Management of Congenital Pouch Colon: Surgical Outcomes and Long-Term Follow-Up

Management of Congenital Pouch Colon: Surgical Outcomes and Long-Term Follow-Up | 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 Management of Congenital Pouch Colon: Surgical Outcomes and Long-Term Follow-Up Lu Huang, Xing rong Xia, Cheng yan Tang, Zhu Jin, yuan mei liu, and 5 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6160098/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 Objective This study aimed to summarize the clinical features, surgical treatments, and follow-up outcomes of 11 cases of congenital pouch colon (CPC) to provide a reference for the clinical diagnosis and treatment of similar diseases. Methods A retrospective analysis was conducted on the general data, surgical methods, prognosis ,and follow-up of 11 CPC patients admitted to the Pediatric Surgery Department of the Affiliated Hospital of Zunyi Medical University from 2017 to 2023. CPC classification was performed using the Saxena-Mathur system. Results General Information : Of the 11 cases, 7 were male and 4 female, with 1 being type I,3 being type II and 7 being type IV CPC cases. Surgical Procedures : eleven patients underwent laparoscopic spherical colectomy and anoplasty,. Postoperative Pathology : Histopathology revealed proliferation of the interosseous plexus with reduced neuronal numbers and sizes. Prognosis : One patient with type I CPC and one with type II CPC died one month after surgery. The median follow-up duration of patients with CPC was 48 (12–84) months. The Kelly score was poor in 11.1% of the patients, good in 11.0% of patients, and excellent in 55.6%of patients. In terms of Krickenbeck's score, 66.7% of patients had regular bowel movements, 11.1% had frequent fouling, 22.2% had mild fouling, 22.2% had occasional constipation which was treated conservatively, and 77.8% had no constipation. Conclusions Type I-II CPC is associated with a poor prognosis and higher postoperative complications and mortality. Individualized treatment of CPC with complete resection of the dilated colon and laparoscopic-assisted procedures is recommended. congenital pouch colon anorectal malformation clinical features surgical treatments follow-up outcomes Figures Figure 1 Figure 2 Figure 3 1. Introduction According to the Krickenbeck International Classification of Anorectal Malformations, CPC is a rare subtype of anorectal malformation (ARM) characterized by varying degrees of cystic dilatation of the colon [ 1 – 2 ]. The incidence of CPC among ARM cases ranges from 2–37%, with the majority of reports originating from India[ 3 – 4 ],with sporadic cases identified in other countries[ 5 – 6 ]. The etiology of CPC remains unclear, but some studies suggest that factors such as dietary patterns, environmental exposures, and genetic predisposition may play a role[ 7 ]. CPC is commonly classified into five types using the Saxena-Mathur classification system [ 8 ]. At present, surgical intervention is the only effective treatment for CPC. There is no consensus on the treatment plans for different types. Colonoplasty has been recommended for Type I-II CPC (short colon) [ 9 ], while others prefer total resection of the band. Total removal of the cystic band is usually the treatment of choice for Type III-IV CPC [ 10 ].This study summarizes the clinical characteristics, diagnostic approaches, surgical management, and prognosis of 11 patients with CPC treated at the Pediatric Surgery Department of the Affiliated Hospital of Zunyi Medical University between 2017 and 2023. 2. Patients and methods 2.1 General information A total of 11 children with CPC were admitted to the Department of Pediatric Surgery at the Affiliated Hospital of Zunyi Medical University between 2017 and 2023. This study was reviewed and approved by the Ethics Review Committee of the Affiliated Hospital of Zunyi Medical University (KLL-2024-507), and informed consent was obtained from the family members of the children. The diagnostic criteria included abdominal radiographs and imaging studies suggestive of CPC, confirmed by intraoperative exploration of a cystically dilated colon. The Saxena-Mathur classification system was applied to categorize the cases.Type I is the complete absence of the normal colon, with the ileum directly communicating with the pouch colon; type II is the presence of a normal cecum between the ileum and the pouch colon; type III is the presence of a normal ascending and transverse colon connected to the pouch colon; type IV is the normal colon, with a pouch located in the sigmoid region; and type V is the two segments of the pouch colon connected by a short normal colon[ 11 ]. 2.2 Surgical procedures and follow-up The surgical procedures included laparoscopic spherical colectomy combined with anorectoplasty and robot-assisted laparoscopic spherical colectomy with anorectoplasty. Postoperative outcomes were evaluated through regular follow-up visits, which included Questionnaire surveys assessing postoperative complications, defecation frequency, stool control, soiling,autonomous defecation ability, and constipation.Anal fingerprinting measuring muscle tension and anorectal function; and Kelly Scoring [ 12 ] and Krickenbeck Protocols [ 13 ] used to analyze defecation control and continence outcomes. The follow-up process aimed to comprehensively assess anorectal function and quality of life in children post-surgery. 