Bowel and Bladder Research: Children with Spina Bifida

Achieving fecal continence in patients with spina bifida: a descriptive cohort study.

Vande Velde S, Van Biervliet S, Van Renterghem K, Van Laecke E, Hoebeke P, Van Winckel M.

Department of Pediatric Gastroenterology and Surgery, University Hospital Ghent, Ghent, Belgium.  J Urol. 2007 Dec;178(6):2640-4; discussion 2644. Epub 2007 Oct 22.

PURPOSE: Fecal incontinence is a major problem in patients with myelomeningocele. We evaluate the results of a stratified approach aimed at obtaining fecal pseudo-continence in patients with myelomeningocele.

MATERIALS AND METHODS: We conducted a cross-sectional descriptive study of last file data in 80 patients 5 to 18 years old with myelomeningocele followed at our center. Beginning at birth patients with myelomeningocele were seen at least annually by the pediatric gastroenterologist, a member of the multidisciplinary “spina team.” Constipation was treated with diet and osmotic laxatives. Starting at age 5 years, treatment was targeted at achieving pseudo-continence. A toilet training scheme was started, associated with induced defecation by digital stimulation. Retrograde tap water enemas were used in patients with unsatisfactory results. If retrograde enemas were unsuccessful, an antegrade continence enema procedure was proposed. For children unable to sit on a toilet regular manual evacuation of stools was advised.

RESULTS: Eight of the 80 patients were fecal continent. Pseudo-continence was achieved in 50 of the 72 incontinent patients (69%), including 5 of 5 following only a strict toilet scheme, 21 of 24 (87.5%) performing retrograde enemas, 16 of 20 (80%) performing orthograde enemas through an antegrade continence device and 8 of 10 performing regular manual evacuation of stools. In 4 of the 20 patients (20%) performing orthograde enemas complications led to closure of the antegrade continence device. Treatment failed and was stopped in 17 patients. Success of treatment was not related to level of spinal lesion or degree of mobility.

CONCLUSIONS: Fecal pseudo-continence was achieved in 58 of 80 patients (72.5%) with myelomeningocele.


An artificial somatic-autonomic reflex pathway procedure for bladder control in children with spina bifida.

Xiao CG, Du MX, Li B, Liu Z, Chen M, Chen ZH, Cheng P, Xue XN, Shapiro E, Lepor H.

Departments of Urology, Tongji Medical College, Xiehe Hospital, Huazhong University of Science and Technology, Wuhan, China.  J Urol. 2005 Jun;173(6):2112-6.  Comment in:  J Urol. 2005 Jun;173(6):1850-1.

PURPOSE: Neurogenic bladder is a major problem for children with spina bifida. Despite rigorous pharmacological and surgical treatment, incontinence, urinary tract infections and upper tract deterioration remain problematic. We have previously demonstrated the ability to establish surgically a skin-central nervous system-bladder reflex pathway in patients with spinal cord injury with restoration of bladder storage and emptying. We report our experience with this procedure in 20 children with spina bifida.

MATERIALS AND METHODS: All children with spina bifida and neurogenic bladder underwent limited laminectomy and a lumbar ventral root (VR) to S3 VR microanastomosis. The L5 dorsal root was left intact as the afferent branch of the somatic-autonomic reflex pathway after axonal regeneration. All patients underwent urodynamic evaluation before and after surgery.

RESULTS: Preoperative urodynamic studies revealed 2 types of bladder dysfunction- areflexic bladder (14 patients) and hyperreflexic bladder with detrusor external sphincter dyssynergia (6). All children were incontinent. Of the 20 patients 17 gained satisfactory bladder control and continence within 8 to 12 months after VR microanastomosis. Of the 14 patients with areflexic bladder 12 (86%) showed improvement. In these cases bladder capacity increased from 117.28 to 208.71 ml, and mean maximum detrusor pressure increased from 18.35 to 32.57 cm H2O. Five of the 6 patients with hyperreflexic bladder demonstrated improvement, with resolution of incontinence. Urodynamic studies in these cases revealed a change from detrusor hyperreflexia with detrusor external sphincter dyssynergia and high detrusor pressure to nearly normal storage and synergic voiding. In these cases mean bladder capacity increased from 94.33 to 177.83 ml, and post-void residual urine decreased from 70.17 to 23.67 ml. Overall, 3 patients failed to exhibit any improvement.

