Myelodysplasia and Neurogenic Bladder Dysfunction Treatment & Management

Updated: Jul 09, 2019
  • Author: Terry F Favazza, MD; Chief Editor: Marc Cendron, MD  more...
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Treatment

Medical Care

Medical care of children with myelodysplasia who have a neurogenic bladder requires constant vigilance and adaptation to new problems. Therapy is based on a few basic goals, as follows:

  • To ensure safe intravesical pressures
  • To prevent urinary stasis and urinary tract infections (UTIs)
  • To promote urinary continence

The ultimate goal of medical therapy is to preserve renal function. In older children, medication may help maintain continence.

Infection

UTIs are common in children with myelodysplasia. Antibiotics are used when indicated to treat acute infections. In the absence of reflux, patients with UTIs are treated symptomatically. Patients with vesicoureteral reflux (VUR) are often placed on prophylactic antibiotics to reduce the chance of upper UTI or pyelonephritis. Bacteriuria is seen in as many as 55% of individuals who have received clean intermittent catheterization (CIC). Patients who are completely asymptomatic do not need treatment.

Reflux

Reflux occurs in 3-5% of infants with myelodysplasia and is usually associated with detrusor hyperreflexia or detrusor-sphincter dyssynergy.

Treatment consists of antibiotic prophylaxis to prevent infection, anticholinergic medications to lower detrusor filling and voiding pressures, and a method of bladder emptying (most commonly, CIC). In children with lower-grade reflux who empty their bladders completely, treatment may be limited to prophylactic antibiotics. In children with high-grade reflux, CIC is started to ensure complete emptying.

Children who are unable to empty their bladders, regardless of reflux, are treated with CIC (see below). Children with detrusor hyperreflexia (with or without hydronephrosis) are started on anticholinergic therapy to decrease intravesical pressures and possibly decompress the upper genitourinary (GU) tract. Reflux treated in this manner has shown a dramatic response, resolving in 30-55% of children. Avoid the Crede maneuver (voiding by suprapubic pressure) in children with reflux, because it can increase pressures and aggravate the degree of reflux.

Inability to empty bladder

Because most patients with myelodysplasia are unable to empty their bladders spontaneously, numerous methods have been devised to potentiate bladder emptying. Initially, large numbers of patients underwent urinary diversion; however, the frequency of renal failure was substantial. This changed dramatically with the introduction of CIC.

Bladder catheterization on a regular basis is a safe, effective method of emptying the bladder and, if performed under clean conditions, does not appear to significantly increase the risk of infection. More than any single concept, the practice of CIC has changed the treatment of and approach to patients with neurogenic bladders. [8]

Currently, urinary diversion is rarely performed in pediatric patients.

Incontinence

Although not an issue in infancy, continence becomes more important as patients age. When children reach school age and social interactions increase, managing incontinence becomes a larger priority.

Medical therapy consists of anticholinergic medications to increase the functional bladder volume and to reduce involuntary and uninhibited bladder contractions. This serves to decrease urgency and incontinence and potentially to increase the bladder's functional storage capacity.

Additionally, alpha agonists have been used infrequently in children to increase sphincter tone at the bladder neck, initiating a state of urinary retention in an effort to decrease incontinence. Thus far, the therapy has had limited use in patients with myelodysplasia.

Studies have reported the use of botulinum toxin directly injected into the detrusor of children with myelodysplasia as being successful in treating incontinence. [9, 10, 11, 12, 13, 14]  However, this treatment is not yet widely used.

Impaired bowel function

Often, children with myelodysplasia have disturbances of bowel as well as urinary function. This is managed most commonly with mild laxatives, such as mineral oil, combined with enemas or digital stimulation to facilitate removal of bowel contents.

Constipation can affect bladder emptying adversely via a mechanism that is not yet fully understood but is likely to be related to altered tone of the pelvic floor musculature or the physical compression of hard stool distorting the geometry of the bladder. The need for a program to combat constipation by maintaining soft stools and facilitating complete evacuation of bowel contents is an integral part of treatment in children with myelodysplasia.

