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    31congenital Glaucomas

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    Shari' Si Wahyu
    1. This document discusses various types of congenital glaucoma including primary congenital glaucoma, iridocorneal dysgenesis syndromes like Axenfeld-Rieger anomaly and Peters anomaly, and glaucoma associated with phacomatoses like Sturge-Weber syndrome and neurofibromatosis type 1. 2. Primary congenital glaucoma is most common, affects about 1 in 10,000 births, and can cause corneal edema, buphthalmos, and optic disc cupping if elevated pressure is not treated early through procedures like goniotomy or trabeculotomy. 3. Syndromes like Axenfeld-Rieger anomaly,

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    31congenital Glaucomas

    Uploaded by

    Shari' Si Wahyu
    72 views12 pages
    1. This document discusses various types of congenital glaucoma including primary congenital glaucoma, iridocorneal dysgenesis syndromes like Axenfeld-Rieger anomaly and Peters anomaly, and glaucoma associated with phacomatoses like Sturge-Weber syndrome and neurofibromatosis type 1. 2. Primary congenital glaucoma is most common, affects about 1 in 10,000 births, and can cause corneal edema, buphthalmos, and optic disc cupping if elevated pressure is not treated early through procedures like goniotomy or trabeculotomy. 3. Syndromes like Axenfeld-Rieger anomaly,

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    31Congenital Glaucomas

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    1. This document discusses various types of congenital glaucoma including primary congenital glaucoma, iridocorneal dysgenesis syndromes like Axenfeld-Rieger anomaly and Peters anomaly, and glaucoma associated with phacomatoses like Sturge-Weber syndrome and neurofibromatosis type 1. 2. Primary congenital glaucoma is most common, affects about 1 in 10,000 births, and can cause corneal edema, buphthalmos, and optic disc cupping if elevated pressure is not treated early through procedures like goniotomy or trabeculotomy. 3. Syndromes like Axenfeld-Rieger anomaly,

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    72 views12 pages

    31congenital Glaucomas

    Uploaded by

    Shari' Si Wahyu
    1. This document discusses various types of congenital glaucoma including primary congenital glaucoma, iridocorneal dysgenesis syndromes like Axenfeld-Rieger anomaly and Peters anomaly, and glaucoma associated with phacomatoses like Sturge-Weber syndrome and neurofibromatosis type 1. 2. Primary congenital glaucoma is most common, affects about 1 in 10,000 births, and can cause corneal edema, buphthalmos, and optic disc cupping if elevated pressure is not treated early through procedures like goniotomy or trabeculotomy. 3. Syndromes like Axenfeld-Rieger anomaly,

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    of 12

    CONGENITAL GLAUCOMAS

    1. Primary

    2. Iridocorneal dysgenesis
    Axenfeld-Rieger anomaly
    Peters anomaly
    Aniridia

    3. In phacomatoses
    Sturge-Weber syndrome
    Neurofibromatosis - 1
    Primary congenital glaucoma
    1:10,000 births, 65% boys
    Most sporadic - 10% autosomal recessive
    Absence of angle recess with iris inserted directly into trabeculum

    Flat iris insertion Concave iris insertion


    Clinical features of primary congenital glaucoma
    Depend on age of onset
    Bilateral in 75% but frequently asymmetrical

    Corneal oedema associated with Buphthalmos if IOP becomes elevated


    lacrimation and photophobia prior to age 3 years.

    Breaks in Descemet membrane Optic disc cupping


    Management of primary congenital glaucoma

    Measurement of IOP and


    corneal diameters Goniotomy Trabeculotomy
    Axenfeld anomaly
    Bilateral but asymmetrical
    Glaucoma is uncommon

    Posterior embryotoxon Attached strands of iris to posterior


    embryotoxon
    Rieger anomaly
    Autosomal dominant
    Bilateral but asymmetrical
    Glaucoma in 50%

    Stromal hypoplasia and corectopia Ectropion uveae

    Full-thickness iris atrophy Angle anomalies


    Rieger syndrome

    Rieger anomaly Dental and facial anomalies


    Peters anomaly
    Usually sporadic
    Bilateral in 80%
    Glaucoma in 50%

    Corneal opacity with iris adhesions Corneal opacity with lenticular adhesions
    Systemic Implications of Aniridia
    AN-1 - 85%
    Autosomal dominant
    Isolated

    AN-2 (Miller syndrome) - 13%


    Deletion of short arm of chromosome 11
    Wilm tumour, genitourinary anomalies and mental handicap

    AN-3 (Gillespie syndrome) - 2%


    Autosomal recessive
    Mental handicap and cerebellar ataxia
    Signs of aniridia

    Partial absence Subtotal absence

    Synechial angle-closure Occasional cataract and lens


    glaucoma in 75% subluxation
    Glaucoma in Sturge-Weber syndrome
    Glaucoma Causes

    Glaucoma in 30% Caused by raised episcleral venous


    Ipsilateral to facial haemangioma pressure associated with episcleral
    haemangioma
    Buphthalmos in 60% Angle anomaly may also be responsible
    Glaucoma in neurofibromatosis - 1
    Glaucoma Causes

    Glaucoma is ipsilateral to neurofibroma Caused by angle anomaly with or


    without ectropion uveae
    of upper eyelid in 50% of cases
    Angle neurofibroma may also be
    responsible

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