3 Pediatric Therapy
A 7-year-old boy enters your office with his eyes downcast. He is withdrawn and will not answer your questions. His mother tells you that he is still wetting the bed and has never been continent of stool. He has no friends and is at risk of failing second grade. He constantly fights with his father, and this past week almost set the house on fire as he tried to incinerate his soiled underpants. The diagnosis is nocturnal enuresis and diurnal encopresis.
The medical description of enuresis is the persistence of involuntary voiding beyond the age of anticipated control [Rushton 1995]. Encopresis is described as the involuntary loss of stool day or night.
These definitions are rather bland in comparison with the reality of pediatric incontinence.
Incidence of Enuresis
Enuresis can be classified as diurnal, nocturnal, or mixed. Mixed enuresis is when the child has symptoms of both daytime and night-time wetting. The accepted age for a child to attain diurnal urinary continence is 4 years. However, in most areas of the United States, children have to be successfully toilet-trained by the age of 3 if they are enrolled in daycare or preschool instruction. This forced continence places a large amount of pressure on the parent and child. Many children’s bladders and sphincters are not sufficiently developed neuromuscularly by the age of 3 to allow continence. The lower urinary tract requires coordination between several levels of the central nervous system (CNS) in order to function properly. Forcing continence on a child whose CNS is not fully matured can generate behavioral problems and poor bowel and bladder habits. The prevalence of diurnal enuresis in the United States is reported to be 29% for those aged 3, 13% for age 4, and 5–10% for ages 5–8. Diurnal enuresis occurs most commonly in girls [Rushton 1995]. A recent study by Sureshkumar et al. [2000] reported a higher than expected incidence of daytime incontinence in children between the ages of 4 and 6 years. The authors found that 19% of these children had had at least one episode of daytime wetting in the previous 6 months.
Nocturnal enuresis occurs in 5–7 million children between the ages of 6 and 18 in the United States. The incidence is reported to be 45% at age 3, 23% at age 5, 6% at age 10, and 1% at ages over 16 years [von Gontard et al. 2001]. In the study by Sureshkumar et al. [2000], nocturnal enuresis was defined as at least one episode during the previous week. The incidence of nocturnal wetting was 11% in children aged 4–6. The study also reported a 4% incidence of combined daytime and night-time wetting in this age group [Sureshkumar et al. 2000]. Spontaneous resolution of sleep enuresis occurs at a rate of approximately 15% annually, starting at age 4. Children with bed-wetting incidents after the age of 18 have a poor prognosis for recovery. Approximately 25% of children who attain dryness by the age of 12 will have periods of relapse during stressful periods. Nocturnal enuresis is more common in boys than girls, with a ratio of three to two up to the age of 11. At age 11, the incidence levels out between boys and girls [von Gontard et al. 2001].
The impact of pediatric incontinence is financial as well as psychological. The psychological impact on the child involves loss of self-esteem, development of anxiety and depression, rejection by peers, and enhanced tension between parents and child. The financial numbers are also felt in the health-care arena. Some 16–60% of pediatrician visits are for urinary incontinence, both diurnal and nocturnal. The wide range of variation depends on the severity of the problem. The frequency with which medical assistance is sought is directly proportional to the severity of the problem [Sureshkumar et al. 2000].
Incidence of Encopresis
Pediatric gastroenterologists in the United States report that 15 % of visits to their offices are for encopresis complaints. Studies indicate a prevalence of 1–3 % of the general pediatric population, with boys affected three to four times more frequently than girls. Enuresis is present concurrently with encopresis in approximately 25% of the cases [Chaney 1995]. Constipation complaints account for up to 25 % of all pediatric gastroenterology outpatient visits [Sutphen et al. 1997].
There is a ten times greater rate of attention deficit hyperactivity disorder (ADHD) in children with encopresis [Chaney 1995]. Boys with primary encopresis were significantly more likely to have both developmental delays and enuresis. Boys with secondary encopresis experience more psychosocial adversity and have comorbid behavioral disorders [Mikkelsen 2001].
Classification of Enuresis
Enuresis is often considered to have both a physical and a psychological or behavioral cause. The American Psychiatric Association (APA) defines enuresis as the repeated voiding of urine during the day or at night into bed or clothes at least twice per week for at least 3 months. The wetting must cause clinically significant distress or impairment in social, academic, or other areas of functioning. To be considered pathological, the child must be of an age at which continence could be expected. That age is chronologically 5 years, or a mental age of 5 for children with developmental delays [Mikkelsen 2001].
Many clinicians use the term “enuresis”to mean only nocturnal or sleep incontinence. This is due to the fact that the majority of enuresis is nocturnal. The lay term for nocturnal enuresis is bed-wetting. Diurnal enuresis is often mistakenly referred to as urinary urge incontinence or stress incontinence. Children can have urge incontinence, diurnal enuresis, stress incontinence, or a combination of any of the three. Urge incontinence usually involves small, frequent loss of urine coinciding with urgency. There may be a diurnal and nocturnal component. Nocturnal urge incontinence is not the same as nocturnal enuresis. In nocturnal urge incontinence, only moistening of the underwear occurs, rather than the soaking due to complete bladder emptying in enuresis. Children with urge incontinence may complain of perineal or suprapubic pain. They demonstrate a typical deferment behavior known as Vincent’s curtsey. This is where the child squats down with one leg under the perineum, applying pressure to the clitoris in girls or the base of the penis in boys. This pressure causes inhibition of ascending sensory information from the bladder and suppresses pelvic motor outflow to the detrusor, thus allowing the deferment of voiding. Urge incontinence usually occurs in girls. Urge incontinence can be a primary cause of incontinence, due to repeated detrusor contractions or instability. Urge incontinence may also develop secondarily, due to conditioning the bladder to remain empty. This conditioning occurs when the child attempts to avoid incontinence by frequently emptying the bladder. Pure diurnal enuresis is a clinical entity in children, usually in boys, in which they delay emptying the bladder until it becomes too late. They rarely report urgency, and when they have an incontinent episode they often have complete bladder emptying. Their voiding mechanism is normal, but infrequent. True stress incontinence due to incompetence of the sphincter is rare in children [Fisher and Frank 2000]. It may occur due to congenital anomalies or nerve damage from trauma or surgery.
Pediatric enuresis is further divided into primary and secondary categories.
Primary or type I enuresis occurs when the child has never achieved dryness or continence.
Successful toilet or “potty”training means having achieved dryness or continence for a period of 2–3 months. Children with type I incontinence have never been successfully “potty-trained.”Primary incontinence does not mean that the child has an incontinent episode every day, but rather that the child has never been able to achieve an accident-free period of 2–3 months. Primary incontinence in diurnal enuresis is often related to voiding dysfunctions. One-third of daytime incontinence patients have normal urinary diagnostic studies. However, many will be found to have detrusor instability. Primary nocturnal enuresis can be subdivided into primary monosymptomatic nocturnal enuresis and primary nonmonosymptomatic nocturnal enuresis.
Children diagnosed with monosymptomatic nocturnal enuresis have normal control over voiding during the day and have normal bladder function, but wet the bed during sleep. Nonmonosymptomatic nocturnal enuresis is defined as primary night-time wetting with daytime voiding problems [Nijman 2000, von Gontard et al. 2001].
Primary incontinence may be a symptom of a serious medical condition. This is particularly true if the child has both enuresis and encopresis. Most cases of nocturnal enuresis—80–90%–involve primary incontinence.
Secondary or type II incontinence is when the child has achieved successful continence for more than 3 months and then has a relapse.
Secondary diurnal enuresis is often related to periods of stress, urinary tract infections (UTIs), voiding dysfunctions, and behavior problems. Children with secondary nocturnal enuresis often have periods of continence lasting up to 6 months. The relapse is usually due to a medical, psychological, or emotional condition.
Enuresis risoria (“giggle incontinence”) is another form of diurnal incontinence. It occurs in association with laughter or emotional changes. The emotional changes precipitate alteration of pelvic floor muscle tone, causing sudden weakness or paralysis. Enuresis risoria is associated with a positive family history and with the narcolepsy syndrome complex. This complex includes: sleep attacks, cataplexy, sleep paralysis, and hypnagogic phenomena. Cataplexy is momentary paralysis that occurs in association with sudden emotional reactions. The patients become “weak with laughter”[Sher and Reinberg 1996].
Classification of Encopresis
The APA defines encopresis as “the repeated passage of feces in inappropriate places” with a frequency of at least once a month for 3 months in children over 4 years of chronological or mental age.
The same nomenclature is used for encopresis as with enuresis, with a distinction between primary and secondary conditions. There are two subtypes of encopresis: retentive encopresis and nonretentive encopresis.
Retentive encopresis is defined as overflow fecal incontinence with constipation, while nonretentive encopresis is overflow fecal incontinence without constipation [Mikkelsen 2001].
Normal Continence Development
Infants are born with an autonomous bladder. All of their pelvic organs are in an emptying position. Voiding occurs due to a supraspinal reflex. When the bladder becomes distended by urine, stretch receptors in the detrusor wall send a sensory impulse, via autonomic afferent fibers, to the pons. The autonomic efferent outflow that results causes the smooth muscle of the detrusor to contract. This micturition reflex occurs simultaneously with relaxation of the urethral smooth muscle and periurethral striated skeletal muscle sphincter, allowing coordination of voiding [Moss 1997]. Infants reflexively void 20 times a day. Micturition never occurs during quiet sleep, only with cortical arousal and a full bladder [Yeung et al. 1995]. The bladder capacity of an infant is approximately 30–60 mL (1–2 fluid ounces). As the infant grows, after 6 months of age, the bladder volume increases and frequency decreases [Rushton 1995].
