Hydrocephalus is the medical term used to describe "fluid on the brain," a condition that causes head swelling and increased pressure on the brain. The neuroscience teams at Nemours Children’s Health System include renowned board-certified pediatric neurosurgeons, neurologists and neuroradiologists who are at the forefront of the diagnosis and treatment of congenital hydrocephalus and hydrocephalus in children. We use advanced imaging (including fetal diagnostic testing) and minimally-invasive endoscopic techniques. In fact, our neurosurgeons are internationally-recognized researchers and innovators wholly committed to helping babies and kids of all ages grow up happy and healthy without limitations.
Hydrocephalus in children is a condition that’s typically the result or symptom of another medical problem. But sometimes, there’s no apparent cause (this is referred to as "idiopathic"). We work with perinatal and fetal specialists, neonatologists and pediatric specialists in other medical disciplines who can discover what’s causing hydrocephalus in your child and how best to treat it.
What Is Hydrocephalus in Children?
Hydrocephalus happens when the cerebrospinal fluid ("CSF," the fluid that continuously circulates through the brain and spinal cord) doesn’t drain properly. The condition can be present at birth (this is called "congenital hydrocephalus") or occur overtime in older children (this is "acquired hydrocephalus").
Cerebrospinal fluid is produced in and carried through a series of passages referred to as the "ventricular system." CSF is responsible for floating or providing buoyancy for the brain inside the skull. When there’s a problem with this process, CSF accumulates and causes increased pressure within the skull and head swelling. Fluid can accumulate due to:
- obstruction (the flow of CSF is blocked within the ventricular system)
- improper absorption (the CSF doesn’t make its way out of the ventricular system)
- overproduction (too much CSF is produced and can’t be absorbed fast enough)
The good news is that hydrocephalus is a surgically treatable condition, particularly when discovered early — and many children can go on to live productive lives.
Causes of Congenital Hydrocephalus and Acquired Hydrocephalus in Children
There are many different causes of congenital hydrocephalus and acquired hydrocephalus in children, including:
- complications from premature birth (intraventricular hemorrhage or "bleeding" in the ventricles)
- subarachnoid hemorrhage (bleeding in the space between the brain and the membrane that covers it)
- genetic changes during fetal development (mutations in certain chromosomes)
- congenital conditions (Chiari malformation type II, craniosynostosis)
- neural tube defects (spina bifida, encephocele)
- tumors or cysts (cancerous or noncancerous)
- infections (meningitis)
- head trauma (traumatic brain injury, surgical complications)
Left untreated, hydrocephalus in children can damage the central nervous system and lead to delays in cognitive and physical functioning. That’s why early detection — which we can do while the baby is still in the womb — is important for the best treatment outcomes.
Hydrocephalus Research at Nemours Children’s Health System
Our neurosurgeons are leading the way in bringing new technologies and electrosurgical tools and instrumentation (using high-frequency electricity) to operating rooms around the globe — innovations with applications across medical specialties to help children with many different conditions. We’re also discovering evidence-based protocols to better understand, diagnose and manage the outcomes of neurological diseases and procedures that reduce the effects of neurosurgery.
Why Families Choose Nemours
Families choose Nemours because we’re wholly dedicated to building strong partnerships and providing family-centered care. Our team might know your child’s condition, but only you know your child best. We want you to be an active participant in the decision-making process, which is why we take the time to explain the diagnosis and treatment in understandable terms, present all of the options and invite your input to create the right treatment plan for your child.
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The neurosurgeons at Nemours/Alfred I. duPont Hospital for Children provide expert care for congenital hydrocephalus and hydrocephalus in children of all ages. As part of the Nemours Neuroscience Center, our neurosurgeons lead a multidisciplinary (multiple specialty) treatment center for hydrocephalus in children of all ages, including newborns.
Our team of top pediatric neurosurgeons, neurologists and neuroradiologists is changing lives by researching and offering new ways to help kids heal faster after surgery with fewer side effects. These innovations — such as unique intraoperative image-guided technologies and tools — provide hope-filled futures for children with neurological and many other medical conditions requiring surgery.
Why Choose Nemours/Alfred I. duPont Hospital for Children
Families from throughout the Delaware Valley/Greater Philadelphia, come to Nemours/Alfred I. duPont Hospital for Children in Wilmington, Del., for care for congenital hydrocephalus and hydrocephalus in children of all ages because of our comfortable, high-tech facility and our pediatric specialists and surgeons who are consistently recognized among the very best in clinical care and research.
