Symptoms and Signs of Primary Congenital Glaucoma

Medical Author: John P. Cunha, DO, FACOEP
Medically Reviewed on 11/8/2021

Doctor's Notes on Primary Congenital Glaucoma

Primary congenital glaucoma (PGC) is a condition present at birth that is characterized by an abnormality in the development of the eye's drainage channel (a structure in the eye called the trabecular meshwork) that results in elevated intraocular pressure (high pressure inside the eye). This pressure can damage to the optic nerve (glaucoma) and could result in permanent vision loss if not treated promptly.

Symptoms of primary congenital glaucoma include

  • excessive tearing (epiphora),
  • sensitivity to light (photophobia), and
  • spasms or squeezing of the eyelid (blepharospasm).

Any child who exhibits these main symptoms should be evaluated by an ophthalmologist (eye specialist). Other symptoms of primary congenital glaucoma include the appearance of an enlarged eyeball (buphthalmos) and a cloudy, whitish-gray appearance to the cornea (the clear front layer of the eye).

What Is the Treatment for Primary Congenital Glaucoma?

The mainstay of treatment for primary congenital glaucoma is angle surgery. Medical therapy in the form of pills or eye drops is typically used as a temporary measure prior to surgery and to help decrease corneal clouding and to supplement intraocular pressure (IOP) control after surgery.   

Most medications in the United States have not been approved for children, however, many studies have been performed that inform practitioners on their safety and efficacy in children. Types of medications used in primary congenital glaucoma include:

  • Beta-blockers
  • Carbonic anhydrase inhibitors
  • Topical carbonic anhydrase inhibitors
  • Combination beta-blocker/carbonic anhydrase inhibitor
  • Adrenergic agonists
  • Combination beta-blocker/alpha-2 adrenergic agonists
  • Prostaglandin analogs 

Surgery is the preferred treatment for PCG. There are five major surgical options for PCG:

  • Angle surgery
    • The first procedure of choice
    • The most successful in infantile-onset 
  • PCG Goniotomy
  • Trabeculotomy ab externo (“trabeculotomy”)
  • Combined trabeculotomy and trabeculectomy (CTT)
  • Filtering surgery with glaucoma drainage device implantation

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REFERENCE:

Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.