Continence development in toddlers changes from reflexive voiding to the development of bladder sensations. By 18 months, the child is able to defer voiding. Toilet training begins at the age of 2–3, when the toddler starts to develop volitional control. The amount of volitional control depends on the amount of neuromuscular maturation in the midbrain and cerebral cortex. These regions of the brain control the external urethral sphincter and voluntary initiation of detrusor contraction. Voiding frequency decreases from 20 to 10 times a day. Bladder capacity in creases to approximately 30 mL for every year of age. The male bladder has a slightly larger capacity. Some neurologically normal children have a temporary delay in the maturation of the sphincter and detrusor. These children exhibit significant voiding dysfunction at ages 4, 5, and 6. However, it is believed that they develop detrusor instability and incontinence as adults [Moss 1997].
Nocturnal bowel control | Age 2–3 |
Daytime bowel control | Age 3–4 |
Daytime bladder control | From age 4 |
Nocturnal bladder control | From age 6 |
By age 4, the child has mastered daytime control by achieving control of the detrusor, vesicoureteral segment, and external urinary sphincter. Differences in degrees of mastery are due to congenital factors, childhood training, and motivation. Night-time urinary continence is usually mastered by the age of 6 [Nijman 2000].
Bowel control is achieved before mastery of daytime bladder control. The early acquisition of bowel control is due to decreased colon motility and tone during sleep. Nocturnal bowel control occurs at around the age of 2–3. Daytime bowel control usually occurs just before daytime bladder control is achieved, between the ages of 3 and 4. Toilet training tends to occur simultaneously for both bowel and bladder, but most children master the bowel first. Infants defecate up to four times a day. Bowel transit time in infants is 8 h. As children get older and more solid foods are introduced, the stools become firmer and fewer. Children between the ages of 1 and 4 defecate one or two times a day. The transit time for food through the gut is approximately 24 h in children and 48 h in adults [Spiro 1993].
Etiology of Enuresis
There are many unfortunate myths surrounding pediatric incontinence. Common myths are that the child is lazy; that the child sleeps too deeply; that he or she is doing it on purpose to annoy parents, teachers, guardians, or caregivers; or that the child is emotionally disturbed. The true etiologies of pediatric incontinence are: genetic, psychological, behavioral, voiding dysfunction, anatomical, or physiological.
Family history is important in both diurnal and nocturnal enuresis. Many congenital urologic anomalies are familial. These include external anomalies, renal disease, enzymatic disorders, and bed-wetting. Some urological abnormalities are due to intrauterine problems, and a detailed gestational history is important. Questions should be posed as to the baby’s birth weight, any maternal illness, medications taken while the mother was pregnant, and substance abuse before and during pregnancy [Gillenwater et al. 1991].
Genetic factors play a large role in nocturnal enuresis. Bed-wetting is an inherited trait in more than 40% of cases of primary enuresis. Children who have one parent with a history of sleep enuresis have a 43% chance of having sleep enuresis. When both parents have a positive history, the chance of their child developing nocturnal enuresis increases to 77%. A family history of nocturnal enuresis has been found to be the strongest predictor for the age of obtaining dryness with monosymptomatic nocturnal enuresis. Children with at least two first-degree relatives with a positive history of monosymptomatic nocturnal enuresis experienced a developmental delay in nocturnal bladder control of 18 months [von Gontard et al. 2001]. The exact cause of nocturnal enuresis is unknown, as most of these children are biologically and psychologically normal. They simply fail to waken when their bladder is full and contracting.
Psychological or behavioral etiologies are especially important if they occur during the developmental period of 2–4 years of age. These etiologies are listed in Table 3.2. Children who achieve bladder control after the age of 5 have a three times greater risk of relapse that those who become dry before the age of 5. The second risk factor is the number of adverse life events preceding the relapse. If the child experiences four or more stressful life events in a year, the risk for secondary nocturnal enuresis is two and a half times greater [von Gontard et al. 2001].
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Voiding dysfunction is caused by incorrect hydration, incomplete emptying of the bladder, chronic constipation, and recurrent urinary tract infections. Incorrect hydration can consist of overhydration, dehydration, and ingestion of bladder irritants. Overhydration can set up a pattern of frequency and create a potential for diurnal urge incontinence. Ingesting too much fluid before going to bed can exacerbate nocturnal enuresis. Dehydration can cause voiding dysfunction by causing the urine to concentrate, making it an irritant to the bladder. Many people erroneously believe that decreasing their fluid intake will reduce the number of urgency episodes. Children need to be adequately hydrated with fluids that do not irritate the bladder; correct hydration is sometimes all that is needed for the bladder to function properly. The same beverages that irritate adult bladders also irritate children’s bladders; Table 3.3 provides a list of substances that irritate children’s bladders. The amounts of beverages consumed by children in the United States that contain large amounts of sugar and dye and also caffeine are disconcerting. Children may be consuming sufficient fluid to keep them hydrated, but they are underconsuming water, milk, and fruit juice. Constipation is also a consequence of poor hydration and is a major contributing factor to enuresis. Loening-Baucke [1997] found that constipation contributed to 29 % of diurnal enuresis, 34% of nocturnal enuresis, and 11 % of urinary tract infections. Constipation was found to be present in 35 % of children with diurnal enuresis and urinary tract infections, compared with 25 % of children with diurnal enuresis and no UTIs [Kodman-Jones et al. 2001].
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Incomplete emptying of the bladder is a common problem in children. Many children are in too much of a rush to void properly. They want to hurry up and get back to playing so they force their bladder to empty. They use the Valsalva maneuver, Crede maneuver, and abdominal contraction to empty the bladder forcefully, leading to incomplete emptying. One result of incomplete emptying is that the child often leaks immediately after voiding, due to the abnormally elevated postvoiding residual (PVR). Over time, the child becomes unable to void except by using forceful voiding methods. Children do not understand that the bladder will contract when the pelvic floor is relaxed. They eventually develop concomitant contraction of the pelvic floor muscles (PFM) due to excessive straining with voiding. This sets the stage for the development of bladder sphincter dyssynergia, with the bladder emptying against a closed external sphincter. It also may lead to the development of a nonrelaxing puborectalis muscles, which can be a major cause of chronic constipation. Constipation and recurrent UTIs are discussed below.
The anatomical and physiological causes of enuresis are maturational lag or developmental delays, decreased production of plasma arginine vasopressin, sleep disorders, organic urinary tract disease, upper and lower urinary tract anomalies, tumors, chronic constipation, neurogenic bladder, hypertonic or hypotonic bladder, detrusor areflexia or hyperreflexia, Hinman’s syndrome, decreased functional bladder capacity, and food allergies. Urological function and dysfunction in children are different from function and dysfunction in adults. The dynamics of the urinary tract in children are more complex, as the development from simple reflex-controlled infant bladder function to mature bladder function takes place during the first 5 years of life. The most crucial event in this development is the maturation of inhibition, which takes place in the growing urinary tract. In addition to the development of neurological control over the lower urinary tract, there is physical growth of the bladder sphincter unit. Children can also have many congenital structural and organic diseases of the lower urinary tract that can disturb normal development [Hoebeke et al. 1995].
Children in whom enuresis is caused by delayed development have a reduction in functional bladder capacity of more than 50%. They have inadequate cortical inhibition over afferent bladder stimuli. This delay in acquiring continence may be associated with other developmental delays in fine and gross motor control, perceptual dysfunction, speech delays, and central nervous system (CNS) regulation. Others believe that the developmental delay does not result from a delay in neuropsychological maturation but from a deficiency in learning proper voiding habits [Rushton 1995].
Plasma arginine vasopressin is the antidiuretic hormone produced by the posterior pituitary gland. As part of the body’s circadian rhythm, the posterior pituitary gland increases production of this hormone at night to cause decreased urine production while the body sleeps. Some children have a decreased production of arginine vasopressin, causing their bladders to continue to produce urine at daytime levels. Nocturnal polyuria is a condition in which the kidneys produce excess amounts of urine at night, exceeding functional bladder capacity. This condition is believed to be due to a developmental delay in the circadian rhythm [Nijman 2000]. Further research is continuing on the relationship between arginine vasopressin and urine osmolality, abnormal tubal processing of sodium, and urinary excretion of potassium and sodium [Mikkelsen 2001]. A recent study investigated nocturnal polyuria and hypercalciuria as a cause of nocturnal enuresis. Night-time polyuria was found in 65% of the children, and hypercalciuria was present in 40%. These higher calcium levels in nocturnal urine may cause an increase in nocturnal diuresis and thus lead to polyuria. The results of this study indicate that there are three parameters that need to be taken into consideration in the nocturnal metabolic balance in enuretic children: the urinary calcium–creatine ratio; levels of antidiuretic hormone; and polyuria [Aceto et al. 2003].
Sleep disorders and their role in nocturnal enuresis are still under debate. It is true that if a child is not sleeping sufficiently, he or she will be more prone to urinary accidents due to muscle fatigue and decreased attention to body signals. Sleep requirements for children are listed in Table 3.4.
