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Aim of the Study: The aim of this study was to evaluate the impact of implementing a Bowel Management Program (BMP) on the quality of life (QOL) in children with pseudoincontinence., Method: Children aged 2.5-13 years with pseudoincontinence were included. Evaluations were performed before and after BMP implementation. Perceptions from parents about QOL were assessed by a QOL questionnaire, and the severity of fecal incontinence (FI) was assessed using the fecal incontinence index (FII) of the Wexner scale., Results: 115 children (mean age of 7.54 ± 2.48) were studied over a 3-12 month period (mean duration 4.36 months). QOL improved from 2.45 ± 1.57 to 14.36 ± 1.37, P˂0.05. There was a significant improvement of the FII: 18.65 ± 1.25 versus 0.13 ± 0.39, P˂ 0.05. There was a significant inverse correlation between the final scores of QOL (14.36 ± 1.37) and FII (0.13 ± 0.39) after implementation of the BMP (r = -0.53; P ˂ 0.05)., Conclusion: This is the largest case series examining QOL in pseudoincontinent children. It demonstrates that BMP significantly improves the QOL of these children in the short and midterm. In addition, it is feasible to apply the FII in assessing incontinence in children. To our knowledge, this is the first study of its kind in our region., Level of Evidence: Level IV., (Copyright © 2019. Published by Elsevier Inc.)

