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The Dysfunctional Elimination Syndrome in Children—Is Sacral Neuromodulation Worth the Trouble?
The Dysfunctional Elimination Syndrome in Children—Is Sacral Neuromodulation Worth the Trouble? DURING the last 15 years the dysfunctional elimination syndrome (DES) has become recognized as a condition distinguished by dysfunctional voiding and bowel dysfunction in the absence of a known neurological disorder. The syndrome manifests itself in various ways, and is often associated with urinary tract infections and vesicoureteral reflux. Incontinence, nocturnal enuresis, urgency and frequency are the most common urinary symptoms to prompt an evaluation. Constipation and encopresis are the most typical presenting bowel symptoms. Importantly, DES is a diagnosis of exclusion. The treatment methods for DES are varied. Dietary modifications, adjustments to fluid intake and timed voiding regimens are considered standard, and implemented in all patients. These modifications lead to continence for almost two-thirds of children and completely relieve urinary symptoms in just under half,1 although encopresis can persist in 80% of patients with DES.2 Biofeedback therapy improves daytime incontinence in 80% of children, urinary frequency in 70% to 100% and urinary urgency in 70% to 90%. Constipation and vesicoureteral reflux are improved in 20% to 100% of children.1 Associated improvements in objective findings (eg postvoid residual, urodynamic study) have been modest, and a randomized controlled trial comparing biofeedback and conservative therapy found no statistically significant difference in initial success.3 Antimuscarinic medicines promote continence in approximately 60% of patients4 and improve urodynamic parameters in about 75%.5 Alpha-adrenergic antagonists lead to subjective improvement of symptoms in 85% of children with bladder neck dysfunction.6 After intravesical injection of botulinum toxin A, 75% of children in whom biofeedback and alpha-adrenergic antagonists had failed were dry at the first postoperative visit.7 Select patients with DES can benefit from clean intermittent catheterization,8 and bladder augmentation, bladder neck suspension, urethral slings5 and artificial sphincters are rarely used. DES refractory to common treatments can lead to problems in school and social settings such as bullying, embar0022-5347/12/1884-1076/0 THE JOURNAL OF UROLOGY® © 2012 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION
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rassment and self-exclusion from age specific social customs (eg sleepovers) as patients try to cope with symptoms considered socially unacceptable. Sacral neuromodulation via removable implantable device did not emerge as an alternate therapy for children with refractory DES until after 1997, when the Food and Drug Administration approved it for urge incontinence in adults alone. Children with neurogenic bladder dysfunction were treated with sacral neuromodulation in 2004,9 and we subsequently began using the InterStim® implantable device to treat children who had refractory DES. In 2006 we published the first outcomes study of sacral neuromodulation for refractory DES in children.10 We have since accumulated a series of more than 100 patients (median followup 18.2 months). In this issue of The Journal Groen et al (page 1313) report their 15-year experience with sacral neuromodulation in 18 children with refractory voiding symptoms, 2 of whom had DES.11 Patients with incontinence episodes as well as those performing self-catheterization had statistically significant decreases in the number of times they were incontinent or in need of catheterization. In terms of the 2 patients with DES, both had objective improvements in postvoid residual bladder volumes with a decrease in urinary tract infections in 1 patient, and a decrease in suprapubic pain and dysuria in the other. Of the combined patients with DES, overactive bladder and Fowler’s syndrome 6 of 13 (46%) had a durable full response (ie 100% resolution of symptoms or more than 90% subjective improvement). Late failure (ie less than 50% improvement of symptoms) followed an initial full response in 2 of 13 (15%) patients, and it followed an initial partial response in 1 of 13 (8%). Early failure occurred in 2 of 13 (15%) patients who did not proceed to permanent implantation, and the remaining 2 (15%) had durable partial responses. Explantation of the device occurred in 1 of 13 (8%) children in this group as a result of infection. Among the entire cohort studied by Groen et al infection and dislocation/loss of effect led to a sum total of 8 complications requiring surgical intervention (8 of 18, 44%). Battery depletion http://dx.doi.org/10.1016/j.juro.2012.07.059 Vol. 188, 1076-1077, October 2012 Printed in U.S.A.
