Hydrocephalus: symptoms, causes and treatment

The NHS says damage to the brain from hydrocephalus can cause a wide range of symptoms, including headache, being sick, blurred vision and difficulty walking. The symptoms differ with the type and the age of the person.

In babies born with hydrocephalus, the NHS lists an unusually large head, a thin and shiny scalp with easily visible veins, a bulging or tense fontanelle (the soft spot on top of the head) and downward-looking eyes, and also poor feeding, irritability, vomiting, sleepiness and muscle stiffness and spasms in the lower limbs.

In children or adults with acquired hydrocephalus, the NHS says headaches may be worse on waking because fluid does not drain as well while lying down, and sitting up for a while may improve the headache, but as the condition progresses headaches may become continuous. Other symptoms include neck pain, feeling or being sick (often worse in the morning), sleepiness that can progress to coma, confusion, blurred or double vision, difficulty walking, and loss of bladder or bowel control.

Hydrocephalus is a build-up of fluid in the brain. In the past it was called water on the brain, but the NHS explains the brain is surrounded by cerebrospinal fluid (CSF), which protects the brain, removes waste products and provides nutrients. The brain constantly produces new CSF (about a pint a day) while old fluid is absorbed into the blood vessels; if this is interrupted, CSF can quickly build up and cause pressure.

The NHS describes 3 main types: congenital hydrocephalus (present at birth), acquired hydrocephalus (develops after birth) and normal pressure hydrocephalus (NPH), which usually only develops in older people.

The NHS says hydrocephalus is treated with surgery, and that babies and children or adults usually need prompt treatment to reduce pressure on the brain. If it is not treated, the increase in pressure will cause brain damage. Both congenital and acquired hydrocephalus are treated with either shunt surgery or neuroendoscopy.

In shunt surgery, a thin tube called a shunt is implanted in the brain so excess CSF flows to another part of the body, usually the tummy, where it is absorbed into the blood. A valve inside controls the flow. The operation is done under general anaesthetic by a neurosurgeon, usually takes 1 to 2 hours, and you may stay in hospital for a few days. Further treatment may be needed if the shunt becomes blocked or infected.

An endoscopic third ventriculostomy (ETV) is an alternative: the surgeon makes a hole in the floor of the brain so trapped CSF can escape to the brain's surface and be absorbed. The NHS says ETV is not suitable for everyone, but could be an option when a blockage causes the build-up (obstructive hydrocephalus). It takes around 1 hour, has less risk of infection than shunt surgery, and long-term results are similar, although a blockage can occur months or years later.

The NHS says the causes are poorly understood. Congenital hydrocephalus may be the result of a brain defect restricting the flow of CSF. It can be caused by health conditions such as spina bifida, an infection the mother develops in pregnancy such as mumps or rubella, premature birth with bleeding in the brain, genetic conditions and arachnoid cysts. In many cases the cause is unknown.

Acquired hydrocephalus is usually the result of an injury or illness, including bleeding in the brain such as subarachnoid haemorrhage, blood clots in the brain, meningitis, brain tumours, head injury and stroke. Normal pressure hydrocephalus may follow brain injury, bleeding or infection, but often the cause is not clear and it may be linked to conditions that affect blood flow such as diabetes, heart disease or high cholesterol.

NHS inform says congenital hydrocephalus can sometimes be diagnosed during a routine antenatal ultrasound scan or the newborn physical examination, and other types are diagnosed from a brain scan, normally CT or MRI.

For normal pressure hydrocephalus, the NHS says a checklist is used, assessing how you walk, your mental ability and symptoms that affect bladder control. It is important to diagnose NPH correctly because, unlike Alzheimer's disease, the symptoms can be relieved with treatment. Lumbar drainage or a lumbar infusion test can find out whether shunt surgery will benefit you.

The NHS says many babies born with hydrocephalus have permanent brain damage, which can cause long-term complications such as learning disabilities, speech problems, memory problems, short attention span, problems with organisational skills, vision problems, problems with physical coordination and epilepsy. Children with learning disabilities will need extra support from nursery or school.

Normal pressure hydrocephalus is uncommon and poorly understood, most often affects people over 60, and has three sets of symptoms: changes in walking (such as difficulty taking the first step or shuffling), urinary incontinence and slowed thinking. These develop gradually and can look like Alzheimer's disease. The NHS says not everyone with NPH benefits from shunt surgery.

NHS inform says to phone 999 or go to A&E if you or your child has symptoms of hydrocephalus that have developed very suddenly or rapidly, and to contact your GP practice if you think you or your child might have symptoms. The NHS says to call a GP or use NHS 111 if you think you or your child may have symptoms.

If you or your child has a shunt, NHS inform says to phone your surgical team immediately for signs of infection such as redness and tenderness along the shunt, a high temperature, headache, vomiting, neck stiffness, tummy pain, or irritability or sleepiness in babies. It says a shunt blockage can be very serious and can lead to brain damage, bringing back the symptoms of hydrocephalus.

On the NHS, hydrocephalus is diagnosed from scans and treated by neurosurgical teams, with ongoing support from paediatric or adult specialists. The NHS points to Shine, the spina bifida and hydrocephalus charity, for details of local support groups.

Private neurosurgery is available for adults. Because shunts can block or infect, ask who you should contact quickly if symptoms return and how follow-up scans are arranged.