We’re also home to the only Level IV Neonatal Intensive Care Unit (NICU) in the state of Delaware. That means we provide the highest level of care for critically ill newborns, with onsite cardiothoracic surgery and the state’s only neonatal extracorporeal membrane oxygenation (ECMO) center (which includes ECMO transport from other regional facilities). So babies born with hydrocephalus can get the immediate care they need — when it matters most.
Some neurosurgical procedures typically done with "open surgery," which requires larger "open" incisions, can be performed through tiny incisions an inch or smaller. And that often means shorter hospital stays, quicker recovery times, less pain and discomfort, reduced chance of infection and bleeding, plus much smaller scars. One example of a type of minimally invasive procedure that helps reduce complications after surgery is called "endoscopic third ventriculostomy," or "ETV." Used to treat congenital hydrocephalus and hydrocephalus in children, the surgery was actually pioneered by one of our former neurosurgeons at Nemours/Alfred I. duPont Hospital for Children.
The operation involves making a small hole in the bottom of the third ventricle (one of the spaces in the brain) and letting the extra cerebrospinal fluid flow out of the brain. By using this groundbreaking procedure, we’re helping children avoid having to get a shunt. With a shunt, one end of a flexible tube is surgically implanted into a ventricle of the brain and the other end is placed in the abdominal cavity, chambers of the heart or the space around the lungs where fluid is drained and absorbed by the bloodstream. But an ETV is shown to be more effective (especially over the long-term), with a lower risk of infection and less need for repeat procedures and follow-up than shunting.
Highlights of our pediatric neurosurgery care at Nemours/Alfred I. duPont Hospital for Children include:
- world-class facilities featuring the most advanced diagnostic techniques and equipment available
- academic medical center (which means we’re at the leading edge of treating highly complex neurosurgical conditions)
- fully-equipped neurosurgery operating rooms
- innovative surgical techniques that minimize surgery risks, recovery time and pain
- extensive imaging and navigational equipment for precise instrument guidance
- customized treatment plans based on research and best practices
- care without delay (which means you have seamless, rapid access to specialists)
Your child’s coordinated medical team includes board-certified pediatric neurosurgeons, registered nurses, physician’s assistants and certified health providers (social workers and Child Life specialists) who are experienced in the physiological and psychological changes that occur as children grow and develop. We also work closely with pediatric specialists at the hospital to discover and treat the underlying cause of congenital hydrocephalus and hydrocephalus in children, as well as any complications that may occur due to the condition. Your child’s care team may include pediatric:
- neurologists (specialize in brain and nervous system disorders)
- neonatologists (newborn intensive care specialists)
- interventional radiologists (perform image-guided minimally invasive procedures)
- neuroradiologists (radiologists with expertise in diagnosing nervous system conditions)
- neuropsychologists (specialize in brain functioning)
- fetal/perinatal diagnostic testing and treatment specialists
- geneticists (perform genetic testing and counseling)
- oncologists (cancer doctors)
- ophthalmologists (eye doctors and surgeons)
- otolaryngologists (ear, nose and throat, or "ENT," specialists and surgeons)
- pathologists (diagnose diseases by examining body tissues, fluids and organs)
- radiologists (provide diagnosis through medical imaging)
- surgeons (orthopedic, maxillofacial, plastic and general surgery)
- therapy and rehabilitation specialists (physical, occupational and speech therapists)
- urologists (specialize in bladder and urinary tract diseases and disorders)
- behavioral health professionals (psychologists, psychiatrists)
Congenital Hydrocephalus and Hydrocephalus in Children
Hydrocephalus is a condition caused by a malfunctioning ventricular system, the part of the brain where cerebrospinal fluid (or "CSF").
Responsible for floating or providing buoyancy for the brain inside the skull, CSF can build up, or accumulate, anywhere along the ventricular system, including:
- the choroid plexus (where the cerebral spinal fluid is produced in the ventricles)
- within the passages that connect the ventricles (most commonly the passage called the "cerebral aqueduct")
- after the fluid leaves the passages for absorption (from the cisterns to the bloodstream)
The ventricular system is made up of four fluid-filled ventricles, or "cavities," that are connected by narrow passages. The system includes two lateral ventricles (left and right) that connect to the third ventricle (through a passage called the "cerebral aqueduct"). The fourth ventricle, positioned at the base of the head, is where the fluid drains into either the central spinal canal or reservoirs called "cisterns" located in the subarachnoid space within the lining of the brain (called "meninges"). From here, the CSF bathes the spinal cord and the surface of the brain and then reabsorbs back into circulation.