SACRAL NEUROMODULATION FOR DYSFUNCTIONAL ELIMINATION SYNDROME
led to intervention for an additional 2 patients (11%), which the authors viewed as an indication of a successful intervention. In our experience we have not had surgical interventions prompted by battery depletion, but infection has caused us to explant the device in 5 of 118 (4%) patients in our DES cohort. In total, 28 of 118 (24%) patients have required reoperation due to complications. The most common complication requiring surgery was lead breakage (17 of 118, 14%), which has seemed to be associated with thinner children after minor falls. Explantation for ineffectiveness (ie late failure) occurred in 3% (4 of 118) of children at an average of 29.7 months after placement. Complete, stable resolution of symptoms enabled us to remove devices entirely from 11% (13 of 118) of our cohort at an average of 40.9 months after placement. The psychosocial benefit of curing incontinence with sacral neuromodulation in a patient with DES is tremendous. Having had the constant fear of having an incontinent episode dissipated, patients have commented, “I always felt bad because I felt like I was a burden.” “I had a hard time living my life.” “I was always embarrassed.” “Accidents at night controlled my life.” In followup visits after successful procedures, parents often remark that their child is interacting more with others and performing better in school. Patients explain how success has been life changing. “I’m looking forward to many opportunities . . . traveling, sleepovers, college, marriage and simply enjoying normal current/future experiences.” “Now I can stop worrying.” “InterStim will allow me to live a normal life and grow up to be a successful soldier,
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police officer and father.” “InterStim made my new life possible.” In our practice we intend to begin offering first stage placement of the temporary external stimulator device more liberally. Our modified surgical technique has eliminated the first stage incision and the second stage use of fluoroscopy, thereby improving cosmesis and minimizing radiation. Thus, a wider application of the first stage procedure would enable more children to benefit from temporary treatment of DES with fewer drawbacks while theories about the benefits of a more prompt diagnosis and earlier treatment of DES in childhood can be investigated. Because the procedure is not technically challenging, takes little time to perform and can be learned quickly, the use of sacral neuromodulation will likely broaden. There are drawbacks to implantation, but our overall complication rate of 29% (34 of 118, median followup 29.7 months) with a 4% infection rate is balanced by the more than 90% of patients who have symptom improvement, not to mention the immense impact on the lives of the 15% (12 of 80) of patients whose incontinence is cured completely. The dramatic effect of success on the life of a child with refractory DES is a compelling incentive and we maintain that sacral neuromodulation is indeed worth it. Moira E. Dwyer Mayo Clinic Rochester, Minnesota and
Yuri E. Reinberg Pediatric Surgical Associates Minneapolis, Minnesota
REFERENCES 1. Desantis DJ, Leonard MP, Preston MA et al: Effectiveness of biofeedback for dysfunctional elimination syndrome in pediatrics: a systematic review. J Pediatr Urol 2011; 7: 342. 2. Feng WC and Churchill BM: Dysfunctional elimination syndrome in children without obvious spinal cord diseases. Pediatr Clin North Am 2001; 48: 1489. 3. Abdol-Mohammad K, Sharifi-Rad L, Ghahestani SM et al: Animated biofeedback: an ideal treatment for children with dysfunctional elimination syndrome. J Urol 2011; 186: 2379. 4. Schröder A and Thüroff JW: New strategies for medical management of overactive bladder in children. Curr Opin Urol 2010; 20: 313.
5. Lopez Pereira P, Miguelez C, Caffarati J et al: Trospium chloride for the treatment of detrusor instability in children. J Urol 2003; 170: 1978.
termittent catheterization in neurologically and anatomically normal children. BJU Int 2002; 89: 923.
