Relieving the pressure within: preparing for my surgery (part 1)

Most involved . . .

Since 2008, I have five other surgical procedures: three stints (2016), bi-ventricular pacemaker (2014), and an Orchiectomy (2008). The surgery to implant a shunt or open a ventriculostomy will be, by far, the most involved carrying the greatest benefit to allow me to return - at least for the biggest part - to helping others. Are there risk? Yes, but at this point, I think the benefits far exceed the risk. To quote an article I read "Most cases of symptomatic hydrocephalus must be treated before permanent neurologic deficits result or neurologic deficits progress" That same article goes on to explain that when the etiologic factor(s) are known, the hydrocephalus can be treated with temporary measures while the underlying causation is also being treated. In my situation, the etiology is congenital aqueductal stenosis which causes my aqueduct of Sylvius to be completely blocked which means cerebrospinal fluid is always present in my skull and cannot be reabsorbed by my body.

Questions, forms, and tests (Oh my!)

I haven't even had the consultation for the surgery yet, and I am blown away by the questions to be answered, forms to be filled out (some in duplicate and triplicate), and tests that have to be completed. I am somewhat embarrassed to admit this, but as I was educating myself on what was about to happen and the website mentioned "informed consent" but, for whatever reason, my mind saw "implied consent". It wasn't until later than evening when I mentioned it to my sister Hope, that she pointed out my gaffe. "Informed consent", by the way, means that I fully understand what the surgery is intended to do, benefits and, of course, potential risks. I do.

But it's not just paperwork when you're preparing for brain surgery (pardon my repeated use of those words . . . it's my way of making what is about to happen real). Prior my consultation visit with my neurosurgeon (which will most likely occur late next and when I am admitted to the hospital, I will have undergone three (3) imaging studies including an Cine MRI which will allow the neurosurgeon to the flow of my cerebrospinal fluid or CSF. This is important for two reasons: 1) It will allow the neurosurgeon to see any blockages that exists in addition to the aqueductual stenosis that we are already aware of; and 2) It will confirm if their suspicions are correct and that I also have Chiari malformation where the cerebellar tonsils (lowest part of the brain) has actually descended into the upper part of my spinal column.

Let's talk about shunt surgery

I would have never thought it

For those who follow this blog regularly, you know that I developed hydrocephalus about one week after I was born.  You might also remember that I became symptomatic last October. Up to this point I have done okay treating the symptoms with medication, but they are getting worse, so, this coming week, I have an appointment at Emory University Hospital for a surgical consultation to either implant a ventriculoperitoneal (VP) shunt, do an endoscopic third ventriculostomy (ETV), or, maybe, both. Whichever Dr. Olson and I agree on, I am ready and look forward - following my recovery - to returning to a quality of life that I enjoy and that benefits those around me.

As I say, I am at peace with the surgery, however, after seeing a video on YouTube, one aspect of the shunt implant raise my anxiety (and pain level) a notch. It involves what I refer to as the "Moses stick" (surgical, stainless steel rod) they use to guide the tubing toward its final destination in my peritoneal cavity. I have been assured by my hydrocephalus family that I will be getting the "good stuff" for pain so any discomfort I feel should be minimal.

They will keep me going

Regardless of how it plays - surgical intervention or continued treatment with medication - I have the most awesome support system a guy could ask for and I believe it is because they have "been there, done that" so they are not only helping me to grasp what's before me, but calming my fears. I love my sweet "hydro family". ❤

Complications and malfunctions of shunt systems

Sadly, much of my inspiration for blogs on hydrocephalus come about because of a bad situation that occurs either to me or one of my friends with hydrocephalus. This is one of those occasions. Early Monday morning, one of my friends passed away after tubing from his shunt broke and came into contact with his brain stem. You might wonder how such a thing could happen. So named because of its stem-like appearance, the brain stem is where the base of the brain attaches to the spinal cord. (WebMD, 2016) it's main job is to control the flow of messages between the brain and the rest of the body; it is also controls basic body functions such as breathing, heart rate, and blood pressure.

What can go wrong?

Hydrocephalus is treated by surgically implanting a shunt into the patient's brain, however, complications can develop or the shunt can malfunction. Interestingly, in pediatric patients, there is a fifty percent (50%) shunt failure rate after only two (2) years. The exact cause for this is unclear.

Shunt malfunction

A partial or complete blockage of the shunt system is know as a shunt malfunction. (Hydrocephalus Association, 2014) When this occurs, cerebrospinal fluid builds up and causes symptoms similar to those seen with untreated hydrocephalus.

