Management of neurogenic fecal incontinence in myelodysplastic children by a modified continent appendiceal stoma and antegrade colonic enema

OBJECTIVES: Antegrade colonic enemas for neurogenic fecal incontinence via reverse reimplanted appendices (Mitrofanoff principle) have been primarily reported by Malone and coworkers in 1990. We used a modification of the described surgical technique and treated the first 10 patients with neurogenic fecal incontinence due to spina bifida. The surgical procedure and the results are reported. METHODS: Since November 1991, we have used a surgical procedure similar to the appendiceal continence mechanism in urinary diversion to establish a continent colonic cutaneous stoma for antegrade enemas in 10 myelodysplastic patients (4 females, 6 males; median age 13.2 years [range 6 to 26]) with severe neurogenic fecal incontinence. The average follow-up is now 26.4 months (range 12.5 to 50). All patients had neurogenic bladder dysfunction successfully managed by clean intermittent catheterization, anticholinergic drugs, or artificial sphincter implantation. The surgical technique for fecal incontinence included the partial orthotopic submucosal imbedding of the appendix into a cecal tenia and the fixation of the ileocecal region at the inner side of the abdominal wall after creation of an appendicocutaneous catheterizable stoma. RESULTS: All patients reached fecal continence for at least 38 hours (median 45.3) by using antegrade colonic enemas with 1.5% saline solution (n = 9) or GoLYTELY solution (n = 1), 0.5 to 1.5 L every 2 to 3 days. All other therapies (diet, oral medication, rectal purgative, or enema) to reach fecal continence had previously failed. There were only two complications seen at the follow-up. One boy with an artificial urinary sphincter presented with infection of the sphincter system, which led to explantation. Another boy presented 15 months after creation of the colonic appendiceal stoma with saline intoxication possibly due to a homemade saline solution. CONCLUSIONS: We conclude that the antegrade colonic enema via an orthotopic continent appendiceal stoma is a safe and highly effective treatment modality for fecal incontinence in patients with neurogenic bowel dysfunction if nonsurgical management has failed.     

Malone antegrade continence enema for adults with neurogenic bowel disease

PURPOSE: We describe the outcomes of adults with neurogenic bowel disease who underwent a Malone antegrade continence enema procedure with or without concomitant urinary diversion. MATERIALS AND METHODS: Consecutive adult patients with neurogenic bowel disease who underwent an antegrade continence enema procedure (continent catheterizable appendicocecostomy for fecal impaction) were retrospectively reviewed. RESULTS: Of the 7 patients who underwent an antegrade continence enema synchronous urinary procedure (ileal conduit, augmentation ileocystoplasty with continent catheterizable abdominal stoma or augmentation ileocystoplasty) was also performed in 6. Mean patient age was 32 years and mean followup was 11 months. Of the 7 patients 6 who self-administered antegrade continence enemas regularly were continent of stool per rectum and appendicocecostomy, using the appendicocecostomy as the portal for antegrade enemas. All 6 compliant patients reported decreased toileting time and improved quality of life. Preoperative autonomic dysreflexia resolved postoperatively in 3 patients. All urinary tracts were stable. In 4 patients 5 complications occurred, including antegrade continence enema stomal stenosis requiring appendicocutaneous revision (1), antegrade continence enema stomal stenosis requiring dilation (1), superficial wound infection (1), small bowel obstruction requiring lysis of adhesions (1) and urinary incontinence (1 who underwent continent urinary diversion). CONCLUSIONS: Patients with neurogenic bladder and bowel disease may benefit from antegrade continence enema performed synchronously with a urinary procedure. Antegrade continence enema may be indicated alone for neurogenic bowel. Patient selection is important.     

