International Children's Continence Society's recommendations for therapeutic intervention in congenital neuropathic bladder and bowel dysfunction in children.

PURPOSE: We present a consensus view of members of the International Children's Continence Society on the therapeutic intervention in congenital neuropatic bladder and bowel dysfunction in children. MATERIAL AND METHODS: Discussions were held by a group of pediatric urologists and gastroenterologists appointed by the board. The following draft review document was open to all the ICCS members via the ICCS web site. Feedback was considered by the core authors and by agreement, amendments were made as necessary. The final document is not a systematic literature review. It includes relevant research when available as well as expert opinion on the current understanding of therapeutic intervention in congenital neuropatic bladder and bowel dysfunction in children. RESULTS: Guidelines on pharmalogical and surgical intervention are presented. First the multiple modalities for intervention that do not involve surgical reconstruction are summarized concerning pharmacological agents, medical devices, and neuromodulation. The non-surgical intervention is promoted before undertaking major surgery. Indicators for non-surgical treatments depend on issues related to intravesical pressure, upper urinary tract status, prevalence of urinary tract infections, and the degree of incontinence. The optimal age for treatment of incontinence is also addressed. This is followed by a survey of specific treatments such as anticholinergics, botulinum-A toxin, antibiotics, and catheters. Neuromodulation of the bladder via intravesical electrical stimulation, sacral nerve stimulation, transcutaneous stimulation, and biofeedback is scrutinized. Then follows surgical intervention, which should be tailored to each individual, based on careful consideration of urodynamic findings, medical history, age, and presence of other disability. Treatments mentioned are: urethral dilation, vesicostomy, bladder, augmentation, fascial sling, artificial urinary sphincters, and bladder neck reconstruction and are summarized with regards to success rates and complications. Finally, the treatment on neuropathic bowel dysfunction with rectal suppositories irrigation and transrectal stimulation are scrutinized.

The management of adolescents with neurogenic urinary tract and bowel dysfunction.

Most children with neurogenic bladder dysfunction arrive into adolescence with reasonably managed lower urinary tract function only to experience bladder and kidney function deterioration after puberty. The aim of this article is to identify issues that contribute to adverse changes in bladder and renal function during adolescence and to highlight strategies to preserve urinary tract integrity, social continence, patient autonomy, and independence. Surveillance of bladder function requires patient attendance at review appointments and compliance with treatment plans. While encouraging independence and treatment compliance the clinician also needs to consider altered mental concentrating ability and fine motor skills of these patients. A keen eye for imminent loss of patient compliance to treatment protocol should be the mainstay of each encounter during puberty and adolescence. Annual surveillance of adolescent neurogenic bladder patients facilitates early identification of risk factors for urinary tract deterioration. Investigations include renal and bladder ultrasonography, urodynamic study when indicated, substantiated by videocystometry when anatomical status dictates. Serum creatinine should be measured and renal scintigraphy performed when upper urinary tract dilation, renal scarring, or atrophy are suspected. Optimal management of adolescents with neurologic disease of the urinary tract included strategies to reduce elevated detrusor pressure, maintain bladder compliance, and maximize dryness. Antimuscarinic medications, botulinum toxin A, and surgical procedures are enhanced by bowel management regimens and regular nurse or urotherapist patient contact. Caring for the patient as a whole requires discussion of sexuality, fertility status, and behaviors that increase the risk of progressive urinary tract damage.

Sacral agenesis and neurogenic bladder: Long-term outcomes of bladder and kidney function.

