Hydrocephalus

Hydrocephalus is a condition characterised by an abnormal build-up of cerebrospinal fluid (CSF) within the ventricles of the brain. This excess fluid increases pressure within the skull, potentially causing damage to brain tissues. CSF normally flows through the ventricles, providing nutrients to the brain and removing waste. When CSF circulation is disrupted, fluid accumulates, leading to ventricular enlargement. In infants, this may also result in head enlargement due to unfused skull bones. This condition can affect people of all ages, from infants to older adults, and may be congenital (present at birth) or acquired later in life due to injury, disease, or other factors.

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Dr. Teo Kejia
MBBS (Singapore) | MRCS (Edinburgh) | FAMS (Neuro.Surg) | FRCS Ed (Neuro.Surg) | IFAANS

Symptoms of Hydrocephalus

The symptoms of hydrocephalus vary depending on age, cause, and individual factors, presenting differently across age groups.

  • Headache

    Persistent or recurrent headaches often occur due to increased intracranial pressure. These headaches may worsen in the morning or with position changes.

  • Nausea and Vomiting

    Pressure changes within the brain can trigger nausea and vomiting, unrelated to food intake or illness.

  • Vision Problems

    Increased pressure can affect the optic nerves, resulting in blurred or double vision, reduced visual field, or sensitivity to light.

  • Balance and Coordination Issues

    Disruption of brain function may lead to difficulty with balance, coordination, and walking. Affected individuals might experience an unsteady gait or frequent falls.

  • Cognitive Changes

    Memory problems, confusion, irritability, and changes in concentration can develop as brain tissues are compressed by excess fluid.

  • Bladder Control Problems

    Pressure on brain regions controlling bladder function can lead to urinary incontinence or urgency.

  • Enlarged Head In Infants

    Rapid enlargement of the head circumference is a key sign in babies due to unfused skull bones.

  • Bulging Fontanelle in Infants

    The soft spot on an infant’s head may appear tense or bulging when the baby is upright and not crying.

  • Developmental Delays In Infants

    Affected babies may miss developmental milestones or show regression in skills they have previously acquired.

Causes and Risk Factors

Hydrocephalus results from an imbalance in CSF production, flow, or absorption, with various potential causes and risk factors.

  • Congenital Factors

    Genetic abnormalities or developmental disorders, such as spina bifida or aqueductal stenosis, can affect brain structure.

  • Intraventricular Haemorrhage

    Bleeding into the ventricles, particularly common in premature infants, can block CSF pathways or impair absorption.

  • Brain Tumours

    Growths in or around the ventricles can block the flow of CSF, leading to fluid accumulation.

  • Infections

    Meningitis, encephalitis, or other infections can cause inflammation of brain tissues or CSF-filled spaces, disrupting normal fluid dynamics.

  • Head Injury

    Traumatic brain injuries may damage the ventricles or CSF-draining structures, affecting the balance of fluid production and absorption.

  • Age-Related Factors

    Normal pressure hydrocephalus, which typically affects adults over 60, has an unclear cause.

  • Previous Brain Surgery

    Surgical interventions in the brain can sometimes lead to scarring or other changes that affect CSF circulation.

  • Subarachnoid Haemorrhage

    Bleeding in the space surrounding the brain can impair CSF absorption through the arachnoid villi.

Diagnostic Methods

Neurological Examination

A comprehensive assessment of mental status, coordination, reflexes, and sensory function helps evaluate neurological impact and detect specific deficits.

Head Circumference Measurement

For infants and young children, head circumference is tracked on growth charts. Abnormally rapid head growth can be an early sign of hydrocephalus in babies, as their unfused skull bones can expand with increased pressure.

Computed Tomography (CT)

A quick imaging method that provides clear images of the ventricles, identifying enlargement, obstruction, or other abnormalities. CT scans are particularly useful in emergency situations as they can be performed quickly and readily show ventricular size.

Magnetic Resonance Imaging (MRI)

MRI provides detailed images of brain structure and CSF flow dynamics. This technique offers superior soft tissue contrast compared to CT scans and can better visualise the causes of obstruction such as tumours, cysts, or congenital malformations.

Ultrasonography

In infants with open fontanelles, ultrasound can be used to visualise the ventricles through the soft spots in the skull. This non-invasive technique is particularly valuable for monitoring ventricular size in premature babies at risk for intraventricular haemorrhage.

Lumbar Puncture

This procedure measures CSF pressure and allows analysis of fluid composition. In normal pressure hydrocephalus, a large-volume lumbar puncture may temporarily improve symptoms, which can be both diagnostic and predictive of shunt response.

Intracranial Pressure Monitoring

In complex cases, a pressure sensor may be placed within the brain to record pressure changes over time. This continuous monitoring helps distinguish between true hydrocephalus and conditions that mimic it, particularly in cases where imaging findings are ambiguous.

CSF Flow Studies

Specialised MRI techniques visualise CSF flow patterns, identifying obstructions and guiding surgical planning.