The Malone antegrade colonic enema enhances the quality of life in children undergoing urological incontinence procedures

PURPOSE: Functional alterations of the gastrointestinal and genitourinary tracts, and physical limitations in children with spina bifida, imperforate anus and spinal cord injury challenge the ability to have independent fecal and urinary continence. Urologists have successfully helped these patients achieve urinary continence. We report our experience with the antegrade colonic enema procedure, which allows select individuals to achieve continence of stool, enhancing quality of life. MATERIALS AND METHODS: Since December 1992, 18 antegrade colonic enema procedures were performed in 12 female and 6 male patients 5 to 31 years old of whom 14 had spina bifida, 2 had imperforate anus and 2 had spinal cord injury. Simultaneous urological continence procedures were performed in 8 patients, including appendicovesicostomy in 4, augmentation cystoplasty in 2 and augmentation cystoplasty plus an ileal Mitrofanoff procedure in 2. Four patients previously underwent urological reconstruction. RESULTS: In 24 months of followup (average 6.6) all patients with a functioning stoma remained continent of stool and 17 were continent of urine. Complications related to the antegrade colonic enema procedure occurred in 4 children (22%) of whom 3 required further surgery. Three patients (17%) had minor stomal stenosis. CONCLUSIONS: The antegrade colonic enema procedure is easily performed and it should be considered for any child with significant physical limitations and/or refractory fecal incontinence before urological continence promoting procedures are done.     

Reproductive aging is an evolutionarily programmed strategy that no longer provides adaptive value

PURPOSE:The aim of this study was to assess the results of the Malone (antegrade colonic enema) procedure for fecal incontinence. METHODS: By a retrospective review of patients treated between 1990 and 1996 in a tertiary referral center, 36 patients were treated with a Malone procedure. Age at operation was 8.3 (range, 3 to 14) years, the mean period of follow-up was 39 (range, 9 to 72) months. The indication was fecal soiling in 35 and chronic constipation in one. The underlying diagnosis was an anorectal anomaly in the majority of patients. The appendix was used in 30 patients and a cecal flap in six, and a submucosal antireflux procedure was also performed in 10. In 35 patients, a circular stoma was created and in one a V flap was used. Antegrade colonic enemas were performed daily in 10, alternate days in 23, and in three patients the stoma was no longer used. Enemas were performed with a 10F catheter using a mixture of phosphate enema (or liquorice) and saline. RESULTS: Fecal soiling was completely controlled in 28 patients, and eight children soiled more than once a week. Complications occurred in 15 patients; the main problem was stenosis of the conduit, which occurred in 9 of 30 appendiceal stomas and three of six cecal stomas. Stomal stenosis was treated with surgical revision in eight patients. Additional complications were reflux through the stoma (n=2), pain on catheterisation (n=1), and small bowel obstruction (n=1). In one patient the Malone has been converted to a colostomy. CONCLUSIONS: The Malone procedure is a simple technique that can effectively control fecal incontinence in the majority of cases. It appeared to be better in older children. Stomal stenosis is a frequently encountered problem that may require surgical revision.     

Antegrade colonic enemas

Thirteen children aged 6-14 (mean 8) years in whom an antegrade colonic enema procedure was performed were reviewed retrospectively. All presented with refractory constipation or faecal soiling over a 3-year period. Nine of the children had previously undergone pull-through procedures for Hirschsprung's disease or high anorectal malformations. Two were suffering from spina bifida and two from idiopathic functional constipation. The operation was performed through a right iliac fossa incision. A catheterizable conduit was created. The appendix was brought out to the wound edge and made continent by intussuscepting the appendix base into the caecum. When the appendix was absent or unusable, a caecal tube was formed. Five patients suffered minor morbidity, six required a further operative procedure and two eventually required a sigmoid colostomy. However, the eventual outcome of a continent stoma was attained in 11 of the 13 children, all of whom would have been considered for sigmoid colostomy before introduction of the antegrade colonic enema procedure.     

Complications related to different continence mechanisms in ileocecal reservoirs

PURPOSE: We compared the incidence, treatment and outcome of complications related to different continence mechanisms in a single institutional series of continent urinary diversions using an ileocecal reservoir. MATERIALS AND METHODS: From November 1990 through October 1996 in 193 consecutive cases an ileocecal pouch (Mainz I) was used as a low pressure, high capacity reservoir. A submucosally embedded in situ appendix was used in 96 patients (mean age 57.2 years, mean followup 35.6 months) and an ileal intussusception valve was used in 106 (mean age 58.4, mean followup 33.1 months). Without exception the stoma was placed in the umbilicus. RESULTS: In 172 patients (85.2%) no stoma related complication was observed. In 17 patients (17.7%) with appendix stoma 23 reinterventions were performed, for appendico-umbilical stenosis in all but 2 cases (15.6%), occurring after a mean of 20.4 months. Two complete appendix necroses required replacement by ileal nipple. Stomal stenoses could be corrected as minor outpatient procedures. In 13 of 106 patients (12.3%) with intussuscepted ileal nipple a second operation became necessary after a mean interval of 9.6 months (partial/complete necrosis of nipple in 4 cases, dislocation of nipple from ileocecal valve in 3, detachment from fascia in 4 and stomal stenosis in 2). Whereas no calculi were observed in the appendix group, stones had to be removed from 3 patients (2.8%) with ileal nipple. CONCLUSIONS: In situ appendix and intussuscepted ileal valve techniques are satisfactory in providing ileocecal reservoir continence. Besides the known advantages of the appendix as the primary reconstructive approach, the treatment of subsequent complications is simple. Therefore, whenever an appropriate appendix is encountered it should be the intestinal segment of choice in forming a continence mechanism.     

