Hydrocephalus, CSF disorders and spina bifida

Service detail

Hydrocephalus, commonly known as ‘water on the brain’, is a condition that can affect all age groups from babies to the elderly. In hydrocephalus, there is an accumulation of the normal brain fluid within the fluid cavities (ventricles) of the brain, leading to an increase in pressure within the brain. Untreated, hydrocephalus can be life-threatening. 

Hydrocephalus affects one in every 500 births, and is one of the most common developmental disabilities in children. The condition also affects older children and adults of all ages and can be secondary to a variety of causes including intracranial tumours, haemorrhage and infection. 

Hydrocephalus is also associated with spina bifida, in which the spinal column does not develop normally during pregnancy. 

Cerebrospinal fluid (CSF) is produced at a constant rate by the brain. It flows through the brain within the normal fluid cavities (ventricles). It circulates over the brain and the spinal cord and finally is reabsorbed by structures called arachnoid villi into the blood stream. 

CSF functions:           

• Buoyancy to protect the brain and spinal cord
• Removal of metabolic waste
  
  
  

Causes of hydrocephalus

There are many causes of hydrocephalus, including:

• Tumours e.g. glioma, metastasis, colloid cysts
• Congenital e.g. spina bifida, Chiari malformation
• Haemorrhage e.g. neonatal haemorrhage, subarachnoid haemorrhage
• Infection e.g. meningitis
• Others e.g. idiopathic intracranial hypertension (IIH), normal pressure hydrocephalus (NPH).

Treatment

Treatment aims to re-establish the CSF circulation. This can be achieved by:

• Removing the cause e.g. tumours
• Inserting a shunt system
• Endoscopic third venticulostomy (ETV)

Shunt systems

Shunts divert fluid through a silicone tube from the ventricles to another part of the body. Depending on the end target the shunt system is named accordingly:

• Ventriculo-peritoneal (abdomen)
• Ventriculo-atrial (heart)
• Ventriculo-pleural (chest cavity)

The amount of CSF diversion is regulated by a valve that forms part of the shunt:

• Fixed valves - permit CSF diversion at a fixed brain pressure
• Programmable valves - CSF diversion can be adjusted according to the needs of the patients

Endoscopic third ventriculostomy (ETV)

With the help of an endoscope a small perforation on the floor of the third ventricle is created to re-establish the CSF circulation by an alternative route. In certain cases this prevents the need to have a permanent shunt system implanted.

Normal pressure hydrocephalus (NPH)

This condition is most commonly seen in patients over 55 years old, with enlarged ventricles and a characteristic triad of symptoms:

• Short wide based ataxic walking
• Memory problems, progressive cognition decline
• Urinary incontinence (patients are not aware of the sensation to urinate).

These patients may benefit from insertion of a shunt. At The Walton Centre potential candidates are carefully assessed using a trial of CSF drainage via a lumbar drain. Patients who clinically improve are offered a permanent shunt.

Idiopathic intracranial hypertension (IIH), also known as benign intracranial hypertension (BIH)

With this condition patients have raised intracranial pressure and often have headaches, visual disturbances or both. IIH tends to occur more frequently in young women with a high body weight. Untreated patients can develop blindness.

The purpose of the treatment is to alleviate the headache and to preserve and protect visual disturbances. Treatment options include:

• Repeated lumbar puncture (spinal tap) and diuretics
• Insertion of a permanent shunt system

Walton Centre hydrocephalus surgeons

Mr Neil Buxton

Mr Michael Jenkinson

Mr Ajay Sinha

Mr Farouk Olubajo

Miss Catherine McMahon

 

Support for patients with hydrocephalus

The Hydrocephalus Nurse Specialist for The Walton Centre provides ongoing support and review for patients who for various reasons, have required CSF diversion surgery following a diagnosis of hydrocephalus. This aims to offer and provide a high standard of care to this particular patient group.

The role involves seeing patients at all stages of their patient journey, from pre-operative assessment, through admission and as follow up in outpatients, on a regular basis. The nurse specialist ensures information is available to patients, both during and following admission to enable them to manage their condition as independently as possible.

To contact the hydrocephalus nurse specialist ring 0151 525 3611 via bleep 5340, during the hours of 9am-5pm Monday to Friday.

Our hydrocephalus services

• Our hydrocephalus team is made up of a dedicated group of medical and nursing staff specialising in supporting patients with hydrocephalus and CSF flow disorders
• We provide rapid access clinics on Monday, Wednesday and Friday, run by our hydrocephalus specialists, as well as urgent ward assessment Monday to Friday
• We have rapid access to ophthalmology services (led by consultant ophthalmologist Miss Carmel Noonan) for patients requiring urgent visual assessment
• We provide transitional care for hydrocephalus patients moving from paediatric care to adult services
• We use the latest image-guided surgery technique to ensure accurate placement of shunts into the ventricles