Background: The provision of timely and comprehensive transition of care from pediatric to adult surgical providers for patients who have undergone childhood operations remains a challenge. Understanding the barriers to transition from a patient and family perspective may improve this process., Methods: A cross-sectional survey was conducted of patients with a history of anorectal malformation (ARM) or Hirschsprung Disease (HD) and their families. The web-based survey was administered through two support groups dedicated to the needs of individuals born with these congenital abnormalities. Categorical variables were compared using Chi-squared and Fisher's exact test with Student's t test and ANOVA for continuous variables., Results: A total of 118 surveys were completed (approximately 26.2% response). The average age of patients at time of survey was 12.3years (SD 11.6) with 64.5% less than 15years old. The primary diagnosis was reported for 78.8% patients and included HD (29.0%), ARM (61.3%), and cloaca (9.7%). The average distance traveled for ongoing care was 186.6miles (SD 278.3) with 40.9% of patients traveling ≥30miles; the distance was statistically significantly greater for patients with ARM (p<0.001). With regards to ongoing symptoms, 44.1% experience constipation, 40.9% experience diarrhea, and approximately 40.9% require chronic medication for management of bowel symptoms; only 3 respondents (3.2%) reported fecal incontinence. The majority of patients, 52.7% reported being seen by a provider at least twice per year and the majority continued to be followed by a pediatric provider, consistent with the majority of the cohort being less than 18years of age. Conversations with providers regarding transitioning to an adult physician had occurred in fewer than 13% of patients. The most commonly cited barrier to transition was the perception that adult providers would be ill-equipped to manage the persistent bowel symptoms., Conclusion: Patients undergoing childhood procedures for ARM or HD have a high prevalence of ongoing symptoms related to bowel function but very few have had conversations regarding transitions in care. Early implementation of transitional care plans and engagement of adult providers are imperative to transitions and may confer long-term health benefits in this patient population., Level of Evidence: Level IV, case series with no comparison group., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Aims: To compare anorectal manometry (AM) in patients with different types of anorectal malformations (ARMs) in relation to functional outcomes., Methods: A single-institution, cross-sectional study. After ethical approval, all patients ≥7years old treated for anterior anus (AA), perineal fistula (PF), vestibular fistula (VF), or rectourethral fistula (RUF) from 1983 onwards were invited to answer the Rintala bowel function score (BFS) questionnaire and to attend anorectal manometry (AM). Patients with mild ARMs (AA females and PF males) had been treated with minimally invasive perineal procedures. Females with VF/PF and males with RUF had undergone internal-sphincter saving sagittal repairs., Results: 55 of 132 respondents (42%; median age 12 (7-29) years; 42% male) underwent AM. Patients with mild ARMs displayed good anorectal function after minimally invasive treatments. The median anal resting and squeeze pressures among patients with mild ARMs (60 cm H2O and 116 cm H2O respectively) were significantly higher than among patients with more severe ARMs (50 cm H2O, and 80cm H2O respectively; p≤0.002). The rectoanal inhibitory reflex was preserved in 100% of mild ARMs and 83% of patients with more severe malformations after IAS-saving sagittal repair. The functional outcome was poor in 4/5 patients with an absent RAIR (BFS≤11 or antegrade continence enema-dependence). Rectal sensation correlated significantly with the BFS., Conclusions: Our findings support the appropriateness of our minimally invasive approaches to the management of mild ARMs, and IAS-saving anatomical repairs for patients with more severe malformations., Level of Evidence: III., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background/purpose: Knowledge about the functional outcome in adults with anorectal malformations is essential to organize structured transition to adult care for this patient group. The aim of this study was to investigate the functional outcome and quality of life in adults with anorectal malformations characterized according to the Krickenbeck classification., Methods: Of 256 patients diagnosed with anorectal malformations at our institution in 1961-1993, 203 patients could be traced and were invited to participate in the study. One hundred and thirty-six patients replied (67%) and were compared with one hundred and thirty-six population based sex and age-matched controls. Patients and controls were evaluated with both a validated questionnaire as well as a study-specific questionnaire to assess bowel function. SF-36 was used for quality of life. Outcome in nine incontinence-related parameters, 10 constipation-related, 6 urogenital function-related, and 13 quality of life parameters were assessed in the patients and compared to the outcome of controls as well as to the type of anorectal malformations according to the Krickenbeck classification., Results: The ARM-patients had an inferior outcome (P<0.05) for all incontinence parameters, 8 of 10 parameters for constipation, 2 of 6 for urogenital function and 7 of 13 quality of life parameters. Patients with rectobulbar and vestibular fistulas had the worst statistical outcome but patients with cloaca and rectoprostatic/bladder-neck fistula had worse outcome in absolute numbers. Forty-four patients (32%) reported incontinence of stool at least once a week and 16 (12%) had a permanent colostomy., Conclusions: The functional outcome and quality of life in adults with anorectal malformations are closely related to the type of malformation. A large proportion of the patients have persistent fecal incontinence, constipation and sexual problems that have a negative influence on their quality of life. Structured multidisciplinary follow-up of adults with anorectal malformations by pediatric and colorectal surgeons, as well as urologists and gynecologists is therefore advocated., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: Functional bowel outcome in patients with anorectal malformation often is poor. For fecal incontinence resulting from sphincter dysfunction, biofeedback (BFB) training appears to be effective. The aim of study was to investigate the bowel function in incontinent children treated for ARM, using a clinical score, a manometric and pelvic magnetic resonance evaluation, in order to establish predictive parameters of response after BFB., Methods: 25 children (median age of 6.5 years) with true fecal incontinence were evaluated by clinical score, anorectal manometry and magnetic resonance imaging (MRI). According to these evaluations patients were divided in 4 groups: group 1 (favorables manometry and MRI); group 2 (favorable manometry and unfavorable MRI); group 3 (unfavorable manometry and favorable MRI); group 4 (unfavorables manometry and MRI). All groups started a cycle of BFB and six months after end of BFB, were reevaluated by clinical score and manometry., Results: The overall response to BFB was excellent in 44%, discrete in 40% and poor in 16%; a better response was found in groups 1 and 2 than groups 3 and 4. The differences between groups before BFB proportionally correlated with values after BFB; a correlation with genitourinary and spinal anomalies was found., Conclusions: Our results showed that BFB is an effective for fecal incontinence when the assessment pretreatment (functional and morphologic) is favorable; the manometry can evaluate the potential sphincterial recovery after BFB with a further prognostic benefit if correlated to morphologic evaluation with MRI., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: To examine the effectiveness of posterior tibial nerve stimulation (PTNS) for the treatment of fecal and urinary incontinence in children with malformations of the bowel or neurological pathologies., Introduction: Treatment of fecal and urinary leaks, in cases of congenital malformations remains a challenge. Recent studies in adults have shown the effectiveness of PTNS., Method: Eight children: 4 with anorectal malformations, 3 with neurological causes (1 medullary lipoma, 1 Arnold Chiari malformation, 1 sacrococcygeal teratoma) and 1 with Hirschsprung's disease presenting with serious anal incontinence, despite extensive bowel management during at least 2 years, were treated with PTNS. Six children had associated urinary leaks. Jorge-Wexner score for defecation and Schurch score for urine were used before treatment and after the second and sixth months of stimulation., Results: After six months, five patients had no more fecal leakage, two patients were improved and one did not respond. Five out of the 6 patients with urinary leaks were continent at 6 months., Conclusion: PTNS is a noninvasive technique and painless modality which seems to be effective for the treatment of fecal and urinary leaks in children even with congenital digestive pathologies or neurological malformations. These results will be confirmed in a prospective study., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Introduction: Anorectal manometry (ARCM) provides valuable information in children with chronic constipation and fecal incontinence but may not be tolerated in the awake child. This study aimed to evaluate the effect of ketamine anesthesia on the assessment of anorectal function by manometry and to evaluate defecation dynamics and anal sphincter resting pressure in the context of pathophysiology of chronic functional (idiopathic) constipation and soiling in children., Methods: This was a prospective study of children who were investigated for symptoms of chronic constipation and soiling between April 2001 and April 2004. We studied 52 consecutive children who had awake ARCM, biofeedback training and endosonography (awake group) and 64 children who had ketamine anesthesia for ARCM and endosonography (ketamine group). We age matched 31 children who had awake anorectal studies with 27 who had ketamine anesthesia., Results: The children in awake and ketamine groups were comparable for age, duration of bowel symptoms and duration of laxative treatments. ARCM profile was comparable between the awake and the ketamine groups with regard to anal sphincter resting pressure, rectal capacity, amplitude of rectal contractions, frequency of rectal and IAS contractions and functional length of anal canal. Of 52 children who had awake ARCM, dyssynergia of the EAS muscles was observed in 22 (42%) and median squeeze pressure was 87mm Hg (range 25-134). The anal sphincter resting pressure was non-obstructive and comparable to healthy normal children. Rectoanal inhibitory reflex was seen in all children excluding diagnosis of Hirschsprung disease., Conclusions: Ketamine anesthesia does not affect quantitative or qualitative measurements of autonomic anorectal function and can be used reliably in children who will not tolerate the manometry while awake. Paradoxical contraction of the EAS can only be evaluated in the awake children and should be investigated further as the underlying cause of obstructive defecation in patients with chronic functional constipation and soiling., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: To clarify the puborectalis muscle (PM) function in pediatric patients with soiling after ileal J-pouch-anal anastomosis (IPAA) for ulcerative colitis, the author examined the neurological functions of the PM., Methods: Twelve patients 3 months after IPAA were studied (average age 13.6 years). Five patients showed soiling every day (group A1) and 7 patients showed soiling 2 to 3 days per week (Group A2). All patients 1.5 years after IPAA showed continence (group B). Twenty subjects served as a control (Group C; average age 13.5 years). Right, left and posterior sides of the sacral nerve terminal motor latency (SNTML) were measured by magnetic stimulation., Results: On the right, left and posterior sides, patients in group A1 exhibited significant prolongation of the SNTML compared with patients in groups A2, B and C (P<.05 each). Group A2 exhibited significant prolongation compared with groups B and C (P<.05 each). There were no significant differences of the SNTML between right and left sides, between right and posterior sides and between left and posterior sides in groups A1, A2, B and C., Conclusions: The SNTML of the PM demonstrates significant latency in those children who have early post-IPAA soilage. The neurological function of the PM potentially injured during an operation may recover, and correlates with normalization of continence., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Aims: Transanal colonic irrigation has been shown to be effective in bowel management program in adults. However, there exist limited data in children. We appraised the effectiveness of this technique in a series of children with incontinence or constipation and overflow soiling., Methods: Following ethical approval, a review of children with incontinence or constipation on a bowel management program with Peristeen® transanal colonic irrigation treated between 2007 and 2012 was performed. Irrigations were performed with a volume of 10-20 ml/kg of water with schedules depending on patient response. Data are reported as median (range)., Results: Twenty-three patients were reviewed. Median age at commencement of irrigations was 7 (2-15) years. Median follow-up is 2 (0.7-3.4) years. Diagnoses include the following: spina bifida (n=11), anorectal anomaly (n=6), Hirschsprung's (n=1), and other complex anomalies (n=5). Sixteen (70%) patients had associated anomalies. Twelve (52%) had constipation and overflow soiling, and 11 (48%) had fecal incontinence. Twenty (87%) had associated urinary wetting. Sixteen (70%) children used alternate-day irrigations, 4 (17%) daily irrigations, and 3 (13%) every third-day irrigations. Nine (39%) patients were taking oral laxatives. Sixteen (70%) reported to be clean and 3 (13%) reported a significant improvement, although were having occasional soiling. Four patients (17%) did not tolerate the irrigations and underwent subsequent colostomy formation for intractable soiling., Conclusions: In our experience, Peristeen® transanal colonic irrigation is an effective method of managing patients with focal soiling in childhood. Majority (83%) of children achieve social fecal continence or a significant improvement with occasional soiling. This was accompanied by high parental satisfaction. Peristeen® transanal colonic irrigation is a valid alternative to invasive surgical procedures and should be considered the first line of treatment for bowel management in children with soiling where simple pharmacological maneuvers failed to be effective., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: The main aim of the present study was to investigate how fecal incontinence might influence different aspects of quality of life (QOL) in children and adolescents with anorectal malformations (ARMs). We considered both the influence of fecal incontinence at time 1 of the study and the influence of fecal incontinence at time 2 (4 years later) on QOL measured at time 2., Methods: A total of 175 parents from the Italian Parents' and Patients' Association for Anorectal Malformations completed a questionnaire about fecal incontinence in their children at time 1 of the study. Four years later, 97 of these parents completed the Hirschsprung Disease/Anorectal Malformation Quality of Life questionnaire. Patient ages ranged from 4 to 17 years. Quality of life areas from the Hirschsprung Disease/Anorectal Malformation Quality of Life questionnaire were considered as outcome variables in a hierarchical regression analyses where fecal incontinence at time 1 and at time 2 served as predictors., Results: The principal findings indicated that fecal incontinence at time 2 significantly predicted constipating diet, presence of diarrhea, urinary continence, social functioning, emotional functioning, body image, and physical symptoms, also when severity of the malformation was taken into account., Conclusions: It is extremely important that pediatric surgeons continue to promote effective bowel management programs and that they work with other specialists and support associations to offer emotional and psychological support to patients with ARM and their families., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: Fecal incontinence and constipation are common problems in follow-up of anorectal malformations (ARMs). We evaluated the anal sphincters using the 3-dimensional endoanal ultrasonography (3D-EAUS) and the anorectal manometry after ARMs repair., Methods: Seventeen patients, divided into 3 groups according to Wingspread classification, underwent anorectal manometry and 3D-EAUS. Clinical, manometric, and endosonographic scoring systems were used., Results: The average anal resting pressure (aARP) was significantly higher in low ARMs than in intermediate and high ARMs. The anal squeeze pressure was not statistically different between the 3 groups. Three-dimensional EAUS visualized internal anal sphincter (IAS) disruptions in 7 of 17 patients and absence of IAS in 6 of 17 children with high ARMs. Scars of the external anal sphincter were localized in low ARMs and generalized in the other groups. In the case of IAS disruption with aARP greater than 20 mm Hg, fecal incontinence and constipation improved with biofeedback and/or laxatives, whereas daily enemas were necessary in absence of IAS with aARP less than 20 mm Hg. Statistical correlation was observed between endosonographic and manometric findings and clinical outcomes., Conclusions: Lesions of the anal sphincter are common in ARMs. Three-dimensional EAUS and anorectal manometry ensure a complete assessment of the anal sphincter and could provide useful information to define the most appropriate treatments to improve the quality of life., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: The pathophysiology of fecal incontinence from fecal impaction and rectal distension is poorly understood. We hypothesize that fecal impaction elicits up-regulation of cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-activated mucosal chloride channel., Methods: The anus was ligated to produce 75% stenosis in rats. Controls received ligation without inducing stenosis. 24 to 48 hours after ligation the colon was removed. Mucosal short-circuit current was measured by Ussing chamber. Western blot analysis was used to detect CFTR expression in the colonic mucosa. Ligated rats failed to defecate, whereas control rats stooled normally., Results: Ligated colons were markedly stool filled and dilated. Water content of feces was significantly increased to 66.5% ± 1.1% (P < .01, n = 12) 24 hours after ligation, vs controls (49.5 ± 5.2%, n = 12). Baseline short-circuit current was significantly increased in the distal (78.8 ± 7.4 μA/cm(2), n = 8, P < .01) and mid colon (24.5 ± 2.5 μA/cm(2), n = 8, P < .05) 24 hours after ligation, compared to control rats (12.5 ± 3.2 μA/cm2, n = 8). CFTR expression was significantly increased 24 hours after ligation in the mid and distal colon., Conclusion: We observe that fecal impaction from anal ligation induces early compensatory up-regulation of CFTR, altering function from net absorption to net secretion in the mid and distal colon., (Published by Elsevier Inc.)

