**5. Diagnosis and evaluation**

obstruction, increase the intravesical pressure. Increased intravesical pressure can pro‐ mote VUR through a possible marginal competence in the valve mechanism [34, 35]. Re‐ cent studies have shown that the prevalence of VUR among children with idiopathic LUTD is between 14% and 46% [11, 12]. Some reports have emphasized bilateral reflux

Van Gool et al. addressed the relationship between DV and reflux for the first time with a retrospective questionnaire in 1992 [37]. The prevalence of uncoordination between the de‐ trusor and urethral sphincter approached 18% and included voiding pattern abnormalities such as urge syndrome, staccato voiding, fractionated and incomplete voiding, and voiding postponement. They also found that, in those children who had spontaneous resolution of

It is important to carefully assess all children with reflux for subtle signs of DV. Chil‐ dren with DV are more likely to have recurrent UTIs, have mild bilateral reflux with less spontaneous resolution, and are less likely to have success with surgical management. The treatment of DV in such children can improve the chances of spontaneous resolution of the reflux and may also reduce recurrent urinary infection. Koff et al. [3] reported on their series of children with VUR, who either resolved spontaneously or were surgically treated, and found that DES was observed in 43% of children with primary VUR and in 77% of a subset of these children who had breakthrough UTI. The presence of DES pat‐ terns was associated with a longer time for spontaneous resolution of low-grade reflux and with unsuccessful surgical outcomes. Of children in the surgically treated group, on‐ ly those with DES developed recurrent and/or contralateral reflux. Children with untreat‐ ed DV undergoing ureteral re-implantation may be at a higher risk for developing

Contrary to these results, Chen and colleagues reported that VUR and UTI are not inde‐ pendent of DV [39]. Their findings are new observations contradicting the previous belief that both UTI and VUR are independently associated with DES. Chen et al. performed a multivariate analysis on 2759 paediatric urology patients, further examining the relationship between DES, VUR, and UTI. Their data demonstrated a higher rate of DES in girls than in boys: 43.7% compared with 23.8%. This group also found that there was no difference in the presence of DES in patients with unilateral and bilateral VUR. Surprisingly, they observed no association of VUR or UTI individually with DES but rather DES was only noted when both of these issues were present. Although this large-scale multivariate analysis is a more statistically powerful study than previous smaller retrospective analyses, the data could be potentially skewed in that all of the patients were recruited from a paediatric urology popu‐ lation, which is not representative of the general paediatric population. Patients with known reflux could potentially be protected from DES by prophylactic antibiotics or continued clin‐ ical follow-up in a specialty setting. On the other hand, it is suggested that there may be two types of reflux: one that is primary or congenital in nature and another that is secondary and in part due to DES and UTI. This study is in line with the neuroplastic theory that postulates that hypertrophy of the bladder and bowel musculature is caused by trophic factors re‐ leased during pelvic floor hyperactivity secondary to central nervous disturbances [40]. Hy‐

associated with LUTD [12, 36].

94 Recent Advances in the Field of Urinary Tract Infections

their VUR, there was a lower prevalence of DV.

recurrent reflux or a new bladder diverticulum [38].

Clinical symptoms may vary from mild incontinence to severe disorders with endpoints of irreversible bladder dysfunction with VUR, UTI and resulting nephropathy [8]. Children with DV voiding often present with urinary incontinence both during the day as well as at night. They may have urinary frequency, urgency, urge incontinence or nocturnal enuresis. Such storage symptoms may result from associated detrusor overactivity, urinary infection or reduced bladder capacity consequent to large residual urine and may be aggravated by constipation or behavioural disorders. A distinctive facial expression may be noted in some of these patients [8].

Diagnosis relies heavily on a good history and physical examination, but also includes radi‐ ologic and urodynamic evaluation. The history should be directed towards the identification of children with neurologic or anatomic causes of their symptoms, and then distinguish be‐ tween which form or pattern of voiding dysfunction is present. Components of this history include maternal medical issues, perinatal history, developmental milestones, scholastic per‐ formance, behavioural history, specifics around toilet training, patterns of voiding and bow‐ el movements, history of UTI, and family history of voiding dysfunction. The use of a 3-day voiding diary is often helpful to identify the frequency of voiding, voided volumes, and tim‐ ing of incontinent episodes [9].

