Challenges and Outcomes in the Management of Rectal Atresia and Stenosis: A Comprehensive Review

Challenges and Outcomes in the Management of Rectal Atresia and Stenosis: A Comprehensive 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 Challenges and Outcomes in the Management of Rectal Atresia and Stenosis: A Comprehensive Review Juan Ignacio Siffredi, Lucila Alvarez, Maria Victoria Dominguez Marquez, and 5 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-9316945/v1 This work is licensed under a CC BY 4.0 License Status: Under Review Version 1 posted 9 You are reading this latest preprint version Abstract Purpose Rectal atresia (RA) and rectal stenosis (RS) are rare anorectal malformations (ARM), comprising 1–2% of all cases. No consensus exists regarding optimal surgical technique. This study evaluated surgical approaches, complications, and long-term functional outcomes at a single referral center. Methods Retrospective review of patients with RA or RS treated between 2003 and 2020. Surgical technique, complications, and bowel function were analyzed. Fecal continence was assessed using the Krickenbeck classification in patients older than 3 years with minimum 6-month follow-up after colostomy closure. Results Eleven patients were included (7 RS, 4 RA), representing 1.2% of 900 ARM cases. Surgical approaches included posterior sagittal anorectoplasty (PSARP) in 4 (36.4%), transanal in 4 (36.4%), abdominoperineal pull-through in 1 (9.1%), and laparoscopic endorectal technique in 2 (18.2%). Two patients required redo surgery. Of 9 eligible for functional assessment, 6 (66.7%) achieved fecal continence, 3 without additional treatment. Three had constipation and soiling due to poor adherence. Median follow-up was 100.4 months (range 13–168). Conclusion Despite surgical complexity, most patients achieved satisfactory outcomes. The high incidence of constipation, particularly in RS, underscores the need for long-term follow-up and early intervention. The laparoscopic endorectal technique offers a reproducible, sphincter-preserving approach to rectal web correction. Rectal atresia Rectal stenosis Anorectal malformations Laparoscopic endorectal technique Fecal continence Long-term outcomes Figures Figure 1 Introduction Rectal atresia (RA) and rectal stenosis (RS) are among the rarest forms of anorectal malformations (ARM), comprising 1–2% of all cases according to the Krickenbeck classification [ 1 , 2 ]. Both entities are characterized by the presence of an anatomically normal anus within an intact sphincter complex, a feature that distinguishes them from other ARM subtypes and poses unique diagnostic and surgical challenges. Both congenital defects are characterized by the presence of a normal anus located within the anal sphincter complex. Sometimes it appears skin lined and it is known as funnel anus. The diagnosis of RA is suspected when a newborn presents obstruction and impossibility to pass a rectal catheter through an apparently normal anal canal. Conversely, patients with RS may pass meconium, mimicking Hirschsprung disease or severe constipation, leading to suspicion due to the inability to pass a rectal catheter. Several surgical techniques have been described for the correction of RA and RS, including posterior sagittal anorectoplasty (PSARP), transanal (TA) approaches, abdominoperineal pull-through (APT), and laparoscopic-assisted techniques [ 3 – 9 ]. However, the rarity of these conditions has precluded the development of evidence-based consensus guidelines, and surgical decision-making remains largely individualized based on institutional experience and anatomical findings. The aim of this study was to describe our single-center experience in the surgical management of RA and RS, with emphasis on technique selection rationale, complications, and long-term functional outcomes. We additionally describe our experience with a laparoscopic endorectal (LE) technique for rectal web correction as a reproducible, minimally invasive option in selected cases. Methods Study design and patient selection A retrospective review was conducted of all patients diagnosed with RA or RS and treated at our institution between January 2003 and December 2020. RA and RS were analyzed as distinct entities given their differences in presentation, timing of diagnosis, and surgical management. The study was approved by the institutional review board. Patients with fewer than 6 months of postoperative follow-up were excluded. Preoperative workup All patients underwent systematic preoperative evaluation including abdominal and lumbosacral ultrasound and plain radiography to assess sacral anatomy and screen for presacral masses. Magnetic resonance imaging (MRI) was performed in selected cases based on clinical suspicion. Distal colostogram or barium enema was conducted to delineate rectal anatomy and guide surgical planning. The sacral ratio was calculated according to standard methodology [ 1 ]. Surgical techniques Four surgical approaches were employed based on anatomical findings and clinical context: PSARP: indicated for RA and RS with complex anatomy or those requiring combined perineal and abdominal access. Transanal (TA): used for RS with accessible anatomy allowing primary correction without colostomy in selected patients. Abdominoperineal pull-through (APT): employed in one patient with extensive rectal fibrosis and complex anatomy; the specific operative rationale was not fully retrievable from the available records given the retrospective nature of the study. Laparoscopic endorectal (LE) technique: applied in patients with rectal web and severe caudal regression precluding standard prone jackknife positioning. Three trocars were placed (4 mm umbilical, 3 mm left flank, 5 mm right flank). The anterior rectal wall was opened longitudinally at the caliber change zone. The web was excised under direct laparoscopic vision, and rectal wall closure was performed transanally or laparoscopically according to the distance between the rectal opening and the anal canal (Fig. 1 ). Patients with RA underwent diverting colostomy in the neonatal period prior to definitive correction. Three RS patients with adequate fecal decompression capacity underwent primary correction without colostomy using a TA approach. All patients underwent digital dilations beginning 15 days post-surgery, extending until colostomy closure or, in cases of primary correction, for 1 month following a weekly dilations schedule. Outcome assessment The primary outcome was fecal continence at follow-up, assessed