CONGENITAL GLAUCOMAS
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Transcript of CONGENITAL GLAUCOMAS
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CONGENITAL GLAUCOMAS
1. Primary
3. In phacomatoses
2. Iridocorneal dysgenesis• Axenfeld-Rieger anomaly• Peters anomaly• Aniridia
• Sturge-Weber syndrome• Neurofibromatosis - 1
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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
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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
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Management of primary congenital glaucoma
Goniotomy TrabeculotomyMeasurement of IOP and corneal diameters
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Axenfeld anomaly• Bilateral but asymmetrical• Glaucoma is uncommon
Posterior embryotoxon Attached strands of iris to posterior embryotoxon
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Rieger anomaly• Autosomal dominant• Bilateral but asymmetrical
Stromal hypoplasia and corectopia Ectropion uveae
Full-thickness iris atrophy Angle anomalies
• Glaucoma in 50%
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Rieger syndrome
Rieger anomaly Dental and facial anomalies
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Peters anomaly• Usually sporadic
• Glaucoma in 50%
Corneal opacity with iris adhesions
• Bilateral in 80%
Corneal opacity with lenticular adhesions
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Systemic Implications of Aniridia
• Autosomal dominantAN-1 - 85%
• Isolated
AN-2 (Miller syndrome) - 13%
• Deletion of short arm of chromosome 11• Wilm tumour, genitourinary anomalies and mental handicap
• Autosomal recessive• Mental handicap and cerebellar ataxia
AN-3 (Gillespie syndrome) - 2%
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Signs of aniridia
Subtotal absence
Synechial angle-closureglaucoma in 75%
Occasional cataract and lenssubluxation
Partial absence
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Glaucoma in Sturge-Weber syndrome
• Glaucoma in 30%• Ipsilateral to facial haemangioma• Buphthalmos in 60%
• Caused by raised episcleral venous pressure associated with episcleral haemangioma• Angle anomaly may also be responsible
Glaucoma Causes
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Glaucoma in neurofibromatosis - 1
• Glaucoma is ipsilateral to neurofibroma of upper eyelid in 50% of cases
• Caused by angle anomaly with or without ectropion uveae• Angle neurofibroma may also be responsible
Glaucoma Causes