Persistent and newly developed gastrointestinal symptoms after surgery for intestinal malrotation in children: Dysmotility or disorders of gut and brain interaction?

OBJECTIVES: Surgery for intestinal malrotation (IM) aims to correct the defect and improve symptoms; however, many have persistent gastrointestinal (GI) symptoms postoperatively. We evaluated the incidence, clinical presentation, and long-term outcomes of children with surgically repaired IM and its possible association with disorders of gut and brain interaction (DGBI). METHODS: Multicenter retrospective study was conducted in patients from 0 to 21 years old, who had surgery for IM from 2000 to 2021 across three pediatric tertiary care centers. Data analyzed included demographics, time to diagnosis, idiopathic diagnosis, incidental diagnosis, postoperative follow-up, surgical time, and the need for surgery including bowel detorsion. Outcome variables were the presence of postoperative GI symptoms and DGBIs, and overall resolution of symptoms. We also evaluated the potential association of demographics and other included variables with our outcome variables. RESULTS: Ninety-two patients with surgically corrected IM were included, 54% were male, and median age of diagnosis and surgical correction was 4.9 and 7.8 months, respectively. Median follow-up after surgery was 64 months. A total of 77% had postoperative GI symptoms, and notably, 78% of patients without symptoms before surgery (incidental diagnosis) developed GI symptoms postoperatively and 27% of patients met Rome IV criteria for a one or more DGBI. No factors were associated to the presence of postoperative symptoms or DGBIs in multivariate analysis. Female gender was the only factor associated with lack of resolution of symptoms at follow-up. CONCLUSION: Pediatric IM is commonly associated with postoperative GI symptoms and DGBI well beyond surgery. An increased awareness about the prevalence of DGBI in these patients may help reach a prompt and accurate diagnosis, and improve their quality of life.

The Clinical Utility of Antroduodenal Manometry in the Evaluation of Children with Upper Gastrointestinal Symptoms.

OBJECTIVES: Antroduodenal manometry (ADM) measures antral and small bowel motility and is clinically used to evaluate upper gastrointestinal (UGI) symptoms. We aimed to evaluate its utility in guiding treatment, predicting response, and association with clinical findings. METHODS: Retrospective review of 200 children undergoing ADM. ADM interpretation and parameters were compared to outcomes (response to first therapy after ADM and overall response), predominant symptom (group A, abdominal distention and/or vomiting and group B, abdominal pain and/or nausea), etiology (idiopathic or with known comorbidity), and ADM indication [suspected chronic intestinal pseudo-obstruction (CIPO) or unexplained UGI symptoms]. RESULTS: We found an association between a normal intestinal phase III of the migrating motor complex (MMC) and idiopathic etiology, group B symptoms and unexplained UGI symptoms. No variable was associated with initial successful response. However, normal small bowel phase III of the MMC and idiopathic etiology were associated with overall successful response to treatment (including feeding tolerance and weaning of parenteral nutrition). No antral ADM parameter was associated with outcomes or other comparisons. The time to overall successful treatment response was significantly shorter in patients with a normal ADM and presence of a normal phase III of the MMC. CONCLUSIONS: The presence of the phase III of the MMC was the single ADM parameter predictive of overall treatment response, also associated to group B symptoms and idiopathic etiology. Our findings suggest that small bowel ADM parameters are more useful to predict outcomes and ADM should be performed primarily in patients presenting with abdominal distention and/or vomiting and those being evaluated for CIPO.

Hirschsprung's disease associated enterocolitis: A comprehensive review.

Hirschsprung's disease (HSCR) is a congenital disorder characterized by failure of the neural crest cells to migrate and populate the distal bowel during gestation affecting different lengths of intestine leading to a distal functional obstruction. Surgical treatment is needed to correct HSCR once the diagnosis is confirmed by demonstrating the absence of ganglion cells or aganglionosis of the affected bowel segment. Hirschsprung's disease associated enterocolitis (HAEC) is an inflammatory complication associated with HSCR that can present either in the pre- or postoperative period and associated with increased morbidity and mortality. The pathogenesis of HAEC remains poorly understood, but intestinal dysmotility, dysbiosis and impaired mucosal defense and intestinal barrier function appear to play a significant role. There is no clear definition for HAEC, but the diagnosis is primarily clinical, and treatment is guided based on severity. Here, we aim to provide a comprehensive review of the clinical presentation, etiology, pathophysiology, and current therapeutic options for HAEC.

