Pediatric Aerodigestive Medicine: Advancing Collaborative Care for Children With Oropharyngeal Dysphagia.

OBJECTIVES: Aerodigestive disorders encompass various pathological conditions affecting the lungs, upper airway, and gastrointestinal tract in children. While advanced care has primarily occurred in specialty centers, many children first present to general pediatric gastroenterologists with aerodigestive symptoms necessitating awareness of these conditions. At the 2021 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the aerodigestive Special Interest Group held a full-day symposium entitled, Pediatric Aerodigestive Medicine: Advancing Collaborative Care of Children with Aerodigestive Disorders. The symposium aimed to underline the significance of a multidisciplinary approach to achieve better outcomes for these complex patients. METHODS: The symposium brought together leading experts to highlight the growing aerodigestive field, promote new scientific and therapeutic strategies, share the structure and benefits of a multidisciplinary approach in diagnosing common and rare aerodigestive disorders, and foster multidisciplinary discussion of complex cases while highlighting the range of therapeutic and diagnostic options. In this article, we showcase the diagnostic and therapeutic approach to oropharyngeal dysphagia (OPD), one of the most common aerodigestive conditions, emphasizing the role of a collaborative model. CONCLUSIONS: The aerodigestive field has made significant progress and continues to grow due to a unique multidisciplinary, collaborative model of care for these conditions. Despite diagnostic and therapeutic challenges, the multidisciplinary approach has enabled and greatly improved efficient, high-quality, and evidence-based care for patients, including those with OPD.

Demographics of children with feeding difficulties from a large electronic health record database.

BACKGROUND: Feeding difficulties are among the most common concerns expressed by parents in younger children. However, few studies have reported on the characteristics of patients with clinically significant feeding diagnoses. The aim of the current study is to describe the characteristics of patients diagnosed with feeding difficulties including concurrent conditions, age, and sex, sampled nationwide utilizing the Cerner Health Facts Database. METHODS: We identified patients with a diagnosis of feeding difficulties (ICD-9 783.3 or ICD-10 R63.3), age 7 months to 17 years, with an outpatient visit between 2010 and 2017. The demographics and complex clinical conditions of this population were categorized. The cohort was then collapsed into a matrix defining recognized phenotype codes for ICD-9 and ICD-10 diagnoses to identify associated conditions. RESULTS: We identified 39,674 patients (0.95%) representing 101,684 encounters from 68 health systems across the United States; 43% of patients were female. Gastrointestinal conditions were the most common, followed by malnutrition, developmental and behavioral diagnoses, and neurologic conditions. CONCLUSIONS: This study is one of the most robust studies defining the prevalence, demographic characteristics, and phenotypic profiling of patients with feeding difficulties. Our observations have implications on screening and resource allocation to recognize and manage this poorly understood population.

Mucosal eosinophils, mast cells, and intraepithelial lymphocytes in youth with rumination syndrome.

BACKGROUND: Rumination syndrome has been associated with increased duodenal eosinophils and intraepithelial lymphocytes in adults. The aims of the current study were to assess densities of antroduodenal eosinophils and mast cells and duodenal intraepithelial lymphocytes in youth with rumination syndrome and to compare cell densities in those with and without abdominal pain or early satiety. METHODS: Twenty-eight youth fulfilling Rome IV criteria for rumination syndrome who had undergone endoscopy were identified and compared to 10 controls. Antral and duodenal biopsies were assessed to determine densities of eosinophils, mast cells, and intraepithelial lymphocytes. Cell densities were also compared between rumination patients with and without abdominal pain and those with and without early satiety. KEY RESULTS: Antral mast cell (peak 18.5±6.5 vs. 12.5±2.7) and eosinophil (peak 9.6±5.2 vs. 4.9±2.1) densities were significantly greater in patients with rumination syndrome as compared to controls. Duodenal intraepithelial lymphocyte densities were also increased in rumination syndrome (18.9 ± 5.1 vs. 11.7 ± 1.5; p<.001). Associations were independent of the presence of abdominal pain or early satiety. CONCLUSIONS AND INFERENCES: In conclusion, we found an increase in eosinophil and mast cell densities in the gastric antrum and an increase in intraepithelial lymphocytes in the duodenum in youth with rumination syndrome which was independent of the presence of abdominal pain or early satiety. These findings suggest a potential role for inflammation in the pathophysiology of rumination syndrome. Future studies should address whether treatment directed at these cells are beneficial in treating rumination syndrome.

