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Background: Pathophysiology of fecal incontinence (FI) in children with anorectal malformations (AM) is not well understood. Standard or high-resolution anorectal manometry (ARM) does not identify radial asymmetry or localize abnormal sphincter function. 3D high-definition anorectal manometry (HDARM) provides detailed topographic and 3D pressure gradient representation of anal canal., Aims: To compare intra-anal pressure profiles between children with AM and controls using HDARM and to determine the association between manometric properties and reported predictors of fecal continence (AM type, spinal anomaly, and sacral integrity)., Methods: HDARM tracings of 30 children with AM and FI referred for ARM were compared with 30 age and sex-matched children with constipation. 2D pressure profiles were used to measure length of high-pressure zone (HPZ). Longitudinal and radial measurements of sphincter pressure at rest and squeeze were taken along each segment in 3D topographic views and compared between groups., Key Results: 3D measurements demonstrated longitudinal and radial differences between groups along all quadrants of HPZ. At rest, intra-anal pressures were lower along the four segments longitudinally across the anal canal and radially along the quadrants in AM group (P < .01). At squeeze, all quadrant pressures were lower in segments 1-4 in AM group (P < .01). Sensation was abnormal in AM group (P < .01). Intra-anal pressures longitudinally and radially were not associated with predictors of fecal continence., Conclusions and Inferences: Children with AM had abnormal sensation and lower pressures longitudinally and radially along all quadrants of anal canal. Manometric properties at rest were not associated with reported predictors of fecal continence., (© 2020 John Wiley & Sons Ltd.)

Background: We evaluated the changes in antroduodenal manometry (ADM) parameters and interpretation when the test is performed the day of catheter placement and the following day., Methods: Catheter was placed endoscopically under anesthesia and recorded on day 1 and repeated on day 2. Study parameters including antrum and small bowel motility index (MI) during fasting, meal, postprandial, erythromycin (EES), and octreotide (OCT) challenge phases, the presence of the phase III of the migrating motor complex (MMC), visual postprandial response, and study interpretation were compared between both days., Key Results: Twenty patients were studied. Antrum and small bowel MI during fasting, postprandial, and EES challenge phases were significantly higher on day 2 than on day 1 (P < 0.05). The proportion of patients having a phase III of the MMC was significantly higher on day 2 compared to day 1 (65% vs 15%; P = 0.006). Study interpretation changed from day 1 to day 2. On day 1, 70% of the patients had a normal study and 30% had an abnormal study. On day 2, 67% of the patients with an abnormal study on day 1 changed to normal and 33% remained abnormal. All patients with a normal study on day 1 remained normal on day 2., Conclusions and Inferences: ADM parameters are affected the day of catheter placement. The MI and presence of the phase III of the MMC were significantly higher on day 2 compared to day 1. Overall, ADM study interpretation changed from day 1 to day 2 in 20% of the patients., (© 2018 John Wiley & Sons Ltd.)

Background: Upper gastrointestinal symptoms in children are common and motility disorders are considered in the differential diagnosis. High resolution esophageal manometry (HRM) has revolutionized the study of esophageal physiology, and the addition of impedance has provided new insights into esophageal function. Antroduodenal motility has provided insight into gastric and small bowel function., Purpose: This review highlights some of the recent advances in pediatric esophageal and antroduodenal motility testing including indications, preparation, performance, and interpretation of the tests. This update is the second part of a two part series on manometry studies in children (first part was on anorectal and colonic manometry [Neurogastroenterol Motil. 2016;29:e12944]), and has been endorsed by the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and the American Neurogastroenterology and Motility Society (ANMS)., (© 2017 John Wiley & Sons Ltd.)