Clinical Utility of Ileal Motility in Children With Defecation Disorders and Children With Chronic Intestinal Pseudo-Obstruction.

BACKGROUND: Little is known about ileal motility patterns and their utility in children. Here, we present our experience with children undergoing ileal manometry (IM). METHODS: A retrospective review of children with ileostomy comparing IM between 2 groups: A [chronic intestinal pseudo-obstruction (CIPO)] and B (feasibility of ileostomy closure in children with defecation disorders). We also compared the IM findings with those from antroduodenal manometry (ADM), and evaluated the joint effect of age, sex, and study indication group on IM results. RESULTS: A total of 27 children (median age 5.8 years old, range 0.5-16.74 years, 16 were female) were included (12 in group A and 15 in group B). There was no association between IM interpretation and sex; however younger age was associated with abnormal IM ( P = 0.021). We found a significantly higher proportion of patients with presence of phase III of the migrating motor complex (MMC) during fasting and normal postprandial response in group B than in group A ( P < 0.001). Logistic regression analysis revealed that only Group B was associated with normal IM ( P < 0.001). We found a moderate agreement for the presence of phase III MMC and postprandial response between IM and ADM (kappa = 0.698, P = 0.008 and kappa = 0.683, P = 0.009, respectively). CONCLUSION: IM is abnormal in patients with CIPO and normal in patients with defecation disorders, suggesting that IM may be not needed for ostomy closure in those with defecation disorders. IM has a moderate agreement with ADM and could be used as a surrogate for small bowel motility.

Clinical utility of colonic low-amplitude propagating contractions in children with functional constipation.

BACKGROUND: Colonic high-amplitude propagating contractions (HAPC) are generally accepted as a marker of neuromuscular integrity. Little is known about low-amplitude propagating contractions (LAPCs); we evaluated their clinical utility in children. METHODS: Retrospective review of children with functional constipation undergoing low-resolution colon manometry (CM) recording HAPCs and LAPCs (physiologic or bisacodyl-induced) in three groups: constipation, antegrade colonic enemas (ACE), and ileostomy. Outcome (therapy response) was compared to LAPCs in all patients and within groups. We evaluated LAPCs as potentially representing failed HAPCs. KEY RESULTS: A total of 445 patients were included (median age 9.0 years, 54% female), 73 had LAPCs. We found no association between LAPCs and outcome (all patients, p = 0.121), corroborated by logistic regression and excluding HAPCs. We found an association between physiologic LAPCs and outcome that disappears when excluding HAPCs or controlling with logistic regression. We found no association between outcome and bisacodyl-induced LAPCs or LAPC propagation. We found an association between LAPCs and outcome only in the constipation group that cancels with logistic regression and excluding HAPCs (p = 0.026, 0.062, and 0.243, respectively). We found a higher proportion of patients with LAPCs amongst those with absent or abnormally propagated (absent or partially propagated) HAPCs compared to those with fully propagated HAPCs (p = 0.001 and 0.004, respectively) suggesting LAPCs may represent failed HAPCs. CONCLUSIONS/INFERENCES: LAPCs do not seem to have added clinical significance in pediatric functional constipation; CM interpretation could rely primarily on the presence of HAPCs. LAPCs may represent failed HAPCs. Larger studies are needed to further validate these findings.

Pediatric Neurogastroenterology and Motility Disorders: What Role Does Endoscopy Play?

Although pediatric neurogastroenterology and motility (PNGM) disorders are prevalent, often debilitating, and remain challenging to diagnose and treat, this field has made remarkable progress in the last decade. Diagnostic and therapeutic gastrointestinal endoscopy emerged as a valuable tool in the management of PNGM disorders. Novel modalities such as functional lumen imaging probe, per-oral endoscopic myotomy, gastric-POEM, and electrocautery incisional therapy have changed the diagnostic and therapeutic landscape of PNGM. In this review, the authors highlight the emerging role of therapeutic and diagnostic endoscopy in esophageal, gastric, small bowel, colonic, and anorectal disorders and disorders of gut and brain axis interaction.

Diverting Ileostomy in Children With Functional Constipation: A Study Evaluating the Utility of Colon Manometry.

