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The Congenital ( (Developmental Glaucomas

The Congenital ( (Developmental Glaucomas. Primary congenital (infantile) glaucoma Abnormalities in anterior chamber angle development that obstruct aqueous outflow in the absence of systemic anomalies or other ocular malformation. At birth or within the first few years of life.

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The Congenital ( (Developmental Glaucomas

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  1. The Congenital ((Developmental Glaucomas

  2. Primary congenital (infantile) glaucoma Abnormalities in anterior chamber angle development that obstruct aqueous outflow in the absence of systemic anomalies or other ocular malformation. At birth or within the first few years of life. • Secondary glaucoma in infants Associated with inflammatory, neoplastichamartomatous, metabolic, or other congenital abnormalities. • Primary juvenile glaucoma generally after 3 years of age

  3. Primary congenital glaucoma • 50%-70% of the congenital glaucoma • 1 in 10,000 births • 65% of patients are male • Genetics (usually occurs sporadically , AR with incomplete or variable penetrance ) • Bilateral in about two-thirds

  4. CLINICAL PRESENTATION

  5. Corneal edema

  6. Clinical Examination • Tonometry and intraocular pressure lOP is best measured using topical anesthesia in a cooperative child A complete evaluation of infants requires an examination under anesthesia Most general anesthetic agents and sedatives lower lOP except ketamine Dehydration before GA lower IOP The normal lOPin an infant under anesthesia may range from 10 to 15 mm Hg

  7. Horizontal measurement of corneal diameter • Normal:9.5-10.5 mm in full-term newborns • increases to the adult diameter 11.5 to 12 mm by 2 years of age. • A diameter greater than 12 mm in an infant is highly suggestive of congenital glaucoma.

  8. Gonioscopy

  9. Trabeculodysgenesis Flat anterior iris insertion Concave iris insertion

  10. Ophthalmoscopy • The optic nerve head of an infant without glaucoma is pink, with a small physiologic cup • Cup-to-disc ratios greater than 0.3 are rare in normal infants and must be considered highly suspicious of glaucoma • Asymmetry of optic nerve cupping is also suggestive of glaucoma, particularly differences greater than 0.2 between the two eyes.

  11. Photographic documentation • Ultrasonography • Cycloplegic refraction and treatment of amblyopia

  12. Differential Diagnosis • Excessive tearing

  13. Corneal enlargement X-linked megalocornea Shallow orbits Exophthalmos

  14. Corneal clouding Metabolic disorders Corneal malformations Inflammation Birth trauma Dermoid CHED

  15. Optic nerve abnormalities Pit Coloboma Malformation Hypoplasia

  16. GLAUCOMA ASSOCIATED WITH CONGENITAL ANOMALIES

  17. Aniridia • Bilateral ,2/3 AD,1/3 sporadic • Fovealhypoplasia • Cataract • Corneal opacification • Glaucoma (late childhood or early adulthood)

  18. Axenfeld-Rieger syndrome • Bilateral ,1/2 AD,1/2 sporadic • 50% glaucoma

  19. Peters anomaly • Bilateral 80% • Usually sporadic • 50% glaucoma

  20. lens anomalies

  21. Sturge-Weber syndrome

  22. Glaucoma occurs in 30%-70% of children with this syndrome • Glaucoma more often occurs when the ipsilateral facial hemangioma • The glaucoma that occurs in infancy similar to glaucoma associated with isolated trabeculodysgenesis and responds well to goniotomy. • The glaucoma that appears later in life is probably related to elevated episcleral venous pressure from arteriovenous fistulas.

  23. NEUROFIBROMATOSIS (VON RECKLINGHAUSEN'S DISEASE)

  24. Posterior segment abnormalities: PFV ,ROP , FEVR , iris or ciliary body tumors • Inflammation • Trauma • Corticosteroid use • Intraocular tumors • Congenital rubella • Lowe syndrome

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