Pediatric hydrocephalus

What is pediatric hydrocephalus?

The brain floats in the cerebrospinal fluid (CSF) so that its weight doesn’t cut off blood supply to the lower parts. CSF also serves as a cushion to stop contact of the brain with the hard, boney skull. The fluid’s constant circulation around the brain and spinal cord provides nutrients and removes waste products.

Too much of this fluid leads to an abnormal enlargement of the head. Think of blowing up a balloon in a container. Eventually the balloon gets too big and has nowhere to go. The pressure keeps building up until something breaks.

Hydrocephalus can permanently damage the brain, leading to severe problems with mental and physical development.

What are the different types of pediatric hydrocephalus?

There are two main types in infants and children.

1. Congenital hydrocephalus is present at birth. It can be the result of some diseases the mother had during pregnancy, such as rubella. There can be a hereditary reason passed down in families. In other cases, a failure of the spine to close entirely occurs.

2. Acquired hydrocephalus develops at birth or sometime after. Instead of being present at birth, it comes from injuries or illnesses such as infections of the brain and spinal column (meningitis), bleeding from burst blood vessels in the brain, severe head injuries, brain tumors or cysts. Acquired hydrocephalus can also occur spontaneously.

Communicating or Non-communicating hydrocephalus

• Communicating is when the blockage of CSF happens outside of the brain. It is called “communicating” because CSF can still circulate between the two halves of the brain or ventricles.

• Non-communicating hydrocephalus happens when the flow of CSF is blocked along the narrow passageways that connect the ventricles. This may also be called obstructive hydrocephalus.

What are the signs and symptoms of pediatric hydrocephalus?

Symptoms in infants (birth to 1 year)

• Bulging or tense soft spot (fontanel) on the top of the head

• Irritability

• Poor feeding

• Poor growth 

• Rapid increase in the size of the head

• Seizures

• Sleepiness

• Unusually large head

• Vomiting

Symptoms in toddlers and older children (1-18 years)

• Abnormal enlargement of the head

• Blurry or double vision

• Changes in personality

• Decline in academic performance

• Delay in meeting milestones, or losing skills the child previously had

• Difficulty remaining awake or waking up

• Headache

• Irritability

• Nausea or vomiting

• Poor appetite

• Problems with coordination

• Seizures

• Sleepiness

• Trouble balancing

How is pediatric hydrocephalus diagnosed?

• During pregnancy, hydrocephalus can be caused by the abnormal development of the baby's nervous system or by certain infections of the uterus. Ultrasound screening, which bounces safe sound waves off your baby and other nearby structures, can show birth defects or early increases in the size of the baby’s head.

• Blood tests may also be used to look for specific proteins that signal an increased risk for fetal problems.

• After birth and through childhood, the first step in the diagnosis is a history and physical examination. You will be asked questions about any signs and symptoms you may see. In younger children where the soft spot remains, doctors will look at the area for hardening or bulging.

• Next will be a neurological exam to assess brain function. Doctors will assess your child’s reflexes, muscle strength and tone, sense of touch, vision and eye movements. If your child is old enough, the exam will include hearing tests, and an assessment of his or her mental status and mood.

• Brain imaging tests use X-rays or ultrasound to look for large ventricles caused by extra cerebrospinal fluid. They may also help identify tumors, cysts or other blockages in the brain.

  • Ultrasound 

  • Magnetic resonance imaging (MRI) 

  • Computed tomography (CT)

What are the causes of pediatric hydrocephalus?

What causes hydrocephalus is not well known. It has been linked to genetic abnormalities and things that go wrong during development in the womb. It can be seen as a complication of premature birth such as bleeding inside the brain and diseases including meningitis and traumatic head injury (TBI).

How is pediatric hydrocephalus treated?

There are no medications that adequately treat hydrocephalus. Surgery offers the best treatment for this condition.

Prompt surgery for hydrocephalus reduces brain damage. The needs of your child and the cause of their hydrocephalus determine the type of surgery chosen.

The two general types of surgery are:

1. Shunt - where a flexible tube is placed in the brain that sends cerebrospinal fluid to another part of the body where it can be absorbed. The shunt is implanted under the skin.

2. Endoscopic third ventriculostomy - involves a hole that is made in the wall of one of the fluid chambers in the brain. This gives the cerebrospinal fluid a way to bypass the blockage.

After treatment, your child will need regular check-ups to make sure there are no further problems. Tests are regularly done to check the child's developmental and for intellectual, neurological or physical problems. 

Visiting nurses, social services, support groups and local agencies can provide emotional support and assist with the care of a child with hydrocephalus who has significant brain damage.