Anorectal Manometry Findings in Relation with Long-term Functional Outcomes of The Patients Operated on for Hirschsprung's Disease Compared to The Reference-Based Population

Anorectal Manometry Findings in Relation with Long-term Functional Outcomes of The Patients Operated on for Hirschsprung's Disease Compared to The Reference-Based Population | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Anorectal Manometry Findings in Relation with Long-term Functional Outcomes of The Patients Operated on for Hirschsprung's Disease Compared to The Reference-Based Population Tung Trinh Huu, Ngoc Minh NGUYEN, Kim Thien LAM, Thach Ngoc PHAM, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-2527378/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 17 Feb, 2023 Read the published version in Pediatric Surgery International → Version 1 posted 7 You are reading this latest preprint version Abstract Purpose This study investigated anorectal manometry (AM) findings and bowel function of patients operated on for Hirschsprung’s disease (HD). Methods A cross-sectional study was conducted at Children’s Hospital 2. Patients operated on for HD from January 2015 to January 2020 were reviewed. Their clinical characteristics, bowel function, and manometric findings were investigated and compared with the references. Results Ninety-five patients and 95 references were enrolled. Mean ages were 6.6 ± 2.2 years and 7.2 ± 2.9 years; fecal incontinence rates were 25.3% and 2.1%, and constipation rates were 12.6% and 4.2 for the patients versus the references, respectively. Anal resting pressures were significantly decreased in the patients compared to the references (53.2 ± 16.1 mmHg versus 62.2 ± 14.0 mmHg; p < 0.05). Among the patients, the anal resting pressure was significantly decreased in the incontinents than in the continents (46.0 ± 10.6 mmHg versus 55.6 ± 16.9 mmHg, p < 0.05). During the sensation test, the value of maximum tolerated volume was significantly decreased in the incontinents than in the continents (135.9 ± 47.9 mL versus 166.6 ± 58.3 mL, p < 0.05). Conclusion AM is an objective method providing beneficial information that could guide a more adapted management in HD patients with defecation disorders. Hirschsprung’s disease anorectal manometry long-term outcomes constipation fecal incontinence Figures Figure 1 Figure 2 Figure 3 Introduction Hirschsprung's disease (HD) is a common pediatric surgery characterized by congenital malformation of the enteric nervous system. The prevalence is about 1 per 5000 live births, and the estimated prevalence of 1.4 per 5000 live births in Asian populations [ 1 ]. Defecation disorders are noticed in a non-negligible number of patients after definitive surgery for HD. Fecal incontinence and constipation are the two main complaints reported in broad ranges of incidence from several to even more than 50% in the literature [ 2 – 6 ]. Clinical assessment of fecal continence is often challenging. Continence problems may be due to either sequela of the surgical techniques or functional motility disorders [ 7 ]. In the literature, authors reported that resection of a part of the large bowel and intra-operative injuries to the anal sphincters could be responsible for postoperative defecation disorders. Otherwise, anorectal function in patients after surgery for HD is still unclear [ 8 – 11 ]. Clinically, anorectal manometry (AM) could help objectively discriminate between acquired troubles and associated functional disorders [ 9 , 11 – 14 ]. So, this study investigated the interests of anorectal manometry and its relationship with long-term bowel function in patients after surgery for HD at Children's Hospital 2 in Ho Chi Minh City, Vietnam. Materials And Methods Study design A cross-sectional study was conducted at Children's Hospital 2. The hospital's research ethics committee approved this study under IRB number 747/NĐ2-CĐT. Operated patients All consecutive charts of patients operated on for HD in our department between January 2015 to January 2020 were retrieved. Data have been collected on gender, date of birth, age at surgery, operative characteristics, and postoperative complications from their medical records stored in the hospital's electronic database. Patients over four years of age with at least twelve months of postoperative follow-up were contacted and invited to participate in this study. The investigators interviewed patients and their parents to complete the questionnaires about bowel function [15]. During the interview, a careful clinical examination was done to rule out the possibility of distal anorectal stricture or other anomalies. The patients were asked to perform AM. Manometric findings were collected for analysis. Informed consent was obtained from all participants. Patients with neurological impairments or AM contraindications were excluded from this study. Operative procedures: The transanal endorectal pull-through technique was applied for the cases with classic HD forms (recto-sigmoid aganglionosis). In patients with long segments of aganglionosis, a Soave pull-through procedure with an abdominal approach (open Soave) or with laparoscopic assistance was used in our institution. An enterostomy was indicated in cases of severe enterocolitis or failure with nursing care (washout). An ileoanal anastomosis without a reservoir was performed for patients with total colon aganglionosis. Reference-based population The reference-based population was volunteers recruited from the pool of patients of Children’s Hospital 2 in Ho Chi Minh City, Vietnam. The inclusion criteria of the references were without any antecedent digestive surgical intervention. They were asked to complete an AM and provided with all information regarding the study aims, objectives, and possible anorectal HRAM adverse effects. All participants and their parents/ relatives provided written consent before participating in the study. The references were matched with the cases by the year of age and gender with a ratio of 1:1. Before AM tests, the clinical and digital rectal examinations were indicated to ensure that participants were free from any anorectal disorders or diseases. Data have been collected on gender, date of birth, bowel function, and AM findings. Bowel function Bowel function was investigated using variables related to clinical aspects of the patient’s stooling patterns, such as fecal incontinence and constipation, as defined in the following paragraphs. Fecal continence was based on the Wingspread classification, which defines the following four levels of continence: excellent : clean (totally continent, toilet trained with no medication); good : staining (rarely soiling, except during stressful exercise); fair : intermittent fecal soiling, urge incontinence; poor: constant fecal soiling or smearing [16]. Patients classified as “ fair ” or “ poor ” were considered fecally incontinent; the other categories were considered fecally continent. We considered that fecal continence was commonly acquired in children older than four [17]. Constipation : Patients were studied for stooling frequency, laxatives, and enema over the months preceding the investigation. A patient was considered suffering from constipation if defecation was only possible with laxatives or occurred less than three times per week according to the ROME IV criteria for functional constipation [18]. Equipment and preparations Water-perfused high-resolution anorectal manometry with eight channels was applied; using the MMS® database software (version 9.3k, copyright (c) 1988-2015 by Medical Measurement System). Rectal enema is routinely before the tests. Patients were in the supine position, their hips and knees flexed, and asked to get familiar with the test. The sequence of the test was as follows: Profile of manometric measurements Anal resting pressure (mmHg) was measured for one minute at rest. The length of the high-pressure zone (HPZ) on AM, considered the anal canal length, was calculated. Anal pressure at coughing maneuver (mmHg) - three single coughs, separated by a thirty-second rest period, were performed. Anal pressure at squeeze maneuver (mmHg) - three squeezes of five-second duration, separated by a thirty-second rest period, were recorded. The maximal time enabled to perform an endurance squeeze maneuver was recorded (seconds). Recto-anal inhibitory reflex (RAIR) - the balloon was inflated within 3 - 5 seconds and deflated; the initial volume was 10 ml and was subsequently increased by increments of 10 ml for the following times. This test was repeated several times to detect the presence of the RAIR. A drop of at least 25% of resting pressure has to occur with subsequent restoration to at least two-thirds of resting pressure for it to be deemed present [7]. Sensation test: the balloon was inflated at a rate of 5 ml every 5 seconds, and the patient was asked to report consequently: the first sensation, urge to defecate, and finally, a maximum tolerated volume, and the sequential volumes were noted (mL) [11]. Balloon expulsion maneuver: the balloon was filled with about 20 ml of air, and the patient asked to try to strain the balloon. While the patient tried evacuating the balloon, we measured the anal sphincter pressure. The anal sphincters (internal and external) must normally relax during defecation. Failure to evacuate the balloon with increased abnormal anal sphincter pressure (IAAP) indicates dyssynergic defecation [11]. Data analysis and statistics Data are reported as mean and standard deviation (SD) or as median and range for continuous variables according to the normality of the distribution and as number and proportion for discrete data. Chi-square or Fisher exact tests were used to compare categorical data. Independent t -tests were used to compare means of two groups. All tests were two-sided, and a p-value < 0.05 was considered significant. IBM SPSS Statistics version 20 (SPSS Chicago, IL) was used for statistical analysis. Results In total, 95 interviewed patients who fulfilled the inclusion criteria and underwent AM were enrolled in this study. All the cases had histopathological results confirming normal ganglionic innervation at the level of anastomosis. Rectosigmoid aganglionosis was the most seen form of HD, representing 69.5% (66 out of 95) of cases operated with TERPT. Otherwise, 30.5% (29 out of 95) of patients had long segment HD that required abdominal assisted surgery, including 16 patients with open Soave and 13 with LATEP. On the other hand, 95 references were also recruited in this study. Patient characteristics Table 1 shows patient characteristics and their association with either fecal continence or constipation at investigation. The mean age of the patients at investigation was 6.6 ± 2.2 years, with 68% male. The mean age of the patients at surgery was 3.3 ± 2.2 years. The mean follow-up time was 3.4 ± 1.9 years. Table 1 . Clinical characteristics and their association with fecal incontinence or constipation, identified by investigators at the last follow-up of the patients operated on for Hirschsprung’s disease (N=95) Variables All N Prevalence of fecal incontinence ( n/N, %) P Prevalence of constipation (n/N, %) P Age at investigation, (years) (Mean ± SD) 6.6 ± 2.2 6.1 ± 1.4 - 7.8 ± 3.2 - Age groups, (n, %) • § 4 - <10 years • § 10 - 16 years 85 10 23 (27.1) 1 (10.0) 0.005 10 (11.8) 2 (20.0) 0.6 Age at surgery, ( years) (Mean ± SD) 3.3 ± 2.2 3.0 ± 1.7 - 3.9 ± 3.3 - Follow-up time, (years) (Mean ± SD) 3.4 ± 1.9 3.2 ± 1.4 - 3.7 ± 2.0 - Gender, (n, %) • § Male • § Female 46 49 10 (21.7) 14 (28.6) >9.99 * 6 (13.0) 6 (12.2) 0.4 * Enterostomy, (n, %) • § Yes • § No 10 85 4 (40.0) 20 (23.5) 0.3 2 (20.0) 10 (11.8) 0.6 Operative procedure, (n, %) • TERPT • LATEP + Open Soave (*) 66 29 16 (24.2) 8 (27.6) 0.52 * 5 (7.6) 7 (24.1) 0.12 * Resected segment • Rectosigmoid colon • Descending colon • Transversal colon • Total colon 66 21 6 2 16 (24.2) 4 (19.0) 2 (33.3) 2 (100) - 5 (7.6) 4 (19.0) 3 (50.0) 0 • Complications, (n, %) • § Anastomotic leak • § Bowel obstruction • § Residual aganglionosis • Total complications (#) 5 0 2 7 3 (60.0) 0 0 3 (42.9) 0.36 0 0 2 (100) 2 (28.6) 0.64 Abbreviation: SD, standard deviation; TEPRT, transanal endorectal pull-through without abdominal assistance; LATEP, laparoscopically assisted trans-anal endo-rectal pull-through procedure. (#) Data for postoperative complications reported by the number of encountered cases. (*) Chi-squared test, otherwise Fisher’s exact test. Note: Bold values (p < 0.05) were considered as statistically significant. Bowel function The patient chart review and a thorough medical history taken during the interview showed that 25.3% (24 out of 95) of the patients clinically presented with fecal incontinence and 12.6% (12 out of 95) of other patients. In contrast, the prevalence of fecal incontinence and constipation were 2.1% (2 out of 95) and 4.2% (4 out of 95), respectively, in the references, as shown in Table 2. Notably, the prevalence of these two symptoms was statistically higher in the patients than in the references. Regarding fecal incontinence, the prevalence was significantly higher in the patients younger than ten years compared with the older groups. Otherwise, none of the other characteristics of the patients were significantly associated with the prevalence of fecal incontinence (Table 1). Regarding constipation, the prevalence was not significantly different according to all characteristics of the patients (Table 1). Table 2 . Patients’ characteristics and defecation patterns of the patients operated on for Hirschsprung’s disease (N=95) versus the references (N=95) who underwent an anorectal manometry Clinical characteristics Patients (N=95) Reference s (N=95) P-value Age, (year) (Mean ± SD) 6.6 ± 2.2 7.2 ± 2.9 0.17 * Age groups, (n, n/N %) • § 4 - <10 years • § 10 - 16 years 69 (72.6) 26 (27.4) 69 (72.6) 26 (27.4) - Gender, (n, n/N %) • § Male • Female 49 (68.0) 46 (32.0) 49 (68.0) 46 (32.0) - Constipation, (n, n/N %) • § Yes • § No 12 (12.6) 83 (87.4) 4 (4.2) 91 (95.8) 0.07 Fecal continence # , (n, n/N %) • § Excellent • § Good • § Fair • Poor 37 (38.9) 34 (35.8) 6 (6.4) 18 (18.9) 85 (89.5) 8 (84.) 