Functional results following the antegrade continence enema procedure

BACKGROUND: Forty children who underwent the antegrade continence enema (ACE) procedure for faecal soiling were studied to determine factors predictive of outcome. METHODS: There were four patient groups: (1) ambulant with spinal dysraphism (n = 13), (2) wheelchair bound with spinal dysraphism (n = 14), (3) ambulant with miscellaneous disorders (n = 11) and (4) wheelchair bound with miscellaneous disorders (n = 2). Effectiveness of the procedure was assessed using technical evaluation and quality-of-life improvement (QOLI) scores (0-5). Objective assessment included colonic transit time (CTT) and anorectal manometry. Median follow-up was 21 (range 5-37) months. RESULTS: Some 28 of 40 children achieved continence. The procedure was reversed in four of 40 children. Of the other 36 children with a functioning ACE stoma, all reported improvement in quality of life (mean QOLI score 3.5). There were no significant differences in technical evaluation score, QOLI score, CTT, manometry findings or continence between ambulant groups and the wheelchair-bound group with miscellaneous disorders. QOLI score, anorectal squeeze pressure and continence were significantly poorer in those who were wheelchair bound with spinal dysraphism. Absent squeeze pressure was associated with poor outcome. CONCLUSION: Wheelchair-bound children with spinal neuropathy have a poorer outcome following the ACE procedure. Although ACE is an effective method of promoting faecal continence, it is essential to determine the aetiology of incontinence and sphincter function before operation.     

Myotonic dystrophy kinase modulates skeletal muscle but not cardiac voltage-gated sodium channels

PURPOSE: To evaluate the technique used for and long-term results of percutaneous cecostomy tube placement for the treatment of fecal incontinence in children. MATERIALS AND METHODS: After an initial pilot study in 15 patients, 42 additional patients with fecal incontinence aged 2-20 (mean, 11.5) years and weighing 9.9-109.0 (mean, 39.2) kg underwent percutaneous cecostomy tube placement. Twenty-nine patients had spina bifida, nine had imperforate anus, three had cloacal anomalies, and one had Hirschsprung disease. Mean follow-up was 265 days (range, 8-503 days). RESULTS: Tube placement was successful in all patients. One patient developed local inflammation after accidental early retention-suture removal, which was treated with suture replacement and intravenous antibiotics. Another developed postprocedural ileus, which resolved. Late complications included constipation in one patient (treated with diet alteration), granulation tissue in seven patients (treated with silver nitrate cautery), and accidentally dislodged tubes in three patients (two successfully replaced at home and one replaced at the radiology suite). Vomiting related to the phosphate enema occurred in two patients. Resolution of soiling was achieved in all patients. CONCLUSION: Percutaneous cecostomy and antegrade enemas are very successful in achieving fecal continence and patient independence and acceptability, with minimal early and late complications.     

The failed anoplasty: successful outcome after reoperative anoplasty and sigmoid resection

BACKGROUND/PURPOSE: Children with anorectal malformations often have less than optimal results after repair. The authors report on five patients (ages 3 to 17 years) born with imperforate anus and treated with anoplasty as a newborn. At presentation, all patients were completely incontinent of stool. None had ever experienced voluntary bowel movements, and all wore diapers continuously. METHODS: Perineal examination with the nerve stimulator showed the muscle complex was largely intact with good contraction, but the neoanus was outside of the muscle complex. Contrast enema showed massive dilation of the rectosigmoid colon and fecal impaction. A tethered spinal cord was excluded by magnetic resonance imaging (MRI). We treated these patients with a combined reoperative anoplasty via the posterior sagittal approach and sigmoid resection. RESULTS: Within 6 months after the procedure, all patients had achieved complete continence. They had from one to three voluntary bowel movements per day without soiling. CONCLUSIONS: Children with severe constipation and fecal incontinence after anoplasty should undergo evaluation by a surgeon. If examination shows a reasonably intact muscle complex and correctable anatomic defects, an excellent result can be achieved with appropriate reoperation.     

