Conference proceedings: Inaugural meeting of the consortium for autism, genetic neurodevelopmental disorders, and digestive diseases.

OBJECTIVES: Individuals with neurodevelopmental disorders (NDDs), including autism spectrum disorder (ASD), often experience a higher prevalence of gastrointestinal (GI) symptoms but have complex medical and behavioral comorbidities that make diagnosis and treatment difficult. A multi-stakeholder conference was convened to (a) determine patient and family experiences related to GI symptoms in NDDs, (b) review the clinicians' and researchers' perspectives, and (c) determine actionable steps for future research. METHODS: The Consortium for Autism, Neurodevelopmental Disorders and Digestive Diseases (CANDID; www.candidgi.com) virtually over 2 days in 2022 and consisted of four key activities: (1) an electronic family survey to assess underlying NDDs and GI symptoms, (2) a session focused on family perspectives, (3) review current clinical care and research, and (4) discussion to identify key next steps. Survey results were obtained electronically via the REDCap platform, and descriptive statistics were generated. The sessions were recorded, and themes were identified. RESULTS: The pre-conference survey ran for ~2 months and 739 families provided responses, with 634 completing all items. 83% had a child with an NDD under age 18, and most patients were White (85%) and non-Hispanic (87%). Constipation (80%), gastrointestinal reflux disease (51%), and bloating (49%) were the most frequently reported symptoms. Families gave unstructured feedback that the measures used in the surveys were often difficult to answer for patients with NDDs or who were nonspeaking. Family and clinical/scientific sessions identified several common themes, including (1) the need for less invasive diagnostic modalities, (2) the need to validate or adapt existing diagnostic measures (e.g., the Rome IV criteria) and outcome assessments, and (3) the need for enhanced attention to parent and caregiver input in treatment plans. CONCLUSIONS: Those providing care to children with NDDs, especially those with communication and cognitive challenges, should be aware of the differing needs in this community and consider family perspectives in managing, treating, and measuring GI issues. Future research should focus on adapting or creating diagnostic and research measures for those with NDDs, developing new diagnostic methods to account for diversity in neurodevelopment and communication, and improving methods for family and caregiver engagement in the care of GI disorders.

A multicenter registry study on percutaneous electrical nerve field stimulation for pediatric disorders of gut-brain interaction.

OBJECTIVES: Percutaneous electrical nerve field stimulation (PENFS) has demonstrated promise in single-center trials for pediatric abdominal pain-related disorders of gut-brain interaction (DGBI). Our aim was to explore efficacy of PENFS as standard therapy for DGBI in a registry involving multiple pediatric gastroenterology referral centers. METHODS: This was a multicenter, prospective open-label registry of children (8-18 years) undergoing PENFS for DGBI at seven tertiary care gastroenterology clinics. DGBI subtypes were classified by Rome IV criteria. Parents and patients completed Abdominal Pain Index (API), Nausea Severity Scale (NSS), and Functional Disability Inventory (FDI) questionnaires before, during therapy and at follow-up visits up to 1 year later. RESULTS: A total of 292 subjects were included. Majority (74%) were female with median (interquartile range [IQR]) age 16.3 (14.0, 17.7) years. Most (68%) met criteria for functional dyspepsia and 61% had failed ≥4 pharmacologic therapies. API, NSS, and FDI scores showed significant declines within 3 weeks of therapy, persisting long-term in a subset. Baseline (n = 288) median (IQR) child-reported API scores decreased from 2.68 (1.84, 3.58) to 1.99 (1.13, 3.27) at 3 weeks (p < 0.001) and 1.81 (0.85, 3.20) at 3 months (n = 75; p < 0.001). NSS scores similarly improved from baseline, persisting at three (n = 74; p < 0.001) and 6 months later (n = 55; p < 0.001). FDI scores displayed similar reductions at 3 months (n = 76; p = 0.01) but not beyond. Parent-reported scores were consistent with child reports. CONCLUSIONS: This large, comprehensive, multicenter registry highlights efficacy of PENFS for gastrointestinal symptoms and functionality for pediatric DGBI.

