The Wonderful World of Hydrocephalus: Part 5, Shunt Malfunctions

Shall we start with a wee-bit of history. It’s just so interesting. To be brief, a guy had a son. The son was born with hydrocephalus. The father was an engineer and invented the shunt in order to save his son’s life. Unfortunately, his son passed away before they “perfected” the shunt. But his passion to save his son’s life has saved millions since. The invention of the shunt has given people with hydrocephalus the chance to live–who otherwise wouldn’t–long healthy lives for the last 50+ years. For that, we thank you John Holter for your amazing work.
“Perfected” is in quotes above because the shunt has flaws to this day. A shunt’s structure and function is simple in design yet how each person handles it is another story. One that shunt manufacturers can’t quite grasp. One that I’m not sure anyone will ever be able to grasp. My neurosurgeon explained to me that there are just too many factors involved. Our bodies naturally respond and adapt to changes in our environment in order to keep us healthy. A shunt can’t be programmed to do the same. Your age, proteins in your blood, hormone levels, the weather, the time of day, what you consume, etc…all contribute to how your body creates CSF and how your body responds to your shunt. It’s a miracle shunts work as well as they do to control hydrocephalus.
I met a young woman of 17 years last weekend who received a VP shunt soon after birth. She has not had one malfunction since. She is extremely lucky. I read about a woman who was shunted at age 4 and had over 30 revisions (surgeries due to a shunt malfunction) by age 29. That’s more than 1 revision a year! I’ve also heard of people who had a bunch of revisions for a while, and then none for years after. You can see how different it is per case.
What is a shunt malfunction?
A shunt malfunction is when a shunt stops working due to obstruction/blockage, an infection, or component failure.
If a shunt malfunctions due to an obstruction, CSF can’t be diverted from the ventricles and will back up from the site of obstruction. A shunt can have a small obstruction (partial) that still allows CSF to flow through or a severe obstruction (complete blockage) that will not allow any CSF to flow through. In either case, immediate action should be taken so no pressure is put on the brain causing brain damage. Obstructions can happen in any components of a shunt but it is most common that tissue blocks the ventricular catheter. Second to that, blood cells or bacteria can clog the catheters or the valve.
Like any surgery, infection is a risk. If a shunt stops working due to an infection, action must be taken immediately and seriously. Most commonly, this happens at time of surgery by the person’s own bacterial organisms that live on the surface of their skin, in their sweat glands, and deep within the skin of their hair follicles. These infections most commonly occur from time of surgery to 3 months after surgery but can occur up to 6 months after surgery. My daughter’s neurosurgeon said, your odds are pretty slim after 3 months but there is still a small chance of infection even up to 1 year after surgery.
Shunts are very durable but they can still break due to wear, a child’s growth, impact, or have a mechanical malfunction. I do not have any stats on this but I have heard these complications are rare.
What are the symptoms of a shunt malfunction?
I touched on this in WELCOME TO THE WONDERFUL WORLD OF HYDROCEPHALUS: PART 2, THE BASICS but here’s a more extensive list.
• Headache
• Vomiting
• Irritability
• Sleepy-difficulty waking or staying awake
• Loss of appetite
• Vision problems
• Personality change
• Loss of coordination or balance
• Decline in academic performance
• Swelling along the tract
• Fever*
• Redness along the shunt tract*
Infants/Toddler symptoms may include:
• Head enlargement
• Fontanelle is full and tense when the infant is upright and quiet
• Prominent scalp veins
• Downward deviation of the eyes
• Loss of previous abilities (milestones regressing)
* Symptoms of an infection.
Here’s where I get really annoyed. Symptoms are considerably different per person. This makes it extremely hard to know if you or your loved one is simply having a headache or a shunt malfunction. Asking advice from others that have been through shunt malfunctions doesn’t seem productive since symptoms vary so much. I guess the one thing anyone that has been through it before can say is, “When in doubt check it out.” Once you go through one malfunction, supposedly future malfunctions render similar symptoms.
My daughter has had one malfunction due to blockage and her only symptom was nothing more than waking up every other night for three nights around 1:30 AM screaming. By the third night she vomited and I knew it was a malfunction since she had no other symptoms of being sick. It could have been easily ignored assuming she ate something bad or had a quick stomach bug. I’m glad I went with my motherly instinct.
What should I do if me or someone I know is showing symptoms of a shunt malfunction?
Go to the ER immediately! If it is a malfunction you’ll be glad you go it taken care of ASAP, if it’s not, you’ll be glad you found out it wasn’t a malfunction. The bottom line here is don’t wait.
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Next time in Part 6 I’ll go over shunt revisions.
– – – – – – – – – – –

The Wonderful World of Hydrocephalus: Part 1, The Facts

The Wonderful World of Hydrocephalus: Part 3, How Hydrocephalus Works
The Wonderful World of Hydrocephalus: Part 4, How Shunts Work

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My amazing neurosurgeon-Dr. George

2 Responses to “The Wonderful World of Hydrocephalus: Part 5, Shunt Malfunctions”
  1. carey gooden says:

    Your information is so helpful!
    I recently had a little girl with a shunt — her mother never informed us that she had one!
    We just happened to notice it one day!
    Her mom hadn’t known that it was supposed to be monitored…..
    Thankfully she had not had any adverse symptoms, but now Mom knows.
    Oh, my.

    • pforpeanuts says:

      I’m glad to know my series has helped you and possibly caught another child’s malfunction before it was too problematic.

      The scary thing is that when people don’t have malfunctions, they tend to forget about it completely. That’s when it can become dangerous.

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