Introduction: The antegrade continence enema (ACE) has been shown to be a safe and effective method for managing fecal incontinence in the pediatric population. The purpose of this study was to examine our experience with the ACE procedure using the appendix as a catheterizable conduit in children with anorectal malformations (ARMs)., Methods: We reviewed the charts of all patients who underwent an ACE procedure using the appendix as a catheterizable conduit between January 1992 and January 2010. Preoperative diagnosis (ARM type), operative details, functional outcomes, and postoperative complications were assessed. Technical modifications over time included selective cecoplication, implementation of the umbilical V-V appendicoplasty technique, and laparoscopy for cecal mobilization., Results: Mean age was 9.9 ± 0.6 years, and 67% were male. The most common preoperative diagnosis was rectourethral fistula in boys (39%) and persistent cloaca in girls (61%). Forty-five complications occurred in 41 patients with an overall incidence of 25.6% (stricture, 18%; leakage, 6%; prolapse, 4%; intestinal obstruction, 0.6%). The incidence of stomal leakage was lower in patients when a cecoplication was performed (2.9% [4/138] vs 29.4% [5/17]; P < .01), and the incidence of stricture was lower in patients when the umbilical anastomosis was created using the V-V appendicoplasty technique (11% [11/100] vs 30% [18/60]; P < .01). Successful management of incontinence was reported by 96% of all patients., Conclusions: The ACE procedure using the umbilical V-V appendicoplasty provides an effective and cosmetically superior means for bowel management in children with ARMs. The rate of late complications is not insignificant however, and preventative strategies should focus on careful operative technique and ensuring compliance with catheterization protocols well past the initial postoperative period., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: The purpose of this study is to discuss the effect of pelvic floor muscle training on fecal incontinence., Methods: A retrospective study was performed on patients who received pelvic floor muscle training from March 2002 to April 2007. There were 55 patients with fecal incontinence (male, 32 cases; female, 23 cases; mean age, 9.4 years old from 6 to 14), including 39 cases of anorectal malformation and 16 cases of Hirschsprung's disease. Pelvic floor muscle training was performed using biofeedback for 2 weeks in hospital, 2 times each day, and 30 minutes each time. The patients were then instructed to carry out self-training at home without the biofeedback device daily and received training evaluations in the hospital outpatient department monthly. All patients completed the training regimen and were followed up for 1 year. Anal manometry and clinical score were evaluated before and after training., Results: Anal continence of 30 patients had satisfactory improvement, but not for the other 25 cases after training. The mean anal squeeze pressures of the group that had good results and the group that had poor results were 98.4 +/- 7.3 and 47.4 +/- 13.6 mm Hg, respectively, before training. There were 31 patients whose anal squeeze pressures were above 80 mm Hg, and 26 of these had satisfactory anal continence improvement, including all patients with Hirschsprung's disease. On the contrary, only 4 of 24 cases whose anal squeeze pressure was below 80 mm Hg acquired satisfactory anal continence improvement., Conclusions: Pelvic floor muscle training could achieve good results in some patients with fecal incontinence. Baseline measurements during anorectal manometry appear to provide good prediction of prognosis and effective management.

