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.

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.

You may also hear your child’s doctor talk about communicating or non-communicating hydrocephalus.

Communicating

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

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.

Symptoms in infants (birth to 1 year*)

Signs and symptoms of hydrocephalus in infants include:

• 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**

Signs and symptoms of hydrocephalus in toddlers (age 1 to 3) and older children (age 4 to 18) include:

• 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

*Age of infants as defined by the World Health Organization (WHO)
**Age of toddlers and older children as defined by the Centers for Disease Control (CDC)

Prenatal Ultrasound

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.

Lab work

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

After the child is born

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.

Neurological exam

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.

Imaging (radiology)

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. Other imaging may include:

• Ultrasound 
• Magnetic resonance imaging 
• Computed tomography 

Medication

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

Surgery

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:

• 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.
• 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.

Follow up care

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.