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Syndromes and anomalies associated with glaucoma

Glaucoma in babies and children is rare and is sometimes part of a wider anomaly, syndrome or condition that the child is born with. This section describes three of the most common syndromes or conditions where glaucoma is present.

Sturge Weber syndrome

Sturge Weber syndrome affects the development of blood vessels in the brain, skin and eyes. People with Sturge Weber syndrome have a ‘port-wine’ stain on the face and scalp. 

If the port-wine stain affects the eye lid or conjunctiva (surface of the eye), there is a chance that high pressure in the eye can occur. This increase in pressure can damage the optic nerve and cause glaucoma. It mostly occurs at an early age.

If you have a port-wine stain near an eye, it is important to get your eyes checked regularly for glaucoma.

If the glaucoma is mild, the most common treatment is eye drops. However, in most cases, surgery is needed to improve the drainage of fluid from the eye. Common surgical treatments are trabeculectomy or aqueous shunt.

Axenfeld-Rieger syndrome

What is Axenfeld-Rieger syndrome?

Axenfeld-Rieger syndrome is a spectrum of inherited conditions mainly affecting the front of the eye, also known as the anterior segment. The syndromes affect about one in 200,000 people. Those with Axenfeld-Rieger may have a thin or underdeveloped iris (the coloured part of the eye), and a pupil that is off-centre. They may have extra holes in the iris that can look like multiple pupils. The syndromes can also result in the cornea not developing properly.

Link to glaucoma

About half of the people affected by Axenfeld-Rieger will develop glaucoma because of problems with the development of the trabecular meshwork and drainage channels. These structures are vital in helping to drain aqueous and keep the eye pressure in the normal range. Some people may be born with these syndromes and have glaucoma at birth, but the majority of cases develop in adolescence and early adulthood.

Treatment options

Treatment is similar to other types of chronic open angle glaucoma. In general, treatment starts with pressure lowering eye drops. Should the pressure in the eye remain at a level which causes, or is likely to cause, damage to the field of vision, then surgery will be considered.

The most common operation is called a trabeculectomy and aims to reduce eye pressure by creating a new drainage channel within the eye, but aqueous shunt surgery is also often needed.

Peters’ anomaly

Peters’ anomaly is a disorder of the eye which develops before birth. It affects the development of the structures at the front (anterior chamber) of the eye including the cornea, lens and iris. It results in the central area of the cornea (the clear window in the front of the eye) becoming thin and cloudy.

The iris (coloured part of the eye) may be stuck to the back of the cornea and the lens. In some cases the lens is also cloudy and this may be stuck to the back of the cornea.

In addition there may issues with the drainage channels in the eye and this can result in a rise in eye pressure. If the pressure is high for a long period it may cause damage to the optic nerve (glaucoma).

Most cases of Peters’ anomaly occur without there being any family history of the condition, although it can sometimes run in families. In the majority of cases both eyes are affected, and in about half of these cases the eye pressure is, or may become, raised.

Treatment for Peters’ anomaly

Treatment of Peters’ anomaly may include performing a surgical iridotomy (making a hole in the iris) to improve vision, or sometimes performing a corneal transplant.

In these cases, raised eye pressure is hard to treat with eye drops alone, and surgery is the most common option. Doctors may perform a trabeculectomy or insert aqueous shunt drainage tubes. If surgery is not fully successful, then the use of laser treatment of the ciliary body (the part of the eye which produces fluid) may be advised to reduce the pressure of the eye and control the glaucoma.

This may be described as ‘cyclodiode laser’ or ‘cyclo-photocoagulation’.