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Congenital Glaucoma (childhood)
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Demetrios Vavvas, MD, PhD
Cynthia Grosskreutz, MD, PhD
Louis Pasquale, MD
October 15, 2002

What is congenital Glaucoma?
- Glaucoma is a disease of the optic nerve. The optic nerve is composed FROM the processes of 1 million cells (ganglion cells). In glaucoma, these cells die, leaving the patient with less and less peripheral (surrounding) vision. Usually central, high acuity vision is preserved until the late stages of the disease.

- Primary congenital or infantile glaucoma is glaucoma that develops in the first few years of life and is thought to be the result of abnormal formation of anterior chamber angle (site of draining the eye fluid), causing obstruction of the fluid outflow and elevated eye pressures.

- It may be associated with or without other eye and/or widespread abnormalities.

- Juvenile glaucoma is glaucoma that develops after the 3rd year of life

- In some children secondary glaucoma may develop as a result of trauma or other eye diseases such as inflammatory, neoplastic, hamartomatous, and metabolic diseases.

How frequent is primary congenital glaucoma?
- 1 out of 10,000 babies is affected.

- It affects both eyes in 2/3rds of cases. It affects males twice as often as females and has no racial preference

Does it affect high acuity, central vision?
- Half of the patients will have visual acuity better than 20/50. But 2%-15% of the affected patients may become legally blind (vision less than 20/400 or restricted tunnel vision).

Is it inheritable? If I have it what are the chances my child will have it?
- Genetic inheritance of congenital glaucoma is complex. Several genetic mutations have been discovered in patients with congenital glaucoma. Probably others will be discovered in the future. There are some cases in which there is autosomal recessive inheritance (both parents must have the defective gene) and some in which there is autosomal dominant (only one defective gene is necessary).

- If there is no family history and a parent is affected, then there is a 5% chance the 1st child will be affected and a 5% that the second child will have the disease. If the first two children are affected then each subsequent child has 25% chance of being affected.

What do I usually see in my child?
- The classic three symptoms of congenital glaucoma are tearing (epiphora), bothered by light (photophobia) and eyelid spasm (blepharospasm).

- In addition, an enlarged eye(s) (buphthalmos) may be noted.

- The cornea may appear cloudy and swollen (corneal edema). Horizontal or concentric streaks may be seen. (Haab's striae)

- The optic nerve has an abnormal appearance (cupped). Only your eye doctor can see that.

- The pressure inside the eye is elevated usually in the 30 to 40 mmHg range. Normal is less than 16 mmHg in children. Only your eye doctor can determine that.

- Other diseases can give tearing (tear duct obstruction, conjuctivitis (pink eye), corneal abrasions..), eye/corneal enlargement (X-linked megalocornea, protruding eye (exophthalmos), nearsighted…), cloudy corneas (dystrophies, infections, congenital anomalies, metabolic disorders, skin disorders, birth trauma…) and optic nerve anomalies (colobomas, pits, hypoplasia…). Only your eye doctor can make a definitive diagnosis of congenital glaucoma.

How is congenital glaucoma treated?
- The primary treatment is surgical and much less medical (eye drops).

- Procedures like goniotomy, trabeculotomy and trabeculectomy are performed to increase the outflow of the eye fluid and DROP the pressure inside the eye.

- In cases not responding to the techniques described above, your doctor may implant a special device (valve or tube) to help with the outlflow (draining) of eye fluid and lower the pressure.

- If the above techniques fail your doctor may destroy the production site for the eye fluid (ciliary body) by laser treatment (cyclophotocoagulation) or application of a very cold probe (cryotherapy).

- Medical treatment involves the application of eye drops and/or pills or liquid taken by mouth.

- Medical therapy is used while waiting for the surgery, and it may be used afterwards to supplement.

What is the prognosis?
- There is good prognosis in 80-90% of the patients if caught early.
Some patients may have complications FROM this disease such as: amblyopia (developing a lazy eye), becoming nearsighted, developing retina detachment, astigmatism (irregular cornea) and lens dislocation.

The information and recommendations appearing on these pages are informational only and is not intended to be a basis for diagnosis, treatment or any other clinical application. For specific information concerning your personal medical condition, the DJO suggests that you consult your physician.