Bowel function assessment and affecting factors in patients operated for Hirschsprung’s disease and Anorectal malformations in a resource-limited area: A five-year review

Bowel function assessment and affecting factors in patients operated for Hirschsprung’s disease and Anorectal malformations in a resource-limited area: A five-year review | 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 Bowel function assessment and affecting factors in patients operated for Hirschsprung’s disease and Anorectal malformations in a resource-limited area: A five-year review Maru Gama Erge, Tafese Gudissa Merga, Hana Abebe Gebreselassie This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7330027/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Introduction: Hirschsprung disease (HSD) and anorectal malformation (ARM) are among the common congenital anomalies encountered in the practice of pediatric surgery. Children who have undergone definitive repair for these disorders may suffer from various complications. This study aims to assess bowel function and the associated factors in patients from a low-income country, as this matter has remained largely unreported in this setting. Methods A retrospective review of children who underwent definitive surgical repair for HSD and ARM, and who were 3 years or older, was included in the study. Rintala's bowel function score was used to assess the outcome. The chi-square test was used to assess the association between several variables and bowel function score. All associations with a P value of < 0.05 were considered significant. Results A total of 132 children were operated on in the study period, and 100 of them were above 3 years and beyond 6 months after the surgery. Among these, the charts of 81 patients were available for analysis. HSD patients accounted for 58(71.6%) of the study participants with a mean age of 4.2 +/- 1.4 years and male to female ratio of 2.4:1. Patients for ARM constituted for 23(28.4%) of the study population with mean age of 3.9 +/- 1.4 years and male to female ratio of 0.9:1. Among children who have undergone pull through, the Rintala bowel function score was good in 37(64%), fair in 16(27.6%) and poor in 5(8.6%) of patients while it was good in 37(74%), fair in 3(13%) and poor in 3(13%) of ARM patients. Late presentation with chronic constipation (P = 0.045, OR = 1.474) was found to be significantly associated with poor bowel function score in patients with HSD, while the type of ARM (P = 0.000, OR = 2.545) and late age at presentation (P = 0.024, OR = 1.247) were found to be the significant variables for ARM patients. Conclusion A significant number of children who have undergone definitive repair for HSD and ARM have fair to poor bowel function scores. Late presentation of children with these pathologies is found to be a significant contributor to the poor outcome in low-income countries. Hirschsprung’s disease anorectal malformation Bowel function score Figures Figure 1 Background Anorectal malformation and Hirschsprung’s disease are among the common gastrointestinal congenital disorders encountered in pediatric surgery(1,2). According to a study done in TASH, Ethiopia, ARM accounts for about 57% of neonatal obstruction, and HSD is the third most common cause following ARM and intestinal atresia(3,4). ARM is a congenital disorder characterized by the absence or abnormal opening of the anal orifice with a wide spectrum of anomalies involving colorectal, urogenital, vertebral spine, and perineal musculature(1,2). Worldwide, the average incidence is about 1 in 5000 live births, with geographic variation. Recto-vaginal fistula is the most common ARM in females, while Recto-urethral fistula is the most common type in males. ARM is known for its association with other congenital anomalies, presenting as part of VACTERL anomalies in about half of the cases(1,5–7). Similarly, HSD is a congenital developmental disorder of the intrinsic component of the enteric nervous system characterized by aganglionosis of the colon. It is estimated that the incidence is 1:5,000 live births and is more commonly in males by a 4:1 ratio compared to females(8–10). The disease is commonly associated with a range of congenital anomalies, namely nervous, renal, and cardiac malformations, among which Down’s syndrome is undoubtedly the most noteworthy(11). The majority of the patients with these disorders will develop symptoms within the first few days of life to the first month of life, while diagnosis during adolescence remains rare. The most common presentation of these patients is usually failure/delayed passage of meconium, abdominal distension, and constipation(1,6,12). Some patients with HSD can present with acute illness related to HSD-associated enterocolitis and, rarely, intestinal perforation and sigmoid volvulus (13,14). Pull-through Procedure is the only definitive management for HSD, with different techniques sharing the common purpose of resecting the distal aganglionic bowel segment and reconstructing the rectum and anus. As to the definitive surgical management of ARM, due to the wide spectrum of anomalies, variable procedures ranging from cut-back anoplasty to complex rectal and genitourinary reconstructions are undertaken. Despite the advancements in surgical procedures, in patients with HSD and ARM, inadvertent injuries to adjacent organs and their innervation may lead to long-term complications. Even though many patients may attain normal bowel function following surgery, defecation disorders, such as constipation, faecal soiling, to incontinence, may persist(5,15–19,20–22). Despite many patients suffering from bowel function disorders, different studies on bowel function outcomes report conflicting results. This is due to a lack of consistency in the methods and tools measuring bowel function, the use of several different scoring tools to evaluate bowel function, variation in age groups, type of surgery, and variation in the extent of aganglionosis in different studies. The advantage of the Rintala score is that it has been validated in healthy normal subjects(23,24). This study aims to provide information on bowel function outcome scores in HSD and ARM surgeries, with associated factors for good and poor outcomes. Detecting factors determining the outcome will help reduce the complications. Also, since there is a significant paucity of data in resource-limited areas, it will help as a base for future researchers. Methods The study was conducted at SPHMMC, one of the largest tertiary hospitals in Addis Ababa, Ethiopia. An institution-based retrospective cross-sectional review was conducted in patients diagnosed with HSD or ARM and who underwent definitive surgical intervention at SPHMMC from January 2018 to December 2022. All patients who have undergone definitive surgical intervention and whose current age is above 3 years and, 6 months away from the last surgery are included in the study. A structured questionnaire was prepared for the interview with the parents of the patients. Data was collected by reviewing patients’ charts, interviewing families at an outpatient clinic, and on phone calls. The collected data was analyzed with SPSS version 26. The association between the independent and dependent variables was considered significant when P < 0.05. The strength of association between dependent and independent variables was also assessed using adjusted odds ratio (AOR). The bowel function outcome was assessed by the Rintala bowel function score as listed in the following table (Table 1). Table 1: Rintala bowel function score Rintala Bowel Function Score A. Ability to hold back defecation Score Always 3 PProblems less than 1/week 2 Weekly problems 1 No voluntary control 0 B. Feels/reports the urge to defecate Always 3 Most of the time 2 Uncertain 1 Absent 0 C. Frequency of defecation Every other day to twice a day 2 More often 1 Less often 0 D. Soiling Never 3 Staining less than 1/week, no change of underwear required 2 Frequent staining, change of underwear often required 1 Daily soiling requires protective aids 0 E. Accidents Never 3 Fewer than 1/week 2 Weekly accidents often require protective aids 1 Daily, requires protective aids during the day and night 0 F. Constipation No constipation 3 Manageable with diet 2 Manageable with laxatives 1 Manageable with enemas 0 G. Social problems No social problems 3 Sometimes (foul odours) 2 Problems causing restrictions in social life 1 Severe social and/or psychological problems 0 Total score The score is out of 20 and categorized as poor if the score is less than or equal