2 (2.1) 0 <0.001 Abbreviation: SD, standard deviation. (#) Fecal continence levels according to the Wingspread’s classification. (*) Independent t-test, otherwise Fisher’s exact test. Note: Bold values (p < 0.05) were considered as statistically significant. Manometric findings Table 3 shows the manometric findings of the patients compared with the references. Anal pressures The anal resting pressure was significantly lower in the patients than in the references (53.2 ± 16.1 mmHg versus 62.2 ± 14.0 mmHg; p<0.05), as shown in Figure 1. Moreover, in the operated group, the anal resting pressure was significantly decreased in the incontinent patients than in the continent patients (46.0 ± 10.6 mmHg versus 55.6 ± 16.9 mmHg; p<0.05). When performing cough maneuvers and squeeze maneuvers, the anal pressures were significantly lower in the patients than in the references. However, the difference was not significant between the two subgroups: incontinent and continent patients in the operated group (p>0.05), as shown in Table 3. Endurance squeeze test (time completed) Measurements of time completed during the endurance squeeze test had significantly decreased in the patients than in the references (p0.05) (Table 3). Anal canal length (high-pressure zone) The length of HPZ on AM, considered the anal canal length, was significantly longer in the patients than in the references (2.2 ± 0.3 cm versus 2.1 ± 0.2 cm, p0.05). Rectal anal inhibitor reflex Fifty of 95 patients (52.6%) showed a re-establishment of RAIR. In contrast, RAIR was normally reported in all the references. Sensation tests The volumes of the first sensation, urge to defecate, and Vmax during the sensation tests showed no significant difference in the patients from that in the references (p>0.05), as shown in Table 3. Whereas, in the operated group, the incontinent patients had shown significantly decreased volumes regarding the first sensation, urge to defecate, and Vmax than that of the continent patients (p<0.05) (Table 3). The qualitative results of the Vmax values are illustrated in Figure 3, with a significant decrease in the Vmax values in the incontinent patients compared to the other groups. Dyssynergic defecation The incidence of IAAP was higher in the patients than in the references, but without significant difference. In addition, in the operated group, there was no significant difference between the continent and the incontinent patients (p>0.05). Table 3 . Manometric findings according to fecal continence status of the operated patient (N=95) versus the references (N=95) Manometric findings Operated patients References (N=95) P * All (N=95) Continence (n=71) Incontinence (n=24) P # Anal pressure (mean ± SD, mmHg) • § At resting 53.2 ± 16.1 55.6 ± 16.9 46.0 ± 10.6 0.004 62.2 ± 14.0 <0.001 • § Cough maneuver 126.4 ± 34.5 123.7 ± 34.1 134.3 ± 35.1 0.20 139.8 ± 43.2 0.02 • Squeeze maneuver 111.8 ± 38.7 115.2 ± 39.8 101.5 ± 34.3 0.13 126.7 ± 38.5 0.01 Anal canal length (high-pressure zone, cm) (mean ± SD) 2.2 ± 0.3 2.2 ± 0.3 2.2 ± 0.2 0.54 2.1 ± 0.2 0.02 Endurance squeeze test ( time completed, second) (mean ± SD) 21.2 ± 7.2 21.4 ± 7.4 20.5 ± 6.8 0.6 24.1 ± 6.5 0.005 Recto-anal inhibitory reflex (n, n/N %) 50 (52.6) 38 (53.5) 12 (50.0) 0.77 β 95 (100) - Sensation test (mean ± SD, mL) • § First urge 93.5 ± 44.8 100.3 ± 45.5 71.8 ± 93.5 0.01 100.9 ± 44.5 0.26 • § Urge to defecate 128.5 ± 54.5 138.2 ± 53.5 97.7 ± 46.4 0.002 143.5 ± 63.5 0.09 • § Vmax 159.4 ± 57.3 166.6 ± 58.3 135.9 ± 47.9 0.02 172.7 ± 63.5 0.22 IAAP (n, n/N%) 21 (22.1) 14 (66.7) 7 (29.2) 0.4 β 14 (14.7) 0.26 β Abbreviation: SD, standard deviation; IAAP, increased abnormal anal sphincters pressures when performing balloon expulsion test; Vmax, maximum tolerated volume. (*) P-value of the comparisons between the references and the operated patients ; (#) P-value of the comparisons between the two subgroups of the operated group : continent and incontinent patients ; (β) Fisher’s exact test, otherwise independent t-test. Note: Bold values (p < 0.05) were considered as statistically significant. Fecal continence status according to the Wingspread’s classification. Discussion After a pull-through surgery, HD patients are at high risk for defecation disorders [19, 20]. Consequently, these disorders could negatively impact their emotional and social development [15, 21-23]. Therefore, the long-term outcomes of these patients remain key points in postoperative management. However, the assessment of bowel function is difficult to objectify, especially in long-term follow-ups [24, 25]. Long-term outcomes This cohort series reported long terms outcomes of the patients operated on for HD with an average follow-up time was 3.4 ± 1.9 years. As a result, the prevalence of fecal incontinence in patients with HD was 25.3%, compared with 2.1% in the references. This result was comparable to Neuvonen et al. reporting that in Finland, 25% of patients presented socially with fecal incontinence versus 2% in the controls [26]. A systematic review by Ying Dai et al. reported that the pooled prevalence of fecal incontinence was 20% (95% CI: 13% - 28%) [19]. In contrast, constipation was less common than continence problems, as reported in the literature and in this study as well. In our series, the prevalence of constipation was 12.6% for the patients compared with 4.2% for the references. According to Neuvonen et al. , the constipation rate was 5% in patients versus 4% in controls [26]. Ying Dai et al. reported the pooled prevalence of constipation was 14% (95% CI: 6% - 25%) [19]. Discordance of these outcomes between the studies may be explained by differences in the definition of defecation disorders and the exactitude of clinical assessment [15]. These issues may result from either dysmotility of the proximal pulled-through colon or anatomical changes in the anorectal structure. As suggested by several authors, AM has been introduced as a helpful tool to assess patients with persistent continence problems, allowing for individualized lesions in each patient [11, 27-29]. In this study, we used AM to investigate the anal sphincter function, the neo-rectum, and the associated functional troubles if persistent of the patients compared with the references. There are few reports about AM during follow-up of children operated on for HD, and the role of AM seems still controversial [8-11]. One point force of this study was the establishment of manometric data in the reference-based population that is still lacking, especially the data on children. That helped to discriminate the changes of manometric parameters in the operated group compared to the references [30]. Anal sphincters pressures and anal canal length In this report, the anal resting pressure of the patients was significantly lower than in the references. In addition, the incontinent patients had significantly lower anal resting pressure than continent patients in the operated group (Figure 1) [31]. Our report shows, as described in the literature that patients with severe fecal incontinence present significantly lower resting and squeeze pressure than continent subjects [32, 33]. That could be due to partial or complete destruction of the anal sphincters due to intraoperative overstretching of the anus during surgery [29]. Otherwise, some authors have raised a hypothesis that the problematic Soave cuff could lead to postoperatively obstructed symptoms in patients with the Soave procedure [34]. The Soave procedure - a worldwide used technique as well as in our series - was explicitly to leave a muscular cuff outside the rectal wall when performing an endorectal dissection. Consequently, the longer the cuff, the higher incidence of obstructive symptoms, whereas the shorter the cuff, the higher incidence of fecal incontinence [28]. However, in this cohort series, the anal sphincter pressures seemed to be impaired in the patients compared to the references, as shown in Table 3 [35, 36]. On the other hand, the length of the HPZ was longer in the patients than in the references. Nevertheless, there was no significant difference in the length of HPZ between the incontinent and the continent patients (Table 3). Rectal anal inhibitor reflex The RAIR is thoughtfully absent during follow-up of the patients operated on for HD as its pathophysiology [11]. However, some authors recently reported the reappearance of RAIR in patients after an operation for HD [9, 12, 28]. Gad El-Hak et al. reported RAIR becoming intact in 11.5% of HD patients four years after surgery and improving rectoanal sensation [37]. Although, this phenomenon did not significantly associate with clinical fecal continence of the patients [9, 28]. In this series, we noted that 50 patients (52.6%) got their RAIR back. Again, the re-establishment of RAIR was also not correlated to any positive long-term outcome in our series (Table 1). However, those who re-established RAIR were older patients, suggesting a quite normal rectal function as an adaptation of the neo-rectum in long-term follow-up. A false RAIR re-establishment due to technical issues when performing an AM should always be considered [38]. Sensation tests Concerning continence pathophysiology, the rectal sensation is the essential determinant. This finding was studied by measuring rectal distention during sensation tests (e.g., the first sensation, the urge to defecate, and the Vmax) [39]. Although we could not measure a precise rectal compliance index in this study, the Vmax indirectly provides helpful information about the elasticity and reserve capacity of the neo-rectum. Our results reported a significant decrease in the Vmax of the incontinent patients compared to the continent patients in the operated group (Figure 2). However, there was no significant difference in Vmax between the references and the operated patients (p>0.05) (Table 3). The hypothesis is that fecal incontinence could be due to the Vmax values being significantly impaired or below average ( increased perception ) [28, 33]. Of note, the average values of Vmax adapted by Meinds et al. were 135 mL in children six years of age and 160 mL in children twelve years of age [33]. In our series, the Vmax value of the references was 172.7 ± 63.5 mL compared to 135.9 ± 47.9 mL in the incontinent patients (Table 3). Clinically, we observed that these patients with severe reduction of Vmax presented with a high degree of fecal incontinence, as shown in Figure 2. These patients were considered rectal hypersensitivity or hypo-compliance . So, in cases with increased perception, the reservoir function of the neorectum is significantly impaired, leading to the uncontrolled leakage of the stool. Of course, we must rule out any possible inflammation, e.g., proctitis or irritable bowel syndrome [39]. Otherwise, this problem could also be due to surgical issues, such as a colon twist or stretching on the inferior mesenteric artery during colon lowering, which is responsible for chronic poor vascularization of the descended colon [15, 40]. We advocate therefore the routine use of laparoscopy in releasing the tension of mesenteries during pull-through surgery and avoiding the torsion of the pull-through colon by laparoscopic control [41]. In contrast, rectal hyposensitivity, or hyper-compliance , also known as a decreased perception , is another complex problem in which their Vmax values were noted with very high volumes but without or significant impairment of the sensation to defecate during the sensation tests. Biofeedback training can improve rectal sensation in patients with decreased perception [42]. Overall, in this series, the increased perception was likely more apparent in the incontinent patients who presented a poor prognosis with severe and persistent fecal incontinence (Figure 2). Furthermore, as concluded in this study, fecal incontinence is a more common problem than constipation. Dyssynergic defecation The etiology of dyssynergic defecation is still unknown. Dyssynergic defecation occurs due to the sphincter apparatus's relaxation disorders and inadequate intra-rectal pressure during defecation [43]. Meinds et al. reported that AM could help to visualize dyssynergic defecation [11]. A shortcoming of the AM system used in this study was not measuring the pressure gradients between intra-rectum