A systematic review on bowel management and the success rate of the various treatment modalities in spina bifida patients

Velde SV, Biervliet SV, Bruyne RD, Winckel MV. (2013). A systematic review on bowel management and the success rate of the various treatment modalities in spina bifida patients.

Spinal Cord. 2013 Oct 15. doi: 10.1038/sc.2013.123. [Epub ahead of print]

Abstract

Study design:Systematic review.Objectives:To determine the different treatment modalities aimed at achieving fecal continence in spina bifida (SB) patients and their effectiveness.Setting:International literature.Method:Electronic databases were searched ('Pubmed', 'Web of science', 'CINAHL' and 'Cochrane') identifying studies published since the mid-eighties and screened for relevance according to the Centre for Reviews and Dissemination procedure guidelines. A total of 37 studies were selected for inclusion.Results:Studies on toilet sitting, biofeedback, anal plug, retrograde colon enemas (RCE) and antegrade colon enemas were found. Fecal continence was achieved in 67% of SB patients using conservative methods (n=509). In patients using RCE (n=190) an 80% continence rate was reached. Patients following surgical treatment (n=469) reached an 81% continence rate, however, 23% needed redo surgery because of complications. Better fecal continence was associated with an improved quality of life, which was negatively influenced by the amount of time spent on bowel management.Conclusion:Evidence favors an individually tailored stepwise approach with surgery as a final step in case of failure of conservative measures. Continued specialized support throughout life remains important to maintain continence. Cross-over and comparative trials are needed in order to optimize treatment.Spinal Cord advance online publication, 15 October 2013; doi:10.1038/sc.2013.123.

 

PMID:24126852

After the honeymoon comes divorce: long-term use of the antegrade continence enema procedure.

Yardley IE, Pauniaho SL, Baillie CT, Turnock RR, Coldicutt P, Lamont GL, Kenny SE. (2009) After the honeymoon comes divorce: long-term use of the antegrade continence enema procedure. Journal of Pediatric Surgery. 2009 Jun;44(6):1274-6; discussion 1276-7.

Department of Paediatric Surgery, Alder Hey Children's Hospital, Liverpool, UK.

BACKGROUND: Having reported that 18% of children discontinue use of the antegrade continence enema (ACE) after 5 years, we aimed to determine long-term use after an ACE procedure.

METHODS: A postal/telephone questionnaire was conducted. Subjects were consecutive children undergoing an ACE between 1993 and 1999. Outcome measures were use of ACE, reasons for nonuse, complications, and overall satisfaction.

RESULTS: Of 84 eligible subjects, data were available on 61 (73%) aged 22.4 years (15.5-35.1 years). Underlying diagnoses included spina bifida (n = 27), anorectal malformations (n = 18), constipation (n = 11), Hirschsprung's disease (n = 1), sacral agenesis (n = 2), and trauma/tumor (n = 2). Follow-up was 11.02 years (8.34-14.39 years). Thirty-six (59%) of 61 patients were still using their ACE. Reasons for nonuse were lack of effectiveness (n = 14), complications (n = 5), psychologic issues (n = 2), and poor compliance (n = 2). There was no association between diagnosis and nonuse (chi(2), P = .63). In those still using ACE, the overall satisfaction score was 4.1 (1-5). Several individuals reported feeling abandoned on becoming adults and losing the support they had in childhood.

CONCLUSION: There is a late "failure" rate for the ACE procedure. However, satisfaction was high among those still using the ACE. This study further emphasizes the need for robust transitional care arrangements.

PMID: 19524753

Treatment of fecal incontinence with a comprehensive bowel management program.

Bischoff A, Levitt MA, Bauer C, Jackson L, Holder M, Peña A. (2009) Treatment of fecal incontinence with a comprehensive bowel management program. Journal of Pediatric Surgery. 2009 Jun;44(6):1278-83; discussion 1283-4.

Department of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.

PURPOSE: Many articles describe the antegrade continence enemas (ACEs), but few refer to a bowel management program. A successful ACE may not help a patient without such management. Valuable lessons were learned by implementation of bowel management in 495 fecally incontinent patients.