3. Results Among the 11 cases of CPC, 7 patients were male, and 4 were female(Table 1 ). The age at diagnosis ranged from 1 day to 1 year, while surgical resection of the spherical colon was performed between 4 months and 1 year of age. Of these cases, 1 was classified as type I,3 were classified as type II and 7 as type IV, according to the Saxena-Mathur classification(Table 2 ,Fig. 3 ). Table 1 Clinical features of types of CPC patients (n = 11). Serial number No. of cases % Anorectal malformations 11 100 Total no. of high anorectal malformations 6 54.5(of total ARM) Total no. of intermediate anorectal malformations 5 45.5(of total ARM) Total no. of ARM (low, intermediate and high) 0 0 Congenital pouch colon 11 Total no. of male congenital pouch colon 7 63.6(of total CPC) Total no. of female congenital pouch colon 4 36.4(of total CPC) Male: female ratio of congenital pouch colon 7:4 1.74 Clinical presentation Age on presentation (average) 63.45 days - Age on presentation (range) 01-365 days - Weight on admission 2.5 kg or more 11 100 Less than 2.5 kg 0 0 Clinical (preoperative) diagnosis of congenital pouch colon 9 of 11 81.8 Table 2 Surgical procedures performed in 11 cases of CPC patients Serial number No. of cases % Type of congenital pouch colon Incomplete/partial CPC (Type III and IV) 7 (Type III-0, IV-07) 63.6 Complete/total CPC (Type I and II) 4 (Type I-01, II-03) 36.4 Initial operative procedures performed (n = 10) Right transverse colostomy 6 60 Ileostomy 4 40 Definitive operative procedures performed laparoscopic spherical colectomy combined with anorectoplasty 10 90.9 robot-assisted laparoscopic spherical colectomy with anorectoplasty 1 9.1 Staged procedures done Three-staged procedures 10 90.9 Single-staged procedures 1 9.1 Two-staged procedures 0 0 Surgical procedures: Ten patients underwent laparoscopic spherical colectomy with anoplasty, one underwent robot-assisted laparoscopic spherical colectomy with anoplasty, and one patient underwent colostomy only(Table 2 ). Postoperative pathology: Postoperative pathological analysis in 11 cases revealed interosseous plexus hyperplasia, with reduced neuron counts or in size. Immunohistochemical results commonly showed positive staining for Calretinin, CgA, and NeuN, with S100 displaying empty bright areas(Fig. 1 ). Surgical prognosis: One type I CPC patient died one month post-colostomy due to complications from preexisting complex congenital heart disease combined with sepsis. Another type II CPC patient died due to dehydration at home one month postoperatively following repeated episodes of diarrhea and delayed medical attention. The outcomes for type IV CPC were more favorable. Follow-up: Nine patients were followed for 12 to 84 months postoperatively. According to the Kelly score and Krickenbeck protocols, all demonstrated good defecation function without significant developmental delays compared to children of the same age. Imaging studies, including abdominal and urinary ultrasounds and barium enemas conducted six months postoperatively, showed no significant abnormalities. Short-term postoperative follow-up indicated the occurrence of perianal dermatitis in 1 /9 patients and rectal mucosal prolapse in 1/9 cases. One type II CPC patient required further surgery due to a spinal embolism. This patient initially showed poor bowel control and unshaped stools during the first postoperative year but showed improved bowel control over seven years of follow-up, though frequent fecal soiling persisted and achieved a poor Kelly score (1/9). Four other CPC patients followed for 7 years had regular bowel movements and excellent Kelly scores (4/9). Among patients followed for over three years, three showed good outcomes with good Kelly scores (3/9). Two patient experienced mild fecal impaction(2/9), another had anal laxity with occasional incontinence(1/9), and two had regular bowel movements with occasional constipation, which improved with conservative treatment(2/9). Seven patients (7/9) had no constipation. One patient was initially presented with anal stenosis during the first postoperative year, which improved after conservative treatment with anal dilatation with an excellent Kelly score (1/9)(Table 3 ). Table 3 Association of each bowel function scoring protocol. Scoring system Outcome standardization No. of patients (n) Kelly score Good(6 − 5) Fair(4 − 3) Poor(2 − 0)_ 4 3 1 Krickenbeck score VBM yes, soiling grade 0, constipation grade 0, VBM yes, soiling grade 1–2, constipation grade 1–2, VBM no, soiling grade 2–3, constipation grade 2–3, VBM no, soiling grade 3, constipation grade 3. 