given knowledege about congenital glaucoma in simpe term...

1. CONGENITAL
GLAUCOMA
MADE BY : SWATI PANARA
FROM : BHARTIMAIYA COLLEGE OF
OPTOMETRY
3rd YEAR 6th SEMESTER

2. GLAUCOMA
• Glaucoma is not a single disease process but a
group of disorders characterized by a
progressive optic neuropathy resulting in a
characterstic appearance of the optic disc and
a specific pattern of irreversible visual field
defects that are associated frequently but not
invariably with raised intraocular pressure
(IOP).

3. TYPES
CONGENITAL
GLAUCOMA
PRIMARY
ADULT
GLAUCOMA
SECONDARY
GLAUCOMA

4. CONGENITAL GLAUCOMA
• The congenital glaucoma are a group of
diverse disorders in which abnormal high
intraocular pressure results due to
developmental abnormalities of the angle of
anterior chamber obstructing the drainage of
aqueous humour.

5. TYPES
• PRIMARY DEVELOPMENTAL /
CONGENITAL GLAUCOMA
• DEVELOPMENTAL GLAUCOMA
WITH ASSOCIATED SPECIFIC
OCULAR OR SYSTEMIC
CONGENITAL ANOMALIES

6. PRIMARY DEVELOPMENTAL /
CONGENITAL GLAUCOMA
• It refers to abnormally high IOP which results
due to developmental anomaly of the angle of
the anterior chamber, not associated with any
other ocular or systemic anomaly.
• There are three types of primary congenital
glaucoma.

7. NEW BORN
GLAUCOMA
INFANTILE
GLAUCOMA
JUVENILE
GLAUCOMA

8. NEW BORN GLAUCOMA
• when IOP is raised during intrauterine life and
child is born with ocular enlargement.
• It occurs in about 40 percent of cases.

9. INFANTILE GLAUCOMA
• when the disease manifests prior to the child's
third birthday.
• It occurs in about 50 percent of cases.

10. JUVENILE GLAUCOMA
• the rest 10 percent of cases who develop
pressure rise between 3-16 years of life.
• Gonioscopy is normal.

11. • Most infant corneas measure less than
10.5mm in horizontal diameter.
• A measurement over 12mm is considered
diagnostic of congenital glaucoma.
• These eyes, hazy and enlarged, appear so
grotesque that the term buphthalmos.

13. PREVALENCE AND GENETIC PATTERN
• Most cases are sporadic.
• About 10 percent cases exhibit an autosomal recessive
inheritance with incomplete peneterance.
• Genes implicated are GLC3A , GLC3B & GLC3C.
• Although sex linkage is not common in inheritance,
over 65 percent of the patients are boys.
• The disease is bilateral in 75 percent cases, though the
involvement may be asymmetric.
• The disease affects only 1 child in 10,000 births.

14. PATHOGENESIS
• Maldevelopment of trabeculum including the
iridotrabecular junction is responsible for
impaired aqueous outflow resulting in raised
IOP.
• In primary congenital glaucoma the
trabeculodysgenesis is not associated with any
other major ocular anomalies.

15. CLINICAL FEATURES
• Photophobia, blepharospasm,lacrimationandeyerubbingoften
occurtogether.
• Cornealsigns.Cornealsignsincludeitsoedema, enlargement and
Descemet’s breaks.
• IOPis raisedwhichis neithermarkednoracute.
• Axialmyopiamayoccurbecauseof increasein axial length which
may give rise to anisometropic amblyopia

18. • Sclerabecomesthinandappearsbluedue to underlying uveal
tissue.
• Anteriorchamberbecomesdeep.
• Irismayshowiridodonesisandatrophicpatches in late stage.
• Lensbecomesflatdue to stretchingof zonulesand may even
subluxate.
• Opticdiscmayshowvariablecuppingandatrophy especially
after third year.

19. EXAMINATION
• A complete examination under general
anaesthesia should be performed on each child
suspected of having congenital glaucoma:
(1) MEASUREMENT OF IOP : Schiotz or
preferably hand held Perkin’s applanation
tonometer since scleral rigidity is very low in
children.
(2) MEASUREMENT OF CORNEAL
DIAMETER.

20. (3) OPHTHALMOSCOPY TO EVALUATE OPTIC
DISC
(4) GONIOSCOPIC EXAMINATION
(5) CENTRAL CORNEAL THICKNESS
(6) ULTRA SOUND

22. DIFFERENTIAL DIAGNOSIS
• Cloudy cornea. In unilateral cases the commonest cause is trauma
with rupture of Descemet’s membrane. In bilateral cases causes
may be trauma, interstitial keratitis and corneal endothelial
dystrophy.
• Large cornea due to buphthalmos should be differentiated from
megalocornea.
• Lacrimation in an infant is usually considered to be due to
congenital nasolacrimal duct blockage and thus early diagnosis of
congenital glaucoma may be missed.
• Photophobia may be due to keratitis or uveitis.
• Raised IOP in infants may also be associated with retinoblastoma,
retinopathy of prematurity, persistent primary hyperplastic
vitreous, traumatic glaucoma and secondary congenital glaucoma
seen in rubella, aniridia.

23. TREATMENT
• Treatment of congenital glaucoma is primarily
surgical :
(1) Incisional angle surgery
(2) Filteration surgery

24. DEVELOPMENTAL GLAUCOMAS WITH
ASSOCIATED ANOMALIES
• A wide variety of systemic and/or ocular
anomalies have an associated raised IOP, usually
due to developmental defects of the anterior
chamber angle.
(1) Glaucoma associated with iridocorneal
dysgenesis. These include: posterior embryotoxon
characterized by a prominent Schwalbe’s ring
Rieger anomaly, Rieger syndrome, Peter’s
anomaly and combined Rieger syndrome and
Peter’s anomaly.
(2) Glaucoma associated with aniridia (50% cases).

25. SCHWALBE’S RING

26. PETERS ANOMALY

27. ANIRIDIA

28. RIEGER SYNDROME

29. • (3) Glaucoma associated with ectopia lentis
syndromes, which include Marfan’s syndrome,
Weil - Marchesani syndrome and
homocystinuria.
• (4) Glaucoma associated with phakomatosis is
seen in Sturge-Weber syndrome ( 50% cases)
and Von Recklinghausen’s neurofibromatosis
(25% cases).

31. • (5) Miscellaneous conditions. Lowe’s syndrome
naevus of Ota, nanophthalmos, congenital
ectropion uveae, congenital microcornea and
rubella syndrome.