What is Congenital Hydrocephalus?

Congenital hydrocephalus happens when there is an excessive buildup of fluid in the brain. This condition can occur during fetal development and may lead to complications. The causes of congenital hydrocephalus are related to abnormalities in the brain's development.

Understanding the underlying factors can help healthcare providers provide appropriate care and support to individuals affected by this condition. If you or a loved one have been diagnosed with congenital hydrocephalus, discussing treatment options with a medical professional is crucial for managing the condition effectively.

Symptoms of Congenital Hydrocephalus

Symptoms of congenital hydrocephalus include an enlarged head, bulging fontanelle, rapid increase in head circumference, vomiting, irritability, seizures, and poor feeding. Other signs can include downward deviation of the eyes (sunsetting sign), difficulty with balance and coordination, and developmental delays.

If your child shows any of these symptoms, seek medical attention promptly for proper evaluation and treatment.

Headaches: People with congenital hydrocephalus may experience frequent headaches due to increased pressure in the brain.
Nausea and vomiting: Some individuals with congenital hydrocephalus may feel nauseous and vomit, especially in the morning.
Vision problems: Blurred or double vision can occur in individuals with congenital hydrocephalus due to pressure on the optic nerves.
Changes in behavior: Some people with congenital hydrocephalus may exhibit irritability, sleepiness, or other changes in behavior.
Poor coordination: Difficulty with balance and coordination can be a symptom of congenital hydrocephalus, leading to problems with walking or fine motor skills.

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Causes of Congenital Hydrocephalus

Other causes can include brain malformations, bleeding within the brain, or complications during delivery. These factors can disrupt the normal flow or absorption of cerebrospinal fluid in the brain, leading to the accumulation of fluid and subsequent hydrocephalus.

Genetic factors, such as mutations in genes related to brain development, can lead to congenital hydrocephalus in newborns.
Infections during pregnancy, such as toxoplasmosis or rubella, can increase the risk of congenital hydrocephalus in the fetus.
Developmental abnormalities in the brain, such as neural tube defects, can disrupt the flow of cerebrospinal fluid and cause congenital hydrocephalus.
Bleeding in the brain of the fetus, known as intraventricular hemorrhage, can result in congenital hydrocephalus.

Types Of Congenital Hydrocephalus

Congenital hydrocephalus can be categorized into two main types: communicating and non-communicating. Communicating hydrocephalus occurs when cerebrospinal fluid flows normally but is not properly absorbed.

Non-communicating hydrocephalus, on the other hand, happens when there is a blockage that prevents the fluid from flowing freely. Both types can lead to an accumulation of fluid in the brain, causing increased pressure and potential complications.

Communicating Hydrocephalus: In this type, there is a blockage in the flow of cerebrospinal fluid (CSF) within the ventricular system of the brain, leading to an accumulation of fluid in the ventricles.
Non-communicating Hydrocephalus: Non-communicating hydrocephalus occurs when there is a blockage that prevents CSF from flowing between the ventricles, often caused by a structural issue such as a tumor or cyst.
Acquired Hydrocephalus: While most cases of hydrocephalus are congenital, acquired hydrocephalus can develop later in life due to conditions such as infections, bleeding within the brain, or head trauma.

Risk Factors

Risk factors for congenital hydrocephalus include genetic factors such as certain genetic disorders or a family history of hydrocephalus. Maternal factors like infections during pregnancy, inadequate prenatal care, or exposure to certain medications or toxins can increase the risk.

Prematurity and certain birth defects are also associated with a higher likelihood of developing congenital hydrocephalus.

Genetic factors, such as having a family history of hydrocephalus, can increase the risk of congenital hydrocephalus in newborns.
Maternal infections during pregnancy, such as toxoplasmosis or rubella, can be a risk factor for the development of congenital hydrocephalus in the fetus.
Exposure to certain toxins or chemicals during pregnancy, like alcohol or certain medications, may contribute to the risk of congenital hydrocephalus in the baby.
Fetal hemorrhage or bleeding within the brain during development can lead to congenital hydrocephalus as a result of disrupted cerebrospinal fluid flow.

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Diagnosis of Congenital Hydrocephalus

Congenital hydrocephalus is diagnosed through physical exams, imaging tests like ultrasound, CT scans, or MRI, and monitoring symptoms like head growth and developmental delays.

Doctors will assess these findings and may recommend a shunt to drain excess fluid from the brain. Early diagnosis and treatment are crucial to manage this condition effectively and prevent complications.

Ultrasound: An ultrasound of the brain is often used to diagnose congenital hydrocephalus in infants by visualizing the enlarged ventricles.
CT Scan: A computed tomography (CT) scan provides detailed images of the brain and can help identify hydrocephalus by showing the accumulation of cerebrospinal fluid.
MRI: Magnetic resonance imaging (MRI) is another imaging technique that offers detailed views of the brain and can help confirm the presence of hydrocephalus.
Intracranial Pressure Monitoring: In cases where the diagnosis is unclear, measuring the pressure within the brain through intracranial pressure monitoring can help confirm hydrocephalus.

Treatment for Congenital Hydrocephalus

Treatment options for congenital hydrocephalus may include surgical placement of a shunt to redirect excess cerebrospinal fluid, endoscopic third ventriculostomy to create a new pathway for fluid drainage, or occasionally, monitoring without immediate intervention.

Your healthcare provider will determine the most appropriate treatment based on individual factors. Regular follow-ups are essential to monitor progress and make any necessary adjustments.

Surgical intervention, such as the placement of a shunt system, is a common treatment for congenital hydrocephalus. This procedure involves inserting a tube to drain excess cerebrospinal fluid from the brain to another part of the body, relieving pressure.
Endoscopic third ventriculostomy (ETV) is another treatment option for congenital hydrocephalus, where a small hole is made in the floor of the third ventricle to allow excess fluid to flow out of the brain, bypassing the need for a shunt.

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Frequently Asked Questions

How do I recognize the signs of congenital hydrocephalus?

Look for symptoms like an enlarged head, bulging fontanelle, rapid increase in head size, vomiting, irritability, and poor feeding in infants.

What are the recommended do's and don'ts for managing congenital hydrocephalus?

Do: Regular follow-ups, shunt monitoring, symptom observation. Don't: Ignore symptoms like headaches or changes in behavior, delay seeking medical help.