5 6 2 2 0 0 1 0 Overall, type IV CPC cases demonstrated good long-term outcomes, with regular bowel movements in all patients during follow-up. Occasional complications, such as mild fecal soiling, anal laxity with incontinence, and frequent fecal soiling, were managed effectively with conservative interventions. 4. Discussion CPC is a rare form of high ARM that is frequently misdiagnosed and mistreated due to the lack of knowledge about the disease [ 14 ]. With advancements in diagnostic technologies and increased clinical experience, the diagnosis and management of CPC have significantly improved. Abdominal radiographs (inverted position) are an important diagnostic tool and can often detect CPC during routine evaluations for ARM(Fig. 2 ). CPC typically appears as an isolated, broad, fluid-filled pouch containing air and meconium [ 15 – 16 ]. Without a diagnosis and treatment experience, it is easy to confuse the overlap of colon and stool shadow, and type I-II CPC is difficult to identify on enteroangiography due to the short colon, potentially leading to a missed diagnosis. In this study, all cases of CPC were associated with high ARM and genitourinary fistulae, often accompanied by other structural anomalies. The rate of concomitant malformations in CPC is high, and some anomalies can be fatal[ 17 ]. For instance, one male child with type I CPC was presented with appendiceal agenesis, Meckel's diverticulum, and rectovesical fistula.. He died due to complications from complex congenital heart disease one month after surgery. Other observed anomalies included genitourinary and gastrointestinal defects such as hydronephrosis, vesicoureteral reflux, appendiceal malformations, and spinal embolism. These findings highlight the need for thorough preoperative assessment to identify associated malformations. CPC must be differentiated from Congenital Segmental Dilation of the Colon (CSD) [ 18 ], a condition characterized by well-defined localized intestinal dilatation. Unlike CPC, CSD features visible migratory segments between proximal and distal intestinal tubes, no intestinal obstruction, and no abnormal innervation. Pathological examination in CSD may reveal classifications such as myxomatous hypertrophy, weakened myxomatous layers, or an absent mucosal appearance [ 19 ]. Our center managed a case of CSD in which resection of the dilated colon revealed no significant abnormalities in ganglion cell development. The patient had a good prognosis with no complications. They resumed normal school activities and daily life within two weeks post-surgery, and follow-up at six months showed normal outcomes. Surgical intervention remains the only definitive treatment for CPC [ 20 ], and the choice of surgical approach depends on the patient's specific condition. The most widely recommended strategy is a staged surgical approach, adapted from protocols for congenital high ARM. This typically involves three phases: the first stage involves an ileostomy or colostomy, the second stage includes spherical colectomy anoplasty with spherical colectomy (resection of the entire dilated colon), or bulbous colioplasty.With advances in laparoscopic technology, laparoscopic-assisted anoplasty has become the preferred surgical approach for treating congenital high ARM [ 21 ]. This technique offers unique advantages, including easier separation of fistulae between the rectum and the rectourethra or rectovagina, more precise fistula management, and reduced risk of urethral injury. In patients with high ARM combined with CPC, laparoscopic spherical colectomy is particularly advantageous for achieving clearer dissection and complete resection under direct laparoscopic visualization. At this center, laparoscopic spherical colectomy was performed in all cases. In recent years, the application of the da Vinci robotic surgical system for ARM has also increased [ 22 ], and one case of robotic-assisted surgery for CPC was also performed here.In this study, all CPC patients underwent radical spherical colectomy. For patients with high ARM, the staged approach involved enterostomy in the first stage, thus enterostomy is recommended for patients with type I-II CPC. The shortness of the colon presents difficulties in fistula removal, and after ileostomy, it is convenient to pull out the ileum during the second operation with anastomosis with the anus. Both CPC patients who died, died one month after the second surgery, one case possibly related to concurrent severe cardiac malformations. In another case, the colon was removed completely, resulting in difficulties in stool storage and water absorption by the intestine. This led to diarrhea, electrolyte disorders, severe dehydration, and death in the absence of timely medical treatment. Singh et al. [ 9 ] proposed the use of spherical colonoplasty to avoid the complications of ileal dragging, with the patients having good prognosis and follow-up outcomes. At present, there is insufficient high-quality evidence from large-sample multicenter randomized controlled trials to reach a conclusion regarding the safety and effectiveness of colonoplasty. It remains controversial whether spherical colioplasty can mitigate postoperative