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Presence of transition zone (TZ) in the pulled colon can impact the outcome of surgery in children with Hirschsprung’s disease. There is a wide variation in terminology used to define TZ and its management. We present our series of managing 11 such children with considerations for conservative management. Eleven of 114 children operated for Hirschsprung’s disease had features of TZ on the 4-quadrant doughnut assessment of proximal anastomosing margin. They were followed up for development of obstructive symptoms, failure of pull-through procedure or bowel-related complications. Intervention done were observation with laxatives, dilatation, Botox injection and redo pull-through. Of the 11 children, 6 underwent Duhamel’s procedure and 5, transanal endorectal pull-through (TERP). Features identified on HPE were presence of hypertrophic nerve bundles involving 2 or 3 quadrants in the circumferential doughnut biopsy of proximal anastomosing margin. Observed symptoms included constipation, enterocolitis, increased bowel frequency and soiling. Intervention done were use of laxatives with bowel management program in six and Botox injections in four. Only one child with TZ in 3 quadrants required redo surgery. Mean follow-up was 5.2 years with resolution of symptoms in most. This study highlights the role of conservative management with good outcomes in children with TZ bowel pull-through having hypertrophic nerve fibers and normal ganglion pattern. Children who underwent Duhamel’s procedure had little impact with the presence of TZ at anastomotic margin and majority of those undergoing TERP benefitted from Botox injection. Conservative management can be attempted successfully to prevent redo surgical interventions as they can lead to poorer outcomes. Only those children not responding to conservative measures need to be planned for revision surgery.

To evaluate the psychosocial functioning of caregivers and patients with anorectal malformation (ARM), Hirschsprung disease (HD), spinal conditions, and idiopathic constipation (IC) during the beginning of participation in bowel management program (BMP). In this retrospective study, Parent Stress Scale (PSS, parent-report) and Strengths and Difficulties Questionnaire (SDQ, parent-proxy; SDQ-S, self-report for 11 years and older) were used to evaluate parental stress levels and behavioral functioning, respectively. Descriptive and comparative statistical approaches were applied to summarize the data and to determine differences in scores between diagnoses, gender, and developmental functioning. Two hundred patients and caregivers participated in BMP during the study period. PSS scores were significantly higher for caregivers of patients with IC than ARM. Statistical differences in SDQ were found for patients with IC versus those with ARM, male versus female patients, and patients with developmental delays versus those without delays. No significant differences were found in SDQ-S scores between these groups. Key findings suggest that level of parental stress and behavioral concerns were significantly influenced by diagnoses, and partly by gender and presence of developmental delay. Thus, psychosocial support may need to be tailored based on these findings to provide optimum quality of care for patients and families.

To compare the quality of life (QoL) in children with spina bifida with a control group of their peers using a validated questionnaire, the Neurogenic Bowel Dysfunction Score (NBDS). The NBDS questionnaire was prospectively distributed to children attending a multi-disciplinary Spina Bifida clinic and healthy controls attending pediatric urology clinics. A score (out of 41) was assigned to each child based on their responses to the validated questionnaire. A lower score indicates better bowel function-related quality of life. SPSS software (v.25) was used for all statistical analysis. There were 98 respondents to the questionnaire, 48 children with spina bifida and 50 controls. The average age of respondents was 7.88 years (3–16 years). Of those with Spina Bifida, 33 (69%) were on retrograde rectal irrigations, [19 (58%) Peristeen® system, 11 (33%) tube rectal irrigations, and 3 (9%) Willis system], 6 (12%) were on laxatives, and 9 (19%) were on no treatment. The median NBDS for Spina Bifida patients was significantly higher 13.5 (2–32) compared to the control group 2 (0–26, p

To define the practice of management for Hirschsprung disease (HD) in Australia and New Zealand.Online survey of Australian and New Zealand Association of Paediatric Surgeons (ANZAPS) members.56/80 (70%) members from 17 centres responded.100% perform suction rectal biopsies; 40% perform a contrast enema.HE (94%), ACHE (70%) and calretinin (75%).Primary pull-through (PT) is performed by 88% (100% by 6/12 months). The Soave-Boley PT is the preferred approach (85%), with laparoscopic assistance (77%) and muscle cuff division (93%). Routine post-operative dilatations are performed by 63% of respondents. If symptoms persist following PT, majority adopt a conservative approach (enemas/laxatives 90%; Botox 74%). If a long-segment is identified at PT, 60% fashion a stoma and delay definitive surgery. If total colonic aganglionosis is identified at PT, 76% fashion a stoma and delay definitive surgery. A dedicated bowel management program is available in 45% of centres with transition to adult services in 29%.A laparoscopic-assisted Soave-Boley PT is the most common technique for recto-sigmoid HD. Differences are noted in both the management of long-segment/total aganglionosis HD and post-operative management/follow-up.

