Hydrocephalus refers to a condition of fluid build-up in the brain, resulting in excess pressure on the brain.^1,2 This pressure can lead to damage to the brain and can even be fatal if left untreated.¹

Hydrocephalus affects approximately 85 per 100,000 individuals worldwide.³

This article will explore how hydrocephalus impacts and influences patient quality of life. Quality of life is a concept that aims to capture and measure the overall well-being of a population or individual.⁴

Understanding the influence of conditions such as hydrocephalus on quality of life is important, to improve patient care and health outcomes, as well as mental health, by focusing on emotional, social, and psychological aspects of patient wellbeing.

Understanding hydrocephalus 

What causes hydrocephalus?

The brain is surrounded by cerebrospinal fluid (CSF). CSF is very important, with three key functions, protecting the brain from damage, removing waste products, and providing key nutrients for the brain to function properly.¹ New CSF is constantly produced, and normally, old fluid is released from the brain and absorbed into blood vessels to prevent excess accumulation of fluid. However, sometimes this goes wrong, resulting in hydrocephalus, where CSF cannot drain into the bloodstream, resulting in excess CSF in chambers of the brain, compressing surrounding tissue and raising the pressure inside the skull.²

Symptoms and causes

General symptoms of hydrocephalus include headaches, sickness, blurred vision, and difficulty walking.¹ There are varying causes, for example, differences in the way the brain develops, all the way through to damage to the brain tissue through an injury or infection.² However, there are three main types of hydrocephalus, each with unique causes and symptoms.

Congenital hydrocephalus 

The baby is born with excess fluid in the brain.

Causes: conditions such as Spina Bifida, or infections developed by the mother during pregnancy.

Symptoms: babies born with hydrocephalus typically show:¹

• An unusually large head
• A thin and shiny scalp with easily visible veins
• A bulging or tense fontanelle
• Downward looking eyes
• Poor feeding, irritability, sickness, sleepiness.

Acquired hydrocephalus

Develops after birth, in children or adults.

Causes: this may be due to serious head injuries or complications of medical conditions such as a brain tumour.

Symptoms:¹

• A headache that may be worse when you wake up in the morning and may be soothed by sitting upright. The headaches may also become continuous, and unaffected by lying down or sitting upright, particularly as the condition worsens.
• Neck pain
• Feeling sick
• Sleepiness
• Changes in mental state (e.g. confusion)
• Blurred or double vision
• Difficulty walking
• Inability to control your bladder or bowel.

Normal pressure hydrocephalus (NPH)

More commonly develops in older patients, over 60.

Causes: can be due to injury/stroke, but sometimes the cause is unknown.

Symptoms: NPH has three sets of distinctive symptoms, affecting:¹

• How you walk (mobility) – typically the first noticeable symptom of NPH is difficulty walking, eventually leading to increasing unsteadiness and the likelihood of falling.
• The urinary system – can include a frequent or urgent need to pee and loss of bladder control
• Mental abilities – can include slow response to questions and situations and slow processing of information

Diagnosis

Brain scans, such as CT and MRI scans can be used to diagnose congenital and acquired hydrocephalus, showing the brain in detail, and any build-up of fluid as well as structural defects that may be causing the problem.¹ To help diagnose NPH, a doctor will look at a patient's ability to walk, mental ability, as well as symptoms affecting the bladder. Diagnosing NPH correctly is important, as it can often be mistaken for Alzheimer's disease. Symptoms of NPH can be relieved by treatment unlike Alzheimer's!

Treatment options

The treatment approach for hydrocephalus focuses on diverting CSF to a place in the body where it can be absorbed, reducing the excess pressure on the brain.⁵ This is important to reduce pressure on the brain, and prevent brain damage!¹ This can be typically achieved through surgical interventions:

Shunt Systems: hydrocephalus is often treated using a shunt (a thin tube), that’s surgically implanted into the brain and drains the excess fluid away, controlling the pressure.⁵

Endoscopic third ventriculostomy (ETV): an alternative to shunt surgery, normally chosen to be used when the build-up of pressure is caused by a blockage to CSF drainage. During ETV surgery, a hole is made in the floor of the brain to allow the trapped fluid to escape to the surface where it can be absorbed. 