intestinal complications in children. In this group of cases, intraoperative assessment of frozen pathological sections indicated the presence of reduced numbers and sizes of ganglion cells in the colon. To avoid the development of constipation caused by poor residual bowel peristalsis, laparoscopic spherical colectomy + anal plasty was performed in the second stage. Bowel function was evaluated postoperatively using clinical history, Kelly scores, and Krickenbeck protocols [ 23 ]. Barium enema imaging was performed six months after surgery to assess the terminal bowel and its anatomical location. Routine abdominal and urinary ultrasounds were conducted to identify postoperative abnormalities, and voiding cystourethrography was performed in cases of urinary dysfunction. As CPC is also complicated with a high likelihood of rectal malformation, postoperative complications are mostly associated with anal plasty surgery. Improvements were seen after short-term follow-up, with the development of rectal mucosal prolapse which was removed 3 months later. One case with a loss of internal control one year after treatment for type II CPC was associated with spinal cord tethering; the condition improved with age. Cases with Type I-II CPC, because of more complex malformations, disease severity, and poor prognosis, should have increased follow-up, together with improved education about the condition, so that in the event of fever or diarrhea, treatment should be sought immediately. However, pelvic computed tomography (CT) or magnetic resonance imaging (MRI), as well as a detailed evaluation of pelvic skeletal and muscular anatomy, were not included in this study's follow-up protocol. The sample size was small, and the study was retrospective. The follow-up time was relatively short and associated with very different outcomes, and functional assessment of postoperative defecation was not uniform. In the next step, more high-quality multicenter prospective randomized controlled studies, as well as long-term follow-up studies, should be performed. It is important to consider the prognosis of CPC and conduct long-term follow-up assessments. 5. Conclusion The prognosis for type I-II CPC remains poor due to complications such as dehydration and severe associated malformations. For children with type IV CPC, individualized treatment strategies should be implemented. Complete resection of the dilated colon during anoplasty is recommended, with laparoscopic-assisted resection of dilated intestinal segments being the preferred surgical approach. It is important to improve the management of rare diseases such as CPC by establishing robust long-term follow-up mechanisms. Declarations Human Ethics and Consent to Participate declarations: This study was conducted in accordance with the Declaration of Helsinki and was approved by the Ethics Review Committee of the Affiliated Hospital of Zunyi Medical University (approval number:KLL-2024-507). Informed consent was obtained from all participants. Funding Declaration: No funding was received for this study. Clinical trial number: not applicable. Data Availability Declaration: The datasets used and analyzed during the current study are available from the corresponding author upon reasonable request. Competing Interest declaration: The authors declare no competing interests. Author Contribution declaration: Huang Lu:Writing,Original Draft,validation.Xia Xing Rong: Conceptualization. Tang ChengYan:Data Curation,Visualization.Jin Zhu:Project administration Funding Acquisition,Writing-Review& Editing.Liu YuanMei:Formal analysis.Zheng ZeBing :Investigation.Du Qin:Methodology.Zhou WanKang: Resources.Zhu DaiWei: Software. Li ZePing: Supervision. References Nah SA, Ong CC, Lakshmi NK, Yap TL, Jacobsen AS, Low Y. Anomalies associated with anorectal malformations according to the Krickenbeck anatomic classification. J Pediatr Surg. 2012;47(12):2273-2278. doi:10.1016/j.jpedsurg.2012.09.017 Chadha R, Bagga D, Mahajan JK, Gupta S. Congenital pouch colon revisited. J Pediatr Surg. 1998;33(10):1510-1515. doi:10.1016/s0022-3468(98)90487-0 Holschneider A, Hutson J, Peña A, Beket E, Chatterjee S, Coran A, et al. Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. 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Robotic-assisted anorectal pull-through for anorectal malformations. J Pediatr Surg. 2011;46(9):1794-1797. doi:10.1016/j.jpedsurg.2011.04.019 Shaari MK, Tan YW, Abdullah MY, Sharudin MF, Osman M, Teoh THQ, et al. Comparing Consistency and Usability of Common Bowel Function Scoring Systems in Anorectal Malformation Patients. J Pediatr Surg. 2024;59(4):571-576. doi:10.1016/j.jpedsurg.2023.12.002 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. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-6160098","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":434189769,"identity":"eaa32b9a-3b85-460f-aa47-5974233344fc","order_by":0,"name":"Lu Huang","email":"","orcid":"","institution":"Affiliated Hospital of Zunyi Medical University","correspondingAuthor":false,"prefix":"","firstName":"Lu","middleName":"","lastName":"Huang","suffix":""},{"id":434189770,"identity":"9ece7479-9fc7-4585-87ab-871aaf630c1d","order_by":1,"name":"Xing rong Xia","email":"","orcid":"","institution":"Affiliated Hospital of Zunyi Medical University","correspondingAuthor":false,"prefix":"","firstName":"Xing","middleName":"rong","lastName":"Xia","suffix":""},{"id":434189771,"identity":"00cd13b5-c2a5-48aa-88b8-1158ef5bfada","order_by":2,"name":"Cheng yan Tang","email":"","orcid":"","institution":"Affiliated Hospital of Zunyi Medical University","correspondingAuthor":false,"prefix":"","firstName":"Cheng","middleName":"yan","lastName":"Tang","suffix":""},{"id":434189772,"identity":"03d8b450-c244-4f3b-a44b-1b433ce8a45d","order_by":3,"name":"Zhu Jin","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA9klEQVRIiWNgGAWjYBAC9mYGBmYGBgkeNvbmgw8+QAQN8GphhGixkOPnOZZsOIMoLQ1gLRXGkjN8zKR5iNLSznv4c2GbROKGGwwG0jZ/tiU2sDdvk2CouYPHYXwJxjNBWm43JBjn8NxObOA5VibBcOwZHi08Bsm8IC13DhxIzpEAapHIMZNgbDiMV8thsJYbiQ2HLQyAWuTf4Nci2Mxj2AzUAvR+MjD0EkC28ODXIs3MY8zMc04CFMjMjD0Hbhu38aQVWyQcw62Fj/+M8WeesjpgVPZ///Hjz23ZfvbDG298qMGtBROwgYgEEjSMglEwCkbBKMAEAAOfUc7EUYjoAAAAAElFTkSuQmCC","orcid":"","institution":"Affiliated Hospital of Zunyi Medical University","correspondingAuthor":true,"prefix":"","firstName":"Zhu","middleName":"","lastName":"Jin","suffix":""},{"id":434189773,"identity":"6700534a-3e0e-4c35-9c3b-41058b9f7df8","order_by":4,"name":"yuan mei liu","email":"","orcid":"","institution":"Affiliated Hospital of Zunyi Medical College: Affiliated Hospital of Zunyi Medical University","correspondingAuthor":false,"prefix":"","firstName":"yuan","middleName":"mei","lastName":"liu","suffix":""},{"id":434189774,"identity":"6273b532-e910-4fb4-a353-bb7beb1ec44c","order_by":5,"name":"Ze bing zheng","email":"","orcid":"","institution":"Affiliated Hospital of Zunyi Medical University","correspondingAuthor":false,"prefix":"","firstName":"Ze","middleName":"bing","lastName":"zheng","suffix":""},{"id":434189775,"identity":"3c7cca8d-f113-4c75-8cb8-af5afef08319","order_by":6,"name":"qing du","email":"","orcid":"","institution":"Affiliated Hospital of Zunyi Medical University","correspondingAuthor":false,"prefix":"","firstName":"qing","middleName":"","lastName":"du","suffix":""},{"id":434189776,"identity":"23887fee-dc4b-4722-8e6f-4fe6cfd02001","order_by":7,"name":"wan kang zhou","email":"","orcid":"","institution":"zun yi yi xue yuan fu shu yi yuan: Affiliated Hospital of Zunyi Medical College","correspondingAuthor":false,"prefix":"","firstName":"wan","middleName":"kang","lastName":"zhou","suffix":""},{"id":434189777,"identity":"c5e93ee9-5dc6-4255-931a-514520bec7e4","order_by":8,"name":"dai wei Zhu","email":"","orcid":"","institution":"Affiliated Hospital of Zunyi Medical University","correspondingAuthor":false,"prefix":"","firstName":"dai","middleName":"wei","lastName":"Zhu","suffix":""},{"id":434189778,"identity":"ff8e1385-cc6c-414b-b096-b14b2a6eef06","order_by":9,"name":"ze ping li","email":"","orcid":"","institution":"Affiliated Hospital of Zunyi Medical University","correspondingAuthor":false,"prefix":"","firstName":"ze","middleName":"ping","lastName":"li","suffix":""}],"badges":[],"createdAt":"2025-03-05 07:47:55","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6160098/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6160098/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":80581981,"identity":"37d055b7-1c85-4c38-8e2d-ced78a2d96b3","added_by":"auto","created_at":"2025-04-14 23:25:01","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":103158,"visible":true,"origin":"","legend":"\u003cp\u003eHE and IHC staining for neuron-specific enolase showing diffuse cytoplasmic positivity in ganglionic cells in a CPC specimen. (A. The arrow indicates the hyperplasia of the myenteric plexus and a decrease in the number of neurons, HE×40; B. The arrow indicates the ganglion cells with reduced volume under high magnification, HE×100; C. The arrow indicates the NeuN(+)-stained ganglion cells, ×40; D. The arrow indicates the clear areas in s100 of ganglion cells, HE×100).\u003c/p\u003e","description":"","filename":"1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6160098/v1/9472cbece3ecf1d8cdb49f2e.jpg"},{"id":80581979,"identity":"530d0a75-414f-4450-b1da-9814e285801e","added_by":"auto","created_at":"2025-04-14 23:25:01","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":37243,"visible":true,"origin":"","legend":"\u003cp\u003eRadiological film demonstrating type 2 CPC（A.Abdominal inversions of type IICPC,B:rectovesical fistula,C:Iodinol contrast of the proximal part of the fistula.）\u003c/p\u003e","description":"","filename":"2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6160098/v1/b41a3c9c3d32332c361b6e7c.jpg"},{"id":80582532,"identity":"4492368b-235d-4e12-ab67-e910fafa6bd7","added_by":"auto","created_at":"2025-04-14 23:33:01","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":48674,"visible":true,"origin":"","legend":"\u003cp\u003eIntraoperative photograph of CPC.(A:type 1 CPC,B+C:type 2 CPC,D+E:type 4 CPC.)