Fluid accumulation can occur due to obstruction (a blockage) or problems with CSF absorption (CSF doesn’t get to the bloodstream) or overproduction (when too much CSF is made), which lead to increased pressure on the brain and head swelling.
Classification and Types of Hydrocephalus We Treat
Hydrocephalus in children can be congenital (present at birth) or acquired (happens over time). There are many different causes for hydrocephalus, such as developmental problems, infections or other medical conditions, although in some cases it’s not known why hydrocephalus occurs. Left untreated, hydrocephalus can cause significant brain damage and can be fatal. Thankfully, hydrocephalus is a surgically treatable condition and many children go on to lead productive lives.
There are two main classifications of hydrocephalus, including:
- noncommunicating hydrocephalus, or "obstructive hydrocephalus" (when CSF is blocked in the narrow passages that connect the ventricles or in the reservoir at the base of the head within the subarachnoid space)
- communicating hydrocephalus (when cerebrospinal fluid still flows within the ventricular system but backs up after it leaves the ventricles because of problems with absorption)
The most common form — noncommunicating "obstructive" congenital hydrocephalus, also called "aqueductal stenosis" — occurs when there’s narrowing in the passage between the third and fourth ventricles (known as the "aqueduct of Sylvius").
"Congenital hydrocephalus" means the condition is present at birth. Most cases of congenital hydrocephalus are due to some disruption during fetal development and can often be diagnosed while the baby is in the womb. It’s estimated that one child in 500 is born with hydrocephalus.
Problems that cause congenital hydrocephalus can include inherited genetic changes (mutations in certain chromosomes) and underlying medical conditions such as:
- Chiari malformation Type II (structural problems with the cerebellum or back part of the brain, and the opening of the spinal cord)
- craniosynostosis (when the boney plates of the skull close too soon)
- Dandy-Walker syndrome (problems with the cerebellum or back part of the brain and the fluid that surrounds it)
- vein of Galen malformation (type of vascular malformation or arteriovenous fistula, a blood vessel abnormality)
Acquired hydrocephalus in children can occur anytime after the baby is born and even into late childhood, due to environmental or physiological factors, such as:
- complications from prematurity (intraventricular hemorrhage or bleeding)
- subarachnoid hemorrhage (bleeding between the brain and the tissue that covers it)
- tumors or cysts (cancerous or noncancerous)
- infections (meningitis)
- head trauma (traumatic brain injury, surgical complications)
- neural tube defects (spina bifida, encephalocele)
Symptoms of Congenital Hydrocephalus and Hydrocephalus in Children
The main indicator of hydrocephalus in babies and children is large head size. But there are other symptoms that can help us make the appropriate diagnosis and discover what’s causing it.
In addition to large head size, other symptoms of congenital hydrocephalus and acquired hydrocephalus in babies can include:
- feeding problems
- downward looking eyes (called “sun setting”)
Some symptoms of hydrocephalus in older children can include:
- nausea and vomiting
- blurred vision
- trouble walking
- problems with coordination
- loss of bladder control
Our Approach to Caring for Kids With Hydrocephalus
Neurosurgery for hydrocephalus (such as shunt placement or ventriculostomy) is almost always necessary to drain the fluid and reduce the intracranial pressure it causes, it’s important to feel comfortable with your child’s care team. It’s good to know that the neuroscience team at Nemours/Alfred I. duPont Hospital for Children and other specialists your child might see will provide comprehensive, conservative and appropriate care and treatment that begins even before your baby is born (if needed) and lasts through recovery, follow-up and transition to adult health care.
Hydrocephalus Evaluation and Diagnosis in Children and Babies
Using advanced fetal neuroimaging, we’re able to diagnose hydrocephalus in utero (while the baby is still in the womb). But we can’t always tell the severity of the condition or what’s causing it until after the baby is born. During your child’s first visit to Nemours/Alfred I. duPont Hospital for Children, our neurosurgical team will take an extensive medical history, evaluate your child’s symptoms, perform a complete neurological exam, address your child’s immediate needs, and develop a plan for further testing and treatment.
