Introduction and classification of
glaucoma
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andCongenital Glaucoma
.
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Learning Objectives
? At the end of this class the students shall be
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able to :? Define and classify glaucoma.
? Define congenital glaucoma.
? Understand the aetio-pathogenesis and clinical
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features of congenital glaucoma.
? Understand the fundamentals of managing
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congenital glaucoma.2
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Excessive
blinking +/-
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watering
Hazy cornea
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Large corneas3
Question
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? A child presents with watering , photophobia and an
enlarged cornea with a diameter of 13mm.
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Examination of the eye reveals double contouredopacities concentric to the limbus. Which of the
following is the most likely diagnosis:
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? Superficial keratitis
? Deep keratitis
? Thyroid eye disease
? Congenital glaucoma
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4
What is glaucoma ?
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? The term glaucoma is derived
from the Greek word "glaukos"
meaning "gray blue"
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? Second leading cause of
blindness worldwide
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? Third most common cause ofblindness in India
? Not reversible
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5
Definition of glaucoma
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? Group of disorders characterized by progressiveoptic neuropathy resulting in characteristic
morphological changes at the optic disc leading to a
specific pattern of irreversible visual field defects
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(with or without a raised IOP).6
Classification of glaucoma
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GlaucomaPrimary
Childhood
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Secondary
Glaucoma
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GlaucomaGlaucoma
Open angle
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Angle
glaucoma
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closureglaucoma
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Primary glaucoma
? Open angle glaucoma
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? Primary Open angle glaucoma? Normal Tension glaucoma
? Juvenile Open angle glaucoma
? Secondary Open angle glaucoma
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? Steroid induced glaucoma? Pigmentary glaucoma
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Primary glaucoma
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? Angle closure glaucoma
? Primary angle closure glaucoma
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? Secondary angle closure glaucoma? Swollen lens
? Posterior segment tumours
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? Neovascular glaucoma
? Plateau iris syndrome
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9Childhood glaucoma
? Primary congenital glaucoma
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? Glaucoma associated with ocular abnormalities
? Glaucoma associated with systemic abnormalities
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10Secondary glaucoma
Glaucomas after ocular surgery
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Steroid induced glaucomaTraumatic glaucoma
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Childhood glaucoma-Introduction
? Diverse group of disorders
? Primary congenital glaucoma-
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Developmental abnormality of angle of anterior chamberleading to high intraocular pressure(IOP).
? Secondary congenital glaucoma
With associated ocular and systemic anomalies
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12ANGLE OF ANTERIOR CHAMBER
- The peripheral recess of anterior chamber is known as the angle of
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anterior chamber.- It is clinically visualized by gonioscopy.
- Starting at the root of iris & progressing anteriorly towards the
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cornea, the following structures can be identified in a normalangle in an adult :
1) Ciliary body band (CBB) & root of iris
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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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14---------
Grade
IV
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IIII
I
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015
16
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Childhood glaucomas? True congenital glaucomas- At birth or during
intrauterine period.
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? 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
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? 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)
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? 60% diagnosed by the age of 6 months and 80%
diagnosed within the first year of life
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18Prevalence and genetic pattern
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? Bilateral (about 70%) but asymmetrical? Boys are affected slightly more frequently than girls
(65%)
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? 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
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? Faulty development of angleof anterior chamber from
neural crest derived cells
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(trabeculodysgenesis)
The normal chamber angle: on the left is a
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? Absence of angle recess with histological cross-section; on the right is aflat/concave iris insertion.
drawing of the same
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? Impaired aqueous outflow
? Elevated IOP
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An underdeveloped chamber angle20
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Clinical presentationClassic triad of
? Epiphora
? Blepharospasm
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? Photophobia? Babies rub their eyes
? Enlarged eyes
? Vision impaired
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Corneal signs
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? Corneal oedema? Corneal enlargement (Corneal diameter>13mm)
? Haab's striae : Descemet's membrane is not very
elastic and stretching may result in small
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linear/circumferential tears that cause a certain
degree of corneal opacification.
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22Clinical presentation
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? Buphthalmos: Enlargement ofthe globe as a result of
elevated IOP. Al segments of
the outer eye especially the
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cornea and sclera expandprincipally at the corneoscleral
junction
? The anatomic landmarks are
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Advanced developmental
displaced.
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glaucoma with extensiveenlargement and scarring of the
? The anterior chamber is deep
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cornea.
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Clinical presentation? Sclera becomes thin and appears blue (due to
underlying uveal tissue
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? Iris- atrophic in later stages
? Optic disc- variable cupping
? Intraocular pressure(IOP)- raised
? Axial myopia- due to increased axial length of
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eyeball
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Examination under anaesthesia
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? Mandatory in all cases? Includes :
? Measurement of IOP ?