Background/purpose: The purpose of this report was to review the results of the antegrade continence enema (ACE) procedure and to compare the outcomes of right and left colon ACEs in children., Methods: Thirty patients who underwent an ACE between 1998 and 2005 were analyzed. Data were obtained based on the following parameters: postoperative soiling, catheter insertion time, colonic washout time, quality of life, and abdominal pain during and after the ACE. Twenty-nine patients were followed for an average of 3.8 years (range, 4 months-7.3 years)., Results: Right colon ACEs were performed in 23 patients, and left colon ACEs were performed in 7 patients. The common complications of the ACE included abdominal pain during and after the ACE (51.7%) and stoma strictures (41.4%). The overall ACE success rate was 24/29 (82.8%; right colon ACE, 18/29; left colon ACE, 6/29). Twenty-three patients (95.8%) believed their quality of life was improved. There were no significant differences in complications or outcomes between the right and left ACEs., Conclusions: An ACE is an effective treatment for children with fecal incontinence. A left colon ACE has similar efficacy as a right colon ACE in managing fecal incontinence in children.

Background/purpose: In this study, the patients operated on for anorectal malformations (ARM) were evaluated in terms of segmental (SCTT) and total colonic transit times (TCTT) and clinical status according to Krickenbeck consensus before and after treatments., Methods: Forty-one patients with ARM (28 males/13 females) older than 3 years (median age, 7.7 years; range, 3-25) who had no therapy before were assessed for voluntary bowel movements (VBM), soiling (from 1 to 3), and constipation (from 1 to 3), retrospectively. Distribution of the patients were rectourethral fistula (17), perineal fistula (PF; 8), vestibular fistula (VF; 8), cloaca (3), rectovesical fistula (1), rectovaginal fistula (1), pouch colon with colovestibular fistula (1), no fistula (1), and unknown (1). The patients ingested daily 20 radiopaque markers for 3 days, followed by a single abdominal x-ray on days 4 and 7 if needed. The results were compared with the reference values in the literature., Results: Mean follow-up period was 36 months (range, 1-108.5 months). All patients but 1 had soiling in different degrees. Twenty-one patients who had VBM were divided into group 1, with constipation (n = 9), and group 2, without constipation (n = 12). The other 19 patients who had no VBM were divided into group 3, with constipation (n = 14), and group 4, without constipation (n = 5). The longest TCTT and rectosigmoid SCTT were found in group 3 (69.5 and 35.2 hours, respectively). Group 1 had long SCTT in rectosigmoid but normal TCTT (27.8 and 47.4 hours, respectively). Groups 2 and 4 had normal SCTT and TCTT, and there was no significant difference between them. After the appropriate treatment, of the patients, 45% (18/40) had no soiling, and the soiling score decreased to grade 1 in 27.5% (11/40) and to grade 2 in 10% (4/40). Four had unchanged soiling score, and 3 were excluded from the study because of follow-up problems. Half of the patients in group 3 (4 VF, 2 rectourethral fistula, PF) gained VBM without soiling after laxative treatment. Only four of 23 patients had decreased constipation score (2 cloaca, PF, VF)., Conclusions: In this study, ARM patients complaining of constipation with or without VBM had prolonged SCTT in the rectosigmoid region. Percentage of the improvement in soiling scores was more conspicuous than that of constipation scores. The dismal figure observed at the first examination in the assessment of VBM was not associated with an unfavorable improvement with laxative treatment. So, it is suggested that assessment of VBM initially may be deceptive for clinical status.