Treatment Options

The management of hydrocephalus focuses on restoring normal CSF circulation and relieving pressure on brain tissues.

Non-Surgical Treatment Options

  • Medication: Diuretics such as acetazolamide or furosemide may temporarily reduce CSF production. These medications decrease fluid volume by inhibiting the production mechanisms in the choroid plexus but generally provide only short-term relief and are not definitive solutions for most cases.
  • Serial Lumbar Punctures: For some patients, especially those with normal pressure hydrocephalus, removing CSF through regular lumbar punctures may temporarily alleviate symptoms. This approach may be used while awaiting surgery or in patients who cannot tolerate surgical interventions.
  • Ventricular Tapping: In infants with rapidly enlarging ventricles, direct needle aspiration of CSF from the ventricles may provide temporary relief. This procedure is typically performed as a bridging measure before definitive surgical treatment.
  • Observation: In cases of mild or compensated hydrocephalus, careful monitoring without immediate intervention may be appropriate. Regular imaging studies and neurological assessments track any progression and determine if active treatment becomes necessary.

Surgical Treatment Options

  • Ventriculoperitoneal (VP) Shunt: The most common surgical treatment involves placing a catheter in the ventricle connected to a valve and tubing that diverts excess CSF to the peritoneal cavity where it is absorbed. The valve regulates flow based on pressure differentials, preventing over-drainage.
  • Endoscopic Third Ventriculostomy (ETV): This minimally invasive procedure creates an alternative pathway for CSF flow by making a small opening in the floor of the third ventricle. ETV is particularly effective for obstructive hydrocephalus and allows patients to avoid shunt-related complications, though success rates vary based on cause and patient age.
  • Ventriculoatrial (VA) Shunt: Similar to a VP shunt, it diverts CSF to the right atrium of the heart through a catheter placed in the jugular vein. This approach may be used when the peritoneal cavity is unsuitable due to infection, adhesions, or previous abdominal surgeries.
  • Ventriculopleural Shunt: In this less common variant, CSF is diverted to the pleural space around the lungs. This option may be considered when both peritoneal and atrial placements are contraindicated, though it carries risks of pleural effusion or respiratory compromise.
  • Lumboperitoneal Shunt: Used primarily for communicating hydrocephalus, this shunt drains CSF from the lumbar subarachnoid space rather than directly from the ventricles. The system bypasses the ventricular system entirely, making it suitable for conditions like pseudotumour cerebri.
  • Choroid Plexus Cauterisation: Sometimes combined with ETV, this procedure reduces CSF production by cauterising the choroid plexus tissue. This approach may improve outcomes in infants, particularly in regions with limited resources for shunt maintenance.

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Prevention and Management

While hydrocephalus cannot always be prevented, certain measures can reduce risk. Prenatal care, including folic acid supplements, helps prevent neural tube defects. Wearing helmets during high-risk activities reduces the risk of head injuries that can lead to acquired hydrocephalus. For those with hydrocephalus, regular medical follow-up ensures proper shunt function and neurological monitoring. Recognising signs of shunt malfunction or infection is necessary for timely intervention. Rehabilitation therapies support mobility, communication, and daily functioning, while educational services assist children with learning challenges.

Frequently Asked Questions (FAQ)

How often do shunts need replacement?

Shunts do not have a fixed lifespan and may function effectively for many years. However, they can fail or require adjustments over time due to blockages, infections, or mechanical issues. Regular monitoring helps detect potential problems early, allowing for timely intervention before complications arise.

How does hydrocephalus affect learning and development?

The impact on learning varies. Some children with treated hydrocephalus experience no cognitive difficulties, while others may have challenges with attention, memory, processing speed, or executive function. Early intervention, tailored support, and neuropsychological assessment can help address specific learning needs.

Can hydrocephalus resolve without treatment?

In rare cases, mild hydrocephalus may stabilise if CSF absorption improves. However, most cases require monitoring and treatment. Normal pressure hydrocephalus symptoms may fluctuate, but progressive cases often need surgery to maintain function.

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Dr. Teo Kejia

MBBS (Singapore)|MRCS (Edinburgh)|FAMS (Neuro.Surg)|FRCS Ed (Neuro.Surg)|IFAANS

Dr. Teo’s expertise lies in complex brain tumour surgeries employing advanced techniques like brain mapping and awake brain surgery. He also manages neurovascular conditions, traumatic head injuries and spinal disorders.

  • Dr. Teo graduated from the National University of Singapore with a Bachelor of Medicine and Bachelor of Surgery in 2006. He completed his general surgery training and advanced neurosurgery training at the National University Hospital Singapore. He furthered his education in the United Kingdom, obtaining advanced certifications and completing specialist training in various neurosurgical disciplines.
  • Dr. Teo’s AMDA award facilitated his training at Queen’s Hospital in London, where he specialized in functional neurosurgery, neuro-oncology, neurovascular, skull base, and spinal surgery. He also underwent fellowship training in brain mapping and awake brain surgery at renowned institutions in Italy and France, working with leading experts in the field.

 

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