Glaucoma is a group of eye diseases characterized by optic nerve damage and visual field loss. It is often associated with elevated intraocular pressure but can sometimes occur with normal pressures. The two main types are open-angle glaucoma and angle-closure glaucoma. Congenital glaucoma is present at birth and results from impaired aqueous outflow, often due to developmental abnormalities in the trabecular meshwork. It is typically treated surgically through procedures like goniotomy or trabeculotomy to improve drainage and lower pressure. Long term monitoring is important as the disease can progress if not properly managed.

1. Glaucoma

2. GLAUCOMA
 Definition
The term glaucoma refers to the group of diseases that have in
common a characteristic optic neuropathy with associated visual
function loss. Although IOP is one of the primary risk factors, it’s
presence or absence doesn’t have a role in the definition of the
disease.
Glaucoma leads to a characteristic appearance of the optic nerve head
and a specific pattern of irreversible visual field defects.

3. Why glaucoma is important?
 Second leading cause
of global blindness
 Leading cause of
irreversible visual loss.
 Visual impairment from
glaucoma affects
adults more than
children and women
more than men.
 By the year 2020 :
80.5 million people in
the world with
glaucoma

4. ANATOMY AND PHYSIOLOGY
APPLIED ANATOMY
• Pathophysiology of glaucoma revolves around the aqueous
humour dynamics
• It is essential to understand the production and drainage of aqueous
humour as well as the anatomy of the angle of the anterior chamber
• The principal ocular structures concerned with it are
• Ciliary body
• Angle of anterior chamber
• Aqueous outflow system

5. CILIARY BODY • Ciliary body is forward
continuation of the choroid at
ora serrata
• Triangular in shape
• The anterior side of the triangle
forms the part of the angle of
anterior and posterior chambers
• The anterior part (about 2 mm)
having finger-like ciliary
processes is called pars plicata
and the posterior smooth part
(about 4 mm) is called pars
plana

6. Angle of anterior chamber
• Angle of anterior chamber plays an important role in the process of
aqueous drainage
• Formed by root of iris, anterior-most part of ciliary body, scleral spur,
trabecular meshwork and Schwalbe’s line (prominent end of
Descemet’s membrane of cornea)
• Fig. Section of the anterior ocular structures showing
region of the anterior chamber

7.  Clinically the angle structures can be visualised by gonioscopic examination
• Gonioscopic grading of the angle width
• The most commonly used is Shaffer’s system
• Diagrammatic depiction of various angle structures (SL, Schwalbe's line; TM, trabecular
meshwork; SS, scleral spur; CBB, ciliary body band; ROI, root of iris) as seen in different
grades of angle width (Schaffer's gradingsystem): A, Gonioscopic view; B, Configuration
of the angle in cross section of the anterior chamber

8. Aqueous outflow system
• IT includes
• Trabecular meshwork
• sieve-like structure through
which aqueous humour leaves
the eye
• consists of three layers,which
from inside out are
I. Uveal meshwork
II. Corneoscleral meshwork
III. Juxtacanalicular
(endothelial) meshwork
Schlemm’s canal
Collector channels
 Direct( aqueous veins)
 Indirect
Episcleral veins
The aqueous outflow system

9. APPLIED PHYSIOLOGY
• Concerned with the dynamics of aqueous humour are its production,
drainage and maintenance of intraocular pressure
• Aqueous humour and its production
• Functions and compostion of aqueous humour
• Volume : Anterior chamber (0.25 ml) and posterior chamber (0.06 ml)
• Functions of aqueous humour are
• Maintains a proper intraocular pressure
• Metabolic and nutritional role
• Optical function
• Clearing function

10. • Functions and compostion of aqueous humour cont.
• Refractive index of aqueous humour is 1.336
• Composition
• Water 99.9% , solids 0.1%
• Proteins (colloid content)(5-6 mg%)
• Amino acid 5 mg/kg water
• Non-colloid constituents
• Oxygen
• Aqueous humour: anterior chamber versus posterior chamber
• main differences are : HCO3 and Ascorbate in posterior chamber
Cl in posterior chamber