iKanEat: protocol for a randomized controlled trial of megestrol as a component of a pediatric tube weaning protocol.

BACKGROUND: Although tube feeding routinely saves the lives of children who do not eat by mouth, chronic tube feeding can be a burden to patients, caregivers, and families. Very few randomized trials exist regarding the best methods for weaning children from their feeding tubes. METHODS: The current paper describes a randomized controlled trial of an empirically supported outpatient treatment protocol for moving children from tube to oral eating called iKanEat. Specifically, we describe the methods of randomized double-blind, placebo-controlled trial which includes a 4-week course of megestrol, the only medication used in the iKanEat protocol, to determine whether the addition of megestrol results in improved child outcomes. The primary and secondary aims are to assess the safety and efficacy of megestrol as part of the iKanEat protocol. The third aim is to provide critical information about the impact of the transition from tube to oral feeding on parent stress and parent and child quality of life. DISCUSSION: This trial will provide data regarding whether megestrol is a safe and effective component of the iKanEat tube weaning protocol, as well as important data on how the tube weaning process impacts parent stress and parent and child quality of life. TRIAL REGISTRATION: ClinicalTrials.gov NCT#03815019 . Registered on January 17, 2019.

Proceedings of the 2018 Advances In Motility and In NeuroGastroenterology: AIMING for the Future Single Topic Symposium.

OBJECTIVES: Motility and functional disorders are common in children and often debilitating, yet these disorders remain challenging to treat effectively. At the 2018 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the Neurogastroenterology and Motility Committee held a full day symposium entitled, 2018 Advances In Motility and In NeuroGastroenterology - AIMING for the future. The symposium aimed to explore clinical paradigms in pediatric gastrointestinal motility disorders and provided a foundation for advancing new scientific and therapeutic research strategies. METHODS: The symposium brought together leading experts throughout North America to review the state of the art in the diagnosis and management of motility and functional disorders in children. Presentations were divided into esophageal, antral duodenal, and colorectal modules. Each module included oral presentations by experts in the respective fields, leading to thought-provoking discussions. There were 2 breakout sessions with small group discussions on select topics, focusing on defining scientific insights into the diagnosis and management of pediatric functional gastrointestinal and motility disorders in a systematic, segment-based approach. CONCLUSIONS: The field of neurogastroenterology has made remarkable progress in the last decade. The current report summarizes the major learning points from the symposium highlighting the diagnosis and promising therapies on the horizon for pediatric neurogastrointestinal and motility disorders.

A comparison of the diagnosis of gastroparesis in 4 h pediatric gastric emptying studies versus 2 h studies.

BACKGROUND: In adults, there is a consensus for standards to diagnose gastroparesis utilizing a gastric emptying study as the key diagnostic modality but there is no consensus for a standard in pediatrics. Additionally, some cost savings might be achieved if symptoms could be utilized to predict patients with gastroparesis. The aims of the current study were to confirm the sensitivity of a 4 h study in the pediatric population and to assess whether the severity of symptoms were predictive of delayed gastric emptying. STUDY: This was a single site, two part study. In the first part, results were reviewed for all patients who had completed a 4-h, solid gastric emptying study over the course of a 3 year period. In the second portion of the study, participants scheduled for a gastric emptying study, completed a modified GCSI questionnaire. RESULTS: Out of a total of 109 participants, at 2 h, 14 participants (12.8%) had abnormal studies as compared to 26 (23.85%) participants who had abnormal studies at 4 h (p = .0027). Of the 95 participants with normal studies at 2 h, 15% (14/95) were abnormal at 4 h. There were no differences in symptom severity scores between those with slow and those with normal emptying at either 2 h or 4 h. CONCLUSIONS: Our study adds independent confirmation that extending studies from 2 to 4 h increases the diagnostic yield and should be the standard in children and adolescents as it is in adults.