Objective: In achalasia, absent peristalsis and reduced esophagogastric junction (EGJ) relaxation and compliance underlie dysphagia symptoms. Novel high-resolution impedance manometry variables, that is, bolus presence time (BPT) and trans-EGJ-bolus flow time (BFT) have been developed to estimate the duration of EGJ opening and trans-EGJ bolus flow. The aim of this study was to evaluate esophageal motor function and bolus flow in children diagnosed with achalasia using these variables., Methods: High-resolution impedance manometry recordings from 20 children who fulfilled the Chicago Classification (V3) criteria for achalasia were compared with recordings of 15 children with normal esophageal high-resolution manometry findings and no other evidence suggestive of achalasia. Matlab-based analysis software was used to calculate BPT and BFT., Results: Both BPT and BFT were significantly reduced in achalasia patients compared with children with normal esophageal motility (BPT 3.3 s vs 5.1 s P < 0.01; BFT 1.4 s vs 4.3 s P < 0.001). BFT was significantly lower than BPT (achalasia difference 1.9 s ± 1.3 s, P = 0.001 and normal difference 0.9 ± 0.3 s, P = 0.001). Overall, there was a significant correlation between BPT and BFT (r = 0.825, P < 0.001). We observed a 2-way differentiation of achalasia patients; those in whom the BPT and BFT were proportional, but significantly lower than in patients with normal peristalsis, and those in whom BFT was disproportionately lower than BPT., Conclusions: Calculation of BPT and BFT may help determine whether esophageal bolus transport to the EGJ and/or esophageal emptying through the EGJ are aberrant. For achalasia, this may detect flow resistance at the EGJ, potentially improving both diagnosis and objective assessment of therapeutic effects.

Background: Subtyping achalasia by high-resolution manometry (HRM) is clinically relevant as response to therapy and prognosis have shown to vary accordingly. The aim of this study was to assess inter- and intrarater reliability of diagnosing achalasia and achalasia subtyping in children using the Chicago Classification (CC) V3.0., Methods: Six observers analyzed 40 pediatric HRM recordings (22 achalasia and 18 non-achalasia) twice by using dedicated analysis software (ManoView 3.0, Given Imaging, Los Angeles, CA, USA). Integrated relaxation pressure (IRP4s), distal contractile integral (DCI), intrabolus pressurization pattern (IBP), and distal latency (DL) were extracted and analyzed hierarchically. Cohen's κ (2 raters) and Fleiss' κ (>2 raters) and the intraclass correlation coefficient (ICC) were used for categorical and ordinal data, respectively., Results: Based on the results of dedicated analysis software only, intra- and interrater reliability was excellent and moderate (κ=0.89 and κ=0.52, respectively) for differentiating achalasia from non-achalasia. For subtyping achalasia, reliability decreased to substantial and fair (κ=0.72 and κ=0.28, respectively). When observers were allowed to change the software-driven diagnosis according to their own interpretation of the manometric patterns, intra- and interrater reliability increased for diagnosing achalasia (κ=0.98 and κ=0.92, respectively) and for subtyping achalasia (κ=0.79 and κ=0.58, respectively)., Conclusions: Intra- and interrater agreement for diagnosing achalasia when using HRM and the CC was very good to excellent when results of automated analysis software were interpreted by experienced observers. More variability was seen when relying solely on the software-driven diagnosis and for subtyping achalasia. Therefore, diagnosing and subtyping achalasia should be performed in pediatric motility centers with significant expertise., (© 2017 John Wiley & Sons Ltd.)

Background: We evaluated the change in colon manometry (CM) parameters and interpretation comparing results when the study is performed the same day after the motility catheter is placed under anesthesia or the following day., Methods: CM catheter was placed with colonoscopy under anesthesia and recorded on day 1 and repeated on day 2. Study parameters including motility index during fasting, post-prandial and post-Bisacodyl challenge phase; gastrocolonic response; number, presence and propagation of high amplitude propagating contractions (HAPCs); and, study interpretation were compared between both the days., Key Results: Motility index (fasting, post-Bisacodyl phase, P<.05), HAPC number (10.1 vs 6.6, P=.01) and the proportion of patients having HAPCs (92% vs 70%, P=.002) was significantly higher on day 2 vs day 1. HAPC propagation improved on day 2 vs day 1 (fully propagated, 49% vs 37%; partially propagated, 43% vs 33%; absent 8% vs 30%). Study interpretation changed from day 1 to day 2. On day 1, 37% had a normal study and 63% had an abnormal study. On day 2, all patients with a normal study on day 1 remained normal, and patients with an abnormal study on day 1, 53% remained abnormal and 47% had a normal study., Conclusions & Inferences: CM parameters are affected the day the catheter is placed with colonoscopy under anesthesia. The number, presence, and propagation of HAPCs were significantly higher/improved on day 2 compared to day 1. Overall, CM interpretation changed from abnormal to normal from day 1 to day 2 in 47% of the patients., (© 2017 John Wiley & Sons Ltd.)