Radiology (Medical Imaging) Tests
- ultrasound (look for signs of hemorrhage)
- CT or "CAT" scan/computed tomography (view the ventricles and other structures)
- MRI/magnetic resonance imaging (look for tumors or malformations at the neck and head)
- CINE, pronounced "sin-nee," MRI (assess CSF flow in the ventricles)
- cranial X-rays (view the skull bones)
Nemours/Alfred I. duPont Hospital for Children’s radiology department has highly technical 3-D (three-dimentional) capabilities. This means better diagnostic imaging with less exposure to radiation.
At Nemours/Alfred I. duPont Hospital for Children, we offer specialized testing and comprehensive, interdisciplinary assessment and consultations of fetal abnormalities like hydrocephalus. Our pediatric neuroradiologists use the most advanced MRI and ultrasound technologies that are gentler and safer (with minimal radiation) for both mom and baby.
If you’re pregnant and may be carrying a baby with a congenital condition, our Nemours Partners for Perinatal Management program will work with you and your referring doctor to arrange for fetal diagnostic testing as well as specialty consultations, if needed. This means we can plan ahead and begin care immediately after your baby is born.
Because hydrocephalus is often a symptom or result of some other problem or condition, your child’s care team may recommend additional diagnostic tests and procedures to understand what’s causing your child’s symptoms. These tests can also help us identify and proactively treat potential effects of the hydrocephalus, and may include:
- ophthalmological exams (evaluate potential problems in and around the eyes)
- otolaryngology exams (examine potential ear, nose and throat disorders)
- hearing assessments
- electroencephalography, or "EEG" (evaluate brain wave/seizure activity)
- polysomnography or "sleep study" (evaluate sleep disorders like sleep apnea)
- ongoing assessments for neurological, intellectual or developmental delays
Once we have all of the information we need, we’ll work together with your family to create a personalized treatment plan.
Surgical Treatments and Procedures We Offer at Nemours/Alfred I. duPont Hospital for Children
The treatment goal for hydrocephalus in babies and children is to, first and foremost, normalize the amount of pressure on the brain to protect healthy development. How this is accomplished depends on what’s causing the increased fluid, where the problem is located within the ventricular system, the severity of the condition, how much pressure is on the brain (and how fast it’s increasing), the age of the child, and other considerations.
When cerebrospinal fluid accumulates quickly due to partial or full blockage between the spinal column and the ventricle, this is an emergency situation. To relieve the pressure as soon as possible (and limit potential brain damage) we may perform a temporary procedure before we can design a long-term plan that fits your child’s specific needs. Depending on the condition, we may perform a:
- ventricular reservoir tap (when a "stopper" is placed in the skull to allow the neurosurgeon to access and draw CSF out of the ventricle using a needle)
- lumbar puncture, or "spinal tap" (when cerebrospinal fluid taken through a needle in the spinal column, particularly in cases of intraventricular hemorrhage or CSF overproduction)
Sometimes a temporary procedure is all that’s needed. But most children with hydrocephalus will require a permanent surgical solution.
Endoscopy is a minimally invasive surgical technique that allows surgeons to access and repair problems using small holes (less than a half inch) and natural openings (such as the nose and mouth). With a high-resolution camera on a small scope (called an "endoscope"), our neurosurgeons can precisely locate the area and use very tiny tools to relieve intracranial pressure, remove an obstruction (such as a tumor or cyst) and often fix ventricles, among many other therapies.
Endoscopic third ventriculostomy (ETV), an alternative to shunting, is a bypass procedure that treats non-communicating (obstructive) hydrocephalus like aqueductal stenosis. It’s often referred to as "third ventriculostomy" because most blockages occur in the passage between the third and fourth ventricle and this is where the bypass is made.
What to Expect
When performing an ETV, the neurosurgeon makes a small perforation or hole in the thin membrane (or "floor") of the third ventricle to allow the CSF to flow naturally to the spinal column and reservoirs at the base of the head. If there’s a problem with CSF production, during the procedure the neurosurgeon may also cauterize or "burn" parts of choroid plexus (where CSF is produced) using a safe, high-frequency electrical current. This combination surgery is referred to as "endoscopic third ventriculostomy with choroid plexus cauterization" (or an "ETV/CPC").
