When the bladder does not empty itself, other ways of emptying it are necessary. Here are four ways that the bladder can be emptied. For some treatments that may be on the horizon, click here.
Usually when the child is a newborn and there is small bladder capacity or pressure, you want to avoid damage to the kidney, but it’s difficult to start catheterizing a young infant, so there is a simple surgery that can be done where a small opening of about 1.5 inches is made so that urine can drain into the diaper. That way, the parents don’t have to catheterize the young infant or toddler and the kidneys stay safe because nothing is backing up into them. The bladder doesn’t have any pressure because there is nothing in it. It’s all draining out.
Eventually, usually around age 3 to 5, when there is a desire to get out of diapers then the vesicostomy can be closed. It’s easy to close surgically and after closing it shouldn’t leak. Then the child will begin catheterizing.
CLEAN INTERMITTENT CATHETERIZATION
A catheter is a thin flexible tube that is used to drain urine from the bladder. A catheter inserted into urethra every 3 or 4 hours to drain the urine out of the bladder. When urine remains in the bladder for longer periods of time, it often grows bacteria, leading to infection. Introducing a catheter into the bladder several times a day to drain urine also introduces infection. Infections are typically addressed with low dose antibiotics taken on a daily basis when there is reflux to the kidney. When there is no reflux, even if bacteria grows in the bladder, it is not necessary to treat infection that is asymptomatic.
Sometimes there are difficulties with catheterization. It can be difficult to catheterize when it hard to find the urethra and it may be hard to put in the catheter tube. Some children with SA/CRS are highly sensitive in the area around the urethra and catheterizing can be very painful.
APENDIVESICOSTOMY (MITROFONOFF), also COLOVESTICOSTOMY
These are surgeries, and they involving taking a piece of the intestines, appendix, or colon to make a tunnel from the bladder to the surface of the skin. The appendix is a one-way valve that is part of the intestines but has no purpose, so it can be used to make a channel from the bladder to the skin surface. There is a small hole (stoma) in the skin surface (in a mitrofonoff this hole is made in the belly button so that is is not visible). The catheter tube can pass through this stoma into the bladder for catheterization. This can be a good alternative to catheterizing through the urethra, allowing easier catheterization, preventing reflux and bladder pressures.
There may be some difficulties with these surgeries. About 20% of people will have leakage from the stoma, or stenosis (a narrowing of the stoma) So, there is a fairly high chance that there will be a need for surgical revision down the road.
The leaking occurs because the tunnel between the lining of the bladder wall and muscle to the external part of the bladder is not long enough, so urine passes back out through the tunnel and leaks through the stoma. Before, urine reflux went up to the kidney, but with the apendivesticostomy it refluxes outside to the skin surface This might be addressed by using a bulking agent, (deflux), to bulk up the stoma so the opening is not as wide, or there may be need for surgical revision to make the tunnel longer.
For individuals with high level SA/CRS, there may be leaking because in a sitting position the torso is not supported by the spine. Organs compete for space in and below the rib cage. That may mean increased pressure pushing down on the bladder, making urine pass out of the tunnel and leak through the stoma. This concern has yet to be investigated through research.
The super pubic catheter is a catheter that stays in the bladder and empties into a bag or can be clamped and then drained. It is not often used in pediatric patients because it involves having a foreign body in the bladder. If you look into the bladder using a cystoscope (procedure to examine the inside of the bladder and urethra with the use of a small telescope, or “cystoscope”, you can see that the walls inside the bladder that have had a super public catheter tend to be swollen and irritated. The super pubic catheter tends to also cause bladder spasms and results in a smaller capacity bladder. It would be a last resort for long term use, but may be used for 2-3 weeks following surgery to allow the bladder to recover following surgery.
This surgery is for lowering pressure and/or improving continence. If you have a small or inflexible bladder, and/or high pressure, various methods used to improve size and flexibility. These include medications, but sometimes surgery is necessary. Augmentation cystoplasty is an option to increase the size and elasticity of the bladder. A part of the intestines are used to make a patch on the bladder that becomes part of the bladder.
When an augmentation is done, then reflux can be addressed by reimplanting of the ureter(s). Augmentation is a last resort to save the kidneys and achieve continence. Medication and catheterization should be tried first, because after the augment, there is a 40% chance that there will be bladder stones.
Augmented bladders produce mucus, which needs to be irrigated daily or often, or that mucus leads to bladder stones. Also a 20% chance, any time there is major abdominal surgery; it increases the risk of bowel obstruction, so that’s a concern for the SA/CRS population.
Sometimes, to prevent leakage, the bladder neck is closed off as a part of the augmentation cystoplasty surgery. However, that has other complications such as bladder rupture. A young child, under parent supervision, may comply with a regular schedule of catheterizing to drain the bladder so that it does not become full and burst. A teenager, however, may become resistant, wanting not to deal with a schedule of catheterization, and may have a bladder rupture as a result of non-compliance. Lastly, there is an increased risk of spontaneous bladder rupture . Internationally, 3-5% of patients with the augment may have spontaneous bladder ruptures, and mostly because of not catheterizing on schedule.
Ditropan is medication that relaxes bladder wall and prevents thickening of wall over time-maintains the flexibility of the bladder. Ditropan is used to keep the bladder walls flexible and reduce bladder spasms. Some children do not tolerate ditropan well, particularly the side effects of dry mouth and the need to stay out of the sun to prevent overheating.
Botox, which has to be repeated every 6 to 9 months, may be used to reduce bladder spasms. This is a relatively new approach with varied results.
Antibiotics, taken daily such as microdantin, nutroflorzxin, or bactrim in low doses prevent bacteria from building up in the bladder. The reason for antibiotics has to do with the amount of reflux to the kidneys. If there is no reflux, or even just grade one or two, then there is not much or any infection traveling from the bladder to the kidney, so the infection stays in the bladder and doesn’t need to be treated as an infection. Bacteria stays in the bladder and that’s okay. A study on large number of spina bifida patients and found there is very low risk with having infection it the bladder, so we leave it be, and the same would be true for those with sacral agenesis/caudal regression syndrome. If there is reflux back up to the kidneys, then it’s very necessary to keep the urine sterile. That’s why a low dose of antibiotics are prescribed.
SLINGS AND ARTIFICIAL SPHINCTERS:
Slings and artificial sphincters are used when the patient has a lot of leakage. A sling is placed around the urethra to lift it back into a normal position and to exert pressure on the urethra to aid urine retention. The sling is attached to the abdominal wall.
In the old days, artificial sphincters were done, but not as much any more because the equipment fails about every 5-7 years, so they have to be redone. They are much less popular now than they were before.