Bladder sphincter dysfunction is a common problem seen in up to 40% of patients admitted to pediatric urology outpatient clinics. Bladder and voiding disorders, or briefly voiding disorders, is a general heading given to all of the disorders that cover both the filling and emptying phase of the bladder. The main title of voiding disorders is often confused with the term dysfunctional voiding=voiding dysfunction, which is only a voiding phase disorder. For this reason, the International Childrens Continence Society (ICCS) uses the main title of Lower urinary tract dysfunction (LUT dysfunction) instead of the main title of voiding disorder. Differentiation of lower urinary tract dysfunctions by classification is quite complex because of the heterogeneity of symptoms and significant overlaps/overlappings between symptoms.
In order to evaluate and differentiate the symptoms of bladder and voiding disorders in a standard way, Farhat et al1 developed a Dysfunctional voiding scoring system in 2000. In Turkey, Akbal et al2 published the Dysfunctional voiding and incontinence scoring system in 2005. Although both of these two scoring systems are validated scoring systems in children in their original language, their translation into foreign languages has led to confusion with the main title of voiding dysfunction, which is a voiding phase problem and voiding dysfunction (characterized by intermittent or staccato voiding). Akbal et al3 changed the name of the same score system and republished it as Lower urinary tract symptom score in 2014. Again, Önen 4 from Turkey proposed a modified scoring system in 2014, called the prominent voiding disorders scale, in which constipation symptoms are also evaluated. Whether Turkish validation of the prominent voiding disorders scale has been carried out is not available in the source where it was published.
Confusion between the main title of voiding disorder and voiding dysfunction. First, it started with the difference in usage between Canadian English and American English, and then the same problem continued due to the problem of the English translation of the English-published score systems after the Turkish validation developed by Akbal et al in Turkey. This problem still seems to persist if the name of the scale suggested by Önen is not noticed when translated into English because it still has voiding disorder. 1,2,3,4
As a solution to these confusions, the lower urinary system recommended by ICCS instead of the main title of voiding disorders LUT dysfunction is a terminology that is generally accepted and used. Lower urinary system dysfunctions are examined under three main headings as neurogenic bladder sphincter dysfunctions, non-neurogenic bladder sphincter dysfunctions and structural anomalies.
It is clear that non-neurogenic bladder sphincter dysfunctions are the lower urinary tract symptoms seen in neurologically and anatomically normal children. What is unclear is to differentiate whether there is a neurogenic and anatomical anomaly in these children. If we cannot detect a neurological or anatomical anomaly that can be revealed with today’s technological possibilities, we include these patients in the group of non-neurogenic bladder sphincter dysfunctions. Perhaps in the future, some patients in this group may actually be found to have neurogenic anomalies. The same symptoms are seen in children with anatomical or neurogenic anomalies as well as without any anatomical and neurological disorders. Functional bladder sphincter dysfunctions have very similar symptoms to those seen in, for example, occult spinal dysraphism or stretched spinal cord syndrome. The symptoms of children with an anatomical obstruction at the bladder outlet are often the same as those of children with a diagnosis of dysfunctional voiding who are anatomically and neurogenically completely normal. In short, if we do not know in which symptoms and situations we should consider neurogenic and anatomical anomalies, there is a possibility that we may be wrong even in the main classification.
Lower urinary system symptoms
The International Children’s Continence Society (ICCS) first tried to classify and standardize lower urinary system dysfunction in children in 1998. In 2006, the ICCS standardization committee changed the terminology of lower urinary system dysfunctions in children and adolescents again, and finally updated the terminology of lower urinary system dysfunctions in 2014. 5,6,7 Therefore, the name given to the same disease in 1998 may be completely different from the name given to the same disease in 2014. Therefore, it is very important to use the latest terminology when preparing an article on bladder sphincter dysfunction.
All cases with bladder sphincter dysfunction should be questioned with a structured anamnesis form and special questionnaire forms. Whether there is incontinence (incontinence) and its frequency. If there is incontinence, it can be day, night or both day and night, frequency of voiding, urgency, voided volume, difficulty in starting urination (hesitancy), straining, weak urine stream Evaluation based on parameters such as wet stream, intermittent urination, painful urination (dysuria), instillation after urination, and constipation is required.
