Bladder and Urethra

Introduction

The bladder and urethra normally function as a coordinated unit to store and discharge urine from the body. Both structural and functional disorders of the bladder or urethra can be responsible for bleeding, incontinence, infection, discomfort, pain, and obstruction that can cause upper-tract deterioration to the point of compromising renal function. This chapter focuses on the major diseases and dysfunctional conditions of the bladder and urethra as a unit and the management of such problems.

The bladder and upper urethra are composed of bundles of smooth muscle fibers arranged in a reticular lattice, the outermost bundles being more circular and the inner bundles more longitudinal in orientation at the bladder neck. The smooth muscle bundles blend into the striated muscle of the external urethral sphincter, which is derived from the pelvic diaphragm. The bladder is lined by transitional epithelium, which is sensitive to irritants such as bacterial toxins and various urinary crystals. The urethra and trigone are especially sensitive, and the presence of any irritant in these areas can create significant discomfort.

Proper function of the lower urinary tract depends on intact autonomic and somatic nervous innervation. The detrusor muscle of the bladder is innervated by both sympathetic and parasympathetic fibers. Storage functions are mediated by the sympathetic component, which arises from spinal levels T10–L1. The chemical mediator of this process is norepinephrine, which acts on β-adrenergic receptors in the fundus of the bladder and causes muscle relaxation for low-pressure storage of urine. The same sympathetic stimulus acts on the β-adrenergic receptors of the trigone, bladder neck, and proximal urethra to increase internal sphincter activity and promote continence during urine storage by maintaining outlet resistance. The external urinary sphincter, innervated by the pudendal nerve, progressively increases its tone as the bladder fills, providing additional resistance. As the child develops, the external sphincter may be consciously contracted at times of urgency or stress to prevent the unwanted passage of urine. Properly coordinated function of the external urinary sphincter relies on an intact sacral reflex arc, which should be well developed in normal infants but is variably functional in infants with spinal cord abnormalities or pelvic lesions. ,

The sensation of bladder fullness initiates a response in toilet-trained children that causes them to discharge their urine. When ready, the parasympathetic nervous system, via acetylcholine, causes cholinergic fibers of the detrusor to contract, resulting in a widened and shortened proximal urethra, eliminating its resistance to outflow. With relaxation of the volitional external sphincter, the bladder empties by sustained and complete contraction of the detrusor, leaving a residual urine volume of <5 mL.

Spinal pathways connect the sacral micturition center with three areas in the brain stem, collectively referred to as the pontine micturition center. This center functions to inhibit urination during storage and to produce external sphincter relaxation during the voiding phase. Above this level are areas of cerebral cortex that oversee and modulate the autonomic process. It is the mature, integrated function of all these components that produces urinary continence.

Toilet training is, in large part, a learned phenomenon. It requires adequate recognition by the brain that micturition would be socially unacceptable in a given situation. With maturation, the bladder gains capacity, allowing for longer intervals between voiding. The approximate bladder capacity in milliliters may be estimated for an infant by formula 38 + [2.5 × Age (months)]. For older children, the formula [Age (years) + 2] × 30 can be utilized. Infants void 20 times per day, which decreases to about 10 times per day by age 3 years. The child also learns to resist the urge to void by voluntary contraction of the external sphincter until the detrusor contraction passes and the bladder once again relaxes. Thus, toilet training depends on the development of voluntary cooperation between detrusor and sphincter functions. At times, this process can become dysfunctional. Finally, full bladder control relies on the child developing volitional control over the spinal micturition reflex to be able to initiate or inhibit detrusor contractions. Most children attain day and night continence by 4 years of age.

Urinary incontinence may be due in part to immaturity of the bladder and its nervous system connections. The usual sequence of bladder development is linked to bowel development and is as follows: (1) control of bowel at night, (2) control of bowel during the day, (3) control of bladder during the day, and (4) control of bladder at night.

Childhood Incontinence

Incontinence is the term used for the unintentional loss of urine after toilet training is achieved. The following definitions are clinically useful:

  • Enuresis or nocturnal enuresis : Complaint of intermittent incontinence that occurs during periods of sleep. If it occurs during the main sleep period, then it could be qualified by the adjective “nocturnal.” The patient has to be asleep when enuresis happens and is usually unaware of it.

  • Primary nocturnal enuresis : Complaint of intermittent incontinence that occurs during periods of sleep that has been present lifelong.

  • Secondary nocturnal enuresis : nighttime incontinence following a dry period of at least 6 months

  • Daytime incontinence : Involuntary loss of urine while awake, after toilet training

  • Overactive bladder (OAB): Urinary urgency, usually accompanied by increased daytime frequency and/or nocturia, with urinary incontinence (OAB-wet) or without (OAB-dry), in the absence of urinary tract infection or other detectable disease.

  • Stress incontinence : Complaint of involuntary loss of urine on effort or physical exertion including sporting activities, or on sneezing or coughing.

  • Urgency incontinence : Complaint of involuntary loss of urine associated with urgency.

