Hydrocephalus in Infants and Children
Dealing with your child’s hydrocephalus diagnosis can be overwhelming. It’s common to have countless questions that range from learning more about what hydrocephalus is to what one can expect a child’s experience to be across the life spectrum. Some parents may need medically urgent questions answered and others may be searching for answers to much broader questions.
Hydrocephalus is an abnormal accumulation of fluid in cavities called ventricles inside the brain.
For more detailed information visit, About Hydrocephalus and Cerebrospinal Fluid Dynamics Relevant to Hydrocephalus.
For more detailed information on the special nature of brains with hydrocephalus, watch Brain Matters: Hydrocephalus 101 with Dr. Marion L (Jack) Walker, MD and James P. (Pat) McAllister II, PhD.
How do Infants and Children Develop Hydrocephalus?
There are babies who are born with hydrocephalus. Hydrocephalus that is present at birth is referred to as congenital hydrocephalus. Hydrocephalus that develops later in life in some children, and even in adults, but is caused by a condition that existed at birth, is still considered a form of congenital hydrocephalus. Congenital hydrocephalus is caused by a complex interaction of genetic and environmental factors during fetal development. The most common causes of congenital hydrocephalus are:
- Spina bifida
- Aqueductal stenosis
- Brain malformations
- Head injuries
- Brain tumors
- Intraventricular hemorrhage (brain bleed), commonly as a complication of prematurity
- Meningitis or other infection of the brain or spinal cord
There are other classifications that you might hear when talking to your doctor, particularly around the time of diagnosis. These can include:
- Compensated or arrested hydrocephalus
- Communicating hydrocephalus
- Non-communicating hydrocephalus, also known as obstructive hydrocephalus
For more detailed information on these classifications, visit Types and Causes.
What are the Symptoms of Hydrocephalus in Infants and Children?
In an infant, the most obvious sign of hydrocephalus is an abnormal enlargement of the baby’s head. The soft spot on the top of the head (fontanel) may be tense and bulging. The scalp may appear thin and glistening, and the scalp veins may appear to have unnatural fullness (prominence) as well. When you feel your baby’s head along the suture lines, you may find that the bones are separated. Symptoms to watch for are vomiting, sleepiness, irritability, and a downward deviation of the baby’s eyes (the sun-setting sign where your baby’s eyes are stuck looking down). A baby might also be more irritable or tired than normal.
In children, due to raised intracranial pressure (ICP), often symptoms may include nausea, vomiting, headaches, and vision problems. The child might have problems with balance, delayed development in such areas as walking or talking, or poor coordination. The child may show a change in personality or be unable to concentrate or remember things, and their school performance may decline. Older children may have difficulty waking up and staying awake. While at times the symptoms are very noticeable, other times they can be very subtle and progress so slowly that only in retrospect are they appreciated.
How is Hydrocephalus Diagnosed in Infants and Children?
A diagnosis is made by the history, neurological examination, and the use of brain imaging techniques such as ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI). More tests are may be performed in adults in order to diagnose the condition.
Computed tomography (CT scan) is a reliable procedure for diagnosing and assisting in the management of hydrocephalus. It is a sophisticated technique in which x-ray beams are passed through a patient’s body and pictures of the internal structures, in this case, the brain, are made by the computer. CT scans use higher doses of radiation compared to simpler imaging tests. Improvements in CT equipment, however, has allowed for better images at lower doses for children. These are referred to as low-dose CT scans. You can find accredited imaging centers using low-dose CT scans at acraccredidation.org.
Magnetic Resonance Imaging (MRI), like the CT scan, is a diagnostic technique that produces images of the brain—but unlike CT scanning, MRI does not use x-rays/radiation. Instead, MRI uses radio signals and a very powerful magnet to scan the patient’s body. The signals are then developed into images by the computer. MRI is a painless procedure and has no known side effects. There are two types of MRI scans: The Single Shot Fast Spin Echo (also called a ‘quick brain MRI), which takes about three minutes and rarely requires sedation, is used to assess ventricular size. The full MRI, which takes 30 to 60 minutes and may require sedation, shows more minute details. Before the longer scans are performed, small children may be given a sedative to minimize movement that would cause blurring of the images.
The radiologist will review the scans, write a report, and send the report on to the doctor.
At this point in the diagnostic process, it is important that a neurologist and/or a neurosurgeon become part of your medical team, along with your primary care physician. Their involvement from the diagnostic stage onward is helpful not only in interpreting test results but also in selecting candidates for shunt surgery. They should also discuss the actual surgery and follow-up care, as well as outcomes and possible complications of surgery.
To find a pediatric neurosurgeon and/or neurologist, visit our Physician Directory.
How is Hydrocephalus Treated in Infants and Children?
While there is currently no known way to prevent or cure hydrocephalus, there are three life-saving treatment options that require brain surgery.
The most common treatment for hydrocephalus is a medical device called a shunt, a flexible tube, which is placed in the ventricular system of the brain and connected to a valve. A small hole called a burrhole is made in the skull and the tube is gently guided through the brain to the fluid-filled ventricles. The tube is left in the ventricle to take the cerebrospinal fluid (CSF) to another region of the body, most often the abdominal cavity, or heart, where it can be absorbed. The tube from the brain is connected to a valve that regulates how much fluid leaves the brain.