6. Van Batavia JP, Combs AJ, Horowitz M et al: Primary bladder neck dysfunction in children and adolescents Ill: results of long-term alpha blocker therapy. J Urol 2010; 183: 724.
9. Guys JM, Haddad M, Planche D et al: Sacral neuromodulation for neurogenic bladder dysfunction in children. J Urol 2004; 172: 1673.
7. Franco I, Landau-Dyer L, Isom-Batz G et al: The use of botulinum toxin A injection for the management of external sphincter dyssynergia in neurologically normal children. J Urol 2007; 178: 1775. 8. Pohl HG, Bauer SB, Borer JG et al: The outcome of voiding dysfunction managed with clean in-
10. Humphreys MR, Vandersteen DR, Slezak JM et al: Preliminary results of sacral neuromodulation in 23 children. J Urol 2006; 176: 2227. 11. Groen LA, Hoebeke P, Loret N et al: Sacral neuromodulation with an implantable pulse generator in children with lower urinary tract symptoms: 15-year experience. J Urol 2012; 188: 1313.
1076
www.jurology.com
AND
RESEARCH, INC.
rassment and self-exclusion from age specific social customs (eg sleepovers) as patients try to cope with symptoms considered socially unacceptable. Sacral neuromodulation via removable implantable device did not emerge as an alternate therapy for children with refractory DES until after 1997, when the Food and Drug Administration approved it for urge incontinence in adults alone. Children with neurogenic bladder dysfunction were treated with sacral neuromodulation in 2004,9 and we subsequently began using the InterStim® implantable device to treat children who had refractory DES. In 2006 we published the first outcomes study of sacral neuromodulation for refractory DES in children.10 We have since accumulated a series of more than 100 patients (median followup 18.2 months). In this issue of The Journal Groen et al (page 1313) report their 15-year experience with sacral neuromodulation in 18 children with refractory voiding symptoms, 2 of whom had DES.11 Patients with incontinence episodes as well as those performing self-catheterization had statistically significant decreases in the number of times they were incontinent or in need of catheterization. In terms of the 2 patients with DES, both had objective improvements in postvoid residual bladder volumes with a decrease in urinary tract infections in 1 patient, and a decrease in suprapubic pain and dysuria in the other. Of the combined patients with DES, overactive bladder and Fowler’s syndrome 6 of 13 (46%) had a durable full response (ie 100% resolution of symptoms or more than 90% subjective improvement). Late failure (ie less than 50% improvement of symptoms) followed an initial full response in 2 of 13 (15%) patients, and it followed an initial partial response in 1 of 13 (8%). Early failure occurred in 2 of 13 (15%) patients who did not proceed to permanent implantation, and the remaining 2 (15%) had durable partial responses. Explantation of the device occurred in 1 of 13 (8%) children in this group as a result of infection. Among the entire cohort studied by Groen et al infection and dislocation/loss of effect led to a sum total of 8 complications requiring surgical intervention (8 of 18, 44%). Battery depletion http://dx.doi.org/10.1016/j.juro.2012.07.059 Vol. 188, 1076-1077, October 2012 Printed in U.S.A.
