Introduction 1 14 15 16 Initial approach for the management of spina bifida To preserve renal function, low bladder pressure must be maintained from birth. The status of pelvic floor activity must be assessed shortly after birth to ascertain whether a child is at risk for high detrusor pressures. At first presentation after birth, inspection of the anal sphincter, closed or open, gives an impression of the status of the pelvic floor: overactive or paralyzed. Approximately 50% of children with spina bifida aperta and 25% of children with occult spinal dysraphism have a detrusor/sphincter dyssynergia that carries a serious risk of early upper urinary tract damage by high bladder pressures and urinary tract infections (UTIs). It is important to realize that after closure of the back, pelvic floor behavior can change from paralyzed to overactive in the first 2–3 months of life. That is a reason to delay the first urodynamic study (UDS) until 2 months after birth. Clean intermittent catheterization 17 20 Antimuscarinic therapy Oxybutynin is best started together with CIC immediately after closure of the back. Other antimuscarinic agents have not yet been registered for pediatric use. This subject is more elaborately described in the section “Overactive detrusor”. Infection prophylaxis In principle, all patients are put on low-dose chemoprophylaxis, mostly trimethoprim 2 mg/kg per day. In case of breakthrough infections, nitrofurantoin or ciprofloxacin can be used. Symptomatic infections are treated intravenously with amoxicillin/clavulanic acid and gentamicin until the result of bacterial culture is known. Many centres stop prophylaxis after the age of 1 year, and approximately 50% of patients seem to do well without prophylaxis. Scientific proof is lacking on this subject. To obtain such proof, we are doing a multicenter randomized study with half of the patients on prophylaxis and half without. Specific needs Overactive pelvic floor 2 21 Paralyzed pelvic floor Patients with a paralyzed pelvic floor are incontinent for urine. Their upper urinary tracts are safe as long as the paralytic pelvic floor is left untreated. They need bladder-neck surgery to become dry. If bladder capacity and compliance are insufficient, surgery can be combined with autoaugmentation of the bladder (detrusorectomy) or (rarely) with clam ileocystoplasty or colocystoplasty. Overactive detrusor muscle If UDS reveals detrusor overactivity, patients are treated with antimuscarinics to increase bladder capacity, even if detrusor pressures are safe. Life-long suppression of detrusor overactivity is required in patients with an overactive neuropathic bladder. In some patients, overactivity can be treated surgically by detrusorectomy or ileocystoplasty. In spina bifida, rhizotomies (interruption of spinal roots) are not yet being used routinely to cure overactivity, but they seem to be a promising alternative. Ideally, we would like to start a protocol with neonatal rhizotomies during the first back closure, but so far, practical and ethical considerations have prevented us from doing this. Antimuscarinic therapy is the gold standard for pharmaceutical therapy of neuropathic detrusor overactivity. Oxybutynin has proven to be inexpensive and effective and can be taken orally, intravesically, and transdermally. Several new antimuscarinic agents have been introduced, which may prove valuable in the future. 22 23 Surgical procedures Timing of surgery There is no age-related contraindication for any operation. Thus, indication for surgery is made in mutual agreement with the child’s parents or with the patients themselves after the age of 11 years. The combination of high bladder pressures and vesicoureteral reflux can sometimes force intervention as early as the first few months of life. We combined antireflux surgery, bladder autoaugmentation, and transvaginal sling suspension in a 3-month-old girl with febrile breakthrough infections, with good clinical and urodynamic result after follow-up for more than 10 years. Initially, reserves existed on doing sling suspensions before puberty, especially in boys, out of fear of introducing obstruction during puberty based on prostate growth. Over the years, it has been proven that puberty can safely be passed after sling suspension of the bladder neck, both in girls and boys. Parental burden can be an indication for performing a catheterizable stoma. If a child weighs 20 kg, five daily transfers for CIC can be too much for a parent with lower back pain. Finally, the patient’s privacy can be an important argumentation for construction of a stoma for CIC. Parents are increasingly reluctant for any caregiver to help their child perform CIC transurethrally, as this involves exposure of the genitalia to strangers several times a day. Surgery for incontinence Patients with a paralytic pelvic floor need bladder-neck surgery to achieve continence. There are many surgical options. Our standard approach is an abdominoperineal puboprostatic sling procedure in boys and a transvaginal sling procedure in girls. If there is some persistent leaking after a sling procedure, this is cured by injecting a bulking agent in the bladder neck. Optimal results are obtained if the injection needle is passed into the bladder neck by means of a suprapubic puncture with transurethral endoscopic