The incidence of spina bifida occulta in idiopathic scoliosis

A review of the roentgenograms of the spine of 100 patients with idiopathic scoliosis and 371 parents and siblings used as controls, showed an incidence of spina bifida occulta of 34 per cent and 41 per cent in their siblings. There was no increase in the incidence of spina bifida occulta in the patients with idiopathic scoliosis as compared with the control group. A review of the parents of these patients showed an incidence of spina bifida occulta of the lumbosacral area of 9 per cent in the mothers, and 13 per cent in the fathers, thus showing a decrease in the incidence of spina bifida occulta in the adult as compared with the teenager. The studies in the adult confirm the finding in the teenagers. There is no increased incidence of spina bifida occulta in idiopathic scoliosis.     

Combined Mitrofanoff and antegrade continence enema procedures for urinary and fecal incontinence

PURPOSE: Fecal soiling or intractable constipation frequently occurs in association with urinary incontinence in children undergoing major reconstructive urological operations. To treat double incontinence or the combination of wetting and severe constipation, we constructed a Mitrofanoff conduit and a channel for antegrade continence enemas in 18 patients between 1989 and 1995. We review the underlying pathological conditions, various surgical techniques and outcomes of these operations. MATERIALS AND METHODS: Underlying abnormalities mainly included spinal lesions, bladder exstrophy, imperforate anus and various cloacal anomalies. Patient age ranged from 2 to 18 years (average 8.4). In 13 patients both procedures were done simultaneously. The appendix was used to construct the antegrade continence enema channel in 8 cases and the Mitrofanoff channel in 5. It was long enough to be divided and used for both procedures in 2 cases but it was missing or unsuitable in 3. Alternative antegrade continence enema conduits were cecal flap in 7 patients and ileum in 1, while the ureter, ileum and detrusor tube were used to establish Mitrofanoff channels in 5, 5 and 1, respectively. Stomas were constructed according to the V-flap or V. Z. Q. technique and situated in close proximity in the right lower abdominal quadrant in 13 cases. RESULTS: Convalescence was uneventful except for 1 abscess near an antegrade continence enema stoma. Ten patients needed dilation or minor revisions due to difficulty in catheterizing the antegrade continence enema (5), Mitrofanoff (3) or both conduits (2). Subsequently 3 patients underwent repeat operations for reconstruction of 2 antegrade continence enema channels (cecal flap and ileum) and 1 detrusor tube Mitrofanoff channel. Currently 15 patients are dry on regular clean intermittent catheterization using 10 to 12F catheters. Outcomes of the antegrade continence enema channels are satisfactory in 15 patients who are clean or rarely soil. Failure occurred in 1 patients with severe constipation necessitating colostomy and 2 (1 noncompliant who stopped catheterizing regularly) in whom the channels subsequently closed. CONCLUSIONS: Synchronous construction of antegrade continence enema and Mitrofanoff channels is successful in the majority of doubly incontinent patients. Selection of patients with high motivation is important to obtain satisfactory results.     

Modification of Mitrofanoff principle for continent urinary diversion

In 1980, Mitrofanoff described a method of achieving continent urinary diversion by surgically closing the bladder neck and creating a continent catheterizable stoma from the appendix, which had been implanted in a non-refluxing manner into the bladder, or from a non-refluxing distal ureter. We describe a modification of the Mitrofanoff procedure for continent urinary diversion in 7 children in whom a standard Mitrofanoff procedure was not possible due to either body habitus or appendiceal anatomy. All 7 patients are continent both day and night. Four have required stomal revisions. Renal function has remained stable or improved in all patients. Although the revision rate was high, this modification of the Mitrofanoff principle has provided good long-term results in these patients and may be useful when patient's anatomy does not allow the creation of a standard appendicovesicostomy.     