METHODS: We previously reported 201 patients. Thereafter, another 294 patients participated in our program. On the basis of a contrast enema and symptoms, they were divided as follows: (a) 220 constipated patients and (b) 74 patients with tendency toward diarrhea. Colonic stool was monitored with abdominal radiographs, modifying the management according to the patient's response and radiologic findings. For constipated patients, the emphasis was on using large enemas. For patients with tendency toward diarrhea, we used small enemas, a constipating diet, loperamide, and pectin. Diagnoses included anorectal malformation (223), Hirschsprung's (36), spina bifida (12), and miscellaneous (23).

RESULTS: The management was successful in 279 patients (95%)-higher in constipated patients (98%) and less successful in patients with tendency toward diarrhea (84%).

CONCLUSIONS: The key to a successful bowel management program rests in tailoring the type of enema, medication, and diet to the specific type of colon. The best way to determine the effect of an enema is with an abdominal film. The ACE procedures should be recommended only after successful bowel management.


PMID: 19524754

Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida

Nanigian DK, Nguyen T, Tanaka ST, Cambio A, DiGrande A, Kurzrock EA. Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida. Journal of Urology. 2008 Oct;180(4 Suppl):1770-3; discussion 1773.

Department of Urology, University of California-Davis, Davis Children's Hospital, California, USA.

PURPOSE: Fecal incontinence and constipation in children with spina bifida are recognized to impact quality of life. Most disease specific quality of life instruments on fecal incontinence target adults and/or children without neuropathic bowel. We developed an instrument to evaluate bowel function and its impact on quality of life in children with spina bifida and their caregivers. MATERIALS AND METHODS: A 51-item questionnaire termed the FIC QOL (Fecal Incontinence and Constipation Quality of Life) survey was developed from expert opinion, patient interviews, and modification of previously published adult and pediatric studies for nonneuropathic bowel dysfunction. The items are divided into 7 quality of life factor groupings, including bowel program, dietary management, symptoms, travel and socialization, family relationships, caregiver emotional impact and financial impact. The questionnaire was given to caregivers of children with and without spina bifida. Discriminant validity was evaluated by comparing the spina bifida and control groups. Test-retest reliability was evaluated by having 41 patients complete 2 surveys within 4 to 6 weeks. RESULTS: Comparing questionnaires from 92 index patients and 52 controls showed a statistically significant difference for all 7 quality of life factor groupings. The FIC QOL instrument objectively demonstrated the negative impact of fecal incontinence and constipation on quality of life in these families. Comparing 82 questionnaires at 2 time points demonstrated the reliability of all FIC QOL questions. CONCLUSIONS: The FIC QOL instrument provides a valid and reliable measure of the effect of fecal incontinence and constipation on the quality of life of caregivers and their children with spina bifida.

PMID: 18721959

The Experience of Finding an Effective Bowel Management Program for Children With Spina

Sawin KJ, Thompson NM, The Experience of Finding an Effective Bowel Management Program for Children With Spina Bifida: The Parent's Perspective Journal of Pediatric Nursing, In Press

Many families who have a child with spina bifida identify establishing an effective bowel program as one of the major challenges they face; yet, we know little about a family's experience with this process. The purpose of this qualitative study was
to describe the parent's experience. Seven parents were interviewed by telephone. Content analysis yielded four themes: (a) long complicated journey, (b) the impact of the journey on the child, (c) the family struggle, and (d) the promise of the future. The major contribution of this study is a compelling description of one of the most difficult areas for these children and their families. A better understanding of this issue enhances the health care providers' ability to deliver optimal health care.


Key words: Bowel management; Spina bifida; Children; Family struggle

Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida

Nanigian DK, Nguyen T, Tanaka ST, Cambio A, DiGrande A, Kurzrock EA. Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida. Journal of Urology. 2008 Oct;180(4 Suppl):1770-3; discussion 1773.

Purpose: Fecal incontinence and constipation in children with spina bifida are recognized to impact quality of life. Most
disease specific quality of life instruments on fecal incontinence target adults and/or children without neuropathic bowel. We developed an instrument to evaluate bowel function and its impact on quality of life in children with spina bifida and their caregivers.

Materials and Methods: A 51-item questionnaire termed the FIC QOL (Fecal Incontinence and Constipation Quality of Life) survey was developed from expert opinion, patient interviews, and modification of previously published adult and pediatric studies for nonneuropathic bowel dysfunction. The items are divided into 7 quality of life factor groupings, including bowel program, dietary management, symptoms, travel and socialization, family relationships, caregiver emotional impact and financial impact. The questionnaire was given to caregivers of children with and without spina bifida. Discriminant validity was evaluated by comparing the spina bifida and control groups. Test-retest reliability was evaluated by having 41 patients complete 2 surveys within 4 to 6 weeks.