With the last amendment made by ICCS (International Children’s Continence Society) in 2014; According to which of the above symptoms are present, patients are classified as follows.
1.Over active bladder
2.Voiding postponement
3.Underactive bladder
4.Dysfunctional voiding
5.Bladder outlet obstruction
6.Stress incontinence
7.Giggle incontinence (Giggle incontinence)
8. Vaginal reflux
9. Extraordinary daytime only urinary frequency
10. Primary bladder neck dysfunction
Due to the close anatomical neighborhood between the bladder and the rectum, bladder dysfunctions are frequently accompanied by bowel dysfunctions. A full rectum affects the feeling of bladder filling, while a full bladder reduces the feeling of rectal filling.8 In this case, both bladder and bowel emptying problems are seen together. The ICCS now recommends using the title Bladder bowel dysfunction (BBD) instead of dysfunctional elimination syndrome (DES), which was suggested by Koff et al. If there is concomitant bowel dysfunction; bladder bowel dysfunction, if there is no accompanying bowel dysfunction, the main headings of lower urinary system dysfunction are used.
Diagnosis of lower urinary tract symptoms
When conducting research to diagnose children with lower urinary tract symptoms, a different approach is required before and after toilet training. It is essential to use non-invasive methods while making this evaluation. Invasive diagnostic tools are used in a small number of cases where it is difficult to diagnose. The most used non-invasive methods are; The patient’s anamnesis, voiding diary, voiding volume measurements and lower urinary tract symptoms score questionnaires were taken with a structured form.
The most common lower urinary tract symptoms are frequent urination (frequency), urgency, holding maneuvers (holding manoeuvres). Children with these symptoms can easily be diagnosed with clinically overactive bladder. While it was previously thought that the diagnosis of overactive bladder must be confirmed urodynamically, many studies have been published emphasizing that urodynamics is not absolutely necessary for all children with a prediagnosis of overactive bladder. After the widespread belief that urodynamic verification is not essential, many patients were diagnosed clinically and anticholinergic treatment was initiated. ICCS clinically diagnosed patients; He suggested that patients whose diagnosis is confirmed by overactive bladder and urodynamics be named as overactive detrusor.6 Thus, it can be understood at first glance whether the diagnosis is made only clinically or if it is confirmed urodynamically.
Vicious circle in overactive bladder; The decrease in capacity causes a feeling of urgency, the feeling of urgency causes an increase in contraction of the pelvic floor muscles, followed by the formation of bladder muscle hypertrophy. When clinical diagnosis of overactive bladder is made, it should be kept in mind that in addition to primary idiopathic overactive bladder patients, there may be cases of overactive bladder secondary to dysfunctional voiding, secondary to bladder outlet obstructions, delaying voiding, and even primary bladder neck dysfunctions. We found the association of overactive bladder and dysfunctional voiding to be 90% in Celal Bayar University series. According to Ural et al10, this association was 85.5% in the Ege University series. In general, the association of dysfunctional voiding and overactive bladder has been reported in the range of 85-90%.11 ICCS emphasized that dysfunctional voiding is only a problem of the voiding phase, and although it said that there may be an accompanying overactive bladder, it did not emphasize that the probability of co-occurrence of these two diseases is around 90% on average.7 Bladder Outflow obstructions are also seen with a high rate of overactive bladder in early ages, while they are seen together with underactive bladder in older ages. In our series, due to the large number of cases diagnosed at an advanced age, bladder outlet obstructions were found with less active bladder and less overactive bladder. Teams that frequently deal with patients with lower urinary tract disorders and frequently perform uroflow and urodynamics are of course aware of these associations. However, some physicians who easily diagnose overactive bladder clinically may not be aware that overactive bladder may occur secondary to another pathology such as dysfunctional voiding, bladder outlet obstruction, primary bladder neck dysfunctions. For these reasons, we encounter many patients who have been started on anticholinergic treatment in recent years, but have not responded, and their families are told that they are drug-resistant. When the diagnosis of overactive bladder is made with only clinical symptoms, the symptoms that may be a symptom of dysfunctional voiding and bladder outlet obstruction, intermittent voiding, difficulty in starting to void, fine voiding and urinating by straining should be questioned. Although the patient or his family can give some information about intermittent urination, they generally cannot give information about wavy urination (staccato), long-term urination, and fine urination.