Gastrointestinal motility disorders in the pediatric population are common and can range from benign processes to more serious disorders. Performing and interpreting motility evaluations in children present unique challenges. There are primary motility disorders but abnormal motility may be secondary due to other disease processes. Diagnostic studies include radiographic scintigraphic and manometry studies. Although recent advances in the genetics, biology, and technical aspects are having an important impact and have allowed for a better understanding of the pathophysiology and therapy for gastrointestinal motility disorders in children, further research is needed to be done to have better understanding of the pathophysiology and for better therapies., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: The differential diagnosis of intractable reflux in children includes rumination syndrome, but confirming the diagnosis using antroduodenal manometry is invasive, is costly, and requires anesthesia. High-resolution esophageal manometry with impedance (HRM-MII) overcomes these limitations, and the goal of this study is to validate the use of HRM-MII as a diagnostic tool for rumination and to describe the subtypes of pediatric rumination., Methods: We reviewed the HRM-MII tracings of 21 children presenting with symptoms of intractable reflux in whom rumination was being considered. Patients underwent a standard and post-prandial HRM-MII. Peak intraluminal esophageal pressures, baseline gastric and thoracic pressures, and the timing of the R wave relative to LES relaxations and bolus flow were recorded. Chi-square analyses were used for comparison of proportions and means were compared using t-tests or non-parametric equivalent., Key Results: Forty-one (55.5%) primary and 33 (44.5%) secondary rumination episodes were seen. Three types of primary rumination were identified: i) LES relaxation without retrograde flow preceding the R wave (51% of episodes); ii) LES relaxation after the R wave (20% of episodes); and iii) R waves with no LES relaxation (29% of episodes). Eleven patients had rumination episodes with a peak gastric pressure <30 mm Hg. A total of 44 (60%) rumination episodes occurred during the standard HRM-MII, and 30 (40%) occurred during or after the meal., Conclusions & Inferences: HRM-MII can accurately diagnose rumination in children. We identify three types of primary rumination which may provide insight into therapeutic response., (© 2016 John Wiley & Sons Ltd.)

Background: Children with urinary incontinence (UI) have associated functional constipation (FC) and fecal incontinence (FI). The physiology between lower urinary tract (LUT) and anorectum in children has not been elucidated., Aims: Observe the effect of rectal distention (RD) on LUT function, and bladder filling and voiding on anorectal function., Methods: Children with voiding dysfunction referred to Boston Children's Hospital were prospectively enrolled in combined urodynamic (UDS) and anorectal manometry (ARM). Anorectal and urodynamic parameters were simultaneously measured. Patients underwent two micturition cycles, first with rectal balloon deflated and second with it inflated (RD). Lower urinary tract and anorectal parameters were compared between cycles., Key Results: Ten children (seven UI, four recurrent UTIs, nine FC ± FI) were enrolled. Postvoid residual (PVR) increased (p = 0.02) with RD. No differences were observed in percent of bladder filling to expected bladder capacity, sensation, and bladder compliance with and without RD. Bladder and abdominal pressures increased at voiding with RD (p < 0.05). Intra-anal pressures decreased at voiding (p < 0.05), at 25% (p = 0.03) and 50% (p = 0.06) of total volume of bladder filling., Conclusions & Inferences: The PVR volume increased with RD. Stool in the rectum does not alter filling cystometric capacity but decreases the bladder's ability to empty predisposing patients with fecal retention to UI and UTIs. Bladder and abdominal pressures increased during voiding, demonstrating a physiological correlate of voiding dysfunction. Intra-anal pressures decreased during bladder filling and voiding. This is the first time intra-anal relaxation during bladder filling and voiding has been described., (© 2016 John Wiley & Sons Ltd.)