Inadequate Bowel Preparation in Pediatric Colonoscopy-Prospective Study of Potential Causes.

OBJECTIVES: Inadequate bowel preparation (IBP) for colonoscopy leads to missed diagnosis, longer anesthesia time, higher chance of complications and increased costs. Adult studies have demonstrated that patient characteristics such as male gender and obesity are associated with IBP. Little is known about factors affecting bowel preparation in children. Our aim was to determine factors associated with IBP in children. METHODS: We prospectively enrolled children undergoing outpatient colonoscopy. Quality of bowel preparation was assessed using Boston Bowel Preparation Scale (BBPS) score (range 0-9). Data collected included patient demographics, indication, and type of insurance. Patients were divided into two groups based on BBPS score-adequate (BBPS score > 5) and inadequate (BBPS score < 5) and groups were compared using Student t-test and chi-square test. Possible predictors were analyzed using multivariate logistic regression models. RESULTS: A total of 334 children were prospectively enrolled of whom 321 were studied further (age range 2-18 years; mean age 12.4 years; 60.4% female; 85.9% Caucasian). The mean BBPS score was 6.8 (standard deviation of ±2). IBP was reported in 12.8% (41/321). Multivariable logistic regression analysis did not show statistical differences between the groups in studied patient factors including age, gender, obesity, race, insurance type, and indication for colonoscopy. CONCLUSION: Contrary to several adult studies, the results of our prospective study did not show any relationship between examined patient factors and IBP in children. Interestingly, IBP was less prevalent in our pediatric study compared to published adult data (12.8% vs 20-40%).

Clinical factors and high-resolution manometry predicting response to surgery for achalasia in children.

BACKGROUND: Esophageal achalasia is an uncommon condition in children. Although many interventions exist for the management of this disorder, esophageal (Heller) myotomy offers one of the most durable treatments. Our institution sought to review patients undergoing Heller myotomy concentrating on preoperative clinical factors that might predict postoperative outcomes. MATERIALS AND METHODS: All patients from January 1, 2007, to December 31, 2016, who underwent surgical treatment for achalasia at our tertiary pediatric hospital were identified and included in the study cohort. Electronic medical records for these patients were reviewed for clinical presentation variables, nonsurgical preoperative treatment, surgical approach, clinical response to surgery, need for postoperative treatment for ongoing symptoms, and high-resolution manometry (HRM) data. RESULTS: Twenty-six patients were included in the study, and all underwent myotomy with partial fundoplication (median age: 14.4 y [interquartile range 11.6-15.5]). At a median follow-up of 9.75 mo (interquartile range 3.5-21 mo), 16 (61.5%) patients reported good resolution of their dysphagia symptoms with surgery alone. Two patients (7.7%) had perforation of the gastrointestinal tract requiring surgical intervention. Eight patients (30.8%) required additional treatment for achalasia, with 5 (19.2%) of these undergoing additional surgery or endoscopic treatment. Patients who had preoperative dilation did not have good resolution of their dysphagia (n = 2; P = 0.037). Two of four patients undergoing postoperative dilation had preoperative dilation. None of these patients underwent preoperative manometry. There was a statistically significant difference in the ages of patients who required postoperative intervention and those who did not (14.1 versus 15.2 y old, respectively; P = 0.043). In patients who reported improvement of gastroesophageal reflux disease/reflux type symptoms after Heller myotomy, lower esophageal residual pressure (29.1 versus 18.7 mmHg; P = 0.018) on preoperative HRM was significantly higher than in those who did not report improvement after surgery. Higher upper esophageal mean pressure (66.6 versus 47.8 mmHg; P = 0.05) also predicted good gastroesophageal reflux disease/reflux symptom response in a similar manner. CONCLUSIONS: Current analysis suggests that preoperative dilation should be used cautiously and older patients may have a better response to surgery without need for postoperative treatment. In addition, preoperative HRM can aid in counseling patients in the risk of ongoing symptoms after surgery and may aid in determining if a fundoplication should be completed at the index procedure. Further research is needed to delineate these factors. LEVEL OF EVIDENCE: Level III.

