Hydrocephalus

What is Hydrocephalus?

The term hydrocephalus (commonly referred to as "water on the brain") comes from two words:

• “Hydro” – meaning water
• “Cephalus” – derived from the Greek word "kephalos" referring to the head

Hydrocephalus is a condition in which cerebrospinal fluid (CSF) accumulates in excessive amounts within the brain's ventricles (fluid-filled cavities in the brain). This buildup disrupts brain function due to:

• Increased intracranial pressure
• Compression of structures connecting different brain regions

Functions of Cerebrospinal Fluid (CSF):

1. Delivers nutrients to brain cells and removes metabolic waste
2. Acts as a cushion, absorbing shocks and impacts
3. Regulates pressure changes by flowing between the brain and spinal cord

Hydrocephalus can develop at any age, but it is most commonly diagnosed in infants or adults over 60 years of age.

Causes of Hydrocephalus

Hydrocephalus may be congenital or associated with various developmental anomalies, such as:

• Spina bifida (a congenital spinal and nervous system defect)
• Encephalocele (a brain herniation disorder)

In adults, the most common causes of hydrocephalus include:

• Intracranial hemorrhages
• Head and brain injuries
• Brain tumors
• Infections (e.g., meningitis)

Normal pressure hydrocephalus (NPH), which primarily affects elderly patients, has unknown causes.

Symptoms of Hydrocephalus

Hydrocephalus symptoms vary depending on the patient's age and the type of hydrocephalus. The following symptom descriptions are based on data from the Hydrocephalus Association (MD, USA).

Symptoms in Newborns:

• Enlarged head size
• Bulging fontanelle (soft spot)
• Prominent large-caliber veins beneath the thin scalp
• Excessive sleepiness
• Seizures
• Poor appetite

Symptoms in Infants and Children:

• Head enlargement
• Headaches, nausea, vomiting, fever
• Blurred or double vision
• Balance and coordination difficulties
• Irritability, excessive sleepiness, difficulty waking up
• Delayed walking or speech development
• Difficulty concentrating
• Seizures
• Loss of appetite

If hydrocephalus progresses slowly, children may develop cognitive impairments and struggle academically. Therefore, children with significant learning difficulties should be evaluated to rule out structural brain damage.

Symptoms in Adults:

• Headaches
• Excessive sleepiness, difficulty waking
• Coordination and balance impairments
• Urgent need to urinate or urinary incontinence
• Blurred or double vision
• Personality changes

Symptoms in Older Adults:

• Coordination and balance issues
• Shuffling gait with wide steps
• Memory problems
• Personality changes (dementia-like symptoms)
• Frequent, urgent need to urinate or urinary incontinence

Among elderly patients, normal pressure hydrocephalus (NPH) is the most common form of hydrocephalus.

• CSF accumulates in the brain's ventricular system, causing ventricular enlargement.
• NPH rarely develops before age 60.
• Many cases are misdiagnosed as Alzheimer's disease or other forms of dementia, leading to underdiagnosis and undertreatment.
• Even in developed countries, only 10–20% of patients with NPH are correctly diagnosed and treated.

How Is Hydrocephalus Diagnosed?

Patients experiencing symptoms require additional imaging tests:

• Ultrasound – commonly used in infants and young children
• MRI or CT scans – used in adults

MRI is the gold standard for diagnosing potential causes of hydrocephalus.

Treatment Options for Hydrocephalus

Treatment varies depending on the cause of hydrocephalus.

Surgical Approaches:

1. Tumor removal – If hydrocephalus is caused by a tumor blocking CSF pathways, surgical tumor removal restores CSF flow.

2. Shunt Surgery – Used when hydrocephalus cannot be corrected directly.

   • Ventriculoperitoneal shunting – Redirects CSF from brain ventricles to the abdomen.

   • Ventriculoatrial shunting – Redirects CSF to the right atrium of the heart via venous circulation.

3. Endoscopic Surgery (Ventriculocisternostomy) – Performed in occlusive hydrocephalus, where CSF pathways are narrowed or obstructed.

   • Instead of implanting a shunt system, a minimally invasive procedure creates a new bypass for CSF flow using a small camera and instruments.

If ventricular enlargement is linked to congenital brain abnormalities and causes no symptoms, surgery may not be necessary.

Recovery After Surgery

Shunt procedures (ventriculoperitoneal and ventriculoatrial shunting) are performed using two or three small skin incisions (1–3 cm each).

Hospital stay:

• 1 night to 2 days, depending on the patient's preoperative condition.
• Post-surgery MRI or CT scan is performed the next day.
• Rehabilitation is necessary to accelerate recovery and reduce functional impairments.
• Follow-up appointment with the surgeon occurs 4–6 weeks after surgery.

What Is the Prognosis for Hydrocephalus?

The prognosis depends on:

• Cause of hydrocephalus
• Severity and duration of symptoms
• Treatment approach

Recovery Possibilities:

• Some patients recover quickly and resume normal activities soon after surgery.
• Others experience slow and prolonged recovery, especially in cases involving dementia or structural brain changes.

Early diagnosis and treatment significantly improve success rates.

Unfortunately, individual patient outcomes are difficult to predict. Even if shunt surgery leads to rapid symptom relief, lifelong risks of shunt complications or dysfunction remain.

Shunt Complication Risk:

While some patients require additional surgeries, most shunt-related issues can be effectively managed.

When to Seek Immediate Medical Attention After Surgery

Urgent medical evaluation is required if any of the following occur after hydrocephalus surgery:

• Redness, swelling, or pain around the implanted shunt tube and valve
• Irritability or excessive sleepiness
• Nausea, vomiting, headaches, or double vision
• Fever or elevated body temperature
• Abdominal pain (for patients who underwent ventriculoperitoneal shunting)
• Return of preoperative symptoms