\u003c/p\u003e","description":"","filename":"3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6160098/v1/6f86b2ae0d1ea96c1712666b.jpg"},{"id":91789843,"identity":"4dc9abb7-80af-46f6-9aa6-9fa6982bacf6","added_by":"auto","created_at":"2025-09-21 11:04:05","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":760923,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6160098/v1/97af70c1-89ff-4d17-abe6-b2729b3b286c.pdf"}],"financialInterests":"","formattedTitle":"Management of Congenital Pouch Colon: Surgical Outcomes and Long-Term Follow-Up","fulltext":[{"header":"1. Introduction","content":"\u003cp\u003eAccording to the Krickenbeck International Classification of Anorectal Malformations, CPC is a rare subtype of anorectal malformation (ARM) characterized by varying degrees of cystic dilatation of the colon [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. The incidence of CPC among ARM cases ranges from 2\u0026ndash;37%, with the majority of reports originating from India[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e],with sporadic cases identified in other countries[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. The etiology of CPC remains unclear, but some studies suggest that factors such as dietary patterns, environmental exposures, and genetic predisposition may play a role[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. CPC is commonly classified into five types using the Saxena-Mathur classification system [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. At present, surgical intervention is the only effective treatment for CPC. There is no consensus on the treatment plans for different types. Colonoplasty has been recommended for Type I-II CPC (short colon) [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e], while others prefer total resection of the band. Total removal of the cystic band is usually the treatment of choice for Type III-IV CPC [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e].This study summarizes the clinical characteristics, diagnostic approaches, surgical management, and prognosis of 11 patients with CPC treated at the Pediatric Surgery Department of the Affiliated Hospital of Zunyi Medical University between 2017 and 2023.\u003c/p\u003e"},{"header":"2. Patients and methods","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003e2.1 General information\u003c/h2\u003e \u003cp\u003eA total of 11 children with CPC were admitted to the Department of Pediatric Surgery at the Affiliated Hospital of Zunyi Medical University between 2017 and 2023. This study was reviewed and approved by the Ethics Review Committee of the Affiliated Hospital of Zunyi Medical University (KLL-2024-507), and informed consent was obtained from the family members of the children. The diagnostic criteria included abdominal radiographs and imaging studies suggestive of CPC, confirmed by intraoperative exploration of a cystically dilated colon. The Saxena-Mathur classification system was applied to categorize the cases.Type I is the complete absence of the normal colon, with the ileum directly communicating with the pouch colon; type II is the presence of a normal cecum between the ileum and the pouch colon; type III is the presence of a normal ascending and transverse colon connected to the pouch colon; type IV is the normal colon, with a pouch located in the sigmoid region; and type V is the two segments of the pouch colon connected by a short normal colon[\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e].\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec4\" class=\"Section2\"\u003e \u003ch2\u003e2.2 Surgical procedures and follow-up\u003c/h2\u003e \u003cp\u003eThe surgical procedures included laparoscopic spherical colectomy combined with anorectoplasty and robot-assisted laparoscopic spherical colectomy with anorectoplasty. Postoperative outcomes were evaluated through regular follow-up visits, which included Questionnaire surveys assessing postoperative complications, defecation frequency, stool control, soiling,autonomous defecation ability, and constipation.Anal fingerprinting measuring muscle tension and anorectal function; and Kelly Scoring [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e] and Krickenbeck Protocols [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e] used to analyze defecation control and continence outcomes. The follow-up process aimed to comprehensively assess anorectal function and quality of life in children post-surgery.\u003c/p\u003e \u003c/div\u003e"},{"header":"3. Results","content":"\u003cp\u003eAmong the 11 cases of CPC, 7 patients were male, and 4 were female(Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). The age at diagnosis ranged from 1 day to 1 year, while surgical resection of the spherical colon was performed between 4 months and 1 year of age. Of these cases, 1 was classified as type I,3 were classified as type II and 7 as type IV, according to the Saxena-Mathur classification(Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e,Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e3\u003c/span\u003e).