versus anal sphincters to demonstrate the type of dyssynergia. Our series reported that 22.1% (21 out of 95 patients) with a paradoxical increase in anal sphincter pressures (IAAP). The IAAP phenomenon could represent type I or II of dyssynergic defecation during the balloon expulsion test (Table 3) [44]. In these patients, the initial problem could be retentive constipation and, so far, followed by overflow incontinence. If not managed well, this process may worsen the obstructed symptoms, whereas this is a reversible functional situation in the beginning [11]. In summary, AM could demonstrate objectively the anal sphincter pressure ( hypotonus or hypertonus ), the sensation of the neorectum ( increased or decreased perception ), and the associated functional issues of defecation ( dyssynergia ) in the patients operated on for HD. The lesions could be either single or multiple factors in each concrete patient [45]. Bowel management in long-term follow-ups Regarding long-term outcomes, some patients may suffer from fecal incontinence or constipation even years after surgery. This dilemma is still insufficient or lacking in developing countries [46, 47]. Non-operative management, such as bowel management (laxatives and enemas), should be considered the first option in most cases with high rates of success, even in more severe cases [48]. Moreover, some sophisticated techniques or more aggressive approaches like a transient (sometimes definitive) colostomy [49] or sacral nerve stimulating device implantation in some well-equipped centers for patients with refractory fecal incontinence [50, 51] should be considered. All these management options must be chosen carefully considering the AM results. AM could provide beneficial information on the etiologies for the continence problems. Thus, the therapeutical strategy for each case must be individualized and not only based on the clinical symptoms of fecal incontinence or constipation [10, 15, 28, 34]. In our institution, we customized a tailored “bowel management program” for each patient presented with persistent and severe defecation disorder due to “anorectal outlet disorders” [47]. The program consists of training daily habit defecation (biorhythm), adjusting the dosage of laxatives in cases of fecal retention (fecaloma); precisely determining the amount of water to facilitate enemas if needed (training for regular retrograde active enemas at-home care) according to the value of the Vmax, and adjusting the frequency of enemas depending on the amelioration of the symptoms and the adaptation of the patients as well as their families [52]. In not rare cases with an associated functional problem (animus or dyssynergic defecation), AM could be repeated periodically as biofeedback training courses to educate patients to get used to “a normal defecation reflex” [53, 54]. Otherwise, in the cases with quite normal AM results, the original problems could be due to a higher level such as dysmotility of the colon (rapid or slow colonic transit time) [55]. Finally, one advantage of this study was the use of a reference-based population that helped to build the database for the average values of AM in Vietnamese children and to compare these parameters with one of the operated patients. More data should be collected for the reference parameters with other study results in the future. To our knowledge, this study could help promote long-term follow-ups still lacking in developing countries where the number of HD cases is much higher than in other countries with well-equipped settings [15, 28]. Conclusion Our report shows that AM is an objective method for investigating anorectal malfunction in which the anal pressures and the rectal sensation are the two essential determinants. It provides helpful information that could guide better-adapted management in patients presenting with defecation disorders after an HD operation. Abbreviations - AM - anorectal manometry - HD - Hirschsprung’s disease - IAAP - increased abnormal anal sphincter pressure - LATEP - laparoscopically assisted trans-anal endo-rectal pull-through - RAIR - recto-anal inhibitory reflex - SD - standard deviation - TERPT - transanal endorectal pull-through Declarations Conflict of interest The authors declare that they have no conflict of interest. Acknowledgments This research is part of a collaboration project funded by the Department of Science and Technology of Ho Chi Minh City, Vietnam, under grant number 03/2021/HĐ-QKHCN. 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J Pediatr Gastroenterol Nutr 70(2):238–242. https://doi.org/10.1097/MPG.0000000000002564 Fosby M V, Stensrud K J, and Bjornland K (2020) Bowel function after transanal endorectal pull-through for Hirschsprung disease - does outcome improve over time? J Pediatr Surg 55(11):2375–2378. https://doi.org/10.1016/j.jpedsurg.2020.04.010 Zimmer J, Tomuschat C, and Puri P (2016) Long-term results of transanal pull-through for Hirschsprung's disease: a meta-analysis. Pediatr Surg Int 32(8):743–749. https://doi.org/10.1007/s00383-016-3908-z Neuvonen M I, Kyrklund K, Rintala R J, and Pakarinen M P (2017) Bowel Function and Quality of Life After Transanal Endorectal Pull-through for Hirschsprung Disease: Controlled Outcomes up to Adulthood. Ann Surg 265(3):622–629. https://doi.org/10.1097/SLA.0000000000001695 Levitt M A, Martin C A, Olesevich M, Bauer C L, Jackson L E, and Pena A (2009) Hirschsprung disease and fecal incontinence: diagnostic and management strategies. J Pediatr Surg 44(1):271–277; discussion 277. https://doi.org/10.1016/j.jpedsurg.2008.10.053 Tran V Q, Mahler T, Bontems P, Truong D Q, Robert A, Goyens P, and Steyaert H (2018) Interest of Anorectal Manometry During Long-term Follow-up of Patients Operated on for Hirschsprung's Disease. J Neurogastroenterol Motil 24(1):70–78. https://doi.org/10.5056/jnm17019 Till H, Heinrich M, Schuster T, and D V S (2006) Is the anorectal sphincter damaged during a transanal endorectal pull-through (TERPT) for Hirschsprung's disease? A 3-dimensional, vector manometric investigation. Eur J Pediatr Surg 16(3):188–191. https://doi.org/10.1055/s-2006-924220 Hong J (2014) Clinical applications of gastrointestinal manometry in children. Pediatr Gastroenterol Hepatol Nutr 17(1):23–30. https://doi.org/10.5223/pghn.2014.17.1.23 Powers M K, Brown E T, Hogan R M, Martin A D, Ortenberg J, and Roth C C (2015) Trends in Toilet Training and Voiding Habits among Children with Down Syndrome. J Urol 194(3):783–787. https://doi.org/10.1016/j.juro.2015.03.114 Glia A, Gylin M, Akerlund J E, Lindfors U, and Lindberg G (1998) Biofeedback training in patients with fecal incontinence. Dis Colon Rectum 41(3):359–364. https://doi.org/10.1007/BF02237492 Kumar D (2007) An Anatomical Overview. In: Haslam J. and Laycock J. (Eds.), Editors, Therapeutic Management of Incontinence and Pelvic Pain: Pelvic Organ Disorders, Spinger-verlag, Londonpp 187–197. Levitt M A, Dickie B, and Pena A (2010) Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg 19(2):146–153. https://doi.org/10.1053/j.sempedsurg.2009.11.013 Dickie B H, Webb K M, Eradi B, and Levitt M A (2014) The problematic Soave cuff in Hirschsprung disease: manifestations and treatment. J Pediatr Surg 49(1):77–80; discussion 80 – 71. https://doi.org/10.1016/j.jpedsurg.2013.09.034 Soave F (1964) A New Surgical Technique for Treatment of Hirschsprung's Disease. Surgery 56(1007–1014. https://doi.org/ Gad El-Hak N A, El-Hemaly M M, Negm E H, El-Hanafy E A, Abdel Messeh M H, and Abdel Bary H H (2010) Functional outcome after Swenson's operation for Hirshsprung's disease. Saudi J Gastroenterol 16(1):30–34. https://doi.org/10.4103/1319-3767.58765 Tabbers M M, DiLorenzo C, Berger M Y, Faure C, Langendam M W, Nurko S, Staiano A, Vandenplas Y, Benninga M A, European Society for Pediatric Gastroenterology H, Nutrition, and North American Society for Pediatric G (2014) Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr 58(2):258–274. https://doi.org/10.1097/MPG.0000000000000266 Lee T H and Bharucha A E (2016) How to Perform and Interpret a High-resolution Anorectal Manometry Test. J Neurogastroenterol Motil 22(1):46–59. https://doi.org/10.5056/jnm15168 van de Ven T J, Sloots C E, Wijnen M H, Rassouli R, van Rooij I, Wijnen R M, and de Blaauw I (2013) Transanal endorectal pull-through for classic segment Hirschsprung's disease: with or without laparoscopic mobilization of the rectosigmoid? J Pediatr Surg 48(9):1914–1918. https://doi.org/10.1016/j.jpedsurg.2013.04.025 Tomuschat C, Zimmer J, and Puri P (2016) Laparoscopic-assisted pull-through operation for Hirschsprung’s disease: a systematic review and meta-analysis. Pediatric Surgery International 32(8):751–757. https://doi.org/10.1007/s00383-016-3910-5 Gladman M A, Lunniss P J, Scott S M, and Swash M (2006) Rectal hyposensitivity. Am J Gastroenterol 101(5):1140–1151. https://doi.org/10.1111/j.1572-0241.2006.00604.x Makosiej R, Makosiej A, Bossowski A, Kolejwa M, Wawrusiewicz-Kurylonek N, Legowik A, Goscik J, Piaseczna-Piotrowska A, and Czkwianianc E (2020) Dyssynergic Defecation and Anal Sphincter Disorders in Children in High-Resolution Anorectal Manometry Investigation. J Pediatr Gastroenterol Nutr 71(4):484–490. https://doi.org/10.1097/MPG.0000000000002819 Rao S S and Patcharatrakul T (2016) Diagnosis and Treatment of Dyssynergic Defecation. J Neurogastroenterol Motil 22(3):423–435. https://doi.org/10.5056/jnm16060 Rao S S C (2010) Advances in Diagnostic Assessment of Fecal Incontinence and Dyssynergic Defecation. Clinical Gastroenterology and Hepatology 8(11):910–919.e912. https://doi.org/https://doi.org/10.1016/j.cgh.2010.06.004 Wang Y, Liang H, Wu Q, Zheng H, Liu G, Wen Z, Lan M, Yu J, Zhu D, Liang J, Zhang J, Xu X, and Xia H (2017) Bowel management program for pediatric postoperative fecal incontinence in China: A surgeon's experience. Medicine (Baltimore) 96(22):e7078. https://doi.org/10.1097/MD.0000000000007078 Colares J H, Purcaru M, da Silva G P, Frota M A, da Silva C A, Melo-Filho A A, Bischoff A, and Pena A (2016) Impact of the Bowel Management Program on the quality of life in children with fecal incontinence. Pediatr Surg Int 32(5):471–476. https://doi.org/10.1007/s00383-016-3874-5 Levitt M and Pena A (2009) Update on pediatric faecal incontinence. Eur J Pediatr Surg 19(1):1–9. https://doi.org/10.1055/s-2008-1039190 Catena F, Wilkinson K, and Phillips R K (2002) Untreatable faecal incontinence: colostomy or colostomy and proctectomy? Colorectal Dis 4(1):48–50. https://doi.org/10.1046/j.1463-1318.2002.00315.x Tjandra J J, Chan M K, Yeh C H, and Murray-Green C (2008) Sacral nerve stimulation is more effective than optimal medical therapy for severe fecal incontinence: a randomized, controlled study. Dis Colon Rectum 51(5):494–502. https://doi.org/10.1007/s10350-007-9103-5 Brouwer R and Duthie G (2010) Sacral nerve neuromodulation is effective treatment for fecal incontinence in the presence of a sphincter defect, pudendal neuropathy, or previous sphincter repair. Dis Colon Rectum 53(3):273–278. https://doi.org/10.1007/DCR.0b013e3181ceeb22 van Ginkel R, Büller H A, Boeckxstaens G E, van Der Plas R N, Taminiau J A, and Benninga M A (2001) The effect of anorectal manometry on the outcome of treatment in severe childhood constipation: a randomized, controlled trial. Pediatrics 108(1):E9. https://doi.org/10.1542/peds.108.1.e9 Yates G, Friedmacher F, Cleeve S, and Athanasakos E (2021) Anorectal manometry in pediatric settings: A systematic review of 227 studies. Neurogastroenterol Motil 33(4):e14006. https://doi.org/10.1111/nmo.14006 Moore D and Young C J (2020) A systematic review and meta-analysis of biofeedback therapy for dyssynergic defaecation in adults. Tech Coloproctol 24(9):909–918. https://doi.org/10.1007/s10151-020-02230-9 Ratuapli S K, Bharucha A E, Noelting J, Harvey D M, and Zinsmeister A R (2013) Phenotypic identification and classification of functional defecatory disorders using high-resolution anorectal manometry. Gastroenterology 144(2):314–322.e312. https://doi.org/10.1053/j.gastro.2012.10.049 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 17 Feb, 2023 Read the published version in Pediatric Surgery International → Version 1 posted Editorial decision: Accepted 31 Jan, 2023 Reviews received at journal 31 Jan, 2023 Reviewers agreed at journal 31 Jan, 2023 Reviewers invited by journal 31 Jan, 2023 Editor assigned by journal 31 Jan, 2023 Submission checks completed at journal 31 Jan, 2023 First submitted to journal 29 Jan, 2023 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-2527378","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":172013305,"identity":"52da4544-22a4-416b-a4c5-c9a9d6c336be","order_by":0,"name":"Tung Trinh Huu","email":"","orcid":"","institution":"Children's Hospital 2","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Tung","middleName":"Trinh","lastName":"Huu","suffix":""},{"id":172013306,"identity":"8982e6c7-ff6b-43e8-9705-6650f881b593","order_by":1,"name":"Ngoc Minh NGUYEN","email":"","orcid":"","institution":"Children's Hospital 2","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Ngoc","middleName":"Minh","lastName":"NGUYEN","suffix":""},{"id":172013307,"identity":"6abb88e4-8e46-41de-a847-cd978b9da9a8","order_by":2,"name":"Kim