The discussion of incontinence is divided into sections on nocturnal enuresis and daytime incontinence, realizing that some children have both. The current recommendation for children with nocturnal enuresis and daytime incontinence is to focus on daytime treatment first, followed by nocturnal enuresis therapy.

Nocturnal Enuresis

About 15%–20% of children at 5 years of age continue to have bed wetting. As so many children still wet at night before this age, it is considered within the range of normal and not termed nocturnal enuresis. After age 5, night wetting resolves at the rate of about 15% each year. By age 15 years, it has resolved in 99% of children.

Etiology

Children with monosymptomatic nocturnal enuresis are, in general, physically and emotionally similar to their peers. The difference lies in their inability to awaken during sleep when their bladder is full or contracts. The etiology of this disorder is likely complex, and several factors should be considered.

Genetic

Family history is significant. If both the parents had enuresis, close to 80% of their offspring will as well. If one parent was affected, 44% of the offspring are affected. If neither parent has a history, only 15% of their children have this problem. ,

Psychological

Psychological stress can induce nocturnal enuresis in certain children. Secondary nocturnal enuresis often raises this concern. Common factors include divorce, changing homes, birth of a new sibling, trouble at school, or just starting school.

Developmental

As children grow, bladder capacity increases significantly each year at a proportion greater than urine volume produced. , , Volitional control over bladder and sphincter also may mature at variable rates and may be related to subtle delays in perceptual abilities or fine motor skills.

Urodynamic

Studies show that enuretic episodes occur when the bladder is full, and they simulate normal awake voiding. Although nocturnal enuretic patients have more nighttime unstable bladder contractions, these are at low pressure and do not cause leakage.

Night wetting appears to occur in three ways: wetting associated with significant restlessness and visceral and somatic activity (deep respirations), wetting with a quick contraction and minimal movement, and wetting with no central nervous system response (parasomnia).

Sleep Disorders

Parents of children with nocturnal enuresis are generally convinced that these children sleep deeply and are difficult to arouse. However, this is probably not true. Enuretic patients sleep no more deeply than age-matched controls, wet in all stages of sleep, and show no different awakening patterns. Wetting episodes occur as the bladder fills throughout the night. A more recent systematic review showed conflicting findings in the differences in polysomnography between patients with nocturnal enuresis and controls, but sleep questionnaires did show more problems in patients with nocturnal enuresis. These included parasomnias, breathing disorders, and daytime sleepiness.

Antidiuretic Hormone

Antidiuretic hormone (ADH) is released from the pituitary in a circadian rhythm so that levels are higher at night and thus diminish urine output. Some children may undersecrete ADH at night resulting in bed wetting. Although some patients follow this pattern, others do not; the altered circadian patterns appear to normalize with maturation.

Evaluation

The screening evaluation should include a history, physical examination, and urinalysis. If these are normal, then no other testing is needed because organic disease rarely causes monosymptomatic nocturnal enuresis. Any associated anomaly or problem such as urinary tract infection (UTI), sacral anomalies, or complex enuresis patterns warrant radiographic investigation.

Treatment

The treating physician should recognize enuresis as a symptom and not a disease. Realizing that there may be more than one cause permits the physician to consider more than one treatment option. Specific treatment is generally discouraged before the age of 7 years. Certain measures are sensible in all nocturnal enuretic patients: void just before getting into bed, avoid excessive fluid intake during the evening hours, and avoid caffeine after 3:00 p.m.

Enuretic Alarms

Wetting alarms are devices that fit in the underwear of the patients. When moistened, an alarm is sounded. This type of conditioning therapy requires a motivated patient and parents. A variety of products are available with either an audio alarm, a vibrating alarm, or both. In our experience, the best alarm is simply one that is easy to set up and is able to wake the child. The parent may need to help arouse the child, take him or her to the bathroom, and reset the alarm. This may occur multiple times each night, particularly at the onset of therapy. In two studies, wetting alarms were shown to give the best long-term results when compared with other treatments. , The length of treatment to achieve dryness varied between 18 nights and 2.5 months. Relapse may occur in 20%–30% of treated children, but retreatment can be successful.

Medications

Imipramine, a tricyclic antidepressant, has been used for many years. The exact mechanism of action is unknown. Initial success has been reported in the 50% range. However, a recent review showed only a 20% success with a relapse rate of 96%. Clinical practice reveals that the longer the initial treatment, the more benefit before the effect wanes. It is suggested that the medication be weaned slowly rather than stopped abruptly. Side effects include anxiety, insomnia, dry mouth, nausea, and personality changes. An overdose can cause fatal cardiac arrhythmias. Therefore, medication safety in the home is important. Imipramine may improve response rates to the enuretic alarm.

Desmopressin is an analog of ADH that mimics its urine-concentrating activity without the vasopressor effect. The effect is dose dependent, usually requiring 200–400 μg/day for success.