to 9, fair if it is between 9 and 16, and good if above 16. Ethical clearance for this study was obtained from the SPHMMC Institutional Review Board. The respondent was provided written and verbal informed consent. Confidentiality of the patient’s medical record was maintained throughout the study. Operational definition Voluntary bowel movement : feeling the urge to use the toilet to have a bowel movement, the capacity to verbalize it, and to hold the bowel movement. Constipation : inability to empty the rectum spontaneously without help every day Faecal soiling : involuntary leaking of small amounts of stool, which may be present with or without voluntary bowel movements Faecal incontinence : lack of control over defecation leading to involuntary loss of bowel contents. Neonatal obstruction: Presentation with features of constipation within one month of life Acute obstruction: Presentation with features of constipation within one month of symptom onset Chronic obstruction: Presentation with features of constipation for more than one month Definitive surgical intervention: The Patient underwent a pull-through procedure, or anoplasty and the colostomy is closed. Results A total of 132 children were operated on in the study period, and 100 of them were above 3 years and beyond 6 months after the surgery. All of these patients were included in the data collection. Among these 100 patients, for 12 patients it was impossible to get the chart, and for 7 patients the chart was incomplete, and it was impossible to reach the patient’s caregiver on a phone call. Therefore, eighty-one patients (81%) fulfilling the criteria were included in the study: 58 (71.6%) with HSD patients and 23 (28.4%) patients with ARM (Figure 1). Hirschsprung’s disease Socio-demographic data Among 58 patients with HSD, 41 (70.7%) were male. Age at the time of data collection ranged from 3 to 10 years, with a median and mean age of 3.9 and 4.2 (SD 1.4) years, respectively. The age at diagnosis varies from as early as 2 days to as late as 4 years, with a median of 3 months and a mean age of 10.5month (SD 14.2 months); similarly, the age at definitive surgical intervention ranges from 6 months to 5 years with a median and mean age of 1.7 (SD 1.3) year. Clinical Profile of Patients with HSD Most of these patients were presented with chronic constipation (36 (62.1%)), while neonatal obstruction was the second most common presentation, 21 (36.2%). Only one (1.7%) patient presented with acute obstruction; this patient presented with acute obstruction at the age of 4 months without having any bowel habit complaint previously. Associated congenital anomalies were seen in 8(13.8%) patients: 4(50%) Down’s syndrome, 3(37.5%) renal anomalies and 1(12.5%) undescended testis. All patients had short-segment HSD based on barium enema findings. As to the definitive surgical procedure, Soave pull-through accounted for most of the procedures, 56(96.6%), and only for 2 (3.4%) patients, Swenson pull-through was done. Post-pull-through bowel function score Rintala bowel function score was used to objectively assess the post-pull-through bowel function of patients with the following results:37(63.8%) patients had a score of above 16 (good), 16 (27.6%) patients between 9 and 16 (Fair), and 5(8.6%) patients less than or equal to 9 (Poor). Among these patients with fair and poor BFS, in only 3(6.8%) patients the cause was identified after evaluation under anaesthesia; One anal stenosis and two anastomosis sites below the dentate line, the rest of the patients were not investigated. Associations of post-pull-through bowel function score with different factors The association of bowel function score with the age of the patient at diagnosis, age at surgical intervention, initial clinical presentation, and associated anomalies was evaluated. Patterns of association were analyzed using bivariate and multivariate logistic regression. Among these variables, a significant association was seen between the type of initial presentation of the patient and bowel function outcome, as patients presented with chronic constipation tend to have fair or poor bowel function score postoperatively (P =0.045, AOR = 1.474). Anorectal malformation Socio-demographic profile of patients with ARM Among 23 ARM patients, 12 (52.2%) were female. Age at data collection ranged from 3 to 10 years, with a median and mean age of 3.6 and 3.9 (SD 1.4) years, respectively. The age at diagnosis varies from as early as 1 day to as late as 5 years, with a median of 10 days and mean of 4.5months with a standard deviation of 12.7 months; similarly, the age at definitive intervention ranges from 1 day to 7 years with a median and mean age of 1.9 and 2.3 (SD 1.9) years respectively. Clinical profile of patients with ARM The majority of these patients were referred from the obstetrics side at the time of delivery (12(52.2%)), while 6 (26.1%) patients presented with chronic constipation and 5(21.7%) patients presented after being detected by family. ARM with RVF was the most common type seen in females, while ARM with RUF is the most common anomaly seen in males (Table 2). Associated congenital anomalies were seen in 9(39.1%) patients 6(66.7%) renal anomalies, 2(22.2%) cardiac anomalies and 1(11.1%) hemivertebrae. Table 2: Distribution by type of ARM Type of ARM No Percent ARM with RVF 9 39.1% ARM with RUF 6 26.1% ARM with perineal fistula 3 13.0% ARM with no fistula 1 4.4% Cloaca 1 4.4% Other (Anal stenosis, rectal atresia, and ARM with bladder neck fistula) 3 13.0% Total 23 100.0% Among these patients, in 21(91.3%) of them diversion colostomy was done, and only 2(8.7%) patients underwent definitive surgical intervention without colostomy; these are two of the patients with ARM with perineal fistula. As to the definitive surgical procedure, PSARP was the most common procedure, done in 11(47.8%) patients, followed by ASARP,9(39.1%), 2(8.7%) cutback anoplasty, and 1(4.4%) patient underwent PSRUVP. Post-analplasty bowel function score Rintala BFS was used to assess post-analplasty bowel function outcome. Among these patients, 17(74.0%) have Rintala Bowel function scores of above 16 (good), while 3(13%) each are between 9 and 16 (fair), and less than or equal to 9 (poor). Five of these patients with fair and poor BFS were investigated, and the cause was identified: 4(80%) anal stenosis, and 1(20%) megacolon. Associations of anoplasty bowel function score with different factors The association of bowel function outcome with the age of the patient at diagnosis and surgical intervention, initial clinical presentation, associated anomalies, type of ARM, and type of surgical intervention was evaluated. Patterns of association were analyzed using bivariate and multivariate logistic regression. Among these factors, the type of ARM (P=0.000, OR=2.545) and late age at presentation (P=0.024, OR=1.247) were found to have a significant association with post-anoplasty BFS. This shows that the later the age at diagnosis and definitive intervention, the worse the bowel function outcome and the more severe the ARM, the poorer the outcomes. Discussion Hirschsprung’s disease HSD is one of the common causes of intestinal obstruction in children. According to this study, about 70% of the patients are male, which is comparable with a study done in Tanzania and London Children's Hospital(25,26), but lower than the study done at TAH, Ethiopia, where only 17% were female(27). In this study, the median age at presentation was 3 months, which is a comparable finding with the study at TAH, Ethiopia, but earlier than the findings in Tanzania and later than in London Children's Hospital, where the median age at diagnosis was 2 years and 6.5 days, respectively. The age at definitive surgical intervention was also later than the findings at London Children's Hospital, 1.7 years and 4 months, respectively(25,26,28). Similar to the study at TAH and the study in Tanzania, chronic constipation was the most common presentation of these patients(26,28). Short-segment HSD is the most common type of HSD. All of our patients were diagnosed with short-segment HSD, which is a relatively similar finding to findings at the Hospital for Children and Adolescents, Finland, but higher than findings in London by Joe Davidson and Joe Curr, and in Tanzania, where short-segment HSD accounted for 76% and 67.5% respectively(8,10,26). Associated congenital anomalies were seen in 13.8%, with Down syndrome being the most common (7%) associated congenital anomaly, which is a similar finding to a study at London Children's Hospital (8%) (25,29). As