Results: Comparing questionnaires from 92 index patients and 52 controls showed a statistically significant difference for all
7 quality of life factor groupings. The FIC QOL instrument objectively demonstrated the negative impact of fecal incontinence and constipation on quality of life in these families. Comparing 82 questionnaires at 2 time points demonstrated the
reliability of all FIC QOL questions.

Conclusions: The FIC QOL instrument provides a valid and reliable measure of the effect of fecal incontinence and constipation on the quality of life of caregivers and their children with spina bifida.

Key Words: abnormalities, spinal dysraphism, quality of life, questionnaires, fecal incontinence

PMID: 18721959

Achieving Fecal Continence in Patients With Spina Bifida: A Descriptive Cohort Study

Velde SV, Biervliet SV, Renterghem KV, Laecke EV, Hoebeke P, Winckel MV. Achieving Fecal Continence in Patients With Spina Bifida: A Descriptive Cohort Study. Journal of Urology. 2007 Oct 16; [Epub ahead of print]

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 gastoenterologist, 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.

PMID: 17945290

Factors that influence outcomes of the Mitrofanoff and Malone antegrade continence enema reconstructive procedures in children

Clark T, Pope JC 4th, Adams C, Wells N, Brock JW 3rd. Factors that influence outcomes of the Mitrofanoff and Malone antegrade continence enema reconstructive procedures in children. Journal of Urology. 2002 Oct;168(4 Pt 1):1537-40; discussion 1540.

PURPOSE: Surgical techniques that provide adequate urinary and fecal continence in children with neurogenic bladder and bowel dysfunction are becoming increasingly used. We reviewed our experience and discuss factors that influence outcome.

MATERIALS AND METHODS: Between 1994 and 2000, 65 stomal procedures were performed in 47 patients. For the urinary continent catheterizable channel we used appendix in 60% of cases, a continent bladder tube in 20%, a Yang-Monti tube in 16% and ureter in 4%. For the antegrade continence enema continent catheterizable channel we used appendix in 85% of cases, a Yang-Monti tube in 5% and a cecal tube in 10%. In the 19 patients who underwent simultaneous Mitrofanoff and antegrade continence enema procedures the urinary continent catheterizable channel was appendix in 21%, a Yang-Monti tube in 32% and continent vesicostomy in 47%. Patients were divided into 2 groups based on compliance status. In addition, percentile body weight for age was evaluated.

RESULTS: Stomal continence was achieved in 63 of the 65 cases (97%). Of the patients who underwent the antegrade continence enema procedure 95% achieved continence via the rectum. Except for ureter stenosis rates according to continent catheterizable channel type did not differ greatly, namely 19% for appendix, 11% for the Yang-Monti tube, 22% for the bladder tube, 50% for ureter and 0% for the cecal tube. Infectious complications developed in 16 patients and 4 had stones. The rates of infection (p = 0.004), stomal stenosis (p = 0.001) and revision (p = 0.004) were statistically lower in the compliant group and the stone formation rate showed a trend favoring the compliant group (p = 0.11). No significant difference was noted for incontinence. Percentile weight predicted a higher rate of stomal stenosis with the highest rate of stomal stenosis overall in the greater than 100th percentile group.

CONCLUSIONS: The Mitrofanoff and antegrade continence enema procedures are reliable and effective. Proper patient selection and surgical technique with a tension-free anastomosis are essential. The choice of tissue for constructing the continent catheterizable channel is not as important as patient compliance, age and possibly body habitus. This report reinforces the importance of careful screening, and rigorous preoperative and postoperative teaching to achieve overall patient success.

PMID: 12352454

Appendiceal versus ileal segment for antegrade continence enema.

Tackett LD, Minevich E, Benedict JF, Wacksman J, Sheldon CA. Appendiceal versus ileal segment for antegrade continence enema. Journal of Urology. 2002 Feb;167(2 Pt 1):683-6.

PURPOSE: To assess outcomes after the antegrade continence enema procedure, we present our results with an ileal segment or the appendix in children with severe bowel dysfunction.

