Spina Bifida Family Support

www.spinabifidasupport.com

The goals of urologic care and management of a child with spina bifida center on maintaining normal renal function and gaining urinary continence.  The significant achievements in other specialties particularly neurosurgery have unmasked the importance of maintaining a healthy bladder environment for urinary storage and elimination.  Over 90% of children with spina bifida will have a normal upper urinary tract (kidney and ureter) at the time of birth.  With no followup, half of the children will suffer considerable upper urinary tract damage due to lower urinary tract (bladder and urethra) hostility.  With appropriate urologic management, children at risk for renal damage can be identified early and intervention undertaken to prevent long term compromise of kidney function.  During the first several years of life, the urologic focus on a child’s health is based on maintaining normal kidney function.  As the child begins to approach school age, interest will be directed toward gaining urinary continence.  There are overlapping factors which can effect both kidney function and urinary control.

Several principles must be recognized that are not unique to urology or to children with spina bifida.  The most important is that no two children are alike.  As you meet other families and discuss issues related to their child’s problems and successes, you must remember that what is appropriate and effective for one child may or may not be appropriate for your child.  From a medical point of view, we are unable to simply look at a child’s back and determine what type of bladder or kidney function they will have.  It is for that reason, children with spina bifida require very close urologic evaluation.

The following description of urologic care is based on a protocol used at our Children’s Hospital.  It works well for our children because of our particular resources.  The philosophy presented here can be carried out elsewhere.  However, there are multiple approaches for any particular problem and if your child is managed differently that should not be considered incorrect.  Institutions create a protocol based on their program's philosophy and resources.  The critical factor is that you continue with consistent urologic followup and actively participate in the urologic management that has been recommended for your child.
 
Children will be managed based on the general philosophy of their institution.  Two general management options are available; proactive (attempting to identify children at risk and treating them before a problem occurs) or retrospective (following a child closely and at the first sign of any adverse change begin management).  Pediatric urologists have had good results treating children in either fashion.  The protocol we use is based on a proactive philosophy.
 
Newborn

The infant bladder functions as a reservoir for urinary storage and elimination.  Babies with normal bladder function void strictly by reflex.  The fact that a baby’s diaper is wet does not equate with normal voiding.  Studies need to be performed to determine at what pressure urine is stored and eliminated.  It is also helpful to learn whether unusual bladder contractions are occurring and whether the external urinary sphincter (muscle) is relaxing appropriately when a bladder contraction does occur (This is called bladder sphincter synergy.  When the external urinary sphincter contracts at the same time the bladder contracts, abnormally high voiding pressure occurs which is called bladder sphincter dyssynergy). 

In order to understand how an infant is voiding, special bladder studies (urodynamics) are required.  A baseline assessment of the kidneys should be established with a renal sonogram.  Blood work is obtained to assess kidney function in children with abnormal kidney changes.  We begin intermittent urethral catheterization on all newborns with spina bifida.  Our protocol quickly tapers catheterization based on the volume of urine collected.  We have found it beneficial to begin catheterization early in order to identify the small group of children who are at risk for carrying a significant residual urine.  In addition, all families become familiar with the technique of catheterization which can be very helpful if this treatment is recommended at a later date. 
 
In order to determine whether a child is at risk for injuring their kidneys, bladder function must be assessed.  This can be undertaken with a urodynamic study.  This study assesses the bladder’s ability to store and eliminate urine.  The urodynamic study is complicated and uncomfortable particularly when the child has normal sensation.  The test can be combined with x-ray imaging of the bladder in order to evaluate any structural bladder changes due to nerve damage, and the presence of vesicoureteral reflux (urine backing up, into the kidneys).  Some of the infants discharged from our hospital will be maintained on intermittent catheterization; all neonates will be placed on a low dose of an antibiotic until their first urodynamic study.  Whether medication is continued will be determined by the results of the study.  During the urodynamic study, the bladder is checked for changes which are considered hostile to kidney growth and development (e.g. elevated storage pressure, abnormal bladder contractions, bladder sphincter dyssynergia).  When vesicoureteral reflux is noted, children are continued on prophylactic antibiotics.
 
The results of the urodynamic study will determine what follow-up is required.  When the urodynamic study is normal or shows favorable bladder function, the child will be scheduled for a return visit in appropriately six months to reassess their kidneys with a sonogram and a urine culture.  The urodynamic study will be repeated yearly in order to identify bladder changes which can not be recognized by the baby’s daily voiding routine.  Significant bladder changes noted on multiple urodynamic studies may reflect the effects of a chronic urinary infection, progressive nerve damage, or the development of a tethered spinal cord.  Often times the first indication of spinal cord tethering is manifested by changes in bladder and bowel function.  In infants, it is difficult to determine any adverse changes unless urodynamic analysis has been performed which shows those changes.