SACRAL NEUROMODULATION FOR DYSFUNCTIONAL ELIMINATION SYNDROME
led to intervention for an additional 2 patients (11%), which the authors viewed as an indication of a successful intervention. In our experience we have not had surgical interventions prompted by battery depletion, but infection has caused us to explant the device in 5 of 118 (4%) patients in our DES cohort. In total, 28 of 118 (24%) patients have required reoperation due to complications. The most common complication requiring surgery was lead breakage (17 of 118, 14%), which has seemed to be associated with thinner children after minor falls. Explantation for ineffectiveness (ie late failure) occurred in 3% (4 of 118) of children at an average of 29.7 months after placement. Complete, stable resolution of symptoms enabled us to remove devices entirely from 11% (13 of 118) of our cohort at an average of 40.9 months after placement. The psychosocial benefit of curing incontinence with sacral neuromodulation in a patient with DES is tremendous. Having had the constant fear of having an incontinent episode dissipated, patients have commented, “I always felt bad because I felt like I was a burden.” “I had a hard time living my life.” “I was always embarrassed.” “Accidents at night controlled my life.” In followup visits after successful procedures, parents often remark that their child is interacting more with others and performing better in school. Patients explain how success has been life changing. “I’m looking forward to many opportunities . . . traveling, sleepovers, college, marriage and simply enjoying normal current/future experiences.” “Now I can stop worrying.” “InterStim will allow me to live a normal life and grow up to be a successful soldier,
1077
police officer and father.” “InterStim made my new life possible.” In our practice we intend to begin offering first stage placement of the temporary external stimulator device more liberally. Our modified surgical technique has eliminated the first stage incision and the second stage use of fluoroscopy, thereby improving cosmesis and minimizing radiation. Thus, a wider application of the first stage procedure would enable more children to benefit from temporary treatment of DES with fewer drawbacks while theories about the benefits of a more prompt diagnosis and earlier treatment of DES in childhood can be investigated. Because the procedure is not technically challenging, takes little time to perform and can be learned quickly, the use of sacral neuromodulation will likely broaden. There are drawbacks to implantation, but our overall complication rate of 29% (34 of 118, median followup 29.7 months) with a 4% infection rate is balanced by the more than 90% of patients who have symptom improvement, not to mention the immense impact on the lives of the 15% (12 of 80) of patients whose incontinence is cured completely. The dramatic effect of success on the life of a child with refractory DES is a compelling incentive and we maintain that sacral neuromodulation is indeed worth it. Moira E. Dwyer Mayo Clinic Rochester, Minnesota and
Yuri E. Reinberg Pediatric Surgical Associates Minneapolis, Minnesota
REFERENCES 1. Desantis DJ, Leonard MP, Preston MA et al: Effectiveness of biofeedback for dysfunctional elimination syndrome in pediatrics: a systematic review. J Pediatr Urol 2011; 7: 342. 2. Feng WC and Churchill BM: Dysfunctional elimination syndrome in children without obvious spinal cord diseases. Pediatr Clin North Am 2001; 48: 1489. 3. Abdol-Mohammad K, Sharifi-Rad L, Ghahestani SM et al: Animated biofeedback: an ideal treatment for children with dysfunctional elimination syndrome. J Urol 2011; 186: 2379. 4. Schröder A and Thüroff JW: New strategies for medical management of overactive bladder in children. Curr Opin Urol 2010; 20: 313.
5. Lopez Pereira P, Miguelez C, Caffarati J et al: Trospium chloride for the treatment of detrusor instability in children. J Urol 2003; 170: 1978.
termittent catheterization in neurologically and anatomically normal children. BJU Int 2002; 89: 923.
6. Van Batavia JP, Combs AJ, Horowitz M et al: Primary bladder neck dysfunction in children and adolescents Ill: results of long-term alpha blocker therapy. J Urol 2010; 183: 724.
9. Guys JM, Haddad M, Planche D et al: Sacral neuromodulation for neurogenic bladder dysfunction in children. J Urol 2004; 172: 1673.
7. Franco I, Landau-Dyer L, Isom-Batz G et al: The use of botulinum toxin A injection for the management of external sphincter dyssynergia in neurologically normal children. J Urol 2007; 178: 1775. 8. Pohl HG, Bauer SB, Borer JG et al: The outcome of voiding dysfunction managed with clean in-
10. Humphreys MR, Vandersteen DR, Slezak JM et al: Preliminary results of sacral neuromodulation in 23 children. J Urol 2006; 176: 2227. 11. Groen LA, Hoebeke P, Loret N et al: Sacral neuromodulation with an implantable pulse generator in children with lower urinary tract symptoms: 15-year experience. J Urol 2012; 188: 1313.