Clinical examination must include an assessment of higher mental functions and their ageappropriateness and basic neurological evaluation. On physical examination, attention should be paid to the back and lower spine for cutaneous manifestations of an occult spinal dysraphism and/or sacral agenesis. Neurologic examination should include assessment of lower extremity function, rectal tone, perineal/anal sensation, and intactness of bulbocaver‐ nosus reflex. The external genitalia should also be examined. Bowel function should be eval‐ uated in detail.

voiding and incontinence scoring system" and designed by Akbal et al. They reported that the children with a score of 8.5 or greater had voiding abnormalities with 90% sensitivity and 90% specificity [46]. The last one was devised by Afshar et al, *w*hich was a 14-item 5 point Likert scale questionnaire for children with dysfunctional elimination with a cut-off score of 11 [47]. This questionnaire was valid and reliable for diagnosing dysfunctional elim‐ ination syndrome. The validity of these scoring systems has not yet been evaluated in large

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**Figure 1.** Example of a child with staccato flow and high EMG activity on Uroflow-EMG.

Perineal USG has been used in women with DV to assess sphincter volume [48]. Perineal USG can also be used to evaluate paradoxical pelvic floor movement which may often be seen in children with DV [49]. Abdominal USG has also been used for chronic constipation

Children with DV often show abnormalities on voiding cystourethrogram (VCUG). A VCUG may demonstrate VUR, bladder trabeculation, a diverticulum, a large bladder ca‐ pacity, or a large post-void residual. Girls may exhibit the spinning top urethra during void‐ ing, which results from dilation of the posterior urethra secondary to detrusor-sphincter uncoordination during voiding [51] (Figure 2). Dilatation of the prostatic urethra may be ob‐ served in boys. VCUG should be performed when there is a history of recurrent UTI or a

in children with DV. Rectal diameter greater than 3.5 cm signifies constipation [50].

prospective trials.

Further evaluation of the child with DV continues with a urinalysis and urine culture. The scout film, or plain film of the abdomen (KUB), can be used to assess the spine and sacrum, and for evidence of constipation.

A renal and bladder ultrasonography with a pre-void and post-void image should be obtained to assess for evidence of obstructive uropathy, an ureterocele, bladder wall thickness, and re‐ sidual urine volume. Studies in healthy infants and toddlers have shown that they do not emp‐ ty the bladder completely every time but they do so at least once during a 4-hour observation period. In older children, a consistent residual of >20 ml is considered abnormal [29].

The diagnosis of DV in children hinges upon the repeated demonstration of a staccato pat‐ tern on uroflowmetry testing. The normal uroflow pattern is a bell-shaped curve with a smooth up-slope and down-slope. OAB may produce an explosive voiding contraction that appears in the flow measurement as a high amplitude curve of short duration, that is, a tow‐ er-shaped curve. A child with organic outlet tract obstruction often has low amplitude and rather even flow curve, that is, a plateau-shaped curve. Finally, in case of an underactive or acontractile detrusor when contraction of the abdominal muscles creates the main force for bladder evacuation, the flow curve usually shows discrete peaks corresponding to each strain, separated by segments with zero flow, namely, an interrupted or fractionated flow curve. The staccato pattern of voiding has been considered classical of DV. Sphincter overac‐ tivity during voiding is seen as sharp peaks and troughs in the flow curve, which is as an irregular or staccato flow curve [10] (Figure 1). To label flow as staccato, the fluctuations should be more than the square root of the maximum flow rate. When combined with nee‐ dle or surface EMG, increased striated urethral sphincter-pelvic floor complex activity can be noted [9]. Less invasive uroflowmetry, perineal electromyography, and PVR comprise the preferred modality at our institution for screening and monitoring response to treat‐ ment. Before flow study, bladder ultrasonography is used to ensure adequate volume and exclude patients with overdistention of the bladder. Overdistention of the bladder can ob‐ scure results, causing an artificial increase in PVR volumes in normal children [43].