Purpose: The internal anal sphincter (IAS) plays an important role in the pathophysiology of constipation and incontinence. We hypothesized that functional bowel obstruction in premature infants is because of a poorly developed IAS. We investigated the neuromuscular development of IAS in fetal, newborn, and adolescent pigs., Methods: Paraffin sections of IAS from 5 different age groups, E60, E90, 1 day, 4, and 12 weeks old, were stained with protein gene product 9.5 (PGP9.5), *-smooth muscle actin (*-SMA), caldesmon (CALD), calponin (CALP), and desmin (DES) antibodies. Quantification of results was performed by grading the density of immunostaining., Results: The PGP9.5-positive ganglion cells were observed in the myenteric and submucosal region of the entire length of the IAS at E60. An increase in ganglion cell size and density was observed with increasing age. There were striking differences in the density of PGP9.5, alpha-SMA, DES, CALD, and CALP immunoreactive fibers between prenatal and postnatal period with gradient increase in the number of fibers from after birth to 12 weeks of age., Conclusion: This study shows for the first time that there are age-related differences in the distribution of neurons and smooth muscle cell components in the IAS. The decreased expression of contractile and cytoskeleton proteins in smooth muscle cells together with decreased expression of neurons in the IAS in the perinatal period may lead to motility dysfunction causing functional intestinal obstruction seen in premature infants.

Background/purpose: Little is known about the quality of life (QOL) of children with Hirschsprung's disease (HD) as they grow older. The purpose of this study was to measure the QOL and bowel function of these children as they mature., Methods: All children who were surgically treated for HD at British Columbia Children's Hospital, Vancouver, British Columbia, Canada between 1986 and 2003 were invited to participate. Each family was sent 3 previously validated questionnaires exploring current QOL and bowel function., Results: Fifty-one families participated (49%), with children between the ages of 3 and 21 years. Fecal continence improved significantly with age (P = .04) and was the strongest predictor of QOL scores of all variables in our study. There was no statistically significant difference in QOL scores between children with HD and healthy children, although a clinically relevant impairment in QOL may be present, especially in psychosocial scores., Conclusions: Fecal continence is an important predictor of overall QOL in children surgically treated for HD. Although continence tends to improve with age, a number of older children still have ongoing continence problems, and they seem to be a group at risk for impaired QOL. Our study indicates that interventions for children with incontinence may offer gains in QOL as well as bowel function.

Background: Magnetic resonance imaging (MRI) of the pelvic floor allows better assessment of pelvic pathology and has a potential as an adjunct for therapy planning. In complex congenital malformations of the pelvic floor and continence organs, it plays a major role in assessing urinary and fecal incontinence or constipation, especially when performed as a dynamic investigation such as MRI defecography., Patients and Methods: Twenty-three patients (3-21 years old) with urinary and/or fecal incontinence or constipation attributable to congenital malformations of the pelvic region presented at our institution. The diagnoses were anorectal malformations (18), bladder exstrophy (2), and cloacal exstrophy (3). All patients underwent static and dynamic MRI of the pelvic floor with rest, squeeze, and evacuation in supine position., Results: Morphology and function of the pelvic floor and pelvic organs could be demonstrated in each case. The reason for urinary incontinence, fecal incontinence, or constipation could be defined, and an individual therapeutic management concept was made based upon the data obtained by the investigation., Conclusions: The advantages of this method, in comparison to others, are direct visualization of the pelvic floor muscles and continence organs and their anatomical relationship during different functional actions. Pelvic floor dysfunction is often the reason for fecal and urinary incontinence and can be detected by MRI. Especially in children, minimizing radiation exposure is of major concern. Disadvantages are the costs and long investigation time.

Purpose: The aim of this study was to define the predictive value of colonic manometry and contrast enema before cecostomy placement in children with defecation disorders., Methods: Medical records, contrast enema, and colonic manometry studies were reviewed for 32 children with defecation disorders who underwent cecostomy placement between 1999 and 2004. Diagnoses included idiopathic constipation (n = 13), Hirschsprung's disease (n = 2), cerebral palsy (n = 1), imperforate anus (n = 6), spinal abnormality (n = 6), and anal with spinal abnormality (n = 4). Contrast enemas were evaluated for the presence of anatomic abnormalities and the degree of colonic dilatation. Colonic manometry was considered normal when high-amplitude propagating contractions (HAPC) occurred from proximal to distal colon. Clinical success was defined as normal defecation frequency with no or occasional fecal incontinence., Results: Colonic manometry was done on 32 and contrast enema on 24 patients before cecostomy. At follow-up, 25 patients (78%) fulfilled the success criteria. Absence of HAPC throughout the colon was related to unsuccessful outcome (P = .03). Colonic response with normal HAPC after bisacodyl administration was predictive of success (P = .03). Presence of colonic dilatation was not associated with colonic dysmotility., Conclusion: Colonic manometry is helpful in predicting the outcome after cecostomy. Patients with generalized colonic dysmotility are less likely to benefit from use of antegrade enemas via cecostomy. Normal colonic response to bisacodyl predicts favorable outcome.

Background: The aim of this study was to design an individualized biofeedback program based on the underlying dysfunctions and to assess the short-term and long-term clinical and quality-of-life outcomes for patients with imperforate anus., Methods: Thirty-one patients were treated using an individualized biofeedback program according to their different dysfunctions after having completed the assessment of the anorectal function. Many special biofeedback training methods that consist of strengthening the perianal muscles biofeedback, sensory discrimination biofeedback, synchronizing external sphincter contraction biofeedback, and improving defecation dynamic biofeedback were selected to make up the individualized biofeedback training program. The clinical scores and quality-of-life scores were assessed in the short-term and long-term follow-up., Results: The clinical scores and quality-of-life scores of patients before training, after biofeedback training, at short-term follow-up, and at long-term follow-up were 3.2 +/- 1.1 and 8.7 +/- 1.1, 5.2 +/- 0.6 and 11.0 +/- 0.8, 5.1 +/- 0.6 and 10.8 +/- 0.9, and 4.6 +/- 0.7 and 10.1 +/- 0.7, respectively. After biofeedback training, the strength of the perianal muscles were increased significantly, the abnormal rectal threshold sensation and defecation dynamic reverted to normal in all patients, and 82% patients who have no external anal sphincter reflex acquired the new reflex. In the short-term follow-up, 12 (57%) patients maintained the clinical outcome, and only 9 (43%) patients have regressions slightly. At long-term follow-up, 7 (33%) patients maintained the clinical outcome very well, and 14 (67%) patients had regressed. Although the clinical and quality-of-life scores have decreased slightly at follow-up, they were still significantly increased compared with those before biofeedback training., Conclusions: The individualized biofeedback is more suitable for improvement of the clinical outcome and the quality of life, and maintained a good clinical outcome and quality of life on the short-term and long-term follow-ups.

