Congenital glaucoma in babies and children: signs, tests and treatment

Glaucoma is a group of eye conditions in which the optic nerve, which carries visual information from the eye to the brain, is damaged. In most types the damage is linked to pressure inside the eye. Glaucoma UK explains that the eye makes a watery fluid, the aqueous, which drains away through tiny channels in the angle between the cornea and the iris. If the fluid cannot drain, it builds up, the eye pressure rises and the optic nerve is stressed, which may lead to permanent damage to vision.

In congenital glaucoma the drainage system has not formed properly before birth. Moorfields lists developmental (congenital) glaucoma as one of four main types of glaucoma, describing it as rare but potentially serious, usually present at birth or developing shortly afterwards, and caused by an abnormality of the eye. Glaucoma UK adds that glaucomas of this type tend to run in families, although it also says most glaucoma in children is not obviously inherited, so families with a history may wish to speak to a genetic counsellor or a childhood glaucoma specialist.

Glaucoma UK separates childhood glaucoma into primary congenital glaucoma, where the eye has simply not developed normally, and secondary glaucomas that result from another condition or an injury. Secondary glaucoma in children can follow cataract surgery in babies and young children, though the reason is still not well understood. It can also occur when the eye is inflamed, for example in children with a childhood form of arthritis, because inflammatory cells can block the drainage channels.

It also lists conditions in which glaucoma may develop and which therefore need monitoring: Axenfeld-Rieger anomaly, Peters anomaly, aniridia (where the iris is absent or only partly developed) and Sturge-Weber syndrome, in which there is a blood vessel birthmark on the face, often on the forehead, known as a port wine stain. Knowing the type matters because it shapes the surgery and the long-term plan.

Glaucoma UK explains why the eye can look different. A child's sclera, the outer coat of the eye, is much softer and more flexible than an adult's, so when pressure rises the eye may expand. Glaucoma UK lists the following as possible symptoms.

• Large eyes: an enlarged eye is one of the important indicators of raised pressure in a baby or young child.

• Sensitivity to light.

• Cloudy eyes: if pressure is high enough, fluid is pushed into the cornea, making it waterlogged and cloudy. Glaucoma UK says this clouding can clear if pressure is lowered, though it may take several months.

• Watering eyes, with glare from lights and some swelling of the cornea.

• Poor vision and jerky eye movements (nystagmus).

• A squint, where the eye with poorer vision turns inwards or outwards.

These signs are not specific to glaucoma, and watering or light sensitivity in a baby may have other causes. They should still be looked at promptly. The NHS says a GP or optician is the first step for any concerns about vision, and that sudden eye pain, redness, nausea and vomiting or a headache with eye symptoms calls for 999 or A&E. In a baby, an eye that appears large, cloudy or very sensitive to light is a reason to ask for an urgent assessment by a GP, health visitor or eye casualty rather than waiting.

Glaucoma UK adds that after treatment most of these symptoms improve, which is one reason early recognition matters.

Glaucoma UK says that if signs of high eye pressure are seen the doctor may carry out a more detailed examination, and that babies and children usually have to be examined under anaesthetic. It says that if eye pressure is found to be raised, they may carry out surgery at the same time to save further anaesthetic. Generally, a child aged five and over can be examined without anaesthetic.

Moorfields describes glaucoma operations as bringing the pressure in the eye down and stopping damage to the optic nerve.

Moorfields' information for children explains that the aim is to lower pressure so the optic nerve is not damaged further. Eye drops can help, but they have to be used every day and sometimes the pressure is still not low enough to be safe, in which case it is usually better for children to have an operation. In a glaucoma operation the surgeon opens up the blocked drainage channel or makes a new one so that fluid can escape from the eye; sometimes a laser is used to reduce the amount of fluid the eye makes.

Moorfields says children have a general anaesthetic, a patch on the eye which is usually taken off the next day, and eye drops to use at home a few times a day. Young eyes are delicate: there can be problems such as infection or the pressure being too low or too high again, and extra treatment may be needed. Glaucoma operations usually work and bring pressure down, though sometimes children need more than one.

The NHS says glaucoma has no cure but treatment can slow damage and stop vision getting worse, and that sight already lost cannot be restored. RNIB says that most people with glaucoma will retain good vision with the right treatment if it is detected early. Moorfields says the doctor will need to see children in clinic regularly after an operation, and that eye drops are used every day when they are part of the plan. Moorfields advises children who wear glasses to keep wearing them after an operation.

Support is available. Glaucoma UK says that meeting others who care for family members with glaucoma can build confidence, and it supports glaucoma support groups across the UK, with a helpline and an online forum. Ask your ophthalmology team about school support, visual impairment services and certification where relevant.

Childhood glaucoma is looked after on the NHS by paediatric ophthalmologists, often in a few specialist centres to which children are referred from local hospitals. Private paediatric ophthalmology exists and can speed an initial appointment or provide a second opinion; the examination and surgical techniques are the same, and long-term follow-up usually continues with the same specialist team. Ask who will carry out the examinations under anaesthetic, and who to contact if you notice any new signs between visits.