Traditionally, the care of children and adults has been arbitrarily separated into pediatric and adult medicine and surgery. Despite progress in pediatric surgical techniques, patients born with congenital anomalies still suffer from significant functional sequelae, which persist into adulthood. We aim to describe some of the most common problems experienced by adult patients with congenital colorectal malformations. Following IRB approval, we performed a retrospective database review of all adult patients who were treated by our group from 1983 until 2017. We identified 88 cases. 51 patients had ARM, 18 cloacas, 9 presacral masses, 3 HD, 2 spina bifida and 5 with other diagnoses (3 vaginal anomalies, 1 cloacal exstrophy, 1 obstructed seminal vesical). The specific problems addressed were: complications from previous operations (41), rectal prolapse (25), fecal incontinence (11), gynecologic concerns (12), urologic concerns (6), and recurrent recto urogenital fistula (3). We performed 83 surgical interventions, including 13 rectal prolapse repair, 13 continent appendicostomies, 44 PSARP or redo PSARP, 11 resections of presacral masses, 11 vaginoplasties, 2 examinations under anesthesia, and 2 Mitrofanoff procedures. Five patients were treated medically (bowel management program, obstetric, urologic evaluation). There is a growing need to better prepare adult providers to assume the care of patients born with congenital colorectal disease as these patients transition to adulthood. A collaboration between specialized pediatric referral centers with adult colorectal surgeons, urologists and gynecologists is a potential pathway for the adequate transition of care.

Patients undergoing surgery for anorectal malformation (ARM) may have defecation-related problems throughout their lives, even if they are perfect treated surgically. Assessment methods are needed to standardize the clinical outcomes of patients with ARM. The aim of this study was to compare the scoring systems (SS) with the anorectal manometry (AM) findings. The data of patients operated on for ARM were examined. Holschneider’s, Rintala’s, Krickenbeck’s and Pena’s questionnaires were executed to the patients and AM was performed. Our study was completed with 23 patients. There was a statistically significant relationship between the anal resting pressure and Holschneider’s questionnaire (HQ). There was a statistically significant relationship between the area under the curve in the maximum voluntary squeeze pressure–time graph (AUC) and the HQ and Rintala’s questionnaire (RQ). A statistically significant difference was found between HQ and RQ scores and high type and low type of ARMs. In our study, based on AM data, it was found that the use of HQ and RQ from the four SS we compared could be more effective in patients’ follow-up. It was concluded that Pena’s questionnaire and Krickenbeck’s questionnaire should be used to determine the bowel management program of the patients rather than patients’ follow-up. Level II.

Although most children with Hirschsprung disease ultimately achieve functional and comfortable stooling, some will experience a variety of problems after pull-through surgery. The most common problems include soiling, obstructive symptoms, enterocolitis, and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative soiling in children with Hirschsprung disease. The American Pediatric Surgical Association Hirschsprung Disease Interest Group engaged in a literature review and group discussions. Expert consensus was then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with soiling symptoms following pull-through for Hirschsprung disease. Causes of soiling after pull-through are broadly categorized as abnormalities in sensation, abnormalities in sphincter control, and "pseudo-incontinence." A stepwise algorithm for the diagnosis and management of soiling after a pull-through for Hirschsprung disease is presented; it is our hope that this rational approach will facilitate treatment and optimize outcomes.