MATERIALS AND METHODS: A retrospective review of 45 children who had undergone the creation of a continent cecostomy for severe chronic constipation and fecal incontinence was performed.

RESULTS: The appendix was used to create the continent cecostomy in 28 patients (group 1) and ileum 17 (group 2). Of 16 patients who underwent simultaneous construction of appendiceal Mitrofanoff neourethra, including continent catheterizable stoma, the appendix was split and used for the cecostomy and neourethra in 11. Overall, acceptable continence was achieved in 39 (87%) patients and total continence 31 (69%). No significant difference was noted in the rate of continence between groups 1 and 2. Nonstomal postoperative complications occurred in 5 patients in group 1 and 3 group 2. Complications that required reoperation related to the continent cecostomy occurred in 10 patients, including stomal stenosis in 8, with 6 group 1 and 2 group 2 (p >0.05), and stricture in 2, with 1 group 1 and 1 group 2. There were 2 patients who had previously undergone colostomy for intractable constipation who were undiverted at the time of the creation of continent cecostomy. Both were continent postoperatively. There were 3 patients, including those 2 who presented with chronic severe constipation of unclear etiology, who underwent colostomy for unrecoverable colonic dys-motility, of whom 1 subsequently required total colectomy.

CONCLUSIONS: The creation of a continent cecostomy for antegrade continence enema is a successful management option in children with debilitating fecal incontinence, and may enable undiversion of an existing colostomy. The appendix and ileal segment are viable options for the procedure, with no significant difference noted in continence or complication rates.

PMID: 11792954

Cecostomy in children with defecation disorders

Mousa HM, van den Berg MM, Caniano DA, Hogan M, Di Lorenzo C, Hayes J. Cecostomy in children with defecation disorders. Digestive Diseases and Sciences. 2006 Jan;51(1):154-60.

Administration of antegrade enemas through a cecostomy is a therapeutic option for children with severe defecation disorders. The purpose of this study is to report our 4-year experience with the cecostomy procedure in 31 children with functional constipation (n = 9), Hirschsprung's disease (n = 2), imperforate anus (n = 5), spinal abnormalities (n = 8), and imperforate anus in combination with tethered spinal cord (n = 7). Data regarding complications, antegrade enemas used, symptoms, and quality of life were retrospectively obtained. Placement of cecostomy tubes was successful in 30 of 31 patients. Soiling episodes decreased significantly in children with functional constipation (P = 0.01), imperforate anus (P < 0.01), and spinal abnormalities (P = 0.04). Quality of life improved in patients with functional constipation and imperforate anus. No difference in complications was found between percutaneous and surgical placement. Use of antegrade enemas via cecostomy improved symptoms and quality of life in children with a variety of defecation disorders.

PMID: 16416229

Percutaneous cecostomy for management of fecal incontinence in pediatric patients.

Sierre S, Lipsich J, Questa H, Bailez M, Solana J. Percutaneous cecostomy for management of fecal incontinence in pediatric patients. Journal of Vascular and Interventional Radiology. 2007 Aug;18(8):982-5.

Purpose To report the authors' experience with percutaneous cecostomy and demonstrate its effectiveness in the management of pediatric patients with fecal incontinence. Materials and Methods Between March 2002 and November 2006, 21 percutaneous cecostomy procedures were performed in 20 patients in whom classical therapeutic approaches for the management of fecal incontinence had failed. Eighteen patients had anorectal malformations, one had myelomeningocele, and one had chronic constipation. All procedures were performed under general anesthesia and fluoroscopic guidance. In all cases, an 8.5-F Dawson-Mueller catheter was placed in the cecum and exchanged after 45 days with a cecostomy button (ie, Trapdoor catheter). Data regarding complications, effectiveness of treatment, satisfaction, and quality of life were obtained by interviewing the patients' parents at follow-up consultation. Results Nineteen of 20 procedures were technically successful. There were no major complications. All patients' symptoms of incontinence improved. Ninety percent of patients in our series (n = 18) reported satisfaction with the procedure, mainly related to their independence and quality of life. Conclusions Percutaneous cecostomy is a safe and effective procedure for the management of pediatric patients with fecal incontinence. Percutaneous cecostomy allows antegrade and more physiologic colon irrigation, avoiding the need for multiple retrograde enemas, achieving more patient independence, and improving quality of life.

PMID: 17675615