11. Aqueous humour formation
• Ultrafiltration
• Secretion
• Diffusion
Control of aqueous formation
Drainage of aqueous humour
Trabecular (conventional)
outflow
Uveoscleral
(unconventional) outlow Flow chart depicting drainage of aqueous humour

12. Mechanism of aqueous transport across inner wall
of Schlemm’s canal
Passive filter mechanism
Leaky pores in endothelial cells
Contraction microfilaments
Sondermann’channels
Vacuolation theory-it was one of the most accepted view till
recent past
Aqueous outflow active pump mechanism

13. Vacuolation theory of aqueous transport across the inner wall of the Schlemm's canal: 1. Non-vacuolated stage; 2.
Stage of early infolding of basal surface of the endothelial cell; 3. Stage of macrovacuolar structure formation; 4.
Stage of vacuolar transcellular channel formation;5.Stage of occlusion of the basal infolding.

14. Aqueous outflow pump mechanism, a part of muscular circulatory loop: (A)
during diastole; (B) during systole

15. Intraocular pressure
 The intraocular pressure (IOP) refers to the pressure exerted by the
intraocular fluids on the coats of the eyeball.
 Normal IOP = 10-21 mmHg
 It is determined by the rate of aqueous secretion and rate of
outflow.

16. Factors influencing intraocular pressure
 Local factors
1. Rate of aqueous formation which in turn is influenced by
permeability of ciliary capillaries and osmotic pressure of the blood.
2. Resistance to aqueous outflow
3. Increased episcleral venous pressure may result in rise of IOP
4. Dilatation of pupil in patients with narrow angle can cause rise in
IOP
5. Refractive errors

17.  General factors
1. Hereditary
2. Age - increases after 40 years
3. Sex- mean IOP is greater in females in the older age
group
4. Diurnal variation- usually the IOP is greater in the
morning than evening ( normal <5mmHg)
5. Postural variation- increases from sitting to supine
6. Blood pressure- glaucoma is more prevalent in
hypertensives
7. Osmotic pressure of blood- increase in plasma
osmolarity is associated with decrease in IOP and vice
versa
8. General anaesthetics and drugs

18. CLASSIFICATION OF GLAUCOMA
• Classification
• Clinico-etiologically glaucoma may be classified as follows:
• (A) Congenital and developmental glaucomas
• 1. Primary congenital glaucoma (without associated anomalies).
• 2. Developmental glaucoma (with associated anomalies).
• (B) Primary adult glaucomas
• 1. Primary open angle glaucomas (POAG)
• 2. Primary angle closure glaucoma (PACG)
• 3. Primary mixed mechanism glaucoma
• (C) Secondary glaucomas
• Depending upon etiology
• Depending upon mechanism of raised IOP

19. Epidemiology
• Global prevalence of glaucomas
• 2% of those over the age of 40 years , and
• 10% of those over 80 years of age
• POAG versus PACG in different ethnic groups
• Glaucoma blindness
• Global : 8%
• India : 12.8%

20. Glaucoma – what is happening ?
Either:
the drain blocks
here
Or poor blood
supply here
Damages the optic
nerve..looks ‘caved in’,
called ‘cupped’

21. Pathogenesis of ocular damage
Death of retinal ganglion cells(RGCs) caused by :
Primary insults
1. Mechanical theory(raised IOP)
Neurotrophins are unable to reach the RGCs due to axonal
deformation and ischaemia caused by mechanical stretch on
lamina cribrosa.
2. Vascular insufficiency theory
Failure of autoregulatory mechanism
Vasospasm
Systemic hypotension
Secondary insults
Toxic factors like glutamate, oxygen free radicals, nitric oxide
released when RGCs undergo apoptosis due to primary
insults

22. What happens in glaucoma

23. CONGENITAL / DEVELOPMENTAL
GLAUCOMAS
• TERMINOLOGY
• Types
1. Primary
developmental/congenital
glaucoma
2. Developmental glaucoma
with associated ocular anomalies
3. developmental glaucoma
with associated systemic
anomalies