OBJECTIVES: Pediatric functional constipation (FC) may require invasive evaluations [like colon manometry (CM)] and surgical interventions [including diverting ostomy (DO)]. We evaluated the utility of CM in guiding surgery after DO. METHODS: Children with medically refractory FC undergoing an ostomy were included. Institutional Review Board approval was obtained for this retrospective study. Demographics and CM variables [high amplitude propagating contractions (HAPCs)] were recorded. Outcome measures: response to ostomy closure defined as successful if no need for further surgery after ostomy closure, and improvement on baseline CM after ostomy. A CM-guided ostomy closure algorithm was developed based on previous studies. We evaluated the association between response to ostomy closure and demographics, ostomy indication and CM improvement, and evaluated the role of CM predicting response using algorithm. RESULTS: A total of 60 children underwent ostomy for FC (median age: 7.1 years, range 0.15-23.6 years, 50% female). Ostomy was closed in 30 patients and deemed successful in 23 of 30. CM was performed in 42 of 60 patients before ostomy and in 29 of 30 before ostomy closure. We found no association between ostomy outcome and age, gender, weight, imaging studies, follow-up time, time with ostomy, HAPCs, and CM improvement. We found an association between failed response and ostomy indication of antegrade colonic enemas (ACE) failure ( P = 0.026) and successful response when ostomy closure was guided by algorithm ( P = 0.03). CONCLUSIONS: DO is a useful intervention in selected children with medically refractory FC, improving colon motility in most. CM can successfully guide the timing and type of ostomy closure. Larger studies are needed to further validate our findings.

Evaluation of Gastroesophageal Reflux Disease in Children on the Autism Spectrum: A Study Evaluating the Tolerance and Utility of the BRAVO Wireless pH Monitoring.

OBJECTIVES: Children on the autism spectrum disorder (ASD) may express pain or discomfort through stereotypic or self-injurious behaviors. Gastroesophageal reflux disease (GERD) may be challenging to diagnose in a child who is non-verbal or has impaired communication skills, diagnostic testing for GERD may be the only way to establish the diagnosis. We report our experience using the BRAVO wireless pH monitoring device for the evaluation of GERD in this patient population. METHODS: Tolerance and feasibility as well as pH parameters and symptom correlation of the BRAVO pH were evaluated retrospectively in ASD children and compared it to a large cohort of non-ASD children. Only patients with studies lasting >24 hours were included. RESULTS: A total of 172 patients were included, 27 of those were diagnosed with autism (median age 11 years, 17 male). We found no difference in age and weight between both groups but there was a male predominance in the autism group ( P = 0.007). We found no difference in the ability to complete at least 24 hours of study duration between both groups (24/27 or 89% in ASD vs 133/145 or 92% non-ASD patients, P = 0.632). We also found no difference in the median reflux index on the worst day ( P = 0.27) or the average of both days ( P = 0.75), BRAVO pH parameters and the proportion of abnormal studies between ASD and non-ASD children. When evaluating the overall symptom correlation with GER episodes, we did not find a difference between both groups, but we did find a higher symptom correlation for GER symptom during supine position in ASD children. Study was performed for behavioral indication in 11 ASD children, all had normal esophageal mucosa but 4 of those had an abnormal BRAVO pH study. No significant side effects were reported during the study, only 2 patients (1 non-ASD and 1 ASD) complained of self-limited chest pain. CONCLUSIONS: BRAVO wireless pH is well tolerated and feasible in evaluating GER and behavioral symptoms in ASD children and provides a reasonable alternative to standard trans-nasal pH monitoring.

A Guide to Establishing a Pediatric Neurogastroenterology and Motility Program.

ABSTRACT: The purpose of this document is to provide guidance for establishing a pediatric neurogastroenterology and motility (PNGM) program, including considerations for personnel, equipment, and physical space requirements, and business planning, from members of the neurogastroenterology and motility (NGM) Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) who have developed PNGM programs at various institutions. A business plan defining the needs for required personnel, dedicated physical space, procedures, clinical care, and equipment storage is a prerequisite. Thoughtful logistical planning should address provider schedules, clinical visits, procedure coordination, and prior authorization processes. A business-plan outlining equipment purchase with projected costs, revenue generation, and goals for future growth is desirable for obtaining institutional support, which is imperative to building a successful PNGM program.