BACKGROUND: Sacral agenesis (SA) is a rare congenital condition that refers to the absence of part or all of two or more lower sacral vertebral bodies. It can be associated with neurogenic bladder dysfunction that does not necessarily correlate with the level of spinal or skeletal defect. Patients with SA should undergo urodynamic studies (UDS) to guide lower urinary tract (LUT) management. OBJECTIVE: This review aimed to update the present institutional experience since 1981 of this rare patient population with detailed, long-term follow-up of bladder and kidney function. STUDY DESIGN: A single institution, retrospective, IRB-approved review was performed on patients born after January 1, 1981 with an isolated diagnosis of sacral agenesis without spina bifida, and followed with urologic involvement at Boston Children's Hospital. Records were reviewed for demographics, radiologic imaging, UDS including cystometrogram (CMG) and electromyography (EMG), surgery, and blood chemistries. Comparisons were made between groups of patients based on age at diagnosis, with specific focus on renal function and stability of neurogenic bladder lesion. RESULTS: Forty-three patients were identified: 23 female and 20 male. Thirty-seven children (86%) had a known age of diagnosis. Nineteen were diagnosed before 2 months old, including five who were diagnosed prenatally, 11 were diagnosed between 2 and 18 months, and seven were diagnosed after 18 months. All 43 had UDS, with 24 (55.8%) studied at the time of diagnosis (Summary Table). Twenty had serial full UDS, with 30% demonstrating neurourologic instability. None developed end-stage renal disease (ESRD) or required spinal cord detethering. DISCUSSION: Many children with SA appeared to be diagnosed prenatally or early in life; SA was mostly identified during evaluation of associated anomalies. Though UDS aid in urologic management, testing was not routinely utilized at the time of diagnosis. CONCLUSIONS: This review of long-term follow-up in SA patients showed stable LUT and renal function, with minimal risk of progression to ESRD.

Management of functional nonretentive fecal incontinence in children: Recommendations from the International Children's Continence Society.

BACKGROUND: Fecal incontinence (FI) in children is frequently encountered in pediatric practice, and often occurs in combination with urinary incontinence. In most cases, FI is constipation-associated, but in 20% of children presenting with FI, no constipation or other underlying cause can be found - these children suffer from functional nonretentive fecal incontinence (FNRFI). OBJECTIVE: To summarize the evidence-based recommendations of the International Children's Continence Society for the evaluation and management of children with FNRFI. RECOMMENDATIONS: Functional nonretentive fecal incontinence is a clinical diagnosis based on medical history and physical examination. Except for determining colonic transit time, additional investigations are seldom indicated in the workup of FNRFI. Treatment should consist of education, a nonaccusatory approach, and a toileting program encompassing a daily bowel diary and a reward system. Special attention should be paid to psychosocial or behavioral problems, since these frequently occur in affected children. Functional nonretentive fecal incontinence is often difficult to treat, requiring prolonged therapies with incremental improvement on treatment and frequent relapses.

Clean, intermittent catheterization of infants with neurogenic bladder.

Clean, intermittent catheterization was instituted in 38 babies with myelodysplasia who were thought to be at risk for upper urinary tract deterioration because of neurogenic bladder dysfunction. There were 19 patients 2 weeks to 12 months of age, 11 were 1 to 2 years of age, and 8 were older than 2 years. Effectiveness of clean, intermittent catheterization was determined by maintenance of upper urinary tract stability. Upper urinary tracts improved or remained stable in 13 of 16 infants (81%) with reflux and 16 of 18 infants (89%) with detrusor-sphincter dyssynergia. Bacteriuria was present in 16 (42%), with only 2 infants (5%) having a febrile episode; no infant required hospitalization because of urinary tract infections. No further complications were identified in infants who were cleanly and intermittently catheterized. Most families found clean, intermittent catheterization of their infants easy to master and not stressful, and their children adjusted to it at an early age.

Hypoplastic dysplastic kidney with a vaginal ectopic ureter identified by technetium-99m-DMSA scintigraphy.

Ectopic insertion of a ureter draining a hypoplastic dysplastic kidney is a significant cause of urinary incontinence in girls. In this case, such a kidney was detected with 99mTc-DMSA scintigraphy but not by intravenous pyelography. Scintigraphy facilitated further delineation of the anatomy with CT prior to nephrectomy. Based on this case and a literature review, we suggest that 99mTc-DMSA scintigraphy be performed early when evaluating girls with urinary incontinence.