pull pull-through procedure involves resection of the colon and rectoanal reconstruction, some patients with HSD may develop bowel function disorder. Among patients enrolled in this study 37(63.8%) had a Rintala Bowel function score of above 16, while 16(27.6%) were between 9 and 16, and 5(8.6%) less or equal to 9, which is similar finding with finding at Vellore, India and Hospital for Children and Adolescent university of Helsinki, Finland where good bowel function score (above 16) is 58% and 47–67% respectively(10). For our patients, BFS was relatively better than the findings reported in London by Joe Davidson and Joe Curry, where only 52% of the patients had good Rintala BFS, while 36% had fair and 12% had poor Rintala BFS(8). It is also better than findings at Ottawa, Ontario, Canada, and London Children Hospital, where only 50% of the patients had good BFS, but the score for our patients is lower than the finding at Tygerberg, South Africa where around three fourth of them were having excellent anorectal function after pull through the procedure(8,18,25,30). Patients who develop post-pull-through bowel function disorder (poor and fair BFS) should undergo evaluation including physical examination, evaluation under anaesthesia, and different imaging modalities depending on the type of the disorder. In our patients, even though the bowel function disorder is shown to be common, in only 3 patients the cause was identified: one anal stenosis and two anastomosis sites below the dentate line; the rest of the patients were not investigated. Different factors are shown to affect bowel function outcomes in HSD patients following definitive surgical intervention. Joe Davidson and Joe Curry, London, found that there is a strong association between BFS and age at surgical intervention, preoperative history of HSD-associated enterocolitis, and length of agangliosis(8). Similarly, a study at the Hospital for Children and Adolescents, Finland, showed that age at surgical intervention has an inverse relation with BFS. They also found that associated congenital anomalies and length of agangliosis negatively affect bowel function outcomes (10). In our study, however, there is no relation seen between bowel function outcome and age of the patient, age at diagnosis or definitive surgical intervention, and associated congenital anomalies. However significant association was seen between the type of initial presentation of the patient and bowel function score, as patients presented with chronic constipation (P = 0.045, AOR = 1.474) tended to develop bowel function disorder postoperatively. Anorectal malformation ARM is one of the common congenital neonatal obstructions having due effect on long-term bowel function. According to this study, about 52.2% of the patients were female, which is comparable with the finding at TASH, but contradicting the finding of a study done in Ibadan, Nigeria, where 69.3% were male(1,6). In our study, the age at diagnosis varies from as early as 1 day to as late as 5 years, with a median of 10 days. This is earlier than findings at TASH and Tanzania, where only 20% were diagnosed in the first month of life, and 50 days was the median age at diagnosis, respectively(1,12). The majority of these patients,12(52.2%), were referred from the gynaecology/obstetric side, possibly contributing to relatively early presentation. ARM is known for its association with other congenital anomalies. About 39.1% of the patients had associated congenital anomalies, renal anomalies being the most common anomalies seen. This is higher than the finding in Tanzania, where only 16% had associated congenital anomalies, but lower than the finding at TASH, where almost half of the patients had associated anomalies, the genitourinary system being the most common(1,12). Similar to findings at TASH and in the UK, ARM with RVF and ARM with RUF were the most common type seen in females and males, respectively(1,5). Due to the nature of the disease and surgical intervention affecting anorectal anatomy, including the sphincter mechanism, patients with ARM can develop bowel function disorders. In our study, we found that in patients with ARM after anoplasty is done, 17(74.0%) have Rintala Bowel function scores of above 16, while 3(13%) each are between 9 and 16, and less than or equal to 9. This is a better finding than the finding at the Hospital for Children and Adolescents in Finland, where around 32% had a BFS of less than 17, but comparable with the finding of a study done in Tanzania, where around 16% had poor BFS(12). In patients with ARM, different factors are known to affect BFS. In our study, we have found that age at diagnosis and age at definitive surgical intervention are inversely related to bowel function outcome score. Similar to the findings at the Hospital for Children and Adolescents in Finland and the study done in the UK, the type of ARM was strongly related to bowel function outcome, as the severe ARM type was associated with poor outcomes (5,31). However, we didn’t find a significant association between bowel function outcome and associated congenital anomalies, contradicting findings by Daniel H. Teitelbaum, Tanta University, Egypt, where 44% of patients with associated congenital anomalies had poor bowel outcomes (32). The fact that only a single patient had vertebral anomalies may have contributed to this non-significant association between BFS and associated congenital anomalies in our findings. Conclusion A significant number of children who have undergone definitive repair for HSD and ARM have fair to poor bowel function. However, the majority of these patients did not undergo any type of evaluation due to poor post-operative follow-up. It is also noticed that early presentation and intervention are associated with better outcomes. Recommendation Early surgical intervention and close postoperative follow-up are recommended for better long-term outcomes in patients with HSD and ARM. Abbreviations AOR Adjusted Odd Ratio BFS Bowel function score EC Ethiopian calendar HSD Hirschsprung disease MD Medical doctor SPHMMC Saint Paul’s Hospital Medical Millennium College TASH Tikur Anbessa specialized Hospital TERPT Transanal endorectal pull-through UK United Kingdom Declarations Ethics approval and consent to participate Ethical approval for this study was obtained from the IRB of SPHMMC. Informed written consent was taken from the legal guardians of children who are enrolled in this study. Consent for publication Not applicable. Data availability The datasets used during the study are available from the corresponding author on reasonable request Competing interests The authors declare no competing interests. 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Moore SW, Albertyn R, Cywes Tygerberg S. Clinical Outcome and Long-Term Quality of Life After Surgical Correction of Hirschsprung’s Disease. Pakarinen MP, Koivusalo A, Lindahl H, Rintala RJ. Prospective Controlled Long-term Follow-up for Functional Outcome After Anoplasty in Boys With Perineal Fistula [Internet]. 2007. Available from: http://journals.lww.com/jpgn Hashish MS, Dawoud HH, Hirschl RB, Bruch SW, El Batarny AM, Mychaliska GB, et al. Long-term functional outcome and quality of life in patients with high imperforate anus. J Pediatr Surg [Internet]. 2010;45(1):224–30. Available from: http://dx.doi.org/10.1016/j.jpedsurg.2009.10.041 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted 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. 