Can Propofol Be Used to Assess the Presence of the Rectoanal Inhibitory Reflex During Anorectal Manometry Studies?

OBJECTIVES: To study changes in intra-anal pressure (IAP) and characteristics of the rectoanal inhibitory reflex (RAIR) during anorectal manometry (ARM) in patients undergoing anesthesia induction with propofol. METHODS: Prospective study in which ARM was performed at baseline while patients were awake and repeated after propofol-induced anesthesia. We studied IAP and the presence and characteristics of the RAIR before and after propofol. RESULTS: A total of 27 patients were included (63% male; 9.2 years). Three patients had obstructive symptoms after Hirschsprung disease repair (HSCR), and 24 had intractable constipation. At baseline, the RAIR was present on 21 of 27 patients and absent on 6 of 27. Of the six patients with an absent RAIR, it remained absent in four of six (three known HSCR, and one new diagnosis of IAS achalasia), and two of six had a normal RAIR during propofol. Therefore, RAIR was present in all patients with constipation. The mean resting IAP was significantly lower after propofol. The percentage of IAS relaxation after lower balloon volume inflations was significantly higher during propofol (P < 0.05). No difference was observed over the latency time or the total relaxation time after propofol. CONCLUSIONS: Propofol can be used to assess the presence of the RAIR during ARM in children who are uncooperative and undergoing other procedures under anesthesia. On the other hand, propofol significantly reduces the resting IAP and increases the percentage of internal anal sphincter relaxation after balloon distention. These findings may impact the interpretation to decide if an intervention is needed, or if there is a possible spinal neuropathy.

Melanosis Coli Is Not Associated with Colonic Dysmotility Nor Severity of Pediatric Functional Constipation.

BACKGROUND: Sennosides are commonly used for the treatment of constipation and associated with melanosis coli. In the present study, we evaluated the utility of melanosis coli as a marker of severity and its association with colonic motility in children with functional constipation. METHODS: Prospective study includes pediatric patients undergoing colonic manometry and colonic biopsies. Demographic data, medication history, surgical history, colonic manometry results (gastrocolonic response to a meal, high-amplitude propagating contractions, and nonpropagating contractions), colonic manometry catheter position, and pathologic results were collected and analyzed. We compared those variables with outcome (need for surgery) between both patient groups (presence or absence of melanosis coli). RESULTS: A total of 150 patients were included, median age was 9.9 years (range 2.1-18) and 77 (51.3%) were female, 17 had melanosis. Patients who took sennosides had higher rates of melanosis coli compared to those who did not (adjusted OR 13.88; 95% CI 4.05-47.57; P < 0.001), and we did not find an association between melanosis coli and use of other medications (osmotic laxatives, bisacodyl, lubiprostone), age, gender, weight, and height. We found no significant difference in the results colonic manometry between patients with and without melanosis coli. The rates of surgery for constipation between patients with and without melanosis coli were not statistically different. (OR 3.00; 95% CI 0.45-20.07; P = 0.257). CONCLUSIONS: Melanosis coli is associated with sennosides use, but it does not influence colonic motility nor is associated with increased subsequent need for surgery in pediatric functional constipation.

Upper Gastrointestinal Functional and Motility Disorders in Children.

Children with disorders affecting the sensory and motor functions of the esophagus will present primarily with swallowing dysfunction, dysphagia, and chest pain, and those with disorders affecting the normal function of the stomach will present with symptoms like abdominal pain, nausea, and vomiting. Recent advances in the mechanisms of disease and technology have increased our understanding of gastrointestinal physiology and that knowledge has been applied to develop new diagnostic studies and therapeutic interventions. We present an overview of the clinical presentation, diagnosis, and treatment of common primary and secondary functional and motility disorders affecting the upper gastrointestinal tract in children.

Lower Gastrointestinal Functional and Motility Disorders in Children.

Functional and motility gastrointestinal disorders are the most common complaints to the pediatric gastroenterologist. Disorders affecting the small intestine carry a significant morbidity and mortality due to the severe limitation of therapeutic interventions available and the complications associated with such interventions. Congenital colorectal disorders are rare but also carry significant morbidity and poor quality of life plus the social stigma associated with its complications. In this review, we summarize the clinical presentation, diagnostic evaluations, and the therapeutic interventions available for the most common and severe gastrointestinal functional and motility disorders of the small bowel, colon, and anorectum.

