Hydrocephalus

Hydrocephalus, a condition affecting people of all ages, occurs when cerebrospinal fluid accumulates in the brain's cavities. This buildup causes pressure within the skull, leading to various symptoms and potential complications. Understanding hydrocephalus is crucial for early detection and effective management of this complex neurological disorder. 

What is Hydrocephalus? 

Hydrocephalus is a neurological condition characterised by cerebrospinal fluid (CSF) buildup in the brain's ventricles. The ventricles are deep cavities within the brain that generally contain CSF. When excess fluid accumulates, it causes the ventricles to enlarge, putting undue pressure on the surrounding brain tissues. This pressure affects brain function and can lead to various symptoms. 

Hydrocephalus Types 

Hydrocephalus can be classified into several types based on the underlying cause and cerebrospinal fluid (CSF) buildup location: 

• Communicating Hydrocephalus: This type occurs when CSF flow is blocked after it leaves the ventricles. In this type, CSF can still move between the ventricles, which remain open. 
• Non-communicating Hydrocephalus: This is also known as obstructive hydrocephalus, which happens when CSF flow is blocked along one or more narrow passages connecting the ventricles. A common cause is aqueductal stenosis, a narrowing of the aqueduct of Sylvius (small passage between the third and fourth ventricles). 
• Normal Pressure Hydrocephalus (NPH): NPH is a form of communicating hydrocephalus that can affect people of any age but is most common in older adults. It may result from various factors, including subarachnoid haemorrhage, head trauma, infection, tumour, or surgical complications. 
• Other classifications of hydrocephalus: 
  • Congenital Hydrocephalus: It is present at the time of a child's birth and may be caused by events or influences during foetal development or genetic abnormalities. 
  • Acquired Hydrocephalus: This type develops later in life, can affect people of all ages, and may be caused by injury or disease. 
  • Hydrocephalus ex-vacuo is another form that primarily affects adults. It occurs when stroke, degenerative diseases like Alzheimer's disease or other dementias, or traumatic injury cause brain tissue to shrink... 

Symptoms of Hydrocephalus

The symptoms of hydrocephalus vary and depend on the individual's age and the condition's underlying cause. In infants, the most noticeable sign is often an abnormally large head. Parents might notice that their baby's head seems to be growing faster than the rest of their body. 

Other symptoms in infants can include: 

• Bulging of fontanelle (it is the soft spot on the top of the head) 
• Prominent veins on the scalp 
• The downward gaze of the eyes, also known as "sunset eyes." 
• Irritability and excessive crying 
• Poor feeding and vomiting 
• Seizures 
• Sleepiness or lack of alertness 

In grown-up children and adults, the symptoms can be more subtle and develop over time. These may include: 

• Severe and frequent headaches 
• Nausea and vomiting 
• Blurred or double vision 
• Balance problems and difficulty walking 
• Poor coordination and clumsiness 
• Urinary incontinence or a frequent need to urinate 
• Memory problems and difficulty concentrating 
• Personality changes and mood swings 
• Fatigue and sleepiness 

In cases of normal pressure hydrocephalus, which typically affects older adults, the symptoms often follow a specific pattern known as the "triad": 

• Difficulty walking, often described as a "magnetic" or shuffling gait 
• Loss of bladder control or a frequent, urgent need to urinate 
• Cognitive decline, such as memory problems, slowed thinking, and difficulty with problem-solving 

Causes of Hydrocephalus 

Hydrocephalus causes can be categorised into congenital and acquired factors, each contributing to cerebrospinal fluid (CSF) buildup in the brain's ventricles.  Congenital causes of hydrocephalus are present at birth and may result from genetic factors or developmental issues during pregnancy. Some common congenital reasons for hydrocephalus include: 

• Neural tube defects 
• Aqueductal stenosis 
• Dandy-Walker syndrome 
• Chiari malformation 

Acquired causes develop after birth and can affect individuals of any age. These causes include: 

• Brain tumours that obstruct CSF flow 
• Head injuries or trauma 
• Infections of the central nervous system, like meningitis 
• Bleeding in the brain (intraventricular haemorrhage) 
• Stroke or other vascular problems 

The underlying mechanism of hydrocephalus involves an imbalance in the production, flow, or absorption of CSF. This imbalance can occur due to: 

• Overproduction of CSF (rare) 
• Obstruction of CSF circulation within the ventricular system 
• Impaired absorption of CSF into the bloodstream 

Risk Factors for Hydrocephalus 

Several factors can increase the possibility of developing hydrocephalus, such as: 

• Children with a family history of neural tube defects, such as spina bifida 
• Premature birth 
• Maternal infections such as toxoplasmosis, cytomegalovirus, rubella, and syphilis during pregnancy 
• Brain tumours, particularly those located near the ventricles 
• Traumatic brain injuries, especially those resulting in bleeding within the brain 
• Certain infections affecting the central nervous system, such as meningitis or cysticercosis, 
• Age is a risk factor for normal pressure hydrocephalus (NPH). 