A second surgical treatment option is called an Endoscopic Third Ventriculostomy (ETV). This is typically used for children over the age of 2 with non-communicating (obstructive) hydrocephalus; hydrocephalus caused by a blockage in the brain like aqueductal stenosis. Similar to the shunt surgery, a small hole is made in the skull and an endoscope is gently guided through the brain into one of the lateral ventricles. With the use of a camera, the endoscope then passes down into the third ventricle and punctures the membrane on the floor of the third ventricle. This creates an alternative pathway for CSF to flow out of the ventricles and around the brain. A shunt is not needed.
Not everyone is a candidate for ETV treatment. Learn more, watch our video.
The third treatment option involves an ETV with the addition of a procedure called choroid plexus cauterization (CPC). This treatment is primarily used in children under 2. Once inside the brain, the neurosurgeon uses a device to burn or cauterize choroid plexus tissue to reduce the amount of fluid being introduced into the ventricles. The choroid plexus is vascular tissue within the ventricles of the brain and is the source of CSF production. The fluid then passes normally through the opening made during the ETV and into the subarachnoid space surrounding the surface of the brain. Not everyone is a candidate for ETV/CPC treatment. ETV/CPC is a very technical surgery and should be performed by a neurosurgeon trained and experienced in the procedure.
With early detection and effective treatment, the outlook for children with hydrocephalus is promising. Many people with hydrocephalus lead normal lives with few limitations. Research and experience show that children with hydrocephalus have excellent opportunities to attain their full potential through programs that stimulate their development.
Management of Hydrocephalus in Infants and Children
Investments in research and advances in technology, as well as diagnostic and treatment protocols, are helping more and more people with hydrocephalus to lead full and active lives. But it is important to understand that life with a shunt or ETV requires you to always be well informed and vigilant about complications. While some people can go 20 years or more without a complication, with a complex condition like hydrocephalus, things can change quickly, therefore it’s critical to be prepared.
Complications of a Shunt and an ETV
When things are going well, it’s easy to put the concerns about hydrocephalus and the complications that come with it out of your mind. However, shunts can malfunction or become infected and this requires a shunt revision, which requires brain surgery. An ETV can close at any time and put an individual in an emergency situation. It is critical to understand the signs and symptoms of shunt failure or the closure of an ETV.
Seeking immediate medical attention can identify a resolvable complication and enable you or your family member to avoid brain damage or even death.
What is the Prognosis for Infants and Children with Hydrocephalus?
Many babies born with hydrocephalus go on to lead full lives, though the road you envisioned for your child might not be the road you journey down together. Hydrocephalus is a complex and unpredictable condition and parents caring and nurturing a baby or child with hydrocephalus need to prepare for the road ahead with careful education and planning. We also recommend a network of other hydrocephalus parents you can turn to for both information and friendship.
So what can you expect? Each child is different and hydrocephalus can present varying degrees of cognitive and physical challenges based on the cause of the hydrocephalus the clinical course. It is not uncommon for babies who have shunts to need repeated shunt revisions in the first weeks, months, or years as their bodies adjust to the condition and treatment. Repeated brain surgeries can impact a baby’s normal development for many reasons including repeated intervention in the brain and time away from being able to grow and learn. Also, in addition to hydrocephalus, some infants may be diagnosed with other medical conditions including but not limited to seizures, cortical visual impairment, cerebral palsy, spina bifida, dandy walker, and Chiari malformation, among others.
Some of the challenges we see in babies as they grow include delays in meeting milestones, sensory issues, problems with chewing and/or swallowing, eye-hand coordination, visual-spatial deficits, delayed speech, and delayed gross motor development like rolling over, crawling, and walking.
Do not despair! Babies with hydrocephalus grow into resilient and determined children. With advances in medical treatments, therapies, educational support services, and an entire industry around toys and games to stimulate the brain and encourage learning, most children grow up to fulfill their dreams of making friends, playing sports, winning academic awards, graduating from high school, and going to college.
For resources on raising a child with hydrocephalus visit, Caring for your Child.
Research and Hydrocephalus
As the leading private funder of hydrocephalus research in the country, we are investing in research to improve outcomes as well as prevent the development of hydrocephalus and, ultimately, find a cure. The Hydrocephalus Clinical Research Network (HCRN) is a network of children’s hospitals that conduct clinical research to improve treatment and care for babies and children with hydrocephalus.
You can be a part of research. Enroll in HAPPIER, the only hydrocephalus patient powered registry in the country, and help our doctors and scientists better serve you!
The Hydrocephalus Association regularly supports our scientists and doctors by assisting in gathering data through surveys as well as through helping enroll patients in current research studies. For more detailed information, visit About Our Research.
Information you can trust! This article was produced by the Hydrocephalus Association, copyright 2021. We would like to thank the following individuals for their valuable contributions and expert input: Marion L. (Jack) Walker, MD, and Cathy Cartwright, DNP, RN-BC, PCNS, FAAN.