Complete dryness rates may be highest in patients with a strong family history of success. Efficacy and safety have been demonstrated in a number of studies, but long-term success remains lower than with alarm systems. Desmopressin can occasionally have side effects, including electrolyte changes, nasal irritation, and headaches. Desmopressin is available as a nasal spray. However, this route is not approved for treating nocturnal enuresis due to a higher incidence of hyponatremia. Parents should be warned to avoid overhydration to prevent this side effect.

Oxybutynin is the most common drug used for enuresis. It is effective when day and nighttime wetting occur in the same patient, but has no benefit over placebo when nighttime wetting is the only symptom.

General Treatment Approach

Although many parents consider bed wetting a problem, they often do not consider it significant enough to treat, especially when medications are being considered. For those desiring treatment, an enuretic alarm and desmopressin are considered the first-line therapies in cases of primary nocturnal enuresis. It is often most reasonable to begin with an enuretic alarm, as it has the highest response rate, no side effects, and the lowest relapse rate. Combination therapy with imipramine may be considered when the alarm is not successful. If desmopressin has proved effective in a specific patient, the patient and family may choose to keep it available and use it only on specific nights when dryness is especially desired (e.g., sleepovers, campouts). Some patients do not respond to therapy, and time, reassurance, and a caring approach are all that can be offered.

Daytime Incontinence

The patient’s history is of paramount importance in sorting out the various categories of daytime incontinence. , The physical examination and evaluation should always assess for an abdominal mass or tenderness, distended bladder, abnormal genitalia, signs of spina bifida occulta, perineal sensation, sacral reflexes, gait, lower extremity reflexes, and urinalysis. Radiographic evaluation, usually voiding cystourethrogram (VCUG) and renal ultrasonography (US), are important in patients with UTI or complex incontinence patterns.

Bladder Instability

Bladder instability is by far the most common diagnosis in children with persistent daytime wetting. These children are neurologically intact and usually toilet trained, but later develop increasing “accidents” associated with urgency. They describe not knowing that the bladder contraction was coming. They dash to the bathroom or try to “hold it in.” Boys grab and compress the penis, and girls often cross their legs and dance around or squat with the heel compressed over the perineum ( Vincent’s curtsy ). In our experience, children with hyperactivity disorders or a willful disposition appear prone to this pattern.

Urodynamic (UD) studies are typically performed in refractory cases and demonstrate significant unstable (unwanted) contractions during bladder filling, known as detrusor overactivity. This causes leakage before sphincter contraction (or posturing) can control it. Because these unstable contractions or spasms occur frequently during the day, a retentive pattern of using the external sphincter to “hold on” is developed. When these children do get to the bathroom and try to void, the sphincter relaxes poorly or only intermittently, resulting in stop-and-go voiding, difficulty initiating a urinary stream, straining, and poor emptying, all of which are part of the constellation of symptoms known as voiding dysfunction (VD). The elevated pressure during voiding and the poor emptying may result in secondary vesicoureteral reflux (VUR) and UTI. Finally, the overactivity of the urinary sphincter may carry over to the anal sphincter, making stool retention and encopresis commonly associated findings. When VD is present in the setting of bowel symptoms, the child has bladder and bowel dysfunction (BBD).

Treatment

Effective treatment rests on managing all aspects of this condition simultaneously. Constipation is treated with fiber or laxatives, and mineral oil after initial bowel clean-out. Recurring UTIs are managed with prophylactic antibiotics. Bladder instability is treated with timed voiding at frequent intervals (an alarm watch or phone application for the child is helpful). Use of pharmacologic therapy has been described for lower urinary tract dysfunction and can be used as an adjunct to behavioral therapy and biofeedback. The type of medication employed is typically based on the clinical picture. Anticholinergics are a mainstay for the typical overactive bladder in a child with incontinence and urgency with complete emptying. Oxybutynin is the only Food and Drug Administration (FDA)-approved anticholinergic for use in children, but tolterodine has been well studied, as have other anticholinergics. , More recently, another class of drugs targeting the detrusor muscle have been approved for use in adult patients with overactive bladder symptoms. These medications are agonists of the beta-3 (β3) receptor, which is a β-adrenergic receptor that is mainly expressed in the bladder. The main drug in this class is mirabegron, which has been FDA approved in adults for both neurogenic and nonneurogenic overactive bladder. In children, FDA approval has only been given for neurogenic detrusor overactivity, but studies have shown both safety and efficacy in pediatric patients with nonneurogenic overactive bladder. , Mirabegron has been shown to have comparable efficacy to anticholinergics in pediatric patients with OAB symptoms. , It has also shown some promise in combination with anticholinergics for refractory patients. Alpha-blocker therapy such as tamsulosin has been described in neurologically intact patients with incomplete emptying and a lag time in external sphincter relaxation. ,

Biofeedback has gained popularity for treatment of the VD. Electrodes placed on the perineum near the genitourinary diaphragm can be attached to monitors, an audio signal, or a computer display so children can learn to relax their external sphincter voluntarily, resulting in better voiding coordination. The process typically requires four to eight weekly visits, with follow-up as needed.