Special concerns

A remarkable number (in some studies, as many as 45%) of patients with myelodysplasia develop hypersensitivity to latex, a phenomenon believed to be related to recurrent exposure of peritoneal and mucosal surfaces to medical devices containing latex throughout childhood. Reactions range from contact dermatitis to anaphylaxis and cardiovascular collapse.

Consider all patients with myelodysplasia of spina bifida to have a latex allergy, and make every effort to avoid exposure to latex from birth. Gloves, catheters, crib pads, and bottle nipples are all potential sources and may exacerbate hypersensitivity.

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Surgical Care

Surgery for neurogenic bladder, though once performed on most patients, is now primarily reserved for patients who have progressive renal damage despite maximal medical therapy, those with extremely noncompliant bladders, or those who wish to improve their continence.

Most procedures are designed to allow adequate low-pressure bladder storage (thereby protecting the upper GU tract), to correct persistent reflux and prevent renal scarring, or to aid with continence. Experimental intrauterine fetal surgery performed to limit future morbidity is under investigation at some centers. [15, 16, 17, 18]

Intrauterine surgery

Studies of surgically created neural tube defects in rats demonstrate that the exposed tissue in the myelomeningocele sustains secondary injury from mechanical and chemical factors during its prolonged exposure to the uterine environment. This has led to research on the effects of in-utero closure of the defect.

Although intrauterine closure is considered experimental because of the limited numbers performed, preliminary findings appear to indicate that it can be accomplished with minimal morbidity to the fetus and the mother and that it may decrease the need for ventriculoperitoneal (VP) shunting later in life.

Whether in-utero repair improves the neurologic outcome in these patients remains unclear.

Procedures to correct vesicoureteral reflux

Ureteral reimplantation

Ureteral reimplantation can be performed in patients who have recurrent symptomatic UTIs despite adequate bladder drainage and antibiotic prophylaxis or in patients who have persistent high-grade reflux with demonstrated renal scarring.

The purpose of the procedure is to create a nonrefluxing connection between the ureter and the bladder. Most often, the procedure is performed by tunneling the ureter beneath the detrusor muscle. This treatment is very effective, provided that a regimen is implemented to ensure a low-pressure reservoir and bladder emptying.

Dextranomer/hyaluronic acid (Deflux) injection

This is a much less invasive way to address VUR. Deflux (a viscous gel consisting of dextranomer microspheres and hyaluronic acid) is injected cystoscopically just beneath the ureteral orifice to prevent reflux. It is performed as an outpatient procedure with excellent success rates (70-85% with a single injection) and can be repeated if necessary. [19, 20]

Vesicostomy

In infants who cannot be catheterized or who demonstrate worsening renal function despite medical therapy and CIC, cutaneous vesicostomy can be performed to establish adequate bladder drainage. In this procedure, the bladder is brought out to the skin, and urine drains continually into a diaper. Vesicostomy is an effective temporary procedure that may be reversed at any time.

Bladder augmentation

Bladder augmentation is an option in patients with small bladder capacity and poor bladder compliance despite maximal medical therapy. [21]  By anastomosing a detubularized segment of bowel to the bladder, capacity can be increased and storage pressures can be lowered, minimizing upper GU tract deterioration and improving continence.

Depending on the segment of bowel used, problems with metabolic derangements, mucous production, stone formation, and hematuria can develop but usually respond to medical therapy. If incontinence is a significant problem, a bladder neck sling procedure can be performed along with bladder augmentation.

Urinary diversion and undiversion

In current practice, formal urinary diversion for neurogenic bladder is very rarely performed. The risks of major abdominal surgery, metabolic derangements, and long-term upper GU tract deterioration are present with urinary diversion. Since the advent of CIC, some patients who underwent incontinent urinary diversion as infants have undergone successful undiversion with bladder augmentation.