Uroflow, which is a non-invasive test in children, is not sufficient on its own. Uroflow + EMG test, which is also a non-invasive test, should be preferred. This test consists of making the child’s urine in an ideal sitting position in a container with scales and simultaneously recording pelvic floor activity with 3 self-adhesive transcutaneous EMG electrodes placed on the right and left of the genital area and on the leg. Instead of urinating in the form of a normal bell curve with Uroflow; tower-style voiding (overactive bladder sign) or plateau-style voiding, intermittent voiding and prolonged voiding time (bladder outlet obstruction sign) or staccato voiding (dysfunctional voiding sign) may be detected. In addition, the diagnosis can be made more accurately by observing increased pelvic floor activity (dysfunctional voiding sign), negative EMG Lag time (overactive bladder sign), long EMG Lag time (bladder outlet obstruction or primary bladder neck dysfunction) findings in EMG. Although the presence of these findings in uroflow + EMG is significant for the accuracy of the diagnosis, the normal or abnormality of a single uroflow + EMG test is not evidence of bladder outlet obstruction or primary bladder neck dysfunction in these children. Because children can turn plateau voiding into normal bell curve voiding by increasing bladder pressure. In order for the result of the Uroflow+EMG test to be accepted as correct, it is necessary to urinate at least 100cc and to perform 2 or 3 consecutive times, and the same result should be obtained in all of them.
While performing Uroflow, findings suggestive of external urethral meatal anomaly, which is seen in girls and causes dysfunctional voiding, may also be encountered. These children have an anterior deflected of urinary stream. Even if you question it in the anamnesis, this forward orientation cannot be adequately described. While performing Uroflow, it can be observed that the urine flow is directed forward in the ideal sitting position, and the toilet seat and even the legs get wet with pee. In these children with anterior orientation, a detailed genital examination can be performed and the diagnosis of anomalies described as meatal webbed or covered hypospadias (coverd hypospadias) can be made.
In cases with dysfunctional voiding, bladder outlet obstruction and primary bladder neck dysfunction, residual urine retention and urinary tract infection frequency are very high. Therefore, a complete urinalysis and ultrasonography, which is another non-invasive test, will be required in these cases. With ultrasonography, it can be learned whether there is dilatation in the upper urinary system, bladder trabeculation and wall thickness, bladder capacity, and most importantly, whether there is residual urine in the bladder after voiding. By measuring the rectal diameter with ultrasonography, if the rectal diameter is more than 3 cm, the presence of constipation can be proven. Incomplete emptying of the bladder after voiding, as well as residual urine, can be very important evidence of bladder outlet obstruction or dysfunctional voiding. An underactive bladder should be considered if there is a bladder capacity well above the bladder capacity calculated according to the child’s age by ultrasonography and residual urine in the post-void ultrasonography.
While dysfunctional voiding is more common in girls, urethral anomalies causing bladder outlet obstruction are seen in boys. The most accurate diagnosis among these diseases, which can be seen not only with lower urinary system symptoms but also with uroflow + EMG findings, can only be made with urodynamics, which is a highly invasive test in which intra-bladder and abdominal pressures can also be measured. If there are detrusor contractions that cannot be inhibited in the filling phase of the bladder, and if external urethral sphincter contractions and increased pelvic floor activity are noted on EMG in the voiding phase, and a staccato voiding pattern is observed in the voiding curve, but we are sure that there is no obstructive anomaly in the urethra, we can diagnose dysfunctional voiding. At this stage, we prefer to perform cystoscopy instead of voiding cystourethrography (ISUG). We think that cystography performed by an experienced hand is more valuable than ISUG in demonstrating urethral anomalies. Voiding cystourethrography (iSUG) was previously considered the gold standard in the diagnosis of bladder outlet obstructions, but in a symposium held in this field this year, it was emphasized that cystoscopy is much superior in demonstrating urethral obstruction.