using the Krickenbeck classification [ 1 ]. Patients were eligible for functional assessment if they were older than 3 years and had completed a minimum of 6 months of follow-up after colostomy closure. Patients with severe neurological impairment precluding reliable functional evaluation were excluded from continence assessment. Secondary outcomes included constipation rate, surgical complications, and need for reintervention. Follow-up was conducted by a multidisciplinary team including pediatric surgery, gastroenterology, and urology. . Results Patient demographics and associated anomalies Out of 900 ARM patients managed at our institution during the study period, 11 (1.2%) were eligible for inclusion: 7 RS (63.6%) and 4 RA (36.4%). Seven patients (63.6%) were male and 4 (36.4%) female. Mean age at diagnosis was 2.25 days (range 2–3 days) for RA and 15.43 months (range 0–57 months) for RS, consistent with the expected later presentation of RS due to partial luminal patency. Associated anomalies were identified in 9 patients (81.8%). Urinary anomalies were present in 4 patients, cardiac anomalies in 2, caudal regression in 2, and severe neurological impairment in 1. Presacral masses were identified in 2 patients (18.2%), both anterior meningoceles, which were resected prior to definitive colorectal repair. Sacral ratios were normal (> 0.7) in 2 patients (18.2%), intermediate (0.4–0.7) in 5 (45.5%), and severely abnormal (< 0.4) in 4 (36.4%). Surgical management Mean age at definitive surgery was 26.4 months (27.9 months for RS and 23.8 months for RA). Surgical approach was individualized based on anatomical findings, sacral morphology, and patient positioning constraints (Table 1 ). For RA patients (n = 4): LE technique was used in 2 (50%), PSARP in 1 (25%), and TA approach in 1 (25%). For RS patients (n = 7): PSARP in 3 (42.9%), TA in 3 (42.9%), and APT in 1 (14.3%). The APT approach was selected for a patient with extensive rectal fibrosis and complex anatomy; complete operative documentation was not available for retrospective verification. Three RS patients underwent primary correction without colostomy via TA approach, given adequate fecal decompression capacity. Four RS patients had a preceding diverting colostomy. Two patients (18.2%, one RA, one RS) required redo surgery: one for persistent stenosis and one for rectovaginal fistula, both following PSARP. No intraoperative or immediate postoperative complications were recorded for patients managed with the LE technique. Table 1 Surgical approach by diagnosis and clinical rationale Patient Diagnosis Technique Rationale Outcome 1 RA LE Rectal web + severe caudal regression — positioning constraints Continent — no treatment required 2 RA LE Rectal web + severe caudal regression — positioning constraints Not evaluable — severe neurological impairment 3 RA PSARP Complex anatomy Continent 4 RA TA Accessible anatomy, primary correction Continent 5 RS PSARP Complex anatomy, prior colostomy Redo surgery (persistent stenosis) 6 RS PSARP Complex anatomy, prior colostomy Redo surgery (rectovaginal fistula) 7 RS PSARP Complex anatomy Continent with laxatives 8 RS TA Primary correction — adequate decompression Constipation and soiling (non-adherence) 9 RS TA Primary correction — adequate decompression Constipation and soiling (non-adherence) 10 RS TA Primary correction — adequate decompression Constipation and soiling (non-adherence) 11 RS APT Extensive rectal fibrosis — complex anatomy (operative rationale not fully retrievable) Continent with enemas Functional outcomes Median follow-up was 100.4 months (range 13–168 months). Nine of 11 patients were eligible for fecal continence assessment. Two patients were excluded: one due to severe neurological impairment precluding reliable functional evaluation, and one due to age below 3 years at the time of analysis. Of the 9 eligible patients, 6 (66.7%) achieved fecal continence based on Krickenbeck criteria: 2/3 RA patients (66.7%) and 4/6 RS patients (66.7%). Among continent patients, 3 (50%) required no treatment, 2 (33.3%) used laxatives, and 1 (16.7%) required enemas. Three patients (33.3%) experienced constipation and soiling; all three were RS patients who had undergone TA primary correction and shared a common pattern of non-adherence to the prescribed bowel management program. No patient achieved continence without some degree of constipation management in the RS group. Both patients managed with the LE technique had favorable postoperative courses with no technique-specific complications. One achieved full continence without treatment; the second was excluded from functional assessment due to the aforementioned neurological condition. Discussion This study presents a single-center series of 11 patients with RA and RS with a median follow-up of over 8 years, one of the longest reported in the literature for this rare condition. Our overall continence rate of 66.7% among eligible patients is consistent with published data, which reports continence rates ranging from 50% to 80% depending on the series and follow-up duration [ 7 , 10 , 11 ]. The ARM-Net Consortium recently published the largest multicenter series of RA and RS to date, including 49 patients with a median follow-up of 43 months [ 11 ]. Their reported continence rate of 69% aligns closely with our findings despite methodological differences, lending external validity to our results. Notably, our follow-up period is more than twice as long, providing insight into the long-term trajectory of these patients beyond what multicenter studies have captured. A central finding of our study is the notable incidence of constipation, particularly in the RS group, a pattern consistent with the literature [ 11 , 12 ]. We identified two potential mechanisms that likely contribute to this observation. First, RS falls within the low ARM spectrum, which carries an inherently higher constipation rate due to preserved but dysfunctional anorectal sensory mechanisms. Second, and critically, the ability of RS patients to pass stool through a stenotic lumen delays diagnosis — in our series, only 1 of 7 RS patients (14%) received timely diagnosis, versus all 4 RA patients who were identified neonatally. Prolonged diagnostic delay allows progressive rectal distension and megarectum formation, which likely compounds postoperative constipation regardless of technical outcome. In our series, sacral ratio did not consistently predict functional outcome, with patients across the full range of sacral morphology achieving continence. Given