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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-7330027","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":503858780,"identity":"cb68e473-f29e-460b-b1c4-958f6dbab706","order_by":0,"name":"Maru Gama Erge","email":"","orcid":"","institution":"St. Paul’s Hospital Millennium Medical College","correspondingAuthor":false,"prefix":"","firstName":"Maru","middleName":"Gama","lastName":"Erge","suffix":""},{"id":503858781,"identity":"57de7246-092e-4b0b-bc58-6048edc4a5f0","order_by":1,"name":"Tafese Gudissa Merga","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA2ElEQVRIiWNgGAWjYLACHjYJIMl8AEhIyBBWjdDClgDSwkOsFjBpABfAC+zZ269JvCmzsOdvP/P51Y0aCx4G9sNHN+C1hedMmeSccxKJM87kbrPOOQZ0GE9a2g28WiRy0qR52yQSGA7kbjPOYQNqkeAxI0qLvfz5N8+Mc/4RpSX9GEgL44YbOcyPc9uI0XLmDLMlyC8bbzwzY87tk+BhI+QX9vb2hzfelNXZy51Pfvw551udHD/74WN4tcCiAwTAEcrAhl852J4HMBbzB8KqR8EoGAWjYCQCABGVQvVRB5MAAAAAAElFTkSuQmCC","orcid":"","institution":"St. Paul’s Hospital Millennium Medical College","correspondingAuthor":true,"prefix":"","firstName":"Tafese","middleName":"Gudissa","lastName":"Merga","suffix":""},{"id":503858782,"identity":"444e1390-60aa-4502-87a6-757c6433db50","order_by":2,"name":"Hana Abebe Gebreselassie","email":"","orcid":"","institution":"St. Paul’s Hospital Millennium Medical College","correspondingAuthor":false,"prefix":"","firstName":"Hana","middleName":"Abebe","lastName":"Gebreselassie","suffix":""}],"badges":[],"createdAt":"2025-08-08 20:23:06","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7330027/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7330027/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":89977450,"identity":"93a87879-e613-4850-97e5-399865b24880","added_by":"auto","created_at":"2025-08-27 06:09:32","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":194117,"visible":true,"origin":"","legend":"\u003cp\u003eFlowchart of inclusion and exclusion criteria\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-7330027/v1/a4f9fee2e5d1407e7cc5447e.png"},{"id":94490161,"identity":"ff2614b0-7a52-46b1-bc3f-23ab70253b66","added_by":"auto","created_at":"2025-10-27 17:07:57","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1000264,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7330027/v1/1508a9f9-3996-4da4-948e-e3f10160318c.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Bowel function assessment and affecting factors in patients operated for Hirschsprung’s disease and Anorectal malformations in a resource-limited area: A five-year review","fulltext":[{"header":"Background","content":"\u003cp\u003eAnorectal malformation and Hirschsprung\u0026rsquo;s disease are among the common gastrointestinal congenital disorders encountered in pediatric surgery(1,2). According to a study done in TASH, Ethiopia, ARM accounts for about 57% of neonatal obstruction, and HSD is the third most common cause following ARM and intestinal atresia(3,4). ARM is a congenital disorder characterized by the absence or abnormal opening of the anal orifice with a wide spectrum of anomalies involving colorectal, urogenital, vertebral spine, and perineal musculature(1,2). Worldwide, the average incidence is about 1 in 5000 live births, with geographic variation. Recto-vaginal fistula is the most common ARM in females, while Recto-urethral fistula is the most common type in males. ARM is known for its association with other congenital anomalies, presenting as part of VACTERL anomalies in about half of the cases(1,5\u0026ndash;7).\u003c/p\u003e\u003cp\u003eSimilarly, HSD is a congenital developmental disorder of the intrinsic component of the enteric nervous system characterized by aganglionosis of the colon. It is estimated that the incidence is 1:5,000 live births and is more commonly in males by a 4:1 ratio compared to females(8\u0026ndash;10). The disease is commonly associated with a range of congenital anomalies, namely nervous, renal, and cardiac malformations, among which Down\u0026rsquo;s syndrome is undoubtedly the most noteworthy(11).\u003c/p\u003e\u003cp\u003eThe majority of the patients with these disorders will develop symptoms within the first few days of life to the first month of life, while diagnosis during adolescence remains rare. The most common presentation of these patients is usually failure/delayed passage of meconium, abdominal distension, and constipation(1,6,12). Some patients with HSD can present with acute illness related to HSD-associated enterocolitis and, rarely, intestinal perforation and sigmoid volvulus (13,14).\u003c/p\u003e\u003cp\u003ePull-through Procedure is the only definitive management for HSD, with different techniques sharing the common purpose of resecting the distal aganglionic bowel segment and reconstructing the rectum and anus. As to the definitive surgical management of ARM, due to the wide spectrum of anomalies, variable procedures ranging from cut-back anoplasty to complex rectal and genitourinary reconstructions are undertaken. Despite the advancements in surgical procedures, in patients with HSD and ARM, inadvertent injuries to adjacent organs and their innervation may lead to long-term complications. Even though many patients may attain normal bowel function following surgery, defecation disorders, such as constipation, faecal soiling, to incontinence, may persist(5,15\u0026ndash;19,20\u0026ndash;22).\u003c/p\u003e\u003cp\u003eDespite many patients suffering from bowel function disorders, different studies on bowel function outcomes report conflicting results. This is due to a lack of consistency in the methods and tools measuring bowel function, the use of several different scoring tools to evaluate bowel function, variation in age groups, type of surgery, and variation in the extent of aganglionosis in different studies. The advantage of the Rintala score is that it has been validated in healthy normal subjects(23,24).\u003c/p\u003e\u003cp\u003eThis study aims to provide information on bowel function outcome scores in HSD and ARM surgeries, with associated factors for good and poor outcomes. Detecting factors determining the outcome will help reduce the complications. Also, since there is a significant paucity of data in resource-limited areas, it will help as a base for future researchers.\u003c/p\u003e"},{"header":"Methods","content":"\u003cp\u003eThe study was conducted at SPHMMC, one of the largest tertiary hospitals in Addis Ababa, Ethiopia. An institution-based retrospective cross-sectional review was conducted in patients diagnosed with HSD or ARM and who underwent definitive surgical intervention at SPHMMC from January 2018 to December 2022.\u0026nbsp;All patients who have undergone definitive surgical intervention and whose current age is above 3 years and, 6 months away from the last surgery are included in the study.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eA structured questionnaire was prepared for the interview with the parents of the patients.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eData was collected by reviewing patients\u0026rsquo; charts, interviewing families at an outpatient clinic, and on phone calls. The collected data was analyzed with SPSS version 26. The association between the independent and dependent variables was considered significant when P \u0026lt; 0.05. The strength of association between dependent and independent variables was also assessed using adjusted odds ratio (AOR).\u003c/p\u003e\n\u003cp\u003eThe bowel function outcome was assessed by the Rintala bowel function score as listed in the following table (Table 1).\u003c/p\u003e\n\u003cp\u003eTable 1: Rintala bowel function score\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"502\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"2\" valign=\"top\" style=\"width: 502px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eRintala Bowel Function Score\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eA. Ability to hold back defecation\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003eScore\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eAlways\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003ePProblems less than 1/week\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eWeekly problems\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eNo voluntary control\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eB. Feels/reports the urge to defecate\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eAlways\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eMost of the time\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eUncertain\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eAbsent\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eC. Frequency of defecation\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eEvery other day to twice a day\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eMore often\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eLess often\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eD. Soiling\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003e\u0026nbsp;Never\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eStaining less than 1/week, no change\u003c/p\u003e\n \u003cp\u003eof underwear required\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eFrequent staining, change of\u003c/p\u003e\n \u003cp\u003eunderwear often required\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eDaily soiling requires protective aids\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eE. Accidents\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eNever\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eFewer than 