Is histologic esophagitis associated with dental erosion: a cross-sectional observational study?

BACKGROUND: Gastroesophageal reflux disease (GERD) affects 15-25% of children and adolescents in the United States. The diagnosis of GERD in children is complex as reported symptoms or symptom profiles have been found to be unreliable. Frequently, the diagnosis must be confirmed by objective tests such as pH monitoring or histological evidence of esophagitis on an esophageal biopsy. Dental erosion has been shown to be associated with GERD as an atypical complication and has the potential to be a marker of GERD. The purposes of this study were to compare the frequency and patterns of dental erosion in children and adolescents with and without histologic esophagitis. METHODS: Twenty-five subjects were recruited from patients scheduled for an upper gastrointestinal endoscopy. Information regarding potential GERD symptoms, food habits, and dental hygiene habits were obtained. Intra-oral photographs were taken, and a dental exam for erosion was performed. The results of a standard biopsy taken from the lower third of the esophagus during an endoscopy were used to divide subjects into either the control group or the GERD group (i.e. those with histologic esophagitis). RESULTS: Twenty-two subjects yielded 586 evaluable teeth. No significant difference was found between frequency or erosion patterns of those with and without histologic esophagitis. Dental erosions were more frequent in primary teeth. CONCLUSIONS: Dental erosions do not appear to be associated with histologic esophagitis indicative of GERD.

A Randomized Controlled Trial of an Outpatient Protocol for Transitioning Children from Tube to Oral Feeding: No Need for Amitriptyline.

OBJECTIVE: To assess the role of amitriptyline in the effectiveness of an outpatient protocol for weaning medically complicated children from tube to oral feeding. STUDY DESIGN: Twenty-one children seen in multidisciplinary outpatient feeding teams across 4 sites were recruited to a randomized placebo-controlled trial of a 6-month outpatient treatment protocol with behavioral, oral-motor, nutrition, and medication components. RESULTS: All of the children who completed the 6-month program (73%) were weaned to receive only oral feeding, regardless of group assignment. The transition from tube to oral feeding resulted in decreases in body mass index percentile and pain, some improvements in quality of life, and no statistically significant changes in cost. CONCLUSIONS: Amitriptyline is not a key component of this otherwise effective outpatient, interdisciplinary protocol for weaning children from tube to oral feeding. TRIAL REGISTRATION: ClinicalTrials.gov: NCT01206478.

Acute Recurrent Pancreatitis in Children: A Study From the Pediatric Health Information System.

We investigated acute recurrent pancreatitis (ARP) in children using a national health care database. From 2002 to 2014, 26,435 children had a diagnosis of acute pancreatitis (AP); 10,648 discharges were index hospitalizations. A total of 6159 children had a single hospitalization for AP, whereas 4489 (42%) children underwent 15,787 rehospitalizations. Children experienced a median of 2 ARP-related hospitalizations with a median time between admissions of 86 days. Younger patients with a more severe index episode of AP were at a higher risk of ARP. ARP-related hospitalizations had an increased requirement for intensive care unit care compared with an index episode of AP.

Caring for Tube-Fed Children: A Review of Management, Tube Weaning, and Emotional Considerations.