The blockage is caused by a build-up of blood cells, tissue, or bacteria and occur anywhere system. Both the proximal catheter (which is implanted in the brain) as well as the distal catheter (which can be implanted either in the ventricle of the heart, the peritoneal cavity of the abdomen, or, rarely, in the spine) can be blocked. These blockages originate in either the choroid plexus or the ventricles of the brain. Generally speaking, in adult hydrocephalus patient's, blockages occur with greater frequency in the distal catheter.

Generally, a shunt system is durable, however, components of the system (pictured below) can become disengaged or fractured as the result of normal wear -- particularly in children due to their growth spurts. Also, although rare, a valve can fail due to a mechanical malfunction.

Shunt infection

A shunt infection usually occurs as a result of the person's own bacterial organisms and isn't caused by be exposed to someone who is sick. The most common bacterium to cause an infection is Staphlococcus epidermis. which is normally present on a person's skin as well as in the hair follicles and in the sweat glands. This type of infection is typically seen one (1) to three (3) months after shunt implant surgery, but can occur up to six (6) months later. In persons with a ventriculoperitoneal (VP) shunt is a shunt infection that occurs secondarily to an abdominal infection. Lastly, in persons treated with a ventriculoatrial shunt (which empties into the right ventricle of the heart) a generalized infection can occur.

Other shunt complications

Over drainage causes the ventricles to decrease in size and become slit-like (see: What is slit ventricle syndrome (SVS)?) due to the brain and meninges pulling away from the skull. SVS is most common in young adults who have been shunted since early childhood. A telltale symptom of SVS is severe intermittent headache that improves when the person is laying down.

Under drainage does the exact opposite and causes the ventricles to swell. When this occurs, the shunt might not be able to relieve the hydrocephalus symptoms. In order to restore a balanced flow of CSF, it might be necessary to implant a new shunt with a more accurate pressure valve. If the person has a shunt with programmable valves, the balance of flow can be restored by resetting the opening pressure.

 Subdural hematoma (pictured at left) occurs when a broken blood vessel in the meninges becomes trapped between the skull and the brain. It is seen most commonly in adults with Normal pressure hydrocephalus (NPH) and requires surgical intervention to correct it.

Multiloculated hydrocephalus is located (isolated) CSF compartment in the ventricular system that is enlarged and not in communication with the normal ventricle. It can be the result of trauma at birth, neonatal intraventricular hemorrhage, Ventriculitis, over drainage, or other conditions. This complication might be difficult to identify because it is typically seen in infants and children who might be neurologically compromised. Treatment involves surgery to implant ventricular catheters, Craniotomy and fenestration (opening) of the intraventricular loculations. (John Hopkins Medical, N.D.)

Seizures can sometimes occur in people (both adults and children) with hydrocephalus. Medical research has shown there is NO CORRELATION (emphasis added) between the site a shunt implant (or the number of revisions a person has) and a increased risk of developing seizures. The one possible exception to this involves children who have significant cognitive delays or motor disabilities are at higher risk (italic emphasis added) are more likely to experience seizures compared to children without similar delays or disabilities. Research has also shown that seizures aren't likely to occur at the time a shunt malfunctions, and the more likely explanation for the occurrence of a seizure disorder is related to an associated malformation of the cerebral cortex.

Abdominal complications can also occur in patient's with hydrocephalus who are treated with a shunt. This is due to the fact the distal catheter of a ventriculoperitoneal (VP) shunt is placed in  the peritoneal cavity of the abdomen. Although complications associated with a VP shunt aren't less in frequency (when compared to a ventriculoatrial [VA] shunt), they tend to be less severe and have a lower mortality rate. These complications can include: peritoneal pseudocysts, loss of the distal catheter, bowel perforations, and hernias.

What are the different types of shunts and how do they work?

Introduction

Yesterday I was working on a blog for my Facebook hydrocephalus support group and one of the group admins (adminstrators) to write a blog discussing the different types of shunts -- here it is. Let's start by looking at the different types of shunts, their pathway, their inflow location, and their drainage location.

Shunt pathway              Type      inflow location      Outflow location

Ventriculo-peritoneal    VP           Ventricle (brain)     Peritoneal cavity
Ventriculo-atrial             VA           Ventricle (brain)     Right atrium of the heart
Ventriculo-pleural          VPL         Ventricle (brain)    Pleural cavity
Lumbo-peritoneal          LP            Lumbar spine        Peritoneal cavity

Now that I have probably confused you, let me see if I can make the "Inflow location" and "outflow location" make sense. The inflow catheter of the shunt system (also referred to as the proximal end) is the location where the Cerebropspinal fluid (CSF) is introduced into the shunt system. The outflow catheter of the shunt system (also referred to as the distal end) is the location that the CSF empties into to be reabsorbed by the body.

