1 Q l Primary Congenital Glaucoma l Evident

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1 Q l Primary Congenital Glaucoma l Evident at birth or within first few

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

2 A l Primary Congenital Glaucoma l Evident at birth or within first few

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

3 Q l Primary Congenital Glaucoma l l Evident at birth or within first

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

4 A l Primary Congenital Glaucoma l l Evident at birth or within first

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

5 Q l Primary Congenital Glaucoma l l l Evident at birth or within

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

6 A l Primary Congenital Glaucoma l l l Evident at birth or within

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

7 Q l Primary Congenital Glaucoma l l l Evident at birth or within

7 Q l Primary Congenital Glaucoma l l l Evident at birth or within first few years 1/10 K live births 2/3 are… …bilateral male vs …male female

8 A l Primary Congenital Glaucoma l l l Evident at birth or within

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

9 Q l Primary Congenital Glaucoma l l l Evident at birth or within

9 Q l Primary Congenital Glaucoma l l l Evident at birth or within first few years 1/10 K live births 2/3 are… …bilateral …male …diagnosed by age 6 months

10 A l Primary Congenital Glaucoma l l l Evident at birth or within

10 A l Primary Congenital Glaucoma l l l Evident at birth or within first few years 1/10 K live births 2/3 are… …bilateral …male …diagnosed by age 6 months

11 Q l Primary Congenital Glaucoma l l l Evident at birth or within

11 Q l Primary Congenital Glaucoma l l l Evident at birth or within first few years 1/10 K live births 2/3 are… …bilateral …male …diagnosed by age 6 months l Associated genetically with POAG? No yes/ no

12 A l Primary Congenital Glaucoma l l l Evident at birth or within

12 A l Primary Congenital Glaucoma l l l Evident at birth or within first few years 1/10 K live births 2/3 are… …bilateral …male …diagnosed by age 6 months l Associated genetically with POAG? No

13 Q l Primary Congenital Glaucoma l l l Evident at birth or within

13 Q l Primary Congenital Glaucoma l l l Evident at birth or within first few years 1/10 K live births 2/3 are… …bilateral …male …diagnosed by age 6 months l l Associated genetically with POAG? No Is secondary to angle dysplasia in the absence of other ocular or systemic abnormalities structure

14 A l Primary Congenital Glaucoma l l l Evident at birth or within

14 A l Primary Congenital Glaucoma l l l Evident at birth or within first few years 1/10 K live births 2/3 are… …bilateral …male …diagnosed by age 6 months l l Associated genetically with POAG? No Is secondary to angle dysplasia in the absence of other ocular or systemic abnormalities

15 Q l Primary Congenital Glaucoma l l l Evident at birth or within

15 Q l Primary Congenital Glaucoma l l l Evident at birth or within first few years 1/10 K live births 2/3 are… …bilateral …male …diagnosed by age 6 months l l Associated genetically with POAG? No association with Is secondary to angle dysplasia in the absence of other ocular or systemic abnormalities What if other ocular and/or systemic abnormalities are present? Would be secondary congenital glaucoma (or confusingly, primary developmental glaucoma)

16 A l Primary Congenital Glaucoma l l l Evident at birth or within

16 A l Primary Congenital Glaucoma l l l Evident at birth or within first few years 1/10 K live births 2/3 are… …bilateral …male …diagnosed by age 6 months l l Associated genetically with POAG? No association with Is secondary to angle dysplasia in the absence of other ocular or systemic abnormalities What if other ocular and/or systemic abnormalities are present? Would be secondary congenital glaucoma (or confusingly, primary developmental glaucoma)

17 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

17 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora 2) Photosensitivity 3) Blepharospasm

18 A/Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

18 A/Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora 2) Photosensitivity 3) Blepharospasm

19 Primary Congenital Glaucoma Epiphora Photophobia/blepharospasm Congenital glaucoma: The triad

19 Primary Congenital Glaucoma Epiphora Photophobia/blepharospasm Congenital glaucoma: The triad

20 A/Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

20 A/Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: three words 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm

21 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

21 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasmia

22 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

22 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § Cloudiness secondary to edema

23 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

23 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § Cloudiness secondary to edema

24 Primary Congenital Glaucoma Congenital glaucoma: Corneal cloudiness

24 Primary Congenital Glaucoma Congenital glaucoma: Corneal cloudiness

25 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

25 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § Cloudiness secondary to edema eponym Haab’s striae

26 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

26 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § Cloudiness secondary to edema Haab’s striae

27 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

27 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l § Haab striae represent what sort of pathology, ie, which corneal structure is damaged, and in what way? Cloudiness secondary edema secondary to corneal stretching Tears into. Descemet’s § Haab’s striae Cornea: What is the mechanism? Mechanical deformation caused by the elevated IOP

28 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

28 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l § Haab striae represent what sort of pathology, ie, which corneal structure is damaged, and in what way? Cloudiness secondary edema secondary to corneal stretching Tears into. Descemet’s § Haab’s striae Cornea: What is the mechanism? Mechanical deformation caused by the elevated IOP