the small sample size, no conclusions can be drawn regarding the predictive value of sacral ratio in this specific population. Regarding surgical approach, our experience supports individualized technique selection over a standardized algorithm. The absence of consensus in the literature reflects the anatomical heterogeneity of these conditions rather than a failure of any single technique. The TA approach without prior colostomy was feasible and effective in 3 RS patients with adequate fecal decompression, consistent with recent series reporting successful primary correction in selected cases [ 10 ]. The APT approach was employed in one patient with extensive rectal fibrosis and complex anatomy; although complete operative documentation was not available for retrospective verification, this case illustrates the need for flexible surgical planning in anatomically challenging presentations. The laparoscopic endorectal technique was employed in two patients presenting with rectal web and severe caudal regression precluding standard prone jackknife positioning. This approach allowed direct visualization of the caliber change zone, precise web excision, and valuable intraoperative assessment of anatomical variants, rectal caliber, and the distance between the resection site and the anal canal — which guided the decision between transanal and laparoscopic rectal wall closure. Both patients had uncomplicated postoperative courses. Previous reports have described laparoscopic approaches in rectal atresia [ 6 ]. The laparoscopic endorectal technique described in this article represents a novel approach for rectal web correction that, beyond fulfilling the primary surgical objective of preserving sphincter integrity, offers the additional advantage of intraoperative laparoscopic assessment of anatomical variants, rectal caliber, and the distance between the resection site and the anal canal — information that directly guides the surgical strategy. The reintervention rate of 18.2% in our series (2 patients, both following PSARP) is within the range reported in the literature [ 7 , 11 ]. Both cases involved technically demanding anatomy, and the complications — persistent stenosis and rectovaginal fistula — are recognized challenges of PSARP in this context rather than technique-specific failures. This study has several limitations. The retrospective design and small sample size, inherent to the rarity of these conditions, limit statistical analysis and preclude formal comparison between techniques. The exclusion of two patients from functional assessment reduces the analyzable cohort further. Additionally, the non-adherence to bowel management observed in three RS patients may reflect socioeconomic or systemic barriers not captured in our data, and their outcomes should be interpreted in that context rather than as treatment failures. One patient was excluded from functional assessment due to age below 3 years at the time of data collection; this patient has since reached evaluable age and prospective follow-up is ongoing. Notwithstanding these limitations, our series contributes meaningful data on the long-term natural history of RA and RS, underscores the importance of systematic multidisciplinary follow-up, and provides early technical experience with the LE approach for rectal web correction. Conclusion Rectal atresia and stenosis remain among the most surgically challenging anorectal malformations, yet individualized management yields satisfactory continence outcomes in most patients. Notably, timely diagnosis was achieved in all RA patients, while only 1 of 7 RS patients (14%) was diagnosed without delay — a finding that may reflect the partial luminal patency inherent to RS and warrants heightened clinical awareness. Delayed diagnosis may contribute to progressive rectal distension and megarectum formation, potentially compounding postoperative constipation regardless of surgical technique. The high burden of constipation — particularly in RS — highlights the need for systematic postoperative follow-up and proactive bowel management. The laparoscopic endorectal technique represents a reproducible, sphincter-preserving option for rectal web correction with favorable early outcomes. Multicenter collaboration remains essential to generate sufficient evidence for guideline development in this rare condition. Declarations Compliance with Ethical Standards Conflict of interest : The authors declare no conflict of interest. Ethical approval: This study was approved by the institutional review board. All procedures were performed in accordance with the ethical standards of the institution and with the 1964 Helsinki Declaration and its later amendments. Informed consent: Informed consent was obtained from the parents or legal guardians of all patients included in the study. Funding No funding was received for conducting this study. Author Contribution JIS: Conceptualization, Data collection, Formal analysis, Writing – original draft, Writing – review and editing. LA: Data collection, Formal analysis, Writing – review and editing. MVDM: Data collection. JR, MDN, AMR: Writing – review and editing. VPDB, MMB: Supervision. All authors read and approved the final manuscript. Data Availability The data that support the findings of this study are not publicly available as they are contained in institutional medical records. Data are available from the corresponding author upon reasonable request and subject to institutional approval. References Holschneider A, Hutson J, Peña A, Beket E, Chatterjee S, Coran A, Davies M, Georgeson K, Grosfeld J, Gupta D, Iwai N, Kluth D, Martucciello G, Moore S, Rintala R, Smith ED, Sripathi DV, Stephens D, Sen S, Ure B, Grasshoff S, Boemers T, Murphy F, Söylet Y, Dübbers M, Kunst M (2005) Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. Journal of Pediatric Surgery. octubre de. ;40(10):1521-6. 10.1016/j.jpedsurg.2005.08.002 Peña A, Levitt MA (2005) Imperforate anus and cloacal malformations. En: Ashcraft KW, Holcomb GW, Murphy JP, editors. Pediatric Surgery. 4. a ed. Philadelphia: Elsevier Saunders; pp. 496–517 Hamzaoui M, Ghribi A, Makni W, Sghairoun N, Gasmi M (2012) Rectal and sigmoid atresia: transanal approach. J Pediatr Surg junio de 47(6):e41–e44. 10.1016/j.jpedsurg.2012.02.025 Dias RG, Santiago ADPG, Ferreira MC (1982) Rectal atresia: Treatment through a single sacral approach. J Pediatr Surg agosto de 17(4):424–425. 10.1016/S0022-3468(82)80506-X Zia-ul-Miraj Ahmad M, Brereton RJ, Huskisson L (1995) Rectal atresia and stenosis. J Pediatr Surg noviembre de 30(11):1546–1550. 