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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