Enteral nutrition is the practice of delivering nutrition to the gut either orally or through a tube or other device. Many children are reliant on enteral feedings to either supplement their nutrition or as a complete source of their nutrition. Managing children on tube feedings requires a team of providers to work through such dilemmas as feeding schedules, weaning from tube feeding, sensory implications of tube feeding, treatment of pain or nausea associated with eating, oral-motor issues, and behavioral issues in the child and family. The purpose of the current review is to summarize the multidisciplinary aspects of enteral feeding. The multidisciplinary team consists of a variable combination of an occupational therapist, speech-language pathologist, gastroenterologist, psychologist, nurse, pharmacist, and dietitian. Children who have minimal oral feeding experience and are fed via a nasogastric or gastrostomy tube often develop oral aversions. Limited data support that children with feeding disorders are more likely to have sensory impairment and that early life pain experiences contribute to feeding refusal. There are inpatient and outpatient programs for weaning patients from tube feeding to eating. The parent-child interaction is an important part of the assessment and treatment of the tube-fed child. This review also points out many information gaps, including data on feeding schedules, blenderized tube feedings, the best methods for weaning children off enteral feedings, the efficacy of chronic pain medications with tube-fed children, and, finally, the necessity of the assessment of parental stress among all parents of children who are tube fed.

Effect of colon transection on spontaneous and meal-induced high-amplitude--propagating contractions in children.

BACKGROUND: After Hirschsprung disease (HD) surgery, many children experience fecal incontinence caused by increased number of high-amplitude-propagating contractions (HAPCs) through the neorectum to the anal verge. The aim of this study was to determine whether children with HD have more HAPCs than children with colon transections for reasons other than HD. METHODS: We reviewed 500 colon manometries. Children (age 7.6 ±â€Š5.1 years, 275 boys) with functional constipation (n = 237, age 7.4 ±â€Š5.0 years, 126 boys) and chronic abdominal pain (n = 48, age 9.8 ±â€Š5.8 years, 25 boys) served as controls compared with subjects with HD (n = 56, age 6.9 ±â€Š4.1 years, 44 boys) and colon transection for other reasons (n = 24, age 6.1 ±â€Š5.8 years, 12 boys). We excluded 139 subjects without HAPCs. We documented HAPCs during 1-hour fasting and 1-hour postprandial. Results are in mean ±â€ŠSD. RESULTS: During fasting, HD subjects had more HAPCs (2.2 ±â€Š3.4/hour) versus functional constipation (0.8 ±â€Š2.2/hour, P = 0.0004) and chronic pain (0.5 ±â€Š1.1/hour, P = 0.001), but not more than colon transection (1.9 ±â€Š3.2/hour, P = 1.0). HD showed more postprandial HAPCs (4.0 ±â€Š5.4/hour) than functional constipation (1.5 ±â€Š2.5/hour, P < 0.0001) and chronic pain (0.9 ±â€Š1.6/hour, P < 0.0001), but not more than colon transection (2.4 ±â€Š3.0/hour, P = 0.6). There were more HAPCs fasting and postprandial after colon transection (1.9 ±â€Š3.2/hour and 2.4 ±â€Š3.0/hour) than functional constipation (0.8 ±â€Š2.2/hour, P = 0.3 and 1.5 ±â€Š2.5/hour, P = 1.0) and chronic pain (0.5 ±â€Š1.1/hour, P = 1.0 and 0.9 ±â€Š1.6, P = 1.0). HD subjects were divided by chief complaint: fecal incontinence or constipation. HD subjects with incontinence (n = 23) only had more HAPCs fasting (P = 0.01) and postprandial (P = 0.01) than HD subjects with constipation (n = 28) only. CONCLUSIONS: Increased HAPCs followed colon transection, regardless of a cause. HD subjects with incontinence had more HAPCs than subjects with colon transection for other reasons.

Visceral hypersensitivity and electromechanical dysfunction as therapeutic targets in pediatric functional dyspepsia.