10.1016/0022-3468(95)90153-1 Liem NT, Hien PD (2007) Laparoscopic and transanal approach for rectal atresia: a novel alternative. J Pediatr Surg noviembre de 42(11):e25–e27. 10.1016/j.jpedsurg.2007.08.049 Hamrick M, Eradi B, Bischoff A, Louden E, Peña A, Levitt M (2012) Rectal atresia and stenosis: unique anorectal malformations. J Pediatr Surg junio de 47(6):1280–1284. 10.1016/j.jpedsurg.2012.03.036 Russell KW, Rollins MD, Feola GP, Scaife ER (2014) Magnamosis: a novel technique for the management of rectal atresia. BMJ Case Rep 5 de agosto de 2014:bcr2013201330. 10.1136/bcr-2013-201330 Lane VA, Wood RJ, Reck C, Skerritt C, Levitt MA (2016) Rectal atresia and anal stenosis: the difference in the operative technique for these two distinct congenital anorectal malformations. Tech Coloproctol abril de 20(4):249–254. 10.1007/s10151-016-1435-5 Sharma S, Gupta DK (2017) Varied facets of rectal atresia and rectal stenosis. Pediatr Surg Int agosto de 33(8):829–836. 10.1007/s00383-017-4106-3 De Beaufort CMC, Gorter RR, Iacobelli BD, Midrio P, Sloots CEJ, Samuk I, Van Rooij IALM, Lisi G, for the ARM-Net Consortium, De Blaauw I, Fascetti-Leon F, Vázquez AG, Krois W, Lacher M, Leva E, Schmiedeke E, Schukfeh N, Stanton M (2023) Rectal atresia and rectal stenosis: the ARM-Net Consortium experience. Pediatr Surg Int 28 de julio de 39(1):242. 10.1007/s00383-023-05518-7 Rintala RJ, Lindahl HG, Rasanen M (1997) Do children with repaired low anorectal malformations have normal bowel function? J Pediatr Surg junio de 32(6):823–826. 10.1016/S0022-3468(97)90628-X Additional Declarations No competing interests reported. Cite Share Download PDF Status: Under Review Version 1 posted Editorial decision: Revision requested 30 Apr, 2026 Reviews received at journal 29 Apr, 2026 Reviews received at journal 27 Apr, 2026 Reviewers agreed at journal 18 Apr, 2026 Reviewers agreed at journal 16 Apr, 2026 Reviewers invited by journal 16 Apr, 2026 Editor assigned by journal 07 Apr, 2026 Submission checks completed at journal 07 Apr, 2026 First submitted to journal 03 Apr, 2026 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-9316945","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":625438824,"identity":"66c5be13-d4f1-4525-b079-4a0e745dcf1f","order_by":0,"name":"Juan Ignacio Siffredi","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA70lEQVRIiWNgGAWjYBACxgYQaSDHDyIPfACSbOzEaTGWBNIGB2eAtDATZxlECzMPiE1IC/OM5GcPfxQYSJi3N288bPNrmzwfMwPjh485eBw2I83cmMfAQELmzLGCw7l9tw3bmBmYJWduw6clwUyaweBPnYREjsHh3J7bjEAtbMy8eLWkf5P8AbRFQv6NwWHLntv2RGjJMZMAOUwCSB5m+HE7kbCWnjdl0mAtPGkFB3sbbie3MTM24/WLYXv6Nskff4Ba2A9v/vDjz23b+e3NBz98xKelAcXONjDZgE0lHMijcv/gVTwKRsEoGAUjFAAAug9M5HqYZaIAAAAASUVORK5CYII=","orcid":"","institution":"Garrahan Hospital","correspondingAuthor":true,"prefix":"","firstName":"Juan","middleName":"Ignacio","lastName":"Siffredi","suffix":""},{"id":625438825,"identity":"3a67392d-e3b3-49e5-b999-5a1b67ad5fc4","order_by":1,"name":"Lucila Alvarez","email":"","orcid":"","institution":"Garrahan Hospital","correspondingAuthor":false,"prefix":"","firstName":"Lucila","middleName":"","lastName":"Alvarez","suffix":""},{"id":625438832,"identity":"63957888-7daa-4038-b731-f61335282612","order_by":2,"name":"Maria Victoria Dominguez Marquez","email":"","orcid":"","institution":"Garrahan Hospital","correspondingAuthor":false,"prefix":"","firstName":"Maria","middleName":"Victoria Dominguez","lastName":"Marquez","suffix":""},{"id":625438834,"identity":"c6a18d64-6a38-4271-a199-c067dcec97c4","order_by":3,"name":"Javier Ruiz","email":"","orcid":"","institution":"Garrahan Hospital","correspondingAuthor":false,"prefix":"","firstName":"Javier","middleName":"","lastName":"Ruiz","suffix":""},{"id":625438835,"identity":"be4c1f80-fcc5-42d7-b1be-60ada20d1fe0","order_by":4,"name":"Maria Daniela Neder","email":"","orcid":"","institution":"Garrahan Hospital","correspondingAuthor":false,"prefix":"","firstName":"Maria","middleName":"Daniela","lastName":"Neder","suffix":""},{"id":625438836,"identity":"5e3fc5b9-8c14-49a8-a1a3-5802dbc2a02d","order_by":5,"name":"Ana Marcela Rocca","email":"","orcid":"","institution":"Garrahan Hospital","correspondingAuthor":false,"prefix":"","firstName":"Ana","middleName":"Marcela","lastName":"Rocca","suffix":""},{"id":625438838,"identity":"e64b4f5f-9f3c-4915-bf03-fd65168195e4","order_by":6,"name":"Maria Marcela Bailez","email":"","orcid":"","institution":"Garrahan Hospital","correspondingAuthor":false,"prefix":"","firstName":"Maria","middleName":"Marcela","lastName":"Bailez","suffix":""},{"id":625438840,"identity":"007666dc-192f-4e5b-b420-bcc09b305029","order_by":7,"name":"Victor Pedro Di Benedetto","email":"","orcid":"","institution":"Garrahan Hospital","correspondingAuthor":false,"prefix":"","firstName":"Victor","middleName":"Pedro Di","lastName":"Benedetto","suffix":""}],"badges":[],"createdAt":"2026-04-04 02:23:58","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-9316945/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-9316945/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":107652014,"identity":"9ec07343-cb73-48de-b511-a6a3c9312d37","added_by":"auto","created_at":"2026-04-23 15:11:16","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":23325,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003eLaparoscopic view of rectal stenosis. (A) Dilated proximal stump. (B) Transition zone. (C) Distal rectum\u003c/em\u003e\u003c/p\u003e","description":"","filename":"Imagecaliberchangezonecopy.jpg","url":"https://assets-eu.researchsquare.com/files/rs-9316945/v1/de0f90cd593b1501afa31395.jpg"},{"id":107652071,"identity":"226863b9-ed52-4e23-9bef-37ab9a0d4d52","added_by":"auto","created_at":"2026-04-23 15:11:22","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":220095,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-9316945/v1/e84ddab3-37e6-4418-8627-e9167365b41e.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Challenges and Outcomes in the Management of Rectal Atresia and Stenosis: A Comprehensive Review","fulltext":[{"header":"Introduction","content":"\u003cp\u003eRectal atresia (RA) and rectal stenosis (RS) are among the rarest forms of anorectal malformations (ARM), comprising 1\u0026ndash;2% of all cases according to the Krickenbeck classification [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Both entities are characterized by the presence of an anatomically normal anus within an intact sphincter complex, a feature that distinguishes them from other ARM subtypes and poses unique diagnostic and surgical challenges.