It has been postulated that children with Hirschsprung disease (HD) and mucosal eosinophilia have been thought to have poorer outcome, but supporting evidence is lacking. The objective of our study was to review the outcomes of children with HD and mucosal eosinophilia. A single center, retrospective review was conducted on all patients diagnosed with HD between 1999 and 2016. Pathology specimens were evaluated for mucosal eosinophilia. Demographics, complications, and outcomes were analyzed. A total of 100 patients were diagnosed with HD and 27 had mucosal eosinophilia. Median age at the time of surgery was 12 days (8, 30) and 82 were males. Comparing patients with HD with and without mucosal eosinophilia, there was no statistically significant difference in time to bowel function (2 days vs. 2 days; p = 0.85), time to start feeds (3 days vs. 3 days; p = 0.78) and time to goal feeds (5 days vs. 5 days; p = 0.47). There was no statistically significant difference in feeding issues (13% vs. 9%; p = 1.0) and stooling issues (60% vs. 50%; p = 0.38). There was no statistically significant difference in postoperative complications and readmissions rates (63% vs. 56%; p = 0.53). Hirschsprung-associated mucosal eosinophilia may not increase postoperative complications, and may not change feeding and bowel management. Further prospective studies are in process to evaluate long term follow-up outcomes for this patient population.

Our center has been successfully implementing a bowel management program (BMP) for fecal incontinence consecutive to anorectal malformation and Hirschsprung disease. Recently, the number of patients with spina bifida requiring management for fecal incontinence has increased. The purpose of this study was to review the results of bowel management in patients with spina bifida and the challenges unique to this population. A retrospective chart review was performed including all patients with spina bifida who attended our BMP from February 2016 until April 2018. Data collection included: prenatal intervention, gender, age, characteristics of contrast enema, success rateand challenges faced. Twenty-two patients met inclusion criteria 13 of which were females. Three patients had their myelomeningocele repaired prenatally, the remaining were repaired postnatally. Patient ages ranged from 2 to 24 years. Only nine patients were referred to BMP at proper toilet training age. Three patients came to BMP status post an antegrade enema procedure with reported “accidents” on their current regimen. The colon in the contrast enema was non-dilated in all patients and two behaved as hypermotile requiring loperamide. Seventeen patients (77%) were clean of stool and considered successful. Solution leakage during enema administration was the most common challenge and was corrected by increasing the Foley balloon fill volume. Our bowel management program with enemas is effective for patients with a history of spina bifida. The data support specific considerations for this population including frequent adjustments, close follow-up and specific administration techniques.

To analyze the outcome after untethering surgery in patients with spinal dysraphism (SD), with or without associated anorectal malformation (ARM). Patients operated on for SD, with (Group A) or without (Group B) associated ARM (1999–2015), were included. The post-operative outcome was analyzed in the two groups in terms of improving of clinical symptoms (neuro-motor deficits, bladder dysfunction, bowel dysfunction) and of instrumental examinations (urodynamics, bladder ultrasound, neurophysiology). Fisher’s exact test and χ2 test were used as appropriate; p

Sacral abnormalities range from missing the coccyx, a few sacral vertebrae, or hemi-sacrum, to complete absence with fused iliac bones. The purpose of this study was to review the association between sacral agenesis and fecal incontinence to help inform patient prognosis. A retrospective review was performed of patients who presented for bowel management due to sacral agenesis at a tertiary care children’s hospital between 2016 and 2017 (n = 10). Data collection included: gender, time of diagnosis, sacral ratio, and associated anomalies. Patients with anorectal malformation and sacrococcygeal teratomas were excluded. Four patients were female. Seven patients had a delayed diagnosis ranging from 22 months of age to 9 years. Most common symptoms included failure of age-appropriate toilet training and severe diaper rash. The sacral ratio was zero (6), 0.3 and 0.4 (2), and hemi-sacrum (2). Associated anomalies were present in five patients. Sacral abnormalities should be suspected in patients who present with early severe diaper rash and those who fail to toilet train. An abdominal radiograph can evaluate the sacrum, when the sacral ratio is 0.4 or less, parents should be counseled regarding fecal incontinence and neurogenic bladder.

Epididymo-orchitis (EO) is infrequently reported in anorectal malformation (ARM) cases. Therefore, it is difficult to assess its risk factors. A total of 110 male patients who were operated on for ARM at the same Institution over a period of 13 years were contacted. Association was assessed between EO and the following: spinal dysraphism (SD), symptomatic VUR (VUR), and bowel management (BM) requiring enemas. The data were analyzed with the Chi-square test. A total of 89 patients were contacted. Ten cases of EO were found, and all occurred in patients with recto-urethral (RU) fistula after reconstruction. The patients’ age at first episode ranged between 4 and 11 years. RU fistula patients experiencing EO (Group A, 10 patients) were compared with those without EO (Group B, 33 patients). VUR occurred in 9/10 cases in Group A and in 13/33 cases in group B (Chi-square 7.8658, p = 0.005038). SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). A total of 8/10 cases in Group A and 12/33 cases in Group B were on BM (Chi-square 5.87, p = 0.0015). EO occurs in approximately in 20 % of male cases with ARM, and recto-urinary communication and should be considered the primary diagnosis in the presence of testicular pain. This could avoid unnecessary surgical exploration, and the family should be counseled about this subject

