Congenital Hydrocephalus

A diagnosis of "Congenital hydrocephalus" can scare many people. They probably think: "What is it"? How is it going to affect me? What can be done for it? Who can I turn to for help in understanding? Where can I find information about "congenital hydrocephalus"?

"Congenital"; meaning "originating prior to or during the birthing process". "Hydrocephalus"; meaning "water on the brain". Ok, enough of the boring stuff!!!

There are two "accepted" types of hydrocephalus: 1) congenital and 2) acquired. Yet, there is also what is called "NPH" or "Normal Pressure Hydrocephalus". NPH, is *normally* found in older adults (and by older I mean anyone from 60-100+ yrs old). We already know that "congenital" means "from birth, essentially". Acquired hydrocephalus happens when something impacts the brain and causes a change in either the structure of the brain, or the way the brain functions. Examples of this would include: 1) Encephalitis, 2) Meningitis, 3) strokes *can* lead to hydrocephalus, 4) MVA's or Motor Vehicle Accidents and also 5) Gunshot wounds or even some sports can lead to the onset of "acquired" hydrocephalus. Think of boxing, think of Friday night high school, Saturday College or Sunday Football. With the the *body armor* that the "kids" wear, they sometimes *think* they are "invincible". Well, their brains are *NOT* invincible. I have been watching as more and more kids are taken off the field on stretchers... Very sad.
While I don't want to get all technical on you, if you have questions, feel free to ask.  I will say that I don't know enough about NPH to discuss is and would refer you on to a medical professional.

If you, or someone you love has a diagnosis of hydrocephalus, it is *NOT* a death sentence. I should know. I'm a 51 yr old, who when I was younger was not given a great chance at survival. Yet, here I am. Is life difficult, yes... at times it is. But, as they say "such is life". If the hydrocephalus is treated *properly* and quickly, we have the same life span as everyone else. Are there deaths caused from hydrocephalus, yes. But, I don't have the statistics on that. What I do know is that *in the US*, 1 in every 500 births has a chance at being diagnosed with "congenital hydrocephalus". Hey, the birthing process is severely difficult!!! Yet, there is also in utero operations; for the treatment of hydrocephalus. But, be aware that those types of operations are rare. The neurosurgeon *usually* waits until the baby is born and then operates.

Let's talk about the operation!!! *Typically*, the operation is performed by a pediatric neurosurgeon. The child is put under general anesthesia, a blood pressure cuff is placed on the child's arm. Sometimes, a oxygen saturation monitor is used, as is an EKG/ECG that monitors the heart. Incisions are made. One can be above and behind the ear, or there may be an incision near the front of the head. To get *to* the brain, a section of the skull is removed. That is called the *bur hole*. There may also be another incision near the neck and also the abdomen. Depending on where the shunt tubing is to empty into. If it is a VA or venticulo-atrial shunt, that empties into the superior vena cava (heart). If, however the shunt tubing empties into the abdominal region, that is a VP or Ventriculo-peritoneal shunt. There is a third, less often used shunt LP (lumbar peritoneal) which empties the fluid from the lumbar region of the spine into the peritoneal cavity (belly).

Let's talk about the valve. I call it the brains of the operation. There are two types of valves. 1) Range valves, that have a set range in which they work (some call them fixed rate valves and there are other names for them, depending on the region). They come in "low", "medium" and "high" pressure settings, depending on the amount of fluid to be evacuated from the head.. Then we have 2) the programmable valves. 

When I was first born, they only had "fixed" rate valves. That first valve lasted me 16 yrs. My second lasted twelve. I had two more that didn't last too long. Oh well, that happens when you are dealing with *man-made* equipment. I then had one of the first "programmable valves" put in. It was great!!! It meant, less surgeries... or at least, that was the goal. Reality was a tad different. Hey, that's the life of someone with hydrocephalus.

During the '90's I had what amounted to a surgery every yr. It had nothing to do with the neurosurgeon. But rather, I had a *finicky* brain. During that decade, I had a couple of valves where the tubing that went from the valve to the stomach, disconnected. I traced *both* of the disconnects to a couple of sneezing fits I had.
There was also one recall that I was notified of, and my neurosurgeon who had done the original operation, notified me of a recall of the shunt tubing due to a manufacturing issue. He replaced it and I went on my way.

I want to inform you of a condition called "Slit Ventrical Syndrome", or SVS. It happens mostly in children who have had to endure numerous shunt revision operations. The ventricles become *slit-like*, rather than having the large ventricles, the reverse happens... That alone can lead to excruciating headaches. Those of us who deal with SVS and hydrocephalus; in general; would make awesome weather forecasters... We can often tell when the weather front changes.

I hope you enjoyed reading my first blog. Feel free to comment away. In conclusion, I am *NOT* a medical professional. I don't want to be *perceived* as one. I am only writing this blog to *inform*. Though I can "hold my own" in a discussion about medical issues, I would refer you to your local medical professionals. I *WILL* give opinions, but not offer any advice on what you should do. You probably noticed I left some unanswered questions... Those will be discussed in a later blog. I want to see how well received this one is. Let's call it a "cliff-hanger"...lol