Thien LAM","email":"","orcid":"","institution":"Children's Hospital 2","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Kim","middleName":"Thien","lastName":"LAM","suffix":""},{"id":172013308,"identity":"48d818f9-3342-48aa-aa91-dfdb7c80a42e","order_by":3,"name":"Thach Ngoc PHAM","email":"","orcid":"","institution":"Children's Hospital 2","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Thach","middleName":"Ngoc","lastName":"PHAM","suffix":""},{"id":172013309,"identity":"f021389c-ee9d-4f56-b704-a113563055e1","order_by":4,"name":"Nhan Truong VU","email":"","orcid":"","institution":"Children's Hospital 2","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Nhan","middleName":"Truong","lastName":"VU","suffix":""},{"id":172013310,"identity":"04618518-26ff-4d14-92ec-63549659687f","order_by":5,"name":"Linh Nguyen Uy TRUONG","email":"","orcid":"","institution":"Ho Chi Minh City Medicine and Pharmacy University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Linh","middleName":"Nguyen Uy","lastName":"TRUONG","suffix":""},{"id":172013311,"identity":"8d63de20-9b60-43fb-b52b-df000e336b7e","order_by":6,"name":"Viet Quoc TRAN","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABAElEQVRIiWNgGAWjYDCCA8icBAYGObDgA/xaGBuQtBgYgwUTiNbCwGCQ2AC1Difgu32A/XFFzR3ZDcebn0k8qPiTPj/s8EOgLXZyug3YtUieS2BsPHPsmfGGM8fMJBLOGORuvJ1mANSSbGx2ALsWgzMMjI0NbIcTN9zIYTZIbANqmZ0A0nIgcRteLf9gWv4ZpBvOTv9AWEtjG1gL44PEBoMEeekc/LZInmFsnNnYd9h45pljhg8SjhkbbpDOKTiQYIDbL3xnmA98bPh2WLbvePODgz9q5OTlZ6dv/vChwk4OlxYGaLQgIscArNIAl3J0jSAg34Bb1SgYBaNgFIxMAAA+L2xa29xQjgAAAABJRU5ErkJggg==","orcid":"","institution":"Children's Hospital 2","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Viet","middleName":"Quoc","lastName":"TRAN","suffix":""}],"badges":[],"createdAt":"2023-01-30 00:59:16","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-2527378/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-2527378/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1007/s00383-023-05402-4","type":"published","date":"2023-02-17T18:56:54+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":32389263,"identity":"e8d7533e-e56b-45d7-a7cc-7409bd439032","added_by":"auto","created_at":"2023-02-02 16:02:59","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":76120,"visible":true,"origin":"","legend":"\u003cp\u003eIllustrations of anal pressures were reported as mean ± standard deviation (mmHg) for the patients (N=95) versus the references (N=95) when performing anorectal manometry: [A] at resting; [B] at cough maneuver; [C] at squeeze maneuver. The references were matched for sex and year of age at the time of the study.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-2527378/v1/1cfe972c10f5ebb1969cf79a.png"},{"id":32390089,"identity":"5fd972c9-924a-4934-b66b-89fe711fccfd","added_by":"auto","created_at":"2023-02-02 16:10:59","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":101705,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003eIllustrations of maximum tolerated volume (Vmax) reported as mean ± standard deviation (mL) for the patients (N=95) versus the references (N=95) when performing anorectal manometry. Qualitative results were demonstrated according to fecal continence status. Notably, in the operated group, the Vmax values were significantly decreased in the incontinent patients compared to in the continent patients: 135.9 ± 47.9 mL versus 166.6 ± 58.3 mL, respectively, (\u003c/em\u003ep=0.02\u003cem\u003e).\u003c/em\u003e\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-2527378/v1/91fe30d4a2504a6178b3a2ce.png"},{"id":32389261,"identity":"819c82a6-40e7-42c2-9b21-49e359ac1f31","added_by":"auto","created_at":"2023-02-02 16:02:59","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":5713,"visible":true,"origin":"","legend":"\u003cp\u003eThis Image is not available with this version\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-2527378/v1/4074525c92a33915a9b83f71.png"},{"id":44720005,"identity":"291843ab-efae-4eac-80bf-b2708aac1276","added_by":"auto","created_at":"2023-10-16 19:06:08","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":716085,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-2527378/v1/89d7783a-0f8c-43d0-84d7-10d59ea3f580.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Anorectal Manometry Findings in Relation with Long-term Functional Outcomes of The Patients Operated on for Hirschsprung's Disease Compared to The Reference-Based Population","fulltext":[{"header":"Introduction","content":"\u003cp\u003eHirschsprung's disease (HD) is a common pediatric surgery characterized by congenital malformation of the enteric nervous system. The prevalence is about 1 per 5000 live births, and the estimated prevalence of 1.4 per 5000 live births in Asian populations [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Defecation disorders are noticed in a non-negligible number of patients after definitive surgery for HD. Fecal incontinence and constipation are the two main complaints reported in broad ranges of incidence from several to even more than 50% in the literature [\u003cspan additionalcitationids=\"CR3 CR4 CR5\" citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eClinical assessment of fecal continence is often challenging. Continence problems may be due to either sequela of the surgical techniques or functional motility disorders [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. In the literature, authors reported that resection of a part of the large bowel and intra-operative injuries to the anal sphincters could be responsible for postoperative defecation disorders. Otherwise, anorectal function in patients after surgery for HD is still unclear [\u003cspan additionalcitationids=\"CR9 CR10\" citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Clinically, anorectal manometry (AM) could help objectively discriminate between acquired troubles and associated functional disorders [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e, \u003cspan additionalcitationids=\"CR12 CR13\" citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eSo, this study investigated the interests of anorectal manometry and its relationship with long-term bowel function in patients after surgery for HD at Children's Hospital 2 in Ho Chi Minh City, Vietnam.\u003c/p\u003e"},{"header":"Materials And Methods","content":"\u003cp\u003e\u003cstrong\u003e\u003cem\u003eStudy design\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eA cross-sectional study was conducted at Children\u0026apos;s Hospital 2. The hospital\u0026apos;s research ethics committee approved this study under IRB number 747/NĐ2-CĐT.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eOperated patients\u0026nbsp;\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll consecutive charts of patients operated on for HD in our department between January 2015 to January 2020 were retrieved. Data have been collected on gender, date of birth, age at surgery, operative characteristics, and postoperative complications from their medical records stored in the hospital\u0026apos;s electronic database. Patients over four years of age with at least twelve months of postoperative follow-up were contacted and invited to participate in this study. The investigators interviewed patients and their parents to complete the questionnaires about bowel function [15].\u003c/p\u003e\n\u003cp\u003eDuring the interview, a careful clinical examination was done to rule out the possibility of distal anorectal stricture or other anomalies. The patients were asked to perform AM. Manometric findings were collected for analysis. Informed consent was obtained from all participants. Patients with neurological impairments or AM contraindications were excluded from this study.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eOperative procedures: The transanal endorectal pull-through technique was applied for the cases with classic HD forms (recto-sigmoid aganglionosis). In patients with long segments of aganglionosis, a Soave pull-through procedure with an abdominal approach (open Soave) or with laparoscopic assistance was used in our institution. An enterostomy was indicated in cases of severe enterocolitis or failure with nursing care (washout). An ileoanal anastomosis without a reservoir was performed for patients with total colon aganglionosis.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eReference-based population\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe reference-based population was volunteers recruited from the pool of patients of Children\u0026rsquo;s Hospital 2 in Ho Chi Minh City, Vietnam. The inclusion criteria of the references were without any antecedent digestive surgical intervention. They were asked to complete an AM and provided with all information regarding the study aims, objectives, and possible anorectal HRAM adverse effects. All participants and their parents/ relatives provided written consent before participating in the study. The references were matched with the cases by the year of age and gender with a ratio of 1:1. Before AM tests, the clinical and digital rectal examinations were indicated to ensure that participants were free from any anorectal disorders or diseases. Data have been collected on gender, date of birth, bowel function, and AM findings.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eBowel function\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eBowel function was investigated using variables related to clinical aspects of the patient\u0026rsquo;s stooling patterns, such as fecal incontinence and constipation, as defined in the following paragraphs.\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eFecal continence\u003c/em\u003e was based on the Wingspread classification, which defines the following four levels of continence: \u003cem\u003eexcellent\u003c/em\u003e: clean (totally continent, toilet trained with no medication); \u003cem\u003egood\u003c/em\u003e: staining (rarely soiling, except during stressful exercise); \u003cem\u003efair\u003c/em\u003e: intermittent fecal soiling, urge incontinence; \u003cem\u003epoor:\u003c/em\u003e constant fecal soiling or smearing [16]. Patients classified as \u0026ldquo;\u003cem\u003efair\u003c/em\u003e\u0026rdquo; or \u0026ldquo;\u003cem\u003epoor\u003c/em\u003e\u0026rdquo; were considered fecally incontinent; the other categories were considered fecally continent. We considered that fecal continence was commonly acquired in children older than four\u0026nbsp;[17].\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eConstipation\u003c/em\u003e: Patients were studied for stooling frequency, laxatives, and enema over the months preceding the investigation. A patient was considered suffering from constipation if defecation was only possible with laxatives or occurred less than three times per week according to the ROME IV criteria for functional constipation [18].\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eEquipment and preparations\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWater-perfused high-resolution anorectal manometry with eight channels was applied; using the MMS\u0026reg; database software (version 9.3k, copyright (c) 1988-2015 by Medical Measurement System). Rectal enema is routinely before the tests. Patients were in the supine position, their hips and knees flexed, and asked to get familiar with the test. The sequence of the test was as follows:\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eProfile of manometric measurements\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eAnal resting pressure\u003c/em\u003e (mmHg) was measured for one minute at rest.\u0026nbsp;The length of the high-pressure zone (HPZ) on AM, considered the anal canal length, was calculated.\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eAnal pressure at coughing maneuver\u0026nbsp;\u003c/em\u003e(mmHg)\u003cem\u003e-\u003c/em\u003e three single coughs, separated by a thirty-second rest period, were performed.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eAnal pressure at squeeze maneuver\u003c/em\u003e (mmHg) - three squeezes of five-second duration, separated by a thirty-second rest period, were recorded. The maximal time enabled to perform an\u003cem\u003e\u0026nbsp;endurance squeeze\u003c/em\u003e \u003cem\u003emaneuver\u003c/em\u003e was recorded (seconds).\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eRecto-anal inhibitory reflex (RAIR)\u003c/em\u003e\u003cstrong\u003e\u0026nbsp;-\u003c/strong\u003e the balloon was inflated within 3 - 5 seconds and deflated; the initial volume was 10 ml and was subsequently increased by increments of 10 ml for the following times. This test was repeated several times to detect the presence of the RAIR. A drop of at least 25% of resting pressure has to occur with subsequent restoration to at least two-thirds of resting pressure for it to be deemed present\u0026nbsp;[7].\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eSensation test:\u003c/em\u003e the balloon was inflated at a rate of 5 ml every 5 seconds, and the patient was asked to report consequently: the first sensation, urge to defecate, and finally, a maximum tolerated volume, and the sequential volumes were noted (mL)\u0026nbsp;[11].