\u003c/p\u003e \u003cp\u003eBoth congenital defects are characterized by the presence of a normal anus located within the anal sphincter complex. Sometimes it appears skin lined and it is known as funnel anus. The diagnosis of RA is suspected when a newborn presents obstruction and impossibility to pass a rectal catheter through an apparently normal anal canal. Conversely, patients with RS may pass meconium, mimicking Hirschsprung disease or severe constipation, leading to suspicion due to the inability to pass a rectal catheter.\u003c/p\u003e \u003cp\u003eSeveral surgical techniques have been described for the correction of RA and RS, including posterior sagittal anorectoplasty (PSARP), transanal (TA) approaches, abdominoperineal pull-through (APT), and laparoscopic-assisted techniques [\u003cspan additionalcitationids=\"CR4 CR5 CR6 CR7 CR8\" citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. However, the rarity of these conditions has precluded the development of evidence-based consensus guidelines, and surgical decision-making remains largely individualized based on institutional experience and anatomical findings.\u003c/p\u003e \u003cp\u003eThe aim of this study was to describe our single-center experience in the surgical management of RA and RS, with emphasis on technique selection rationale, complications, and long-term functional outcomes. We additionally describe our experience with a laparoscopic endorectal (LE) technique for rectal web correction as a reproducible, minimally invasive option in selected cases.\u003c/p\u003e"},{"header":"Methods","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eStudy design and patient selection\u003c/h2\u003e \u003cp\u003eA retrospective review was conducted of all patients diagnosed with RA or RS and treated at our institution between January 2003 and December 2020. RA and RS were analyzed as distinct entities given their differences in presentation, timing of diagnosis, and surgical management. The study was approved by the institutional review board. Patients with fewer than 6 months of postoperative follow-up were excluded.\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003ePreoperative workup\u003c/h3\u003e\n\u003cp\u003eAll patients underwent systematic preoperative evaluation including abdominal and lumbosacral ultrasound and plain radiography to assess sacral anatomy and screen for presacral masses. Magnetic resonance imaging (MRI) was performed in selected cases based on clinical suspicion. Distal colostogram or barium enema was conducted to delineate rectal anatomy and guide surgical planning. The sacral ratio was calculated according to standard methodology [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e].\u003c/p\u003e\n\u003ch3\u003eSurgical techniques\u003c/h3\u003e\n\u003cp\u003eFour surgical approaches were employed based on anatomical findings and clinical context:\u003c/p\u003e \u003cp\u003e \u003cul\u003e \u003cli\u003e \u003cp\u003ePSARP: indicated for RA and RS with complex anatomy or those requiring combined perineal and abdominal access.\u003c/p\u003e \u003c/li\u003e \u003cli\u003e \u003cp\u003eTransanal (TA): used for RS with accessible anatomy allowing primary correction without colostomy in selected patients.\u003c/p\u003e \u003c/li\u003e \u003cli\u003e \u003cp\u003eAbdominoperineal pull-through (APT): employed in one patient with extensive rectal fibrosis and complex anatomy; the specific operative rationale was not fully retrievable from the available records given the retrospective nature of the study.\u003c/p\u003e \u003c/li\u003e \u003cli\u003e \u003cp\u003eLaparoscopic endorectal (LE) technique: applied in patients with rectal web and severe caudal regression precluding standard prone jackknife positioning. Three trocars were placed (4 mm umbilical, 3 mm left flank, 5 mm right flank). The anterior rectal wall was opened longitudinally at the caliber change zone. The web was excised under direct laparoscopic vision, and rectal wall closure was performed transanally or laparoscopically according to the distance between the rectal opening and the anal canal (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003c/li\u003e \u003c/ul\u003e \u003c/p\u003e \u003cp\u003ePatients with RA underwent diverting colostomy in the neonatal period prior to definitive correction. Three RS patients with adequate fecal decompression capacity underwent primary correction without colostomy using a TA approach. All patients underwent digital dilations beginning 15 days post-surgery, extending until colostomy closure or, in cases of primary correction, for 1 month following a weekly dilations schedule.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e\n\u003ch3\u003eOutcome assessment\u003c/h3\u003e\n\u003cp\u003eThe primary outcome was fecal continence at follow-up, assessed using the Krickenbeck classification [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Patients were eligible for functional assessment if they were older than 3 years and had completed a minimum of 6 months of follow-up after colostomy closure. Patients with severe neurological impairment precluding reliable functional evaluation were excluded from continence assessment. Secondary outcomes included constipation rate, surgical complications, and need for reintervention. Follow-up was conducted by a multidisciplinary team including pediatric surgery, gastroenterology, and urology.\u003c/p\u003e \u003cp\u003e.\u003c/p\u003e"},{"header":"Results","content":"\u003cdiv id=\"Sec8\" class=\"Section2\"\u003e \u003ch2\u003ePatient demographics and associated anomalies\u003c/h2\u003e \u003cp\u003eOut of 900 ARM patients managed at our institution during the study period, 11 (1.2%) were eligible for inclusion: 7 RS (63.6%) and 4 RA (36.4%). Seven patients (63.6%) were male and 4 (36.4%) female. Mean age at diagnosis was 2.25 days (range 2\u0026ndash;3 days) for RA and 15.43 months (range 0\u0026ndash;57 months) for RS, consistent with the expected later presentation of RS due to partial luminal patency.\u003c/p\u003e \u003cp\u003eAssociated anomalies were identified in 9 patients (81.8%). Urinary anomalies were present in 4 patients, cardiac anomalies in 2, caudal regression in 2, and severe neurological impairment in 1. Presacral masses were identified in 2 patients (18.2%), both anterior meningoceles, which were resected prior to definitive colorectal repair. Sacral ratios were normal (\u0026gt;\u0026thinsp;0.7) in 2 patients (18.2%), intermediate (0.4\u0026ndash;0.7) in 5 (45.5%), and severely abnormal (\u0026lt;\u0026thinsp;0.4) in 4 (36.4%).