Evaluate the impact of implementing Bowel Management Program (BMP) on quality of life (QoL) in children with fecal incontinence (FI). Children 3–12 years referred to the Children’s Treatment Center of FI were included and categorized accordingto the tendency to constipation (dilated colon; group 1) or diarrhea (non-dilated colon; group 2). Evaluations were performed before and after 3 months of BMP implementation. Perceptions from parents and children about QoL were assessed by the instrument PedsQL4.0 and the severity of FI by the fecal incontinence index (FII) of Wexner scale. 43 children (mean age of 7.7 ± 3.1) were studied. 32 (74.4 %) belonged to group 1 and 11 (25.6 %) to group 2. After 3 months of BMP, it was noticed a significant improvement of the FII (14.5 ± 3.7 versus 2.8 ± 2.5; p

Patients with anorectal malformation (ARM) frequently suffer postoperatively from fecal incontinence (25%) and constipation (75%). Depending on the type of malformation, some cases will not have a chance to control bowel movements. For these patients with fecal incontinence, we started to combine bowel management with hydrocolonic ultrasound to keep them clean.From January 2003 until December 2010, overall 63 patients (aged 4-22 years) with ARM and fecal incontinence were treated by specific bowel management. Hydrocolonic ultrasound was used as a diagnostic parameter to determine stool texture and activity of the colon as well as to determine the appropriate volume which is needed to clean the colon. Each patient received an individually adjusted enema. Patients were classified into two groups: Group I: patients with incontinence and tendency to constipation and Group II: patients with incontinence and tendency to diarrhea. Subsequent controls were focused on problems such as bowel dilatation, bowel motility, constipation or too little enema due to the patient's growing.Up to now, 63 patients were investigated in our study (Group I n = 37 patients and Group II n = 26 patients). In addition, the patients were classified according to the type of malformation. With specific bowel management combined with hydrocolonic ultrasound, 97% of patients in Group I (36/37) stayed clean (demonstration of complete bowel control). All 57.7% of patients in Group II (15/26) stayed clean after daily bowel management, and 34.6% with smearing less than three times per week (n = 9/26). In addition, a specific diet and constipating medication were often necessary to get patients in Group II clean.Treating young patients with fecal incontinence is always a challenge. Hydrocolonic ultrasound diagnostically conclusive and less invasive. The combination with bowel management results in better bowel control and serves as a valuable tool in affected infants and adolescents. Thus, hydrocolonic ultrasound may be an essential instrument in postoperative diagnostic procedure and therapy of patients with fecal incontinence.

Background/purpose: Anorectal malformations (ARM) are a spectrum of defects ranging from the very minor ones, with an excellent functional prognosis, to those that are more complex, often associated with other anomalies, difficult to manage and with a poor functional outcome. A significant number of these children suffer from fecal and urinary incontinence despite major advances in the management of ARM patients have significantly improved the quality of life. The role of sacrovertebral anomalies/dysraphism (SD) and neurospinal cord anomalies/dysraphism (ND) associated with ARM on the continence of these patients is still controversial. The authors made a review of their experience in a period of 5 years, focusing on the role of neurospinal cord anomalies in patients with ARM. Materials: At colorectal clinic of our department of pediatric surgery 215 patients who underwent a procedure of posterior sagittal anorectoplasty for ARM are followed-up in a multidisciplinary clinic. Among them 60 patients with either SD or ND were documented. In 37 patients the anomaly involved the spinal cord (ND). 12 of these 37 patients underwent neurosurgical treatment and 25 were managed conservatively. Data collected from their follow-up were analyzed and compared, focusing on their bowel and urinary continence. Results: All 37 patients acquired regular bowel movements with an appropriate bowel management according to Peña's protocols. Urinary incontinence required clean intermittent catheterization in four cases. None of the patients who did not receive neurosurgical treatment developed acute complications due to the progression of the neurospinal anomaly, like acute urinary retention, orthopedic and motility problems or acute hydrocephalus. From literature review we were unable to find good evidence that the presence of ND worsens the functional prognosis of patients with ARM. We were also unable to find convincing evidence to support the practice of prophylactic neurosurgical procedures. Conclusions: The present study supports the theory that for ARM patients the prognosis in terms of continence depends mainly on the type of malformation and is not complicated by the association with ND. In our series neurosurgical treatments did not have any effect in improving the continence of ARM patients and a conservative management of ND did not expose the patients to the sequelae of progressive deterioration, reported elsewhere, requiring rescue neurosurgery. We believe that the correct practice of pediatric surgeons following-up ARM patients is a protocol which includes appropriate investigations to detect the presence of a SD or ND and, once these entities are detected, it is mandatory to manage the patient with a multidisciplinary team, where a conservative non-operative management is initially justified and advocated in the absence of neurosensorymotor symptoms.