CONCLUSIONS: The artificial somatic-autonomic reflex arc procedure is an effective and safe treatment to restore bladder continence and reverse bladder dysfunction for patients with spina bifida.


Augmentation cystoplasty rates at children’s hospitals in the United States: a pediatric health information system database study.

Lendvay TS, Cowan CA, Mitchell MM, Joyner BD, Grady RW.  Children’s Hospital and Regional Medical Center, University of Washington School of Medicine, 4800 Sand Point Way NE, Seattle, WA 98105, USA.  J Urol. 2006 Oct;176(4 Pt 2):1716-20.

PURPOSE: We identified augmentation cystoplasty rates in children with spina bifida at children’s hospitals enrolled in the Pediatric Health Information System database. MATERIALS AND METHODS: The Pediatric Health Information System database tabulates demographic and diagnostic patient data from 35 children’s hospital centers in the United States. Between October 1999 and September 2004 we extracted data on 0 to 19-year-old patients with International Classification of Diseases-9 diagnosis codes for spina bifida. The International Classification of Diseases-9 procedure code for augmentation cystoplasty was cross-referenced with these patients to determine the total number of patients with augmentation, total population augmentation rates and individual institution rates of bladder augmentation.

RESULTS: Staff at enrolled pediatric medical centers submitted inpatient data accounting for 9,059 beds servicing an aggregate metropolitan population of 82 million individuals. In the 5-year period 12,925 unique spina bifida patient encounters were identified, including 665 patients who underwent augmentation cystoplasty. The mean 5-year institutional number of augmentations performed in children with spina bifida was 20 (range 1 to 121) and the mean annual number of augmentations performed per institution was 4. The overall augmentation rate at 33 hospitals contributing data for the full years 2000 to 2003 was 5.4% (range 0.5% to 16.3%, p <0.0001). The male-to-female ratio of those who underwent augmentation was 1:1.2. Median length of stay in children with augmentation was 7 days (mean 9). The median age of children with augmentation was 10.4 years, that is 11.3 years in boys and 9.8 years in girls. The difference in mean age was statistically significant (p <0.003). At institutions where 10 or more augmentations were performed in 5 years (mean 27) mean patient age at operation was 10.1 years. This was significantly younger than the mean patient age of 12.3 years at hospitals where fewer than 10 augmentations (mean 5) were done in 5 years (p <0.05).

CONCLUSIONS: Clinical management for neurogenic bladder conditions has evolved to emphasize nonoperative management. Several studies suggest that aggressive early intervention improves bladder compliance and may protect renal function. However, results from the Pediatric Health Information System database demonstrate no change in augmentation rates during this time and they demonstrate significant interinstitutional variability. To our knowledge this represents the largest series of augmentation cystoplasty in children with spina bifida to date.


Current approaches to the urologic care of children with spina bifida.

Joseph DB.  Section of Pediatric Urology, University of Alabama at Birmingham, Children’s Hospital, 1600 7th Avenue, South, Birmingham, AL 35233, USA.  Curr Urol Rep. 2008 Mar;9(2):151-7.

Most children born with spina bifida, the most common disabling congenital abnormality, have normal renal function. If left untreated, more than half of these children will have serious renal deterioration by age 5. This deterioration is secondary to hostile neurogenic changes of the bladder. Renal development should follow a normal course when close evaluation and intervention are undertaken during the newborn period and toddler years. As children age, attention is directed to quality-of-life issues, such as the establishment of urinary and bowel continence. Teenagers face the responsibility of understanding their medical condition and should begin to assume responsibility for their own care with eventual transition to the adult health care system. This article describes the foundations of management, beginning at birth, for caring for children with spina bifida.


Evaluation of clinico-urodynamic outcome of bladder dysfunction after surgery in children with spinal dysraphism – a prospective study.

