primary congenital glaucoma - aao.org

Q Primary Congenital Glaucoma Evident at birth or within first few years‘age’ period of time

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Evident at birth or within first few years

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births#

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateralunilateralv bilateral

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…malemale vs

female

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…male

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…male…diagnosed by age 6 months

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…male…diagnosed by age 6 months

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…male…diagnosed by age 6 months

Associated genetically with POAG? Noyes/no

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…male…diagnosed by age 6 months

Associated genetically with POAG? No

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…male…diagnosed by age 6 months

Associated genetically with POAG? No Is secondary to angle dysplasia in the absence of

other ocular or systemic abnormalitiesstructure

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…male…diagnosed by age 6 months

Associated genetically with POAG? No Is secondary to angle dysplasia in the absence of

other ocular or systemic abnormalities

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…male…diagnosed by age 6 months

Associated genetically with POAG? No Is secondary to angle dysplasia in the absence of

other ocular or systemic abnormalities

association with

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What if other ocular and/or systemic abnormalities are present?Would be secondary congenital glaucoma (or confusingly, primary developmental glaucoma)

Primary Congenital Glaucoma

A Primary Congenital Glaucoma Evident at birth or within first few years 1/10K live births 2/3 are…

…bilateral…male…diagnosed by age 6 months

Associated genetically with POAG? No Is secondary to angle dysplasia in the absence of

other ocular or systemic abnormalities

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What if other ocular and/or systemic abnormalities are present?Would be secondary congenital glaucoma (or confusingly, primary developmental glaucoma)

association with

Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora2) Photosensitivity3) Blepharospasm

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Primary Congenital Glaucoma

A/Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora2) Photosensitivity3) Blepharospasm

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Primary Congenital Glaucoma

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Congenital glaucoma: The triad

Epiphora Photophobia/blepharospasm

Primary Congenital Glaucoma

A/Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

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three words

Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasmia

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema

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Primary Congenital Glaucoma

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Congenital glaucoma: Corneal cloudiness

Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striaeeponym

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae

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Haab striae represent what sort of pathology, ie, which corneal structure is damaged, and in what way?Tears in Descemet’s secondary to corneal stretching

What is the mechanism?Mechanical deformation caused by the elevated IOP

Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae

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Haab striae represent what sort of pathology, ie, which corneal structure is damaged, and in what way?Tears in Descemet’s secondary to corneal stretching

What is the mechanism?Mechanical deformation caused by the elevated IOP

Primary Congenital Glaucoma

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Congenital glaucoma: Haab striae

Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae

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Haab striae represent what sort of pathology, ie, which corneal structure is damaged, and in what way?Tears in Descemet’s secondary to corneal stretching

What is the mechanism?Mechanical deformation caused by the elevated IOP

Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae

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Haab striae represent what sort of pathology, ie, which corneal structure is damaged, and in what way?Tears in Descemet’s secondary to corneal stretching

What is the mechanism?Mechanical deformation caused by the elevated IOP

Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mmmm

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~1# #

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~14

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~14 In congenital glaucoma: 30-40 unanaesthetized#

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~14 In congenital glaucoma: 30-40 unanaesthetized

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~14 In congenital glaucoma: 30-40 unanaesthetized >20 even under anesthesia#

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms:1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~14 In congenital glaucoma: 30-40 unanaesthetized >20 even under anesthesia

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~14 In congenital glaucoma: 30-40 unanaesthetized >20 even under anesthesia

General anesthetic(s) that raise IOP:Ketamine

General anesthetic(s) that lower IOP:Pretty much everything else

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~14 In congenital glaucoma: 30-40 unanaesthetized >20 even under anesthesia

General anesthetic(s) that raise IOP:Ketamine

General anesthetic(s) that lower IOP:Pretty much everything else

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~14 In congenital glaucoma: 30-40 unanaesthetized >20 even under anesthesia

General anesthetic(s) that raise IOP:Ketamine

General anesthetic(s) that lower IOP:Pretty much everything else

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Clinical presentation

The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction)2) Photosensitivity3) Blepharospasm

Cornea: Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11.5 mm

IOP: Normal IOP: Infancy 10-12 ; age 7 years ~14 In congenital glaucoma: 30-40 unanaesthetized >20 even under anesthesia

General anesthetic(s) that raise IOP:Ketamine

General anesthetic(s) that lower IOP:Pretty much everything else

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Natural history if untreated: Progresses to NLPvisual

acuity

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Natural history if untreated: Progresses to NLP

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(NLP = No light perception)

Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Natural history if untreated: Progresses to NLP Cornea… Opacifies and vascularizesbad sequelae 1 bad sequelae 2

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Natural history if untreated: Progresses to NLP Cornea… Opacifies and vascularizes

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Primary Congenital Glaucoma

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Congenital glaucoma: Corneal opacification, vascularization

Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Natural history if untreated: Progresses to NLP Cornea… Opacifies and vascularizes diameter ~17 mm# (mm)

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Natural history if untreated: Progresses to NLP Cornea… Opacifies and vascularizes diameter ~17 mm

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Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Natural history if untreated: Progresses to NLP Cornea… Opacifies and vascularizes diameter ~17 mm

Pseudoproptosis 2o to buphthalmos (enlargement of globe secondary to chronically elevated IOP)

translation: ox’s eye

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Natural history if untreated: Progresses to NLP Cornea… Opacifies and vascularizes diameter ~17 mm

Pseudoproptosis 2o to buphthalmos (enlargement of globe secondary to chronically elevated IOP)

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Primary Congenital Glaucoma

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Congenital glaucoma: Pseudoproptosis 2ndry to buphthalmos

Primary Congenital Glaucoma

Q Primary Congenital Glaucoma Natural history if untreated: Progresses to NLP Cornea… Opacifies and vascularizes diameter ~17 mm

Pseudoproptosis 2o to buphthalmos (enlargement of globe secondary to chronically elevated IOP)

Amblyopia commoncommon vsuncommon

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Primary Congenital Glaucoma

A Primary Congenital Glaucoma Natural history if untreated: Progresses to NLP Cornea… Opacifies and vascularizes diameter ~17 mm

Pseudoproptosis 2o to buphthalmos (enlargement of globe secondary to chronically elevated IOP)

Amblyopia common

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Primary Congenital Glaucoma