Download MBBS Ophthalmology PPT 24 Congenital Glaucoma Lecture Notes

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and

Congenital 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 corneas

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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 contoured

opacities 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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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 of

blindness in India

? Not reversible

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Definition of glaucoma

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? 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

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(with or without a raised IOP).

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Classification of glaucoma

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Glaucoma

Primary

Childhood

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Secondary

Glaucoma

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Glaucoma

Glaucoma

Open angle

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Angle

glaucoma

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closure

glaucoma

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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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Childhood glaucoma

? Primary congenital glaucoma

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? Glaucoma associated with ocular abnormalities

? Glaucoma associated with systemic abnormalities

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Secondary glaucoma

Glaucomas after ocular surgery

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Steroid induced glaucoma

Traumatic 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 chamber
leading to high intraocular pressure(IOP).
? Secondary congenital glaucoma
With associated ocular and systemic anomalies

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ANGLE 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 normal

angle 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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---------
Grade
IV

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II

II

I

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0

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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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Prevalence 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 angle

of 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 a

flat/concave iris insertion.

drawing of the same

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? Impaired aqueous outflow

? Elevated IOP

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An underdeveloped chamber angle

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Clinical presentation

Classic 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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Clinical presentation

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? Buphthalmos: Enlargement of

the globe as a result of
elevated IOP. Al segments of
the outer eye especially the

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cornea and sclera expand
principally at the corneoscleral
junction

? The anatomic landmarks are

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Advanced developmental

displaced.

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glaucoma with extensive

enlargement 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 ? 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

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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

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? Direct Gonioscopy ? to examine angle of anterior

chamber

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? Koeppe's gonioscopy
lens 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), hyperosmotic

agents(Mannitol), carbonic anhydrase inhibitors

(acetazolamide/dorzolamide)

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? Miotics and Alpha-2 agonists are not used in

children.

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Goniotomy/Trabeculotomy

Trabeculectomy with

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trabeculotomy

Modified Trabeculectomy

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Glaucoma drainage implant

Cyclodestructive 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 therapy

Normal 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 months

Poor prognosis

VISUAL

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Record IOP, CDR, VA

REHABILITATION

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Consider

Axial length, VF (if possible)

Drainage implant

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Cyclodestruction31

Goniotomy

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? Safe procedure when performed

skilfully.

? Performed with direct

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visualization of trabecular

meshwork

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? Aims to transect Schlemm's

canal by ab-interno approach

? Incises only superficial

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trabecular tissues, necessary to

cure this disease

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Trabeculotomy

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? Ab-externo trabeculotomy has good success rates.

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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 trabeculotomy

Scleral flap

Ds sDissection upto grey limbus

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Trabecular meshwork cut

Diffuse subconjunctival bl

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Role 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-destruction

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What is a Glaucoma Drainage Device?

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Glaucoma drainage devices

(GDDs) create an alternate

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aqueous pathway from the

anterior 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 an

equatorial 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 1950

BIETTI

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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 pupillary

Trans-scleral route

diode laser

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810 nm wave length

Penetrates through sclera

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Contact delivery through

fibre optic cable

Diode laser is prefer ed

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Melanin in the ciliary

epithelium bet er absorbs

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this wavelength

Causes 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 media

opacities

? Amblyopia therapy to achieve

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binocular stereoscopic vision

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VISUAL REHABILITATION

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? Low vision aids
vTelescopes (hand-held or
spectacle-mounted)

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vHand or pocket
magnifiers (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 is

as 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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