2. Definition of Terms
Primary congenital/ infantile glaucoma-
present at birth or 1st few years of life
Anterior chamber angle abnormalities
No systemic anomalies
Juvenile- > 3 y/o
Secondary infantile glaucoma
Associated with inflammatory, neoplastic,
hamartomatous, metabolic or other congenital
abnormalities
3. Epidemiology and Genetics
Primary Congenital Glaucoma
Rare
1- 10,000
50- 70 % of congenital glaucomas
60%- diagnosed by 6 months
80 %- 1st year of life
M > F (65 %)
Bilateral > unilateral – ( 70 %)
4. Inheritance:
AD
AR with variable penetrance
GLC3A/B/C
Ch 2, Ch 1, Ch 14
CYP1B1 gene- congenital glaucoma gene at the
GLCA3 locus
5. PATHOPHYSIOLOGY
Exact mechanism- unknown
1. cellular or membranous abnormality in the TM
Impermeable TM or a BARKAN membrane
2. abnormal insertion of the ciliary muscle
Developmental arrest in the late embryonic period
11. Examination under GA
Most GA agents and sedatives lower IOP, except
KETAMINE
Dehydration- lowers IOP
Normal IOP – 10- 15
12. Gonioscopy
High and flat iris insertion
Absence of angle recess
Peripheral iris hypoplasia
Tenting of peripheral iris pigment epithelium
Thickened uveal TM
Open angle
13. OPTIC DISC
Direct and indirect ophthalmoscopy
Optic nerve photography
Glaucomatous cupping:
Superior and inferior
Cup enlargement
Reversible
Indicates control of IOP
19. PROGNOSIS and FOLLOW- UP
Surgery- preferred treatment
Goniotomy
Trabeculotomy
Trabeculectomy, aqueous shunts-
Cyclophotocoagulation
20.
21.
22.
23. MEDICAL
temporizing
Control IOP, clear the cornea
B- blockers
Apnea
Hypotension
Cough
CAI
Acidosis
hypoK
A- adrenergic agonist
CNS adverse effect
Should not be used in patients < 3 y/o
With caution- < 10 y/o
24. Better prognosis- asymptomatic at birth,
symptomatic before 24 months old
Guarded- symptomatic at birth, and diagnosed after
2 y/o
COMPLICATIONS:
Amblyopia, corneal scarring, strabismus,
anisometropia, cataract, lens subluxation, recurrent
glaucoma