Initial success with any treatment is often followed by later relapse. If initial treatment is unsuccessful, it may be successful if retried later. Patients older than 8 years who fail treatment should be considered for UD testing. Secondary VUR usually resolves (80%) as bladder function improves. Failure of behavioral and medical therapy is rare, with 2% in one large recent series failing nonoperative management. Further management typically involves either cystoscopic injection of botulinum toxin (Botox) or a form of neuromodulation. Patients who are likely candidates for these treatments should be evaluated with imaging and UD studies prior to further intervention. Injection of Botox has been FDA approved for nonneuropathic overactive bladder in adults, while it remains off-label in neurologically intact children with lower urinary tract dysfunction. Despite this, cystoscopic Botox injection of the detrusor in children with an overactive bladder or detrusor overactivity, and injection of Botox into the sphincter complex in patients with incomplete emptying and dysfunctional voiding, have been recently shown to be effective.

Neuromodulation has been used mainly in adults who have a refractory overactive bladder; however, there has been recent use in children. Transcutaneous electrical nerve stimulation (TENS) has been popular because of its noninvasive nature, though it requires numerous sessions. The TENS unit is thought to inhibit bladder activity via the pudendal–pelvic nerve reflex. Initial studies show promise with improvements ranging from 73% to 100% in small series , and improvement in UD parameters.

Surgically implanted sacral neuromodulators have also recently been shown to be successful in patients with refractory bladder and bowel dysfunction. While this therapy is off-label in children, it has shown durable improvement in quality of life and validated symptom scores. While it is promising, the rate of complications is high, with one study reporting 27% of patients in their series requiring reoperation due to a complication. Of those patients who do have success, there are a number of patients who have a complete cure and in whom the device can then be explanted (usually after at least 2 years of treatment).

Isolated Frequency Syndrome

A separate, and much less common, group of children present with acute onset of urinary frequency. They appear healthy, are normal on examination, and have a normal urinalysis and culture. They do not have true urgency or any wetting, but feel that they must urinate frequently, sometimes every 5–10 minutes. They void a very small amount each time. Most sleep through the night and void a large amount on awakening. The pattern may come and go over weeks or months.

The cause is unclear but is related to emotional stress in many cases. Careful assessment is crucial, and reassurance to parent and child is paramount. Sometimes, setting an alarm to progressively lengthen voiding intervals with a reward for success is helpful. This condition is benign and self-limited, although it may persist intermittently for months. Anticholinergics have no benefit, and further evaluation is not needed.

Infrequent Voider/Underactive Bladder

On the other end of the voiding spectrum are those children who void only once or twice daily, and may not urinate until afternoon after waking in the morning. These children have developed urinary retentive behavior without any bladder instability and have dilated, high-capacity, low-pressure bladders. Some show an aversion to bathrooms or exhibit excessive neatness, whereas many others appear reasonably adjusted. They may be somewhat prone to UTIs and stress incontinence.

It is important to exclude a neurologic cause and a structural obstruction to emptying. US can demonstrate good emptying if performed before and after voiding. A timed voiding regimen is usually required to get these children to urinate regularly if problems are occurring. This pattern tends to improve with age. Treatment with biofeedback and neuromodulation have also been described in management of refractory cases. ,

Continuous Incontinence

Patients who present with total incontinence and constant dribbling have a higher probability of urinary tract anomaly or pathology and require radiographic and possibly UD evaluation.

Hinman Syndrome

A small number of children demonstrate persistent incontinence, repeated febrile UTI, VUR, high bladder storage pressures, and very poor emptying. This appears to be a deeply ingrained, “learned” disorder of severe voluntary detrusor sphincter dyssynergia (DSD). In these patients, the urinary tract has the appearance of a patient with a neurogenic bladder. There is hydronephrosis, a trabeculated bladder, reflux, and sometimes progressive loss of renal function ( Fig. 54.1 ).

Fig. 54.1

(A) This cystogram shows the typical findings in a patient with Hinman syndrome: Trabeculated bladder and severe reflux. (B) This voiding study in the same patient demonstrates dilation of the posterior urethra (asterisk) as a result of chronic contraction of the external sphincter during voiding.

Aggressive therapy with prophylactic antibiotics, anticholinergics, alpha-blockers, UD biofeedback training, timed voiding, or clean intermittent catheterization (CIC) may be required. , Some recalcitrant cases may require bladder diversion or augmentation to avoid renal failure. As with many “functional” disorders, the severity of Hinman syndrome tends to wane with maturation, but progressive deterioration may not permit the surgeon to wait.

Neurogenic Bladder

True neurogenic dysfunction of the bladder in childhood results from acquired or congenital lesions that affect bladder innervation. Acquired lesions may occur from trauma to the brain, spinal cord, or pelvic nerves, or as a result of tumor, infection, or vascular lesions affecting these same structures. Congenital lesions include spina bifida and other neural tube defects (most common), degenerative neuromuscular disorders, cerebral palsy, tethered cord, sacral agenesis, and other causes.

