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Primary Congenital Glaucoma

Primary Congenital Glaucoma Facts

By definition, primary congenital glaucoma (PCG) is present at birth. It is usually diagnosed at birth or shortly thereafter, and most cases are diagnosed during the first year of life. PCG is characterized by an abnormality in the development of the eye's drainage channel (a structure in the eye called the trabecular meshwork). A clear fluid called aqueous continuously flows within the eye from the area behind the iris where it's secreted to the sieve-like trabecular meshwork where it drains back into the bloodstream. Because the trabecular meshwork does not function properly in PCG, the aqueous outflow is impaired, resulting in high pressure inside the eye. This elevated intraocular pressure can result in damage to the optic nerve (glaucoma) and may result in permanent vision loss if not treated promptly.

What Are Causes of Primary Congenital Glaucoma?

Most cases of primary congenital glaucoma occur sporadically. However, some cases are genetic and therefore inherited. In these cases, genetic testing of the parents and siblings of the affected child may be considered.

What Are Primary Congenital Glaucoma Symptoms?

Patient Comments

Three symptoms typically characterize primary congenital glaucoma:

  • Excessive tearing (called epiphora)
  • Sensitivity to light (called photophobia)
  • Spasms or squeezing of the eyelid (called blepharospasm)

If an infant or a young child shows any of these symptoms, parents or caregivers should seek medical care as soon as possible from an ophthalmologist as they may be signs of glaucoma.

What Are Primary Congenital Glaucoma Signs?

Elevated eye pressure can put pressure on the wall of the eye and cause expansion. The eye may appear enlarged. This is called buphthalmos. The cornea (the clear front layer of the eye) can also become cloudy, appearing whitish-grey.

When Should Someone Seek Medical Care for Primary Congenital Glaucoma?

Any child suspected of having glaucoma should be seen as soon as possible by an ophthalmologist. The earlier the diagnosis is made and treatment initiated the better the odds of preventing permanent vision loss. The initial eye examination is performed in the office, and additional testing may be performed in an operating room setting under sedation if warranted. (See Exams and Tests.)

Medically Reviewed by a Doctor on 7/8/2016
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Patient Comments & Reviews

The eMedicineHealth doctors ask about Primary Congenital Glaucoma:

Primary Congenital Glaucoma - Symptoms

What were the symptoms you experienced from primary congenital glaucoma?

Primary Congenital Glaucoma - Treatment

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Primary Congenital Glaucoma - Surgery

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Read What Your Physician is Reading on Medscape

Glaucoma, Primary Congenital »

By definition, primary congenital glaucoma is present at birth; however, its manifestations may not be recognized until infancy or early childhood.

Read More on Medscape Reference »


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