Incidence of Low Seroimmunity to Hepatitis B Virus in Children With Inflammatory Bowel Disease.

OBJECTIVES: Patients with inflammatory bowel disease (IBD) often receive immunosuppressive therapy, which may make them vulnerable to infections such as hepatitis B. We hypothesized that hepatitis B virus titers are low in the vaccinated pediatric population with IBD. The aims of our study were to identify the incidence of lower titers of hepatitis B surface antibody (HBsAb) and determine which patient factors may be associated with lower HBsAb titers. METHODS: Patients with diagnosis of IBD, ages 5 to 18 years, were prospectively enrolled. Patients were confirmed to have had a full series of hepatitis B vaccination. Quantitative serum HBsAb titers were measured and logistic regression analysis with independent variables of age, sex, race, disease phenotype, surgery, medications and a dependent variable of adequate HBsAb titers (> 10 mIU/mL) was performed. RESULTS: Of the 116 patients enrolled, 57 were boys and 59 were girls. 75 patients had a diagnosis of Crohn disease; 32 had a diagnosis of ulcerative colitis; and 9 patients had been diagnosed as having indeterminate colitis. At the time of the study, 15 patients were taking corticosteroid, 66 on an immunomodulator, and 53 on a biologic. Sixty percent of patients in the 5- to 10-year age group had protective titers versus 22% to 27% in the older groups, P = 0.04. Only 28% of the 116 patients had HBsAb titers of >10m IU/mL. Twenty percent of the patients taking corticosteroids, 27% taking immunomodulators, and 24% taking biologics were found to be seroimmune. CONCLUSIONS: Nearly two-thirds of pediatric patients with IBD have low titers against hepatitis B virus. Titers were highest in the younger patients. No patient-specific variable, such as the use of immunosuppressants, appeared to influence these low titers.

Effect of Octreotide on Colonic Motility in Pediatric Patients With Chronic Recalcitrant Constipation.

OBJECTIVE: The aim of the present study was to study the effect of octreotide on colonic motility in pediatric patients with recalcitrant chronic constipation/encopresis and other suspected colonic motility disorders. METHODS: This was a nonrandomized, single-center, open-label, prospective study evaluating the effect of a single subcutaneous dose of octreotide on colonic motility. RESULTS: Thirteen patients (5 boys) were enrolled in the study. The age range was 4.6 to 16.2 years. Eleven patients (84%) had normal colonic manometry and 2 patients (16%) had colonic neuropathy. Motility Index (MI) (mmHg) for the 15 minutes before and after octreotide infusion was 6.03 ±â€Š1.26 (95% confidence interval [CI] 5.35-6.72) and 5.32 ±â€Š1.66 (95% CI 4.42-6.23), respectively, with P value of 0.08. MI for the 30 minutes before and after octreotide infusion was 6.89 ±â€Š1.37 (95% CI 6.14-7.64) and 6.71 ±â€Š1.47 (95% CI 5.91-7.52), respectively, with P value of 0.55. MI for the 45 minutes before and after octreotide infusion was 7.73 ±â€Š1.32 (95% CI 7.01-8.45) and 7.53 ±â€Š1.38 (95% CI 6.78-8.28), respectively, with P value of 0.8. CONCLUSION: Our study showed that the administration of octreotide resulted in no significant changes in colonic MI in pediatric patients with chronic recalcitrant constipation.

Neuroenteric Staining as a Tool in the Evaluation of Pediatric Motility Disorders.