Complications of Hydrocephalus 

One of the primary complications of hydrocephalus is brain damage. The increased pressure within the skull can compress delicate brain tissues, impairing function in affected areas. Other complications are: 

• Hydrocephalus can also significantly impact motor function, resulting in problems with coordination, balance, and fine motor skills. In severe cases, this can lead to partial or complete paralysis. 
• Vision problems 
• Seizures 
• Emotional and behavioural changes 
• In infants and young children, hydrocephalus can have severe consequences on development. 
• Without proper treatment, hydrocephalus can be life-threatening. 

Diagnosis 

The diagnostic process typically begins with thoroughly assessing the patient's hydrocephalus symptoms. 

• A general physical exam and a detailed neurological evaluation follow this. 
• During the neurological exam, the doctor assesses muscle condition, movement, sensory abilities, and overall neurological function. The specific tests conducted may vary based on the patient's age. 
• Imaging studies provide detailed views of the brain structure and can reveal enlarged ventricles caused by excess cerebrospinal fluid. The most commonly used imaging techniques include: 
  • Ultrasound 
  • Magnetic Resonance Imaging (MRI) 
  • Computed Tomography (CT) scan 
• In addition to these imaging tests, doctors may use other diagnostic tools, particularly for adult patients. These may include: 
  • Spinal tap (lumbar puncture) 
  • Intracranial pressure monitoring (ICP) 
  • Fundoscopic exam 

Treatment for Hydrocephalus 

Hydrocephalus treatment aims to reduce the hydrocephalus pressure on the brain caused by excess CSF. The surgical procedures used to cure this condition are: 

• Shunt Surgery: It is the most common hydrocephalus therapy. It involves implanting a thin tube called a shunt in the brain, which allows excess CSF to flow from the brain to another part of the body. 
• Endoscopic third ventriculostomy (ETV): In this procedure, surgeons create a hole in the floor of the brain to allow trapped CSF to escape to the brain's surface for absorption. 

When to See a Doctor 

Emergency medical intervention should be sought immediately if an infant or toddler exhibits: 

• Continuous crying in high-pitch 
• Problems with sucking or feeding 
• Recurrent vomiting with no apparent cause 
• Seizures 

For older children, symptoms that warrant medical attention include: 

• Persistent headaches 
• Nausea and vomiting 
• Vision problems 
• Developmental delays 

Adults experiencing the following symptoms should consult a doctor: 

• Chronic headaches 
• Nausea and vomiting 
• Vision problems 
• Excessive fatigue 
• Problems with balance and coordination 
• Short-term memory loss 
• Difficulties walking (gait disturbances) 
• Mild dementia 
• Forgetfulness 
• Loss of bladder control 

Prevention of Hydrocephalus 

While it's not possible to completely prevent hydrocephalus, there are ways people can lower the risk of developing this condition, such as: 

• Expectant mothers should attend regular check-ups throughout their pregnancy. 
• Vaccinations against illnesses and infections that have been associated with hydrocephalus. 
• Using appropriate safety equipment, such as helmets, during activities like cycling 
• Always wear a seatbelt while in a vehicle. 
• Car seats, strollers, and other infant gear should meet safety standards. 

Conclusion 

While prevention is not always possible, You can take several preventive steps to reduce the risk of developing hydrocephalus. Regular prenatal care, vaccinations, and safety precautions play a vital role in decreasing the risks of this condition. For those diagnosed with hydrocephalus, ongoing medical care and regular monitoring are musts for the best possible outcomes and quality of life. 

FAQ's

1. Can hydrocephalus go away on its own? 

Hydrocephalus does not go away on its own and requires special treatment. Without treatment, hydrocephalus can lead to severe complications and even be fatal. 

2. Is hydrocephalus treatable? 

Yes, hydrocephalus is treatable. The condition can be managed with surgery. 

3. Why do cerebrospinal fluid levels increase? 

An increase in CSF levels can occur due to the following: 

• Overproduction of CSF (rare) 
• Obstruction of CSF circulation within the ventricular system 
• Impaired absorption of CSF into the bloodstream 

4. Is hydrocephalus painful? 

Hydrocephalus can cause pain, particularly headaches. The pain experienced varies and depends on the type of hydrocephalus and the 
age of the person affected. 

Dr. Parag Aradhey

MBBS, DNB (MED), DNB (Neurology), MNAMS
Ganga CARE Hospital Limited, Nagpur

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