Ophthalmology Book

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Congenital Glaucoma

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  1. 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
  2. Pathophysiology
    1. Intraocular Pressure abnormally increased
    2. Damages the Optic Nerve
  3. 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
  4. 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)
  5. Signs
    1. Optic Nerve "cupping"
    2. Buphthalmos
      1. Injected red eye
      2. Hazy or "steamy" Cornea (Corneal Opacification)
      3. Swollen eye
  6. Differential Diagnosis
    1. Nasolacrimal duct obstruction
  7. Management
    1. Surgical Direct communication made between
      1. Anterior chamber
      2. Aqueous Humor draining system (Schlemm's canal)
    2. Long term Ophthalmology follow-up
  8. References
    1. Berson (1987) Ophthalmology Study Guide, AAO, p. 95-110
    2. Essman (1992) Am Fam Physician 46(4):1243
    3. Mills (1999) Am Fam Physician 60(3):907
    4. Russell-Eggitt (1997) J {a 6313} Refract Surg 23:664
    5. Simon (2001) Am Fam Physician 64(4):623

Hydrophthalmos (C0020302)

Definition (MSH)Congenital open-angle glaucoma that results from dysgenesis of the angle structures accompanied by increased intraocular pressure and enlargement of the eye. Treatment is both medical and surgical.
Definition (NCI)Abnormally large eyeball.
ConceptsCongenital Abnormality (T019) , Disease or Syndrome (T047)
ICD9365.14, 743.2, 743.20
EnglishBuphthalmia, Buphthalmos, BUPHTMALMOS, Congenital glaucoma, Developmental glaucoma, Glaucoma of childhood, Hydrophthalmos, Infantile glaucoma, Juvenile glaucoma, Megophthalmos, Newborn glaucoma
Spanishbuftalmía, buftalmia, buftalmos, glaucoma congénito, glaucoma congenito, glaucoma de la niñez, glaucoma de la ninez, glaucoma del recién nacido, glaucoma del recien nacido, glaucoma infantil, glaucoma juvenil, hidroftalmía, hidroftalmia
CreditsDerived from the NIH UMLS (Unified Medical Language System)



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