\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003eSurgical management\u003c/h3\u003e\n\u003cp\u003eMean age at definitive surgery was 26.4 months (27.9 months for RS and 23.8 months for RA). Surgical approach was individualized based on anatomical findings, sacral morphology, and patient positioning constraints (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eFor RA patients (n\u0026thinsp;=\u0026thinsp;4): LE technique was used in 2 (50%), PSARP in 1 (25%), and TA approach in 1 (25%). For RS patients (n\u0026thinsp;=\u0026thinsp;7): PSARP in 3 (42.9%), TA in 3 (42.9%), and APT in 1 (14.3%). The APT approach was selected for a patient with extensive rectal fibrosis and complex anatomy; complete operative documentation was not available for retrospective verification. Three RS patients underwent primary correction without colostomy via TA approach, given adequate fecal decompression capacity. Four RS patients had a preceding diverting colostomy.\u003c/p\u003e \u003cp\u003eTwo patients (18.2%, one RA, one RS) required redo surgery: one for persistent stenosis and one for rectovaginal fistula, both following PSARP. No intraoperative or immediate postoperative complications were recorded for patients managed with the LE technique.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eSurgical approach by diagnosis and clinical rationale\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"5\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatient\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eDiagnosis\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eTechnique\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eRationale\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eOutcome\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eLE\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eRectal web\u0026thinsp;+\u0026thinsp;severe caudal regression \u0026mdash; positioning constraints\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eContinent \u0026mdash; no treatment required\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eLE\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eRectal web\u0026thinsp;+\u0026thinsp;severe caudal regression \u0026mdash; positioning constraints\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNot evaluable \u0026mdash; severe neurological impairment\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003ePSARP\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eComplex anatomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eContinent\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eTA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eAccessible anatomy, primary correction\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eContinent\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003ePSARP\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eComplex anatomy, prior colostomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eRedo surgery (persistent stenosis)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003ePSARP\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eComplex anatomy, prior colostomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eRedo surgery (rectovaginal fistula)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003ePSARP\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eComplex anatomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eContinent with laxatives\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eTA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003ePrimary correction \u0026mdash; adequate decompression\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eConstipation and soiling (non-adherence)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eTA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003ePrimary correction \u0026mdash; adequate decompression\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eConstipation and soiling (non-adherence)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e10\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eTA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003ePrimary correction \u0026mdash; adequate decompression\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eConstipation and soiling (non-adherence)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e11\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eAPT\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eExtensive rectal fibrosis \u0026mdash; complex anatomy (operative rationale not fully retrievable)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eContinent with enemas\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e\n\u003ch3\u003eFunctional outcomes\u003c/h3\u003e\n\u003cp\u003eMedian follow-up was 100.4 months (range 13\u0026ndash;168 months). Nine of 11 patients were eligible for fecal continence assessment. Two patients were excluded: one due to severe neurological impairment precluding reliable functional evaluation, and one due to age below 3 years at the time of analysis.\u003c/p\u003e \u003cp\u003eOf the 9 eligible patients, 6 (66.7%) achieved fecal continence based on Krickenbeck criteria: 2/3 RA patients (66.7%) and 4/6 RS patients (66.7%). Among continent patients, 3 (50%) required no treatment, 2 (33.3%) used laxatives, and 1 (16.7%) required enemas. Three patients (33.3%) experienced constipation and soiling; all three were RS patients who had undergone TA primary correction and shared a common pattern of non-adherence to the prescribed bowel management program. No patient achieved continence without some degree of constipation management in the RS group.\u003c/p\u003e \u003cp\u003eBoth patients managed with the LE technique had favorable postoperative courses with no technique-specific complications. One achieved full continence without treatment; the second was excluded from functional assessment due to the aforementioned neurological condition.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eThis study presents a single-center series of 11 patients with RA and RS with a median follow-up of over 8 years, one of the longest reported in the literature for this rare condition. Our overall continence rate of 66.7% among eligible patients is consistent with published data, which reports continence rates ranging from 50% to 80% depending on the series and follow-up duration [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe ARM-Net Consortium recently published the largest multicenter series of RA and RS to date, including 49 patients with a median follow-up of 43 months [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Their reported continence rate of 69% aligns closely with our findings despite methodological differences, lending external validity to our results. Notably, our follow-up period is more than twice as long, providing insight into the long-term trajectory of these patients beyond what multicenter studies have captured.