Kumar R, Singhal N, Gupta M, Kapoor R, Mahapatra AK.  Department of Neurosurgery, Sanjay Gandhi Institute of Postgraduate Medical Sciences, Lucknow, India.  Acta Neurochir (Wien). 2008 Feb;150(2):129-37. Epub 2008 Jan 30.

BACKGROUND: The aim was to asses the clinical profile and urodynamic findings and the outcome of surgery in children with spinal dysraphism.

METHOD: Twenty five children with spinal dysraphism who were treated at our institute between January 2005 and June 2006 were studied prospectively. There were 10 with an open spina bifida and 15 closed. The clinical profile of bladder dysfunction was assessed and urodynamic evaluation done pre-operatively in each child. An ultrasound study of the kidneys was also done to assess for hydro-uretero-nephrois. All underwent surgery for their primary and associated malformations. Post-operatively, re-evaluation of bladder dysfunction and urodynamic parameters was performed at 6-8 months. RESULTS: Before operation there was a history indicating that the bladder was involved in 14 of the 25 children. Six of the 10 cases with an open spina bifida showed clinical involvement of the bladder as compared to 8 of 15 with a closed pattern. Urodynamic studies showed evidence of bladder dysfunction in 19 children. Of 10 with a meningomyelocele, there were abnormal urodynamics in 9 as compared to 10 of 15 with closed dysraphism. Follow up urodynamic studies showed improvement in 9 of 20 children 3 of 7 with a meningomyelocele and 6 of 13 with closed dysraphism. CONCLUSION: Children with open spina bifida, as compared to closed dysraphism, tend to have more bladder dysfunction as exemplified on clinical history and urodynamic assessment. A pre-operative urodynamic study helps to identify severity of bladder dysfunction which is clinically overt cases and also identifies subtle bladder dysfunction in clinically silent cases. Evaluation after operation tends to shows better outcome in children with closed dysraphism. The study also identifies deterioration in some patients with seemingly clinical improvement.


14 years of experience with the artificial urinary sphincter in children and adolescents without spina bifida.

Ruiz E, Puigdevall J, Moldes J, Lobos P, Boer M, Ithurralde J, Escalante J, de Badiola F.  Section of Pediatric Surgery and Pediatric Urology, Department of Pediatrics, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.  J Urol. 2006 Oct;176(4 Pt 2):1821-5.

PURPOSE: The efficacy of the artificial urinary sphincter to treat sphincteric incontinence in pediatric patients with spina bifida has been clearly reported. The possibility of maintaining spontaneous voiding has usually been the main reason for prosthetic device surgery. We reviewed our experience with the artificial urinary sphincter in patients without spina bifida who had had previous surgery of the bladder neck or proximal urethra.

MATERIALS AND METHODS: From 1990 to 2004, 112 children and adolescents underwent implantation of an AMS 800 artificial urinary sphincter. Of the patients 19 males and 4 females (20.5%) between ages 4 and 17 years (mean 8.1) had no spina bifida. Instead there were bladder exstrophy in 12 patients, anorectal malformation with a rectourethral or vesical fistula in 7 and epispadias in 4. A bladder neck cuff between 5.5 and 7.5 cm, and a 61-70 balloon were used in all patients.

RESULTS: Only 1 patient was lost to followup. In 22 patients (95.6%) mean followup was 80 months (range 4 to 155). Three sphincters in patients with exstrophy were removed because of erosion and/or infection 5, 49 and 60 months after initial surgery, respectively. A total of 19 sphincters remained in place (86.3% survival rate) with 5 revisions (26.3%) because of the pump (2), the cuff (2) or balloon fluid leakage. In this group 13 patients (68.4%) voided spontaneously and 6 (31.6%) performed clean intermittent catheterization, although 3 also voided spontaneously. Overall continence was good in 87% of patients because 2 were still incontinent at night.

CONCLUSIONS: The artificial urinary sphincter is a good long-term solution to urinary incontinence secondary to sphincter incompetence despite multiple previous surgeries of the bladder neck or proximal urethra. Patients with bladder exstrophy and many previous bladder procedures are more exposed to complications such as erosion compared with patients with epispadias or anorectal malformation. The high percent of patients maintaining spontaneous voiding and the good rate of continence are the most important benefits of this type of surgical option for sphincter incompetence.