The diagnosis of enteric neuromuscular disorders has come a long way since the first description of an enteric neuropathic disorder by the Danish physician Harald Hirschsprung in 1886. Advances in specialized enteric histopathological staining techniques have made it possible to identify subtle neuropathies and myopathies that cause intestinal motility disorders, from the common and now better understood and relatively easily diagnosed Hirschsprung's disease to the less common and more severe and not well-characterized chronic idiopathic intestinal pseudoobstruction, which continues to present a diagnostic challenge to the gastroenterologist and histopathologist alike. This article will discuss the common gastrointestinal motility disorders and some of the specialized histological stains, such as the relatively common enzyme stain, acetylcholinesterase, used to diagnose Hirschsprung's disease; advanced tinctorial stains, such as Masson trichrome, which may aid in diagnosis of enteric myopathies causing pseudoobstruction; and immunohistochemical stains such as C-Kit or PG 9.5, which may aid in the diagnosis of enteric neuropathies causing pseudoobstruction.

Vomiting.

Vomiting can be the presenting symptom of a variety of disorders, ranging from self-limited diseases to life-threatening diseases. The causes of vomiting vary with age of presentation, and pediatricians should develop the skill to identify serious conditions at the earliest stage based on the age of presentation. Bilious emesis at any age is a sign of intestinal obstruction until proven otherwise and needs immediate attention. Vomiting is not always due to a GI disorder, and pediatricians should look for causes outside the GI tract if no GI disease is identified.

Can colonic manometry studies be done on the day of colonic motility catheter placement?

BACKGROUND AND AIMS: Colonic manometry has been used to assess colonic neuromuscular integrity in pediatric patients with severe constipation unresponsive to standard medical therapy and to tailor their treatment plans. There are presently no available standard protocols for conducting colonic manometry studies. The aim of the present study was to determine whether colonic manometry studies can be conducted on the same day the colonic motility catheters are placed and to compare the effects of inhaled sevoflurane versus intravenous propofol, used during catheter placement, on colonic motility. METHODS: Twenty patients, randomized to receive sevoflurane or propofol during catheter placement, underwent colonic manometry on the day of catheter placement as well as the day after. The total motility index (MI), change in MI in response to a meal and bisacodyl, and presence of high-amplitude propagating contractions were compared between the 2 studies for each patient. RESULTS: Ten patients were allocated to sevoflurane and 10 patients to propofol. A total of 8 (80%) patients in the sevoflurane group and 9 (90%) patients in the propofol group had no differences in their studies between days 1 and 2 when the tracings were interpreted manually for gross evidence of high-amplitude propagating contractions and gastrocolonic reflex. Similarly, there was no change in the total MI between studies done on days 1 and 2 in either sevoflurane (978 ± 232 vs 978 ± 184; P = 0.99) or propofol (968 ± 200 vs 1078 ± 227; P = 0.29) group. When comparing change in MI in response to a meal or bisacodyl between the 2 days, there was no statistical difference noted in either group. CONCLUSIONS: Colonic manometry studies can be conducted as early as 4 hours following catheter placement with either propofol or sevoflurane used for anesthesia.

Esophageal pH monitoring with the BRAVO capsule: experience in a single tertiary medical center.

AIM: The aim of the present study was to report the global experience with placement, complication rate, and recording of esophageal pH using the BRAVO capsule at our institution. PATIENTS AND METHODS: We recorded the rate of any technical problems and complications during placement in all of the patients (ages 4-22 years) who received this device during a 2-year period. All of the patients undergoing esophagogastroduodenoscopy with the placement of BRAVO pH capsule were included in this analysis. We also examined the pH data recorded on days 1 and 2 for significant day-to-day variability during 2 days of pH monitoring. RESULTS: Two hundred eighty-nine BRAVO pH probes were placed from January 1, 2006 to December 31, 2008. At least 1 day of data was obtained in 278 patients (96.2%). Two days of data were obtained in 274 patients (94.8%). Of all of the reported complications, 1% occurred before deployment of the capsule, 4% occurred during deployment of the capsule, and 9% occurred after successful deployment of the capsule. One patient experienced a superficial esophageal tear that was associated with failure of the capsule to release from the delivery system. No patient requested removal of the capsule and all of the capsules detached within 14 days. In 9.12% of our patients, reflux index was normal on day 1 and abnormal on day 2. There was no statistically significant difference between reflux index recorded on day 1 versus day 2 (P = 0.686). CONCLUSIONS: The BRAVO pH capsule is easy to place, safe, and well tolerated by children. Performing a 48-hour study detected abnormal reflux in an additional 9% of our patients.

