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 years ‘age’ period of time
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 few years 1/10 K live births #
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 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 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 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 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 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 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 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 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 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 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 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 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 signs/symptoms: 1) Epiphora 2) Photosensitivity 3) Blepharospasm
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
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 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 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 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
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 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 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 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
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 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 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 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 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 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 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 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 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 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 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 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 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 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 Progresses to NLP
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 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 to NLP Cornea… § Opacifies and vascularizes
48 Primary Congenital Glaucoma Congenital glaucoma: Corneal opacification, vascularization
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 to NLP Cornea… § § Opacifies and vascularizes diameter ~17 mm
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 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
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 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
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