Evaluation of Fecal Incontinence in Pediatric Functional Constipation: Clinical Utility of Anorectal and Colon Manometry.

OBJECTIVES: The aim of the study is to evaluate the utility of motility studies in pediatric functional constipation with/without fecal incontinence. PATIENTS AND METHODS: Patients with functional constipation and failure to conventional therapy undergoing colonic manometry (CM) and/or anorectal manometry (ARM) manometry were classified as functional constipation without fecal incontinence (FC) or with fecal incontinence (FCI). Clinical data, motility parameters, and treatment outcomes were compared. RESULTS: A total of 280 were included, and all patients underwent CM (229 FC and 51 FCI) and 219 ARM. We found no difference in CM interpretation and presence of normal high amplitude propagating contractions (HAPCs) between groups; however, patients with FCI had higher frequency and presence of HAPCs and normal gastrocolonic meal response (GC). No CM parameter predicted outcomes. In FC, more patients with an abnormal CM responded to therapy compared to those with a normal study (79% vs 65% respectively, P = 0.04). FCI patients had lower median anal resting pressure compared to FC (49 vs 66 mmHg, respectively, P = 0.03); no other ARM parameter differentiated FC from FCI. We found no association between therapy response and ARM interpretation (P = 0.847) or any ARM parameter. A multivariate analysis found only male gender was associated with FCI (P < 0.001). CONCLUSIONS: FCI patients have higher frequency of normal CM parameters compared to FC, but overall interpretation was no different. CM helped predict response to therapy in FC but not in FCI. ARM demonstrated no added benefit in the evaluation of functional constipation with/without soiling. Patients with both normal ARM and CM had a lower response to therapy than those with abnormal studies.

Development of Entrustable Professional Activities and Standards in Training in Pediatric Neurogastroenterology and Motility: North American Society for Pediatric Gastroenterology, Hepatology and Nutrition and American Neurogastroenterology and Motility Society Position Paper.

ABSTRACT: Neurogastroenterology and motility (NGM) disorders are common in childhood and are often very debilitating. Although pediatric gastroenterology fellows are expected to obtain training in the diagnosis and management of patients with these disorders, there is an ongoing concern for unmet needs and lack of exposure and standardized curriculum. In the context of tailoring training components, outcome and expressed needs of pediatric gastroenterology fellows and programs, members of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and American Neurogastroenterology and Motility Society (ANMS) developed guidelines for NGM training in North America in line with specific expectations and goals of training as delineated through already established entrustable professional activities (EPAs). Members of the joint task force applied their expertise to identify the components of knowledge, skills, and management, which are expected of NGM consultants. The clinical knowledge, skills and management elements of the NGM curriculum are divided into domains based on anatomic regions including esophagus, stomach, small bowel, colon and anorectum. In addition, dedicated sections on pediatric functional gastrointestinal (GI) disorders, research and collaborative approach, role of behavioral health and surgical approaches to NGM disorders and transition from pediatric to adult neurogastroenterology are included in this document. Members of the NASPGHAN-ANMS task force anticipate that this document will serve as a resource to break existing barriers to pursuing a career in NGM and provide a framework towards uniform training expectations at 3 hierarchical tiers corresponding to EPA levels.

Diagnostic and clinical utility of the wireless motility capsule in children: A study in patients with functional gastrointestinal disorders.

BACKGROUND: The wireless motility capsule (WMC) evaluates gastrointestinal motility and transit simultaneously. We evaluated the utility of the WMC in children with functional gastrointestinal symptoms. METHODS: Study in children comparing WMC transit and motility parameters between those with upper (UGI) or lower (LGI) gastrointestinal symptoms, nuclear medicine gastric emptying time (NMGET) and/or a colonic radiopaque marker (CROM) study. KEY RESULTS: We prospectively recruited 57 children (median age 16.45y, range 8.78-17.8y, 44 Female) and 50 completed the study (24 UGI/26 LGI). We found no association between WMC study interpretation, motility and transit parameters and symptoms. WMC and NMGET interpretation agreement observed in 24/34 (70%) (κ = 0.351, p = 0.026) and with CROM in 17/21 (81%) patients (κ = 0.576, p = 0.007). WMC detected abnormal gastric transit in 41% vs. 24% with NMGET (p = 0.04) and abnormal colon transit in 62% vs. 71% of patients by CROM (p = 0.01). We found significant correlation (r = 0.574, p = 0.01) and no difference in median colon transit (p = 0.421) by WMC and CROM. A single WMC motility parameter, mean peak amplitude, was associated with NMGET (p = 0.04), none with CROM. Capsule retention >5 days (n = 9, all passed <2 weeks) was associated with prolonged colon transit, not with symptoms, age and gender. CONCLUSIONS: WMC is well tolerated in children as young as 8 years old. We found no association between WMC and symptoms, fair agreement with NMGET and strong agreement with CROM. WMC increases the yield of finding gastrointestinal transit abnormalities. Capsule retention in children is associated to prolonged colon transit. Larger studies are needed to further validate these findings.