How does a shunt system work?

Also known as a "CSF diversion device", shunts have been used for 68 years (the first one was successfully implanted in 1949) to divert CSF from the ventricles within either the brain or subarachnoid space(s) to another part of the body where it can be reabsorbed. Implantation of a shunt creates an alternate pathway for the CSF which is constantly produced within the brain and usually (italics added for emphasis) restores the balance between CSF production, flow, and absorption when one (or more) of these functions becomes impaired. There are valves within the shunt's pathway that act as "on-off switches" when the Differential pressure (DP) - the pressure difference across the valve - exceeds the valve's opening pressure.

My sources:

Hydrocephalus Association (shunt fact sheet)
Neuropathology: an illustrative interactive course

Recovery after VP shunt implant surgery (part 2)

Yesterday, we looked at placement and recovery following ventriculo-peritoneal surgery. Today, we will look at recovery once the patient gets home and potential complications that can occur.

Recovery at home

After surgery, the patient may experience tenderness in their neck or belly since these are both incision sites. Overall, shunt implant surgery is VERY safe procedure, however, complications can occur both during the procedure and during the recovery period. These include: 1) Infection either in the brain or in the shunt itself; 2) Bleeding inside the brain; 3) Damage to brain tissue; or 4) Edema (swelling) of the brain.

Additionally, in rare cases, patients might experience fever (>36.0 celsius), headache, abdominal pain, fatigue, or a spike in blood pressure (or experiencing symptoms similar to before the shunt was implanted) are indications of either an infection, shunt malfunction, or shunt failure. THESE ARE POTENTIALLY LIFE-THREATENING -- NOTIFY YOUR DOCTOR OR PROCEED TO THE NEAREST EMERGENCY DEPARTMENT.

What is the prognosis for implant patients?

Shunting is successful in reducing intracranial pressure (ICP) in most patients. One drawback to the shunting process is the fact that it generally (italics added) has to be replaced after several years -- particularly in small children. For infants, the anticipated life of a shunt is two (2) years. After age two and in adults with a shunt, this increases to eight (8) or more years. (I have a friend whose shunt is 37 years-old). Shunt systems require frequent monitoring to prevent: 1) Mechanical failure; 2) Obstruction; or 3) Infection.

My sources:

What is a vetriculoperitoneal shunt? (Healthline.com)

Recovery after VP shunt implant surgery (part 1)

Some background . . .

A few minutes ago on one of my Facebook hydrocephalus support groups, we had a mom ask about recovery after shunt implant surgery. Her infant son was several days post surgery and, while his incision was healing well, the group was concerned about abnormal swelling around the incision site. Being the group's resident "egg-head" (pun intended) I sprang into action to find the answers to her questions. This blog is the results of my research.

What is a VP shunt?

A ventriculo-peritoneal (VP) shunt is used to remove excess cerebrospinal fluid (CSF) from the skull of patients suffering from Hydrocephalus. It is so named because the distal end of the shunt is placed in the peritoneal cavity of the abdomen where it empties and the CSF is reabsorbed by the body.

How is the implant done?

Surgical implant of a VP shunt requires the neurosurgeon to make one (1) burr (drill) hole and  two (2) incisions: a hole is drilled (burred) using a surgical drill behind the patient's ear to place the proximal end of the shunt into the affected ventricle of the patient's brain, one incision is made into the neck to aid in the placement of the tubing into the chest / abdomen area; and one incision is made in the patient's abdomen to receive the distal end of the shunt. Barring any unforeseen complications, the procedure usually takes about (italics added) 1.5 hours.

Are there risks associated with shunt implant surgery?

Simply put, as with any surgical procedure, there are risk involved. Generally, however, the benefits of shunt implant surgery out-weigh the risks. In addition to breathing issues, possible reaction to medication(s), and bleeding or blood clots, shunt implant surgery carries several unique risks: 1) Bleeding from the brain or blood clot(s); 2) Swelling of the brain; 3) Development of bowel perforation (a hole) following the surgery; 4) Leakage of CSF fluid under the skin; 5) Infection of the shunt, brain, or in the abdomen; 6) Damage to brain tissue; or 7) Seizures.

In order to prevent potential complications (and to facilitate faster healing) the patient might (italics added) be required to lie flat for the first 24-hours after the surgery. The duration of the post-operative hospital stay is dependent on why the shunt was originally needed. For example, a shunt being placed to try congenital hydrocephalus might not require as long of a hospital stay as one for a shunt placed to treat Acquired hydrocephalus which is caused by a traumatic brain injury (TBI) or an infection. The health care team will carefully monitor the situation.