Pressure-flow analysis allows assessing esophageal bolus transport in relation to esophageal pressures. This study aimed to characterize pressure-flow metrics in relation to dysphagia in paediatric patients. We analysed esophageal pressure-impedance recordings of 5 ml liquid and viscous swallows from 35 children (17 M, mean 10.5 ± 0.8 years). Primary indication for referral was gastroesophageal reflux disease (GERD) (9), post-fundoplication dysphagia (5), idiopathic dysphagia (16), trachea-esophageal fistula (2) and other (3). Peristaltic function was assessed using the 20 mmHg iso-contour defect and the timing between bolus pressure and flow was assessed using the Pressure Flow Index, a metric elevated in relation to dysphagia. Patients were stratified in relation to dysphagia and to peristaltic defect size. Dysphagia was characterized by a weaker peristalsis for liquids and higher Pressure Flow Index for viscous. When patients were stratified based on weak or normal peristalsis, dysphagia with weak peristalsis related to a larger iso-contour defect size and dysphagia with normal peristalsis related to higher Pressure Flow Index., Conclusion: Pressure-flow analysis enables differentiation of patients with dysphagia due to weak peristalsis (poor bolus clearance) from abnormal bolus flow resistance (esophageal outflow obstruction). This new dichotomous categorization of esophageal function may help guide the selection of optimal treatment such as pharmacological or endoscopic therapy., What Is Known: • Pressure-flow analysis (PFA) can detect abnormalities in esophageal motility using integrated analysis of bolus propulsion and bolus flow during swallowing. • AIM analysis has recently been reported to be useful in identifying subtle pre-operative esophageal dysfunction in adult patients who developed post-fundoplication dysphagia as well as in patients with non-obstructive dysphagia., What Is New: • Pressure-flow parameters can distinguish the cause of dysphagia in paediatric patients. • Combined high-resolution manometry and impedance measurements with pressure-flow analysis can differentiate paediatric patients with dysphagia symptoms in relation to either weak peristalsis (poor bolus clearance) or over-pressurization (abnormal bolus flow resistance). HOW MIGHT IT IMPACT ON CLINICAL PRACTICE IN THE FUTURE? • This study supports the use of a novel objective analysis method on recordings that are readily used in paediatric clinical practice. • The pressure-flow approach allows discriminating esophageal dysfunction in relation to dysphagia symptoms in children. This has not been achieved in children with current analysis methods. • The new findings of this study allow a dichotomous categorization of esophageal function, which may help to guide the selection of the most optimal treatment such as pharmacological or endoscopic therapy.

Background: The Chicago Classification (CC) facilitates interpretation of high-resolution manometry (HRM) recordings. Application of this adult based algorithm to the pediatric population is unknown. We therefore assessed intra and interrater reliability of software-based CC diagnosis in a pediatric cohort., Methods: Thirty pediatric solid state HRM recordings (13M; mean age 12.1 ± 5.1 years) assessing 10 liquid swallows per patient were analyzed twice by 11 raters (six experts, five non-experts). Software-placed anatomical landmarks required manual adjustment or removal. Integrated relaxation pressure (IRP4s), distal contractile integral (DCI), contractile front velocity (CFV), distal latency (DL) and break size (BS), and an overall CC diagnosis were software-generated. In addition, raters provided their subjective CC diagnosis. Reliability was calculated with Cohen's and Fleiss' kappa (κ) and intraclass correlation coefficient (ICC)., Key Results: Intra- and interrater reliability of software-generated CC diagnosis after manual adjustment of landmarks was substantial (mean κ = 0.69 and 0.77 respectively) and moderate-substantial for subjective CC diagnosis (mean κ = 0.70 and 0.58 respectively). Reliability of both software-generated and subjective diagnosis of normal motility was high (κ = 0.81 and κ = 0.79). Intra- and interrater reliability were excellent for IRP4s, DCI, and BS. Experts had higher interrater reliability than non-experts for DL (ICC = 0.65 vs ICC = 0.36 respectively) and the software-generated diagnosis diffuse esophageal spasm (DES, κ = 0.64 vs κ = 0.30). Among experts, the reliability for the subjective diagnosis of achalasia and esophageal gastric junction outflow obstruction was moderate-substantial (κ = 0.45-0.82)., Conclusions & Inferences: Inter- and intrarater reliability of software-based CC diagnosis of pediatric HRM recordings was high overall. However, experience was a factor influencing the diagnosis of some motility disorders, particularly DES and achalasia., (© 2014 John Wiley & Sons Ltd.)