There have been efforts to standardize scoring systems in the evaluation of children with DV. These scoring systems would be beneficial in classifying the type and severity of DV to determine necessary treatment modalities. Farhat et al. introduced the Dysfunctional Void‐ ing Scoring System (DVSS) by comparing the scores of the children aged 3-10 years with age-matched controls across 10 questions related to urinary incontinence, voiding habits, ur‐ gency, posturing, bowel habits and stressful life conditions [44]. Nine of these questions are scored between 0 and 3 depending on whether the problem is noted almost never (0), less than half the time (1), about half the time (2) or almost every time (3). The last question is addressed to the parents to identify a stress situation in the family. The authors derived cutoff values of 6 and 9 for girls and boys respectively for making a diagnosis of DV. A small prospective cohort was analyzed by Upadhyay and colleagues to determine the validity of the DVSS in children with reflux. A positive correlation between symptom score improve‐ ment and resolution of VUR was found [45]. Another scoring system is the "Dysfunctional voiding and incontinence scoring system" and designed by Akbal et al. They reported that the children with a score of 8.5 or greater had voiding abnormalities with 90% sensitivity and 90% specificity [46]. The last one was devised by Afshar et al, *w*hich was a 14-item 5 point Likert scale questionnaire for children with dysfunctional elimination with a cut-off score of 11 [47]. This questionnaire was valid and reliable for diagnosing dysfunctional elim‐ ination syndrome. The validity of these scoring systems has not yet been evaluated in large prospective trials.

nosus reflex. The external genitalia should also be examined. Bowel function should be eval‐

Further evaluation of the child with DV continues with a urinalysis and urine culture. The scout film, or plain film of the abdomen (KUB), can be used to assess the spine and sacrum,

A renal and bladder ultrasonography with a pre-void and post-void image should be obtained to assess for evidence of obstructive uropathy, an ureterocele, bladder wall thickness, and re‐ sidual urine volume. Studies in healthy infants and toddlers have shown that they do not emp‐ ty the bladder completely every time but they do so at least once during a 4-hour observation

The diagnosis of DV in children hinges upon the repeated demonstration of a staccato pat‐ tern on uroflowmetry testing. The normal uroflow pattern is a bell-shaped curve with a smooth up-slope and down-slope. OAB may produce an explosive voiding contraction that appears in the flow measurement as a high amplitude curve of short duration, that is, a tow‐ er-shaped curve. A child with organic outlet tract obstruction often has low amplitude and rather even flow curve, that is, a plateau-shaped curve. Finally, in case of an underactive or acontractile detrusor when contraction of the abdominal muscles creates the main force for bladder evacuation, the flow curve usually shows discrete peaks corresponding to each strain, separated by segments with zero flow, namely, an interrupted or fractionated flow curve. The staccato pattern of voiding has been considered classical of DV. Sphincter overac‐ tivity during voiding is seen as sharp peaks and troughs in the flow curve, which is as an irregular or staccato flow curve [10] (Figure 1). To label flow as staccato, the fluctuations should be more than the square root of the maximum flow rate. When combined with nee‐ dle or surface EMG, increased striated urethral sphincter-pelvic floor complex activity can be noted [9]. Less invasive uroflowmetry, perineal electromyography, and PVR comprise the preferred modality at our institution for screening and monitoring response to treat‐ ment. Before flow study, bladder ultrasonography is used to ensure adequate volume and exclude patients with overdistention of the bladder. Overdistention of the bladder can ob‐

period. In older children, a consistent residual of >20 ml is considered abnormal [29].

scure results, causing an artificial increase in PVR volumes in normal children [43].

There have been efforts to standardize scoring systems in the evaluation of children with DV. These scoring systems would be beneficial in classifying the type and severity of DV to determine necessary treatment modalities. Farhat et al. introduced the Dysfunctional Void‐ ing Scoring System (DVSS) by comparing the scores of the children aged 3-10 years with age-matched controls across 10 questions related to urinary incontinence, voiding habits, ur‐ gency, posturing, bowel habits and stressful life conditions [44]. Nine of these questions are scored between 0 and 3 depending on whether the problem is noted almost never (0), less than half the time (1), about half the time (2) or almost every time (3). The last question is addressed to the parents to identify a stress situation in the family. The authors derived cutoff values of 6 and 9 for girls and boys respectively for making a diagnosis of DV. A small prospective cohort was analyzed by Upadhyay and colleagues to determine the validity of the DVSS in children with reflux. A positive correlation between symptom score improve‐ ment and resolution of VUR was found [45]. Another scoring system is the "Dysfunctional

uated in detail.

and for evidence of constipation.

96 Recent Advances in the Field of Urinary Tract Infections

**Figure 1.** Example of a child with staccato flow and high EMG activity on Uroflow-EMG.

Perineal USG has been used in women with DV to assess sphincter volume [48]. Perineal USG can also be used to evaluate paradoxical pelvic floor movement which may often be seen in children with DV [49]. Abdominal USG has also been used for chronic constipation in children with DV. Rectal diameter greater than 3.5 cm signifies constipation [50].