Background/purpose: A simple and objective method evaluating the bowel functions of patients with anorectal anomalies is necessary. The authors applied fecoflowmetry and saline enama test for patients with anorectal anomalies to evaluate the fecodynamics and anorectal motilities in these children., Methods: The bowel functions of 16 patients who underwent repair for anorectal malformations and 5 normal controls were evaluated by saline enema test and fecoflowmetry. The correlations between the clinical scores for the bowel functions and the parameters in fecodynamic studies were investigated., Results: Seven of 16 patients exhibited periodical contractions of the rectum synchronized with relaxations of the anal canal during saline infusion as did the controls and had significantly higher clinical scores than the other patients. Two patients with severe chronic constipation lacked rectal contractions. Among the fecoflowmetric parameters, the maximum flow, average flow, and tolerable volume of saline infused into the rectum were significantly lower in the patients with low clinical scores than those of the controls. The maximal squeeze pressure and resting anal pressure were not significantly different between the patients and controls., Conclusion: Fecodynamic studies, such as fecoflowmetry and saline enema test, help in obtaining clinical indicators for the bowel functions of patients with anorectal anomalies., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Background/purpose: The aim of the study was to assess anal sphincter performance in relation to clinical fecal continence in adult patients who have Hirschsprung's disease., Methods: Fifty-four adult patients (mean age, 29 +/- 7.2 years; 46 men; 8 women) who had undergone surgery for Hirschsprung's disease during their childhood underwent anorectal manometry and clinical examination. Fecal continence was evaluated with a quantitative scoring method (scoring, 0-14; 14, normal bowel function; 10-13, good continence, no social problems; 5-9, fair continence, marked social limitations; 0-4, total incontinence). Thirty healthy adults were used as controls., Results: Fourteen patients had normal bowel habits according to the quantitative scoring. The median anal resting pressure of these patients was 25 cm H2O (range, 15-37.5). The median resting pressure of patients with good continence (n = 30; median, 20 cm/H2O; range, 5-27.5) and with fair continence (n = 6; median, 15 cm/H2O; range, 5-27.5) was significantly lower (P < .01) than in patients who had normal continence. There was no statistical difference in maximal squeeze pressure between the patient groups (median normal, 52.5; good, 45; fair, 52.5). In the controls, the median resting pressure (61.5 cm H2O; range, 34-105) and maximal squeeze pressure (86 cm H2O; range, 55-148) were significantly higher than in all patient groups (P < .0001). The voluntary sphincter force (maximal squeeze pressure minus resting pressure) was similar in patients and controls (patients median, 27 cm H2O; controls median, 16 cm H2O, NS)., Conclusions: There is a positive correlation between functional outcome and anal resting pressure in adults who have repaired Hirschsprung's disease. The overall low resting pressure reflects internal sphincter dysfunction, which may be caused by operative trauma. Despite this, most patients have a satisfactory functional outcome, which is probably related to normal voluntary sphincter performance.

A consecutive series of 90 children with anorectal malformations was reviewed for urologic problems related to lower urinary tract dysfunction. Neurogenic bladder-sphincter dysfunction was seen in 22 patients (24%), all but one of whom had sacral agenesis. Vesicoureteral reflux was present in 60% of the patients with dysfunction, and 32% had reflux nephropathy. Urinary incontinence was present in 91% of the patients who had bladder-sphincter dysfunction. However, the management of bladder-sphincter dysfunction and urinary incontinence was not as straightforward as for patients with myelodysplasia because the parents were more reluctant to accept the therapeutic measures. It was particularly difficult to introduce clean intermittent catheterization (CIC), especially in older patients, because genital and urethral sensation often was undisturbed. Only if CIC had been started in the neonatal period or early infancy were there no problems with acceptance and parental compliance. The authors emphasize the importance of urodynamic testing of neonates and infants who have an anorectal malformation and associated sacral agenesis in identifying those who have neurogenic bladder-sphincter dysfunction. Consequently, patients with lower urinary tract dysfunction should receive prompt treatment, including CIC if necessary, to prevent or reduce secondary urologic morbidity, especially loss of renal function.

From 1985 to 1994, 27 patients with high- or intermediate type imperforate anus who underwent anorectoplasty were studied for postoperative function, particularly with respect to the rectoanal relaxation reflex and continence. Fourteen of the patients had a rectourogenital fistula and were treated with posterior sagittal anorectoplasty using the fistular end as the neoanus (internal sphincter-saving). Nine patients had a blind rectal pouch and received posterior sagittal anorectoplasty using the trimmed bowel end for reconstruction of the neoanus (incomplete internal sphincter-saving). The other four had Rehbein's mucosa-stripping endorectal pull-through combined with anterior sagittal anorectoplasty (none internal sphincter-saving). A positive rectoanal relaxation reflex was found in 8 of 14 (57.1%), 7 of 9 (77.8%), and 3 of 4 (75%), respectively. It appears that the internal sphincter-saving procedure is not essential for the development of the rectoanal relaxation reflex. Compensation or adaptation most likely contributes to the presence of the rectoanal relaxation reflex, and perhaps to postoperative continence.