The most practical way to classify neurogenic bladder abnormalities is by a simple functional system: failure to store, failure to empty, or a combination of both. Failure to store urine may be caused by the detrusor muscle itself or by the bladder outlet. Detrusor hyperactivity or poor compliance causes elevated bladder pressures and may lead to upper-tract deterioration and incontinence on this basis. An incompetent bladder neck or urethral sphincter mechanism can be the cause of failure to store urine even if storage pressures are reasonable. Failure to empty can be secondary to the hypotonic neurogenic bladder, which may not generate enough pressure to empty, or increased outlet resistance secondary to striated or smooth muscle sphincter dyssynergia. Increased storage pressures in this setting may lead to upper-tract deterioration and/or overflow incontinence. This classification helps to guide treatment based on UD data.

Myelomeningocele

The most common cause of neurogenic bladder in childhood is neural tube defects, which range from occult spinal dysraphism to myelomeningocele. , Myelomeningocele is most common, reported in about 1 in 1000 live births with notable geographic variations. , The etiology is multifactorial, with a clear familial association (2%–5% sibling risk) and evidence that periconceptual folic acid supplementation (0.4 mg/day) reduces the risk by 60%–80%. Improved treatment for the neurosurgical aspects of this lesion since the late 1970s has increased the survival rate.

Urinary incontinence, recurrent urinary tract infections, and deterioration of renal function are a major source of morbidity among patients with myelomeningocele. Early urologic evaluation and continued follow-up is critical in these patients; however, the optimal approach for evaluation and treatment of these patients has not been established. The Centers for Disease Control and Prevention began a collaboration in 2014 among nine centers across the United States. An iterative quality improvement protocol was utilized to evaluate and manage patients for the first 5 years of life. The UMPIRE study (Urologic Management to Preserve Initial Renal function) has been a valuable source of information in recent years and was extended for 5 more years in 2019, adding an additional center. An early paper from the study showed that over 90% of newborns with myelomeningocele have a normal renal scan in infancy. This highlights the importance of close follow-up to preserve renal function in these patients.

Evaluation of the Newborn with Myelomeningocele

Fetal closure of myelomeningocele has been evaluated in a multiinstitutional randomized controlled trial and is now more widely available. Results have demonstrated ventriculoperitoneal shunting was reduced by 50% at 12 months of age, with improved psychomotor development and motor function seen as well in children who underwent fetal repair. However, fetal intervention is associated with increased maternal and fetal risks and most patients who underwent prenatal closure were still not able to ambulate independently at 30 months. Initial studies with short follow-up of these patients did not show a decrease in the need for CIC. A more recent study evaluating results in school-age children demonstrated that most children who underwent fetal closure were still either incontinent or on CIC. However, there was a lower percentage of patients on CIC and more patients who were voiding volitionally when compared to patients who underwent postnatal closure. The results were based on parent-reported outcomes and there was no protocol directing the management of these patients. In addition, urodynamic data did not differ among the two groups. Longer-term data is needed to determine if prenatal closure truly has an impact on bladder function.

Generally, the newborn with myelomeningocele has had a thorough neurologic assessment, closure of the lumbar defect if this was not performed in utero, and possibly even ventriculoperitoneal shunting before any evaluation of the urinary tract. The level of the bony defect does not predict the functional cord level because the spinal cord lesion may be partial and patchy. Before discharge, renal and bladder US should be performed to evaluate parenchymal quality, the presence of hydronephrosis, and the size and function of the bladder. A small number of these patients have an abnormal US. In such cases, VCUG and possibly a renal scan should be performed. If the US is normal, other studies can probably be delayed a few months. About 3%–5% of these patients have VUR in the newborn period. The incidence of VUR increases with time, particularly in patients with untreated DSD or detrusor hyperactivity. Antibiotic prophylaxis has been universally recommended in the past; however, data from the UMPIRE study demonstrated that the incidence of UTI is low and found that patients on CIC and those with higher grades of hydronephrosis had the highest risk of UTI. Risk stratification of patients with initiation of antibiotic prophylaxis for higher-risk patients appears to be a more prudent approach.

Serum creatinine should be obtained but will reflect maternal creatinine levels initially. Baseline creatinine should start indicating neonatal renal function at 48 hours and should normalize within 2 weeks of age, while premature infants may take longer. A postnatal renal function panel at 48 hours and again at 2–4 weeks of age is a reasonable approach to screening these infants.

After closure of the defect, postvoid residuals should be measured before discharge from the hospital. CIC is begun if the residual urine is consistently >30 mL. A Credé maneuver should be avoided because it is ineffective in emptying the bladder and magnifies the detrimental effects of high intravesical pressure if DSD or VUR are present.