Long-term Use of Bisacodyl in Pediatric Functional Constipation Refractory to Conventional Therapy.

OBJECTIVES: Standard therapy for pediatric constipation includes osmotic laxatives with stimulant laxatives use only as rescue therapy. Limited information is available on regular and long-term use of bisacodyl in pediatric population despite its common use in adult and pediatric constipation. METHODS: Retrospective review of patients with functional constipation refractory to conventional therapy (regular use of osmotic laxatives and intermittent use of stimulant laxatives only as a rescue therapy) referred to tertiary care children's hospital (January 2007-December 2014). Patients had a bowel movement (BM) frequency of ≤2 per week and were treated with bisacodyl regularly for longer than 4 weeks. Demographic variables, bisacodyl dose and treatment duration, number of BM/week before and after treatment, side effects, and length of follow-up were recorded. Response to therapy was successful when frequency of BM increased from baseline to ≥3 BM/wk. RESULTS: A total of 164 patients were included, 52% girls, median age 9.45 years (0.9-21 years). Bisacodyl median dose was 5 mg/day, median duration of treatment was 14 months (1-77 months) with 90% of patients taking the medication for <36 months. Median number of BM/wk doubled after initiation of bisacodyl from 2 to 4 bm/w (P < 0.001). Approximately 57% of patients had successful response. At long-term follow-up 55% of patients were successfully weaned off bisacodyl (median time of 18 months). Side effects reported in 9% of patients. CONCLUSIONS: Bisacodyl is effective and well tolerated in the long-term treatment of pediatric functional constipation refractory to conventional therapy. Most of patients with a favorable response were successfully weaned off the medication.

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.

Utility of Colon Manometry in Guiding Therapy and Predicting Need for Surgery in Children With Defecation Disorders.

OBJECTIVES: Colon manometry (CM) has emerged as a tool to evaluate children with defecation problems. Our aim was to evaluate the utility of CM in guiding therapy and predicting surgery in pediatric constipation. METHODS: Retrospective review of children undergoing CM for 4 indications: constipation, fecal incontinence, postsurgical evaluation and chronic intestinal pseudo-obstruction. Variables included age, sex, follow-up, and CM parameters: gastrocolonic response (GC) and quality/quantity of high-amplitude propagating contractions (HAPCs). INTERVENTIONS: medical, surgical or no intervention. OUTCOMES: response to change of therapy guided by CM, response to first intervention guided by CM (CMI) and CM predicting surgery (CMS). Response to therapy was classified according to study indication. RESULTS: Five hundred fifty-five studies (448 patients, 54.4% female; median age 8.9 years) were included, 24% of studies were normal. Change of therapy guided by CM was associated with a high response rate (P = 0.003). Overall response to stimulant laxatives was 48% and was not associated with CM findings. Surgical interventions had a higher response rate than medical or other interventions (P < 0.001). We found no association between the CM interpretation and CMI, but an abnormal CM was predictive of surgery (P < 0.01). GC and presence/number of HAPCs were not associated with CMI or CMS. We also found no association between HAPC quality and CMI but partially propagated HAPCs were predictive of surgery (P < 0.001). Logistic regression analysis showed no factors associated with CMI; however, longer follow up and partially propagated HAPCs were predictive of surgery. CONCLUSIONS: CM is useful in pediatric defecation disorders, although not predictive of successful medical intervention, an abnormal CM is predictive of surgery. CM should be performed only after medical interventions have failed and surgery is contemplated.