My sources:

1) Ventriculo-peritoneal shunting           (Medine.com)
2) What is a vetriculoperitoneal shunt? (Healthline.com)

Hydrocephalus and hospitalization (Part 1 of 2)

The ambulance bay at Southern Regional
Hospital.

Today I decided to broach a subject that I don't have much experience with since I am not shunted and that is hospitalization and hydrocephalus. I went to Hydrocephalus Association (HA) website and downloaded their fact sheet on the subject. As I was reading the information and deciding what information to include, I was struck by a profound statement made by fellow hydrocephalus patient Amy Herrington: "shunts are not capable of paying attention to a family's social calendar, or a child's school calendar". I sat here for a couple of minutes letting that soak in and was again reminded how blessed I am that my dad opted to not have me shunted. Make no mistake, shunts are a good thing because they can prevent an enlarged head such as mine, but with a BIG price including the risk of infection and the need for periodic revisions.

Amy hit it squarely on the head when she said "no matter whether you're a parent, child, or adult dealing with hydrocephalus and the possible need for shunt revisions, the common threat of hospitalization looms in the midst". Although I have never experienced it, I can only imagine what a family goes through when they face the possibility of a loved one having to endure yet another surgery and the range of emotions they will face. I have always heard that "growing old isn't for sissy's" well, I have news for you, hydrocephalus isn't either. Here are some tips that Amy offers for dealing with those hospitalizations:

General suggestions for all patients and families

• You have the right to review your own medical records (or those of your minor child) as well as to know what procedures you (or your child) will receive while in the hospital. (Health Insurance Portability and Accountability Act (HIPAA)

• Bring familiar items from home to make your hospital room look more inviting.

• Keep a written or tape-recorded journal while you hospitalized. Concentrate specifically on your hopes, dream, fears, or anxieties. Doing so is an important part of the emotional healing process.

For parents of infant children

• Remember that you are the advocate for your infant child. You have the right to ask questions (and receive proper answers) regarding your baby’s health. Become educated on what YOU can do to help your baby.

• If possible, hold your baby. Touch is crucial to their development. If it is not possible to hold your baby, talk quietly to him or her. Your voice is literally music to their ears.

For parents of a young child (ages 2 - 6)

• As with an infant, you are still the advocate for your young child. Inquire about your child’s health and learn what their needs are.

• Draw a map with your child that depicts the distance between the hospital and home. This will help your child understand where he/she is.

• Some children feel they have to be brave in the hospital -- especially around parents who care about them. Remind them that it is perfectly acceptable to express feelings of anger, sadness, or frustration in the hospital. Have them draw pictures of how they feel both physically and emotionally while they are hospitalized. This serves as an outlet to relieve tension.

For additional information: Hydrocephalus and hospitalization

When shunts go bad: complications experienced with shunt systems

X-ray images of a 20-month old hydrocephalus patient
experiencing shunt malfunction. As a result, the child
experienced sutural diastasis.

Hydrocephalus can be treated with a shunt system, however, this treatment often results in complications. Research has shown that an estimated fifty percent (50%) of shunts in pediatric patients fail within two (2) years often requiring repeated neurosurgery. The most commonly seen of these complications are: infection and malfunction (emphasis added).

I. Common complications

Shunt malfunction

A shunt malfunction involves either a partial or complete blockage (of the tubing) causing the shunt to function intermittently or not at all. As a result of the blockage cerebrospinal fluid (CF) accumulates and, as illustrated in the image at right, can result in diastasis where a fracture line traverses one (or more) sutures of the skull resulting in a widening of the suture.

The blockage can result from blood cells, tissue, or bacteria and occur in any part of the shunt. Both the ventricular catheter (the portion of the system placed in the brain) and the distal catheter (the portion of the system that drains CSF to other part of the body) can become blocked by tissue from the choroid plexus (which produces CSF) or ventricles. In adults, the blockage occurs most commonly in the distal portion of the shunt system.

Shunts - by their very nature - are very durable, but their components can become disengaged or fractured as a result of wear or as a child grows. In rare cases valve failure can occur due to a mechanical malfunction.

Shunt infection

Typically a shunt infection occurs because of the person's own bacteria and isn't acquired from someone else who is ill. The most common infection to occur in a shunt system is Staphylococcus Epidermidis which is know for attacking indwelling medical devices such as a shunt. which is normally found both on the surface of a person's skin as well as in the sweat glands. Normally a shunt infection occurs with a one (1) to three (3) month window following shunt implant surgery, but has been known to occur up to six (6) months following the surgery. Research has shown that patients receiving a ventriculoperitoneal (VP) shunt are statistically at the highest risk for developing a shunt infection secondary to abdominal infection.