1/week\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eWeekly accidents often require protective aids\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eDaily, requires protective aids during the day and night\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eF. Constipation\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eNo constipation\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eManageable with diet\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eManageable with laxatives\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eManageable with enemas\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eG. Social problems\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eNo social problems\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eSometimes (foul odours)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eProblems causing restrictions in social life\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eSevere social and/or psychological problems\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 403px;\"\u003e\n \u003cp\u003eTotal score\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 99px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eThe score is out of 20 and categorized as poor if the score is less than or equal to 9, fair if it is between 9 and 16, and good if above 16.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eEthical clearance for this study was obtained from the SPHMMC Institutional Review Board. The respondent was provided written and verbal informed consent. Confidentiality of the patient\u0026rsquo;s medical record was maintained throughout the study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eOperational definition\u003c/strong\u003e\u003c/p\u003e\n\u003cp id=\"_Toc25798\"\u003e\u003cstrong\u003eVoluntary bowel movement\u003c/strong\u003e: feeling the urge to use the toilet to have a bowel movement, the capacity to verbalize it, and to hold the bowel movement.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConstipation\u003c/strong\u003e:\u0026nbsp;inability to empty the rectum spontaneously without help every day\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFaecal soiling\u003c/strong\u003e:\u0026nbsp;involuntary leaking of small amounts of stool, which may be present with or without voluntary bowel movements\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFaecal incontinence\u003c/strong\u003e: lack\u0026nbsp;of control over defecation leading to involuntary loss of bowel contents.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eNeonatal obstruction:\u0026nbsp;\u003c/strong\u003ePresentation with features of constipation within one month of life\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcute obstruction:\u0026nbsp;\u003c/strong\u003ePresentation with features of constipation within one month of symptom onset\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eChronic obstruction:\u0026nbsp;\u003c/strong\u003ePresentation with features of constipation for more than one month\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eDefinitive surgical intervention:\u003c/strong\u003e The Patient underwent a pull-through procedure, or anoplasty and the colostomy is closed.\u003c/p\u003e"},{"header":"Results","content":"\u003cp\u003eA total of 132 children were operated on in the study period, and 100 of them were above 3 years and beyond 6 months after the surgery. All of these patients were included in the data collection. Among these 100 patients, for 12 patients it was impossible to get the chart, and for 7 patients the chart was incomplete, and it was impossible to reach the patient\u0026rsquo;s caregiver on a phone call. \u003cstrong\u003e\u0026nbsp;\u003c/strong\u003eTherefore, eighty-one patients (81%) fulfilling the criteria were included in the study: 58 (71.6%) with HSD patients and 23 (28.4%) patients with ARM (Figure 1).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eHirschsprung\u0026rsquo;s disease\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eSocio-demographic data\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Among 58 patients with HSD, 41 (70.7%) were male. Age at the time of data collection ranged from 3 to 10 years, with a median and mean age of 3.9 and 4.2 (SD 1.4) years, respectively. The age at diagnosis varies from as early as 2 days to as late as 4 years, with a median of 3 months and a mean age of 10.5month (SD 14.2 months); similarly, the age at definitive surgical intervention ranges from 6 months to 5 years with a median and mean age of 1.7 (SD 1.3) year.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eClinical Profile of Patients with HSD\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eMost of these patients were presented with chronic constipation (36 (62.1%)), while neonatal obstruction was the second most common presentation, 21 (36.2%). Only one (1.7%) patient presented with acute obstruction; this patient presented with acute obstruction at the age of 4 months without having any bowel habit complaint previously.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAssociated congenital anomalies were seen in 8(13.8%) patients: 4(50%) Down\u0026rsquo;s syndrome, 3(37.5%) renal anomalies and 1(12.5%) undescended testis. All patients had short-segment HSD based on barium enema findings. As to the definitive surgical procedure, Soave pull-through accounted for most of the procedures, 56(96.6%), and only for 2 (3.4%) patients, Swenson pull-through was done.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePost-pull-through bowel function score\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eRintala bowel function score was used to objectively assess the post-pull-through bowel function of patients with the following results:37(63.8%) patients had a score of above 16 (good), 16 (27.6%) patients between 9 and 16 (Fair), and 5(8.6%) patients less than or equal to 9 (Poor). Among these patients with fair and poor BFS, in only 3(6.8%) patients the cause was identified after evaluation under anaesthesia; One anal stenosis and two anastomosis sites below the dentate line, the rest of the patients were not investigated.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAssociations of post-pull-through bowel function score with different factors\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe association of bowel function score with the age of the patient at diagnosis, age at surgical intervention, initial clinical presentation, and associated anomalies was evaluated. Patterns of association were analyzed using bivariate and multivariate logistic regression. Among these variables, a significant association was seen between the type of initial presentation of the patient and bowel function outcome, as patients presented with chronic constipation tend to have fair or poor bowel function score postoperatively (P =0.045, AOR = 1.474).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAnorectal malformation\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eSocio-demographic profile of patients with ARM\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAmong 23 ARM patients, 12 (52.2%) were female. Age at data collection ranged from 3 to 10 years, with a median and mean age of 3.6 and 3.9 (SD 1.4) years, respectively. The age at diagnosis varies from as early as 1 day to as late as 5 years, with a median of 10 days and mean of 4.5months with a standard deviation of 12.7 months; similarly, the age at definitive intervention ranges from 1 day to 7 years with a median and mean age of 1.9 and 2.3 (SD 1.9) years respectively.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eClinical profile of patients with ARM\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe majority of these patients were referred from the obstetrics side at the time of delivery (12(52.2%)), while 6 (26.1%) patients presented with chronic constipation and 5(21.7%) patients presented after being detected by family.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eARM with RVF was the most common type seen in females, while ARM with RUF is the most common anomaly seen in males (Table 2). Associated congenital anomalies were seen in 9(39.1%) patients 6(66.7%) renal anomalies, 2(22.2%) cardiac anomalies and 1(11.1%) hemivertebrae.