\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eBalloon expulsion maneuver:\u003c/em\u003e the balloon was filled with about 20 ml of air, and the patient asked to try to strain the balloon. While the patient tried evacuating the balloon, we measured the anal sphincter pressure. The anal sphincters (internal and external) must normally relax during defecation. Failure to evacuate the balloon with increased abnormal anal sphincter pressure (IAAP) indicates dyssynergic defecation\u0026nbsp;[11].\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eData analysis and statistics\u0026nbsp;\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eData are reported as mean and standard deviation (SD) or as median and range for continuous variables according to the normality of the distribution and as number and proportion for discrete data. Chi-square or Fisher exact tests were used to compare categorical data. Independent \u003cem\u003et\u003c/em\u003e-tests were used to compare means of two groups. All tests were two-sided, and a p-value \u0026lt; 0.05 was considered significant. IBM SPSS Statistics version 20 (SPSS Chicago, IL) was used for statistical analysis.\u003c/p\u003e"},{"header":"Results","content":"\u003cp\u003eIn total, 95 interviewed patients who fulfilled the inclusion criteria and underwent AM were enrolled in this study. All the cases had histopathological results confirming normal ganglionic innervation at the level of anastomosis. Rectosigmoid aganglionosis was the most seen form of HD, representing 69.5% (66 out of 95) of cases operated with TERPT. Otherwise, 30.5% (29 out of 95) of patients had long segment HD that required abdominal assisted surgery, including 16 patients with open Soave and 13 with LATEP. On the other hand, 95 references were also recruited in this study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003ePatient characteristics\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eTable 1 shows patient characteristics and their association with either fecal continence or constipation at investigation. The mean age of the patients at investigation was 6.6 \u0026plusmn; 2.2 years, with 68% male. The mean age of the patients at surgery was 3.3 \u0026plusmn; 2.2 years. The mean follow-up time was 3.4 \u0026plusmn; 1.9 years.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable\u0026nbsp;\u003c/strong\u003e\u003cstrong\u003e1\u003c/strong\u003e. Clinical characteristics and their association with fecal incontinence or constipation, identified by investigators at the last follow-up of the patients operated on for Hirschsprung\u0026rsquo;s disease (N=95)\u003c/p\u003e\n\u003ctable border=\"1\" cellpadding=\"0\" cellspacing=\"0\" width=\"672\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd width=\"32.291666666666664%\"\u003e\n \u003cp\u003e\u003cstrong\u003eVariables\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e\u003cstrong\u003eAll\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003eN\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"18.75%\"\u003e\n \u003cp\u003e\u003cstrong\u003ePrevalence of\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003efecal incontinence (\u003c/strong\u003en/N, %)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003eP\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e\u003cstrong\u003ePrevalence of\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003econstipation\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e(n/N, %)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"11.011904761904763%\"\u003e\n \u003cp\u003eP\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"6\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eAge at investigation,\u003c/strong\u003e (years)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e(Mean \u0026plusmn;\u0026nbsp;SD)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e6.6 \u0026plusmn; 2.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"18.75%\"\u003e\n \u003cp\u003e6.1 \u0026plusmn; 1.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e7.8\u0026nbsp;\u0026plusmn; 3.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"11.011904761904763%\"\u003e\n \u003cp\u003e\u003cstrong\u003e-\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"6\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eAge groups, (n, %)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;4 - \u0026lt;10 years\u003c/li\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;10 - 16 years\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e85\u003c/p\u003e\n \u003cp\u003e10\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" width=\"18.75%\"\u003e\n \u003cp\u003e23 (27.1)\u003c/p\u003e\n \u003cp\u003e1 (10.0)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.005\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e10 (11.8)\u003c/p\u003e\n \u003cp\u003e2 (20.0)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"11.011904761904763%\"\u003e\n \u003cp\u003e0.6\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"6\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eAge at surgery, (\u003c/strong\u003eyears)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e(Mean \u0026plusmn; SD)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e3.3 \u0026plusmn; 2.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"18.75%\"\u003e\n \u003cp\u003e3.0 \u0026plusmn; 1.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e3.9 \u0026plusmn; 3.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"11.011904761904763%\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e\u003cstrong\u003eFollow-up time, (years)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"18.75%\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"11.011904761904763%\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e(Mean \u0026plusmn;\u0026nbsp;SD)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e3.4 \u0026plusmn; 1.9\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"18.75%\"\u003e\n \u003cp\u003e3.2\u0026nbsp;\u0026plusmn; 1.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003e\u003cstrong\u003e-\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e3.7\u0026nbsp;\u0026plusmn; 2.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"11.011904761904763%\"\u003e\n \u003cp\u003e\u003cstrong\u003e-\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"6\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eGender, (n, %)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Male\u003c/li\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Female\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e46\u003c/p\u003e\n \u003cp\u003e49\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" width=\"18.75%\"\u003e\n \u003cp\u003e10 (21.7)\u003c/p\u003e\n \u003cp\u003e14 (28.6)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003e\u0026gt;9.99\u003csup\u003e*\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e6 (13.0)\u003c/p\u003e\n \u003cp\u003e6 (12.2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"11.011904761904763%\"\u003e\n \u003cp\u003e0.4\u003csup\u003e*\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"6\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eEnterostomy,\u0026nbsp;\u003c/strong\u003e\u003cstrong\u003e(n, %)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Yes\u003c/li\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;No\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e10\u003c/p\u003e\n \u003cp\u003e85\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" width=\"18.75%\"\u003e\n \u003cp\u003e4 (40.0)\u003c/p\u003e\n \u003cp\u003e20 (23.5)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003e0.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e2 (20.0)\u003c/p\u003e\n \u003cp\u003e10 (11.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"11.011904761904763%\"\u003e\n \u003cp\u003e0.6\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"6\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eOperative procedure,\u0026nbsp;\u003c/strong\u003e(n, %)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e\u0026bull; TERPT\u003c/li\u003e\n \u003cp\u003e\u0026bull; LATEP + Open Soave \u003csup\u003e(*)\u003c/sup\u003e\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e66\u003c/p\u003e\n \u003cp\u003e29\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"18.75%\"\u003e\n \u003cp\u003e16 (24.2)\u003c/p\u003e\n \u003cp\u003e8 (27.6)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003e0.52\u003csup\u003e*\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e5 (7.6)\u003c/p\u003e\n \u003cp\u003e7 (24.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"11.011904761904763%\"\u003e\n \u003cp\u003e0.12\u003csup\u003e*\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"6\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eResected segment\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e\u0026bull; Rectosigmoid colon\u003c/li\u003e\n \u003cp\u003e\u0026bull; Descending colon\u003c/li\u003e\n \u003cp\u003e\u0026bull; Transversal colon\u003c/li\u003e\n \u003cp\u003e\u0026bull; Total colon\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e66\u003c/p\u003e\n \u003cp\u003e21\u003c/p\u003e\n \u003cp\u003e6\u003c/p\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"18.75%\"\u003e\n \u003cp\u003e16 (24.2)\u003c/p\u003e\n \u003cp\u003e4 (19.0)\u003c/p\u003e\n \u003cp\u003e2 (33.3)\u003c/p\u003e\n \u003cp\u003e2 (100)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"8.035714285714286%\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e5 (7.6)\u003c/p\u003e\n \u003cp\u003e4 (19.0)\u003c/p\u003e\n \u003cp\u003e3 (50.0)\u003c/p\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"11.011904761904763%\"\u003e\n \u003cp\u003e\u0026bull; \u0026nbsp;\u003c/li\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"6\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eComplications,\u0026nbsp;\u003c/strong\u003e\u003cstrong\u003e(n, %)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"32.291666666666664%\"\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Anastomotic leak\u003c/li\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Bowel obstruction\u003c/li\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Residual aganglionosis\u003c/li\u003e\n \u003cp\u003e\u0026bull; Total complications \u003csup\u003e(#)\u003c/sup\u003e\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.267857142857142%\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003cp\u003e7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"18.75%\"\u003e\n \u003cp\u003e3 (60.0)\u003c/p\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003cp\u003e3 (42.9)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.035714285714286%\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e0.36\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"19.642857142857142%\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003cp\u003e2 (100)\u003c/p\u003e\n \u003cp\u003e2 (28.6)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"11.011904761904763%\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e0.64\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003cem\u003eAbbreviation: SD, standard deviation;\u0026nbsp;\u003c/em\u003e\u003cem\u003eTEPRT, transanal endorectal pull-through without abdominal assistance;\u0026nbsp;\u003c/em\u003e\u003cem\u003eLATEP, laparoscopically assisted trans-anal endo-rectal pull-through procedure.\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003e\u003csup\u003e(#)\u003c/sup\u003e\u003c/em\u003e\u003cem\u003e\u0026nbsp;Data for postoperative complications reported by the number of encountered cases.\u0026nbsp;\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003e\u003csup\u003e(*)\u003c/sup\u003e\u003c/em\u003e\u003cem\u003e\u0026nbsp;Chi-squared test,\u0026nbsp;\u003c/em\u003e\u003cem\u003eotherwise Fisher\u0026rsquo;s exact test.\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eNote: Bold values (p \u0026lt; 0.05) were considered as statistically significant.\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eBowel function\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe patient chart review and a thorough medical history taken during the interview showed that 25.3% (24 out of 95) of the patients clinically presented with fecal incontinence and 12.6% (12 out of 95) of other patients. In contrast, the prevalence of fecal incontinence and constipation were 2.1% (2 out of 95) and 4.2% (4 out of 95), respectively, in the references, as shown in Table 2. Notably, the prevalence of these two symptoms was statistically higher in the patients than in the references.\u003c/p\u003e\n\u003cp\u003eRegarding fecal incontinence, the prevalence was significantly higher in the patients younger than ten years compared with the older groups. Otherwise, none of the other characteristics of the patients were significantly associated with the prevalence of fecal incontinence (Table 1). Regarding constipation, the prevalence was not significantly different according to all characteristics of the patients (Table 1).