Exploring potential noninvasive biomarkers in eosinophilic esophagitis in children.

BACKGROUND AND AIMS: Eosinophilic esophagitis (EE) continues to present clinical challenges, including a need for noninvasive tools to manage the disease. To identify a marker able to assess disease status in lieu of repeated endoscopies, we examined 3 noninvasive biomarkers, serum interleukin (IL)-5, serum eosinophil-derived neurotoxin (EDN), and stool EDN, and examined possible correlations of these with disease phenotype and activity (symptoms and histology) in a longitudinal study of children with EE. SUBJECTS AND METHODS: Children with EE were studied for up to 24 weeks (12 weeks on 1 of 2 corticosteroid therapies and 12 weeks off therapy). Twenty children with normal esophagogastroduodenoscopies with biopsies were enrolled as controls. Serum IL-5, serum EDN, and stool EDN were measured at weeks 0, 4, 12, 18, and 24 in children with EE, and at baseline alone for controls. Primary and secondary statistical analyses (excluding and including outlier values of the biomarkers, respectively) were performed. RESULTS: Sixty subjects with EE (46 [75%] boys, mean age 7.5 ±â€Š4.4 years) and 20 normal controls (10 [50%] boys, mean age 6.7 ±â€Š4.1 years) were included. Significant changes in serum EDN (significant decrease from baseline to week 4, and then rebound from week 4 to week 12) occurred. Serum EDN levels were stable after week 12. Serum IL-5 and stool EDN levels in subjects with EE were not statistically different from those of the control subjects when each time point for the cases was compared with the controls' 1-time measurement. CONCLUSIONS: Serum EDN levels were significantly higher in subjects with EE than in controls, and the results suggest a possible role, after additional future studies, for serum EDN in establishing EE diagnosis, assessing response to therapy, and/or monitoring for relapse or quiescence.

Pediatric bowel preparation: Sodium picosulfate, magnesium oxide, citric acid vs polyethylene glycol, a randomized trial.

BACKGROUND: Bowel preparation in children can be challenging. AIM: To describe the efficacy, safety, and tolerability of sodium picosulfate, magnesium oxide, and citric acid (SPMC) bowel preparation in children. METHODS: Phase 3, randomized, assessor-blinded, multicenter study of low-volume, divided dose SPMC enrolled children 9-16 years undergoing elective colonoscopy. Participants 9-12 years were randomized 1:1:1 to SPMC ½ dose × 2, SPMC 1 dose × 2, or polyethylene glycol (PEG). Participants 13-16 years were randomized 1:1 to SPMC 1 dose × 2 or PEG. PEG-based bowel preparations were administered per local protocol. Primary efficacy endpoint for quality of bowel preparation was responders (rating of 'excellent' or 'good') by modified Aronchick Scale. Secondary efficacy endpoint was participant's tolerability and satisfaction from a 7-item questionnaire. Safety assessments included adverse events (AEs) and laboratory evaluations. RESULTS: 78 participants were randomized, 48 were 9-12 years, 30 were 13-16 years. For the primary efficacy endpoint in 9-12 years, 50.0%, 87.5%, and 81.3% were responders for SPMC ½ dose × 2, SPMC 1 dose × 2, and PEG groups, respectively. Responder rates for 13-16 years were 81.3% for SPMC 1 dose × 2 and 85.7% for PEG. Overall, 43.8% of participants receiving SPMC 1 dose × 2 reported it was 'very easy' or 'easy' to drink, compared with 20.0% receiving PEG. Treatment-emergent AEs were reported by 45.5% of participants receiving SPMC 1 dose × 2 and 63.0% receiving PEG. CONCLUSION: SPMC was an efficacious and safe for bowel preparation in children 9-16 years, with comparable efficacy to PEG. Tolerability for SPMC was higher compared to PEG.

Hypertrophic pyloric stenosis in an adolescent.