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eTable 2: Distribution by type of ARM\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"564\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 354px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eType of ARM\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 66px;\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 144px;\"\u003e\n \u003cp\u003ePercent\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 354px;\"\u003e\n \u003cp\u003eARM with RVF\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 66px;\"\u003e\n \u003cp\u003e9\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 144px;\"\u003e\n \u003cp\u003e39.1%\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 354px;\"\u003e\n \u003cp\u003eARM with RUF\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 66px;\"\u003e\n \u003cp\u003e\u0026nbsp;6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 144px;\"\u003e\n \u003cp\u003e26.1%\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 354px;\"\u003e\n \u003cp\u003eARM with perineal fistula\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 66px;\"\u003e\n \u003cp\u003e\u0026nbsp;3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 144px;\"\u003e\n \u003cp\u003e13.0%\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 354px;\"\u003e\n \u003cp\u003eARM with no fistula\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 66px;\"\u003e\n \u003cp\u003e\u0026nbsp;1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 144px;\"\u003e\n \u003cp\u003e4.4%\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 354px;\"\u003e\n \u003cp\u003eCloaca\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 66px;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 144px;\"\u003e\n \u003cp\u003e4.4%\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 354px;\"\u003e\n \u003cp\u003eOther (Anal stenosis, rectal atresia, and ARM with bladder neck fistula)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 66px;\"\u003e\n \u003cp\u003e\u0026nbsp;3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 144px;\"\u003e\n \u003cp\u003e13.0%\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 354px;\"\u003e\n \u003cp\u003eTotal\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 66px;\"\u003e\n \u003cp\u003e\u0026nbsp;23\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 144px;\"\u003e\n \u003cp\u003e100.0%\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eAmong these patients, in 21(91.3%) of them diversion colostomy was done, and only 2(8.7%) patients underwent definitive surgical intervention without colostomy; these are two of the patients with ARM with perineal fistula. As to the definitive surgical procedure, PSARP was the most common procedure, done in 11(47.8%) patients, followed by ASARP,9(39.1%), 2(8.7%) cutback anoplasty, and 1(4.4%) patient underwent PSRUVP.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePost-analplasty bowel function score\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eRintala BFS was used to assess post-analplasty bowel function outcome. Among these patients, 17(74.0%) have Rintala Bowel function scores of above 16 (good), while 3(13%) each are between 9 and 16 (fair), and less than or equal to 9 (poor).\u0026nbsp;Five of these patients with fair and poor BFS were investigated, and the cause was identified: 4(80%) anal stenosis, and 1(20%) megacolon.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAssociations of anoplasty bowel function score with different factors\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe association of bowel function outcome with the age of the patient at diagnosis and surgical intervention, initial clinical presentation, associated anomalies, type of ARM, and type of surgical intervention was evaluated. Patterns of association were analyzed using bivariate and multivariate logistic regression. Among these factors, the type of ARM (P=0.000, OR=2.545) and late age at presentation (P=0.024, OR=1.247) were found to have a significant association with post-anoplasty BFS. This shows that the later the age at diagnosis and definitive intervention, the worse the bowel function outcome and the more severe the ARM, the poorer the outcomes.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cdiv id=\"Sec16\" class=\"Section2\"\u003e\u003ch2\u003eHirschsprung\u0026rsquo;s disease\u003c/h2\u003e\u003cp\u003eHSD is one of the common causes of intestinal obstruction in children. According to this study, about 70% of the patients are male, which is comparable with a study done in Tanzania and London Children's Hospital(25,26), but lower than the study done at TAH, Ethiopia, where only 17% were female(27). In this study, the median age at presentation was 3 months, which is a comparable finding with the study at TAH, Ethiopia, but earlier than the findings in Tanzania and later than in London Children's Hospital, where the median age at diagnosis was 2 years and 6.5 days, respectively. The age at definitive surgical intervention was also later than the findings at London Children's Hospital, 1.7 years and 4 months, respectively(25,26,28).\u003c/p\u003e\u003cp\u003eSimilar to the study at TAH and the study in Tanzania, chronic constipation was the most common presentation of these patients(26,28).\u003c/p\u003e\u003cp\u003eShort-segment HSD is the most common type of HSD. All of our patients were diagnosed with short-segment HSD, which is a relatively similar finding to findings at the Hospital for Children and Adolescents, Finland, but higher than findings in London by Joe Davidson and Joe Curr, and in Tanzania, where short-segment HSD accounted for 76% and 67.5% respectively(8,10,26). Associated congenital anomalies were seen in 13.8%, with Down syndrome being the most common (7%) associated congenital anomaly, which is a similar finding to a study at London Children's Hospital (8%) (25,29).\u003c/p\u003e\u003cp\u003eAs pull pull-through procedure involves resection of the colon and rectoanal reconstruction, some patients with HSD may develop bowel function disorder. Among patients enrolled in this study 37(63.8%) had a Rintala Bowel function score of above 16, while 16(27.6%) were between 9 and 16, and 5(8.6%) less or equal to 9, which is similar finding with finding at Vellore, India and Hospital for Children and Adolescent university of Helsinki, Finland where good bowel function score (above 16) is 58% and 47\u0026ndash;67% respectively(10).\u003c/p\u003e\u003cp\u003e For our patients, BFS was relatively better than the findings reported in London by Joe Davidson and Joe Curry, where only 52% of the patients had good Rintala BFS, while 36% had fair and 12% had poor Rintala BFS(8). It is also better than findings at Ottawa, Ontario, Canada, and London Children Hospital, where only 50% of the patients had good BFS, but the score for our patients is lower than the finding at Tygerberg, South Africa where around three fourth of them were having excellent anorectal function after pull through the procedure(8,18,25,30).\u003c/p\u003e\u003cp\u003ePatients who develop post-pull-through bowel function disorder (poor and fair BFS) should undergo evaluation including physical examination, evaluation under anaesthesia, and different imaging modalities depending on the type of the disorder. In our patients, even though the bowel function disorder is shown to be common, in only 3 patients the cause was identified: one anal stenosis and two anastomosis sites below the dentate line; the rest of the patients were not investigated.\u003c/p\u003e\u003cp\u003eDifferent factors are shown to affect bowel function outcomes in HSD patients following definitive surgical intervention. Joe Davidson and Joe Curry, London, found that there is a strong association between BFS and age at surgical intervention, preoperative history of HSD-associated enterocolitis, and length of agangliosis(8). Similarly, a study at the Hospital for Children and Adolescents, Finland, showed that age at surgical intervention has an inverse relation with BFS. They also found that associated congenital anomalies and length of agangliosis negatively affect bowel function outcomes (10). In our study, however, there is no relation seen between bowel function outcome and age of the patient, age at diagnosis or definitive surgical intervention, and associated congenital anomalies. However significant association was seen between the type of initial presentation of the patient and bowel function score, as patients presented with chronic constipation (P\u0026thinsp;=\u0026thinsp;0.045, AOR\u0026thinsp;=\u0026thinsp;1.474) tended to develop bowel function disorder postoperatively.