects with trabecular dysgenesis
1.Peripheral lesions (upto 2mm into clear cornea):
Posterior Embryotoxon (Axenfeld’s anomaly)
2.Mid peripheral lesions:
Rieger’s anomaly (Sector defects….opacities with adherent iris)
3.Central defects:
Peter’s anomaly (opacification + central stromal thinning + Zone of clear cornea between opacity and limbus)

20. PATHOGENESIS
Barkan (1955)  imperforate membrane, barkan's membrane
 no such membrane on histology or electron microscopy
Allen & colleugues  faulty cleavage of mesodermal tissue.
Maumeene theory  abnormal iris insertion causes obstruction
Andersons theory  excessive collagen matrix deposition in T.M,
 prevents CB & IRIS to sliding back
Kaiser theory  faulty migration or differentiation of neural crest cells

21. CLINICAL FEATURES
Classic Triad ……symptoms
1. Epiphora
2. Photophobia
3. Blepharospasm
Other symptoms are
cloudy cornea , red eye, enlarged cornea or eye , irritability , poor vision and pain
In older children, astigmatism, progressive axial myopia cause symptomatic decreased VA and
refractive amblyopia.

22. Excessive blinking
+/- watering
Hazy cornea
Large corneas

23. SIGNS
IOP
Corneal oedema Haabs striae

24. 1. Breaks in Descmet’s membrane –as
cornea stretches- acute localized edema
& deposition of new basement
membrane into hyaline ridges.
2. Horizontal, curvilinear and parallel to
the limbus in the peripheral cornea.
Haab’s striae

25. SIGNS
IOP
buphthalmos Sclera thin
and blue

26. SIGNS
IOP
Flat and
subluxated lens iridodonesis

27. 1. Anterior chamber becomes deep
2. Optic nerve cupping
◦ Develop early and rapidly
◦ Reversal of cupping with IOP reduction

28. EVALUATION
Examination in clinic
Observe
1. Cornea Size and Clarity
2. degree of tearing/photophobia
3. Any discharge /swelling –sac area
4. Iris/pupil Hypoplasia/corectopia and Aniridia

29. EVALUATION UNDER GA
1. Tonometry ( After Induction And Before Intubation )
2. External Examination
3. Anterior Segment Examination
4. Corneal Diameter Measurement
5. Gonioscopy
6. Fundus Examination
7. CCT
8. Ultrasound (Axial Length Measurement / B Scan )
9. Optic Nerve Photography
10. Refraction

30. TONOMETRY
1. IOP should be measured using Perkin’s hand-held applanation tonometer or
tonopen
2. Most anesthetic agents reduce IOP
3. Should be measured early during earliest possible moments after anesthesia
induction, before intubation.
4. Asymmetric IOP more suggestive of abnormality than B/L borderline IOP

32. Chloral hydrate is the agent doesn’t have any effect on IOP,so best…..

33. EXTERNAL EXAMINATION
1. Exclude other causes of epiphora, photophobia and blepharospasm
2. Most common cause of epiphora in newborn and infant – congenital NLDO
3. Others ; conjucntivitis (infectious, chemical)
corneal epithelial defect/ abrasion
keratitis
inflammed ant segmant (uveitis, trauma)

34. CORNEAL DIAMETER
Age Normal suspicious
Birth – 6m 9.5 to 11.5 mm > 12
1 – 2 years 10 to 12 mm > 12.5
> 2 years < 12 mm > 13
Markowitz et al Can J Ophthalmol :20; 96 1985

35. Horizontal diameter from white to white……..
The vertical diameter is fallaciously lowered by
encroachment of sclera at the superior limbus

36. Also Document
Corneal edema/ clouding, breaks in Descemet’s membrane (Haab’s striae)
Other causes of corneal clouding
Sclerocornea
Tears in D membrane
Ulcers
Metabolic disease
Peters anomaly
Endothelial dystrophies
Dystrophy e.g congenital hereditary stromal dystrophy

37. GONIOSCOPY
Koeppe (14-16mm) lens provides surgeon with appropriate view of the angle.
In normal newborn
1. Iris inserts posterior to scleral spur.
2. TM appears more translucent than that of adult.
In primary congenital glaucoma
1. Anterior insertion of iris (flat or concave) directly into TM.
2. Surface of TM has stippled appearance and meshwork may appear thicker
3. Occasionally loops of blood vessels from major arterial circle may be seen
(LOCH NESS monster phenomenon)

38. FUNDOSCOPY
1. C:D ratio >0.3 and asymmetry are suggestive evidence of glaucoma.
2. The infant glaucomatous cup is more often round , steep walled central &
surrounded by uniform pink NRR. Tends to enlarge circumferentially with
progression of glaucoma.
3. Cupping is reversible

39. MANAGEMENT
I. Parent counselling.
II. A complete general physical examination by a pediatrician
III. Treatment

40. Parent counselling
1. What is glaucoma ?
2. Need for surgery , multiple surgery.
3. Need for life time follow up
4. Combination of problems
◦ IOP
◦ Amblyopia management
◦ Refractive correction
◦ Keratoplasty
5. Counsel regarding choice of profession