Newborn UD evaluation has been shown to have prognostic value in determining bladder risk. Bladder pressures higher than 40 cm H 2 O at the point of urinary leakage and those with DSD are much more likely to show upper-tract deterioration or VUR. Other factors known to indicate bladder “hostility” include hyperreflexic contractions and high storage pressures. Patients with high storage pressures and DSD are initially managed medically with anticholinergic therapy and CIC.

While early UD evaluation (typically by 12 weeks of age) is important to determine bladder risk, the interpretation of these studies has proven to be problematic. A multicenter study of the interrater reliability of interpreting pediatric urodynamic tracings showed that agreement on the presence of detrusor overactivity and DSD was poor. Despite this limitation, early evaluation is still recommended to serve as a baseline for follow-up studies and to identify patients at greatest risk of upper-tract deterioration. Such information can be useful to determine the frequency of follow-up studies as well as the timing and initiation of bladder therapy programs.

Childhood Management

Periodic reassessment of the anatomy and function of the urinary tract in patients with a neurogenic bladder is important because the clinical and UD picture may change with growth and spinal cord tethering. Initiation of a bladder management program is generally undertaken when there is worsening bladder function: VUR, hydronephrosis, or infection. It may also be initiated if findings on UD studies are worrisome. If the urinary tract is stable, such management may be delayed until social continence is desired.

The cornerstone of treatment programs for neurogenic bladder is CIC. Popularized in the early 1970s, CIC has revolutionized the treatment for these children. The purpose of CIC is to provide periodic low-pressure emptying of the bladder, which can prevent or improve deterioration of the upper tracts. , In younger children, this task is performed by the caretaker. As children become older and more responsible, they can assume this task.

CIC is associated with a high incidence of bacteriuria, varying greatly in different series. , Bacteriuria is eventually found in at least 60% of patients on CIC within 1 year, often with one or two symptomatic episodes per year. In patients with no VUR and with normal intravesical pressure, asymptomatic bacteriuria appears to have little clinical significance. However, in patients with high storage pressures and/or VUR, the likelihood for upper-tract infection increases significantly. As indicated previously, CIC has been shown to increase the risk of infection among infants with myelomeningocele and initiating antibiotic prophylaxis in these infants should be considered.

Pharmacologic therapy for neurogenic bladder coupled with CIC is aimed at decreasing the pressures in the hypertonic, noncompliant bladder or increasing bladder outlet resistance to aid in obtaining continence. Anticholinergic drugs, such as oxybutynin or tolterodine, can be used to lower bladder storage pressures by blocking hypertonic detrusor activity. Imipramine may also be useful alone or in combination with the anticholinergic agents because it can both relax the detrusor and tighten the outlet. Inadequate vesical outlet resistance may also respond to α-adrenergic medications, such as pseudoephedrine. In some cases, the combination of anticholinergics, α-agonists, and CIC is required to achieve adequate continence. Side effects of anticholinergics, including constipation and dry mouth, can sometimes limit their use. Extended-release formulations are reported to cause fewer side effects than immediate-release anticholinergics. The side-effect profile of one anticholinergic over another may vary from patient to patient, and changing to a different anticholinergic may be worthwhile if the side effects become problematic. Instillation of oxybutynin directly into the bladder can lessen the side effects and still maintain a therapeutic response. Also, the oxybutynin cutaneous patch may offer improved treatment for these patients.

Mirabegron is a beta-3 adrenoreceptor agonist that has been shown to have similar efficacy to standard anticholinergic therapy with an improved side effect profile. It received FDA approval in 2021 for use in neurogenic detrusor overactivity in children. A recent retrospective study in children with neurogenic bladder showed improvements in compliance, bladder capacity, and end fill pressures.

Finally, cystoscopic injection of botulinum toxin type A received FDA approval in 2021 for neurogenic detrusor overactivity in children over 5 years of age. Its use in pediatric patients with myelomeningocele has been rising in the United States, while bladder augmentation rates have remained relatively stable. The effects of Botox injection are temporary, but repeat injections result in a durable and often progressive improvement in UD parameters along with sustained symptomatic improvement. However, injections must be repeated approximately every 4–6 months for a continued effect, necessitating periodic exposure to general anesthesia in most pediatric patients along with the associated costs of the operating room. Botox has also been shown to be ineffective in patients with fibrotic, noncompliant bladders.

UD assessment can be elaborate in certain situations but is usually a simple measurement of the pressure–volume relationship of the bladder during filling. It is performed using a double-lumen catheter in the bladder and involves simultaneous assessment of external sphincter function with a perineal electrode. It can also be performed with contrast material and monitored fluoroscopically to add information. Evaluation of bladder compliance, hyperreflexic contractions, leak-point pressure, stress leak-point pressure, and sphincter dyssynergia can be extremely helpful in choosing among treatment options. Fig. 54.2 demonstrates the effect of anticholinergics in shifting the pressure–volume curve to the right and thus permitting the bladder to store more urine at any given pressure. Fig. 54.3 shows the effect of α-adrenergic agonists on raising the leak-point pressure and thus improving continence.

