Hydrocephalus, IIH and normal pressure hydrocephalus

What is it?

Cerebrospinal fluid (CSF) cushions the brain and spinal cord. Problems with its flow or absorption can cause several conditions in adults:

Hydrocephalus: a build-up of CSF within the fluid spaces (ventricles) of the brain, raising pressure. It may be caused by a tumour, bleeding, infection or a narrowing of the fluid pathways, or it may have been present since childhood (including people who had a shunt as a child).

Idiopathic intracranial hypertension (IIH), also called benign raised intracranial pressure: raised pressure of the fluid around the brain without a tumour or blockage. It most often affects women of childbearing age and is associated with weight gain. Although once called 'benign', it can threaten vision.

Normal pressure hydrocephalus (NPH): enlarged ventricles without consistently raised pressure, usually in older adults.

What symptoms can it cause?

• Hydrocephalus: headaches (often worse in the morning), nausea and vomiting, blurred or double vision, drowsiness. People with a shunt may notice symptoms similar to those they had before.
• IIH: headaches, pulsing noises in the ears, brief visual blurring, and loss of vision if untreated.
• NPH: a typical combination of difficulty walking (a slow, shuffling, 'magnetic' gait), bladder urgency or incontinence, and slowing of memory and thinking.

How is it assessed?

• Hydrocephalus: a CT or MRI scan of the brain; for people with a shunt, sometimes shunt X-rays.
• IIH: an eye examination for swelling of the optic nerves (papilloedema) and visual field testing, an MRI and venogram to exclude other causes, and a lumbar puncture to measure the pressure. Care is shared with neurologists and ophthalmologists.
• NPH: MRI scan, walking and memory assessment, and often a test removal of fluid (a lumbar tap test or lumbar drain) to see whether walking improves.

What are the treatment options?

• Hydrocephalus: a shunt, which drains fluid to the abdomen or elsewhere, or, where suitable, endoscopic third ventriculostomy (ETV). Some stable people are monitored.
• IIH: weight loss, medication to reduce CSF production (such as acetazolamide) and treatment of headaches are the main treatments. Surgery, such as a shunt or, through the neurology or radiology teams, venous sinus stenting, is considered when vision is threatened despite medical treatment.
• NPH: a shunt for people who are likely to benefit, usually guided by the tap test.

When might surgery be considered?

Surgery may be advised for hydrocephalus causing symptoms, a shunt that is not working, IIH with threatened vision despite medical treatment, or NPH when tests suggest a good chance of improvement. The likely benefits and risks are discussed with you.

What to expect from recovery and follow-up

After shunt surgery most people stay in hospital for a few days. Shunts can block or become infected, so knowing the warning signs matters. In NPH, walking tends to improve more than memory. Regular follow-up continues, with eye checks in IIH.

When to seek urgent help

If you have a sudden severe headache, a seizure, new weakness, confusion, difficulty speaking, or loss of bladder or bowel control, go to the nearest emergency unit or call 10177. If you are under our care, also call the hospital switchboard and ask for Dr Labuschagne (Netcare Unitas Hospital 012 677 8000; Nelson Mandela Children's Hospital 010 133 0600). If you have a shunt, also seek urgent review for worsening headaches, vomiting, drowsiness or fever. In IIH, any change in vision needs prompt review.

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