Initial urologic management of myelomeningocele.

Snodgrass WT, Adams R.  Pediatric Urology and Developmental Disabilities, Texas Scottish Rite Hospital for Children, 2222 Welborn Street, Dallas, TX 75219, USA.  Urol Clin North Am. 2004 Aug;31(3):427-34, viii.

Disordered innervation of the detrusor musculature and external sphincter in patients with myelomeningocele adversely affects bladder function and potentially threatens the upper urinary tracts. Children with this condition can be categorized into high and low- risk groups for secondary damage from a neurogenic bladder based on intravesical pressure. Those with elevated pressure are at risk for hydronephrosis or reflux,and evidence suggests that early management of high pressure protects the bladder from additional damage, reducing the need for augmentation. Management decisions made during infancy potentially impact long-term outcomes for preserving renal function and achieving urinary continence. This discussion focuses on the initial urologic care of infants with myelomeningocele, emphasizing the controversy regarding urodynamic versus radiologic-based management of the neurogenic bladder.

Is an anal plug useful in the treatment of fecal incontinence in children with spina bifida or anal atresia?

Van Winckel M, Van Biervliet S, Van Laecke E, Hoebeke P.

Department of Paediatrics, University Hospital Ghent, De Pintelaan 185, B-9000 Gent, Belgium.  J Urol. 2006 Jul;176(1):342-4.

PURPOSE: We evaluated the efficacy and tolerance of the Conveen anal plug in children with spina bifida or anal atresia with persistent fecal incontinence necessitating diapers despite bowel management.

MATERIALS AND METHODS: Seven 4 to 12-year-old patients with high congenital imperforate anus and 9 who were 6 to 13 years old with spina bifida, no mental retardation and no involuntary urine loss on clean intermittent catheterization were included in the study. During a prospective, 6-week crossover descriptive study after a test period to find the most comfortable plug with a diameter of 37 or 45 mm patients and parents completed a diary with the number of soiling episodes, stool frequency, stool consistency and the number of diapers used during 3 weeks without and with the plug, respectively. They provided a final assessment of the device.

RESULTS: Two of the 7 patients with congenital imperforate anus discontinued use because of pain and discomfort, 1 had a decrease in soiling episodes and 4 achieved full continence and needed no diapers while using 2 plugs daily (range 1 to 4). All patients preferred the smaller plug. Two of the 9 patients with spina bifida always lost the plug within 1 hour after introduction, 5 had a decrease in soiling episodes but continued to need diapers and 2 achieved full continence using 2 plugs daily (range 1 to 4). All patients preferred the larger plug.

CONCLUSIONS: The Conveen anal plug is an adjuvant treatment option for fecal incontinence in children with congenital imperforate anus or spina bifida, enabling a minority to stop using diapers. The Conveen anal plug is not a universal solution for fecal incontinence problems in these patients.

Location of spina bifida occulta and ultrasonographic bladder abnormalities predict the outcome of treatment for primary nocturnal enuresis in children.

Miyazato M, Sugaya K, Nishijima S, Owan T, Ogawa Y.  Division of Urology, Department of Organ-Oriented Medicine, Faculty of Medicine, University of the Ryukyus, Okinawa, Japan.  Int J Urol. 2007 Jan;14(1):33-8.

OBJECTIVE: We examined whether the existence of spina bifida occulta or ultrasonographic bladder abnormalities was related to the outcome of treatment for primary nocturnal enuresis in children.

METHODS: Between April 1996 and September 2005, a total of 77 subjects (53 boys and 24 girls, aged 5-18 years; mean age, 9.9 years) with primary nocturnal enuresis were studied. Plain X-ray films of the spine and ultrasonographic bladder measurements (bladder wall thickness, bladder neck descent and bladder neck opening) were obtained and the correlations with the outcome of treatment using imipramine hydrochloride were assessed.