Vesical manifestations of chronic granulomatous disease in children. Its relation to eosinophilic cystitis.

Two children with chronic granulomatous disease involving the bladder are reported. Their clinical course was characterized by lower urinary tract symptoms, decreased vesical capacity, unilateral ureterovesical junction obstruction, and an intense eosinophilic infiltrate on biopsy specimens in each. Chronic granulomatous disease and eosinophilic cystitis appear to have remarkably similar clinical manifestations. A review of urinary tract involvement in chronic granulomatous disease and its comparison with eosinophilic cystitis is presented.

Complete duplication of bladder and urethra.

An unusual case of complete duplication of bladder and urethra is reported. The pertinent literature is reviewed and treatment discussed.

Megaureters in children.

The results of surgery for megaureter are reviewed in 40 children. Special attention is focused on the etiologic causes of these dilated ureters in relation to the surgical results. Thirty of 31 children with obstructive or refluxing megaureters and normal bladders had satisfactory results. Less satisfactory results occurred in children with abnormal bladder dynamics. No child in this series required upper ureteral tapering. A discussion of the causes and further management of the surgical failures is presented.

Dilemma of small pyelonephritic kidney associated with vesicoureteral reflux.

Nineteen children with severe unilateral pyelonephritic scarring secondary to reflux and infection were followed up for at least five years after ureteral reimplantation. The small kidneys in these children grew at a rate which paralleled expected normal, and this growth was independent of the degree of hypertrophy in the contralateral kidney or the size difference between each pair of renal units. The authors differentiate between obligate and compensatory renal growth and conclude that children with a small pyelonephritic kidney should undergo reimplantation rather than nephrectomy because of the recuperative powers of the growing kidney and its ability to become a potentially life-sustaining organ in the future.

Urachal anomalies: defining the best diagnostic modality.

OBJECTIVES: Urachal abnormalities are uncommon and the literature is primarily comprised of case reports. Conclusions regarding the presentation and diagnosis of these abnormalities may be elucidated by reviewing a large experience. METHODS: The records of 45 patients with urachal abnormalities in the pediatric age group were reviewed from 1970 to 1997. This included 24 boys and 21 girls with an age range from 1 day to 20 years (average 4.0 years). The presenting complaint was periumbilical discharge in 19 patients (42%), umbilical cyst or mass in 15 (33%), abdominal or periumbilical pain in 10 (22%), and dysuria in 1 (2%). The diagnosis consisted of a urachal sinus in 22 children (49%), a urachal cyst in 16 (36%), and a patent urachus in 7 (15%). Various radiographic studies were used to establish the diagnosis. RESULTS: Patients with a urachal sinus had 16 voiding cystourethrograms performed (only 1 diagnostic), 9 sinograms (all diagnostic), 8 ultrasounds (4 diagnostic), and 1 excretory urogram (normal). Those with a urachal cyst had 8 voiding cystourethrograms (1 diagnostic), 5 excretory urograms (all normal), 4 ultrasounds (all diagnostic), and 1 computed tomography scan (diagnostic). Children with a patent urachus had 2 excretory urograms (both diagnostic), 1 voiding cystourethrogram (diagnostic), and 2 ultrasounds (normal). One baby with a patent urachus was diagnosed prenatally during ultrasound screening. The diagnosis was made by history and physical examination alone in 5 children and at the time of surgery in 1. Treatment consisted of surgical excision of the urachal abnormality with a cuff of bladder in 22 children, surgical excision without a bladder cuff in 22, incision and drainage of a urachal cyst (1%), and laparoscopic excision of a patent urachus with a bladder cuff in another (1%). There were three wound infections postoperatively. None developed any long-term sequelae. CONCLUSIONS: The diagnosis of urachal abnormalities can be made with certainty if a good physical examination and the appropriate radiographic test are performed. A patient who presents with periumbilical drainage should have a sinogram performed, which should be diagnostic for both a urachal sinus and a patent urachus. Any child who presents with a periumbilical mass should have an ultrasound performed, which should be diagnostic for a urachal cyst.