II. Less common complications

Over drainage

CT scan shows hydroceplaus
patient with SVS.

This complication causes ventricles to decrease in size causing slit-like ventricles due to the brain and its meninges pulling away from the skull. Known as slit-ventricle syndrome or SVS for short (pictured at right) , it is most commonly seen in young adults (ages 20 - 39) who were shunted as a child. One symptom that is unique to SVS is the severe intermittent headache that can be relieved by lying down. In order to confirm a diagnosis of SVS, imaging must be completed that shows the smaller size ventricles.

Under drainage

Results in the exact opposite problem as over drainage and that is an increase in the size of ventricles as well as the inability to relieve the symptoms associated with the hydrocephalus. In some cases, to restore a balanced flow of CSF, it might be necessary to implant a new shunt with a more accurate pressure valve. In patient's with a programmable shunt, it is possible to restore a balanced flow by simply re-setting the opening pressure.

Subdural hematoma

Control unit of a programmable shunt.

This is generally seen in older adults (ages 60 - 80) and is caused when blood from a broken vessel in the meninges becomes trapped between the skull and the brain. Surgical intervention is required to correct this complication.

Multiloculated hydrocephalus

This condition occurs when a located (isolated) compartment in the ventricular system becomes enlarged and not in communication with the normal ventricle. There a numerous documented causes including: neonatal intraventricular hemorrhage, trauma that occurs at birth, ventriculitis (inflammation of the ventricles), or shunt-related infection. Typically it can be difficult to identify due to the fact that it is most often seen in infants and children who might be neurologically compromised.

 
For additional information: Complications of shunt systems

Shunting as a means to manage hydrocephalus

I. Overview

Subarachnoid spaces in the brain

I. Overview

Over the years, the management of hydrocephalus has challenged neurologist, engineers, and medical device manufacturer alike due to the unique nature of cerebrospinal fluid (CSF) dynamics in each person. Known as a "CSF diversion device", a shunt has become the primary therapy used in hydrocephalus management for over sixty (60) years. The shunt, which is surgically implanted within a ventricle in the patient's brain (or in the subarachnoid spaces around the brain), works by diverting the CSF to another part of the body where it is absorbed. This creation of an alternate pathway typically restores the physiological balance between CSF production, flow, and absorption when one or more of these functions has been impaired. Once it has been inserted, valves within the shunt's pathway act like on/off switches, opening when the differential pressure, that is, the pressure difference across the valve, exceeds the valve's opening pressure.

Blog author Walter Little with his
grandparents, James and Sara Sanders,
and Officer Dorsey Goss (1973)

II. What are the complications of shunting?

As I alluded to above, a shunt provides an alternate pathway through which CSF can bypass obstruction(s) in the fluid compartments (ventricles) of the brain. Such a bypass relieves the excess fluid backup the causes hydrocephalus. When both the CSF production and absorption are in balance, the hydrocephalus is considered to be "compensated". In contrast, when production exceeds aborption, complications such as elevated pressure or overdrainage occur and can mimic a malfunctioning shunt.

More detailed information can be found on the Hydrocephalus Association website, but here is a brief overview of some of the most common complications:

Malfunction

As is the case with any piece of equipment, a shunt might break. Additional they are subject to becoming disconnected, migrating (moving), or, most commonly, becoming blocked. (It should be noted that the disconnection and migration are particularly prevalent in children with a shunt due to the child's growth.)

Infection

Shunts can become colonized with bacteria or - in rare instances - fungi which typically occurs at the time the shunt is implanted. In an effort to combat this, some manufacturers have began to add anti-microbial coating to their shunts which appears to be reducing the rate of post-surgical infection.

Material degradation

Originally, barium sulfate (BaSO4) was mixed with silicone to allow the shunt catheter to be visible on an x-ray. These would eventually dissolve making the tubing surface rough. When tissue in-growth occurred to the tubing, it would bind at that location resulting in deterioration and/or breakage. Over time the design of shunt tubing has changed and a clear silicone elastomer now covers the surface of the tubing greatly decreasing the likelihood of degradation.

 Shunt revision(s)

I put an "s" on revision because the average person with hydrocephalus undergoes 2.66 shunt revisions over the course of their lifetime. These can be required at any time to correct one (or more) of the complications outlined above or to compensate for growth in the case of children and young adults. If a blockage is suspected, it must be confirmed by a neurosurgeon who evaluates the implanted system to determine whether the problem is the result of a complete or partial blockage, if a disconnection has occurred, or whether the current system just can't no longer meet the needs of the individual.

Additional information:

Shunt system fact sheet