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable\u0026nbsp;\u003c/strong\u003e\u003cstrong\u003e2\u003c/strong\u003e. Patients\u0026rsquo; characteristics and defecation patterns of the patients operated on for Hirschsprung\u0026rsquo;s disease (N=95) versus the references (N=95) who underwent an anorectal manometry\u003c/p\u003e\n\u003ctable border=\"1\" cellpadding=\"0\" cellspacing=\"0\" width=\"670\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd width=\"37.014925373134325%\"\u003e\n \u003cp\u003e\u003cstrong\u003eClinical characteristics\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"28.208955223880597%\"\u003e\n \u003cp\u003e\u003cstrong\u003ePatients\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e(N=95)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"24.029850746268657%\"\u003e\n \u003cp\u003e\u003cstrong\u003eReference\u003c/strong\u003e\u003cstrong\u003es\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e(N=95)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.746268656716419%\"\u003e\n \u003cp\u003e\u003cstrong\u003eP-value\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"4\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eAge,\u0026nbsp;\u003c/strong\u003e(year)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"37.014925373134325%\"\u003e\n \u003cp\u003e(Mean\u0026nbsp;\u0026plusmn;\u0026nbsp;SD)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"28.208955223880597%\"\u003e\n \u003cp\u003e6.6 \u0026plusmn; 2.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"24.029850746268657%\"\u003e\n \u003cp\u003e7.2 \u0026plusmn; 2.9\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"10.746268656716419%\"\u003e\n \u003cp\u003e0.17\u003csup\u003e*\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"4\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eAge groups,\u0026nbsp;\u003c/strong\u003e(n,\u0026nbsp;n/N\u0026nbsp;%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"37.014925373134325%\"\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;4 - \u0026lt;10 years\u003c/li\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;10 - 16 years\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"28.208955223880597%\"\u003e\n \u003cp\u003e69 (72.6)\u003c/p\u003e\n \u003cp\u003e26 (27.4)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"24.029850746268657%\"\u003e\n \u003cp\u003e69 (72.6)\u003c/p\u003e\n \u003cp\u003e26 (27.4)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"10.746268656716419%\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"4\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eGender,\u0026nbsp;\u003c/strong\u003e(n, n/N %)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"37.014925373134325%\"\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Male\u003c/li\u003e\n \u003cp\u003e\u0026bull; Female\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"28.208955223880597%\"\u003e\n \u003cp\u003e49 (68.0)\u003c/p\u003e\n \u003cp\u003e46 (32.0)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"24.029850746268657%\"\u003e\n \u003cp\u003e49 (68.0)\u003c/p\u003e\n \u003cp\u003e46 (32.0)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"10.746268656716419%\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"4\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eConstipation,\u0026nbsp;\u003c/strong\u003e(n,\u0026nbsp;n/N\u0026nbsp;%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"37.014925373134325%\"\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Yes\u003c/li\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;No\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"28.208955223880597%\"\u003e\n \u003cp\u003e12 (12.6)\u003c/p\u003e\n \u003cp\u003e83 (87.4)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"24.029850746268657%\"\u003e\n \u003cp\u003e4 (4.2)\u003c/p\u003e\n \u003cp\u003e91 (95.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"10.746268656716419%\"\u003e\n \u003cp\u003e0.07\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"4\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eFecal continence \u003csup\u003e#\u003c/sup\u003e\u003c/strong\u003e,\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e(n,\u0026nbsp;n/N\u0026nbsp;%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"37.014925373134325%\"\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Excellent\u003c/li\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Good\u003c/li\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Fair\u003c/li\u003e\n \u003cp\u003e\u0026bull; Poor\u003c/li\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"28.208955223880597%\"\u003e\n \u003cp\u003e37 (38.9)\u003c/p\u003e\n \u003cp\u003e34 (35.8)\u003c/p\u003e\n \u003cp\u003e6 (6.4)\u003c/p\u003e\n \u003cp\u003e18 (18.9)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"24.029850746268657%\"\u003e\n \u003cp\u003e85 (89.5)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;8 (84.)\u003c/p\u003e\n \u003cp\u003e2 (2.1)\u003c/p\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"10.746268656716419%\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003cem\u003eAbbreviation: SD, standard deviation.\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003e\u003csup\u003e(#)\u0026nbsp;\u003c/sup\u003e\u003c/em\u003e\u003cem\u003eFecal continence levels according to the Wingspread\u0026rsquo;s classification.\u0026nbsp;\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003e\u003csup\u003e(*)\u003c/sup\u003e\u003c/em\u003e\u003cem\u003e\u0026nbsp;Independent t-test,\u0026nbsp;\u003c/em\u003e\u003cem\u003eotherwise Fisher\u0026rsquo;s exact test.\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eNote: Bold values (p \u0026lt; 0.05) were considered as statistically significant.\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eManometric findings\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eTable 3 shows the manometric findings of the patients compared with the references.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAnal pressures\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe anal resting pressure was significantly lower in the patients than in the references (53.2 \u0026plusmn; 16.1 mmHg versus 62.2 \u0026plusmn; 14.0 mmHg; p\u0026lt;0.05), as shown in Figure 1. Moreover, in the operated group, the anal resting pressure was significantly decreased in the incontinent patients than in the continent patients (46.0 \u0026plusmn; 10.6 mmHg versus 55.6 \u0026plusmn; 16.9 mmHg; p\u0026lt;0.05).\u003c/p\u003e\n\u003cp\u003eWhen performing cough maneuvers and squeeze maneuvers, the anal pressures were significantly lower in the patients than in the references. However, the difference was not significant between the two subgroups: incontinent and continent patients in the operated group (p\u0026gt;0.05), as shown in Table 3.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eEndurance squeeze test (time completed)\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eMeasurements of time completed during the endurance squeeze test had significantly decreased in the patients than in the references (p\u0026lt;0.05). In contrast, there was no significant difference in time completed between the incontinent and the continent patients in the operated group (p\u0026gt;0.05) (Table 3).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAnal canal length (high-pressure zone)\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe length of HPZ on AM, considered the anal canal length, was significantly longer in the patients than in the references (2.2 \u0026plusmn; 0.3 cm versus 2.1 \u0026plusmn; 0.2 cm, \u003cem\u003ep\u0026lt;0.05\u003c/em\u003e). However, there was no significant difference in the length of the anal canal between the incontinent and the continent patients in the operated group (2.2 \u0026plusmn; 0.3 cm versus 2.2 \u0026plusmn; 0.2cm, p\u0026gt;0.05).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eRectal anal inhibitor reflex\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eFifty of 95 patients (52.6%) showed a re-establishment of RAIR. In contrast, RAIR was normally reported in all the references.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eSensation tests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe volumes of the first sensation, urge to defecate, and Vmax during the sensation tests showed no significant difference in the patients from that in the references (p\u0026gt;0.05), as shown in Table 3. Whereas, in the operated group, the incontinent patients had shown significantly decreased volumes regarding the first sensation, urge to defecate, and Vmax than that of the continent patients (p\u0026lt;0.05) (Table 3). The qualitative results of the Vmax values are illustrated in Figure 3, with a significant decrease in the Vmax values in the incontinent patients compared to the other groups. \u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eDyssynergic defecation\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe incidence of IAAP was higher in the patients than in the references, but without significant difference. In addition, in the operated group, there was no significant difference between the continent and the incontinent patients (p\u0026gt;0.05).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable\u0026nbsp;\u003c/strong\u003e\u003cstrong\u003e3\u003c/strong\u003e\u003cstrong\u003e.\u003c/strong\u003e Manometric findings according to fecal continence status of the operated patient (N=95) versus the references (N=95)\u003c/p\u003e\n\u003cdiv align=\"center\"\u003e\n \u003ctable border=\"1\" cellpadding=\"0\" cellspacing=\"0\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd rowspan=\"2\" width=\"28.7856071964018%\"\u003e\n \u003cp\u003e\u003cstrong\u003eManometric findings\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd colspan=\"4\" valign=\"top\" width=\"49.92503748125937%\"\u003e\n \u003cp\u003e\u003cstrong\u003eOperated patients\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd rowspan=\"2\" valign=\"top\" width=\"12.593703148425787%\"\u003e\n \u003cp\u003e\u003cstrong\u003eReferences\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e(N=95)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd rowspan=\"2\" width=\"8.695652173913043%\"\u003e\n \u003cp\u003eP\u003csup\u003e*\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.44311377245509%\"\u003e\n \u003cp\u003eAll\u003c/p\u003e\n \u003cp\u003e(N=95)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"28.44311377245509%\"\u003e\n \u003cp\u003eContinence\u003c/p\u003e\n \u003cp\u003e(n=71)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cp\u003eIncontinence\u003c/p\u003e\n \u003cp\u003e(n=24)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"14.37125748502994%\"\u003e\n \u003cp\u003eP\u003csup\u003e#\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"7\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eAnal pressure\u0026nbsp;\u003c/strong\u003e\u003cem\u003e(mean\u003c/em\u003e\u003cem\u003e\u0026nbsp;\u003c/em\u003e\u003cem\u003e\u0026plusmn;\u003c/em\u003e\u003cem\u003e\u0026nbsp;SD,\u0026nbsp;\u003c/em\u003e\u003cem\u003emmHg)\u003c/em\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cul\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;At resting\u003c/li\u003e\n \u003c/ul\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e53.2 \u0026plusmn; 16.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e55.6 \u0026plusmn; 16.9\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e46.0 \u0026plusmn; 10.6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.004\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e62.2 \u0026plusmn; 14.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cul\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Cough maneuver\u003c/li\u003e\n \u003c/ul\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e126.4 \u0026plusmn; 34.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e123.7 \u0026plusmn; 34.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e134.3 \u0026plusmn; 35.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e0.20\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e139.8 \u0026plusmn; 43.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.02\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cul\u003e\n \u003cp\u003e\u0026bull; Squeeze maneuver\u003c/li\u003e\n \u003c/ul\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e111.8 \u0026plusmn; 38.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e115.2 \u0026plusmn; 39.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e101.5 \u0026plusmn; 34.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e0.13\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e126.7 \u0026plusmn; 38.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.01\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"7\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eAnal canal length\u0026nbsp;\u003c/strong\u003e\u003cem\u003e(high-pressure zone, cm)\u003c/em\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cp\u003e(mean\u0026nbsp;\u0026plusmn;\u0026nbsp;SD)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e2.2 \u0026plusmn; 0.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e2.2 \u0026plusmn; 0.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e2.2 \u0026plusmn; 0.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e0.54\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e2.1 \u0026plusmn; 0.