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Congenital Hydrocephalus

Nurses face patients of all ages suffering from various health concerns. Learning about them puts you in a better position to achieve positive patient outcomes and deliver patient education to help. When it comes to children, there is one significantly dangerous condition you might come across. Today, we will discuss hydrocephalus in children.

What is Congenital Hydrocephalus?

Hydrocephalus, once called water on the brain, is a term that describes excessive fluid buildup in the cavities (ventricles) of the brain.  Typically, cerebrospinal fluid (CSF) passes through the ventricles. The body generates this fluid to deliver nutrients and remove waste from the brain. The clear fluid also protects the brain from injury by keeping it buoyant and cushioned. The CSF is eventually absorbed in the bloodstream, however, in hydrocephalus, there is an imbalance of CSF production and absorption, causing a buildup within the ventricles, resulting in increased pressure within the brain, which can cause a host of medical problems. this blog will focus on hydrocephalus in children.

Congenital hydrocephalus is caused by a complex interaction of genetic and environmental factors during fetal development. Hydrocephalus that develops later in life but can be attributed to conditions that existed at birth is still considered congenital hydrocephalus. Potential causes of congenital hydrocephalus include:

  • Aqueductal Stenosis – The most common cause of congenital hydrocephalus is an obstruction, occurs when the passageway between the third and fourth ventricles (the aqueduct of Sylvius) is narrowed or blocked.
  • Neural Tube Defect (NTD) – Occurs when the spinal cord is exposed at birth and often leaks CSF, called a myelomeningocele. The condition is often referred to as spina bifida. This kind of defect usually leads to the Chiari II malformation, causing part of the cerebellum and the fourth ventricle to push downward through the opening at the base of the skull into the spinal cord area, blocking CSF flow out of the fourth ventricle.
  • Arachnoid Cysts – Can occur anywhere in the brain but are often located at the back of the brain (posterior fossa) and in the area of the third ventricle. The fluid trapped by the cysts may block the CSF pathways, causing hydrocephalus.
  • Dandy-Walker Syndrome – the fourth ventricle becomes enlarged because its outlets are partly or completely closed, and part of the cerebellum fails to develop. Dandy-Walker syndrome may also be associated with abnormal development in other parts of the brain and sometimes leads to aqueductal stenosis.
  • Chiari Malformation – There are four types of Chiari malformation. Hydrocephalus is most commonly associated with Chiari I and Chiari II malformation. Both types occur at the bottom of the brain stem where the brain and spinal cord join, causing the lowest portion of the brain to be displaced, making it lower than normal and pushing it down into the spinal column.
  • X-Linked Hydrocephalus –A rare genetic disorder that is characterized by narrowing of the aqueduct of Sylvius and severe ventriculomegaly. It is part of a group of conditions linked by mutations in the L1CAM gene. Collectively the conditions are called L1 syndrome, whose symptoms can vary greatly in severity.
Symptoms

Hydrocephalus symptoms can vary in children based on their age.

In infants, hydrocephalus may have symptoms that include:

  • A bulging or tense soft spot (fontanel) on the top of the head
  • A rapid increase in the size of the head
  • An unusually large head
  • Eyes fixed downward (sunsetting of the eyes)
  • Irritability
  • Nausea and vomiting
  • Poor eating
  • Problems with muscle tone and strength
  • Seizures
  • Sleepiness or sluggishness (lethargy)

In toddlers and older children, the condition may have symptoms that include:

  • Abnormal enlargement of a toddler’s head
  • Abnormal eye movements
  • Blurred or double vision
  • Change in personality
  • Decline in school performance
  • Delays or problems with previously acquired skills, such as walking or talking
  • Headache
  • Irritability
  • Loss of bladder control or frequent urination
  • Nausea or vomiting
  • Poor appetite
  • Poor coordination
  • Sleepiness or sluggishness
  • Unstable balance
Effects on Brain Development

In most cases, hydrocephalus progresses over time, which means permanent complications, including intellectual developmental and physical disabilities can occur if left untreated. Hydrocephalus can also be life-threatening if left untreated. While treating hydrocephalus can prevent further brain damage, little can be done to help a patient recover from the damage already caused.

Treatments Options

Presently, there are two methods to treat hydrocephalus:

Endoscopic Third Ventriculostomy (ETV): This is a surgical procedure in which a specialist makes a small hole in the third ventricle floor, allowing the CSF to bypass the obstruction and flow toward the site of resorption around the surface of the brain. Patients two years or older may need this procedure.

Inserting a shunt: The typical treatment for the condition involves the surgical placement of a shunt; a device that channels CSF sway from the brain to another part of the body where it can be absorbed. Most shunt systems consist of three components:

  • A collection catheter situated within the cerebral ventricles
  • A valve mechanism to control how much CFS flows
  • An exit catheter to drain the CSF to another part of the body

Many children suffering from hydrocephalus may have lifelong brain damage. Depending on the progression of the condition, how early it is treated, and any complications that arise, proper care delivery can help reduce the effects of the condition.

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