Single-stage transanal endorectal pull-through (TEPT) procedure is now widely used for rectosigmoid Hirschsprung’s disease (HD). However, this procedure is associated with some common complications like stricture, enterocolitis, constipation, anastomotic dehiscence and perianal excoriation. The aim of this study was to evaluate the clinical outcomes of a modified TEPT operation in the management of rectosigmoid HD. 45 patients with rectosigmoid HD admitted between August, 2004 and July, 2008 were included in this study. Pre-operative barium enema was done in all of them. Patients in whom the transition zone was well delineated were included in the study. Frozen section biopsy was done to confirm the presence of ganglion cells in the pull-through bowel. All odd number patients were included in group A, where conventional TEPT procedure was performed. All even number patients and the last five patients of the series were in group B. In this group, a modified transanal endorectal procedure was done. The initial part of submucosal dissection and mobilization of aganglionic rectum was the same as in the conventional procedure. The anorectal mucosa was anastomosed end-to-side to the seromuscular coat of the ganglionic bowel. Mobilized bowel was divided at about 2 cm distal to the anastomosis. Six hitching stitches were applied from the free edge of the bowel to the perianal skin so that the anastomosis remains outside the anal verge. The distal redundant bowel was excised on eighth post-operative day. Pre-operative bowel preparation was done in all patients in group A. The bowel preparation was not mandatory in group B patients. Post-operative results of both these groups were compared and analyzed using the Mann–Whitney U test. Mean operative time in group A was 86 min (range 75–95 min) versus 90 min (range 70–100 min) in group B. Average length of the bowel resected was 20 cm (range 10–26 cm) in group A and 22 cm (12–40 cm) in group B. Post-operative feeding was started after 48 h in group A and after 6 h in group B. Average hospital stay was about 8 days in both the groups. Median follow-up was 36 months in group A (range 14–54 months) and 32 months (range 12–52 months) in group B. Anastomotic stricture or stenosis was noted in two patients in group A and none in group B (p < 0.01). Partial anastomotic dehiscence was noted in two patients in group A and none in group B (p < 0.01). Average bowel movement in group A was 6–8 per day at 1 month and 2–6 per day at 6 months. In group B, it was 8–10 at 1 month and 2–8 at 6 months. Cuff narrowing causing constipation was noted in two patients in group A and none in group B (p < 0.01). Enterocolitis following pull-through was noted in two patients in group A and three patients in group B. Four patients in group A and one patient in group B had post-operative constipation. All of them responded to bowel management program: only one of them (group A) required enema; soiling was noted in one patient each in both the groups. Modified TEPT procedure is associated with excellent clinical outcome with fewer complications, and permits early post-operative feeding. Operating time, hospital stay and post-operative bowel movement were comparable with the conventional technique.

Anorectal malformations represent a continuing challenge for the paediatric surgeon. Faecal and urinary incontinence can occur even with an excellent anatomic repair. We undertook a prospective evaluation of comorbidity and psychosocial needs in children and adolescents with anorectal malformation to test the hypothesis whether psychosocial workup in concert with an improved continence situation would lead to a better functional outcome in patients suffering from defecating disorders. Treatment of defecating disorder was based on differentiation between overflow pseudo-incontinence and true faecal incontinence. Patients who presented with soiling regardless of the type of anomaly were included in the study: 44 patients were investigated, 14 patients did not meet the criteria of the study, 30 patients aged 4–18 were evaluated. The surgical diagnostic program comprised a careful clinical history, physical examination, exact classification of the malformation, evaluation for associated defects, stool protocol. Patients were grouped in two different management programs according to their continence situation. Patients with pseudo-incontinence were treated with enemas, diet, anal hygiene, behavioural therapy and physiotherapy. Patients with true faecal incontinence were included in a bowel management program: 28 patients were free of symptoms of soiling after 3 days of hospital treatment and remained free of symptoms 6 months and 1 year later at re-evaluation. Two patients did not follow the therapeutic regime and therefore did not show an improved condition concerning soiling in the long run. Differentiating between overflow pseudo-incontinence and true faecal incontinence is the basis of an effective treatment of defecating disorders. All patients born with anorectal malformation can be kept clean of stool if they are subjected to an adequate treatment. A multidisciplinary approach is a valuable supplement to standard therapy.

