CONGENITAL GLAUCOMA

GLAUCOMA
DEFINITION
Glaucoma is a group of disorders characterized by
progressive optic neuropathy resulting in characteristic
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
CONGENITAL / DEVELOPMENTAL
GLAUCOMA
It is a group of disorders in which abnormal intraocular pressure results due to
developmental abnormalities of the angle of anterior chamber obstructing the
drainage of aqueous humor.
Two Types:
1. PRIMARY CONGENITAL/DEVELOPMENTAL GLAUCOMA
2. SECONDARY CONGENITAL/ DEVELOPMENTAL GLAUCOMA
PRIMARY CONGENITAL
GLAUCOMA
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.
Depending upon the age of onset the developmental glaucoma are termed as :
1. Newborn glaucoma(40%) : True congenital glaucoma . IOP raised during intrauterine life
and child is born with ocular enlargement.
2. Infantile glaucoma (55%) : disease manifests prior to child’s third birthday
3. Juvenile glaucoma (5%) : develop pressure rise after 3 years but before adulthood.

BUPHTHALMOS : When the disease manifest prior to age of 3 years, the eyeball enlarges and so
the term buphthalmos (bull like eyes ). It results due to retention of aqueous humor.
Prevalence and genetic pattern
Sporadic occurrence 90%
Autosomal recessive inheritance in 10%
Bilateral occurrence is seen in 70%
Prevalence : 1 child in 10,000 births
 pathogenesis
 primary congenital glaucoma is due to failure or abnormal development of the trabecular
meshwork

 Maldevelopment, from neural crest derived cells, of trabeculum including the iridotrabecular
junction ( trabeculodysgenesis) is responsible for impaired aqueous outflow resulting in raised IOP.
 Clinically, trabeculodysgenesis is characterised by absence of the angle recess with iris having a
flat ( more common ) or concave direct insertion into surface of trabeculum .
 The iris may not completely separate from the cornea that the angle remain closed by persistent
embryonic tissue .
 CLINICAL FEATURES
1. Lacrimation, photophobia and blepharospasm – classical triad. Often associated with eye rubbing.
2. Corneal signs
 Corneal oedema : First sign that arouses suspicion. At first it is epithelial , but later there
stromal involvement and permanent opacities can occur.
 Corneal enlargement : Associated with buphthalmos
 Haab’s striae : Tears and breaks in descement’s membrane as it is less elastic than the
corneal stroma
 Hazy cornea with frosted glass appearance
3. Sclera : Thin and appears blue due to underlying uveal tissue.
4. Anterior chamber : Deep
5. Iris : Iridodonesis and atrophic patches in later stages
6. Optic disc : Variable cupping and atrophy especially after third year
7. IOP is raised
8. Axial myopia may occur due to increase in axial length
9. Large eyeball –buphthalmos
EXAMINATION
A complete examination under general anesthesia should be done . The examination should
include
1. Measurement of IOP , preferably with perkin’s applanation tonometer , should be done since
scleral rigidity is low in children.
2. Measurement of corneal diameter by callipers
3. Slit lamp examination
4. Ophthalmoscopy to evaluate optic disc
5. Gonioscopic examination of angle of anterior chamber reveals trabeculodysgenesis with
either flat or concave insertion. It is performed under general anesthesia using koeppe’s lens
DIFFERENTIAL DIAGNOSIS
1. LACRIMATION : in infants may be due to congenital nasolacrimal duct blockage. Other
causes in small child include corneal abrasion , Meesmann’s corneal dystrophy , Reis- Buckler
dystrophy.
2. PHOTOPHOBIA : Keratitis or Uveitis
3. CLOUDY CORNEA : In unilateral cases , the cause may be trauma with rupture of
descement’s membrane. In bilateral cases , causes may be trauma ,
mucopolysacchridosis , inerstitial keratitis and corneal epithelial
dystrophy.
4. LARGE CORNEA : Megalocornea , sclerocornea and high myopia
5. RAISED IOP : Retinoblastoma , retinopathy of prematurity , persistent primary hyperplastic
vitreous , traumatic glaucoma and secondary congenital glaucoma

6. OPTIC DISC CHANGES : Congenital anomalies of disc such as coloboma , hyperplasia ,

tinted disc and large physiological cup
 TREATMENT
MEDICAL TREATMENT
Medications are not very effective
IOP must be lowered by hyperosmotic agents , carbonic anhydrase
inhibitors and beta blockers ( timolol 0.5% ) till surgery is taken up.
Miotics are not used because they paradoxically increase IOP.
Alpha 2 agonist - CNS depression in children and hence contraindicated.
SURGICAL PROCEDURES
Incisional angle surgery
1. Goniotomy ( internal approach )
A Barkan’s goniotomy knife is passed through the limbus on temporal side.
Under the gonioscopic control the knife is passed across the anterior
chamber to the nasal part of the angle. An incision made in the angle
approximately midway between root of the iris and Schwalbe’s ring through
approximately 75 degree. The knife is then withdrawn.
TRABECULOTOMY (External approach)

This is useful when corneal clouding prevents visualization of the angle or in case where

goniotomy has failed.

In this canal of schelmm is exposed at about 12O’ clock position by a vertical scleral
incision after making a conjunctival flap and partial thickness flap. The lower prong of
harm’s trabeculotome is passed along the Schelmm’s canal on one side and the upper
prong is used as the guide. Then the trabeculotome is rotated so as to break the inner
wall over one quarter of the canal. This is then repeated on the other eye.
FILTERATION SURGERY
1. Trabeculectomy with antimetabolite
2. Combined trabeculotomy and trabeculectomy with
antimetabolites

GLUCOMA DRAINAGE DEVICES

JUVENILE OPEN ANGLE GLAUCOMA

JOAG is labelled when IOP is raised between 3 to 40 years of age and fulfill following criteria :

Meet glaucoma definition with open angles

No congenital anomalies or syndrome
No ocular enlargment
SECONDARY DEVELOPMENTAL
GLAUCOMA
Secondary developmental glaucoma with associated ocular
anomalies
1. Glaucoma associated with iridodysgenesis
Glaucoma associated with aniridia
Glaucoma associated with familial iris hypoplasia
Glaucoma associated with congenital ectropion uvea
Glaucoma associated with congenital microcornea
Glaucoma associated with congenital nanophthalmos
Glaucoma associated with iridiocorneal dysgenesis
1. Posterior embryotoxon
2. Axenfeld Rieger syndrome
3. Peter’s anomaly
4. Combined Rieger’s syndrome and Peter’s anomaly
Secondary developmental glaucoma associated with systemic
anomalies
1. Glaucoma associated with chromosomal disorders such as down’s syndrome , Edward’s
syndrome , Turner’s syndrome
2. Glaucoma associated with ectopia lentis syndrome which include Marfan’s syndrome ,
homocystinuria
3. Glaucoma associated with phakomatosis seen in Sturge Weber syndrome and Von
Recklinghausen’s neurofibromatosis
4. Glaucoma associated with metabolic syndromes such as Hurler’s syndrome
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