An experimental model in the pig rendered incontinent of feces was developed to assess the sphincteric activity of the transposed antropylorus. In the control group, normal defecation was studied clinically, radiologically, and manometrically. Nineteen 7- to 10-week-old pigs were rendered incontinent by resection of 20 cm of colon and rectum to below the dentate line. The antropylorus was prepared on its own blood supply and transposed to the anus, initially with a colostomy, which was closed 15 to 21 days later. Clinically these pigs passed semisolid stool in a piecemeal fashion. Contrast defecography showed hold-up at the pylorus, reflux of contrast into the colon, with pyloric contraction independent of antral stimulation. Manometry showed pyloric contraction with rise in antral pressure and independence. The authors conclude that transposition of an antropyloric segment to the anus provides a sphincter-like mechanism and could have application in fecal incontinence.

The feasibility of skeletal muscle transposition and electrical stimulation (dynamic myoplasty) for treatment of fecal incontinence has been shown in adults. It might be attractive to use such a technique in pediatric patients. Therefore, the influence of growth on skeletal muscle transposition and stimulation was studied in five puppies. In each dog, two neosphincters were constructed around a Thiry-Vella loop by using a dissected sartorius muscle with the neurovascular supply intact. In each of these five puppies, one of these muscles was electrically stimulated during a mean of 19 weeks (one puppy died during the first postoperative week). Muscle biopsies showed an increase in the percentage type I, fatigue-resistant muscle fibers from 61 to 94 in electrically stimulated sartorius neosphincters, but also an increase from 57 to 67 percent in transposed nonstimulated sartorius muscles. The diameter of these type I fibers during growth increased 36% in eletrically stimulated sartorius neosphincters and 55% in nonstimulated sphincters. Function of the neosphincters was tested with the inflow of saline in the Thiry-Vella loop. It was shown that the stimulated neosphincters were capable of inhibiting flow (which corresponded to manometric pressure registrations), but the nonstimulated sphincters were unable to inhibit flow. The experiments were complicated by infection and necrosis around the implanted stimulators in four puppies (which required reimplantation). We conclude that a dynamic myoplasty for fecal incontinence is feasible in growing puppies but that the technique is unacceptable during rapid growth because of the risks of infection and dislocation of the implanted device.

The aim of this study was to assess whether an electrically stimulated graciloplasty (dynamic graciloplasty) can achieve continence in nine patients with anal atresia (median age, 28 years; range, 18 to 40). As the first procedure, a gracilis muscle was transposed. Six weeks later, intramuscular electrodes were implanted and connected to a pulse generator. Eventually, the muscle was gradually trained, by electrical stimulation, to achieve fecal continence. Continence was obtained in five patients (55%). Manometry demonstrated an increase in mean anal pressure, from 36 mm Hg (without stimulation) to 52 mm Hg (with stimulation), after 8 weeks (mean increase, 16 mm Hg, [95% confidence interval, 8, 24 mm Hg; n = 9; P < .01). Failures resulted from a noncontracting distal part of the gracilis muscle (in three patients) and a nondistending rectum (in one patient). We conclude that dynamic graciloplasty can achieve continence in a substantial number of patients with thus-far untreatable incontinence after surgical correction for anal atresia.

Sixteen patients aged 12 to 16 years (mean, 14.9) with corrected low anorectal anomalies, and eight age-matched controls were studied. Eight patients were totally continent, six had staining, and two had fecal soiling. Half the patients had trouble with constipation. The grade of incontinence was related to significantly reduced resting and squeeze pressure in the anal canal. Girls had significantly worse results than boys. The anal sphincter muscle complex was visualized by anal endosonography, and images of the external anal sphincter corresponded to the results of the surface electromyography. In addition, anal endosonography provided a detailed picture of all perianal structures including the internal anal sphincter. Anal endosonography supplements other imaging techniques for assessing the perianal structures in patients who have an accessible anal canal.

Anorectal function was clinically, manometrically, and radiologically followed-up in 40 patients who underwent an internal sphincter-saving posterior sagittal anorectoplasty in 1984 to 1989. The functioning internal sphincter was manometrically verified in 83% (33/40) of the patients. Soiling related to sphincter insufficiency was found in only 4 of the 33 patients with a functioning internal sphincter; 5 of the 7 patients without an internal sphincter had soiling. Symptomatic constipation was found in 73% (24/33) of the patients with a functioning internal sphincter, but in only 28% (2/7) of the patients without this structure. Constipation was not associated with a stenotic anal outlet in any patient. On anorectal manometry, there was no statistical difference in basal and pressures and internal sphincter reflex threshold values between constipated and nonconstipated patients. Histologically, ganglion cells were found in the proximal anal canal in all cases. The radiological size of the rectal pouch before closure of the protecting colostomy had a positive correlation with the severity of constipation. Medical treatment with diet and bulk-laxatives (13 patients) or with stimulant laxatives and occasional enemas (11 patients) successfully relieved constipation in 24 patients. In 2 patients, the symptoms were refractory to medical treatment and a resection of the megarectum was required. In conclusion, the preservation of the internal sphincter in patients with high or intermediate anorectal anomalies gives a good fecal continence outcome, but is associated with a high incidence of symptomatic constipation.

Eight patients with fecal incontinence after surgery for anorectal malformations received one to four sessions of biofeedback therapy. The physiologic status of the anorectum before and after biofeedback therapy was investigated by anorectal manometry and electromyography of the external sphincter. The effectiveness of biofeedback treatment and the indications for its use in patients with fecal incontinence were investigated. Three of the four patients responded well to biofeedback therapy following three or four training sessions. However, in the other five patients who had only one or two sessions, a good response to the therapy was not obtained. Anorectal manometry and electromyography recordings of the external sphincter showed that biofeedback therapy improved voluntary sphincter function. The three good responders had an adequate and resting pressure both before and after biofeedback therapy and had good electrical activity of the external sphincter after therapy. In the presence of adequate resting and pressure, biofeedback therapy should be attempted for the treatment of fecal incontinence after correction of anorectal malformations before resorting to further surgical intervention. However, biofeedback therapy may not be suitable if the sphincters are markedly hypoplastic, or do not surround the anal canal after an inappropriate pull-through operation.

