Hydrocephalus

Hydrocephalus is a chronic and complex neurological condition caused by the buildup of cerebrospinal fluid (CSF) within your brain. CSF has a critical function within your brain and spinal cord – it flows through your brain’s four cavities (ventricles) to protect, provide nutrients and remove waste. When excess CSF accumulates around your brain, it can put harmful pressure on your brain leading to loss of function.

Commonly referred to as “water on the brain,” hydrocephalus has several different types that can be present at birth or acquired later in life from an injury or illness. If caught early, most cases of hydrocephalus can be managed safely with surgery, monitoring and therapy.

Hydrocephalus causes the accumulation of cerebrospinal fluid leading to enlarged ventricles and increased pressure in the brain.

Types of hydrocephalus

Hydrocephalus types are categorized by the cause of the disruption of CSF. Communicating and non-communicating hydrocephalus is most common in infants. Normal pressure hydrocephalus is most common in older adults. Hydrocephalus ex-vacuo occurs after a traumatic brain injury or illness.

The four types of hydrocephalus include:

• Normal pressure hydrocephalus: CSF doesn’t drain properly out of your ventricles, allowing the gradual buildup of CSF with little increase in pressure. This type is slow moving and most common in older adults.
• Communicating hydrocephalus: CSF flows through your ventricles but is blocked after exiting. This type is caused by the thickening of membranes at the base of your brain (arachnoid).
• Non-communicating hydrocephalus: Flow of CSF is blocked due to the narrowing of passages connecting your ventricles (aqueductal stenosis). It’s also known as obstructive hydrocephalus.
• Hydrocephalus ex-vacuo: CSF builds up in your enlarged ventricles, left by shrinking brain tissue caused by a brain injury or illness. Head pressure usually remains normal.

Hydrocephalus causes

A combination of factors – including genetics and illness – may cause hydrocephalus. Hydrocephalus can be congenital (present at birth) or acquired (occurs after birth). A newborn is born with hydrocephalus in 2 out of every 1,000 births in the United States.

Congenital hydrocephalus causes include:

• Cerebral aqueduct stenosis, which narrows the third and fourth ventricles in the brain
• Spina bifida, which affects the brain and spine, causing a neural tube defect (encephalocele)
• Intraventricular hemorrhage (IVH) that causes bleeding in the ventricles, which can result from premature birth complications
• Infections during pregnancy that cause inflammation in fetal brain tissue, such as rubella or syphilis

Causes of acquired hydrocephalus include:

• Stoke or injury, leading to bleeding in the brain
• Bacterial meningitis or mumps, which affect nervous system function
• Brain hemorrhage
• Brain and spinal cord tumors
• Traumatic brain injuries (TBI)

If left untreated, hydrocephalus can be fatal or lead to life-long complications.

Symptoms of hydrocephalus

Hydrocephalus symptoms vary by age group. Symptoms are more common in newborns and infants. The hallmark symptom of an enlarged head in newborns is often referred to as the “fluid on the brain” symptom. Adults usually experience behavioral and cognitive changes.

Hydrocephalus symptoms in newborns and infants:

• Bulging soft spot on the top of the head (fontanel)
• Unusually large head
• Rapid increase in the size of head
• Downward gaze of the eyes (sunsetting eyes)
• Lethargy
• Irritability
• Vomiting
• Muscle tone and strength problems
• Poor appetite

Hydrocephalus symptoms in children:

• Developmental delays
• Unusually large head
• Headaches
• Irritability
• Nausea and vomiting
• Vision problems
• Unusual eye movements
• Balance and coordination problems
• Poor appetite
• Loss of bladder control

Hydrocephalus symptoms in adults:

• Headaches
• Balance and coordination problems
• Nausea and vomiting
• Memory and concentration issues
• Vision problems
• Tiredness
• Loss of bladder control and frequent urination

Hydrocephalus symptoms in older adults:

• Mild dementia
• Loss of bladder control and frequent urination
• Memory loss
• Trouble walking
• Balance and coordination problems

Hydrocephalus diagnosis

Your doctor will first ask about your symptoms and perform a neurological exam. To find the source of hydrocephalus, a variety of tests may be recommended, including:

• An MRI
• A CT scan
• Lumbar puncture (also known as a spinal tap)
• Intracranial pressure monitoring
• A variety of neurological and psychological tests to rule out other conditions

Hydrocephalus treatment options

Our trusted neurocritical care specialists will discuss the best treatment plan, using the latest technologies. Our multidisciplinary team is specially trained in the care of the nervous system.

The most common type of treatment for hydrocephalus in newborns, children and adults is a hydrocephalus shunt. The shunt system is surgically placed in the brain or spinal cord. The system drains the excess CSF fluid using a small, flexible tube to another part of the body like the stomach, where it can be more easily absorbed.

If you receive a shunt, you’ll need to be regularly monitored to prevent any possible complications. Most shunts can be managed externally using a magnet. Possible complications include an infection or break in the shunt system, which can be repaired.

An alternative to a shunt system, endoscopic third ventriculostomy (ETV) is another type of surgery to treat hydrocephalus. This surgery creates a pathway to regain the normal flow of CSF by creating a small hole in the floor of your third ventricle.

In addition to surgical treatments, you may receive rehabilitation to help with any cognitive or physical problems.