Hypertrophic pyloric stenosis presenting beyond infancy is an uncommon occurrence. The etiology of adult onset pyloric stenosis is unknown. In this case report, we describe a 14-year-old boy who presented with nausea, intermittent vomiting, and abdominal pain. He underwent upper gastrointestinal series, which demonstrated persistent narrowing of the pylorus. He was referred to Pediatric Surgery for evaluation and subsequently underwent laparoscopic pyloroplasty. Postoperatively, the patient did well, and at 20 months, he continues to be asymptomatic.

Comparison of oral prednisone and topical fluticasone in the treatment of eosinophilic esophagitis: a randomized trial in children.

BACKGROUND & AIMS: Although eosinophilic esophagitis is recognized increasingly, outcome data guiding therapy are limited. We conducted a prospective randomized trial comparing oral prednisone (P) and swallowed fluticasone (F) for histologic and clinical response. METHODS: Patients were randomized to receive P or F for 4 weeks, followed by an 8-week weaning protocol. Esophageal histology was evaluated at baseline and after 4 weeks of therapy. Clinical assessments were performed at weeks 0, 4, 12, 18, and 24. RESULTS: Eighty patients with eosinophilic esophagitis were enrolled: 40 in the P arm and 40 in the F arm. Histologic improvement was seen in 30 of 32 P and 34 of 36 F patients, with a greater degree of histologic improvement in the P group. All P and 35 of 36 F patients were free of presenting symptom(s) at week 4. Symptom relapse was seen in 45% of patients by week 24. Kaplan-Meier analysis showed no difference between P and F with regard to relapse rate (P = .7399). No significant difference in time to relapse was found between groups (P = .2529). Systemic adverse effects were noted in 40% of the P arm, whereas esophageal candidal overgrowth was seen in 15% of the F arm. CONCLUSIONS: Systemic and topical corticosteroids were effective in achieving initial histologic and clinical improvement. P resulted in a greater degree of histologic improvement, without evidence of an associated clinical advantage over F in terms of symptom resolution, relapse rates, or time to relapse. Symptom relapse was common to both groups upon therapy discontinuation, highlighting the need for maintenance treatment protocols.

Accuracy and tolerability of the Bravo catheter-free pH capsule in patients between the ages of 4 and 18 years.

OBJECTIVE: The aim of this study was to determine if the Bravo pH capsule is comparable to the nasally placed pH catheter in terms of pH-metry, safety, and tolerability in children. METHODS: Ten patients each in the age ranges of 4 to 6 years, 7 to 10 years, and >10 years were tested simultaneously with the catheter and the capsule. Six each were tested with the catheter alone or the capsule alone. Subjects recorded adverse events and graded tolerance (in terms of activity, appetite, and satisfaction) on a scale of 1 to 5, with a score of 5 indicating that the device was well tolerated. A 24-hour reflux index and 24- and 48-hour reflux indices were generated from the catheter and capsule, respectively. Student t test, Mann-Whitney U test, and Fisher exact test were used to compare reflux index, tolerability, and adverse events between the catheter and capsule. RESULTS: Sixty-six patients 4 to 16 years of age (mean, 9.4 years) were enrolled. There was no statistically significant difference between the mean reflux indices (RIs) obtained simultaneously with the catheter and capsule in all patients combined on day 1 (P = 0.0665). There was a significant difference between day 2 and days 1 and 2 combined with the capsule versus the catheter (P = 0.007 and P = 0.0107); however, a discordant result of normal RI on day 1 and pathological RI on day 2 was seen in only 1 patient. The capsule was better tolerated than the catheter in terms of appetite (P = 0.029), activity (P = 0.001), and satisfaction (P = 0.003). There were no significant complications. CONCLUSIONS: The Bravo pH capsule was as accurate and safe and better tolerated than the conventional pH catheter in children 4 years of age and older.

At what age is a suction rectal biopsy less likely to provide adequate tissue for identification of ganglion cells?