Children with DV often show abnormalities on voiding cystourethrogram (VCUG). A VCUG may demonstrate VUR, bladder trabeculation, a diverticulum, a large bladder ca‐ pacity, or a large post-void residual. Girls may exhibit the spinning top urethra during void‐ ing, which results from dilation of the posterior urethra secondary to detrusor-sphincter uncoordination during voiding [51] (Figure 2). Dilatation of the prostatic urethra may be ob‐ served in boys. VCUG should be performed when there is a history of recurrent UTI or a febrile UTI, thickened bladder wall on ultrasound, children older than 5 years with day and nighttime incontinence, or children at puberty with persistent enuresis.

**6. Treatment of DV**

id intake and diet.

from incontinence.

recurrent UTIs.

pelvic floor during voiding.

cilitated at the initiation of management.

Urotherapy is the nonsurgical, nonpharmacologic treatment of lower urinary tract function and can be defined as a bladder re-education or rehabilitation program aiming at correction of filling and voiding difficulties. Standard urotherapy is a combination of informing the child and the family about the normal lower urinary tract function and what is abnormal in the patient's voiding and correcting the abnormal voiding habits, lifestyle with regard to flu‐

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In the setting of discomfort with voiding or dysuria, all efforts should be made to eliminate any dietary irritants, such as caffeine, carbonated beverages, citrus juices, and chocolate. Furthermore, skin care should be initiated in children with eroded or irritated perineal areas

Education emphasizing timed voiding, fluid management, and pelvic floor exercises are key components of the initial management for DV. In addition, education concerning proper posture during voiding should be emphasized to minimize abdominal musculature strain‐ ing. Proper sitting technique with buttock and foot support and comfortable hip position is necessary to enable voiding without recruitment of the abdominal muscles [53, 54]. Hygiene education is also important to limit local skin inflammation that may contribute to holding maneuvers and DV. In this way, coordinated voiding with a relaxed pelvic floor can be fa‐

Treatment of constipation is also important component of the initial management for DV [55]. Fecal impaction must be managed prior to maintenance therapy. For this purpose laxa‐ tives, stool softeners, and enemas is recommended. Maintenance with balanced diet, fibre supplementation, and oral medications such as mineral oil, polyethylene glycol and lactu‐ lose are recommended to maintain a goal of one bulky bowel movement a day. Treatment of constipation alone has been shown to resolve lower urinary tract abnormalities. Enuresis re‐ solved in 63% and daytime incontinence in 89% of patients presenting with constipation and incontinence in one study [56]. In the same study, resolution of constipation also resolved

Biofeedback therapy is the next line of treatment after conservative approaches have been initiated. Biofeedback is a specific treatment modality that aims to retrain the patient's void‐ ing with the assistance of a computer game. Biofeedback has been used in children as young as four years of age [57]. There is no standard protocol for the correct teaching of biofeed‐ back, but in general there are 2 methods. Real-time uroflowmetry allows the patient to view the urine flow rate that in turn can be used to teach the child to relax his/her pelvic floor musculature with voiding. This method is recommended in children with pelvic floor hy‐ peractivity and no OAB symptoms. Sphincter or pelvic floor electromyography can also be used to teach patients how to voluntarily control their pelvic floor musculature during void‐ ing and thus reduce or prevent detrusor-sphincter incoordination. The advantage in using this method lies in its ability to teach a guarding reflex in addition to the relaxation of the

**Figure 2.** A 6-year old female child with spinning top urethra and right grade 4 VUR on VCUG.

In patients with suspicion of anatomical abnormalities of the lower urinary tract, a diagnos‐ tic cystoscopy may be performed. Routine diagnostic cystoscopy is not recommended [52].

Urodynamic evaluation remains an uncomfortable test for children. Cooperation of the child and the narrow size of the urethra are the major hurdles. A relaxed child-friendly environment and patience on the part of the technician are paramount. Full urodynamic studies are considered invasive and should be reserved for children with neurogenic bladder dysfunction, severe DV, myogenic failure, or symptoms that do not improve with therapy [8, 52]. Urodynamic criteria for DV include too large or small bladder ca‐ pacity, poor bladder compliance, detrusor overactivity or premature contractions, an un‐ sustained voiding contraction, excessive voiding pressure, an intermittent uroflow pattern, or elevated residual urine.