29 Primary Congenital Glaucoma Congenital glaucoma: Haab striae

29 Primary Congenital Glaucoma Congenital glaucoma: Haab striae

30 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

30 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l § Haab striae represent what sort of pathology, ie, which corneal structure is damaged, and in what way? Cloudiness secondary edema secondary to corneal stretching Tears into. Descemet’s § Haab’s striae Cornea: What is the mechanism? Mechanical deformation caused by the elevated IOP

31 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

31 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l § Haab striae represent what sort of pathology, ie, which corneal structure is damaged, and in what way? Cloudiness secondary edema secondary to corneal stretching Tears into. Descemet’s § Haab’s striae Cornea: What is the mechanism? Mechanical deformation caused by the elevated IOP

32 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

32 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11. 5 mmmm

33 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

33 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11. 5 mm

34 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

34 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11. 5 mm IOP: § # Normal IOP: Infancy 10 -12 ; age 7 years ~1#

35 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

35 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11. 5 mm IOP: § Normal IOP: Infancy 10 -12 ; age 7 years ~14

36 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

36 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l 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

37 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

37 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l 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

38 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

38 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l 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

39 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

39 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l 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

40 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

40 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11. 5 mm IOP: § § Normal IOP: Infancy 10 -12 ; age 7 years ~14 General anesthetic(s) that raise IOP: In congenital glaucoma: Ketamine § 30 -40 unanaesthetized § >20 even under anesthesia General anesthetic(s) that lower IOP: Pretty much everything else

41 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

41 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11. 5 mm IOP: § § Normal IOP: Infancy 10 -12 ; age 7 years ~14 General anesthetic(s) that raise IOP: In congenital glaucoma: Ketamine § 30 -40 unanaesthetized § >20 even under anesthesia General anesthetic(s) that lower IOP: Pretty much everything else

42 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

42 Q l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11. 5 mm IOP: § § Normal IOP: Infancy 10 -12 ; age 7 years ~14 General anesthetic(s) that raise IOP: In congenital glaucoma: Ketamine § 30 -40 unanaesthetized § >20 even under anesthesia General anesthetic(s) that lower IOP: Pretty much everything else

43 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of

43 A l Primary Congenital Glaucoma l Clinical presentation l The classic triad of signs/symptoms: 1) Epiphora (common misdiagnosis: Congenital lacrimal sac obstruction) 2) Photosensitivity 3) Blepharospasm l Cornea: § § § l Cloudiness secondary to edema Haab’s striae Horizontal diameter > 11. 5 mm IOP: § § Normal IOP: Infancy 10 -12 ; age 7 years ~14 General anesthetic(s) that raise IOP: In congenital glaucoma: Ketamine § 30 -40 unanaesthetized § >20 even under anesthesia General anesthetic(s) that lower IOP: Pretty much everything else

44 Q l Primary Congenital Glaucoma l Natural history if untreated: l visual acuity

44 Q l Primary Congenital Glaucoma l Natural history if untreated: l visual acuity Progresses to NLP

45 A l Primary Congenital Glaucoma l Natural history if untreated: l Progresses to

45 A l Primary Congenital Glaucoma l Natural history if untreated: l Progresses to NLP (NLP = No light perception)

46 Q l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses

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

47 A l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses

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

48 Primary Congenital Glaucoma Congenital glaucoma: Corneal opacification, vascularization

48 Primary Congenital Glaucoma Congenital glaucoma: Corneal opacification, vascularization

49 Q l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses

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

50 A l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses

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

51 Q l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses

51 Q l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses to NLP Cornea… § § l Opacifies and vascularizes diameter ~17 mm translation: ox’s eye Pseudoproptosis 2 o to buphthalmos (enlargement of globe secondary to chronically elevated IOP)

52 A l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses

52 A l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses to NLP Cornea… § § l Opacifies and vascularizes diameter ~17 mm Pseudoproptosis 2 o to buphthalmos (enlargement of globe secondary to chronically elevated IOP)

53 Primary Congenital Glaucoma Congenital glaucoma: Pseudoproptosis 2 ndry to buphthalmos

53 Primary Congenital Glaucoma Congenital glaucoma: Pseudoproptosis 2 ndry to buphthalmos

54 Q l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses

54 Q l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses to NLP Cornea… § § l l Opacifies and vascularizes diameter ~17 mm Pseudoproptosis 2 o to buphthalmos (enlargement of globe secondary to chronically elevated IOP) common vs Amblyopia common uncommon

55 A l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses

55 A l Primary Congenital Glaucoma l Natural history if untreated: l l Progresses to NLP Cornea… § § l l Opacifies and vascularizes diameter ~17 mm Pseudoproptosis 2 o to buphthalmos (enlargement of globe secondary to chronically elevated IOP) Amblyopia common