\u003c/p\u003e\u003c/div\u003e\u003cdiv id=\"Sec17\" class=\"Section2\"\u003e\u003ch2\u003eAnorectal malformation\u003c/h2\u003e\u003cp\u003eARM is one of the common congenital neonatal obstructions having due effect on long-term bowel function. According to this study, about 52.2% of the patients were female, which is comparable with the finding at TASH, but contradicting the finding of a study done in Ibadan, Nigeria, where 69.3% were male(1,6). In our study, the age at diagnosis varies from as early as 1 day to as late as 5 years, with a median of 10 days. This is earlier than findings at TASH and Tanzania, where only 20% were diagnosed in the first month of life, and 50 days was the median age at diagnosis, respectively(1,12). The majority of these patients,12(52.2%), were referred from the gynaecology/obstetric side, possibly contributing to relatively early presentation.\u003c/p\u003e\u003cp\u003eARM is known for its association with other congenital anomalies. About 39.1% of the patients had associated congenital anomalies, renal anomalies being the most common anomalies seen. This is higher than the finding in Tanzania, where only 16% had associated congenital anomalies, but lower than the finding at TASH, where almost half of the patients had associated anomalies, the genitourinary system being the most common(1,12). Similar to findings at TASH and in the UK, ARM with RVF and ARM with RUF were the most common type seen in females and males, respectively(1,5).\u003c/p\u003e\u003cp\u003eDue to the nature of the disease and surgical intervention affecting anorectal anatomy, including the sphincter mechanism, patients with ARM can develop bowel function disorders. In our study, we found that in patients with ARM after anoplasty is done, 17(74.0%) have Rintala Bowel function scores of above 16, while 3(13%) each are between 9 and 16, and less than or equal to 9. This is a better finding than the finding at the Hospital for Children and Adolescents in Finland, where around 32% had a BFS of less than 17, but comparable with the finding of a study done in Tanzania, where around 16% had poor BFS(12).\u003c/p\u003e\u003cp\u003eIn patients with ARM, different factors are known to affect BFS. In our study, we have found that age at diagnosis and age at definitive surgical intervention are inversely related to bowel function outcome score. Similar to the findings at the Hospital for Children and Adolescents in Finland and the study done in the UK, the type of ARM was strongly related to bowel function outcome, as the severe ARM type was associated with poor outcomes (5,31). However, we didn\u0026rsquo;t find a significant association between bowel function outcome and associated congenital anomalies, contradicting findings by Daniel H. Teitelbaum, Tanta University, Egypt, where 44% of patients with associated congenital anomalies had poor bowel outcomes (32). The fact that only a single patient had vertebral anomalies may have contributed to this non-significant association between BFS and associated congenital anomalies in our findings.\u003c/p\u003e\u003c/div\u003e"},{"header":"Conclusion","content":"\u003cp\u003eA significant number of children who have undergone definitive repair for HSD and ARM have fair to poor bowel function. However, the majority of these patients did not undergo any type of evaluation due to poor post-operative follow-up. It is also noticed that early presentation and intervention are associated with better outcomes.\u003c/p\u003e\u003cdiv id=\"Sec19\" class=\"Section2\"\u003e\u003ch2\u003eRecommendation\u003c/h2\u003e\u003cp\u003eEarly surgical intervention and close postoperative follow-up are recommended for better long-term outcomes in patients with HSD and ARM.\u003c/p\u003e\u003c/div\u003e"},{"header":"Abbreviations","content":"\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 132px;\"\u003e\n \u003cp\u003eAOR\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 408px;\"\u003e\n \u003cp\u003eAdjusted Odd Ratio\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 132px;\"\u003e\n \u003cp\u003eBFS\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 408px;\"\u003e\n \u003cp\u003eBowel function score\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 132px;\"\u003e\n \u003cp\u003eEC\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 408px;\"\u003e\n \u003cp\u003eEthiopian calendar\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 132px;\"\u003e\n \u003cp\u003eHSD\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 408px;\"\u003e\n \u003cp\u003eHirschsprung disease\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 132px;\"\u003e\n \u003cp\u003eMD\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 408px;\"\u003e\n \u003cp\u003eMedical doctor\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 132px;\"\u003e\n \u003cp\u003eSPHMMC\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 408px;\"\u003e\n \u003cp\u003eSaint Paul\u0026rsquo;s Hospital Medical Millennium College\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 132px;\"\u003e\n \u003cp\u003eTASH\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 408px;\"\u003e\n \u003cp\u003eTikur Anbessa specialized Hospital\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 132px;\"\u003e\n \u003cp\u003eTERPT\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 408px;\"\u003e\n \u003cp\u003eTransanal endorectal pull-through\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 132px;\"\u003e\n \u003cp\u003eUK\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 408px;\"\u003e\n \u003cp\u003eUnited Kingdom\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eEthical approval for this study was obtained from the IRB of SPHMMC. Informed written consent was taken from the legal guardians of children who are enrolled in this study.\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets used during the study are available from the corresponding author on reasonable request \u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no competing interests.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e: This study is not funded\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eClinical trial number:\u003c/strong\u003e Not applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eMGE participated in the conceptualization and designing of the study. TGM participated in the conceptualization, designing data collection and writing of the manuscript. HGA participated in conceptualization and designing, data analysis, clearing and interpretation.\u0026nbsp;\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eGama M, Tadesse A. Management of Anorectal Malformation: Experience from Ethiopia. Annals of African Surgery. 2018;15(1):25\u0026ndash;8. \u003c/li\u003e\n\u003cli\u003eMoore SW, Sidler D, Hadley GP. Anorectal malformations in Africa. South African Journal of Surgery. 2005;43(4):174\u0026ndash;5. \u003c/li\u003e\n\u003cli\u003eMohammed M, Amezene T, Tamirat M. Intestinal Obstruction in Early Neonatal Period: A 3-Year Review Of Admitted Cases from a Tertiary Hospital in Ethiopia. Ethiop J Health Sci. 2017 Jul 1;27(4):393\u0026ndash;400. \u003c/li\u003e\n\u003cli\u003eKindsew A. Treatment outcome of Neonatal Intestinal Obstruction and its Associated factors at Tibebegion Comprehensive Specialized Hospital and Felegehiwot Comprehensive Specialized Hospital; North West Ethiopia [Internet]. 2020. Available from: http://dspace.orghttp://ir.bdu.edu.et/handle/123456789/13706\u003c/li\u003e\n\u003cli\u003eGoyal A, Williams JM, Kenny SE, Lwin R, Baillie CT, Lamont GL, et al. Functional outcome and quality of life in anorectal malformations. J Pediatr Surg. 2006;41(2):318\u0026ndash;22. \u003c/li\u003e\n\u003cli\u003eOgundoyin OO, Olulana DI, Lawal TA. Experience with the management of anorectal malformations in Ibadan, Nigeria. Pan African Medical Journal. 2021;38. \u003c/li\u003e\n\u003cli\u003eDewberry L, Alaniz V, Wilcox DT, de La Torre L, Pe\u0026ntilde;a A, Bischoff A. From Prenatal Diagnosis Through Transition to Adult Care: What Everyone Should Know About Anorectal Malformations. Vol. 6, Current Treatment Options in Pediatrics. Springer; 2020. p. 91\u0026ndash;100. \u003c/li\u003e\n\u003cli\u003eDavidson JR, Kyrklund K, Eaton S, Pakarinen MP, Thompson DS, Cross K, et al. Long-term surgical and patient-reported outcomes of Hirschsprung Disease. J Pediatr Surg. 2021 Sep 1;56(9):1502\u0026ndash;11. \u003c/li\u003e\n\u003cli\u003eGunadi, Karina SM, Dwihantoro A. Outcomes in patients with Hirschsprung disease following definitive surgery. BMC Res Notes. 