Although surgery is the primary treatment option for hydrocephalus, by directly resolving the issue of pressure build-up, there can sometimes be complications. It’s important to discuss possible complications with a surgeon before surgery.

Non-surgical treatments such as medications to reduce CSF production, or management of symptoms through lifestyle changes are useful but do not provide permanent solutions for patients with hydrocephalus. Additionally, many drugs that have been used for hydrocephalus in the past have unpleasant side effects and are not hugely successful.

Quality of life: an overview

Quality of life takes into account positive and negative aspects of an individual’s overall well-being, taking into account elements that contribute to a person’s physical, mental, and social well-being.⁵

Quality of life is complex, taking into account multiple different factors, but can be defined by:

• Physical well-being
• Mental and emotional well-being
• Social well-being
• Environmental well-being
• Economic well-being
• Spiritual well-being
• Overall life satisfaction

Several factors influence the quality of life for patients with hydrocephalus, for example:

• Effectiveness of treatment and frequency of medical complications (e.g. shunt functioning, sometimes shunts can become infected or blocked)
• Comorbidities (e.g. epilepsy, chronic pain, headaches)
• Mobility and physical abilities
• Pain management
• Cognitive impairments (memory, learning difficulties affecting concentration, reasoning, short-term memory, etc)²
• Emotional well-being (anxiety, depression), and access to mental health support
• Social interactions and relationships
• Support systems (family, friends, caregivers)

Several strategies and interventions can be put in place to improve quality of life:

Mental, emotional and social well-being

The symptoms and challenges of hydrocephalus are often invisible to others, which can lead to feelings of isolation and loneliness for those with the condition.⁶ Therefore, it is important to surround yourself with people who can support and understand what you’re going through. Additionally, understanding the condition, as well as methods for management of the condition allows you to feel more in control, and able to make informed decisions regarding your medical treatment and lifestyle.

• Support groups and community resources
• Family and caregiver support
• Counselling and therapy, for example, cognitive behavioural therapy
• Workplace accommodations and advocacy

Physical well-being

• Regular follow-ups and monitoring
• Physical therapy, occupational therapy, speech therapy

Although these can be separated into largely affecting mental and social well-being, versus physical well-being, the two undoubtedly cross over hugely, and so all are important to take into account.

It is also important to remember that the effects of hydrocephalus vary greatly from one individual to another, and so some people will have very few, if any problems, while some may have more.²

Resources

Shine: The Spina Bifida and Hydrocephalus Charity²

• Shine is a charity that can provide details of local support groups and organizations. Their website also contains lots of information about hydrocephalus, as well as details of where you can access support and related events.

The Hydrocephalus Association:⁶

• A nonprofit organization focused on supporting individuals affected by hydrocephalus as well as promoting awareness about the condition, and medical research. 

Summary: hydrocephalus and quality of life

Hydrocephalus is a condition characterized by the accumulation of cerebrospinal fluid (CSF) in the brain, leading to increased pressure and potential brain damage. It affects approximately 85 per 100,000 individuals globally and can be classified into three types: congenital, acquired, and normal pressure hydrocephalus (NPH), each with distinct causes and symptoms. Treatment typically involves surgical interventions like shunt systems or endoscopic third ventriculostomy (ETV) to divert excess CSF.

Quality of life for individuals with hydrocephalus is influenced by various factors, including treatment effectiveness, comorbidities, mobility, cognitive impairments, and emotional well-being. Key aspects of quality of life include physical, mental, social, and economic well-being. Strategies to enhance quality of life include support groups, therapy, and physical rehabilitation. Resources like Shine and the Hydrocephalus Association provide support and information for affected individuals. Understanding and addressing both the physical and emotional impacts of hydrocephalus are crucial for improving overall well-being.