Functional gastrointestinal disorders (FGID) are common clinical syndromes diagnosed in the absence of biochemical, structural, or metabolic abnormalities. They account for significant morbidity and health care expenditures and are identifiable across variable age, geography, and culture. Etiology of abdominal pain associated FGIDs, including functional dyspepsia (FD), remains incompletely understood, but growing evidence implicates the importance of visceral hypersensitivity and electromechanical dysfunction. This manuscript explores data supporting the role of visceral hypersensitivity and electromechanical dysfunction in FD, with focus on pediatric data when available, and provides a summary of potential therapeutic targets.

Aggressive juvenile polyposis in children with chromosome 10q23 deletion.

Juvenile polyps are relatively common findings in children, while juvenile polyposis syndrome (JPS) is a rare hereditary syndrome entailing an increased risk of colorectal cancer. Mutations in BMPR1A or SMAD4 are found in roughly half of patients diagnosed with JPS. Mutations in PTEN gene are also found in patients with juvenile polyps and in Bannayan-Riley-Ruvalcaba syndrome and Cowden syndrome. Several previous reports have described microdeletions in chromosome 10q23 encompassing both PTEN and BMPR1A causing aggressive polyposis and malignancy in childhood. These reports have also described extra-intestinal findings in most cases including cardiac anomalies, developmental delay and macrocephaly. In this report we describe a boy with a 5.75 Mb deletion of chromosome 10q23 and a 1.03 Mb deletion within chromosome band 1p31.3 who displayed aggressive juvenile polyposis and multiple extra-intestinal anomalies including macrocephaly, developmental delay, short stature, hypothyroidism, atrial septal defect, ventricular septal defect and hypospadias. He required colectomy at six years of age, and early colectomy was a common outcome in other children with similar deletions. Due to the aggressive polyposis and reports of dysplasia and even malignancy at a young age, we propose aggressive gastrointestinal surveillance in children with 10q23 microdeletions encompassing the BMPR1A and PTEN genes to include both the upper and lower gastrointestinal tracts, and also include a flowchart for an effective genetic testing strategy in children with juvenile polyposis.

Colon manometry proves that perception of the urge to defecate is present in children with functional constipation who deny sensation.

BACKGROUND AND OBJECTIVE: Children with functional constipation often state an inability to sense an urge to defecate and/or inability to feel incontinence. We used colon manometry to assess whether there was a sensory abnormality in patients who denied sensation. METHODS: A physician observed all of the colon manometries in the preceding 20 years, and included behavioral observations in the procedure reports. We reviewed the charts of these patients. RESULTS: Of 150 subjects with normal manometry and a diagnosis of functional constipation, 56 volunteered that they had no urge to defecate or complained of abdominal pain. For all who denied sensation, the first high-amplitude propagating colonic contraction (HAPC) was associated with retentive posturing and facial grimaces. When queried, all reported they felt nothing. The examiner explained the HAPC was causing pain, and informed the child that the pain would resolve if they defecated. With subsequent HAPCs, every patient acknowledged an urge to defecate and successfully defecated. Most agreed that a similar pain sensation was present daily, but was misinterpreted to be abdominal pain. CONCLUSIONS: Colon manometry may be useful not only for objective findings to discriminate neuromuscular disease from functional symptoms but also to understand psychological issues and aid in helping the child and family understand the maladaptive behaviors in functional constipation.

Parental illness encouragement behavior among children with functional gastrointestinal disorders: a factor analysis with implications for research and clinical practice.

The objective of the current study was to assess the factor structure of the Illness Behavior Encouragement Scale (IBES) by Walker and Zeman (1992) among children with functional gastrointestinal disorders (FGIDs). Two hundred seventy nine children (63 % female), and 135 primary caregivers (90.8 % mothers), recruited from a large Midwestern children's hospital completed the IBES, a 12-item measure of parental behavior in response to abdominal pain episodes. Findings suggested the IBES possesses two conceptually distinct scales that are invariant across parent self- and child-report, and are consistent with previous factor analysis in a Dutch sample of children with headaches. Different types of parental behaviors exist that naturally cluster and diverge in reliable ways. Future research is warranted to determine if these different types of parental behavior may differentially influence illness outcomes among children with FGIDs.