RESULTS: On plain X-ray films of the spine, spina bifida occulta (lumbar vertebrae in three, lumbosacral vertebrae in 19 and sacral vertebrae in 31) was recognized in 53 children (69%). Ultrasonographic bladder abnormalities were recognized in 40 children (52%). Children with lumbar and lumbosacral spina bifida occulta showed a higher rate of concomitant ultrasonographic bladder abnormalities (P = 0.006) and had a poorer response to treatment (P = 0.041) compared with the children who had sacral spina bifida occulta. Children with ultrasonographic bladder abnormalities had a worse response to treatment (P = 0.005) compared to the children without bladder abnormalities.

CONCLUSIONS: The presence of lumbar spina bifida occulta and ultrasonographic bladder abnormalities was related to the outcome of treatment for primary nocturnal enuresis in children, suggesting that spinal radiography and vesical ultrasonography may be useful predictive tests.


Lower urinary tract reconstruction for spina bifida — does it improve health related quality of life?

Macneily AE, Morrell J, Secord S.  Division of Urology, University of British Columbia, Vancouver, BC, Canada.  J Urol. 2005 Oct;174(4 Pt 2):1637-43; discussion 1643.

PURPOSE: Reconstruction for incontinent myelomeningocele is assumed to improve health related quality of life (HRQOL) yet there are no published data to support this assumption.

MATERIALS AND METHODS: A retrospective cohort study was performed of 36 consecutive incontinent meningomyelocele cases undergoing surgery (augmentation, with or without Mitrofanoff, bladder neck reconstruction and cecostomy). Controls were matched 2:1 for age, lesion level, parental marital status, ambulatory status and shunt status. HRQOL was assessed with a previously validated disease specific discriminative instrument that stratifies for ages 12 years or less and 13 years or greater. An additional 5-point Likert questionnaire was used for self-scoring of bladder and bowel continence. RESULTS: Responses were 89% and 84% for cases and controls, respectively. The 2-sample t testing for subjects 12 years old or younger revealed no significant difference in mean HRQOL score between those who underwent reconstruction (12, mean 165 +/- 23) and those who did not (9, mean 162 +/- 27, p=0.73). Results in older subjects were similar for cases (20, mean 190 +/- 23) and controls (3, mean 192 +/- 26, p=0.80). This finding occurred despite the fact that 78% of reconstructed cases achieved urinary continence for 3 hours or more with equal or superior self-reported bladder and bowel continence compared to controls.

CONCLUSIONS: We were unable to demonstrate superior HRQOL in patients over controls. Several possible interpretations exist, such as surgery may have no impact on HRQOL, patients might have scored much lower without surgery, or perhaps only caregiver quality of life improves. The impact of urinary tract reconstruction upon quality of life in the myelomeningocele population warrants further study.


Robotic-assisted laparoscopic mitrofanoff appendicovesicostomy and antegrade continent enema colon tube creation in a pediatric spina bifida patient.

Lendvay TS, Shnorhavorian M, Grady RW.  Division of Pediatric Urology, Children’s Hospital and Regional Medical Center, Seattle, WA 98105, USA. J Laparoendosc Adv Surg Tech A. 2008 Apr;18(2):310-2.

Robotic-assisted laparoscopic surgery has been applied to pediatric surgery, especially for technically challenging reconstructive procedures owing to the improved suturing capabilities over pure laparoscopic techniques when using fine suture material. In this paper, we report the techniques of creating both an appendicovesicostomy and an antegrade continent enema colon tube in a 9-year-old female with a neurogenic bladder and bowel secondary to myelomeningocele.


Surgical management of the neurogenic bladder and bowel.

Mingin GC, Baskin LS.

Pediatric Urology, Children’s Hospital, University of California, San Francisco, California 94143-0738, USA.  Int Braz J Urol. 2003 Jan-Feb;29(1):53-61.