Children with primary nocturnal enuresis wet the bed during all stages of sleep, irrespective of the state of arousal. This suggests that during sleep, inhibitory signal processing in the brain-stem is unable to prevent bladder emptying without voluntary cortical control. The signal from the distended bladder is not registering in the subcortical centers that inhibit micturition [Ornitz et al. 1999]. Children without nocturnal enuresis awaken when they receive the signal from the bladder that there is a need to void. Child with sleep enuresis are either not receiving the signal or failing to respond to the signal from their bladder. In a study of 1413 schoolchildren, enuresis was found to be associated with difficulty in waking up at night and confusion when woken from sleep [Neveus et al. 1999].
The enuretic event is a predominantly a phenomenon of nonrapid eye movement (NREM) sleep. Sleep has six stages: stage 0 is the prelude to sleep; stages 1 through 4 are NREM sleep. A person sleeps deepest in stage 4 and if aroused will be groggy and confused. Rapid eye movement (REM) sleep alternates with NREM sleep every 30–40 min throughout the night. During a typical night, a person completes four to six sleep cycles, not including stage 0, with each cycle lasting approximately 90 min. The early part of the night is spent in stages 3 and 4, while the later part of the night involves stage 2 and REM sleep. REM sleep appears to be important for daytime alertness.
Age (y) | Sleep required (h) |
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3 | 12 or more |
4 | 11.5 |
5 | 11 |
6 | 10.75 |
7–8 | 9–10 |
9–12 | 9.25 |
Anatomical anomalies of the urinary tract can occur at the level of the kidneys, ureters, bladder, and urethra. Kidney anomalies that can affect the symptoms of enuresis include malrotation, ectopic kidney, and renal fusion. Malrotation is when the kidney rotates around the ureter, usually on a vertical axis. Ectopy is a condition in which the kidney does not occupy its usual lumbar position. It may be located in the pelvis, abdomen, or on the opposite side of the body. Renal fusion is the anatomic union of two or more kidneys. Clinical symptoms of renal anomalies include recurrent urinary tract infections, hematuria, abdominal pain, and obstruction. Children with renal anomalies are usually first seen by rehabilitation professionals if they need to have retraining in voiding after corrective surgery has been carried out.
Ureter anomalies include: ectopic ureters, strictures, ureteral duplication, megaureters, ureteroceles, ureteral diverticula, ureteral stenosis, and strictures. Ureteral ectopy occurs when the ureter opens into a location other than the bladder. Most ectopic ureters are reported in girls. Abnormal ureteral terminations in girls are often located in the vestibule (38 %); the urethra (32%); the upper vagina (27%); and the cervix and uterus (3%). These children may present with urinary tract infections, diurnal incontinence, and obstruction. They may complain of only leaking urine when standing or sitting. This may be due to a dilated ureter that serves as a collecting system, only draining when the child in an upright position. Approximately 50 % of ectopic ureters are not diagnosed until adult life [Kelalis et al. 1985].
In boys, ureteral ectopy may occur in the prostatic urethra, seminal vesicle, and vas deferens. Urinary incontinence is not usually a symptom with boys, because their ectopic ureters always drain within the external urinary sphincter. They may instead complain of urinary tract infections and flank pain. A ureterocele is a congenital cystiform dilation of the terminal ureter that causes ballooning at the posterolateral trigone and projects intravesically. Symptoms are usually infection and retention, and rarely obstruction or incontinence. The ureterocele can prolapse in girls to a point at which it becomes visible at the urethral meatus during straining and micturition [Walsh et al. 1998].
Congenital bladder abnormalities include bladder exstrophy, patent urachus, and bladder formation anomalies. Most congenital bladder anomalies require bladder reconstruction, often with the creation of bladder augmentation, urinary diversion, and genital reconstruction. The ability to regain continence after this extensive surgery depends on how significant the reconstruction is and the amount of neurological damage to the structures that occurs. Many of the children will regain continence, but may need to have retraining in voiding. Others will need long-term catheterization. The exstrophic bladder is located external to the abdominal wall, with an openly draining urethra and separation of the symphysis pubis. Bladder, urethral, and genital reconstruction are required. A patent urachus occurs when the urachal tube fails to close in the fourth and fifth months of gestation. Failure of this normal closure results in continued communication between the bladder and umbilicus. Bladder formation anomalies can include agenesis, duplication, and multilocular bladder [Kelalis et al. 1985].
Urethral anomalies occur mostly in boys. These anomalies can consist of posterior or anterior placement of the urethral valves, megalourethra, urethral duplications, and hypospadias. Hypospadias is an abnormal urethral opening along the shaft of the penis. It occurs in one in 300 live male births. Hypospadias is often associated with anomalies of the upper urinary tract, imperforate anus, and myelomeningocele. Hypospadias requires reconstructive surgery for the urethra and often for the chordee [Walsh et al. 1998].
A hypertonic bladder is caused by contraction of the external sphincter, with dilation of the proximal urethra. This is known as a “spinning-top” bladder, due to the image of a dilated urethra over a closed external sphincter that is seen on video cystourethrography. A child with hypertonic bladder has a small bladder capacity, with elevated detrusor pressures at voiding, a thickened/trabeculated bladder wall, and vesicoureteral reflux. Vesicoureteral reflux is urine flowing in a retrograde direction from the bladder to the upper urinary tract (ureters and kidneys). The primary cause of reflux is a congenital anomaly of the ureterovesical junction, leading to inadequate ureteral valvular control of urine flow. A secondary etiology of reflux is bladder obstruction and consequently elevated pressures forcing the urine back into the ureters. Children with hypertonic bladders have symptoms of primary diurnal and nocturnal enuresis, staccato voiding, recurrent urinary tract infections, urgency, frequency, dysuria, and use of Vincent’s curtsey.
Hypotonic bladders are also known as lazy bladders. They have hypercapacity with normal contractions, a diminished sense of bladder fullness and elevated PVR. Children with a hypotonic bladder start with a normal voiding and bladder capacity as infants, but receive an inadvertent reinforcement to retain their urine. This may consist of pain during a urinary tract infection, fear of using public bathrooms, or discouragement about voiding when the urge was present. As a result, the bladder becomes overstretched and the child does not respond to normal cues for the need to void. The elevated PVR predisposes the child to recurrent urinary tract infections, as the extended bladder provides an ideal medium for bacterial growth. The recurrent urinary tract infections reinforce the child’s avoidance of voiding, and the bladder continues to overstretch. Children with a hypotonic bladder typically wait until the last minute to void. They only void two or three times in a 24-h period. They may not use the bathroom during school, deferring it until they get home. They have complaints of chronic constipation or encopresis and strain to urinate.
Detrusor areflexia is a condition in which the bladder overflows without warning. The child has both daytime and night-time wetting. This may be due to a perinatal cerebral insult or infection. There are inhibited bladder contractions and a trabeculated bladder wall. Detrusor hyperreflexia, in which the bladder muscle undergoes uncontrollable spasms, causing urgency, frequency, sudden incontinence, and enuresis, may be associated with a family history of delayed continence. There is no evidence of outlet obstruction, and the child has a normal upper urinary tract, normal developmental milestones, and normal motor coordination and learning abilities. However, the child may have mild hyperreflexia of the lower extremities and reveal uninhibited contractions of the bladder during filling on urodynamic studies. There may be delayed maturation of the reticulospinal pathways and/or the inhibitory centers in the midbrain and cerebral cortex [Kelalis et al. 1985].
Hinman syndrome, also known as Hinman– Allen syndrome, is described as “non–neurogenic neurogenic bladder.” It is characterized by structural and urodynamic changes that suggest neurogenic bladder, but without an underlying neuropathic lesion being present. The incontinence is due to active contraction of the external urethral sphincter when voiding, causing outflow obstruction. With this disorder, there is an increased risk of upper tract damage due to reflux. Vesicoureteral reflux occurs in 50 % of children with this syndrome. The condition is believed to be caused by acquired behavioral and psychosocial disorders, resulting in voiding dysfunction that mimics neurologic disease. This syndrome is more common in boys between the ages of 6 and 10 years. The affected children usually have a background of problems in the family, with alcoholism, divorce, severe discipline, and emotional abuse. The classic signs and symptoms of Hinman syndrome are: staccato voiding, hurried voiding, sphincter dyssynergia, high-amplitude voiding pressures, marked bladder trabeculation, recurrent urinary tract infections, high PVR, vesi-courethral reflux, urge incontinence, nocturnal enuresis, encopresis, constipation, fecal incontinence, and detrusor instability [Hinman and Baumann 1973, Allen 1977].
Genetic
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Psychological/behavioral
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Micturition dysfunction
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Anatomical/physiological
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It was once believed that enuresis was due to emotional problems, sleep disorders, and congenital anomalies. However, urinary tract obstruction and emotional disorders account for only 5 % of the cases. Studies have revealed that enuretic children have decreased functional bladder capacity and void twice often as continent children. Continent children between the ages of 4 and 6 normally void between five and six times within a 24-h period. Enuretic children in the same age group void 10–12 times a day. The normal bladder capacity in children aged 4 and older is believed to be 300 mL or more. Enuretic children have been found to have bladder capacities of less than 300 mL. Cystometrograms in children with primary enuresis showed that 75% had a small bladder capacity and uninhibited detrusor contractions [Gillenwater et al. 1991]. Table 3.5 provides an overview of the etiology of enuresis.
