II. Epidemiology

  1. Rare pediatric eye disease
  2. Incidence: 1:12,500 live births
    1. Sporadic: 80-90%
    2. Familial: 10-20%
  3. Onset under age 1 year
  4. Bilateral eyes involved in 75% cases

III. Pathophysiology

  1. Intraocular Pressure abnormally increased
  2. Damages the Optic Nerve

IV. Etiology

  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. Steroid induced
  9. Uveitis

V. Symptoms (presents during first months of life)

  1. Epiphora (chronic tearing) or other Lacrimation
  2. Eye discomfort
  3. Photophobia (light sensitivity)
  4. Excessive Eyelid squeezing (blepharospasm)

VI. Signs

  1. Optic Nerve "cupping"
  2. Buphthalmos
    1. Injected Red Eye
    2. Hazy or "steamy" Cornea (Corneal Opacification)
    3. Swollen eye

VII. Differential Diagnosis

VIII. Management

  1. Surgical Direct communication made between
    1. Anterior chamber
    2. Aqueous Humor draining system (Schlemm's canal)
  2. Long term Ophthalmology follow-up

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