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, neoplastic hamartomatous, 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 lOP in an infant under anesthesia may range from 10 to 15 mm Hg

7. Horizontal measurement of corneal diameter
Normal: 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 Foveal hypoplasia 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