Diffuse esophageal spasm in children referred for manometry.

Diffuse esophageal spasm (DES) causes chest pain and/or dysphagia in adults. We reviewed charts of 278 subjects 0 to 18 years of age after esophageal manometry to describe the frequency and characteristics of DES in children. Patient diagnoses included normal motility (61%), nonspecific esophageal motility disorder (20%), DES (13%, n=36), and achalasia (4%). Of patients with DES, the most common chief complaint was food refusal in subjects younger than 5 years (14/24, 58%) and chest pain in subjects older than 5 years (4/12, 33%). Comorbid medical conditions, often multiple, existed in 33 subjects. DES should be considered when young children present with food refusal.

Sleep problems and functional disability in children with functional gastrointestinal disorders: an examination of the potential mediating effects of physical and emotional symptoms.

BACKGROUND: Sleep disturbances are increasingly recognized as a common problem for children and adolescents with chronic pain conditions, but little is known about the prevalence, type, and impact of sleep problems in pediatric functional gastrointestinal disorders (FGIDs). The objectives of the current study were two-fold: 1) to describe the pattern of sleep disturbances reported in a large sample of children and adolescents with FGIDs; and, 2) to explore the impact of sleep by examining the inter-relationships between sleep disturbance, physical symptoms, emotional problems, and functional disability in this population. METHODS: Over a 3-year period, 283 children aged 8-17 years who were diagnosed with an FGID and a primary caretaker independently completed questionnaires regarding sleep, emotional functioning, physical symptoms, and functional disability during an initial evaluation for chronic abdominal pain at a pediatric tertiary care center. A verbal review of systems also was collected at that time. Descriptive statistics were used to characterize the pattern of sleep disturbances reported, while structural equation modeling (SEM) was employed to test theorized meditational relationships between sleep and functional disability through physical and emotional symptoms. RESULTS: Clinically significant elevations in sleep problems were found in 45% of the sample, with difficulties related to sleep onset and maintenance being most common. No difference was seen by specific FGID or by sex, although adolescents were more likely to have sleep onset issues than younger children. Sleep problems were positively associated with functional disability and physical symptoms fully mediated this relationship. Emotional symptoms, while associated with sleep problems, evidenced no direct link to functional disability. CONCLUSIONS: Sleep problems are common in pediatric FGIDs and are associated with functional disability through their impact on physical symptoms. Treatments targeting sleep are likely to be beneficial in improving physical symptoms and, ultimately, daily function in pediatric FGIDs.

Utility of pH/multichannel intraluminal impedance probe in identifying operative patients in infants with gastroesophageal reflux disease.

INTRODUCTION: Gastroesophageal reflux is a common clinical problem in infants, but identifying which infants may benefit from a fundoplication remains a conundrum. Esophageal pH and multichannel intraluminal impedance (MII) measurements are useful diagnostic tools in adults and older children, but their diagnostic efficacy in infants is unclear. Therefore, we reviewed our experience with the combined pH/MII probe in this population. SUBJECTS AND METHODS: A retrospective review of patients ≤ 6 months of age who were studied with the pH/MII probe from 2006 to 2010 was performed. Test results, interventions, and outcomes were reviewed. Patients were divided into operative and nonoperative groups, and pH probe and MII results were compared. RESULTS: Fifty-seven patients (53% male) were identified. Mean age at the time of pH/MII probe was 3.1 months. Regarding the operative group (n = 33), 21% had an abnormal pH probe, and 100% had an abnormal MII; 97% had symptom improvement at a mean follow-up of 16 months (range, 0.4-38 months). In the nonoperative group (n = 24), 29% had an abnormal pH probe, and 100% had an abnormal MII. There was no significant difference in reflux index, Boix-Ochoa score, or percentage of acid and non-acid reflux between the two groups. CONCLUSIONS: The pH and MII studies are poor indicators of pathologic reflux in infants and do not adequately discern which patients will benefit from fundoplication.