OBJECTIVE: The objective of this study was to determine at what age suction rectal biopsy is less likely to provide adequate tissue to detect submucosal ganglion cells in a child being evaluated for Hirschsprung disease. PATIENTS AND METHODS: Children > or =1 year of age undergoing a rectal biopsy at a single children's hospital had 1 biopsy each obtained simultaneously with a suction biopsy device and a grasp biopsy forceps. The biopsies were examined by 2 pathologists for adequacy of the submucosa (none, scant, adequate, or ample) and the presence of ganglion cells. The 2 specimens were compared with each other. RESULTS: One hundred fifty-two children 1 to 17 years of age were included. Fifty-three were female. Subjects were grouped into 4 age categories: 1 to 3 years (group A), 4 to 6 years (group B), 7 to 9 years (group C), and > or =10 years (group D). Similar numbers of patients were recruited for each group. Ganglion cells were identified in 73% and 90% by the suction and grasp devices, respectively, in group A. In groups B through D, ganglion cells were identified in 50% to 53% vs 92% to 97% of the suction and grasp biopsies, respectively (P < 0.001). Submucosa was present in 88% (suction) vs 98% (grasp) in group A, 70% vs 95% in group B, 69% vs 94% in group C, and 45% vs 92% in group D. CONCLUSION: The suction rectal biopsy is less likely to provide adequate submucosa for identification of ganglion cells after 3 years of age.

Contemporary short- and long-term outcomes in patients with unremitting constipation and fecal incontinence treated with an antegrade continence enema.

PURPOSE: The primary aim of this study is to determine the natural history of patients who undergo an antegrade continence enema (ACE) procedure including complications, functional results, and long-term outcomes. METHODS: Patients aged 3-18years who underwent an ACE procedure from 2008 to 2015 for unremitting constipation and fecal incontinence with at least thirty day follow-up were included. Patients with congenital anatomic disorders of the spine, rectum, and anus were excluded. RESULTS: A total of 93 patients were included in the analysis with an average age of 10+/-4years and follow-up of 26+/-41months. The ACE procedure was rapidly effective, with 99% of patients experiencing improvement at 1month. At the end of follow-up, 83% of patients had normal bowel function, and 95% of patients noted improvement. Amongst patients with at least 24months of follow-up (n=51), 43% successfully stopped using their ACE at an average of 40+/-27months. Overall morbidity was 55%, mostly related to minor complications. However, 13% of patients required an additional operation. CONCLUSION: The ACE procedure is very successful in the treatment of unremitting constipation with fecal incontinence in appropriately selected patients. LEVEL OF EVIDENCE: Level IV.

Assessment of the effectiveness of biofeedback in children with dyssynergic defecation and recalcitrant constipation/encopresis: does home biofeedback improve long-term outcomes.

The purpose of this study was to determine whether biofeedback benefits children with dyssynergic defecation and constipation/encopresis, and whether home biofeedback improves long-term outcomes. Thirty-six patients with chronic constipation who had failed at least 6 months of conventional treatment and demonstrated dyssynergic defecation at anorectal manometry were randomized to biofeedback in the laboratory alone (group 1, n=24) or in the laboratory and at home (group 2, n=12) and followed up at 2, 4, and a mean of 44 months. Thirty patients were available for long-term follow-up. Bowel movements increased in all from a mean of 1.4/week to 5.1, 5.8, and 5.1 per week at 2 months, 4 months, and long-term, respectively (p < or = 0.001). Soiling decreased in all from a mean of 5.5/week to 0.6, 0.1, and 1 per week at 2 months, 4 months, and long-term, respectively (p < or = 0.001). Laxative use decreased from a mean of 4.1 days/week to 0.6, 0.3, and 0.7 per week at 2 months, 4 months, and long-term, respectively (p < or = 0.001). Twenty-seven of 30 parents ranked their satisfaction a mean of 2.2 (range 1-excellent to 3-good). There were no significant differences in outcomes between the laboratory alone group and the laboratory plus home group. Biofeedback is beneficial for some children with chronic constipation and dyssynergic defecation. Supplemental home biofeedback does not improve long-term outcomes.