Some children are born with or develop bladders that do not function properly. These children may have a history of myelomeningocele (spina bifida), have had a traumatic spinal injury, were born with bladder exstrophy (bladder open face at the skin), or have had a congenital bladder obstruction. These patients often have urinary incontinence and may develop dilatation of their ureters and kidneys. Whether due to abnormal nerve innervation of the bladder muscle and/or urethral sphincters, or to chronic obstructive bladder damage most patients with this bladder condition are unable to empty their bladder on their own.
After obtaining pertinent historical information relative to the child or adolescents present condition as well as performing a thorough physical exam, a few x-ray tests and a bladder physiology test are usually necessary to best understand each individual's anatomy and plan future management. A renal and bladder ultrasound is often obtained to assess if there is any dilatation of the kidneys or ureters suggestive of partial ureteral or kidney obstruction or blockage. A VCUG test (voiding cystourethrogram) is also performed to assess the contour of the bladder and bladder neck region, as well as identifying if any ureteral reflux is present. Ureteral reflux is urine flowing backwards from the bladder up toward the kidney instead of being completely urinated or emptied into the toilet by catheterization. Refluxing urine, when infected can lead to permanent renal damage and therefore must be identified and appropriately treated. The next study needed is called a CMG (cystometrogram). This is performed in our office, where a small catheter is placed through the urethra into the bladder and the bladder is filled at a constant flow and important physiologic information is obtained. At the completion of the cystometrogram study, we will be able to identify at what pressure and volume urinary leakage occurs, if it does occur in the individual, as well as assess the compliance of the bladder. Compliance in a normal individual is very good with the bladder filling to large volumes at low pressure. In patient's that have a neurogenic bladder, their compliance may be poor so that as their bladder increases in volume, the pressure also increases. This pressure, once elevated, is vented by creating pressure on the ureters and kidneys with resultant dilatation and/or causing increased urinary leakage. Once all studies and tests have been reviewed, coupled with the individual's symptoms, history, and physical examination, treatment plans are proposed.
If the child or adolescent has a bladder that does not empty on it's own, clean intermittent catheterization (CIC) is highly encouraged. Although it may seem odd and intimidating in the beginning to catheterize your son or daughter, the techniques are often easily picked up as one becomes proficient at it within a day or two. In some cases, clean intermittent catheterization is all that is required to keep the individual dry between catheterizations and his/her upper urinary tracts without any undue pressure. However, it is not uncommon that the child or adolescent may need medicine, to help further relax the bladder, the most common of which is Ditropan. Ditropan is a bladder relaxant which allows the bladder to store urine at higher volumes and lower pressure and stops most bladder contractions that occur without control called uninhibited contractions. Combined with clean intermittent catheterization, Ditropan may allow the child to become dry. Ditropan is an anticholinergic medicine and may have some side effects. If this is the case, other anticholinergic medicines may be tried or the Ditropan medicine may be placed directly into the bladder. Installation of Ditropan into the bladder is done at the completion of clean intermittent catheterization, when the bladder is empty. A 5mg Ditropan tablet is then dissolved in approximately 10cc's of sterile saline and then flushed through the catheter into the bladder. When Ditropan is taken this way (known as intravesically), the relaxation of the bladder is fully maximized even more so than when Ditropan is taken orally or by the mouth.
Management of children or adolescents with neurogenic bladder conditions are individualized to each patient and their parents. Our main goals are to preserve function of the kidneys and then to obtain full urinary continence in between urinating which is most often assisted with catheterizations if catheterization is required. In those individuals in which we have fully maximized their medical management and clean intermittent catheterization skills and they remain wet or are having progressive dilatation of their ureters or kidneys, we encourage moving ahead to surgical intervention to optimize their condition.
There are three common scenarios in which patients may require surgical intervention. In the first the bladder has good compliance, but the urethral resistance is low. These patients have good preservation of their kidneys, but are constantly wet no matter how compliant they are with their medications or catheterization. These patients will need to have something done to increase their bladder outlet resistance. There are currently 3 procedures that we consider as options to increase resistance at the bladder outlet, the artificial urinary sphincter (AUS), bladder neck sling or cinch, and periurethral injection of contigen. In the second scenario, the bladder compliance is poor but the urethral resistance is high. These patients are at risk for increased renal pressure and eventual renal damage. These patients often require bladder augmentation. Standard augmentations with either colon or ileum bowel segments are most often performed. On occasion a stomach segment may be used and when possible we offer a seromuscular colocystoplasty lined with urothelium. The latter type of augmentation preserves the bladder lining and places the colon segment stripped of it's mucosal lining into the bladder. The advantage of this bladder augmentation is that it is non-mucous secreting. The third scenario is a combination of the first and the second with a high pressure bladder and low urethral resistance. These patients will often require both bladder augmentation in addition to a procedure to łtighten˛ the bladder neck region.
In patients who are on clean intermittent catheterization and are having difficulty passing the catheter through the urethra into the bladder or would just prefer an easier more accessible rout for catheterization we've been able to offer them the Mitrofanoff procedure. The Mitrofanoff is a catheterizable non-leaking dime size stoma which we conceal by placing it in the base of the umbilicus (belly button). The appendix is used to communicate the bladder to the umbilicus and is fashioned so to accept an 8-14 french catheter for emptying the bladder and not allow urinary leakage. The patient simply lifts his/her T-shirt places the catheter into the concealed opening in the umbilicus and then empties the bladder. For children or adolescents who are confined to a wheelchair and/or this would allow for their catheterizations independent of others' assistance, this has been most gratifying.
In patients who have achieved complete urinary continence but who have poor bowel control so that they con not make the transition from diapers to underwear there have been several technical advancements that we have been offering patients. If the use of diet, suppositories or oral medicines to control bowel function have been unsuccessful and enemas are difficult to perform, the Malone Procedure or the cecostomy button have worked in these circumstances. The Malone Procedure uses the appendix (if not already used for a Mitrofanoff) which is tunneled into the cecum (the first portion of the large bowel) and brought up to the skin level as a dry dime size stoma. This stoma is then catheterized once per day and fluid is placed into it flushing the colon clean of stool. This process takes between 10-25 minutes. The patient is then without fecal soilage between daily irrigations. The cecostomy button is the same principle without a stoma but with a plastic device the size of a quarter at the skin level which is connected to the cecum. Flushes are done in an identical fashion as with the Malone.
Overall, we can offer children or adolescents with neurogenic bladders a wide array of successful options to both preserve their renal function and gain urinary as well as fecal continence. All treatment plans are individualized for each patient starting with the most conservative measures first then advancing as necessary to achieve the goals desired.