\u003c/p\u003e \u003cp\u003eA central finding of our study is the notable incidence of constipation, particularly in the RS group, a pattern consistent with the literature [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e, \u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. We identified two potential mechanisms that likely contribute to this observation. First, RS falls within the low ARM spectrum, which carries an inherently higher constipation rate due to preserved but dysfunctional anorectal sensory mechanisms. Second, and critically, the ability of RS patients to pass stool through a stenotic lumen delays diagnosis \u0026mdash; in our series, only 1 of 7 RS patients (14%) received timely diagnosis, versus all 4 RA patients who were identified neonatally. Prolonged diagnostic delay allows progressive rectal distension and megarectum formation, which likely compounds postoperative constipation regardless of technical outcome.\u003c/p\u003e \u003cp\u003eIn our series, sacral ratio did not consistently predict functional outcome, with patients across the full range of sacral morphology achieving continence. Given the small sample size, no conclusions can be drawn regarding the predictive value of sacral ratio in this specific population.\u003c/p\u003e \u003cp\u003eRegarding surgical approach, our experience supports individualized technique selection over a standardized algorithm. The absence of consensus in the literature reflects the anatomical heterogeneity of these conditions rather than a failure of any single technique. The TA approach without prior colostomy was feasible and effective in 3 RS patients with adequate fecal decompression, consistent with recent series reporting successful primary correction in selected cases [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. The APT approach was employed in one patient with extensive rectal fibrosis and complex anatomy; although complete operative documentation was not available for retrospective verification, this case illustrates the need for flexible surgical planning in anatomically challenging presentations.\u003c/p\u003e \u003cp\u003eThe laparoscopic endorectal technique was employed in two patients presenting with rectal web and severe caudal regression precluding standard prone jackknife positioning. This approach allowed direct visualization of the caliber change zone, precise web excision, and valuable intraoperative assessment of anatomical variants, rectal caliber, and the distance between the resection site and the anal canal \u0026mdash; which guided the decision between transanal and laparoscopic rectal wall closure. Both patients had uncomplicated postoperative courses. Previous reports have described laparoscopic approaches in rectal atresia [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. The laparoscopic endorectal technique described in this article represents a novel approach for rectal web correction that, beyond fulfilling the primary surgical objective of preserving sphincter integrity, offers the additional advantage of intraoperative laparoscopic assessment of anatomical variants, rectal caliber, and the distance between the resection site and the anal canal \u0026mdash; information that directly guides the surgical strategy.\u003c/p\u003e \u003cp\u003eThe reintervention rate of 18.2% in our series (2 patients, both following PSARP) is within the range reported in the literature [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Both cases involved technically demanding anatomy, and the complications \u0026mdash; persistent stenosis and rectovaginal fistula \u0026mdash; are recognized challenges of PSARP in this context rather than technique-specific failures.\u003c/p\u003e \u003cp\u003eThis study has several limitations. The retrospective design and small sample size, inherent to the rarity of these conditions, limit statistical analysis and preclude formal comparison between techniques. The exclusion of two patients from functional assessment reduces the analyzable cohort further. Additionally, the non-adherence to bowel management observed in three RS patients may reflect socioeconomic or systemic barriers not captured in our data, and their outcomes should be interpreted in that context rather than as treatment failures.\u003c/p\u003e \u003cp\u003eOne patient was excluded from functional assessment due to age below 3 years at the time of data collection; this patient has since reached evaluable age and prospective follow-up is ongoing.\u003c/p\u003e \u003cp\u003eNotwithstanding these limitations, our series contributes meaningful data on the long-term natural history of RA and RS, underscores the importance of systematic multidisciplinary follow-up, and provides early technical experience with the LE approach for rectal web correction.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eRectal atresia and stenosis remain among the most surgically challenging anorectal malformations, yet individualized management yields satisfactory continence outcomes in most patients. Notably, timely diagnosis was achieved in all RA patients, while only 1 of 7 RS patients (14%) was diagnosed without delay \u0026mdash; a finding that may reflect the partial luminal patency inherent to RS and warrants heightened clinical awareness. Delayed diagnosis may contribute to progressive rectal distension and megarectum formation, potentially compounding postoperative constipation regardless of surgical technique. The high burden of constipation \u0026mdash; particularly in RS \u0026mdash; highlights the need for systematic postoperative follow-up and proactive bowel management. The laparoscopic endorectal technique represents a reproducible, sphincter-preserving option for rectal web correction with favorable early outcomes. Multicenter collaboration remains essential to generate sufficient evidence for guideline development in this rare condition.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e \u003ch2\u003eCompliance with Ethical Standards\u003c/h2\u003e \u003cp\u003e \u003cb\u003eConflict of interest\u003c/b\u003e: The authors declare no conflict of interest.