Etiology of Encopresis
Encopresis is related not to a single variable, but to several factors. The possible causes of encopresis include avoidance, congenital deformity, impaired rectal and sigmoid sensation, chronic constipation, slow mobility, rectal prolapse, rectal injury, poor diet, food allergy, medications, and family or social stress factors. Levine [1982] developed a list of potentiating factors in the development of encopresis. He considered that the development of encopresis could start as early as in infancy and the toddler years with simple infant constipation, parental overconcern, congenital anorectal anomalies, acquired anorectal problems (e. g., fissures) and coercive physical treatment of constipation. The toilet-training years of ages 2–5 may bring a host of other factors that can precipitate encopresis. The factors include extreme family stress at the time of training, overaggressive training, extremely permissive training, continuing painful defecation, and toileting fears. During the early school years, factors such as fear of school bathrooms, prolonged or severe gastroenteritis, attention deficits causing incomplete defecation, food intolerance, hectic lifestyle, and psychosocial stresses can contribute to encopresis [Levine 1982].
Psychological or behavioral factors contributing to encopresis are found to be hyperkinetic syndromes and emotional and conduct disorders. In one small study, 65% of children with encopresis and/or daytime incontinence had externalizing, internalizing, delinquent, anxious, or depression problems [von Gontard and Hollmann 2004]. A larger study of children with constipation and soiling showed no relation between colonic/anorectal function and behavioral profiles, but revealed that children with defecation disorders have more behavioral problems than control individuals [Benninga et al. 2004]. The question then becomes one of whether the child’s bowel disorder is due to the behavioral problem, or whether it was the behavioral condition that led to the defecation dysfunction. Children with primary encopresis have been shown to have more difficult and disruptive toilet-training experiences, including interruption of toilet training, punishment, constipation, and abdominal pain resulting in fear of the toilet [Fishman et al. 2002].
The process of avoidance contributing to encopresis usually starts with the child experiencing one large, painful bowel movement. A large bowel movement can cause a midline sagittal tear in the anal mucosa, an anal fissure. The pain may be due to the development of this anal fissure. In addition, pain with a bowel movement may be due to perianal dermatitis or perianal and anorectal abscesses. The pain and anal fissure may result in pelvic floor muscle spasm, leading to difficulty in evacuating and ultimately constipation [Leung et al. 1996]. This pain may also cause fear. Fear of the pain recurring with each bowel movement results in avoidance of toileting. Due to the avoidance, the stool collects in the rectum. As the stool enlarges, it causes the walls of the colon and rectum to stretch. Stretching of the rectal walls sends sensory signals of urge for the child to defecate, but the child ignores the urge. As stool continues to collect, it exerts pressure on the internal anal sphincter, causing it to relax reflexively. As the internal anal sphincter relaxes, the stool exerts pressure on the external anal sphincter. The child valiantly tries to resist defecating, but the external anal sphincter is skeletal muscle and therefore becomes fatigued over time. Soft or liquid stool then involuntarily leaks out from around the solid stool. The leakage starts as a mild staining of the undergarments and progresses to larger amounts of soft feces. This leakage, known as overflow incontinence, is often misinterpreted as diarrhea. If the pattern of avoidance continues, the child develops a megacolon. A megacolon is an enlarged colon and rectum. The vault is so large that the stool is unable to exert pressure on the walls to trigger the defecation urge. This has a negative effect on the child’s ability to defecate normally, and the incontinence becomes more pronounced. The typical defecation pattern of encopresis is staining of the undergarments, with increasing leakage over time and then passage of a very large volume of stool, often large enough to obstruct the toilet. This is followed by a brief remission of soiling. Symptoms of encopresis are dull, cramping abdominal pain, often relieved by defecation. Anorexia, general malaise, headaches, and rectal bleeding may also be present [Chaney 1995].
Constipation is defined as difficult or infrequent passage of feces. Chronic constipation consists of bowel movements with a frequency of less than every 3 days, with straining more than 25 % of the time to defecate small, hard feces. Constipation may be due to functional causes such as poor diet, avoidance of bowel movements, and muscle incoordination, or may have physiological causes such as disturbed colonic motility and impaired sensation. Ninety-five percent of cases of childhood constipation are functional in nature. Functional constipation can occur early. Neonates may have the defecation disorder known as infant dyschezia. Symptoms of this disorder are straining and screaming or crying with defecation, despite the passage of soft or liquid stools. It is thought that this disorder is due to incoordination during defecation, with increased intra-abdominal pressure and relaxation of the pelvic floor muscles. This behavior occurs in the first few months of an infant’s life and usually resolves once the infant has developed improved muscle coordination with pelvic floor relaxation and increased intra-abdominal pressure. Some young children under the age of 5 have functional constipation. This involves infrequent defecation of hard, firm feces. It often starts when the child is weaned from breast milk and introduced to whole milk and solid foods. There is no underlying anatomical anomaly or medical condition causing the constipation. The children’s colonic motility is normal. Often the only abnormal motor activity is found in the rectum, which has become so dilated and atonic that it may not be able to generate enough pressure to propel stool into the anal canal. Dietary changes and proper toileting habits can improve the consistency of the feces and aid with defecation. If the condition is not addressed at an early age, the child will develop long-standing habits of retention and encopresis, usually within 2 years [Youssef and Di Lorenzo 2001].
Toileting habits are believed to have a major impact on constipation. Some problems common with constipated children are avoidance of defecation or deferring the urge and not evacuating completely. Children may avoid defecating due to the pain issues discussed above, as well as concerns about privacy, cleanliness, and lack of willingness to take time away from play. Children who do not evacuate completely may do so due to being in a rush to return to play, a nonrelaxing puborectalis muscle, discomfort with the bathroom setting, and lack of sensation. A study conducted to investigate toileting behavior in encopretic children, not only constipated children, found that toileting behaviors did not play as large a role in encopresis as they do in enuresis. Borowitz et al. [1999] compared toileting-specific behaviors in encopretic children to those in asymptomatic siblings and asymptomatic non-siblings. The results showed that toileting behaviors in children with chronic encopresis were similar to those in asymptomatic siblings and controls.
Some medications commonly given to children can also lead to the development of constipation. These include methylphenidate (Ritalin), phenytoin (Dilantin), imipramine (Tofranil), iron-containing preparations, and cough syrups with codeine [Chaney 1995]. It is important to bear these in mind, as methylphenidate is often given to children with ADHD, imipramine is used with diurnal enuresis, and many children take over-the-counter multivitamin preparations with iron supplementation. Prolonged usage of laxatives to treat constipation may actually exacerbate the condition by causing a loss of intestinal muscle tone and loss of normal defecation reflexes, resulting in continued constipation [Leung et al. 1996].
Impaired rectal or sigmoid sensation occurs in 97 % of chronically constipated children. The most common condition causing impaired recto-sigmoid sensation is Hirschsprung’s disease. This disease is a sex-linked multifactorial inherited disease that occurs predominantly in boys, with a ratio of four to one. It occurs in one in 5000 live births. Hirschsprung’s disease is the most frequent intrinsic intestinal neuromuscular disease. There is an absence of neural elements in the bowel wall, allowing distention to occur due to a lack of awareness of urge. In 75% of the cases, the disease is limited to the rectosigmoid region. Peristaltic activity is absent or abnormal in the involved segment. These two abnormalities cause the development of megacolon and the resultant fecal obstruction. The children are often constipated from birth. The majority of children with this disease will be symptomatic within the first weeks to months of life, with vomiting, abdominal distension, and failure to thrive. Children who present later in life with the condition have symptoms of chronic constipation, ribbonlike stools, distended abdomen, and failure tothrive. These children do not have encopresis, as the disease is limited to a very short segment of the colon. The diagnostic results of barium enema studies reveal a colon dilated proximal to the fecal obstruction. The diagnosis of Hirsch-sprung’s disease is definitively made with a rectal biopsy that shows an absence of nerve ganglia in the colon [Youssef and Di Lorenzo 2001].
Congenital deformities that are common with encopresis include neurogenic bowel, prune belly syndrome, bowel atresia, imperforate anus, and sacral agenesis. Prune belly syndrome is a congenital absence, deficiency, or hypoplasia of the abdominal musculature accompanied by a large hypotonic bladder, dilated and twisted ureters, and bilateral cryptorchidism (undescended testis) and malrotation of the cecum. These children have chronic constipation, leading to mega-colon, usually due to the absence of abdominal musculature. They also have musculoskeletal anomalies such as clubfoot and congenital hip dislocation [Kelalis et al. 1985]. Neurogenic bowel is found in children with diagnoses of myelodysplasia , myelomeningocele, lipomeningocele, and spinal cord tumors or trauma. Children with myelomeningocele often have primary enuresis associated with neurogenic bladder. Bowel atresia is a congenital absence or closure of the anus, ileum, or duodenum. Children with anal atresia often have a fistula from the anal pouch to the urethra or perineum. These children need surgery to repair their deficit or to create a structure for defecation.
Imperforate anus is present in one in 5000 live births per year, mostly in males. It can range in severity from anal stenosis, to anal or rectal atresia, to incomplete joining of the intestines to the rectum. Imperforate anus is classified into groups based on the level of the imperforation relative to the puborectalis muscle or the pelvic floor [Moss 1997]. Anal stenosis is incomplete rupture of the anal membrane. Rectal atresia is the presence of an anus that does not communicate with the colon. In anal atresia, there is no anus. In both rectal and anal atresia, the colon communicates externally via fistulas. Fistulas develop between the intestines or rectum and the bladder, vagina, and urethra instead of the anus. The anus is often not in its normal location. Surgical repair is necessary. Imperforate anus may be associated with many urological problems. There are often congenital upper tract anomalies. Urethral strictures or sphincter damage can occur during surgical dissection, resulting in urinary incontinence. Neurogenic vesicle dysfunction is common in children with imperforate anus, as are abnormalities of the lumbosacral spine [Moss 1997].