Trajectories of autonomy development across the adolescent transition in children with spina bifida.

Objective: The current study investigated individual growth in autonomy development across the adolescent transition, comparing the trajectories of children with and without spina bifida. Method: Individual growth curve modeling procedures were utilized to describe the developmental course of autonomy across four waves of data collection, from ages 9 to 15, and to test whether illness status [spina bifida vs. matched comparison group (N = 68 for both groups at Time 1)] would significantly predict individual variability in autonomy development. Potential moderators [child gender, SES, and Peabody Picture Vocabulary Test (PPVT) score] of the association between illness status and autonomy development were also examined. Results: Children with spina bifida demonstrated distinct developmental trajectories, though the nature of the group differences varied by type of autonomy development (emotional vs. behavioral), context (i.e. school vs. family), and reporter. Significant interactions with PPVT score and child gender were found. Conclusion: Overall, children with spina bifida show considerable developmental resiliency, but may lag behind their peers in specific areas of autonomy. Boys with spina bifida, and children with spina bifida who have lower than average levels of verbal intelligence, appear to be at greater risk for exhibiting delays in autonomy development. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Experience with the hemi-Kock ileocystoplasty with a continent abdominal stoma

We describe our experience with the hemi-Kock ileocystoplasty with a continent abdominal stoma as an alternative to an indwelling catheter or supravesical diversion in 14 women and 4 men with various problems who could not perform intermittent urethral self-catheterization. The aim of management was also to provide, if possible, a competent urethra for additional access. Mean patient age was 37 years (range 22 to 75) and mean followup was 26 months (range 5 to 58). Preoperative management in the 11 wheelchair dependent women with neurological disease was an indwelling catheter in 7, urethral intermittent catheterization with the patient in the supine position in 3 and diapers in 1. Two women with a nonneurogenic bladder and a grossly incompetent urethra (1 after multiple incontinence and fistula repairs, and 1 after severe obstetrical trauma) wore diapers, while 1 with urinary retention and inability to perform self-catheterization had an indwelling catheter. The 4 men included 2 wheelchair dependent incontinent spinal cord injury patients who could not be managed with condom drainage, 1 with multiple anomalies who had trouble with self-catheterization, and 1 with an impassable postoperative stricture and a suprapubic tube. Surgery included anti-incontinence procedures in 10 patients and bladder neck closure in 3. A total of 15 patients required bladder augmentation in addition to the stoma and 3 had a stoma alone. Postoperative intervention was necessary in 4 women for stomal incontinence and in 2 of these bladder stones were removed simultaneously. One of these women was later treated for recurrent stones cystoscopically through the stoma. Overall, 17 of 18 patients are dry on intermittent stomal catheterization, with 1 lost to followup. We conclude that this procedure is a good alternative in patients with an end stage urethra or who cannot perform urethral catheterization because of physical disability. Establishing urethral continence and maintaining patency leaves a safety valve should the stoma fail. Since the bladder remains as a reservoir no ureteral surgery is necessary.     

Acute effects of nasal continuous positive airway pressure on 24-hour blood pressure and catecholamines in patients with obstructive sleep apnea

OBJECTIVE: To estimate the risk for spina bifida associated with the common mutation C677T of the MTHFR gene in a country with a relatively low prevalence of NTDs. DESIGN: Case-control study. SUBJECTS: Cases: 203 living patients affected with spina bifida (173 myelomeningocele and 30 lipomeningocele); controls: 583 subjects (306 young adults and 277 unselected newborns) from northern and central-southern Italy. SETTING: Cases: three spina bifida centres; young adult controls: DNA banks; newborn controls: regional neonatal screening centres. MAIN OUTCOME MEASURES: Prevalence of the C677T genotypes in cases and controls by place of birth; odds ratios for spina bifida and estimated attributable fraction. RESULTS: The prevalence of T/T, T/C, and C/C genotype was 16.6%, 53.7%, and 29.7% in controls and 25.6%, 43.8%, and 30.6% in cases, respectively. We found no differences between type of defect or place of birth. The odds ratio for spina bifida associated with the T/T genotype v C/C plus T/C was 1.73 (95% CI 1.15, 2.59) and the corresponding attributable fraction was 10.8%. No increased risk was found for heterozygous patients (OR=0.79, 95% CI 0.53-1.18). CONCLUSION: This study, as well as the meta-analysis we updated, shows that homozygosity for the MTHFR C677T mutation is a moderate risk factor in Europe, and even in Italy where there is a relatively low prevalence of spina bifida. The estimated attributable fraction associated with this risk factor explains only a small proportion of cases preventable by periconceptional folic acid supplementation. Thus, other genes involved in folate-homocysteine metabolism, their interaction, and the interaction between genetic and environmental factors should be investigated further.     