41. Treatment
MEDICAL
Congenital glaucoma is a surgical disease.
Indications
1. reduce IOP temporarily
2. clear cornea to facilitate goniotomy
3. achieve post op IOP control till aquequacy of surgical procedure
4. Cases where surgery poses high risk

42. DRUGS
 Topical beta blockers 1st line
S/E bronchospasm, bradycardia, hypotension
 2nd line are topical CAI
 Others  Miotics, PGA, Alpha agonists
 The a2-adrenergic agonist brimonidine crosses the blood-brain barrier
and therefore has significant effects on the central nervous system
apnea, hypotension, bradycardia, hypotonia, hypothermia, and somnolence
 C/I in children younger than 3 years.

43. SURGICAL
Multiple surgical interventions are often required.
Goal of surgery
◦ Normalize and permanently control IOP off drugs
◦ Clear the cornea
◦ Prevent progression of disc cupping &corneal diameters
◦ Preserve visual field and ocular integrity
◦ To stimulate development of binocular stereoscopic vision

44. Surgical options
I. Goniotomy
II. Trabeculotomy
III. Trabeculectomy + antimetabolites
IV. Trabeculotomy with trabeculectomy

45. Goniotomy
Clear cornea is prerequisite.
Preoperative gonioscopic evaluation is essential.
Success rate 80 – 90% if performed in 1-12 months age
Requisites  barkans lens and knife
Procedure  involves entering the AC with sharp goniotomy knife and making an opening
incision through the abnormally developed TM
 120 DEGRESS can be treated in single sitting
Post op  antibiotics, miotics

47. ADVANTAGES
1. Direct visualisations allows prescice
location
2. Less traumatic
3. Safe
4. Rapid
5. Can be repeated
6. Doesn’t injure conjunctiva
7. No bleb related complications
DISADVANTAGES
1. Lens have to be used to visualise angle
2. Not possible in cloudy corneas
3. Require surgical experience

48. Trabeculotomy
Indications
 Cloudy cornea
 After 2 goniotomies
 Can be combined with trabeculectomy
Success 73-100%
Procedure
 External incision
 Identify schemms canal
 Break through TM to increase aqueous flow, 120-140 degrees can be done

50. ADVANTAGES
1. Can be done in opaque corneas
2. High success rate
3. Lens not required
DISADVANTAGES
1. Angle not visualised directly
2. Schlemms canal not found in 4-20% cases
3. Hypotony may occur

51. Trabeculectomy
1. Lower success rates as compared to adults
2. Problems in children with PCG.
◦ Stretched and thinned out limbus.
◦ Distorted anatomy.
◦ A thick and active tenon capsule.
◦ Exuberant scar tissue due to rapid wound healing.
◦ Large buphthalmic eye with thin sclera and low scleral rigidity.

52. Indications
Failure of goniotomy and trabeculotomy
Poor success rate
Likely to fail due to low scleral rigidity, scarring
Best results with MMC
advantages
1. Post op IOP titrable
2. Lower pressure achievable
3. May clear cloudy cornea
disadvantages
1. Damage conjunctiva
2. Risk of endoph
3. Intraocular damage due to anti
metabolites
4. High risk of scarring

53. Trabeculotomy with trabeculectomy
Provides two major pathways for outflow.
1. Study by Mandal et al in 120 patients.
2. Apart from shallow AC post operatively, no increase in the complications.
3. High success rate –94.4%.
4. Recommend primary trab +trab in all babies presenting with corneal edema at birth.
The best surgical option in the management of PCG and suits the Indian patient population and
its social circumstances
Mandal et al Ophthalmolgy 1998 105: 6: 975-82

54. SURGERY
Clear cornea Corneal haze
goniotomy trabeculotomy
Goniotomy 1 Goniotomy 2
Trabeculotomy 3

55. Failure of goniotomy or trabeculotomy
trabeculectomy
Glaucoma drainage implant
Cyclodestructive procedures

56. Goniotomy/Trabeculectomy/Combined Trab-Trab
Surgical outcome?
EUA after 3 weeks
IOP controlled
Evaluation after 3 months
Normal IOP
Evaluation after 3 months
FU every 3 months
Record IOP, CDR, VA
Axial length, VF (if possible)
IOP not controlled
Add medical therapy
If IOP not controlled
repeat Trab-trab
Controlled
VISUAL
REHABILITATION
uncontrolled
Very poor prog.
Consider repeat trab,
Drainage implant,
Cyclodestruction.
FOLLOW UP

57. End point
• Normalization of IOP
• Clearing of cornea
• Reversal of optic disc cupping
• Stabilization of axial length

58. PROGNOSIS
Best results with isolated trabeculodysgenesis
Best when onset is between 1st and 24th month of life

59. CONCLUSIONS
Early diagnosis and prompt treatment can preserve vision
Responsibility of surgeon does not stop with the surgery.
Visual rehabilitation is equally important as IOP control.
An attempt should be made to familiarize the parents with the protracted course of
the illness.

60. THANK YOU