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CONGENITAL GLAUCOMAS
1. Primary
3. In phacomatoses
2. Iriocornea! ys"enesis• A#en$e!%&ie"er anoma!y
• Peters anoma!y
• Aniriia
• St'r"e%(e)er synrome
• Ne'ro$i)romatosis % 1
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Primary con"enita! "!a'coma• 1*1+,+++ )irths, -/ )oys
• Most sporaic % 1+/ a'tosoma! recessi0e
!at iris insertion Conca0e iris insertion
•
A)sence o$ an"!e recess ith iris inserte irect!y into tra)ec'!'m
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C!inica! $eat'res o$ primary con"enita! "!a'coma
rea4s in 5escemet mem)rane
• 5epen on a"e o$ onset• i!atera! in 6/ )'t $re7'ent!y asymmetrica!
Cornea! oeema associate ith!acrimation an photopho)ia
'phtha!mos i$ IOP )ecomes e!e0ateprior to a"e 3 years.
Optic isc c'ppin"
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Mana"ement o$ primary con"enita! "!a'coma
Goniotomy Tra)ec'!otomyMeas'rement o$ IOP ancornea! iameters
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A#en$e! anoma!y• i!atera! )'t asymmetrica!• G!a'coma is 'ncommon
Posterior em)ryoto#on Attache strans o$ iris to posteriorem)ryoto#on
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&ie"er anoma!y• A'tosoma! ominant• i!atera! )'t asymmetrica!
Stroma! hypop!asia an corectopia Ectropion '0eae
'!!%thic4ness iris atrophy An"!e anoma!ies
• G!a'coma in +/
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&ie"er synrome
&ie"er anoma!y 5enta! an $acia! anoma!ies
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Peters anoma!y• Us'a!!y sporaic
• G!a'coma in +/
Cornea! opacity ith iris ahesions
• i!atera! in 8+/
Cornea! opacity ith !entic'!ar ahesions
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Systemic Imp!ications o$ Aniriia
• A'tosoma! ominant
AN%1 % 8/
• Iso!ate
AN%2 9Mi!!er synrome: % 13/
• 5e!etion o$ short arm o$ chromosome 11
• (i!m t'mo'r, "enito'rinary anoma!ies an menta! hanicap
• A'tosoma! recessi0e
• Menta! hanicap an cere)e!!ar ata#ia
AN%3 9Gi!!espie synrome: % 2/
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Si"ns o$ aniriia
S')tota! a)sence
Synechia! an"!e%c!os're"!a'coma in 6/
Occasiona! cataract an !enss')!'#ation
Partia! a)sence
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G!a'coma in St'r"e%(e)er synrome
• G!a'coma in 3+/
• Ipsi!atera! to $acia! haeman"ioma
• 'phtha!mos in -+/
•
Ca'se )y raise episc!era! 0eno'spress're associate ith episc!era!haeman"ioma
• An"!e anoma!y may a!so )e responsi)!e
G!a'coma Ca'ses
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G!a'coma in ne'ro$i)romatosis % 1
• G!a'coma is ipsi!atera! to ne'ro$i)roma
o$ 'pper eye!i in +/ o$ cases
• Ca'se )y an"!e anoma!y ith oritho't ectropion '0eae
• An"!e ne'ro$i)roma may a!so )eresponsi)!e
G!a'coma Ca'ses
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