\u003c/p\u003e \u003cp\u003e \u003cstrong\u003eEthical approval:\u003c/strong\u003e \u003cp\u003eThis study was approved by the institutional review board. All procedures were performed in accordance with the ethical standards of the institution and with the 1964 Helsinki Declaration and its later amendments.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eInformed consent:\u003c/strong\u003e \u003cp\u003eInformed consent was obtained from the parents or legal guardians of all patients included in the study.\u003c/p\u003e \u003c/p\u003e\u003ch2\u003eFunding\u003c/h2\u003e \u003cp\u003eNo funding was received for conducting this study.\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eJIS: Conceptualization, Data collection, Formal analysis, Writing \u0026ndash; original draft, Writing \u0026ndash; review and editing. LA: Data collection, Formal analysis, Writing \u0026ndash; review and editing. MVDM: Data collection. JR, MDN, AMR: Writing \u0026ndash; review and editing. VPDB, MMB: Supervision. All authors read and approved the final manuscript.\u003c/p\u003e\u003ch2\u003eData Availability\u003c/h2\u003e\u003cp\u003eThe data that support the findings of this study are not publicly available as they are contained in institutional medical records. Data are available from the corresponding author upon reasonable request and subject to institutional approval.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eHolschneider A, Hutson J, Pe\u0026ntilde;a A, Beket E, Chatterjee S, Coran A, Davies M, Georgeson K, Grosfeld J, Gupta D, Iwai N, Kluth D, Martucciello G, Moore S, Rintala R, Smith ED, Sripathi DV, Stephens D, Sen S, Ure B, Grasshoff S, Boemers T, Murphy F, S\u0026ouml;ylet Y, D\u0026uuml;bbers M, Kunst M (2005) Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. 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Pediatr Surg Int agosto de 33(8):829\u0026ndash;836. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1007/s00383-017-4106-3\u003c/span\u003e\u003cspan address=\"10.1007/s00383-017-4106-3\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDe Beaufort CMC, Gorter RR, Iacobelli BD, Midrio P, Sloots CEJ, Samuk I, Van Rooij IALM, Lisi G, for the ARM-Net Consortium, De Blaauw I, Fascetti-Leon F, V\u0026aacute;zquez AG, Krois W, Lacher M, Leva E, Schmiedeke E, Schukfeh N, Stanton M (2023) Rectal atresia and rectal stenosis: the ARM-Net Consortium experience. 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J Pediatr Surg junio de 32(6):823\u0026ndash;826. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/S0022-3468(97)90628-X\u003c/span\u003e\u003cspan address=\"10.1016/S0022-3468(97)90628-X\" 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":false,"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":"Rectal atresia, Rectal stenosis, Anorectal malformations, Laparoscopic endorectal technique, Fecal continence, Long-term outcomes","lastPublishedDoi":"10.21203/rs.3.rs-9316945/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-9316945/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003ePurpose\u003c/h2\u003e \u003cp\u003eRectal atresia (RA) and rectal stenosis (RS) are rare anorectal malformations (ARM), comprising 1\u0026ndash;2% of all cases. No consensus exists regarding optimal surgical technique. This study evaluated surgical approaches, complications, and long-term functional outcomes at a single referral center.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e \u003cp\u003eRetrospective review of patients with RA or RS treated between 2003 and 2020. Surgical technique, complications, and bowel function were analyzed. Fecal continence was assessed using the Krickenbeck classification in patients older than 3 years with minimum 6-month follow-up after colostomy closure.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e \u003cp\u003eEleven patients were included (7 RS, 4 RA), representing 1.2% of 900 ARM cases. Surgical approaches included posterior sagittal anorectoplasty (PSARP) in 4 (36.4%), transanal in 4 (36.4%), abdominoperineal pull-through in 1 (9.1%), and laparoscopic endorectal technique in 2 (18.2%). Two patients required redo surgery. Of 9 eligible for functional assessment, 6 (66.7%) achieved fecal continence, 3 without additional treatment. Three had constipation and soiling due to poor adherence. Median follow-up was 100.4 months (range 13\u0026ndash;168).\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e \u003cp\u003eDespite surgical complexity, most patients achieved satisfactory outcomes. The high incidence of constipation, particularly in RS, underscores the need for long-term follow-up and early intervention. The laparoscopic endorectal technique offers a reproducible, sphincter-preserving approach to rectal web correction.\u003c/p\u003e","manuscriptTitle":"Challenges and Outcomes in the Management of Rectal Atresia and Stenosis: A Comprehensive Review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2026-04-23 15:10:25","doi":"10.21203/rs.3.rs-9316945/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2026-04-30T08:45:32+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-04-29T18:56:48+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-04-27T16:02:13+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"306630964974924151275171160858552965323","date":"2026-04-18T13:12:57+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"144519683870042678369832919817245567665","date":"2026-04-16T17:56:46+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2026-04-16T05:20:30+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2026-04-07T09:11:35+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2026-04-07T05:00:21+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Surgery International","date":"2026-04-04T02:15:48+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":"f75fc437-148e-4d07-b626-7d3b82c27c82","owner":[],"postedDate":"April 23rd, 2026","published":true,"recentEditorialEvents":[{"type":"decision","content":"Revision requested","date":"2026-04-30T08:45:32+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-04-29T18:56:48+00:00","index":17,"fulltext":""}],"rejectedJournal":[],"revision":"","amendment":"","status":"under-review","subjectAreas":[],"tags":[],"updatedAt":"2026-05-17T10:08:18+00:00","versionOfRecord":[],"versionCreatedAt":"2026-04-23 15:10:25","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-9316945","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-9316945","identity":"rs-9316945","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}