When a hostile bladder environment is found on urodynamics, treatment will be recommended based on the specific abnormality.  For most children, treatment will follow various progressive steps, beginning with the easiest to perform and the least involved.  Some children simply have a large residual of urine which can be eliminated by the Crede’ technique (pushing on the bladder with your fingertips above the pubis).  This technique is very helpful but not appropriate for all children particularly if vesicoureteral reflux is present or if the child has been shown to have what is described as the Crede’ reflex (contraction of the external urinary sphincter with compression of the bladder).  Using the Crede’ technique when these problems are present can result in high-pressure elimination of urine which over a long period of time is detrimental to the kidneys.  When excessive bladder contractions (hyperreflexia) are present, medication will be started in an attempt to control the contractions and decrease pressure on the kidneys.  The commonly used medications are oxybutynin (Ditropan), tolterodine (Detrol), hyoscyamine (Levsin) and propantheline.  Medication is first given in an oral fashion but if poorly tolerated, some medications can be given directly into the bladder if the child is catheterized.  
 
Intermittent catheterization is the most important urologic advancement over the past 20 years in the treatment of the neurogenic bladder.  Intermittent catheterization successfully eliminates urine from the bladder mimicking normal voiding.  This can decrease urinary infections and reduce the effects of hostile bladder pressure on the kidneys.  Intermittent catheterization is performed in a clean but not sterile fashion.  All children will eventually have bacteria in their bladder but only about 50% will have symptoms related to the bacteria resulting in a clinical urinary tract infection.  Asymptomatic bacteria in the urine does not need to be treated.  Symptomatic infections are usually easily controlled.  Over 50% of the children who catheterize will require a low dose of an antibiotic in order to remain symptom free.
 
There is a small group of infants with significant bladder hostility who do not respond well to medication or catheterization.  When this occurs, operative intervention may be required.  The most effective operative procedure to eliminate urine and decrease pressure in an infant is creating a vesicostomy.  This is a small opening in the bladder which connects to the abdominal wall and is positioned between the pubic bone and the umbilicus.  When a vesicostomy is created, it is well tolerated and reversible.  It is common for the infant to develop a rash around the opening because of continuous urine dribbling.  This will improve with time but may initially require topical treatment.  The vesicostomy provides excellent bladder decompression and usually improves the pressure on the kidneys due to high-pressure bladder storage and high pressure voiding.  
 
Urinary Continence

As the child ages the upper urinary tract should remain normal and interest can be shifted to focus on gaining urinary continence.  The age at which a child begins to work toward urinary continence is individualized based on their physical capabilities and social situation.  It is practical to consider urinary continence when a child enters school.  While this is a realistic goal, it may not be appropriate for all children.  In order to gain urinary continence it will be important to adhere to a consistent voiding program.  This voiding program may include medications, intermittent catheterization, and possibly operative reconstruction.  When a urinary continence program is initiated, it should be continued while the child is in a structured school environment.  
 
Some children with spina bifida can void on their own and achieve normal urinary continence. The majority of children, however, will require a combination of treatment measures similar to what was described above.  Urodynamic bladder testing will help assist the pediatric urologist in developing a program that is specific for your child’s problem and needs.  Most often, conservative reversible treatment regimens are initiated first which include medications and catheterization. Only when this management has been maximized will operative intervention be recommended.
 
In order to achieve urinary continence, three important factors of bladder function need to be assessed.  The first is the ability of the bladder to store an adequate volume of urine at low pressure.  The second is the ability of the bladder to contract or eliminate urine.  And the third is the ability of the bladder neck and/or external urinary sphincter to remain closed when the child is not voiding in order to remain dry.  Any one or all three of these factors may be dysfunctional.  Treatment will be dependent on what abnormality is present.  
 
When the bladder capacity is small and/or resting bladder pressure is elevated, medications can be tried to enlarge the capacity and decrease the pressure.  The commonly used medications are oxybutynin (Ditropan), tolterodine (Detrol), hyoscyamine (Levsin) and propantheline.   When medications have been unsuccessful the bladder may need to be enlarged or augmented.  Various tissues have been used to augment the bladder, most often the small or large bowel is selected.  There are advantages and disadvantages of using any segment of bowel.  The large bowel is in close proximity to the bladder and its large diameter results in a smaller segment that needs to be isolated.  But there is a greater discharge of mucus and there can be an effect on bowel control for some children.  For those reasons, small bowel (ileum) is the common choice.  When bowel is used, it is reconfigured into a “cup patch” which is added to the bladder.  Reconfiguring the bowel creates a reservoir of greater capacity and also decreases elevated pressure which can occur when the bowel segment contracts.   Some children might benefit from use of stomach, particularly if they have a limited amount of bowel.   Stomach results in minimal mucus secretion and less potential for bladder stones but it can be a source for discomfort and minor bleeding due to normal acid secretion from the segment.
 