Avoidance behaviors
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Congenital malformations
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Impaired anal and sigmoid sensation
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Chronic constipation Metabolic and endocrine disorders |
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Reduced colonic motility |
Rectal prolapse |
Poor nutrition
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Nutritional allergies |
Medications
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Familial or social stress factors
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Psychological
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Sacral agenesis results from failure of ossification of the sacral segments. There is a complete or partial absence of one or more sacral vertebral segments. The neurological deficits may result from absence of spinal roots, compression or entrapment of the spinal cord or roots, and traction on the spinal cord during growth of the vertebral column as a consequence of tethering. Children with sacral agenesis may not develop incontinence until they are older. These children’s symptoms worsen as they grow. Symptoms may include hydronephrosis, recurrent urinary tract infections, vesicourethral reflux, enuresis, encopresis, a decrease in perineal sensation and sphincter tone, hypotonicity or paralysis of the second through fifth sacral roots, flattened gluteal region, and foot deformities [Moss 1997].
The most common gastrointestinal food allergy is to milk protein. Other foods that cause gastric reactions include eggs, soy, fish, shellfish, nuts, chocolate, tomatoes, and citrus fruits. To establish a diagnosis of food allergy, it is necessary to demonstrate that the elimination of the allergen results in the disappearance of the clinical symptoms, that symptoms recur on repeated challenge, and that reaction to the offending food is indeed due to a hypersensitivity reaction. Hypersensitivity reactions can be identified using a skin test or radioallergosorbent test (RAST), which detects immunoglobulin E (IgE)–mediated antibodies [Spiro 1993]. Table 3.6 shows the etiology of encopresis.
Subjective Examination
When working with children, it is important to make them feel welcome and relaxed. The waiting and examination rooms need to have age-appropriate books, games, toys, and activities. There should be a child-sized table and chairs for them to use. Both the parent or guardian and the child should be interviewed. If there appears to be animosity between the child and parent, it may be necessary to interview them separately. Some parents are so frustrated by the incontinence that they can only say hurtful things about their child as they explain the situation. The child should not hear these discussions, as it can be very damaging. At that point, it is best to send the child out with another staff member to play, and to conduct a private interview with the parent. During some interviews, the parent may continually interrupt or correct the child, thus preventing the child from presenting a complete picture. It may become necessary to interview the child privately. It is then prudent to have another staff member in the room for the interview, for the clinician’s legal protection.
Many children are fearful of the clinician, and it can be difficult to interview them. There are many options to make this situation more comfortable and open. One suggestion is that the clinician should avoid wearing a laboratory coat or other type of medical uniform, as these make them less approachable in the eyes of the child.
It can be more productive to interview the child while playing. Coloring and drawing are wonderful avenues for distracting children from the fact that they are being asked difficult questions. One should avoid intimidating children by sitting next to instead of across from them and one should not expect a child to talk or look directly at one. They should be asked questions about their school, friends, siblings, and what they do for fun. Inquiries about their toileting habits can be made with questions such as: When do you go? Do you feel when you need to go? What is the bathroom like at school? Is it a high or low toilet? Do you feel you have privacy? Do you have enough time to go? Can you get up to go when you need to at school, or do you have to ask the teacher or wait until a break? In many school situations, the teachers or class environment make it difficult for children to use the bathroom when they need to [Cooper et al. 2003]. The clinician may need to interview the child’s teacher as well to determine what is occurring at school and whether constructive changes can be made to assist the child.
It is important to find out from the parents what terminology they use for toileting. If a child calls urination “wee-wee” and the clinician calls it “potty,” the child may be confused and the interview will not be accurate. It is just as important to learn the words the parents use for the child’s body parts. Many parents are horrified at their children using the proper terms for penis and anus and teach their children slang or more child-friendly terms instead. Age-appropriate visual aids on the gastrointestinal and genitourinary system should be available—e. g., a water-filled balloon to discuss micturition. These help children understand what is happening to the body and may allow them to be more candid in their discussion. Unfortunately, many children feel that the incontinence is their fault. Once children learn what is happening anatomically and physiologically, they start to understand that the incontinence is not their own fault and that they are not to be blamed. Watching the relief wash across their faces when they realize they are not bad children is a tremendously gratifying experience.
A medical history is important in understanding incontinence. Inquiries should be made into all aspects of the genitourinary, gastrointestinal, neuromuscular, endocrine, and surgical history. A bladder/bowel diary needs to be completed for all children. Table 3.7 provides information on the data to be collected with the diary. The history of toilet-training should be discussed, including when it started and what problems and successes were encountered. Pain should be discussed with both the child and the parent. It should be determined whether there is pain before, during, or after urination or bowel movements. The family medical history is important as well in these children. One should ask about any history of incontinence or urological or gastrointestinal disease in the parents and siblings.
Data to be recorded in a bladder journal
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The social and family history can be more important than the medical history if the clinician is able to ask the right questions. Topics that need to be discussed are sleeping habits, family dynamics, siblings, and the family attitude toward wetting. Inquiries about sleep habits include: is the child getting enough quality sleep at night; are they able to wake up to go to the bathroom; are they afraid of the dark or of going to the bathroom alone, and is the way to the bathroom well lit? Questions about family dynamics should probe into any stressful changes in the child’s life. It is important to discuss the relationship between the child and parents and between the child and siblings. One should inquire about sibling or parental rivalry, tension, or high expectations and determine whether the family is supportive with the wetting issue or whether teasing, punishment, or embarrassing or demeaning behaviors are occurring.
Physical Examination
The physical examination is always carried out with a parent or guardian in the room. The examination needs to be conducted in a nonthreatening manner. It is important to explain to the adult and child what will be visualized and touched. Ascertain that they understand the purpose of the examination and give permission before it starts. Pediatric visual aids are extremely helpful. Demonstrating the examination on a favorite doll can ease anxiety for a young child. Letting them play with an examination glove, blowing it up into balloons and drawing faces on it, can make the situation seem less sterile and alarming for the child.
The musculoskeletal examination should start with functional mobility. One should watch the child walking, running, getting up from the floor, moving onto and off a bed or chair, climbing stairs, and rolling over. To visualize the trunk and lower extremities, it is helpful to have the child put on a gown over underwear. Skin findings may include a dimple, a tuft of hair, or skin tag over the lower lumbar or sacral region, which may suggest spinal cord tethering or spina bifida. One should observe and palpate for any alignment problems in the spine, pelvic girdle, legs, or umbilicus. A gross assessment of the trunk and lower extremity range of motion, strength, sensation, and deep tendon reflexes should be carried out. The examination should include palpation of the abdominal wall for muscle integrity, symmetry, masses, enlarged viscera, and pain.
A pelvic floor examination may need to be deferred until the second appointment, depending on the child’s level of comfort. During a pelvic floor examination, the genitalia are examined visually and palpated. One should look for scars, lesions, wetness, or soiling, and for swollen or protruding tissues. One should visualize the size and completeness of the foreskin, the presence of chordee, and the position of the urethral orifice. One should assess size and position of gonads. The urethral and vaginal orifices, labia, and inguinal areas are examined externally. A rectal examination is helpful in determining perineal muscle tone. If the child is old enough to follow directions, one should assess active perineal contraction and relaxation during the rectal examination. Have the child bear down as if trying to have a bowel movement and observe for leakage, muscle tone, and prolapse from the rectum, vagina, or urethra. One should observe the perineum as the child is asked to tighten the muscles as if they have to go to the bathroom but cannot make it in time. Children have more accessory contraction of the gluteal muscles than adults and find it difficult to distinguish between them. Children do more breath-holding and have more abdominal protrusion as they try to comply with the requests. No intravaginal assessment is carried out in premenstrual girls. The parent or guardian should approve an internal assessment and they must be present in the room at the time of the examination. Strength testing scales for the perineum differ slightly with children, as levels 4 and 5 are not used in young children. They are reserved for intravaginal or internal testing only. A modified Oxford scale is used instead (Table 3.8).
0 | No contraction |
1 | Trace contraction |
2 | Weak contraction |
2+ | Weak contraction with displacement |
3 | Contraction with displacement |
3+ | Strong contraction |
A neurologic evaluation of the pelvic floor includes reflexes, sensation, and proprioception. The two sacral reflexes that may need to be tested in the perineum are the anal wink and the bulbocavernosus reflex. The anal sphincter reflex (wink) is tested by lightly stroking just lateral to the anus, resulting in an anal sphincter contraction (wink). The bulbocavernosus reflex is tested by stroking the clitoral hood or clitoris or by squeezing the glans and causing a contraction of the pelvic floor. The presence of these reflexes indicates that the reflex arc is intact. One should palpate for sensory awareness with a light touch as well as point tenderness in the perineum. Proprioception can be tested by filling the bladder with fluid. This test is usually performed with urodynamic studies.
Diagnostic Urological Studies
A urinalysis and urine culture will determine whether there are any infectious agents contributing to the urinary symptoms. A urine culture determines whether there are any microorganisms in the urine. Cystitis can be infectious or noninfectious. The infection can be due to a bacterial, viral, fungal, or parasitic organism, or may be noninfectious and due to chemical irritation. Urinalysis can detect the presence of glucose, protein, sediment, red and white blood cells, and crystals in the urine. Proteinuria and hematuria are common reasons for referral to a nephrologist to investigate for renal disease [Kelalis et al. 1985].