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Perkins tonometer/Tonopen
(Normal 10-21 mm Hg)
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? Measurement of corneal diameter ? bycallipers
(Normal 9.5mm-10.5mm)
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Examination under anaesthesia? Slit lamp examination- with portable slit lamp
? Ophthalmoscopy- to evaluate optic disc
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Asymmetric disc cupping in a child with developmental glaucoma. (A) Notesteep-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
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? Direct Gonioscopy ? to examine angle of anterior
chamber
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? Koeppe's gonioscopylens is preferable
? Angle is open but
immature in
congenital glaucoma
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Differential Diagnosis
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? Hazy/Cloudy cornea-- -? STUMPED (Sclerocornea, Trauma, Ulcer, Metabolic
disorders, Peter's anomaly, Endothelial dystrophy)
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? Watering and intolerance to light-- -Congenital Naso Lacrimal Duct obstruction
keratitis, conjunctivitis
? Optic cupping - - disc coloboma, hypoplasia,
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physiological cupping? Corneal enlargement -- megalocornea, high myopia
? Descemet's breaks - - Forceps delivery ,birth trauma 28
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Management? Glaucoma surgery is the primary option
? Medications are not very effective
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? Role of medical management is temporary, till
surgery is taken up.
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? Beta blockers (Timolol), hyperosmoticagents(Mannitol), carbonic anhydrase inhibitors
(acetazolamide/dorzolamide)
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? Miotics and Alpha-2 agonists are not used in
children.
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29Goniotomy/Trabeculotomy
Trabeculectomy with
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trabeculotomy
Modified Trabeculectomy
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Glaucoma drainage implantCyclodestructive procedures
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Approach to management
Goniotomy/Trabeculectomy/Combined Trabe-Trab
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Surgical outcome?
EUA after 3-4 weeks
IOP controlled
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IOP not controlled
Evaluation after 3 months
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Add medical therapyNormal IOP
If IOP not control ed
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repeat Trab ? MMC
Evaluation after 3 months Controled Uncontroled
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FU every 3 monthsPoor prognosis
VISUAL
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Record IOP, CDR, VA
REHABILITATION
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ConsiderAxial length, VF (if possible)
Drainage implant
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Cyclodestruction31
Goniotomy
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? Safe procedure when performedskilfully.
? Performed with direct
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visualization of trabecular
meshwork
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? Aims to transect Schlemm'scanal by ab-interno approach
? Incises only superficial
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trabecular tissues, necessary to
cure this disease
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32Trabeculotomy
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? Ab-externo trabeculotomy has good success rates.33
Trabeculotomy with trabeculectomy
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? Most commonly performed surgery in India
? Easy adaptability
? Safe and successful
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? Suitable in compromised corneas? More predictable results
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Steps of Trabeculectomy
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with trabeculotomyScleral flap
Ds sDissection upto grey limbus
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Trabecular meshwork cut
Diffuse subconjunctival bl
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35 ebRole of antimetabolites in paediatric glaucoma
? Significantly more complications associated with the
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use of Mitomycin(MMC) in paediatric glaucomas
? Thin, avascular filtering blebs
? Wound leakage
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? Choroidal detachment? Bleb related endophthalmitis
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Options for refractory glaucoma ?
? Glaucoma Drainage Devices
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? Cyclo-destruction37
What is a Glaucoma Drainage Device?
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Glaucoma drainage devices
(GDDs) create an alternate
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aqueous pathway from theanterior chamber (AC) by
channeling aqueous out of the
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eye through a tube to a
subconjunctival bleb. This tube
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is usually connected to anequatorial plate under the
conjunctiva.
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Cyclodestructive procedures
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? Cyclocryotherapy
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? Cyclophotocoagulation?Transscleral
?Transpupillary
?Endoscopic
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CYCLO CRYOTHERAPY
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TREATMENT OF 1950BIETTI
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Lasers relatively safer energy? Trans-scleral route
? Direct application to ciliary
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epithelium
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Trans pupillaryTrans-scleral route
diode laser
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810 nm wave length
Penetrates through sclera
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Contact delivery throughfibre optic cable
Diode laser is prefer ed
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Melanin in the ciliary
epithelium bet er absorbs
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this wavelengthCauses more targeted
destruction with
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less inflammation
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VISUAL REHABILITATION
? Correction of refractive error
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? Management of mediaopacities
? Amblyopia therapy to achieve
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binocular stereoscopic vision43
VISUAL REHABILITATION
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? Low vision aids
vTelescopes (hand-held or
spectacle-mounted)
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vHand or pocketmagnifiers (2? to 3?)
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CONCLUSIONS
? Glaucoma is a group of disorders characterized by progressive
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optic neuropathy.? Early diagnosis and prompt treatment can preserve vision.
? All children with suspected childhood glaucoma should be
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examined under anaesthesia.
? Mainstay of management of childhood glaucoma is surgery
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? Visual rehabilitation and counseling of the parents of the child isas important as IOP control.
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Question
? Identify the abnormality
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marked by arrow.? Which structure is
involved?
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? What type of slit lamp
illumination is used in
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the photograph?? Mention one
differential diagnosis of
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the condition
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