When any segment of bowel is used in urinary reconstruction, there is a long-term risk for a bowel obstruction.  This risk persists throughout life but remains at a low percent.  Bowel naturally secrets mucus and mucus needs to be intermittently irrigated from the child’s bladder.  Failure to do so will result in excessive accumulation of mucus, poor urinary drainage, increased risk of urinary infections, odor, and the formation of bladder calculi.  Draining the bladder completely and irrigating the bladder on a routine basis helps diminish those risks. 
 
Augmenting the bladder with a portion or bowel will likely result in the child requiring intermittent catheterization.  Therefore, it is beneficial for all children and parents to feel comfortable with the technique of catheterization for several weeks prior to reconstructive surgery.   After the augmentation, it is essential that catheterization is maintained on a very strict schedule.  Often times when children are left to catheterizing themselves, poor technique will lead to either incomplete emptying or excessive stretching of the bladder and augmented segment.  Over a long period of time a bladder rupture can occur which can be a life threatening problem.  Teenagers often are complacent regarding the importance for maintaining a strict catheterization schedule and need to be supervised when they become responsible for their own care.

When the bladder neck or sphincter are not strong enough to hold the urine within the bladder, various operative techniques are used to tighten each area.  Multiple procedures are available because there is no single procedure that is effective for all of the different types of outlet problems.  Some operative techniques use natural bladder tissue which is fashioned into a “one way valve”.  This allows a catheter to be passed for drainage but prevents urine from flowing out.  Another technique uses a strip of fascia (strong tissue that surrounds a muscle) which is placed around the bladder neck and tightened like a belt.  An artificial (mechanical) sphincter can be placed and is helpful for selected children.  Most bladder neck repairs will require the child to catheterize.  The specific bladder neck operation that is recommended for your child will be dependent on the problem that is identified. 
 
It should be appreciated that intermittent catheterization is a common theme for maintaining normal kidney function and urinary continence.  Most often catheterization is begun through the normal position of the urethra (the tube that connects the bladder to the vaginal opening in girls, and travels through the penis in boys).  As the child begins to mature, it may become more difficult to catheterize through the natural urethra.  In addition, some children who use a wheelchair have difficulty reaching their genital area for self catheterization.  It is possible to make a small catheterizable tube that enters the bladder from the body wall above the pubic region.  The stoma (opening) of the tube can be hidden within the umbilicus or positioned very low on the abdominal wall.  The tube is commonly created by using the appendix (Mitrofanoff) or a portion of the small bowel (Monte/Yang). 
 
Bowel Control

It is practical to consider bowel continence at the same time a child is working toward urinary continence.  It is essential for a child to maintain an appropriate stool consistency which can be achieved by a diet that includes plenty of fluid and fiber.  Some children benefit from fiber supplementation.  Even when the stool is of normal consistency, some children need to have assistance in eliminating the stool from their rectum and colon.  There are various techniques recommended including digital manipulation, rectal suppositories, and enemas.  When enemas are employed, only the very terminal end of the colon is washed free of the stool leaving the bulk of stool in the remainder of the colon.  When the above techniques are unsuccessful at achieving bowel continence, it is possible to irrigate the entire colon at its origin (the cecum).  In this situation the appendix or a small tube can be inserted in the cecum with the end brought to the abdominal wall.  This tube is then irrigated, washing the entire colon.  Colonic irrigation may take anywhere from 30 to 90 minutes.  The children remain in the bathroom while their colon is irrigated at which time they can read or proceed with homework.  This procedure is called the ACE (antegrade colonic enema). 
 
Transitional Years

When the child becomes a teenager, the urologic care should slowly make a transition from parent or caretaker to the teenager.  This transition can be difficult for both the teenager and parent or caretaker.  Teenagers should be educated about their particular problem(s) and the reasons they need to perform certain activities.  It is important that the adult allow the teenager to assume the responsibility for bladder and bowel needs.  While the parent or caretaker provides independence, it is necessary for them to continue close monitoring assuring that the teenager is accomplishing the goals of the expected treatment program.  
 
The teen years are a struggle for children with spina bifida just as they are for other children.  However, the medical consequences resulting from the lack of attention paid to specific personal needs is much greater.  Parents may need to refocus their child on what is required to maintain healthy kidneys and urinary continence.
 
With appropriate urologic care most children with Spina Bifida will transition through life with normal renal function and achieve urinary continence.

This information does not constitute medical advice for any individual.  As specific cases may vary from the general information presented here, Spina Bifida Family Support advises readers to consult a qualified medical or other professional on an individual basis.

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