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.02\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"7\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eEndurance squeeze test\u0026nbsp;\u003c/strong\u003e\u003cem\u003e(\u003c/em\u003e\u003cem\u003etime completed, second)\u003c/em\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cp\u003e(mean\u0026nbsp;\u0026plusmn;\u0026nbsp;SD)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e21.2 \u0026plusmn; 7.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e21.4 \u0026plusmn; 7.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e20.5 \u0026plusmn; 6.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e0.6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e24.1 \u0026plusmn; 6.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.005\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"7\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eRecto-anal inhibitory reflex\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cp\u003e(n, n/N %)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e50 (52.6)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e38 (53.5)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e12 (50.0)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e0.77\u003csup\u003e\u0026beta;\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e95 (100)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"7\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eSensation test\u0026nbsp;\u003c/strong\u003e\u003cem\u003e(mean\u0026nbsp;\u003c/em\u003e\u003cem\u003e\u0026plusmn;\u003c/em\u003e\u003cem\u003e\u0026nbsp;SD, mL)\u003c/em\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cul\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;First urge\u003c/li\u003e\n \u003c/ul\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e93.5 \u0026plusmn; 44.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e100.3 \u0026plusmn; 45.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e71.8 \u0026plusmn; 93.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.01\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e100.9 \u0026plusmn; 44.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e0.26\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cul\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Urge to defecate\u003c/li\u003e\n \u003c/ul\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e128.5 \u0026plusmn; 54.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e138.2 \u0026plusmn; 53.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e97.7 \u0026plusmn; 46.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.002\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e143.5 \u0026plusmn; 63.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e0.09\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cul\u003e\n \u003cp\u003e\u0026bull; \u0026sect;\u0026nbsp;Vmax\u003c/li\u003e\n \u003c/ul\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e159.4 \u0026plusmn; 57.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e166.6 \u0026plusmn; 58.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e135.9 \u0026plusmn; 47.9\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.02\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e172.7 \u0026plusmn; 63.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e0.22\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"7\" valign=\"top\" width=\"100%\"\u003e\n \u003cp\u003e\u003cstrong\u003eIAAP\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" width=\"28.74251497005988%\"\u003e\n \u003cp\u003e(n, n/N%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e21 (22.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.221556886227544%\"\u003e\n \u003cp\u003e14 (66.7)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"14.37125748502994%\"\u003e\n \u003cp\u003e7 (29.2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"7.18562874251497%\"\u003e\n \u003cp\u003e0.4\u003csup\u003e\u0026beta;\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"12.574850299401197%\"\u003e\n \u003cp\u003e14 (14.7)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" width=\"8.682634730538922%\"\u003e\n \u003cp\u003e0.26\u003csup\u003e\u0026beta;\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n \u003c/table\u003e\n\u003c/div\u003e\n\u003cp\u003e\u003cem\u003eAbbreviation: SD, standard deviation;\u0026nbsp;\u003c/em\u003e\u003cem\u003eIAAP, increased abnormal anal sphincters pressures when performing balloon expulsion test;\u0026nbsp;\u003c/em\u003e\u003cem\u003eVmax, maximum tolerated volume.\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003e\u003csup\u003e(*)\u003c/sup\u003e\u003c/em\u003e\u003cem\u003e\u003csup\u003e\u0026nbsp;\u003c/sup\u003e\u003c/em\u003e\u003cem\u003eP-value of the comparisons between the references and the operated patients\u003c/em\u003e\u003cem\u003e;\u0026nbsp;\u003c/em\u003e\u003cem\u003e\u003csup\u003e(#)\u003c/sup\u003e\u003c/em\u003e\u003cem\u003e\u003csup\u003e\u0026nbsp;\u003c/sup\u003e\u003c/em\u003e\u003cem\u003eP-value of the comparisons between the\u0026nbsp;\u003c/em\u003e\u003cem\u003etwo\u003c/em\u003e\u003cem\u003e\u0026nbsp;subgroups of the operated\u003c/em\u003e\u003cem\u003e\u0026nbsp;group\u003c/em\u003e\u003cem\u003e: continent and incontinent\u003c/em\u003e\u003cem\u003e\u0026nbsp;\u003c/em\u003e\u003cem\u003epatients\u003c/em\u003e\u003cem\u003e;\u003c/em\u003e\u003cem\u003e\u0026nbsp;\u003csup\u003e(\u0026beta;)\u0026nbsp;\u003c/sup\u003eFisher\u0026rsquo;s exact test, otherwise\u003csup\u003e\u0026nbsp;\u003c/sup\u003eindependent\u0026nbsp;t-test.\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eNote: Bold values (p \u0026lt; 0.05) were considered as statistically significant. Fecal continence status according to the Wingspread\u0026rsquo;s classification.\u003c/em\u003e\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eAfter a pull-through surgery, HD patients are at high risk for defecation disorders [19, 20]. Consequently, these disorders could negatively impact their emotional and social development [15, 21-23]. Therefore, the long-term outcomes of these patients remain key points in postoperative management. However, the assessment of bowel function is difficult to objectify, especially in long-term follow-ups [24, 25].\u003c/p\u003e\n\u003cp\u003e\u003cem\u003e\u003cstrong\u003eLong-term outcomes\u003c/strong\u003e\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eThis cohort series reported long terms outcomes of the patients operated on for HD with an average follow-up time was 3.4 \u0026plusmn; 1.9 years. As a result, the prevalence of fecal incontinence in patients with HD was 25.3%, compared with 2.1% in the references. This result was comparable to\u0026nbsp;Neuvonen\u0026nbsp;\u003cem\u003eet al.\u003c/em\u003e reporting that in Finland, 25% of patients presented socially with fecal incontinence versus 2% in the controls\u0026nbsp;[26]. A systematic review by Ying Dai et al. reported that the pooled prevalence of fecal incontinence was 20% (95% CI: 13% - 28%)\u0026nbsp;[19]. In contrast, constipation was less common than continence problems, as reported in the literature and in this study as well. In our series, the prevalence of constipation was 12.6% for the patients compared with 4.2% for the references. According to Neuvonen \u003cem\u003eet al.\u003c/em\u003e, the constipation rate was 5% in patients versus 4% in controls\u0026nbsp;[26]. Ying Dai \u003cem\u003eet al.\u003c/em\u003e reported the pooled prevalence of constipation was 14% (95% CI: 6% - 25%)\u0026nbsp;[19].\u003c/p\u003e\n\u003cp\u003eDiscordance of these outcomes between the studies may be explained by differences in the definition of defecation disorders and the exactitude of clinical assessment\u0026nbsp;[15]. These issues may result from either dysmotility of the proximal pulled-through colon or anatomical changes in the anorectal structure. As suggested by several authors, AM has been introduced as a helpful tool to assess patients with persistent continence problems, allowing for individualized lesions in each patient\u0026nbsp;[11, 27-29].\u003c/p\u003e\n\u003cp\u003eIn this study, we used AM to investigate the anal sphincter function, the neo-rectum, and the associated functional troubles if persistent of the patients compared with the references. There are few reports about AM during follow-up of children operated on for HD, and the role of AM seems still controversial\u0026nbsp;[8-11]. One point force of this study was the establishment of manometric data in the reference-based population that is still lacking, especially the data on children. That helped to discriminate the changes of manometric parameters in the operated group compared to the references\u0026nbsp;[30].\u003c/p\u003e\n\u003cp\u003e\u003cem\u003e\u003cstrong\u003eAnal sphincters pressures and anal canal length\u003c/strong\u003e\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eIn this report, the anal resting pressure of the patients was significantly lower than in the references. In addition, the incontinent patients had significantly lower anal resting pressure than continent patients in the operated group (Figure 1)\u0026nbsp;[31]. Our report shows, as described in the literature that patients with severe fecal incontinence present significantly lower resting and squeeze pressure than continent subjects\u0026nbsp;[32, 33]. That could be due to partial or complete destruction of the anal sphincters due to intraoperative overstretching of the anus during surgery\u0026nbsp;[29].\u003c/p\u003e\n\u003cp\u003eOtherwise, some authors have raised a hypothesis that the problematic Soave cuff could lead to postoperatively obstructed symptoms in patients with the Soave procedure [34]. The Soave procedure - a worldwide used technique as well as in our series - was explicitly to leave a muscular cuff outside the rectal wall when performing an endorectal dissection. Consequently, the longer the cuff, the higher incidence of obstructive symptoms, whereas the shorter the cuff, the higher incidence of fecal incontinence [28]. However, in this cohort series, the anal sphincter pressures seemed to be impaired in the patients compared to the references, as shown in Table 3 [35, 36]. On the other hand, the length of the HPZ was longer in the patients than in the references. Nevertheless, there was no significant difference in the length of HPZ between the incontinent and the continent patients (Table 3).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eRectal anal inhibitor reflex\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe RAIR is thoughtfully absent during follow-up of the patients operated on for HD as its pathophysiology [11]. However, some authors recently reported the reappearance of RAIR in patients after an operation for HD [9, 12, 28]. Gad El-Hak \u003cem\u003eet al.\u003c/em\u003e reported RAIR becoming intact in 11.5% of HD patients four years after surgery and improving rectoanal sensation [37]. Although, this phenomenon did not significantly associate with clinical fecal continence of the patients [9, 28]. In this series, we noted that 50 patients (52.6%) got their RAIR back. Again, the re-establishment of RAIR was also not correlated to any positive long-term outcome in our series (Table 1). However, those who re-established RAIR were older patients, suggesting a quite normal rectal function as an adaptation of the neo-rectum in long-term follow-up. A false RAIR re-establishment due to technical issues when performing an AM should always be considered [38].\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eSensation tests\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eConcerning continence pathophysiology, the rectal sensation is the essential determinant. This finding was studied by measuring rectal distention during sensation tests (e.g., the first sensation, the urge to defecate, and the Vmax) [39]. Although we could not measure a precise rectal compliance index in this study, the Vmax indirectly provides helpful information about the elasticity and reserve capacity of the neo-rectum. Our results reported a significant decrease in the Vmax of the incontinent patients compared to the continent patients in the operated group (Figure 2). However, there was no significant difference in Vmax between the references and the operated patients (p\u0026gt;0.05) (Table 3). The hypothesis is that fecal incontinence could be due to the Vmax values being significantly impaired or below average (\u003cem\u003eincreased perception\u003c/em\u003e)\u0026nbsp;[28, 33]. Of note, the average values of Vmax adapted by Meinds \u003cem\u003eet al.\u003c/em\u003e were 135 mL in children six years of age and 160 mL in children twelve years of age\u0026nbsp;[33].\u0026nbsp;In our series,\u0026nbsp;the Vmax value of the references was 172.7 \u0026plusmn; 63.5 mL compared to\u0026nbsp;135.9 \u0026plusmn; 47.9 mL in the incontinent patients (Table 3). Clinically, we observed that these patients with severe reduction of Vmax\u0026nbsp;presented\u0026nbsp;with a high degree of fecal incontinence, as shown in Figure 2. These patients were considered rectal\u003cem\u003e\u0026nbsp;hypersensitivity\u003c/em\u003e or \u003cem\u003ehypo-compliance\u003c/em\u003e. So, in cases with increased perception, the reservoir function of the neorectum is significantly impaired, leading to the uncontrolled leakage of the stool. Of course, we must rule out any possible inflammation, e.g., proctitis or irritable bowel syndrome\u0026nbsp;[39].