The most common condition caused by Spina Bifida is hydrocephalus in babies. This comes with its own set of worries and a mountain of information to comprehend. Today we are answering some of the most common questions parents have about hydrocephalus.
What is Hydrocephalus in Babies?
Hydrocephalus is the accumulation of too much cerebrospinal fluid (CSF) in the ventricles of the brain. You may have heard the term “water on the brain,” which is the same thing as hydrocephalus.
There are four ventricles in the brain that produce and house cerebrospinal fluid (CSF). When there is a normal flow of CSF, it circulates in the brain and up and down the spine. In some cases the flow is disrupted due to a blockage. The CSF starts to back up in the brain and overflow creating more and more pressure on the brain as the ventricles continue to expand due to the constant rate of CSF production.
If there is too much pressure on the brain from the cerebrospinal fluid, it could damage brain tissue and cause a range of impairments. It can impair brain function, cause vision problems and developmental delays. If left untreated or not treated timely, it could cause severe brain damage or death.
What Are the Symptoms of Hydrocephalus in Babies?
Symptoms vary depending on the person and the age of the person. The following are the signs and symptoms of hydrocephalus in babies:
- For babies whose head has not fused together, yet, their head size will be quite larger than normal.
- The soft spot (fontanel) will be tense and bulging when sitting in an upright position
- Developmental delays
- A downward deviation of the eyes (also called sunsetting eyes)
- Poor appetite
What is the Main Cause of Hydrocephalus in Babies?
Hydrocephalus in babies is often present even before the baby is born. This is called congenital hydrocephalus. The cause could be a variety of factors such as:
- A genetic defect
- Spina Bifida
- Aqueductal stenosis
- Arachnoid cysts
- An infection in the mother during pregnancy
- Dandy-Walker syndrome
- Chiari malformation
- Complications from premature birth
How is Hydrocephalus in Babies Diagnosed?
Congenital hydrocephalus is often diagnosed before the baby is born through routine ultrasounds. If it is not diagnosed during pregnancy, the most common way it is diagnosed is by measuring the size of the baby’s head. Abnormal enlargement of the head is an indicator that warrants further testing.
When I had my ultrasound at 20 weeks to confirm my son had Spina Bifida, they also diagnosed him with hydrocephalus. They were able to diagnose this by measuring the size of the ventricles in his brain.
As a side note, another indicator that he had Spina Bifida was the shape of his precious baby head. A lemon shaped head is a sign of Spina Bifida although it’s not exclusive to it.
Can Hydrocephalus Go Away?
Hydrocephalus is not curable but it is manageable with timely treatment. Since it is a chronic condition, it is essential to continually follow-up with your child’s neurosurgeon and neurologist. The neurosurgeon will want to have periodic MRI’s to make sure the hydrocephalus is being controlled. The neurologist will make sure your child is meeting his developmental milestones and monitor the signs and symptoms caused by hydrocephalus such as vision problems, possible seizure activity and developmental delays.
How is Hydrocephalus Treated?
The most common treatment for hydrocephalus is the placement of a shunt which acts as a drainage system for the cerebrospinal fluid. A long, flexible tubing is placed in one of the ventricles of the brain. A one-way valve attached to the tubing regulates the pressure of the CSF flow. The valve keeps it flowing in the right direction and at an acceptable rate. Sometimes the rate of the flow will need to be adjusted which can only be done by the neurosurgeon. The long, flexible tubing runs down the neck and into either the belly or a chamber of the heart. The CSF is then absorbed into the bloodstream.
Another way to treat hydrocephalus is by having a surgery called endoscopic third ventriculostomy (ETV). This is where the neurosurgeon uses an instrument called an endoscope to poke a hole in the bottom of the third ventricle in the brain. The purpose of the hole is to allow CSF to flow through the spaces of the brain where it was once blocked. This procedure is usually a recommended alternative to shunting if the neurosurgeon thinks your child is a candidate.
To find out more about ETV, click here to download Hydrocephalus Association’s fact sheet on ETV.
Neither of these treatments will cure hydrocephalus. It is important to follow up regularly with your child’s neurosurgeon for periodic evaluations to make sure the shunt or ETV is working properly.
It is also important to be aware of complications of an ETV and shunt failure, and to see your child’s doctor right away if these symptoms do appear.
What Other Treatments Are Offered?
As mentioned earlier, it is common for there to be physical and mental delays caused by the pressure of CSF on the brain. The kinds of therapies involved include:
- Early intervention services – Please take advantage of this parents! Find out who your local intervention program is through and contact them as soon as possible. The hospital where your baby is born will be able to help you find out this information. In the state I live in, the program provided is funded by the Department of Elementary and Secondary Education. They provide services to families who have children with disabilities and developmental delays. The age range is from birth up to three years. Because of this program, my son has received all the therapies he has needed to reach his milestones.
- Physical therapy – These therapists work with the gross motor functioning such as rolling over, sitting without support, crawling, walking, strength building, balance and coordination, and flexibility exercises.
- Occupational therapy – These therapists aid the physical therapist in crawling and walking but they also help with fine motor skills, sensory issues, hand-eye coordination, social skills, and various other life skills.
- Speech/language therapy – These therapists not only help with speech and language disorders but they also help with feeding disorders. Problems with this could be with chewing the food properly, swallowing, gagging, and sensory issues with foods.
At some point your child should be referred for a vision exam to rule out any vision problems due to the pressure of the CSF behind the eyes. It is not always an issue with children who have hydrocephalus, but it is common. If the child’s vision is normal, then your child will not need to be seen regularly by an ophthalmologist.
Can Babies with Hydrocephalus Lead Normal Lives?
Although hydrocephalus can cause mental and physical developmental problems/delays, most children will lead normal lives with some limitations. Most babies with hydrocephalus will have normal intelligence.
Just as it is with all children, babies with hydrocephalus will develop at their own pace. They may be slower in reaching certain developmental milestones. But that’s okay! As long as they are receiving adequate therapy and the love and support from their parents, they will surely surpass your expectations.
What Can Parents Do to Help?
To make sure your child lives a successful life with hydrocephalus, here is a brief summary of what you can do as a parent to help your child:
- Take your child to all scheduled doctor visits.
- Make sure your child is receiving the recommended therapy to reach critical milestones:
- Early intervention services
- Physical therapy
- Occupational therapy
- Speech/language therapy
- Be aware of the signs and symptoms of shunt failure.
- Educate anyone who will be responsible for the care of your child to recognize the signs of shunt failure.
- Don’t be afraid to call your child’s doctor if you have concerns about your child’s behavior or symptoms. If for some reason you are unable to reach the doctor, seek medical care immediately.
Learn as much as you can about the signs and symptoms of shunt failure and educate those who will be taking care of your child. To help with that, I have included a FREE printable that lists the signs of shunt failure for infants, toddlers, and children. You can hand these out to anyone that will be watching your child. I recommend putting these up everywhere your child will be when not in your care; places like Grandma and Grandpa’s house, the church nursery, on your fridge for the babysitter, childcare center, pre-school. You get the picture. Everyone needs to be aware at all times. These symptoms should not be considered typical in a child with a shunt and medical attention should be sought immediately.
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What other questions do you have about hydrocephalus? Comment below or send me a personal message. I’d love to hear from you!