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