24. PRIMARY DEVELOPMENTAL/CONGENITAL GLAUCOMA
1. Newborn glaucoma
2. Infantile glaucoma
3. Juvenile glaucoma
• Prevalence and genetic pattern
• Pathogenesis
• Maldevelopment of trabeculum including the iridotrabecular
junction (trabeculodysgenesis) is responsible for impaired
aqueous outflow resulting in raised IOP
• Flat iris insertion is more common than the concave iris insertion
• Concave iris insertion is less common

25. Clinical features:-
• 1.Blephrospasm
• 2.Photophobia
• 3.Lacrimation
Triad
Blephrospasm and Photophobia

26. Clinical features
1. Photophobia, blepharospasm, lacrimation ( classic triad)
2. Corneal signs
1. Corneal oedema
2. Corneal enlargement
3. Tears and breaks in Descemet’s membrane(Haab’s striae)
3. Sclera
4. Anterior chamber becomes deep
5. Iris may show iridodonesis and atrophic patchesin late stage
6. Lens becomes flat due to stretching of zonules and may even
subluxate
7. Optic disc may show variable cupping and atrophy especially
after third year
8. IOP is raised which is neither marked nor acute
9. Axial myopia

27. Haab Striae

28. Corneal Haze

29. Examination (Evaluation)
• A complete examination under general anaesthesia (EUA) should be
performed on each child suspected of having congenital glaucoma
• The examination should include following
1. Measurement of IOP
2. Measurement of corneal diameter
3. SLIT LAMP EXAMINATION
4. Gonioscopic examination of angle of anterior chamber
5. Ophthalmoscopy
Differential diagnosis
• It is to be considered for different presenting signs as
follows
• Cloudy cornea, Large cornea, Lacrimation, Photophobia, Raised
IOP, Optic disc changes

30. Cloudy Cornea- STUMPED
Sclerocornea
Tears (e.g. tears in Descemets due to forceps delivery)
Ulcers
Mucopolysaccharidoses
Peters anomaly
Endothelial dystrophy
Dermoids

31. Treatment
• Medical treatment
• Surgical procedures for
congenital glaucoma
1. Incisional angle surgery
1. Goniotomy
2. Trabeculotomy
2. Filteration surgery
1. Trabeculectomy with
antimetabolites
2. Combined trabeculotomy
and trabeculectomy with
antimetabolites
3. Glaucoma drainage
devices(GDD)
Technique of goniotomy : A, showing position
of goniotomy knife in the angle under direct
visualization; B, showing procedure of
sweeping the knife in the angle

32. Technique of trabeculotomy

33. Follow-Up
• Patient should be reviewed 1 month after initial surgery.
• IOP & Corneal diameters should be monitored at regular interval.
• Cycloplegic refraction should be done at 6 monthly interval.

34. • DEVELOPMENTAL GLAUCOMAS WITH ASSOCIATED
OCULAR ANOMALIES
• Glaucoma associated with iridodysgenesis
• ANIRIDIA
• FAMILIAL IRIS HYPOPLASIA
• CONGENITAL ECTROPION UVEA
• WITH CONGENITAL MICROCORNEA
• CONGENITAL NANOPHTHALMOS
• Glaucoma associated with iridocornealdysgenesis
• POSTERIOR EMBRYOTOXON
• AXENFELD-RIEGER SYNDROME
• PETER’S ANOMOLY COMBINED REIGER’S
SYNDROME AND PEYERS ANOMALY

35. DEVELOPMENTAL GLAUCOMAS WITH ASSOCIATED
SYSTEMIC ANOMALIES
1. Chromosomal disorders
2. Ectopic lentis syndromes which include Marfan’s
syndrome, Weil-Marchesani syndrome and
homocystinuria.
3. Glaucoma associated with phakomatosis is seen in
Sturge-Weber syndrome ( 50% cases) and Von
Recklinghausen’s neurofibromatosis (25% cases)
4. Metabolic syndromes