Fig. 54.2

Bladder filling pressure–volume curve in a patient with a neurogenic bladder. Note the shift of the curve to the right (A to B) when anticholinergics relax the detrusor, allowing lower pressure at any given volume.

Fig. 54.3

Bladder filling pressure–volume curve demonstrating a higher leak-point pressure (from A to B), sometimes achieved with α-adrenergic agents such as pseudoephedrine and imipramine. The effect is to decrease incontinence at lower pressures.

It is crucial to understand that when bladder pressures remain >35–40 cmH 2 O, ureteral peristalsis does not effectively empty the upper tracts, and hydronephrosis and renal insufficiency eventually result. Thus, coupling UD data with a particular patient’s estimated (or measured) hourly output permits the clinician to decide what CIC interval would keep bladder pressures in a safe range. Medications can then be adjusted to extend CIC intervals, achieve dryness, and avoid the development or progression of hydronephrosis.

While the objective measurement of bladder pressure is felt to be critical in identifying patients at risk for upper-tract deterioration, it must be recognized that interpretation of UD studies is subjective and can be difficult, particularly in the pediatric population. , Differences in interpretation may be significant enough to alter patient management. Pattern recognition may be as important as obtaining objective pressure measurements.

In children with high bladder storage pressures and deterioration of the upper tracts that cannot be managed by CIC and pharmacologic therapy, temporary diversion with cutaneous vesicostomy may be necessary. , Protection of the upper urinary tracts from high bladder pressures is thus accomplished until such time that other treatments can be effective. We reserve this treatment for infants who have serious deterioration of the upper tract and those who, for social, medical, or anatomic reasons, cannot be managed with the other aforementioned forms of medical treatment.

Surgical Treatment

Although most patients with neurogenic bladder can be managed adequately without surgery, those with VUR, a poorly compliant bladder that is not responsive to medical therapy, or refractory incontinence may benefit from an operative approach.

Treatment of VUR in the neurogenic bladder is much the same as that for the normal bladder. It is imperative that the bladder is adequately treated for poor compliance and hyperreflexia (CIC and anticholinergics) before and after the operation to diminish the risk of recurrence.

In some cases, bladder augmentation may be required. Bladder augmentation is designed to create a reservoir with good compliance and adequate capacity to store urine until it can be emptied by CIC at socially appropriate intervals. Detubularized segments of large or small bowel employed as a patch on the widely opened bladder (enterocystoplasty) are current popular techniques for augmentation ( Fig. 54.4 ). Another approach is gastrocystoplasty, which was used in the 1990s, but has since fallen out of favor. , Its advantages over enterocystoplasty include a decrease in mucus formation, a possible decrease in infection, and maintenance of electrolyte balance in patients with renal insufficiency. Unfortunately, the hematuria–dysuria syndrome may affect up to one-third of patients, which limits its applicability. This problem and other complications of enterocystoplasty—metabolic derangements secondary to the absorption of urine by the gastrointestinal (GI) tract, excessive mucus production, stone formation, and a debatably increased risk of bladder cancer —have led to a search for other approaches.

Fig. 54.4

Bladder enlargement by enterocystoplasty (sigmoid) and bladder neck reconstruction. Enterocystoplasty enlarges the bladder nicely but has significant potential complications, including occasional perforation, which may present as acute abdominal pain.

Bladder autoaugmentation or detrusorectomy is an alternative augmenting technique that may prove useful in selected patients ( Figs. 54.5 and 54.6 ). This approach involves removal of the detrusor muscle over the superior portion of the bladder, leaving the underlying bladder mucosa intact. This creates a large compliant surface, essentially a large diverticulum, which decreases bladder pressures and increases bladder capacity at the time of filling. The advantage of this technique is that the bladder epithelium is preserved and not replaced with GI epithelium as in bowel augmentation, thus eliminating the problems associated with the secretory and absorptive functions of bowel mucosa. Long-term follow-up data on large numbers of children are lacking and smaller series demonstrated mixed results. Nonetheless, this technique remains a viable alternative for use in bladders with a reasonable capacity and primarily poor compliance. The concept has been extended to create “composite” bladders by placing demucosalized bowel or stomach patches over the urothelial bulge created in autoaugmentation. ,

Fig. 54.5

(A) Radiographic and (B) ultrasonographic images of a patient with spina bifida showing a small, poorly compliant bladder and worsening hydronephrosis.

Fig. 54.6

(A) Radiographic and (B) ultrasonographic images in the patient shown in Fig. 54.5 after bladder autoaugmentation, demonstrating improved bladder capacity and better compliance, which resulted in continence and diminished hydronephrosis.

Augmentation with ureter has also been shown to be successful, but its application is limited to patients with significant reflux to a large, dilated ureter, where the kidney or polar moiety has poor function. As the urothelial lining of the ureter is similar to that of the bladder, there are similar advantages of using ureteral augmentation (rather than bowel) as there are with autoaugmentation. In contemporary practice, however, patients with neurogenic bladders typically have close surveillance to prevent massive reflux and ureteral dilation, so this treatment modality only has limited application.