2018;11(1). \u003c/li\u003e\n\u003cli\u003eRintala RJ, Pakarinen MP. Long-term outcomes of Hirschsprung\u0026rsquo;s disease. Semin Pediatr Surg. 2012 Nov;21(4):336\u0026ndash;43. \u003c/li\u003e\n\u003cli\u003eWester T, Granstr\u0026ouml;m AL. Hirschsprung disease\u0026mdash;Bowel function beyond childhood. Semin Pediatr Surg. 2017 Oct 1;26(5):322\u0026ndash;7. \u003c/li\u003e\n\u003cli\u003eMfinanga RJ, Massenga A, Mashuda F, Gilyoma JM, Chalya PL. Clinical profile and outcome of surgical management of anorectal malformations at a tertiary care hospital in Tanzania. Tanzan J Health Res. 2018;20(1):1\u0026ndash;11. \u003c/li\u003e\n\u003cli\u003eMurthi GVS, Raine PAM. Preoperative enterocolitis is associated with poorer long-term bowel function after soave-boley endorectal pull-through for Hirschsprung\u0026rsquo;s disease. J Pediatr Surg. 2003;38(1):69\u0026ndash;72. \u003c/li\u003e\n\u003cli\u003eGosaye AW, Nane TS, Negussie TM. A case report of Hirschsprung\u0026rsquo;s disease presenting as sigmoid volvulus and literature review, Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia. BMC Surg. 2021 Dec 1;21(1). \u003c/li\u003e\n\u003cli\u003eRintala RJ. Congenital anorectal malformations: Anything new? J Pediatr Gastroenterol Nutr. 2009;48(SUPPL. 2). \u003c/li\u003e\n\u003cli\u003eRangel SJ, de Blaauw I. Advances in pediatric colorectal surgical techniques. Semin Pediatr Surg [Internet]. 2010;19(2):86\u0026ndash;95. Available from: http://dx.doi.org/10.1053/j.sempedsurg.2009.11.018\u003c/li\u003e\n\u003cli\u003eDivarci E, Ergun O. General complications after surgery for anorectal malformations. Vol. 36, Pediatric Surgery International. Springer; 2020. p. 431\u0026ndash;45. \u003c/li\u003e\n\u003cli\u003eYanchar NL, Ottawa PS. Long-Term Outcome After Hirschsprung\u0026rsquo;s Disease: Patients\u0026rsquo; Perspectives. \u003c/li\u003e\n\u003cli\u003eMeinds RJ, van der Steeg AFW, Sloots CEJ, Witvliet MJ, de Blaauw I, van Gemert WG, et al. Long-term functional outcomes and quality of life in patients with Hirschsprung\u0026rsquo;s disease. British Journal of Surgery. 2019 Mar 1;106(4):499\u0026ndash;507. \u003c/li\u003e\n\u003cli\u003eRoorda D, Witvliet MJ, Wellens LM, Schulten D V., Sloots CEJ, de Blaauw I, et al. Long-term outcome and quality of life in patients with total colonic aganglionosis in the netherlands. Colorectal Disease. 2018;20(8):719\u0026ndash;26. \u003c/li\u003e\n\u003cli\u003eRintala RJ, Lindahl HG, Rasanen Helsinki M. Do Children With Repaired Low Anorectal Malformations Have Normal Bowel Function? \u003c/li\u003e\n\u003cli\u003eBorg HC, Holmdahl G, Gustavsson K, Doroszkiewicz M, Sill\u0026eacute;n U. Longitudinal study of bowel function in children with anorectal malformations. J Pediatr Surg. 2013 Mar;48(3):597\u0026ndash;606. \u003c/li\u003e\n\u003cli\u003eBischoff A, Bealer J, Pe\u0026ntilde;a A. Critical analysis of fecal incontinence scores. Pediatr Surg Int. 2016 Aug 1;32(8):737\u0026ndash;41. \u003c/li\u003e\n\u003cli\u003eTownley OG, Lindley RM, Cohen MC, Murthi G V. Functional outcome, quality of life, and \u0026lsquo;failures\u0026rsquo; following pull-through surgery for Hirschsprung\u0026rsquo;s disease: A review of practice at a single-center. J Pediatr Surg. 2020 Feb 1;55(2):273\u0026ndash;7. \u003c/li\u003e\n\u003cli\u003eThakkar HS, Bassett C, Hsu A, Manuele R, Kufeji D, Richards CA, et al. Functional outcomes in Hirschsprung disease: A single institution\u0026rsquo;s 12-year experience. J Pediatr Surg. 2017 Feb 1;52(2):277\u0026ndash;80. \u003c/li\u003e\n\u003cli\u003eMabula JB, Kayange NM, Manyama M, Chandika AB, Rambau PF, Chalya PL. Hirschsprung\u0026rsquo;s disease in children: a five year experience at a University teaching hospital in northwestern Tanzania [Internet]. 2014. Available from: http://www.biomedcentral.com/1756-0500/7/410\u003c/li\u003e\n\u003cli\u003eNegash S, Getachew H, Tamirat D, Mammo TN. Hirschsprung disease managed with one-stage transanal endorectal pullthrough in a low-resource setting without frozen section. BMC Surg. 2022 Dec 1;22(1). \u003c/li\u003e\n\u003cli\u003eNegash S, Getachew H, Tamirat D, Mammo TN. Hirschsprung disease managed with one-stage transanal endorectal pullthrough in a low-resource setting without frozen section. BMC Surg. 2022 Dec 1;22(1). \u003c/li\u003e\n\u003cli\u003eGunadi, Monica Carissa T, Stevie, Daulay EF, Yulianda D, Iskandar K, et al. Long-term functional outcomes of patients with Hirschsprung disease following pull-through. BMC Pediatr. 2022 Dec 1;22(1). \u003c/li\u003e\n\u003cli\u003eMoore SW, Albertyn R, Cywes Tygerberg S. Clinical Outcome and Long-Term Quality of Life After Surgical Correction of Hirschsprung\u0026rsquo;s Disease. \u003c/li\u003e\n\u003cli\u003ePakarinen MP, Koivusalo A, Lindahl H, Rintala RJ. Prospective Controlled Long-term Follow-up for Functional Outcome After Anoplasty in Boys With Perineal Fistula [Internet]. 2007. Available from: http://journals.lww.com/jpgn\u003c/li\u003e\n\u003cli\u003eHashish MS, Dawoud HH, Hirschl RB, Bruch SW, El Batarny AM, Mychaliska GB, et al. Long-term functional outcome and quality of life in patients with high imperforate anus. J Pediatr Surg [Internet]. 2010;45(1):224\u0026ndash;30. Available from: http://dx.doi.org/10.1016/j.jpedsurg.2009.10.041\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Hirschsprung’s disease, anorectal malformation, Bowel function score","lastPublishedDoi":"10.21203/rs.3.rs-7330027/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7330027/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eIntroduction:\u003c/h2\u003e\u003cp\u003eHirschsprung disease (HSD) and anorectal malformation (ARM) are among the common congenital anomalies encountered in the practice of pediatric surgery. Children who have undergone definitive repair for these disorders may suffer from various complications. This study aims to assess bowel function and the associated factors in patients from a low-income country, as this matter has remained largely unreported in this setting.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e\u003cp\u003e A retrospective review of children who underwent definitive surgical repair for HSD and ARM, and who were 3 years or older, was included in the study. Rintala's bowel function score was used to assess the outcome. The chi-square test was used to assess the association between several variables and bowel function score. All associations with a P value of \u0026lt;\u0026thinsp;0.05 were considered significant.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e\u003cp\u003eA total of 132 children were operated on in the study period, and 100 of them were above 3 years and beyond 6 months after the surgery. Among these, the charts of 81 patients were available for analysis. HSD patients accounted for 58(71.6%) of the study participants with a mean age of 4.2 +/- 1.4 years and male to female ratio of 2.4:1. Patients for ARM constituted for 23(28.4%) of the study population with mean age of 3.9 +/- 1.4 years and male to female ratio of 0.9:1. Among children who have undergone pull through, the Rintala bowel function score was good in 37(64%), fair in 16(27.6%) and poor in 5(8.6%) of patients while it was good in 37(74%), fair in 3(13%) and poor in 3(13%) of ARM patients. Late presentation with chronic constipation (P\u0026thinsp;=\u0026thinsp;0.045, OR\u0026thinsp;=\u0026thinsp;1.474) was found to be significantly associated with poor bowel function score in patients with HSD, while the type of ARM (P\u0026thinsp;=\u0026thinsp;0.000, OR\u0026thinsp;=\u0026thinsp;2.545) and late age at presentation (P\u0026thinsp;=\u0026thinsp;0.024, OR\u0026thinsp;=\u0026thinsp;1.247) were found to be the significant variables for ARM patients.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e\u003cp\u003eA significant number of children who have undergone definitive repair for HSD and ARM have fair to poor bowel function scores. Late presentation of children with these pathologies is found to be a significant contributor to the poor outcome in low-income countries.\u003c/p\u003e","manuscriptTitle":"Bowel function assessment and affecting factors in patients operated for Hirschsprung’s disease and Anorectal malformations in a resource-limited area: A five-year review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-08-27 06:09:27","doi":"10.21203/rs.3.rs-7330027/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"1dc1b88a-0a8b-4a7a-b019-7577d586dd20","owner":[],"postedDate":"August 27th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-10-27T15:37:37+00:00","versionOfRecord":[],"versionCreatedAt":"2025-08-27 06:09:27","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7330027","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7330027","identity":"rs-7330027","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}