Motility of the rectosigmoid and rectum and reservoir function of the rectum after surgery for anorectal malformations were investigated in 32 patients (17 with high type, 6 with intermediate type, and 9 with low type anomaly) aged 5 to 16 years. All 32 patients were examined manometrically as well as with a newly devised myoelectrical method. Manometry showed that the values of maximum anal pressure and anorectal pressure difference in the high type were significantly lower than those in the low type. However, the incidence of contractile activity of the rectosigmoid was not significantly different between these two groups. The threshold sensation pressure and the maximum tolerable pressure in the high type were significantly higher than those in the low type, and the rectal compliance in the high type was significantly lower than that in the low type. Electromyography was recorded at 8 cm and 5 cm from the anal verge. Two types of slow waves were observed, a faster rhythm and a slower rhythm. Their frequency was similar in the three groups. However, the numbers of spike bursts in the high type and intermediate type were significantly higher than those in the low type. These results indicate that in addition to an inadequate anal resting pressure a loss of optimal rectal sensation or rectal reservoir function might be associated with fecal incontinence in the high type and that increased spike bursts might play some role in rectal motility.

Embryologically a fistula in an anorectal malformation can be regarded as an ectopic anus. Since 1984 the so-called fistula has been saved and used as the new anal canal in the surgical reconstruction of 48 patients with high or intermediate imperforate anus. A positive rectoanal inhibitory reflex indicating internal sphincter function was recorded in 32 of 43 patients investigated with anorectal manometry. These patients showed significantly better anal continence. It is concluded that there is an internal sphincter "anlage" in the fistulous connection from the bowel to the urogenital tract. Internal sphincter function can be obtained in the majority of the patients, which seems advantageous for their anal function.

Of 49 children with imperforate anus, 23 underwent an abdominoperineal procedure for a high and seven for a low maliformation, 17 had a perineoplasty for a low and two for a high malformation. In each subject, rectal and anal sphincteric resting pressures were studied at least 1 yr after surgery. Of 30 subjects who had undergone an abdominoperineal procedure, eight were continent in contrast to 15 out of 19 patients who had had a perineoplasty (p less than 0.001). In patients who had undergone an abdominoperineal procedure, the rectal motility was of the colonic type, with waves of higher amplitude and lower frequency (p less than 0.01) than in the normal rectum in 23 cases and in most of these, peristalsis was recorded down to the anal margin. Incontinence was most frequently associated with abnormally short anal resistance, low anal pressure, reduced sensibility, weak voluntary contraction and absence of rectoanal inhibitory reflex. In the group of patients who underwent perineoplasty, continence was associated with normal mechanical parameters and normal physiologic behavior of the anal sphincter.

In correcting anorectal incontinence, the analysis of all known factors responsible for a lack of function is mandatory. Besides anamnestic and clinical data, manometric investigation is especially helpful. Continence may be improved by conservative measures (biofeedback training, muscle training, and drugs). The indication for surgical procedures depends on clinical and manometric findings: missed portions of the sphincteric muscle complex are reintegrated with a repull-through procedure. In some cases, Kottmeier's levatorplasty or a modification of it has led to some improvement of continence. Whenever a call to stool was present, gracilis transposition has markedly improved anorectal function in 22 of 27 operated patients. Free transplantation of palmaris longus muscle has not proven to be of functional advantage. Doubling the circular colonic musculature and an S-shaped anoplasty at the time of the primary pull-through procedure may lead to continence in the future.

Two hundred and twenty-five patients aged from neonate to 15 years presenting with chronic constipation and soiling had anorectal manometry without sedation, and 142 patients in the same age range and with a similar range of presenting complaints had anorectal manometry using ketamine as an anesthetic. There were no significant differences between the groups in the resting pressures recorded in the anal canal, in the amount of inhibition with rectal distension, or in the frequency or amplitude of rhythmical activity of the internal anal sphincter. Classical inhibitory troughs were seen when expected in both groups as were signs of external sphincter activity. Ketamine anaesthesia is a suitable sedative which enables anorectal manometry to be performed on young or nervous patients and does not alter the qualitative or quantitative responses.

Since the introduction of the levatorplasty, 44 patients were operated upon for anal incontinence on our service. In 31 patients, a levatorplasty was performed. The selection of the levatorplasty as operative procedure was based on the availability of a functional levator as demonstrated by physical examination, anorectal manometry, and defecography. The 31 patients ranged from infancy to 76 years of age and 30 of them were followed up to 7 years with an average of 3 years. In 18 patients with fecal incontinence following operative procedures for anorectal malformations, satisfactory continence was restored in 8, improvement was found in 6, and 4 patients remain incontinent. In 13 patients with incontinence following operative procedures or trauma, the postoperative results were satisfactory in 9 patients, 2 improved, and 2 failed. The review showed that the levatorplasty was most successful in patients in whom the revised but normally innervated levator replaces a destroyed external sphincter in the presence of an intact internal sphincter. Only one half of all patients with major incontinence following an operative procedure for anorectal malformations had an adequate levator that allowed successful restoration of function. The review showed that the levatorplasty can improve or restore anal continence in a variety of conditions in properly selected patients.

The measurement of the strength of the anal sphincters has heretofore involved voluntary cooperation by the patient. In some children one cannot count on the maximum squeeze pressure being generated and in others the child is too young to understand what is required. We have devised an objective method of measuring the anal sphincter force (ASF) and have collected data derived from over 256 children over the course of 24 months. These include patients with Hirschsprung's disease, constipation, postoperative incontinence, spina bifida, and normal children both conscious and anesthetized. The results are presented and the value of the test and its applications are discussed.

Functional results after surgical correction of anorectal malformations were assessed on a clinical basis following Kelly score and by manometric study. Forty-six patients, aged 2--17, were personally interviewed and 25 of these 46 had manometric studies to evaluate postoperative continence. The manometric study was also performed on 35 normal children as a control group. Continent patients characteristically had marked high pressure zones as did the normal subjects. On the other hand, in the patients with fair or poor results, the anorectal pressure profile had no marked high pressure zone in the anal canal. The presence of normal anal pressure at rest as well as adequate ano-rectal pressure difference was found to correlate well with continence. In patients with perineoplasty, the ano-rectal reflex correlated well with continence but not in patients treated by abdominoperineal rectoplasty.