\u0026nbsp;Otherwise, this problem could also be due to surgical issues, such as a colon twist or stretching on the inferior mesenteric artery during colon lowering, which is responsible for chronic poor vascularization of the descended colon\u0026nbsp;[15, 40]. We advocate therefore the routine use of laparoscopy in releasing the tension of mesenteries during pull-through surgery and avoiding the torsion of the pull-through colon by laparoscopic control\u0026nbsp;[41].\u003c/p\u003e\n\u003cp\u003eIn contrast, rectal \u003cem\u003ehyposensitivity,\u003c/em\u003e or \u003cem\u003ehyper-compliance\u003c/em\u003e, also known as a \u003cem\u003edecreased perception\u003c/em\u003e, is another complex problem in which their Vmax values were noted with very high volumes but without or significant impairment of the sensation to defecate during the sensation tests. Biofeedback training can improve rectal sensation in patients with decreased perception [42]. Overall, in this series, the increased perception was likely more apparent in the incontinent patients who presented a poor prognosis with severe and persistent fecal incontinence (Figure 2). Furthermore, as concluded in this study, fecal incontinence is a more common problem than constipation.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eDyssynergic defecation\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe etiology of dyssynergic defecation is still unknown. Dyssynergic defecation occurs due to the sphincter apparatus\u0026apos;s relaxation disorders and inadequate intra-rectal pressure during defecation\u0026nbsp;[43]. Meinds \u003cem\u003eet al.\u003c/em\u003e reported that AM could help to visualize dyssynergic defecation\u0026nbsp;[11]. A shortcoming of the AM system used in this study was not measuring the pressure gradients between intra-rectum versus anal sphincters to demonstrate the type of dyssynergia. Our series reported that 22.1% (21 out of 95 patients) with a paradoxical increase in anal sphincter pressures (IAAP). The IAAP phenomenon could represent type I or II of dyssynergic defecation during the balloon expulsion test (Table 3)\u0026nbsp;[44]. In these patients, the initial problem could be retentive constipation and, so far, followed by overflow incontinence. If not managed well, this process may worsen the obstructed symptoms, whereas this is a reversible functional situation in the beginning\u0026nbsp;[11].\u003c/p\u003e\n\u003cp\u003eIn summary, AM could demonstrate objectively the anal sphincter pressure (\u003cem\u003ehypotonus or hypertonus\u003c/em\u003e), the sensation of the neorectum (\u003cem\u003eincreased or decreased perception\u003c/em\u003e), and the associated functional issues of defecation (\u003cem\u003edyssynergia\u003c/em\u003e) in the patients operated on for HD. The lesions could be either \u003cem\u003esingle\u003c/em\u003e or \u003cem\u003emultiple\u003c/em\u003e factors in each concrete patient [45].\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eBowel management in long-term follow-ups\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eRegarding long-term outcomes, some patients may suffer from fecal incontinence or constipation even years after surgery. This dilemma is still insufficient or lacking in developing countries [46, 47]. Non-operative management, such as bowel management (laxatives and enemas), should be considered the first option in most cases with high rates of success, even in more severe cases [48]. Moreover, some sophisticated techniques or more aggressive approaches like a transient (sometimes definitive) colostomy [49] or sacral nerve stimulating device implantation in some well-equipped centers for patients with refractory fecal incontinence [50, 51] should be considered. All these management options must be chosen carefully considering the AM results. AM could provide beneficial information on the etiologies for the continence problems. Thus, the therapeutical strategy for each case must be individualized and not only based on the clinical symptoms of fecal incontinence or constipation [10, 15, 28, 34].\u003c/p\u003e\n\u003cp\u003eIn our institution, we customized a tailored \u0026ldquo;bowel management program\u0026rdquo; for each patient presented with persistent and severe defecation disorder due to \u0026ldquo;anorectal outlet disorders\u0026rdquo;\u0026nbsp;[47]. The program consists of training daily habit defecation (biorhythm), adjusting the dosage of laxatives in cases of fecal retention (fecaloma); precisely determining the amount of water to facilitate enemas if needed (training for regular retrograde active enemas at-home care) according to the value of the Vmax, and adjusting the frequency of enemas depending on the amelioration of the symptoms and the adaptation of the patients as well as their families\u0026nbsp;[52]. In not rare cases with an associated functional problem (animus or dyssynergic defecation), AM could be repeated periodically as biofeedback training courses to educate patients to get used to \u0026ldquo;a normal defecation reflex\u0026rdquo;\u0026nbsp;[53, 54]. Otherwise, in the cases with quite normal AM results, the original problems could be due to a higher level such as dysmotility of the colon (rapid or slow colonic transit time)\u0026nbsp;[55].\u003c/p\u003e\n\u003cp\u003eFinally, one advantage of this study was the use of a reference-based population that helped to build the database for the average values of AM in Vietnamese children and to compare these parameters with one of the operated patients. More data should be collected for the reference parameters with other study results in the future. To our knowledge, this study could help promote long-term follow-ups still lacking in developing countries where the number of HD cases is much higher than in other countries with well-equipped settings [15, 28].\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eOur report shows that AM is an objective method for investigating anorectal malfunction in which the anal pressures and the rectal sensation are the two essential determinants. It provides helpful information that could guide better-adapted management in patients presenting with defecation disorders after an HD operation.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003e- AM\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003e- anorectal manometry\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003e- HD\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003e- Hirschsprung\u0026rsquo;s disease\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003e- IAAP\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003e- increased abnormal anal sphincter pressure\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003e- LATEP\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003e- laparoscopically assisted trans-anal endo-rectal pull-through\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003e- RAIR\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003e- recto-anal inhibitory reflex\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003e- SD\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003e- standard deviation\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003e- TERPT\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003e- transanal endorectal pull-through\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eConflict of interest\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no conflict of interest.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgments\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis research is part of a collaboration project funded by the Department of Science and Technology of Ho Chi Minh City, Vietnam, under grant number 03/2021/HĐ-QKHCN.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003ePuri P and Montedonico S (2008) Hirschsprung\u0026rsquo;s Disease: Clinical Features. 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Gastroenterology 144(2):314\u0026ndash;322.e312. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1053/j.gastro.2012.10.049\u003c/span\u003e\u003cspan address=\"10.1053/j.gastro.2012.10.049\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-surgery-international","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pesi","sideBox":"Learn more about [Pediatric Surgery International](http://link.springer.com/journal/383)","snPcode":"383","submissionUrl":"https://submission.nature.com/new-submission/383/3","title":"Pediatric Surgery International","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Hirschsprung’s disease, anorectal manometry, long-term outcomes, constipation, fecal incontinence","lastPublishedDoi":"10.21203/rs.3.rs-2527378/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-2527378/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003ePurpose\u003c/h2\u003e \u003cp\u003eThis study investigated anorectal manometry (AM) findings and bowel function of patients operated on for Hirschsprung\u0026rsquo;s disease (HD).\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e \u003cp\u003eA cross-sectional study was conducted at Children\u0026rsquo;s Hospital 2. Patients operated on for HD from January 2015 to January 2020 were reviewed. Their clinical characteristics, bowel function, and manometric findings were investigated and compared with the references.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e \u003cp\u003eNinety-five patients and 95 references were enrolled. Mean ages were 6.6\u0026thinsp;\u0026plusmn;\u0026thinsp;2.2 years and 7.2\u0026thinsp;\u0026plusmn;\u0026thinsp;2.9 years; fecal incontinence rates were 25.3% and 2.1%, and constipation rates were 12.6% and 4.2 for the patients versus the references, respectively. Anal resting pressures were significantly decreased in the patients compared to the references (53.2\u0026thinsp;\u0026plusmn;\u0026thinsp;16.1 mmHg versus 62.2\u0026thinsp;\u0026plusmn;\u0026thinsp;14.0 mmHg; p\u0026thinsp;\u0026lt;\u0026thinsp;0.05). Among the patients, the anal resting pressure was significantly decreased in the incontinents than in the continents (46.0\u0026thinsp;\u0026plusmn;\u0026thinsp;10.6 mmHg versus 55.6\u0026thinsp;\u0026plusmn;\u0026thinsp;16.9 mmHg, p\u0026thinsp;\u0026lt;\u0026thinsp;0.05). During the sensation test, the value of maximum tolerated volume was significantly decreased in the incontinents than in the continents (135.9\u0026thinsp;\u0026plusmn;\u0026thinsp;47.9 mL versus 166.6\u0026thinsp;\u0026plusmn;\u0026thinsp;58.3 mL, p\u0026thinsp;\u0026lt;\u0026thinsp;0.05).\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e \u003cp\u003eAM is an objective method providing beneficial information that could guide a more adapted management in HD patients with defecation disorders.\u003c/p\u003e","manuscriptTitle":"Anorectal Manometry Findings in Relation with Long-term Functional Outcomes of The Patients Operated on for Hirschsprung's Disease Compared to The Reference-Based Population","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2023-02-02 16:02:54","doi":"10.21203/rs.3.rs-2527378/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Accepted","date":"2023-01-31T19:03:07+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2023-01-31T19:00:30+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"214417cc-123c-4578-bbd2-bb9abc982b4f","date":"2023-01-31T18:59:05+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2023-01-31T18:56:08+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2023-01-31T18:43:08+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2023-01-31T06:35:15+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Surgery International","date":"2023-01-30T00:50:20+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-surgery-international","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pesi","sideBox":"Learn more about [Pediatric Surgery International](http://link.springer.com/journal/383)","snPcode":"383","submissionUrl":"https://submission.nature.com/new-submission/383/3","title":"Pediatric Surgery International","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"d87382e1-9e0e-447d-af49-8fff4fd7155c","owner":[],"postedDate":"February 2nd, 2023","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2023-10-16T19:02:26+00:00","versionOfRecord":{"articleIdentity":"rs-2527378","link":"https://doi.org/10.1007/s00383-023-05402-4","journal":{"identity":"pediatric-surgery-international","isVorOnly":false,"title":"Pediatric Surgery International"},"publishedOn":"2023-02-17 18:56:54","publishedOnDateReadable":"February 17th, 2023"},"versionCreatedAt":"2023-02-02 16:02:54","video":"","vorDoi":"10.1007/s00383-023-05402-4","vorDoiUrl":"https://doi.org/10.1007/s00383-023-05402-4","workflowStages":[]},"version":"v1","identity":"rs-2527378","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-2527378","identity":"rs-2527378","version":["v1"]},"buildId":"cBFmMYwuxLRRLfASyISRj","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}