Evaluation and management of children with sacral agenesis.

Sacral agenesis is a rare congenital anomaly of the lower vertebral column which usually produces lower urinary tract dysfunction. Sixteen children with varying degrees of sacral agenesis and urinary symptoms were extensively evaluated. The neurologic lesion varied from no denervation to a complete loss of sacral motor and sensory function. The treatment instituted was individualized and based on specific urodynamic and radiologic findings. Overall, 12 of the 16 children (75%) achieved continence. Clues to the diagnosis including symptoms, physical findings, and the relationship to maternal diabetes are discussed.

Urodynamic evaluation of boy with myelodysplasia and incontinence.

Twenty-two boys with myelodysplasia and incontinence were evaluated urodynamically. Three types of bladder function were noted, but each could not be correlated with any particular neuroligic level. The integrity of the external sphincter innervation was determined by electomyographic monitoring of periurethral striated muscle. Bladder sphincter dyssynergia was found in one of the boys with voluntary control, five with involuntary bladder contractions, and five with adynamic bladders who voided by Credé's method. A radiologically narrow external sphincter on voiding cystography could only be correlated with the bioelectric activity in children with dyssynergia. A narrow sphincter was also noted in three children with synergy and four with complete lower motor neuron lesions. Marked fibrosis was found to be the cause of narrowing of external sphincter area in these boys. Thus, urodynamic evaluation helped define the etiology of outlet obstruction when it was present in the boy with myelodysplasia. A classification of bladder sphincter function is proposed.

Congenital posterior urethral perineal fistulae: a unique form of urethral duplication.

Three unique cases of urethral duplication, often referred to as congenital posterior urethral perineal fistulae, are presented. Presenting signs and symptoms, evaluation, and treatment are discussed, and the literature is reviewed. Although labeled as a fistula in the literature, this entity most likely represents a distinct type of urethral duplication. A functional dorsal urethra separates it from the other forms of urethral duplication.

Urinary undiversion in patients with myelodysplasia and neurogenic bladder dysfunction. Report of a workshop.

This workshop was conducted in an attempt to analyze critically the role of reconstruction of the myelodysplastic patient who had undergone urinary diversion and to develop guidelines for selecting those patients in whom urinary undiversion might be undertaken safely. The collective experience initially seems to be acceptable; however, the authors emphasize the gravity of the decision and the complexity of the evaluation which must be undertaken prior to embarking on such reconstructive surgery. Contrary to some reports, we believe that the defunctionalized bladder frequently can be evaluated. Further, many of the contraindications to urinary undiversion have been identified and several of the hazards involved therein can be avoided. We believe that the neurogenic bladder is no longer an absolute contraindication to undiversion. Our experience suggests that undiversion is a reasonable surgical treatment in select patient with neurogenic bladder dysfunction. But, the decision to remove a satisfactorily functioning conduit must not be undertaken lightly. Patients should be selected only after a thorough, detailed, and properly conducted evaluation. A protocol has been developed which will hopefully assist in this evaluation. Perhaps additional shared experience will further refine and delineate the circumstances appropriate for reconstruction of these patients.

Urologic management of spinal cord injury in children.

This overview of the management of children with spinal cord injury has focused on a program based on bladder dynamics. The bladder is modulated pharmacologically and surgically, if necessary, to create a reservoir that stores urine at low pressure, and emptying is achieved by clean intermittent catheterization. The parent assumes the responsibility for bladder emptying when the child is unable to perform catheterization. Reflex voiding in a balanced fashion is not advocated. The program described not only avoids the deleterious effects of high voiding pressures, it also obviates the wearing of a urinary collection device, which is impractical for most prepubertal boys, and it provides for emptying at predictable intervals in order to make the child more socially acceptable. This approach seems to improve the child's self-image, and it should increase the chances of useful participation in society and a fulfilling life.