Accuracy of pain recall in children.

BACKGROUND AND AIM: Chronic abdominal pain (AP) is common in children. Recall of symptoms is used clinically to determine management, to assess treatment progress, and in drug studies to assess outcomes. Limited data exist on accuracy of AP recall in children. The aim of the present study was to assess ability to accurately recall AP in children. METHODS: The study was a secondary analysis of data obtained from a double-blind, randomized, placebo-controlled trial, evaluating amitriptyline in children with functional gastrointestinal disorders. Children ages 8 to 17 years with AP predominant functional gastrointestinal disorders based on Rome II criteria were recruited from 6 centers. Those with evidence of organic disease were excluded. Patients maintained AP diary daily for 1 month (presence, frequency, and intensity). At the end of the study, patients reported the number of days of AP during previous month. Agreement between daily pain reports and recalled pain was assessed. Univariate analysis was conducted with Spearman rank correlations. RESULTS: We recruited 63 children (45 girls, mean age 12.8 years). Sixteen percent children had perfect agreement on number of days of AP. Fifty-four percent of children recalled fewer episodes of pain. The average number of days with AP by recall was 17.7/month, whereas by diary it was 23.5/month (P = 0.001). Correlation between patient recall of the last week of symptoms (r = 0.47) was no better than correlation between recall of the last 30 days of symptoms (r = 0.48). On comparing AP recall versus various pain intensities, reported AP did not reflect only AP of greater severity. Higher correlation of recall of symptoms was seen in children 11 years or younger (r = 0.59) as compared with children older than 11 years (r = 0.26). CONCLUSIONS: Few children can accurately recall the episodes of AP. Children commonly recall a lower frequency of AP than that assessed by prospective diary reports. Reported recall does not reflect a shorter recollection period. Recall is not related to intensity of pain. Adolescents have worse recall of symptoms.

Colonic hyperactivity results in frequent fecal soiling in a subset of children after surgery for Hirschsprung disease.

BACKGROUND AND AIM: Fecal soiling is a challenging problem in some children after pull-through surgery for Hirschsprung disease (HSCR). The prevailing perception is that soiling results from overflow incontinence; however, its treatment with laxatives yields mixed results. Colonic manometry studies are reported to be normal in most patients in this population. The interpretation of these findings does not support the physiology of fecal overflow incontinence in these children. The aim of the present study was to define the physiology underlying daily, frequent fecal soiling in children after surgery for HSCR using manometric techniques. PATIENTS AND METHODS: Four pediatric motility centers in the United States participated in the study; medical records and manometric tracings (anorectal and colonic) of children (n = 59; 6.5 years; 48 boys) who had pull-through surgery for HSCR and presented with daily, frequent fecal soiling were examined. Children referred for evaluation of constipation who had normal colonic manometry served as controls (n = 25; 6.7 years; 12 boys). The patients with HSCR were divided into 2 groups (Hirschsprung disease groups 1 and 2 [HD1, HD2]) based on the absence or presence of high-amplitude propagated contractions (HAPCs). A control group that included children with chronic constipation was also studied. We compared the mean HAPC frequency between the HD2 and control groups. RESULTS: HD1 included 21 patients who had no HAPCs in fasting or postprandial periods. HD2 included 38 patients who had an average of 0.07 HPACs/min while fasting and 0.13/min in the postprandial state. In this subset the number of HAPCs in the fasting state (P = 0.04) and the postprandial state (P < 0.001) was greater when compared with controls. Additionally, there was a significant increase in HAPCs/min from the fasting to the postprandial state (P = 0.01). In the HD2 group 40% had colonic hyperactivity. CONCLUSIONS: Daily, frequent fecal soiling after pull-through surgery for HSCR may be due to colonic hyperactivity in some children. It is imperative that this unique subset be identified because the management strategy would include avoidance of laxatives, contrary to standard current practice.