Glaucoma

Young child with unilateral congenital glaucoma, left eye. Note the larger size of the left eye (buphthalmos) compared to the right eye. This is a classic sign of congenital, unilateral glaucoma.

Young child with unilateral congenital glaucoma, left eye. Note the larger size of the left eye (buphthalmos) compared to the right eye. This is a classic sign of congenital, unilateral glaucoma.

Congenital glaucoma occurs at birth or shortly thereafter, and approximately 70% of cases are bilateral. It is due to increased intraocular pressure caused by an abnormal outflow or manufacturing of normal eye fluid. Typical intraocular pressure in infant’s is approximately 10 to 15mm Hg., whereas intraocular pressure in infants with congenital glaucoma is often over 30mm Hg. Congenital glaucoma is very different from adult glaucoma. In adult glaucoma, increased intraocular pressure damages the optic nerve function, but the eye size does not change. In infants, however, increased intraocular pressure results in expansion of eye size in addition to damaging the optic nerve. The reason the eye enlarges is that the eye wall in infants is elastic and stretches. As the cornea enlarges, it causes corneal swelling that reduces vision and can lead to amblyopia. After 3 years of age, the eye wall becomes fairly rigid and ocular enlargement secondary to glaucoma does not occur.

Congenital glaucoma, left eye. There is a cloudy cornea, enlarged eye, and strabismus.

Congenital glaucoma, left eye. There is a cloudy cornea, enlarged eye, and strabismus.


Tearing is one of the most common presenting signs of congenital glaucoma, with other symptoms including sensitivity to light, a large cornea, cataract, and corneal clouding. In cases that present with tearing, the diagnosis of congenital glaucoma may be misdiagnosed as a nasolacrimal duct obstruction. In contrast to nasolacrimal duct obstruction where the pupils will have a clear red reflex, congenital glaucoma causes a dull red reflex on the pupils. Juvenile glaucoma, which is extremely rare, has onset after 2-3 years of age with almost no visual signs or symptoms.

The treatment of congenital glaucoma is based on lowering the intraocular pressure in order to prevent optic nerve damage, prevent progressive expansion of the eye, and reduce corneal swelling. Medications have been used to lower intraocular pressures, but they are not effective in most cases, and therapy almost includes surgery to lower the intraocular pressure. The prognosis for congenital glaucoma is fair, with approximately 70% of patients maintaining good, long-term visual acuity.

Unfortunately, those who are in the unfavorable outcome group often go on to blindness. The most important cause of visual loss is attributed to optic nerve damage, which is not reversible. Juvenile glaucoma is more amenable to medical treatment; however, juvenile glaucoma almost always requires surgery.