Download MBBS (Bachelor of Medicine, Bachelor of Surgery) Ophthalmology PPT 24 Congenital Glaucoma Lecture Notes
Introduction and classification of
glaucoma
and
Congenital Glaucoma
.
Learning Objectives
? At the end of this class the students shall be
able to :
? Define and classify glaucoma.
? Define congenital glaucoma.
? Understand the aetio-pathogenesis and clinical
features of congenital glaucoma.
? Understand the fundamentals of managing
congenital glaucoma.
2
Excessive
blinking +/-
watering
Hazy cornea
Large corneas
3
Question
? A child presents with watering , photophobia and an
enlarged cornea with a diameter of 13mm.
Examination of the eye reveals double contoured
opacities concentric to the limbus. Which of the
following is the most likely diagnosis:
? Superficial keratitis
? Deep keratitis
? Thyroid eye disease
? Congenital glaucoma
4
What is glaucoma ?
? The term glaucoma is derived
from the Greek word "glaukos"
meaning "gray blue"
? Second leading cause of
blindness worldwide
? Third most common cause of
blindness in India
? Not reversible
5
Definition of glaucoma
? Group of disorders characterized by progressive
optic neuropathy resulting in characteristic
morphological changes at the optic disc leading to a
specific pattern of irreversible visual field defects
(with or without a raised IOP).
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Classification of glaucoma
Glaucoma
Primary
Childhood
Secondary
Glaucoma
Glaucoma
Glaucoma
Open angle
Angle
glaucoma
closure
glaucoma
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Primary glaucoma
? Open angle glaucoma
? Primary Open angle glaucoma
? Normal Tension glaucoma
? Juvenile Open angle glaucoma
? Secondary Open angle glaucoma
? Steroid induced glaucoma
? Pigmentary glaucoma
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Primary glaucoma
? Angle closure glaucoma
? Primary angle closure glaucoma
? Secondary angle closure glaucoma
? Swollen lens
? Posterior segment tumours
? Neovascular glaucoma
? Plateau iris syndrome
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Childhood glaucoma
? Primary congenital glaucoma
? Glaucoma associated with ocular abnormalities
? Glaucoma associated with systemic abnormalities
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Secondary glaucoma
Glaucomas after ocular surgery
Steroid induced glaucoma
Traumatic glaucoma
11
Childhood glaucoma-Introduction
? Diverse group of disorders
? Primary congenital glaucoma-
Developmental abnormality of angle of anterior chamber
leading to high intraocular pressure(IOP).
? Secondary congenital glaucoma
With associated ocular and systemic anomalies
12
ANGLE OF ANTERIOR CHAMBER
- The peripheral recess of anterior chamber is known as the angle of
anterior chamber.
- It is clinically visualized by gonioscopy.
- Starting at the root of iris & progressing anteriorly towards the
cornea, the following structures can be identified in a normal
angle in an adult :
1) Ciliary body band (CBB) & root of iris
2) Scleral spur (SS)
3) Trabecular meshwork (TM)
4) Schwalbe's line (SL)
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Angle of anterior chamber as seen
on gonioscopy
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---------
Grade
IV
II
II
I
0
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Childhood glaucomas
? True congenital glaucomas- At birth or during
intrauterine period.
? Infantile glaucoma- Upto three years of age.
? Juvenile glaucoma- After three years of age and
upto 35 years of age.
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Prevalence and genetic pattern
? Sporadic occurrence in most cases (90%)
? Autosomal recessive in 10% of cases
? Loci linked with congenital glaucoma are
2p21(GLC3A), 1p36(GLC3B) and 14q24(GLC3C)
? 60% diagnosed by the age of 6 months and 80%
diagnosed within the first year of life
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Prevalence and genetic pattern
? Bilateral (about 70%) but asymmetrical
? Boys are affected slightly more frequently than girls
(65%)
? Prevalence is 1 in 10,000 births
? Chance of second sibling having disease is 3%
? Chance of third sibling (of two affected siblings) having
disease is 25%
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Pathogenesis
? Faulty development of angle
of anterior chamber from
neural crest derived cells
(trabeculodysgenesis)
The normal chamber angle: on the left is a
? Absence of angle recess with histological cross-section; on the right is a
flat/concave iris insertion.
drawing of the same
? Impaired aqueous outflow
? Elevated IOP
An underdeveloped chamber angle
20
Clinical presentation
Classic triad of
? Epiphora
? Blepharospasm
? Photophobia
? Babies rub their eyes
? Enlarged eyes
? Vision impaired
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Corneal signs
? Corneal oedema
? Corneal enlargement (Corneal diameter>13mm)
? Haab's striae : Descemet's membrane is not very
elastic and stretching may result in small
linear/circumferential tears that cause a certain
degree of corneal opacification.
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Clinical presentation
? Buphthalmos: Enlargement of
the globe as a result of
elevated IOP. Al segments of
the outer eye especially the
cornea and sclera expand
principally at the corneoscleral
junction
? The anatomic landmarks are
Advanced developmental
displaced.
glaucoma with extensive
enlargement and scarring of the
? The anterior chamber is deep
cornea.
23
Clinical presentation
? Sclera becomes thin and appears blue (due to
underlying uveal tissue
? Iris- atrophic in later stages
? Optic disc- variable cupping
? Intraocular pressure(IOP)- raised
? Axial myopia- due to increased axial length of
eyeball
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Examination under anaesthesia
? Mandatory in all cases
? Includes :
? Measurement of IOP ?