Pediatric neurourology.

Children with voiding disturbances or neurologic dysfunction of the lower urinary tract present challenges to the practicing clinician. With careful clinical and radiologic evaluation, many of these disturbances can be managed. Complex situations, however, generally require expert urodynamic evaluation to delineate the cause of the bladder dysfunction and to guide management. These investigations combined with long-term follow up should provide new insights for further improvement in the management of neurogenic bladders in children. Urodynamic studies also have given us insights into the pathophysiology of abnormal voiding states secondary to dysfunctional bladders in children with posterior urethral valves, the prune belly syndrome, and the Hinman syndrome.

Evaluation and management of urinary incontinence after surgery for posterior urethral valves.

Posterior urethral valves lead to an overlapping constellation of urodynamic abnormalities, often many years after the initial diagnosis and valve ablation. Voiding dysfunction and urinary incontinence in boys with a history of valves are seldom the result of sphincteric incompetence. Three major categories of bladder dysfunction may cause voiding symptoms: myogenic failure, detrusor hyperreflexia, and bladder hypertonia. The interaction of the severity and duration of valve obstruction may be important factors, but the determinants of ultimate bladder function in the setting of urethral valves remain unclear. A careful urodynamic assessment is needed to define the abnormal bladder and to select and evaluate therapy. Therapy should be designed to provide low-pressure urinary storage for a socially acceptable period of time as well as complete emptying of the bladder. Success may require several lines of treatment and demands patience, not only of the patient and his family, but of the physician.

Management of the obstructed urinary tract associated with neurogenic bladder dysfunction.

The child with neurogenic bladder dysfunction may suffer from lower urinary tract obstruction, which, if untreated, can result in upper urinary tract deterioration. It is prudent to identify those newborns and infants who are at risk for upper urinary tract deterioration and advise an appropriate treatment protocol. In order to identify those children at risk, a thorough examination is required, along with appropriate upper urinary tract imaging studies and urodynamic assessment of the bladder. Infants and children at risk for upper tract deterioration are those who have intermittent or continuous detrusor pressure elevation above 40 cm H2O. We have identified the safe period, which is the time during which the bladder remains at a pressure lower than 40 cm H2O. Our management protocol for each child is individualized and based on increasing the safe period. The safe period can be increased with a combination of intermittent catheterization, administration of medications, and, in some cases, surgical reconstruction. With compulsive evaluation and intensive management, many of the previous upper urinary tract changes associated with the obstructed neurogenic bladder can be averted. With the above factors in mind, we have defined the period of bladder filling and storage where the pressure is below 40 cm H2O as the safe period. The ideal bladder for maintenance of normal upper tracts would have a long safe period and low voiding pressure. Our treatment protocol for the neurogenic bladder is therefore dependent on identifying those children who have very short or nonexistent safe periods. These are the children who are at risk for upper tract deterioration.

Complications of urinary tract undiversion.

The complications of urinary tract undiversion can be divided into those associated with patient selection, choice of techniques, and early and late surgical difficulties. Patient selection must take into account the original reason for diversion (incontinence, hydronephrosis, reflux, or renal functional impairment), current renal function and bladder dynamics, upper urinary tract anatomy, and psychosocial considerations. Technical considerations require versatility and ingenuity on the part of the surgeon. Although direct uroepithelium-to-uroepithelium continuity is ideal, this often cannot be accomplished. A variety of techniques including transureteroureterostomy, psoas hitch of the bladder, bladder augmentation, ileocecal and colon interposition, and autotransplantation can be used to facilitate the refunctionalization of the urinary tract. One must monitor the patient postoperatively for potential early and late complications. Meticulous attention to surgical details will help minimize these. The ultimate goal is to achieve stable renal function and urinary continence.