Background: One of the primary reasons for referral for reflux testing is to correlate reflux events with symptoms such as cough. Adult studies have suggested that symptom recording is flawed and pediatricians feel this is an even more significant problem because there may be errors in both parental and patient reports. We hypothesize that intraesophageal pressure recording (IEPR) provides an objective method to identify coughs during reflux testing in children., Methods: We recruited 20 children undergoing multichannel intraluminal impedance with pH (pH-MII) testing for the evaluation of cough. We placed simultaneous intraesophageal pressure and pH-MII catheters. Tracings were blindly scored by 2 observers without knowledge of patient/parent symptom report. After the blinded scoring, patient/parent report of symptoms was recorded., Results: Ninety-four percent of all coughs were detected by IEPR and only 48% of all coughs were reported by patients/parents. The mean time from the IEPR cough to the patient/parent cough was 11±16 seconds. Using IEPR as the criterion standard for the detection of cough, the sensitivity of patient report for the detection of cough is 46%. Using varying symptom windows because of the increased precision of IEPR, the number of patients with a positive symptom index could be reduced from 30% to 0%, preventing children from being falsely categorized as having reflux-related lung disease., Conclusions: Parental and patient symptom recording in children is inadequate for making the diagnosis of reflux-related lung disease. If patients undergo pH-MII testing for reflux-related cough, IEPR should become the new standard by which to correlate reflux with cough.

Objectives: The pathophysiology of fecal incontinence is not well understood. Standard or high-resolution anorectal manometry (ARM) provides simple two-dimensional (2D) intra-anal pressure measurements and do not identify radial asymmetry or localize abnormal sphincter function. 3D high-definition ARM (HDARM) has 256 pressure sensors distributed circumferentially and provides a detailed topographical and 3D pressure gradient representation of the anal canal. The objective of this study was to use HDARM to characterize intra-anal pressure profiles in children during rest and squeeze., Methods: HDARM manometric tracings of 30 children with constipation referred for ARM were reviewed. 2D pressure profiles using high-resolution manometry were used to measure the length of the high-pressure zone (HPZ). The HPZ was divided into four equal segments from the anal verge to adjust for the variable sphincter length. Longitudinal and radial measurements of the HPZ during rest and squeeze (anterior, left, posterior, right quadrants of the HPZ) were taken along each segment in 2D and 3D topographical views. A 3D reconstruction combining all patients was then constructed., Results: Mean age was 149.3±1.8 months and mean HPZ length was 3.0±0.1 cm. Using 2D manometry, the mean peak HPZ pressure at rest was 72.0±2.5 mm Hg, and was located in the second segment of the HPZ. The mean peak HPZ pressure at squeeze was 202.9±13.1, and was located in the second segment. 3D measurement demonstrated both longitudinal and radial asymmetry along the anterior, left, posterior, and right quadrants of the HPZ. Left and right quadrant pressures were higher than anterior and posterior pressures at the anal verge and segment 1 during rest and squeeze. Anterior pressures were lower than posterior pressures longitudinally and radially in segments 2, 3, and 4 both during rest and squeeze. Our findings also suggest that in pediatrics it may be necessary to adjust pressure measurement to the anal canal length to get a more accurate picture., Conclusions: 3D HDARM allows for a detailed characterization of intra-anal pressures. 3D topographic pressure measurements demonstrate longitudinal and radial asymmetry of the anal canal at rest and during squeeze. This is the first time longitudinal and radial asymmetry of the anal canal has been described in children. 3D HDARM may allow for a better understanding of the mechanisms of fecal continence in children.

Background and Objective: We previously showed that approximately 10% of patients with intractable constipation have spinal abnormalities without any other physical findings. Given that spinal magnetic resonance imaging is costly and often requires deep sedation in children, it would be useful to find a screening tool to determine who has a higher likelihood of having a spinal abnormality. The aim of the study was to determine whether anorectal manometry is a useful screening test in predicting which patients will have abnormal spinal MRIs., Patients and Methods: This is a case-control study comparing the anorectal manometries of 10 children with constipation who had abnormal spinal MRIs (cases) to the manometries of 10 age-matched children with normal MRIs (controls)., Results: The maximum relaxation of the sphincter after balloon distention was achieved with a significantly smaller balloon in the cases as compared with the controls (35 ± 20 vs 60 ± 23 mL; P = 0.02). The dose-response curve of sphincter relaxation at different balloon distention was shifted to the left in patients with spinal lesions. Anal spasms after balloon distention were noted in 60% of the patients with abnormal magnetic resonance images compared with 0% of the controls (P < 0.003). There were no other differences., Conclusions: Patients with spinal cord abnormalities may show changes in anorectal manometry. Anal spasms on anorectal manometry are significant predictors of spinal abnormalities. Also, patients with spinal abnormalities have maximum sphincter relaxations with smaller balloon sizes. Further studies are needed to determine the utility of anorectal manometry as a screening test for spinal abnormalities in patients with constipation.