A major research gap is determining the best age to perform an appendicostomy or cecostomy. This study hypothesizes that performance of appendicostomy/cecostomy prior to starting school (6 years) would improve functional stooling and quality of life (QOL).Patients who underwent appendicostomy/cecostomy for bowel management between 2003 and 2013 were retrospectively identified. Families were prospectively surveyed regarding current stooling habits (17 items) and a (7 item) pediatric QOL survey. Lower stooling survey scores represent better bowel control. Higher QOL scores indicated better quality. The primary outcome was to correlate age of appendicostomy/cecostomy to QOL score. Statistics were performed using paired, unpaired t tests, and Chi-square. p Values ≤0.05 were considered significant.35 patients underwent placement of appendicostomy/cecostomy. Fourteen (40%) patients/families were prospectively contacted (6, n = 6;6, n = 8). Stooling scores (15.17 ± 1.35 vs. 22.25 ± 1.70; for6 vs.6 years old, p = 0.009) and continence scores (6.33 ± 1.45 vs. 11.13 ± 1.64; p = 0.06), at time of contacting families, were significantly better in those undergoing appendicostomy/cecostomy in the6 group. Pre-procedure QOL scores for the two groups were similar (p = 0.89). Post-procedure QOL significantly increased to the good subcategory for both age groups; however improvement was significantly better in the6 age group vs. ≥6 group: 6.33 ± 0.92 vs. 3.13 ± 0.91 points (p = 0.03). A secondary parent survey showed significantly more families wished an appendicostomy/cecostomy were done earlier in the6 vs.6 group (87.5 vs. 33%; p = 0.04).Early placement of cecostomy or appendicostomy as part of a bowel management program may contribute to improved QOL and functional stooling.

Fecal incontinence is a serious problem that may lead to social segregation and psychological problems. Patients with anorectal malformations frequently suffer from fecal incontinence even with an excellent anatomic repair. In these patients, an effective management program with enemas can improve their quality of life. We want to present our experience with hydrocolonic sonography as a diagnostic tool to predict the type and volume of enema needed to initiate an effective bowel management. Patients who presented with soiling regardless of the type of anomaly were included in the study. Thirty patients aged 4-18 were evaluated. The diagnostic program comprised a careful clinical history, physical examination, exact classification of the malformation, evaluation for associated defects, and stool protocol. Twenty patients suffered from true fecal incontinence and were included in a bowel management program. These patients received oral polyethyleneglycol to evacuate stool impaction. Bowel management was initiated with the help of hydrosonography to evaluate bowel motility. The volume of the enema was determined according to the amount of fluid that was needed to fill the colon to the cecum. Twenty patients were investigated with the help of hydrocolonic sonography. Eighteen patients were free of symptoms of soiling after 3 days of hospital treatment and remained free of symptoms 6 months and 1 year later at reevaluation. Two patients did not follow the therapeutic regime and, therefore, did not show an improved condition concerning soiling in the long run. Hydrocolonic sonography is a helpful diagnostic tool to assess colonic volume and motility to predict the type and volume of enema needed for an effective bowel management.

Fecal incontinence is a devastating underestimated problem, affecting a large number of individuals all over the world. Most of the available literature relates to the management of adults. The treatments proposed are not uniformly successful and have little application in the pediatric population. This paper presents the experience of 30 years, implementing a bowel management program, for the treatment of fecal incontinence in over 700 pediatric patients, with a success rate of 95%. The main characteristics of the program include the identification of the characteristics of the colon of each patient; finding the specific type of enema that will clean that colon and the radiological monitoring of the process.