Procedures for incontinence

As patients grow older, continence begins to play a larger role in their lives. Several procedures have been developed to improve continence, with the hope to promote more independent living.

Dextranomer/hyaluronic acid (Deflux) bladder neck injection

This procedure consists of cystoscopically injecting dextranomer/hyaluronic acid into the bladder neck to increase outlet resistance. It has been shown to improve continence, but complete cures are unpredictable, and the durability of the procedure contineus to be evaluated. [22, 23, 24, 25, 26]

Bladder neck sling

This procedure entails placing a sling of either autologous tissue or synthetic polypropylene mesh beneath the urethra in order to increase outlet resistance. There are several variations to the procedure, but overall success rates range from 60% to greater than 85%, with a number of patients becoming fully continent.

Detrusor myoplasty

This procedure, though not commonly done, has the possibility of increasing bladder contractility in some patients. It is performed by harvesting the patient's latissimus dorsi muscle and microsurgically transplanting it so that it wraps around the bladder. This has allowed some patients to spontaneously void and reduce their dependence on catheterization. [27]

Stem cell injection

Stem cell injection remains experimental in this setting, but preliminary studies showed some promise. In this procedure, stem cells are cystoscopically injected into the urinary sphincter. The goal of therapy is to increase sphincter activity and improve continence. Animal and early human trials have been encouraging. [28]

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Diet

Generally, dietary management is the first step to achieving fecal continence. Both constipation and diarrhea must be avoided. The goal is to provide enough bulk to have one bowel movement per day at a socially acceptable time. Usually, digital stimulation, suppositories, or enemas are used to regulate the timing of bowel movements.

If the child has difficulty with constipation, altering the diet to include more fiber and adding bowel lubricants (eg, mineral oil) may help regulate bowel movements. Adequate bowel and bladder management are crucial for optimizing social, school, and work activities.

In addition, because of neurologic and orthopedic issues, mobility is limited, reducing the patient's ability to exercise. These patients are at high risk for obesity.

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Activity

Children with myelodysplasia often have limited development or motion of the extremities; however, no specific activity limitations are required. Children are encouraged to be as active as possible within the limitations of the defect.

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Consultations

Patients with myelodysplasia have a multitude of issues that require constant observation. The following consultations may be warranted:

  • Intervention by a neurosurgeon is needed, starting at birth; patients require initial closure of the spinal defect, cerebrospinal fluid (CSF) shunting, and monitoring for cord tethering or shunt malfunction
  • Often, consultation with a neurologist is required to define defects and watch for any change in symptoms
  • If significant bone abnormalities are present, consultation with an orthopedist may be necessary
  • Parents, and eventually the child, undoubtedly need the support of a psychologist to help deal with the struggles inherent in raising a child or growing up with myelodysplasia
  • Physical therapy may also be needed
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Long-Term Monitoring

General follow-up care

That patients require lifelong supervision and monitoring of renal function cannot be stressed enough. Renal failure can progress slowly or occur with startling rapidity.

Check postvoiding residual volumes every 6-12 months and renal function (blood urea nitrogen [BUN] and creatinine levels) yearly. Perform renal ultrasonography yearly. Many advocate a yearly urodynamic study for the first 5 years of life, followed by biennial evaluation. Perform a repeat urodynamic study any time the patient experiences any change in neurologic symptoms.

Tethered cord

As children age, differing growth rates of the vertebral bodies and the spinal cord can add a dynamic element to the lesion. Fibrosis surrounding the cord at the site of meningocele closure can tether it during growth. This can lead to changes in bowel, bladder, and lower-extremity function. If these are noted, evaluation with magnetic resonance imaging (MRI) is indicated. From the urologic standpoint, MRI and a repeat urodynamic study are warranted when the patient has a change in symptoms or undergoes any neurosurgical procedure.

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