CHAPTER 1 INTRODUCTION

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Ethan Simmons
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3 Introduction Spina bifida is a congenital defect of the spine in 1-3 out of 1000 live born children 1 and still is one of the most common serious congenital malformations. The spinal cord lesion in such patients is localised at variable levels. Many of the lesions are asymmetrical. At any level the motoric and sensitive lesions may occur and depending on the level of the lesion a variety of symptoms can be found. Innervation of the bladder and pelvic floor is complex. Parasymptomatic and symptomatic autonomic nerves innervate the bladder neck and the detrusor muscle of the bladder. The somatic pudendal nerve innervates the pelvic floor. Depending on the level of the lesion the bladder can be overactive or inactive, and pelvic floor and bladder neck can be overactive or inactive. According to odds, in a cross table this means that in 25% of the patients the bladder can be overactive in combination with an inactive sphincter complex and that in 25% both bladder and sphincter can be overactive. The other 50% of the patients will be incontinent as a result of inactivity of the sphincter, 25% of them even with overactivity of the bladder. detrusor inactive detrusor overactive sphincter inactive 25% 25% sphincter overactive 25% = at risk 25% = at high risk Overactivity of the bladder can result in damage of the kidneys due to high pressure in the urogenital system. Especially in combination with overactivity of the sphincter complex a high risk situation develops. Vesico-ureteral reflux, urinary infections and pyelonephritis form a continuous threat to the kidneys. In many institutions it is still accepted as a fact of life that 25-40% of these patients develop pyelonephritic lesions or even end stage renal disease. 2,3 In a study of 132 spina bifida patients from in our institution 4 urodynamic investigation showed that the distribution of risk factors was 9

4 CHAPTER 1 INTRODUCTION as follows: detrusor inactive detrusor overactive sphincter inactive 35 (26%) 42 (32%) sphincter overactive 13 (10%) 42 (32%) In reality, the risk factors were even worse than was expected. In 42% (=10%+32%) the sphincter complex was overactive but in 32% also the detrusor was overactive, resulting in a high risk factor for upper urinary tracts. For this reason high bladder pressure needed to be converted by means of drugs or by means of surgery in order to create a compliant bladder reservoir. Emptying of the bladder was done by cathetrization. 5,6,7,8,9,10,11,12,13 This rationale is further discussed in chapter 2. Therefore from 1988 on, all patients with spina bifida, that were admitted to our institution, were treated with intermittent catheterization and antimuscarinic agents shortly after birth and surgical closure of the spinal defect. In some patients suplementary surgical therapy was needed for treatment of vesico-ureteral reflux, incontinence, persisting high bladder pressures or for the creation of a continent vesicostomy. 14,15,16,17,18,19 Aims of this thesis 1. Is hyperactivity of the detrusor muscle a result of compensation against an overactive sphinctermechanism or of pure neuropathic origin? Urodynamic studies were done in 15 spina bifida patients before and after stopping antimuscarinic agents (Chapter 3). 2. Is augmentation plasty of the bladder also feasible by surgical removal of a large part of detrusor muscle? Detrusorectomy was done in 35 patients, 19 patients because of 10

5 poor compliance and in 16 patients in an attempt to stop antimuscarinic agents (Chapter 4). 3. Is surgical treatment for continence a good option? In 24 girls and in 14 boys with pelvic floor paralysis a sling procedure was done (Chapter 5 and 6). 4. How were the results of the vesicostomies that were created? In a group of 36 patients the results of vesicostomies were studied (Chapter 7). 5. Is kidney function improved by lowering bladder pressures? From January 1988 untill June 2001 a group of 144 spinal dysraphism patients was treated by lowering intravesical pressure and regular evacuation of the bladder by means of clean intermittent catheterisation. Development of kidney function and continence was studied (Chapter 8). 11

6 CHAPTER 1 INTRODUCTION References 1. Pal-de Bruin KM, Buitendijk SE, Hirasing RA, den Ouden AL. [Prevalence of neural tube defects in births before and after promotion of periconceptional folic acid supplementation]. Ned Tijdschr Geneeskd 2000: 144(36): Muller T, Arbeiter K, Aufricht C. Renal function in myelomeningocele: risk factors, chronic renal failure, renal replacement therapy and transplantation. Curr.Opin.Urol Nov; 12 (6): Capitanucci ML, Iacobelli BD, Silveri M, Mosiello G, De Gennaro M. Long-term urological follow-up of occult spinal dysraphism in children. Eur J Pediatr Surg 1996:6 Suppl 1: van Gool JD (1986) Spina bifida and neurogenic bladder dysfunction--a urodynamic study. Impress, Utrecht 5. Drago JR, Wellner L (1977) The role of intermittent catheterization in the management of children with myelomeningocele. J Urol 118: Baskin LS, Howard PS, Duckett JW, Snyder HM, Macarak EJ (1993) Cellular bladder model. I. Smooth muscle characterization. J Urol 149: Brock WA, So EP (1981) Intermittent catheterization in the management of neurogenic vesical dysfunction in children. J Urol 125: Edelstein RA, Bauer SB (1995) The long-term urological response of neonates with myelodysplasia treated proactively with intermittent catheterization and anticholinergic therapy. J Urol 154: Mulcahy JJ, James HF (1977) Oxybutynin chloride combined with intermittent clean catheterization in the treatment of myelomeningocele patients. J Urol 118: Kass EJ, Koff SA (1983) Bladder augmentation in the pediatric neuropathic bladder. J Urol 129: Kass EJ, Koff SA, Diokno AC (1981) Fate of vesico-ureteral reflux in children with neuropathic bladder managed by intermittent catheterization. J Urol 125: Kass EJ, McHugh T, Diokno AC (1979) Intermittent catheterization in children less than 6 years old. J Urol 121: Whitycombe J, Whitaker RH, Hunt G (1978) Intermittent 12

catheterization in the management of children with neurogenic bladder. Lancet ii:981 14. Duckett JW, Snyder HM (1986) Continent urinary diversion-- variations on the Mitrofanoff principle.

7 catheterization in the management of children with neurogenic bladder. Lancet ii: Duckett JW, Snyder HM (1986) Continent urinary diversion-- variations on the Mitrofanoff principle. J Urol 136: Dewan, P. A. and Stefanek, W.: Autoaugmentation gastrocystoplasty: early clinical results. Br J Urol, 1994, 74: Mitrofanoff, P.: Trans-appendicular continent cystostomy in the management of the neurogenic bladder. Chir Pediatr, 1980, 21: Freedman, E. R., Singh, G., Donnell, S. C., Rickwood, A. M. and Thomas, D. G.: Combined bladder neck suspension and augmentation cystoplasty for neuropathic incontinence in female patients. Br J Urol, 73: 621, Elder JS. Periurethral and puboprostatic sling repair for incontinence in patients with myelodysplasia. J Urol 1990; 144: Pérez LM, Smith EA, Broecker BHet al.outcome of sling cystourethropexy in the pediatric population: a critical review. J Urol 1996; 156:

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Early Start to Therapy Preserves Kidney Function in Spina Bifida Patients

european urology 49 (2006) 908 913 available at www.sciencedirect.com journal homepage: www.europeanurology.com Pediatric Urology Early Start to Therapy Preserves Kidney Function in Spina Bifida Patients