Anterior sagittal anorectoplasty (ASARP) was used for the definitive correction in 107 cases of anovestibular fistula (AVF) between 1996 and 2005. These cases were subjected to three different types of treatment regimes during the same period. Majority of the cases (78) were operated in one stage where postoperatively an early oral feed was started (A). Cases were discharged in 2-4 days. In the second group (B), there were ten cases who were also operated in one stage but with prolonged fasting of 9-10 days postoperatively. Nineteen cases (C) were operated under cover of colostomy during the same period. In the immediate postoperative period, among the group A, one case had a major wound disruption requiring a colostomy and a redo surgery. Three cases had subcutaneous leak. In seven cases there was premature dehiscence of mucocutaneous or skin sutures. In groups B and C, there were no significant complications in the immediate postoperative period. In the follow-up period, out of 107 cases, 63 (58.8%) had constipation at the end of 3 months. However, at the end of one year, only 24.3% (26 cases) cases had constipation. Regarding fecal continence, 86 cases (90.5%) were totally continent. Seven had history of occasional soiling and in two cases, soiling was more frequent. As far as repair or correction of AVF or vestibular anus is concerned, we feel that anterior sagittal approach is more suitable as it requires less pelvic dissection. Separation of posterior vaginal wall from rectum, which is considered, is the most important step of the operation, takes place under direct vision. We also feel that AVF can be repaired in one stage with an early postoperative oral feed, provided we are meticulous in pre and postoperative bowel management. It reduces hospital stay and the cost of treatment. This provides a good option to cases who are not able to afford prolonged hospitalization (fasting) or are not willing for a colostomy.

The successful management of faecal incontinence can dramatically improve the quality of life of affected children. The introduction of the non-refluxing, catheterisable appendico-caecostomy provides the opportunity to treat previously resistant patients. Over a 6-year period, 29 children had a Malone antegrade continent stoma for enema administration (MACE). Incontinence was related to spina bifida in 12 children, ano-rectal anomaly in 12, Hirschprung's disease in 2, followed excision of a pelvic tumour in 2, and was secondary to intractable chronic constipation in 1. The conduit was fashioned from the appendix (20), a caecal tube (8), or a gastric tube (1). Surgical complications were stomal stenosis (11), wound infection (1), anastomotic leak (1), MACE stoma prolapse (1), and a pressure sore (1). Colonic irrigation was achieved with washouts of saline (24), saline plus phosphate (4), and saline plus Picolax (1). Twenty-three patients have complete control of bowel function, but 4 still soil. Two remain incontinent, 1 of whom is still being instructed. One child subsequently had a colostomy, but still uses the MACE stoma. Successful bowel management requires motivation, dedication, commitment, and the input of a clinical nurse specialist. The MACE is a relatively straightforward operative procedure that provides an effective washout technique that is acceptable to both parents and children.

We report our experience of electrical stimulation and biofeedback exercise of pelvic floor muscle for children with faecal incontinence after surgery for anorectal malformation (ARM). Electrical stimulation and biofeedback exercise of pelvic floor muscle were performed on children with post-operative faecal soiling following repair of intermediate or high type ARM. Children under the age of 5 years or with learning difficulties were excluded. They had 6 months supervised programme in the Department of Physiotherapy followed by 6 months home based programme. Bowel management including toilet training, dietary advice, medications and enemas were started before the pelvic floor muscle exercise and continued throughout the programme. Soiling frequency rank, Rintala continence score, sphincter muscle electromyography (EMG) and anorectal manometry were assessed before and after the programme. Wilcoxon signed rank test was performed for statistical analysis. From March 2001 to May 2006, 17 children were referred to the programme. Twelve patients (M:F = 10:2; age = 5-17 years) completed the programme. There was a trend of improvement in Rintala score at sixth month (p = 0.206) and at the end of programme (p = 0.061). Faecal soiling was significantly improved at sixth month (p = 0.01) and at the end of the programme (p = 0.004). Mean sphincter muscle EMG before treatment was 1.699 microV. Mean EMG at sixth month and after the programme was 3.308 microV (p = 0.034) and 3.309 microV (p = 0.002) respectively. After the programme, there was a mean increase in anal sphincter squeeze pressure of 29.9 mmHg (p = 0.007). Electrical stimulation and biofeedback exercise of pelvic floor muscle is an effective adjunct for the treatment of faecal incontinence in children following surgery for anorectal malformation.