The concept of urothelial preservation during augmentation is carried forward by current innovative approaches to replace the bladder wall with biodegradable scaffolds, typically seeded with urothelial and detrusor smooth muscle cells. While the concept is promising, a recent multiinstitutional phase II prospective study using autologous cell seeded scaffolds for augmentation did not show improvement in capacity or compliance and had a high rate of adverse events.

Persistent incontinence, despite adequate treatment of the bladder to lower pressures and increase compliance, may require bladder outlet repair to increase resistance. The Young–Dees technique, which lengthens the urethra by infolding and tubularizing the trigone of the bladder, still has some advocates. Kropp’s procedure uses a tubularized anterior bladder strip reimplanted in the submucosa of the trigone to gain continence by a flap valve mechanism. Continence is commonly achieved, but catheterization is sometimes difficult. Pippi Salle’s procedure creates a similar (but easier to catheterize) flap valve by onlaying an anterior bladder wall flap onto a posterior incised strip up the middle of the trigone. Owing to the lack of a pop-off mechanism in both these procedures, if the bladder becomes overfilled, there is an increased potential for bladder rupture or for upper-tract deterioration if high bladder pressure develops.

One of the more popular forms of increasing urethral resistance in a neurogenic bladder is by a bladder neck fascial sling. This procedure has many advocates and involves securing a rectus fascial strip (or other material) around the bladder neck and suspending it from the anterior rectus fascia or pubis. This elevates and compresses the urethra to increase outlet resistance. More recent series combined a bladder neck reconstruction with a sling to improve continence rates. , Finally, bladder neck closure may be required to achieve continence in some patients.

Robotic-assisted techniques have been increasingly used in lower urinary tract reconstruction. A literature review in 2020 highlighted the safety and efficacy of robotic assisted bladder neck procedures. Continence rates for bladder neck procedures ranged from 60% to 100% and similar rates were found in procedures performed with robotic assistance. Although operative times were typically longer, the authors noted decreased blood loss, improved cosmesis, and potentially decreased intraabdominal adhesion formation.

The artificial urinary sphincter (AUS) is a fluid-filled pressurized cuff around the urethra or bladder neck, which can be deflated by a pump-reservoir device that permits the urethra to open and the bladder to drain ( Fig. 54.7 ). The AUS can also be used in higher-pressure bladders in conjunction with bladder augmentation. The main disadvantage is that it is a mechanical device that can erode into the urethra and malfunction over time. Revision rates approach 30%, with erosion occurring in 20%. Erosion at the bladder neck is less common if there has been no prior bladder neck procedure and if the activation is delayed for 6 weeks. Children with neurogenic bladder dysfunction can continue to perform CIC with an AUS in place. In pediatric patients, however, life expectancy is ever-increasing, and the sphincter will eventually need revision or replacement. For this reason, we prefer to use autologous tissue techniques whenever possible.

Fig. 54.7

Typical artificial urinary sphincter. The scrotal pump moves fluid from the cuff to the reservoir to permit bladder emptying.

The periurethral injection of dextranomer/hyaluronic acid copolymer (Deflux), Teflon, or polydimethylsiloxane represents a simple, safe technique for enhancing urethral resistance in selected patients with poor intrinsic sphincter tone. It appears to be most applicable in patients requiring only a minimal increase in stress leak-point pressure. Long-term success is unlikely, and the usefulness of this approach in children is unclear. ,

With all procedures to enhance resistance at the bladder outlet, it is crucial that the storage pressures of the bladder be considered simultaneously. When the bladder outlet is tightened but the bladder is unable to store increasing volumes at low pressure, hydronephrosis or VUR results. When the surgeon is considering bladder-outlet reconstruction, it may be necessary to occlude the bladder neck with a urinary catheter balloon during preoperative UD assessment to determine the bladder capacity and storage pressures to assess whether augmentation is also needed.

One beneficial adjunct in patients unable to self-catheterize their urethra (e.g., owing to spinal deformity, discomfort, or false passage) is the creation of a continent catheterizable stoma. This can be performed using the appendix or another small tubularized structure implanted into the bladder and anastomosed to the skin (Mitrofanoff principle). , The implanted conduit can be hidden at the base of the umbilicus ( Fig. 54.8 ) and CIC carried out using it. Alternatively, the appendix may be left in continuity with the cecum and brought to the skin as a catheterizable channel for irrigation/enemas for the neurogenic colon. In this circumstance, a small segment of ileum can be fashioned as a catheterizable Monti–Yang stoma for bladder access. This concept has been a great adjunct to simplify catheterization for wheelchair-bound patients. A robotic-assisted approach to creating an appendicovesicostomy has gained popularity and demonstrates comparable success rates with a decreased length of hospital stay.

Fig. 54.8

Umbilical positioning of an appendicovesicostomy permits easy access for clean intermittent catheterization.

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May 10, 2026 | Posted by in PEDIATRICS | Comments Off on Bladder and Urethra

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