Spina bifida and myelodysplasia are associated with neurogenic abnormalities of the bladder and bowel function. All children with myelodysplasia require an evaluation of their urinary tract with ultrasound and urodynamics to confirm normal bladder and kidney function. Patients with anatomical and functional abnormalities require treatment, the mainstay being intermittent catheterization and anticholinergic medication. The treatment goals for patients with a neurogenic bladder are the preservation of the upper urinary tract, bladder and bowel continence, independence, autonomy, and facilitation of self-esteem. A minority of children will not respond to conservative therapy and will ultimately require surgical intervention. This review will discuss the surgical options for bladder augmentation, bladder neck reconstruction and closure, as well as the methods for the creation of continent catheterizable stomas. The timing, indications, and description for each procedure will be addressed. Finally, the antegrade continence enema procedure will be described for the management of refractory fecal incontinence.


Treatment of the neurogenic bladder in spina bifida.

de Jong TP, Chrzan R, Klijn AJ, Dik P.  Pediatric Renal Center, Department of Pediatric Urology, University Children’s Hospital, UMCU, P.O. Box 85090, 3508 AB, Utrecht, The Netherlands.  Pediatr Nephrol. 2008 Mar 19. [Epub ahead of print]

Renal damage and renal failure are among the most severe complications of spina bifida. Over the past decades, a comprehensive treatment strategy has been applied that results in minimal renal scaring. In addition, the majority of patients can be dry for urine by the time they go to primary school. To obtain such results, it is mandatory to treat detrusor overactivity from birth onward, as upper urinary tract changes predominantly start in the first months of life. This means that new patients with spina bifida should be treated from birth by clean intermittent catheterization and pharmacological suppression of detrusor overactivity. Urinary tract infections, when present, need aggressive treatment, and in many patients, permanent prophylaxis is indicated. Later in life, therapy can be tailored to urodynamic findings. Children with paralyzed pelvic floor and hence urinary incontinence are routinely offered surgery around the age of 5 years to become dry. Rectus abdominis sling suspension of the bladder neck is the first-choice procedure, with good to excellent results in both male and female patients. In children with detrusor hyperactivity, detrusorectomy can be performed as an alternative for ileocystoplasty provided there is adequate bladder capacity. Wheelchair-bound patients can manage their bladder more easily with a continent catheterizable stoma on top of the bladder. This stoma provides them extra privacy and diminishes parental burden. Bowel management is done by retrograde or antegrade enema therapy. Concerning sexuality, special attention is needed to address expectations of adolescent patients. Sensibility of the glans penis can be restored by surgery in the majority of patients.


Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale.

Metcalfe PD, Luerssen TG, King SJ, Kaefer M, Meldrum KK, Cain MP, Rink RC, Casale AJ.  Department of Pediatric Urology, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine, 702 North Barnhill Drive, Indianapolis, IN 46202, USA. J Urol. 2006 Oct;176(4 Pt 2):1826-9; discussion 1830.

PURPOSE: Occult tethered cord syndrome applies to patients with signs and symptoms consistent with a caudal spinal cord malformation despite normal neuroimaging. Although several reports of successful surgical treatment exist, controversy remains with respect to patient selection and efficacy. We present a large series with excellent clinical followup, neuroimaging and urodynamic characterization.

MATERIALS AND METHODS: We present our experience with 36 patients at a single institution with preoperative clinical findings, neuroimaging and urodynamics available. Postoperative outcomes were assessed clinically and with urodynamics. We determined predictive parameters to improve patient selection.

RESULTS: Approximately 0.04% of pediatric urology clinic visits resulted in neurosurgical referral for the potential of an occult tethered cord. They occurred after failure of a mean of 2 years of aggressive medical management. Daytime urinary incontinence was present in 83% of patients and 47% had encopresis. Preoperative urodynamics were markedly abnormal in all patients with mean bladder capacity 55% of expected capacity. Clinical improvement in urinary symptoms was seen in 72% of patients with resolution of incontinence in 42%. Bowel symptoms improved in 88% of cases, including resolution of encopresis in 53% within 3 months of surgery. Urodynamic improvements were demonstrated in 57% of cases. We were unable to determine preoperative factors that were more likely associated with surgical success. CONCLUSIONS: In a highly select population with severe urinary and fecal dysfunction sectioning a normal-appearing filum terminale can result in significant improvement. We were unable to identify factors that may increase the chance of surgical success.