Congenital glaucoma

What is congenital glaucoma?

Congenital glaucoma is a rare disease, defined by excessive pressure within the eye, leading to pathological lengthening of the eye (buphthalmia) and irreversible damage to the optic nerve with the risk of vision loss if not treated early. 

The forms of glaucoma affecting children are all the more serious as they occur early in life and the visual prognosis is closely linked to the speed of care. Congenital glaucoma is caused by immaturity or an ocular malformation causing an obstacle to the proper evacuation of the intraocular fluid, thus creating too much pressure inside the eye which causes ocular hypertension.

What are signs of glaucoma in children?

Infants with congenital glaucoma have large, watery, bluish eyes and are very easily dazzled by light.

A complete ophthalmological examination can highlight different anomalies:

• Excessively high pressure within the eye
• An increase in the length of the eye (buphthalmia)
• Corneal opacity
• A pathological excavation of the optic nerve (a hollow forms in its centre following the disappearance of the nerve fibres)
• Strabismus or other associated eye malformations.

What are the risk factors?

There are multiple forms of congenital glaucoma, either isolated or associated with other eye or general malformations. Therefore, a complete eye and general assessment with genetic investigation are necessary. The family history is to be taken into account, the main risk factor for developing early glaucoma being a family history of congenital, infantile or juvenile glaucoma. However, there are many cases where a de novo mutation occurs without the presence of affected relatives in the family.

How can congenital glaucoma be treated in children?

Congenital glaucoma represents a surgical emergency that requires early detection from birth. The clinical diagnosis is often confirmed by specific examinations under general anaesthesia performed in the operating room at the same time as the surgery.

The purpose of this surgery is to create an evacuation path for the pressurised intraocular fluid and thus to slow down or ideally stop the destruction of the optical fibres by compression of the optical nerve. Before and after the procedure, eye drops may be prescribed to reduce eye pressure.

Children suffering from congenital glaucoma often have to undergo several general anaesthetics and surgical procedures before their intraocular pressure can be controlled. In addition, regular monitoring of visual development is essential. It is sometimes necessary to correct an amblyopia: your ophthalmologist may prescribe glasses and/or rehabilitation sessions with an orthoptist. Children with this condition have more fragile eyes and require increased protection and regular monitoring for life.

Find out more about children's visual disorders

Do you want to learn more about a particular visual condition? Discover our pages on myopia, strabismus, and astigmatism. We explain these visual disorders and tell you how to treat them to preserve your children’s visual acuity.