Urine testing is especially important if the incontinence is secondary enuresis and is sudden in nature. Recurrent urinary tract infections can create voiding dysfunctions, and conversely, voiding dysfunction can pose an increased risk of urinary tract infections. Urinary tract infections are extremely common in children. Typical symptoms are dysuria, urgency, frequency, and enuresis. Two-thirds of boys and one-third of girls with urinary infection have an underlying structural urologic anomaly. More than three-quarters of girls with an infection will have a recurrence [Gillenwater et al. 1991]. The etiologies of urinary tract infections include chronically elevated PVR, detrusor spasm with retrograde urethral flow, and chronic constipation. Most organisms enter the bladder via the urethra with retrograde flow of the urine. Chronic detrusor spasm conditions can draw the urine back up from the distal urethra. The distal urethra often contains bacteria, which are now deposited in the bladder. Once the organisms have entered the bladder, the most important defense mechanism is frequent and complete bladder emptying. A child with chronically elevated PVR does not have complete emptying and keeps urine in the bladder for long periods of time. This increases the chances of an infection developing, and the problem can be compounded by a low urinary flow rate. A patient with a low urinary flow rate does not wash the bacteria out of the bladder and urethra. There is a definite correlation between constipation and urinary tract infections. However, where the actual causes and effects of constipation and urinary tract infection lie is an educated guess. The hypothesis that there is a relationship between them is based on increased bacterial contamination, mechanical voiding factors related to a large mass of stool compressing the bladder outlet, a relationship between infrequent voiding and constipation, and decreased lymphatic flow from the pelvic girdle with constipation [Kelalis et al. 1985]. Girls with encopresis commonly suffer from urinary tract infections, possibly due to ascending bacteria from incontinent fecal material [Chaney 1995].
The next tests, which are still less invasive, include radiography of the abdomen and lumbar spine, ultrasonography, and measurement of the postvoiding residual. Abdominal radiography is used to exclude the presence of a distended, obstructed, or impacted bowel, renal or urethral calcifications, constipation, and megacolon. Lumbar radiography is used to diagnose spondylolisthesis, spina bifida occulta, and vertebral malformation. Ultrasonography can be used to look for abnormalities of the liver, large vessels, kidneys, ureters, bladder, pelvic organs, and gonads. A post-voiding residual test is used to ascertain whether bladder retention is a problem. It can be carried out via catheterization or bladder ultrasonography. In addition to radiographs, a lumbar magnetic resonance imaging (MRI) examination may have to be carried out to determine whether there is a myelomeningocele or tethered spinal cord syndrome in patients with a positive lumbar spine radiograph.
A series of more comprehensive urologic tests may be indicated, including urodynamic studies, cystoscopy, voiding cystourethrography, or intravenous pyelography. Urodynamic studies provide a comprehensive examination of the bladder, urethra, and external urinary sphincter. The method is useful for evaluating the patient’s ability to urinate and the bladder function. The complete study includes a urinary flow rate, detrusor leak point pressure, urethral pressure profile, cystometrogram, and electromyography. The urinary flow rate is the rate of urine flow measured during urination via a transducer attached to a commode. The urethral pressure profile measures the length of and pressures in the urethra with filling, holding, and emptying. The urethral catheter contains pressure sensors. The catheter is slowly drawn out of the bladder and then moved back in while the patient’s bladder is being filled with fluid. Cystometrography is the procedure that measures the bladder’s ability to fill, store, and empty. In electromyography, surface electrodes are placed on the perineum to measure pelvic floor muscle activity during filling, holding, and voiding. Urodynamics can be used to rule out detrusor instability, detrusor hyperreflexia, neurogenic bladder, bladder outlet obstruction, detrusor sphincter dyssynergia, or discordant voiding.
Cystoscopy is carried out with an illuminated tubular endoscope to examine the interior of the bladder and exclude interstitial cystitis, bladder defects, and urethral syndromes. Voiding cystourethrograms are a series of radiographs used to assess visually the ability of the bladder to void while the bladder is filled with a contrast fluid. The method evaluates the detrusor and bladder outlet mechanism. Voiding cystourethrography can help in the diagnosis of detrusor hyperreflexia, bladder outlet obstruction, detrusor sphincter dyssynergia, discordant voiding, or ureter reflux [Moss 1997]. Intravenous pyelography involves a series of radiographs of the renal pelvis (kidneys) and ureters after a contrast medium has been administered intravenously. The method is used to determine whether there are any anatomical deformities in the upper urinary tract and to measure vesicourethral reflux. It is often difficult to ascertain the exact cause of the urological symptoms without some or all of these diagnostic tests.
Diagnostic Gastrointestinal Tests
Gastrointestinal diagnostic tests are divided into upper gastrointestinal and lower gastrointestinal tests. The upper gastrointestinal tract includes the stomach and small intestine, while the lower gastrointestinal tract includes the large intestine and rectum. One of the first tests is to have the child collect data for a bowel diary. The child may also need to keep a bladder diary as well if urological symptoms are also present. Table 3.7 lists the information that should be collected with a diary.
Numerous diagnostic tests can be performed to determine the cause of encopresis or constipation. Not all of these tests are appropriate in all children. The first and least invasive method is a radiograph of the abdomen. This can be useful for assessing the degree of fecal retention and the need for disimpaction. It also helps assess the lower spine in children with both bowel and bladder complaints. Other less invasive tests include blood laboratory values to assess for infection and establish the endocrine and metabolic status; fecal analysis to test levels of normal flora, infectious organisms, enzymes, blood, and other contents; and guaiac or occult blood testing to look for blood in the feces.
More invasive gastrointestinal tests include: surface and fine-wire electromyography, sigmoidoscopy, colonoscopy, barium meal or upper gastrointestinal series, barium enema, colonic transit study, cinedefecography, nerve latency studies, rectal biopsy, and anorectal manometry. Surface and fine-wire electromyography is used to assess the function of the external anal sphincter. Sigmoidoscopy and colonoscopy use an endoscope to visualize the intestines. The sigmoidoscope assesses just the rectum and colon. The colonoscope inspects the entire colon. A barium meal is used to assess the gastrointestinal tract from the esophagus through the duodenum. The child drinks barium as a contrast medium to allow radiographic viewing of the upper gastrointestinal tract. Barium enemas are used for passive radiographic viewing of the lower gastrointestinal tract and are helpful in assessing patients with Hirschsprung’s disease or other neuronal disorders and in patients undergoing surgery for anal atresia. Children with neuronal disorders will have segments of dilated colon. This test is not indicated in children with a history of functional constipation or functional fecal retention [Youssef and Di Lorenzo 2001, Loening-Baucke 1994]. Cinedefecography also uses a contrast medium, but only in the rectum. During this test, the child is asked to defecate into a commode while being filmed. The test looks for anatomical prolapse, obstruction, and the anorectal angle. Segmental colon transit studies use the ingestion of radiopaque markers at specific intervals. Radiography is carried out at timed intervals to observe the amount of time it takes for the markers to move through the digestive tract. This method helps determine whether there are segments of the colon in which feces are accumulating due to decreased neuromuscular function in the colon. Children with functional constipation have an accumulation of feces in the rectal ampulla. Children with neurological disorders, such as cerebral palsy and myelomeningocele, have abnormal segmental transit times [Youssef and Di Lorenzo 2001]. Nerve latency studies measure the function of the pudendal nerve at the perineum. Pudendal neuropathy has been shown to contribute to constipation and incontinence in adults. However, studies have shown that pudendal nerve latency in encopretic children is normal in comparison with control children [Sentovich et al. 1998]. A rectal biopsy is indicated to confirm the diagnosis of intestinal neuromuscular disorders such as Hirschsprung’s disease.
Anorectal manometry provides an objective assessment of sphincter function and pressure. This test uses microballoon devices and pull-through techniques similar to those of cystometrography. It measures resting and squeeze pressures in the anal canal, the rectoanal inhibitory reflex, rectoanal contractile response, and rectal compliance. The rectoanal inhibitory reflex is the threshold of rectal distention to elicit reflex relaxation of the internal anal sphincter. The rectoanal contractile response is the contractile activity of the external anal sphincter after rectal distention. The external anal sphincter should contract reflexively in response to rectal distention and internal anal sphincter relaxation. Rectal compliance is the rectum’s ability to relax around a bolus. It there is low compliance, the patient is unable to store feces and has an increased urge and frequency. Excessive compliance may suggest a megacolon, in which a patient does not feel any urge and therefore develops constipation and eventually fecal incontinence around the bolus that remains in the rectum. Typical problems seen with children with chronic constipation and encopresis are abnormalities in the ability to defecate a bolus, rectal sensation, awareness of urge to defecate, anal resting pressure, and external anal sphincter control with straining. A study using anal electromyography identified nonrelaxation of the external anal sphincter in 75 % of a group of encopretic children, in comparison with 13% of the control group [Sentovich et al. 1998].
Medical Interventions for Enuresis
Medical interventions include surgery and medication. Surgical treatment for urinary incontinence can range from urethral dilation to bladder reconstruction. Children with bladder anomalies undergo more complex operations. They may also require neurologic, orthopedic, and plastic surgery. Children requiring more complex operations may benefit from rehabilitation for bladder retraining and other physical therapy interventions afterward. Some children may be able to regain normal continence after surgery if the problem was minor and was addressed early enough. The younger the children, the easier it is for them to learn normal continence, as they will not have previously learned incorrect voiding behaviors.
