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CONGENITAL GLAUCOMAS

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.

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CONGENITAL GLAUCOMAS

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

  2. 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

  3. Clinical features of primary congenital glaucoma • 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. Breaks in Descemet membrane Optic disc cupping

  4. Management of primary congenital glaucoma Measurement of IOP and corneal diameters Goniotomy Trabeculotomy

  5. Axenfeld anomaly • Bilateral but asymmetrical • Glaucoma is uncommon Attached strands of iris to posterior embryotoxon Posterior embryotoxon

  6. Rieger anomaly • Autosomal dominant • Bilateral but asymmetrical • Glaucoma in 50% Stromal hypoplasia and corectopia Ectropion uveae Angle anomalies Full-thickness iris atrophy

  7. Rieger syndrome Rieger anomaly Dental and facial anomalies

  8. Peters anomaly • Usually sporadic • Bilateral in 80% • Glaucoma in 50% Corneal opacity with iris adhesions Corneal opacity with lenticular adhesions

  9. 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

  10. Signs of aniridia Subtotal absence Partial absence Occasional cataract and lens subluxation Synechial angle-closure glaucoma in 75%

  11. Glaucoma in Sturge-Weber syndrome Glaucoma Causes • Glaucoma in 30% • Caused by raised episcleral venous • pressure associated with episcleral • haemangioma • Ipsilateral to facial haemangioma • Buphthalmos in 60% • Angle anomaly may also be responsible

  12. Glaucoma in neurofibromatosis - 1 Glaucoma Causes • Caused by angle anomaly with or • without ectropion uveae • Glaucoma is ipsilateral to neurofibroma • of upper eyelid in 50% of cases • Angle neurofibroma may also be • responsible

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