visual control. So far, we have mainly used silicon grains in povidone (Macroplastique®) as a bulking agent. The polymer Deflux® offers a good alternative because it is easier to inject. However, in our experience, Deflux does not work if CIC needs to be carried out through the same channel. A recent survey, yet unpublished, of the results of 76 sling suspensions revealed an 80% success rate. Sling suspension in boys 24 25 24 False routes are a risk after sling suspension. Out of approximately 50 male patients, we had to construct a catheterizable stoma in five: four as a result of a false route, and one because of a huge congenital prostatic cyst. Sling suspension in girls 26 Alternatives 27 Several groups do not opt for urinary continence at an early age and try to maintain safe leak-point pressures by regular dilatation of the female urethra, sometimes also of the male urethra, after making this possible with a perineal urethral stoma. In some patients, they construct a cutaneous vesicostomy for the first few years and perform an ileocystoplasty when the stoma is closed. Alternatives in dealing with a neurogenic bladder When cerebral function is bad and the patient has no dexterity, diapers can also be chosen. For this choice, a low-pressure bladder is necessary. Good results are obtained by cutaneous vesicostomy. In girls, low pressures can be achieved by making a vesicovaginal fistula by endoscopic cutting into the vagina between the ureteric orifices. Temporary low pressures can be achieved by overdistension of the female urethra. In boys, endoscopic external sphincterotomy in the 12 o’clock position produces low outlet resistance for 2–3 years and needs to be repeated when bladder pressures rise. Catheterizable stomas 28 1 Fig. 1 The appendix can be used to make a stoma to the bladder for intermittent catheterization  In the literature, after construction of a catheterizable stoma, complications are described in as many as 50% of patients. These complications are mostly temporary. Stomal stenosis, mostly at skin level, is a frequently occurring temporary complication that can be avoided with a silicon ace stopper left behind in the skin portion of the stoma for 6 months between catheterizations. Stomal leakage of urine can be a frustrating complication. Endoscopic treatment with bulking agents can be tried, but lengthening of the intravesical tunnel or reimplantation of the stoma will often be needed. Leakage of the stoma occurs more often in midline stomas (umbilicus) than in stomas placed on the right side of the lower abdominal wall. The reason is probably that the lateral stoma passes through the rectus muscle and is thus occluded during increases in abdominal pressure due to rectus contraction. In midline stomas we now also pass the stoma through the median margin of one rectus muscle. Stoma stenosis at the level of entrance into the bladder can sometimes be treated endoscopically with success. In those patients, formal reimplantation of the stoma into the bladder will frequently be needed as well. Autoaugmentation or detrusorectomy of the bladder 2 2 21 Fig. 2 The bladder can be augmented by removing the detrusor muscle. A sling is used to improve bladder outlet resistance  Clam cystoplasty 3 Fig. 3 The bladder can be augmented by using a bowel segment  Bowel management and surgery 29 30 4 Fig. 4 The appendix can be used to make an antegrade colonic enema stoma  14 Surgery to improve sexuality in male patients 31 Conclusion Kidney-function preservation and early urinary dryness are important factors for optimal quality of life for spina bifida patients. Another important factor is patient independence regarding his or her bladder and bowel management. For optimal treatment of this difficult group of patients, a multidisciplinary team is needed consisting of members from all the necessary medical specialties, including rehabilitation specialists, physical therapists, specialized nurses, and social workers. Questions Reported death rate due to renal failure is up to 20% in the first year of life Reported renal failure rate is up to 20% in the first year of life Reported death rate due to renal failure is up to 20% in puberty Reported renal failure rate is up to 20% in puberty CIC is best started immediately after closure of the spinal defect CIC is best started immediately after the age of 8–9 years CIC is best started not earlier than after bladder neck surgery CIC is best started not earlier than after several urinary infections and reported renal scarring All patients need low-dose chemoprophylaxis Fifteen percent of all patients need low-dose chemoprophylaxis Scientific proof of the need for infection prophylaxis is still lacking No patients need low-dose chemoprophylaxis Central side-effects occur less frequently if antimuscarinic therapy is administered orally Antimuscarinic therapy is the gold standard for pharmaceutical therapy Repeated injection therapy of the bladder with only 3 U of botulinum toxin can be an alternative to antimuscarinic therapy Antimuscarinic therapy has positive effects on perspiration and body-temperature regulation Kidney-function preservation Dryness Fecal continence All the above Electronic supplementary material Below is the link to the electronic supplementary material.
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