CONGENITAL GLAUCOMAS 1. Primary 3. In phacomatoses 2. Iridocorneal dysgenesis

: CONGENITAL GLAUCOMAS 1. Primary 3. In phacomatoses 2. Iridocorneal dysgenesis

Created by: Aman Joshi
 Page 1


CONGENITAL GLAUCOMAS 
1.  Primary 
3.  In phacomatoses 
2.  Iridocorneal dysgenesis 
•  Axenfeld-Rieger anomaly 
•  Peters anomaly 
•  Aniridia 
•  Sturge-Weber syndrome 
•  Neurofibromatosis - 1 
Page 2


CONGENITAL GLAUCOMAS 
1.  Primary 
3.  In phacomatoses 
2.  Iridocorneal dysgenesis 
•  Axenfeld-Rieger anomaly 
•  Peters anomaly 
•  Aniridia 
•  Sturge-Weber syndrome 
•  Neurofibromatosis - 1 
Primary congenital glaucoma 
•  1:10,000 births, 65% boys 
•  Most sporadic - 10% autosomal recessive 
Flat iris insertion Concave iris insertion 
•  Absence of angle recess with iris inserted directly into trabeculum 
Page 3


CONGENITAL GLAUCOMAS 
1.  Primary 
3.  In phacomatoses 
2.  Iridocorneal dysgenesis 
•  Axenfeld-Rieger anomaly 
•  Peters anomaly 
•  Aniridia 
•  Sturge-Weber syndrome 
•  Neurofibromatosis - 1 
Primary congenital glaucoma 
•  1:10,000 births, 65% boys 
•  Most sporadic - 10% autosomal recessive 
Flat iris insertion Concave iris insertion 
•  Absence of angle recess with iris inserted directly into trabeculum 
Clinical features of primary congenital glaucoma 
Breaks in Descemet membrane 
•  Depend on age of onset 
•  Bilateral in 75% but frequently asymmetrical 
Corneal oedema associated with  
lacrimation and photophobia 
Buphthalmos if IOP becomes elevated  
prior to age 3 years. 
Optic disc cupping 
Page 4


CONGENITAL GLAUCOMAS 
1.  Primary 
3.  In phacomatoses 
2.  Iridocorneal dysgenesis 
•  Axenfeld-Rieger anomaly 
•  Peters anomaly 
•  Aniridia 
•  Sturge-Weber syndrome 
•  Neurofibromatosis - 1 
Primary congenital glaucoma 
•  1:10,000 births, 65% boys 
•  Most sporadic - 10% autosomal recessive 
Flat iris insertion Concave iris insertion 
•  Absence of angle recess with iris inserted directly into trabeculum 
Clinical features of primary congenital glaucoma 
Breaks in Descemet membrane 
•  Depend on age of onset 
•  Bilateral in 75% but frequently asymmetrical 
Corneal oedema associated with  
lacrimation and photophobia 
Buphthalmos if IOP becomes elevated  
prior to age 3 years. 
Optic disc cupping 
Management of primary congenital glaucoma 
Goniotomy Trabeculotomy 
Measurement of IOP and  
corneal diameters 
Page 5


CONGENITAL GLAUCOMAS 
1.  Primary 
3.  In phacomatoses 
2.  Iridocorneal dysgenesis 
•  Axenfeld-Rieger anomaly 
•  Peters anomaly 
•  Aniridia 
•  Sturge-Weber syndrome 
•  Neurofibromatosis - 1 
Primary congenital glaucoma 
•  1:10,000 births, 65% boys 
•  Most sporadic - 10% autosomal recessive 
Flat iris insertion Concave iris insertion 
•  Absence of angle recess with iris inserted directly into trabeculum 
Clinical features of primary congenital glaucoma 
Breaks in Descemet membrane 
•  Depend on age of onset 
•  Bilateral in 75% but frequently asymmetrical 
Corneal oedema associated with  
lacrimation and photophobia 
Buphthalmos if IOP becomes elevated  
prior to age 3 years. 
Optic disc cupping 
Management of primary congenital glaucoma 
Goniotomy Trabeculotomy 
Measurement of IOP and  
corneal diameters 
Axenfeld anomaly 
•  Bilateral but asymmetrical 
•  Glaucoma is uncommon 
Posterior embryotoxon 
Attached strands of iris to posterior  
embryotoxon 
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