Staccato voiding pattern, which ICCS attaches great importance to, and an increase in pelvic floor activity in EMG are seen only in 1/3 of patients with a diagnosis of dysfunctional voiding. The remaining 2/3 patients with a Staccato voiding pattern and no increase in pelvic floor activity on EMG have primary bladder neck obstruction or an underactive bladder.11 In lower urinary tract dysfunctions, not only symptoms but often diagnoses can be seen together in the same patient (Figure 1). therefore, it is the diagnosis made by ISUG (video urodynamics) performed at the same time as the urodynamic examination. However, even with videourodynamics, it is possible to miss some urethral obstructions. For this reason, cystoscopy may be required in cases with difficulty in diagnosis. Causes of obstruction in the urethra in boys; late detected posterior urethral valve (PUV), mild forms of congenital urethral obstructions (Flap valve, mini valve), syringocele, utricular hood, utricular cyst, Cobbs’s colar (Moormann’s ring), meatal stenosis.
In our series, meatotomy and/or meatoplasty were applied to meatal stenosis cases who were circumcised in the newborn period and presented with complaints of daytime urinary incontinence, and to cases with meatal cenosis who complained of urinary incontinence after hypospadias surgery. When syringocele is diagnosed with ISUG or cystoscopy, it should absolutely be drained. Patients with delayed diagnosis of PUV presenting with daytime urinary incontinence should be followed closely after valve ablation. In our series, there are many cases in which we diagnosed as congenital posterior urethral valve (PUV) in boys who presented with complaints of daytime urinary incontinence after toilet training. We also found many cases of incomplete congenital posterior urethral valve (flap valve, mini valve) and Cobb’s colar. We have been endoscopically applying trans urethral incision (TUI) at 5 and 7 o’clock positions with a cold knife in obstructions called flab valve (mini malve) and Cobb’s colar anomaly since 2009. We showed significant improvement in symptoms and uroflow+EMG findings after TUI.12,13 There is no complete agreement between the treatment methods applied to mild forms of congenital urethral obstruction and Cobbs’ colar (Moormann’s ring) cases. Koff 14 stated that every anomaly that prevents urinary flow is an obstruction and will cause kidney damage if left untreated. Some believe that mild forms of congenital urethral obstructions are a misnomer that started when a famous Australian urologist named it congenital obstructive posterior urethral membrane (COPUM), and that an anomaly called Cobb’s colar or Moormann’s ring, which is also known as COPUM, never existed.15 Imaji et al. 16 COPUM. They graded membranous lesions as severe, moderate and mild (severe, moderate, minimal). They reported that 45% of 83 congenital posterior urethral obstructive lesions required a second fulguration. 17 Khiara et al. performed trans urethral incision (TUI) in congenital urethral lesions in boys and reported that it was 80% effective.18 Nakamura et al19 in an article published in 2011 showed significant changes in urethral angles after TUI by performing urodynamics and voiding cystourethrography before and after TUI in mild forms of urethral obstructions, and they stated that transurethral incision provided 87.5% improvement in daytime urinary incontinence symptoms. At the symposium titled Surgical treatment of urinary incontinence in children with non –neurogenic lower urinary tract dysfunction, which I attended this year, treatments applied to urethral anomalies such as Flap valve(mini valve), and Cobb’s collar(Moormann’s ring) were discussed and it was emphasized that they should be surgically discontinued.20 In fact, Cobb’s colar(Moormann’s ring) was discontinued, 25% of cases relapsed and it was necessary to cut it a second time.21
Less known anomalies in the external urethral meatus in girls, especially those that cause dysfunctional voiding; Anomalies such as webbed meatus, covered hypospadias, ring of Lyon may rarely be noticed when inserting a catheter for ISUG or urodynamics. In doubtful cases, it can only be revealed by a detailed examination under general anesthesia. Correction of meatal anomalies seen in girls with meatotomy or even meatoplasty with sutures results in the improvement of dysfunctional voiding without the need for any additional treatment in 45% of the cases. Standard urotherapy was added to the treatment, and a few cases required intensive urotherapy.21
In conclusion; Bladder sphincter dysfunctions constitute an important part of patients who apply to pediatric nephrology and urology outpatient clinics. Unfortunately, there was a period when these patients were diagnosed with overactive bladder based on only some clinical symptoms and immediately given anticholinergic treatment. Apart from primary idiopathic overactive bladder cases that respond very well to anticholinergic treatment, the perspective and the way to find the primary specific cause in secondary overactive bladders are explained in detail in this review article.
References
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