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