• See Also
  1. Eye Examination in Children
  2. Glaucoma
  • Epidemiology
  1. Rare pediatric eye disease
  2. Incidence: 1:12,500 live births (0.05% of children)
    1. Sporadic: 80-90%
    2. Familial: 10-20%
  3. Onset under age 1 year
  4. Bilateral eye involvement in 75% cases
  • Pathophysiology
  1. Narrow iridocorneal angle as a developmental defect
    1. Obstructs aqueous fluid outflow from the anterior chamber
    2. Similar to Narrow Angle Glaucoma in adults
  2. Intraocular Pressure abnormally increased
    1. Damages the Optic Nerve
  • Causes
  1. Congenital Cataract surgery
  2. Congenital Infections
    1. TORCH Virus (especially Rubella)
  3. Sturge Weber Syndrome
  4. Retinopathy of Prematurity
  5. Neurofibromatosis
  6. Aniridia
  7. Aphakia
  8. Corticosteroid induced Glaucoma
  9. Uveitis
  10. Eye Trauma
  11. Anterior or posterior segment malformations
  • Symptoms (Presents during first months of life)
  1. Affects one or both eyes
  2. Epiphora (chronic tearing) or other Lacrimation
  3. Eye discomfort
  4. Photophobia (light sensitivity)
  5. Excessive Eyelid squeezing (blepharospasm)
  • Signs
  1. Optic Nerve "cupping"
  2. Buphthalmos
    1. Injected Red Eye
    2. Hazy or "steamy" Cornea (Corneal Opacification)
    3. Corneal edema and globe edema
  • Differential Diagnosis
  1. Nasolacrimal Duct Obstruction
  • Management
  1. Surgical Direct communication made between
    1. Anterior chamber
    2. Aqueous Humor draining system (Schlemm's canal)
  2. Long term Ophthalmology follow-up
  • References
  1. Berson (1987) Ophthalmology Study Guide, AAO, p. 95-110
  2. Essman (1992) Am Fam Physician 46(4):1243-52 [PubMed]
  3. Mills (1999) Am Fam Physician 60(3):907-16 [PubMed]
  4. Russell-Eggitt (1997) J Cataract Refract Surg 23:664-8 [PubMed]
  5. Simon (2001) Am Fam Physician 64(4):623-8 [PubMed]