Dr. Atul Kumar Anand discusses primary congenital glaucoma, which occurs due to a developmental defect in the trabecular meshwork and anterior chamber angle. It affects about 1 in 10,000 births and is usually diagnosed within the first year of life. The main symptoms are photophobia, epiphora, and blepharospasm. Examination reveals corneal edema, enlargement, and increased intraocular pressure. The primary treatment is surgical, through procedures like goniotomy or trabeculotomy to relieve pressure and allow drainage of fluid from the eye. Follow-up is required over an indefinite period to monitor intraocular pressure and optic nerve changes.

1. Dr. Atul Kumar Anand
Senior Resident
AIIMS Patna

2. INTRODUCTION
Occurs due to
developmental
defect in the
trabecular meshwork
& anterior chamber
angle.

3. 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. Most cases are sporadic
10% familial/hereditary in which may be
autosomal recessive inheritance
three major loci (GLC3A, GLC3B, GLC3C)
identified on chromosome 2, 1, 14
respectively

5. 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. Infantile glaucoma: occurs from birth until 3 years of life
4. Juvenile glaucoma: occurs after the age of 3 to teenage
years
5. developmental glaucoma: if there is associated
anomalies, either ocular or systemic

6. Primary congenital glaucoma : without other
ocular findings.
Secondary congenital glaucoma : in
association with other syndromes, after injury,
congenital cataract extraction, or inflammation.
Buphthalmos: when disease manifest prior to age
of 3 years(infantile glaucoma), eyeball enlarges & so
the term bupthalmos(bull-like eyes)

7. 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

8.  Due to failure or abnormal development of trabecular
meshwork(trabeculodysgenesis).
 Iris may not completely separate from cornea, so that
angle remains closed by embryonic tissue.
 Depending upon degree of obstruction, results in
permanent rise in tension of eye, but since
circulation of aqueous is maintained, although at a
lower rate, by anterior ciliary vein & uveoscleral
outfow, rise in IOP is usually neither marked nor
acute.

9. 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.

10. Usually detected by enlarged or hazy
cornea
As cornea stretches breaks occur in
corneal endothelium ,aq. Enters cornea
causing swelling & haziness (frosted glass
app. )
Photophobia is due to irritation of corneal
nerves occurring as a result of raised iop (
infant burying its head in a pillow to avoid
light )

11. Signs
Corneal :
Corneal edema : usually 1st sign .at first
epithelium later stromal involvement
causing permanent opacity
Corneal enlargement

12. -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

13. -Tear in the descemet’s
membrane(Habb’s striae)
,which are single or multiple,
oriented horizontally or
concentric to the limbus
associated with corneal
edema in the early phases .
- In severe cases Acute
hydrops may occur.

14. - In advanced cases ,the zonules maybecome
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

15. Sclera : thin ,appears blue due to
underlying uveal tissue
A C : becomes deep ( as junction of
cornea & sclera stretches so that cornea is
forced forward )
Iris : may show iridodenesis
Iop: raised (neither marked nor acute ) or
normal
Optic disc : variable cupping
Axial myopia

16. Initial Evaluation
Office examination – Following can be
performed in children more than 5 year of
age-
Slit lamp examination
Applanation tonometery
Gonioscopy
Optic nerve evaluation
Retinoscopy

17.  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

18. 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

19. Cloudy cornea : in u/l commonest cause is
trauma with rupture of descemets
membrane(forceps injury), in b/l cases
causes are trauma ,
mucopolysaccharidosis ,interstitial keratitis
Large cornea (megalocornea)
Lacrimation : in infant usually due to
CNLDO , so early diagnosis of cong
glaucoma may be missed

20. Photophobia : may be due to keratitis or
uveitis
Raised iop : in infant may be associated
with retinoblastoma , ROP, PHPV,
traumatic glaucoma

21. Primarily surgical, however iop lowered by
use of b blockers,
acetazolamides,hyperosmotic agents till
surgery is done

22. Goniotomy
Procedure of choice in eyes
with congenital glaucoma with
clear cornea 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

23. 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

24. Mechanism of 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

25. Disadvantages
- Procedure not possible if media hazy
- Require special instrument
- Need experienced surgeon
- Possibility of corneal endothelial, angle and lens trauma
- Best prognosis for infant presenting between 2-8 months
of age
- Worst prognosis with elevated pressure and cloudy
cornea at birth

26. Procedure of choice; when cornea is opaque
or where goniotomy failed
Identify schlemm canal by external approach
As favorable as initial goniotomy procedure

27. 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

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

29. 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

30. 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

31. - filtration surgery with anti-fibrotic drugs
- glaucoma drainage implants
- cyclodestructive procedure

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

33. 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