Current urologic management of cloacal exstrophy: experience with 11 patients

PURPOSE: Since 1980 the authors have treated 12 infants with cloacal exstrophy (10 classical and 2 variants). Eleven patients had repair, and are all surviving. The initial phases of management that led to improved survival have previously been reported. Quality of life is now a major focus for the cloacal exstrophy patient. During the past 10 years, nine of the 11 patients had lower urinary tract reconstructive procedures. This review evaluates experience with reconstructive efforts to achieve bowel and bladder control and to improve the quality of life in this complex group of patients. METHODS: Through review of patient charts and by patient interviews, data were collected to evaluate the ability to provide urinary and bowel control. A continence score was applied to provide a measure of success: voluntary control, 3; control with an enema program or intermittent catheterization, 2; incontinence with a well-functioning stoma, 1; and incontinence without a stoma, 0. The best continence score is 6 (genitourinary and gastrointestinal). Surgical complications, urodynamic and metabolic sequelae of continent urinary diversion were reviewed. RESULTS: At the time of the authors' previous report, eight of 11 patients had a continence score of 2 or less. Currently, eight of 11 patients have a score of 3 or better (five with enteric stoma and continent urinary diversion, two with enema program and continent urinary diversion, and one with enema program and continent bladder). Urinary-diversion procedures have included two gastric augmentations and five gastric reservoirs, two of which have required subsequent bowel augmentation. Gastric augmentations carry a definite risk of metabolic problems with three of our patients demonstrating significant episodes of metabolic alkalosis. In addition, results of urodynamic monitoring suggests that gastric reservoirs may be less compliant than reservoirs formed using other bowel segments. CONCLUSIONS: Modern principles of continent urinary diversion have been successfully applied to the cloacal exstrophy patient further improving their quality of life. Use of gastric flaps with preservation of intestinal length has been central to urologic reconstructive efforts. Use of stomach alone for formation of urinary reservoirs may produce suboptimal compliance, and composite ileogastric construction should be considered if the gastric flap is of marginal size.     

Depressive symptoms and self-concept in young people with spina bifida

Examined self-reported depressive symptoms in 72 young people with spina bifida, ages 9 to 18:11, and matched able-bodied comparison subjects, using the Dimensions of Depression Profile for Children and Adolescents (Harter & Nowakowski, 1987). Independent variables included gender, self-perceptions (including physical appearance), and perceived social support. Young people with spina bifida were at greater risk of depressive mood, low self-worth, and suicidal ideation. Girls, independent of disability, were at greater risk of depressive mood, low self-worth, and self-blame. Multiple regression analyses suggest that global self-worth serves as a mediating variable for the effect of physical appearance self-concept on depressed mood (particularly in young people with spina bifida), and that perceived parental social support has a direct effect on depressed mood (particularly in girls).     

Trajectories of family processes across the adolescent transition in youth with spina bifida.

The current study investigated change in family processes, including conflict, cohesion, and stress, across the adolescent transition, comparing the developmental trajectories of youth with and without spina bifida. Individual growth curve modeling procedures were utilized to describe the developmental course of family processes across 4 waves of data collection, from ages 9 to 15 years, and to test whether illness status (spina bifida vs. matched comparison group [N = 68 for both groups at Time 1]) would significantly predict individual variability in family processes. Potential moderators (child gender, socioeconomic status [SES], and child verbal ability) of the association between illness status and family functioning were also examined. Differences were found between the trajectories of family processes for families of youth with and without spina bifida. For families of youth with spina bifida, changes in family conflict and cohesion may be less dramatic than or inconsistent with what is expected during typical adolescence. Families of youth with spina bifida from low SES homes appear to demonstrate resilience in terms of family stress. (PsycINFO Database Record (c) 2010 APA, all rights reserved)