Objective: To evaluate esophageal body motor contractions occurring during esophageal reflux in pediatric patients with gastro-esophageal reflux disease (GERD)., Methods: Patients referred for the evaluation of GERD who were evaluated with combined 24-hour pH probe and esophageal manometry test (MP24) were included. Patients were separated into the following groups: Group C -- normal pH probe and normal EGD; Group 1 -- abnormal pH probe and normal EGD; and Group 2 -- abnormal pH probe and EGD with histologic esophagitis. Esophageal motor function during reflux episodes was analyzed., Results: Twenty-five patients were included. All had a normal stationary esophageal manometry. Patients in Groups 1 and 2 had significantly more gastroesophageal reflux by pH probe than Group C (P < 0.01). During the MP24, patients in Group 1 and 2 had significantly fewer contractions per minute pre-, during, and post-GER (P < 0.05). There were significant differences in the number of isolated and prolonged contractions (>7 sec) during prolonged GERD episodes >5 minutes (P < 0.05)., Conclusion: Children with GERD have a decreased number and abnormal esophageal body contractions with esophageal reflux. This suggests that children with GERD with and without esophagitis have impaired esophageal body acid clearance.

Objective: To describe diurnal variations of esophageal motor function in children using combined ambulatory 24-hour esophageal manometry and continuous intraluminal pH measurement (MP24)., Methods: Medical records of all patients referred for the performance of MP24 from 1995 to 2002 at a tertiary care center were reviewed. Patients were selected retrospectively for this study using the following inclusion criteria: (1) no dysphagia, (2) normal upper gastrointestinal barium radiograph, (3) normal esophagogastroduodenoscopy and biopsies, (4) normal stationary esophageal manometry, (5) normal esophageal pH probe, and (6) no gastrointestinal pathology appearing after long-term follow-up. Data from the MP24 of these children were retrospectively analyzed for differences between meal, upright, and supine periods using nonparametric univariate analysis., Results: One hundred twenty-three children had MP24 during the study period. Eleven met the criteria for normality and were included. Their mean age was 12.4 +/- 1.5 years. The number of contractions/minute in the upper, middle, and lower esophageal body differed significantly during meals and in the upright and supine periods (P < 0.01). A significant increase in motor activity occurred during meals (P < 0.01). There was less motor activity observed in the supine position than in the upright position (P < 0.01). There were significant differences in effective (P < 0.05) and total propagated peristalsis among the meal, upright, and supine periods (P < 0.01), with the largest percentage of propagated contractions observed during meals., Conclusion: This study provides the first information on prolonged esophageal motor activity in pediatric patients without esophageal disease. MP24 in children demonstrates significant diurnal variations in esophageal motor function that is similar to the findings in studies of healthy adults.

Background: The diagnosis of Hirschsprung's disease is usually made in neonates but often considered in older infants and children with constipation: these children may be referred for barium enema. Since it is widely accepted that a normal barium enema does not exclude Hirschsprung's disease, some children, after a normal enema, undergo more invasive procedures such as rectal manometry or biopsy. Our study asked how frequently a diagnosis of Hirschsprung's disease was made by biopsy or manometry in children who had normal barium enema., Materials and Methods: We reviewed the medical records and barium enemas of 54 patients older than 28 days with constipation or difficulty passing stool who had a barium enema followed by manometry and/or biopsy., Results: Forty-eight patients had normal enemas: 24 of those patients had biopsies, 16 had manometry, and 8 both manometry and biopsy. Only 1 had manometry suggestive of Hirschsprung's disease, confirmed by biopsy. Six patients had abnormal enemas. Five had biopsy and manometry compatible with Hirschsprung's disease; one had a normal biopsy and manometry study., Conclusion: The barium enema is a good initial screening test for Hirschsprung's disease in severely constipated children since it correlates well with manometry and biopsy. The enema is particularly useful in centers without easy access to pediatric gastroenterology services, and a normal enema in this setting allows the continuation of medical therapy with further evaluation only if there is a lack of response. An abnormal enema, however, requires referral to a facility equipped to perform confirmatory manometry or biopsy.