Numerous drugs are available for the treatment of urinary incontinence. Many are not appropriate for young children, due to their adverse effects. Most of these drugs are not curative and are associated with high relapse rates if the underlying problem has not been resolved or the child’s urinary system has not reached maturation [Glazener et al. 2003b]. The medications can be useful when applied in conjunction with behavioral therapies. Using the medications to help the child stay dry improves self-esteem, which results in increased willingness to work on the behavioral therapies. The children can then be weaned from the medications to assess their progress. If weaning is successful, the drugs are discontinued. Some medications are useful in certain situations such as sleep-overs, long trips, and during the school day. Most medications are not recommended for children under the age of 4 for diurnal enuresis or under the age of 5 in children with nocturnal enuresis.
There are four major categories of medication for urinary incontinence: tricyclic antidepressants, anticholinergics, sympathomimetics, and vasopressin analogues. Tricyclic antidepressants are used to decrease bladder contractility. They are associated with side effects of personality changes, nervousness, and gastrointestinal upset. The most widely used medications for diurnal enuresis are anticholinergics. These reduce bladder spasms and increase bladder capacity. They also can be used for nocturnal enuresis, due to the ability to increase functional bladder capacity by 25%. Sympathomimetics are not as effective with children. They are used to release norepinephrine, to increase contractility in the bladder neck. Vasopressin analogues are used for nocturnal enuresis, to stimulate the production of antidiuretic hormone (vasopressin). Desmopressin is the most common form used in the treatment of nocturnal enuresis. The success of these agents appears to depend on several factors. Studies have shown that if the child has a reduced functional bladder capacity, the response will be adversely affected [Rushton et al. 1996]. Hogg and Husmann [1993] demonstrated the importance of family history and pharmaceutical effectiveness. They found that in children with familial nocturnal enuresis there was a higher success rate of dryness with desmopressin [Hogg and Husmann 1993]. A recent small study was carried out to investigate desmopressin and its relationship to arousability in children. The study found that the number of wet nights per week decreased significantly and that the children slept more soundly when treated with desmopressin. This contrasts with the belief that enuresis is due to the child sleeping too deeply. The authors consider that the findings suggest that one of the causes of primary nocturnal enuresis lies in the structure of the sleep of the affected patients, and further research is recommended [Eggert et al. 2004].
Medical Interventions for Encopresis
Medical interventions for encopresis can range from medications to surgical repair. Children with gastrointestinal and anal anomalies undergo more complex forms of surgery. They may also require neurologic, orthopedic, and plastic surgery. Gastrointestinal operations may also range from minor fissure repair to complex organ reconstruction.
Drugs are widely used in the treatment of fecal incontinence and constipation. The agents are bulk-forming, lubricating, emollient, stimulant, and osmotic laxatives. Most are used to treat constipation and encopresis. Only one assists with fecal incontinence. Bulk-forming laxatives decrease both constipation and loose feces by thickening the stool through the absorption of fluid in the bowel. The feces become more bulky and it is easier to defecate. Lubricant laxatives promote defecation by facilitating the movement of stool. Mineral oil is an example of a lubricant. It coats and penetrates the fecal material, reducing the colonic absorption of water and allowing easier passage. Chronic use can inhibit the absorption of fatsoluble vitamins. Emollient laxatives soften stool without adding bulk, and they increase peristalsis. Most of these agents contain sodium or calcium salt. Stimulant laxatives stimulate peristalsis by promoting water and electrolyte movement into the intestines and inhibiting their absorption. There are also local treatments such as enemas and suppositories and oral stimulants. Chronic use of oral stimulants can lead to an atonic cathartic bowel. Osmotic laxatives are nonabsorbable salts that osmotically retain water in the lumen of the colon. They are used for immediate evacuation of bowel contents, as with impaction or before gastrointestinal tests.
The administration of laxatives in the treatment of encopresis should be carried out in conjunction with behavioral therapy for long-term resolution of fecal incontinence. Laxative use has been found to promote faster resolution of incontinence and a reduction in emotional maladjustment. Children with secondary encopresis do much better with a combination of laxative and behavioral therapy than with one form of treatment alone [Nolan et al. 1991]. A common treatment is administration of a large bolus of milk of magnesia to clean out the bowel. The child is then placed on a gradually decreasing maintenance dose of milk of magnesia to prevent the resumption of constipation. Studies have also investigated the administration of polyethylene glycol without electrolytes instead of milk of magnesia, and showed better success and fewer side effects with this treatment in the long-term management of constipation and encopresis [Loening-Baucke 2002, Pashankar et al. 2003].
Therapeutic Interventions
Therapeutic interventions are age-dependent in children. The ability to learn, follow directions, and make conscious decisions in relation to the bowel and bladder depend on the child’s chronological or mental age. In children aged between 2 and 3, the treatment should involve education of the parents, guardians, or caregivers regarding the maturation of the urological or gastrointestinal systems. Both the parents and children need to be informed regarding proper voiding and defecating habits, proper hydration, toilet-training methods, and bladder irritants. Four-year-olds and their parents should be given the same instructions. Biofeedback training can be initiated at this time as well. Some children respond appropriately to biofeedback, while others express disinterest and some have difficulty in following the instructions. Education and biofeedback can be attempted successfully with children and their parents by the age of 5. After the age of 6, there may be some emotional overlay that may make treatment more difficult. The education and biofeedback should still be pursued. Sessions with a counselor may also have to be started with these children in order to work on issues of self-esteem, learning difficulties, anger management, and other emotional issues discussed earlier in this chapter. Family counseling may also be indicated.
The treatment should be instituted step by step. One can start with one approach and gradually add treatments when they appear appropriate. Children cannot process as much information as adults and can easily be confused by the message. When educating children, the same methods should be used as during the interview described above. Do not wear medical uniforms, use play and age-appropriate materials, avoiding talking down to the child and talking around them to the adult, and do not force them to make eye contact—they are listening. Remember to make the sessions fun. For both children and adults, the session should include information about micturition and defecation. They can be shown the anatomy of the urological and gastrointestinal systems. The children may become fascinated with their body after this and will become more observant about urine and feces. This information alone can make a significant difference to the child’s attitudes to urge and toileting. Discuss the importance and function of the muscles of the pelvic floor, leg, hips, and abdomen and explain to the children that they are not alone with their problem. Information can be given about proper food and fluid intake, voiding and defecating postures, techniques, and frequency. Urge and when to use it or defer it can be discussed. Remember that the way in which this information is presented must depend on the child’s age and condition. If the child is being taken care of during the day by a nanny or teacher, the caregiver may need to receive the same information. Mixed signals on toileting between home and school can create confusion and lead to delayed recovery.
Many children have food and fluid allergies that can contribute to their symptoms. The first thing to do is eliminate all commonly known irritants. In the bladder, these common irritants include carbonation, caffeine, and citrus. If symptoms do not subside, other food or fluid items of concern can gradually be eliminated. These items are those that exacerbate the symptoms or may be ones the child ingests regularly. These items can gradually be eliminated one at a time and any changes in the symptoms can be observed. The elimination should be continued for 2 weeks. If the symptoms remain dormant, one can progressively reintroduce the item once a day for three consecutive days and watch for adverse effects. If no adverse effects appear, one can start again with a new item.
Certain foods can have a negative effect on the gastrointestinal system. Some foods can cause the child to become constipated, while others cause intestinal pain and some can cause explosive diarrhea. Fluid intake is important for both the bowel and bladder. The recommended intake will be less than that in adults. Children need to drink between six and eight 170–225-mL glasses of noncaffeinated, noncarbonated fluid a day. If the child’s problem is nocturnal, the fluid intake should be restricted, but not eliminated, 2 h before bedtime.
Many adults have poor voiding and defecating habits. This is mostly due to having been taught poor habits as children, as well as ignorance of correct habits. It is important to teach correct habits at the initiation of toileting training. Toileting training should start when the child shows an interest in going to the bathroom on the toilet. It is helpful to have a child’s potty seat in the bathroom they use. Other child-friendly methods are a smaller-diameter seat for a regular-size toilet, with a stool for those with short legs. Children need to feel physically safe when they use the toilet, and an adult-sized toilet will make them tense. Many children are afraid of falling into the toilet. Automatic flushing in public toilets can cause great consternation, as children may be afraid not only of falling in but even of being flushed away. Children learn through observation and then imitate what they see. It is therefore helpful to take the young child in with the parent—girls with mom and boys with dad–when they use the toilet. Alternatively, the child can watch a responsible older sibling. This approach can be problematic if the adult has poor toileting habits, as the child may imitate incorrect methods.
The child should be taught not to rush with the toilet, and the bathroom should be made pleasant for the child. Books should be available for them to look at. They need to be taught to relax when they sit down and allow the reflex to work for them. All children should sit initially with voiding. Once boys master continence, they can be taught to void while standing.
It is important for them to learn to not force the urine flow. They can count how long it takes to urinate. If it is only a few seconds, a game can be made of seeing how long they can urinate for—seeing if they can make it take longer each time, up to a target of 10–12 seconds. Discern that they are not using a stop/start technique. This can be done by listening at the bathroom door. The stop/start method can lead to retention.
