There are several spaces inside and around the brain filled with fluid, called cerebrospinal fluid (CSF). CSF is a clear fluid that surrounds and cushions the brain and spinal cord. CSF is constantly being produced, and must flow through the fluid spaces in order to be reabsorbed. Sometimes, too much fluid can build up inside the spaces, and this a called Hydrocephalus. Hydrocephalus can be caused by a blockage in the flow or reabsorption of CSF, causing extra fluid to build up. On rare occasions, hydrocephalus is caused by the body making too much CSF. The extra buildup of CSF can cause the skull to become larger, can increase the pressure inside the brain, and can be harmful to brain development.
Hydrocephalus can be congenital, meaning that it happens before your baby is born. Some reasons for too much cerebral spinal fluid (CSF) in the brain include:
Hydrocephalus can affect the brain and your baby’s development. The extent of the problem depends on the severity of the hydrocephalus, and the presence of other problems such as:
Hydrocephalus may be found during routine prenatal ultrasound but sometimes it is discovered after the baby is born. If your doctor suspects a problem, additional tests may be ordered. These may include:
Some congenital conditions are caused by changes in DNA, or a baby’s genetic make-up. The doctors may suggest genetic testing to find out more about your baby’s DNA.
If a test finds you are carrying a baby with hydrocephalus, you will continue to receive care from your obstetrician. Your obstetrician may refer you to the Ohio Fetal Medicine Collaborative (OFMC), which will help you connect with a team of fetal medicine experts.
There, you will meet with a multidisciplinary team including neonatologists (doctors who specialize in treating newborns), pediatric neurologists (doctors who specialize in treating children with brain abnormalities) pediatric neurosurgeons (doctors who perform brain surgery), and nurses to learn more about your baby’s care after he or she is born. Nurse coordinators will help answer your questions and concerns, guide you through your pregnancy, and prepare you for what to expect.
Your doctor will watch your pregnancy closely. You will deliver your baby at a hospital that is ready to care for high-risk babies like yours. You should discuss your plans for labor and delivery with your obstetrician. Contact your doctor right away if you have any concerns.
Your baby may need to be monitored for these symptoms after birth.
If pressure in the baby’s head builds, symptoms will get worse and may include:
At the delivery hospital, a neonatologist (a doctor who specializes in treating newborns) will manage the care of your baby. The neonatologist will assess your baby’s breathing, heart rate, measure the head size, and examine the baby for other problems. Your baby’s head may appear larger than normal.
Your baby may have several tests to confirm hydrocephalus and look for the cause. Additional pictures of your baby’s brain will be taken by ultrasound and/or magnetic resonance imaging (MRI), to determine how severe the hydrocephalus is, and whether there are any additional problems with the brain. Additional genetic testing may be needed as well.
Depending on your baby’s condition:
If treatment is needed, the goal is to reduce the pressure in the baby’s head and to drain the cerebral spinal fluid (CSF).
Surgery may be needed to treat hydrocephalus. There are two main types of surgical procedures for managing hydrocephalus. Your neurosurgeon with discuss these with you and decide which is best for your baby.
Ventriculo-Peritoneal Shunt (VP Shunt): The neurosurgeon places a mechanical shunting device called a ventriculoperitoneal shunt (VP shunt) in your baby’s head. This shunt helps to drain the extra fluid inside of your baby’s brain and redirects the fluid to another part of
your baby’s body such as the abdomen, where it can be absorbed.
Endoscopic Third Ventriculostomy (ETV) with Chorid Plexus Cauterization (CPC): An alternative to shunt placement for the treatment of hydrocephalus in some patients is endoscopic third ventriculostomy (ETV). The procedure creates an additional opening that allows CSF flow out of fluid spaces in the brain through an opening made deep in the floor of the brain. This procedure may be used in place of a shunt when hydrocephalus is caused by certain CSF flow blockages.
In children younger than 1 year old, ETV must be combined with choroid plexus cauterization (CPC). CPC reduces the amount of CSF made in the brain. If you think of hydrocephalus like a blocked sink, the ETV creates a new drain and the CPC slows the amount of water coming from the faucet.
After surgery, your baby will receive care in the Children’s Hospital Neonatal Intensive Care Unit (NICU). Your baby may need to be on a breathing machine until he or she is fully recovered from surgery.
Other treatments your baby will probably need after surgery are:
Complications From Shunts
Your baby will need regular follow-up appointments to measure growth, development and nutrition. You will want to find a local pediatrician to take care of the routine checkups, immunizations, and doctor’s visits. The program coordinator can help if needed. Your child will need to be seen by the pediatric neurosurgeon to check his growth and development and that the shunt or ETV-CPC is working properly.
National Institute of Neurological Disorders and Stroke
National Association of Rare Diseases