Perkins tonometer/Tonopen
(Normal 10-21 mm Hg)
? Measurement of corneal diameter ? by
callipers
(Normal 9.5mm-10.5mm)
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Examination under anaesthesia
? Slit lamp examination- with portable slit lamp
? Ophthalmoscopy- to evaluate optic disc
Asymmetric disc cupping in a child with developmental glaucoma. (A) Note
steep-wal ed cup. This is typical of glaucomatous cupping in the elastic
infant eye. (B) The left eye has no cupping.
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Examination under anaesthesia
? Direct Gonioscopy ? to examine angle of anterior
chamber
? Koeppe's gonioscopy
lens is preferable
? Angle is open but
immature in
congenital glaucoma
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Differential Diagnosis
? Hazy/Cloudy cornea-- -
? STUMPED (Sclerocornea, Trauma, Ulcer, Metabolic
disorders, Peter's anomaly, Endothelial dystrophy)
? Watering and intolerance to light-- -
Congenital Naso Lacrimal Duct obstruction
keratitis, conjunctivitis
? Optic cupping - - disc coloboma, hypoplasia,
physiological cupping
? Corneal enlargement -- megalocornea, high myopia
? Descemet's breaks - - Forceps delivery ,birth trauma 28
Management
? Glaucoma surgery is the primary option
? Medications are not very effective
? Role of medical management is temporary, till
surgery is taken up.
? Beta blockers (Timolol), hyperosmotic
agents(Mannitol), carbonic anhydrase inhibitors
(acetazolamide/dorzolamide)
? Miotics and Alpha-2 agonists are not used in
children.
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Goniotomy/Trabeculotomy
Trabeculectomy with
trabeculotomy
Modified Trabeculectomy
Glaucoma drainage implant
Cyclodestructive procedures
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Approach to management
Goniotomy/Trabeculectomy/Combined Trabe-Trab
Surgical outcome?
EUA after 3-4 weeks
IOP controlled
IOP not controlled
Evaluation after 3 months
Add medical therapy
Normal IOP
If IOP not control ed
repeat Trab ? MMC
Evaluation after 3 months Controled Uncontroled
FU every 3 months
Poor prognosis
VISUAL
Record IOP, CDR, VA
REHABILITATION
Consider
Axial length, VF (if possible)
Drainage implant
Cyclodestruction31
Goniotomy
? Safe procedure when performed
skilfully.
? Performed with direct
visualization of trabecular
meshwork
? Aims to transect Schlemm's
canal by ab-interno approach
? Incises only superficial
trabecular tissues, necessary to
cure this disease
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Trabeculotomy
? Ab-externo trabeculotomy has good success rates.
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Trabeculotomy with trabeculectomy
? Most commonly performed surgery in India
? Easy adaptability
? Safe and successful
? Suitable in compromised corneas
? More predictable results
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Steps of Trabeculectomy
with trabeculotomy
Scleral flap
Ds sDissection upto grey limbus
Trabecular meshwork cut
Diffuse subconjunctival bl
35 eb
Role of antimetabolites in paediatric glaucoma
? Significantly more complications associated with the
use of Mitomycin(MMC) in paediatric glaucomas
? Thin, avascular filtering blebs
? Wound leakage
? Choroidal detachment
? Bleb related endophthalmitis
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Options for refractory glaucoma ?
? Glaucoma Drainage Devices
? Cyclo-destruction
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What is a Glaucoma Drainage Device?
Glaucoma drainage devices
(GDDs) create an alternate
aqueous pathway from the
anterior chamber (AC) by
channeling aqueous out of the
eye through a tube to a
subconjunctival bleb. This tube
is usually connected to an
equatorial plate under the
conjunctiva.
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Cyclodestructive procedures
? Cyclocryotherapy
? Cyclophotocoagulation
?Transscleral
?Transpupillary
?Endoscopic
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CYCLO CRYOTHERAPY
TREATMENT OF 1950
BIETTI
Lasers relatively safer energy
? Trans-scleral route
? Direct application to ciliary
epithelium
Trans pupillary
Trans-scleral route
diode laser
810 nm wave length
Penetrates through sclera
Contact delivery through
fibre optic cable
Diode laser is prefer ed
Melanin in the ciliary
epithelium bet er absorbs
this wavelength
Causes more targeted
destruction with
less inflammation
VISUAL REHABILITATION
? Correction of refractive error
? Management of media
opacities
? Amblyopia therapy to achieve
binocular stereoscopic vision
43
VISUAL REHABILITATION
? Low vision aids
vTelescopes (hand-held or
spectacle-mounted)
vHand or pocket
magnifiers (2? to 3?)
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CONCLUSIONS
? Glaucoma is a group of disorders characterized by progressive
optic neuropathy.
? Early diagnosis and prompt treatment can preserve vision.
? All children with suspected childhood glaucoma should be
examined under anaesthesia.
? Mainstay of management of childhood glaucoma is surgery
? Visual rehabilitation and counseling of the parents of the child is
as important as IOP control.
45
Question
? Identify the abnormality
marked by arrow.
? Which structure is
involved?
? What type of slit lamp
illumination is used in
the photograph?
? Mention one
differential diagnosis of
the condition
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This post was last modified on 07 April 2022