When toileting, the child needs to learn to defecate without using the Valsalva method. This is holding your breath when you strain to have a bowel movement. It creates an inordinate amount of pressure on the pelvic floor muscles and can cause them to co-contract the sphincters. The child should be taught to exhale while bearing down, or can blow onto a windmill, trying to make it spin. They need to learn how to use the gastrocolic reflex to their advantage. Children with constipation or encopresis tend to defer urge and allow themselves to become constipated.
Behavior modification is the heart of therapeutic interventions. Once the child and parent have received the information they need, it is time to set up a treatment plan. Children should be told that they can be the boss of their bladder and bowel; they do not have to take orders from them. The key to behavior modification is the reward system. The parent, family, and caregivers have to understand that they must not punish, belittle, or embarrass the child. To avoid embarrassment, incontinence should only be discussed with immediate family members and medical personnel. Studies have shown that behavior modification can be effective in up to 25% of cases of diurnal enuresis when used alone, by increasing the child’s motivation. The key is to reward the effort and small improvements as they occur. Star and sticker charts are wonderful tools for behavior modification. Monetary or food rewards should be avoided, and family activities should be promoted instead–for example, “If there are no accidents today, we’ll go to the park”; “If there are no accidents this week, we’ll go to the zoo.”
When treating diurnal enuresis, it is vital to treat constipation first even if the child is not reporting the classic signs and symptoms. In cases in which constipation and enuresis are both present, diurnal enuresis resolves in 89 % of the cases with resolution of the constipation, nocturnal enuresis resolves in 63%, and recurrent urinary tract infections in 100 % of the cases [Loening-Baucke 1997]. A timed schedule for voiding can be very helpful even when medications and other therapeutic methods are being used. The child’s functional bladder capacity may be so small that this is the only way of staying continent. The child can be given a timed schedule based on feedback from the bladder diary. The child should not use the bathroom “just in case,” but should follow the schedule and increase the interval as the accidents decline. Eventually the intervals will be so long, every 3–4 h, that the child will be able to act independently when an urge is felt. The parents should be reminded that the symptoms may increase or return when the child is sick, under mental or physical stress, or lacking sufficient sleep. Many children will ignore their urge if they are engaged in a fun activity such as playing outside with friends, on the computer, or in the middle of a video. They need to learn to stop the activity when it is time to if they are on a schedule, or when they experience urge.
Nocturnal enuresis should not be treated at the same time as diurnal enuresis in a child who has both problems. The daytime problem should be treated first. Once it has been resolved, one can move on to the night-time problem. Behavior modification techniques such as rewards may not work as well with nocturnal problems, as the child may not have volitional control. However, penalties for wet beds appear to be counterproductive [Glazener et al. 2003a]. Start with environmental changes such as placing night lights in the bedroom, hallway, and bathroom. Many children are in a rush all day and never completely empty their bladder. These children are the ones who tend to wet the bed an hour after they have fallen asleep, when the pelvic floor finally relaxes and the bladder empties. They can play quietly for an hour before bedtime to allow the body to settle down, so that they can void more completely. Other simple interventions are lifting and scheduled waking. Lifting is when the parent or guardian takes the still-sleeping child to the toilet to allow him or her to void. Many professionals consider that this is counterproductive, as the child does not learn the sensations of a full bladder, and that it reinforces the behavior of urinating without waking. Scheduled waking involves waking the child at intervals and assisting him or her to the bathroom. A more complex approach to this is dry bed training, in which the parent or guardian starts by waking the child every hour once asleep. The time intervals are gradually increased until a pattern of wetting is found. This is similar to diurnal timed voiding or bladder retraining. In older children, an alarm clock set at intervals can wake them so that they can void. Another approach encourages the child to imagine and play-act getting up out of bed at night to go to urinate. Another simple intervention is retention control training or bladder stretching. Studies have shown that children with primary nocturnal enuresis have a smaller bladder capacity than those who do not bed-wet. The idea behind retention control training is to increase the bladder capacity and thereby reduce the bladder’s need to empty as frequently even as the child sleeps [Glazener and Evans 2004].
Constipation is a major problem in children. The initial approach is to provide information about the gastrointestinal system and gastrocolic reflex. The child has to learn to use the reflex, going to the bathroom within 5–20 min of eating a meal. Once there, children should to sit supported on the toilet or toilet chair for a minimum of 5 min to allow the reflex to occur. They can take in books and a timer so that they know how long to sit. Once they start to see this work, it is possible to find out which meal they have more success after, and it can become a routine for them. Travel can be particularly disruptive to this toileting routine, and consideration needs to be given to keep the child to the routine as much as possible [Leung et al. 1996]. Some children develop megacolon due to a long-term habit of deferring urge. In treating megacolon, the first step is to carry out a bowel purgation using one of the laxatives mentioned above, to eliminate constipation. The next goal is to restore regular bowel functioning for a sufficiently long period of time to establish a normal pattern of defecation. This is accomplished by administering a low-dose laxative and a high-fiber and adequate fluid diet. The type of laxative does not appear to be as important as compliance by the children and families. Constipating foods such as excess dairy products, apples, and bananas should be avoided. The child needs to use the gastrocolic reflex daily. Due to the enlargement of the colon, the child has a decreased sensitivity to the normal urge to defecate. Over time, the megacolon will decrease in size, urge will start to resume, and the medication can be phased out. Biofeedback therapy is warranted, as many of these children paradoxically contract the pelvic floor muscles when they defecate and force bowel movements using a Valsalva maneuver. This has to be corrected to prevent the constipation from returning.
The treatment for encopresis is essentially the same as that for megacolon, as megacolon is often the cause of encopresis. It is important to assess the children for possible food allergies that could be contributing to the symptoms. Bowel accidents are much more traumatic than urinary accidents, due to the odor. Talk with the child’s teacher or caregiver and have the parents provide extra clothes and laundry bags for soiled items. Make sure the child has a safe, private place where he or she can take time to defecate, especially after lunch.
In a 1994 National Institutes of Health study, biofeedback was shown to be 60% effective in the treatment of diurnal incontinence and the elimination of urge. It helps eliminate 75 % of cases of nocturnal enuresis. It is important to remember that the reason for providing biofeedback treatment in these cases is not to strengthen the pelvic floor muscles, as the children’s problem is delayed or disrupted voluntary contraction of the bladder and pelvic floor. The biofeedback is intended to help them learn to coordinate the pelvic floor with their bowel and bladder. The treatment involves achieving control with submaximal contractions and relaxation, quick recruitment, practicing in functional positions, and avoiding breath-holding and increasing intra-abdominal pressure [Wennergren and Oberg 1995]. Biofeedback treatment is carried out with surface electrodes placed perianally. Many computerized biofeedback programs have software that is suitable for children who use computer games and videos, and the interactive computer games have been shown to be clinically effective. A 1999 study showed that postvoiding residual decreased by 57%, urinary diagnostic study values improved by 42 %, pelvic floor muscle relaxation improved by 56%, and strength increased by 78% [McKenna et al. 1999].
Bed alarms, or the bell-and-pad method, are another form of biofeedback used with nocturnal enuresis. When used alone, they have a 55% success rate, although the results can take several months. The most important aspect of the alarm is the loudness of the actual alarm. It has to be loud enough initially to wake both the child and the parents. The parent will have to help the child to bed until the child is conditioned to do so. The loudness is also important to prevent the child from becoming conditioned to the sound. When bed alarms were used along with other behavioral modification techniques, the success rate improved to 85–95%. One effect of alarm treatment is an increase in functional bladder capacity [Oredsson and Jorgensen 1998]. Other behavioral approaches used include: restricting fluids before bedtime, emptying the bladder before going to bed, having the child get enough sleep for his or her age, keeping to regular bedtimes, avoiding excitement before going to bed, and using pelvic floor muscle biofeedback to improve bladder sphincter control [Schwartz 1987]. Once the child has achieved some success with alarm treatment (14 consecutive dry nights), over-learning is recommended. This is a method in which the child no longer restricts fluids before bedtime but actually increases fluid intake to test the ability to wake when the need to urinate occurs. There may initially be wetting, but by using the alarm with over-learning the child eventually will achieve success. Relapse rates are lower when over-learning is added to the alarm treatment [Glazener et al. 2003a]. A review article by Glazener and Evans [2004] found that reward systems and lifting and waking were associated with significantly fewer wet nights, higher cure rates, and lower relapse rates in comparison with controls. There was not enough evidence to compare these simple behavioral and physical interventions with more complex treatments involving bladder training, dry bed training, vasopressin, or bed alarms to determine which is the most effective treatment approach. The study does show that these simple methods could be tried as a first-line therapy before more demanding approaches are considered [Glazener and Evans 2004]. It has been shown that a combination of vasopressin treatment and bed alarms does not improve the success rate for continence in children who were initially nonresponders to vasopressin [Gibb et al. 2004]. Bed alarms are successful with partial responders or nonresponders to drug treatment in 90% and 71 % of cases, respectively, for up to 6 months after the cessation of all treatments [Woo and Park 2004].
Biofeedback with encopretic children has been shown to improve defecation dynamics by 86%. Learning normal defecation dynamics correlated with clinical recovery [Loening-Baucke 1990].
Summary
Toilet training in the majority of children is a simple technique that usually only takes 1–2 weeks. However, for children with genetic, anatomical, and psychological problems with voiding and defecation, life can be very stressful and unhappy. The young boy mentioned at the beginning of this chapter responded slowly, but eventually achieved continence. A combination of individual and family counseling, dietary changes, and behavioral interventions including rewards, biofeedback, and education was required to achieve this positive outcome.
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