Benefits and Risks
The benefits of any image-guided minimally invasive surgery include better accuracy, faster recovery time, less pain and risk for infection and minimal scarring. Endoscopic third ventriculostomy can eliminate the need for shunting and, therefore, eliminate the need for shunt replacement or revision procedures and also the risk for infection common in shunting systems.
As with any surgical procedure there are risks, including the risk of damaging nearby structures. Your neurosurgeon will help you understand the risks involved so you can make an informed decision about your child’s care.
The standard surgical treatment for hydrocephalus is inserting a shunt to move excessive cerebrospinal fluid from the affected ventricle to another part of the body for absorption. A shunt is a long, flexible tube with catheters on each end and a one-way valve that directs the flow of fluid. The valve can be a fixed pressure type (where CSF flow is regulated using a predetermined setting) or adjustable pressure type (where pressure can be changed, if needed, without and invasive procedure using a special external magnetic device).
The most common shunt system is called the "ventriculoperitoneal (or VP) shunt," which reroutes the CSF into the abdomen. There are other shunt systems named for the area or cavity in which the fluid is delivered, for example ventriculopleural (chest), ventriculoatrial (heart) and the lumboperitonel (spinal column to abdomen).
What to Expect
During the procedure, one end of the tube is inserted into the abdomen (or other cavity) and is threaded under the skin to the affected ventricle. The entire procedure takes about an hour and most kids are in the hospital for a couple of days and back to regular activity within a week or two. After the surgery, your child will require frequent follow-up visits to be sure the device is working properly and monitor signs of infection.
Benefits and Risks
The main benefit of shut placement is that it can be done anywhere along the ventricular system, therefore many types of hydrocephalus can be treated this way.
Although our neurosurgeons use innovative, image-guided intraoperative tools that make these procedures safer with fewer side effects, shunt surgery still carries risk of infection, and many kids need shunt replacement or repositioning surgeries over time. This is why close surveillance is necessary, and also the reason we offer innovative and effective procedures to treat hydrocephalus without the need for shunting.
If your child has another medical condition related to hydrocephalus, such as Chiari malformation, craniosynostosis or a tethered spinal cord, our neurosurgeons work to combine surgeries whenever possible to reduce the number of procedures your child needs.
If your child needs surgery, you can trust our pediatric surgical team at Nemours/Alfred I. duPont Hospital for Children. Our pediatric surgical specialists (surgeons, anesthesiologists and nurses) understand what kids need, psychologically and physically. Our guidebook provides step-by-step instructions and information about what to expect before, during and right after surgery.
Take your child on a pediatric surgery video tour with Sheridan on her surgery day. This fun, kid-friendly video shows children and teens what to expect, step-by-step, if they need an operation at Nemours/Alfred I. duPont Hospital for Children.
Nonsurgical Treatments and Ongoing Therapies
Although neurosurgery is the only way to treat hydrocephalus in children, there are other types of treatments we may prescribe before and after surgery.
Medicine is not a long-term treatment for hydrocephalus, but may temporarily relieve pressure caused by cerebrospinal fluid overproduction and/or absorption problems. Medicines may be used for different reasons, such as to delay surgery for premature infants with intraventricular hemorrhage that may correct itself, or until a baby is older and strong enough for surgery.
Convenient Care and Support for the Entire Family
At Nemours/Alfred I. duPont Hospital for Children, we’re dedicated to providing care in a truly family-centered environment. Our team cares about your physical and emotional wellness, and values your involvement throughout the entire process. We understand that dealing with a chronic or complex medical condition can be difficult for your child and family, but you don’t have to go through it alone.
We provide support services that begin on the very first day we meet and continues throughout your journey. Our hospital support services include:
- Child Life services
- mental health counseling
- patient and family education
- spiritual care
- social work
- support groups
In addition to complete services available at Nemours/Alfred I. duPont Hospital for Children in Wilmington, Del., our Nemours pediatric neurosurgery specialists provide inpatient care at Thomas Jefferson University Hospital for children in Philadelphia.
We also maintain regular outpatient appointment hours at satellite Nemours duPont Pediatrics locations throughout the Delaware Valley:
No matter where your child receives care at Nemours, your medical team (including your primary care provider) can access your child’s medical history, test results and visit notes anytime through our award-winning electronic health record system.
You can also view parts of your child's health records, communicate with your Nemours care team, make appointments, request prescription refills and more through our MyNemours online patient portal.