\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\u003eClinical features of types of CPC patients (n\u0026thinsp;=\u0026thinsp;11).\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"3\"\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 \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSerial number\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNo. of cases\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003e%\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAnorectal malformations\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e100\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal no. of high anorectal malformations\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e54.5(of total ARM)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal no. of intermediate anorectal malformations\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e45.5(of total ARM)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal no. of ARM (low, intermediate and high)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCongenital pouch colon\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal no. of male congenital pouch colon\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e63.6(of total CPC)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal no. of female congenital pouch colon\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e36.4(of total CPC)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMale: female ratio of congenital pouch colon\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e7:4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1.74\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eClinical presentation\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAge on presentation (average)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e63.45 days\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAge on presentation (range)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e01-365 days\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eWeight on admission\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2.5 kg or more\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e100\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLess than 2.5 kg\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eClinical (preoperative) diagnosis of congenital pouch colon\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9 of 11\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e81.8\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eSurgical procedures performed in 11 cases of CPC patients\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"3\"\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 \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSerial number\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNo. of cases\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003e%\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eType of congenital pouch colon\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eIncomplete/partial CPC (Type III and IV)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e7 (Type III-0, IV-07)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e63.6\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eComplete/total CPC (Type I and II)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (Type I-01, II-03)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e36.4\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eInitial operative procedures performed (n\u0026thinsp;=\u0026thinsp;10)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eRight transverse colostomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e60\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eIleostomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e40\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDefinitive operative procedures performed\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003elaparoscopic spherical colectomy combined with anorectoplasty\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e90.9\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003erobot-assisted laparoscopic spherical colectomy with anorectoplasty\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e9.1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eStaged procedures done\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eThree-staged procedures\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e90.9\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSingle-staged procedures\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e9.1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTwo-staged procedures\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eSurgical procedures:\u003c/p\u003e \u003cp\u003eTen patients underwent laparoscopic spherical colectomy with anoplasty, one underwent robot-assisted laparoscopic spherical colectomy with anoplasty, and one patient underwent colostomy only(Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003ePostoperative pathology:\u003c/p\u003e \u003cp\u003ePostoperative pathological analysis in 11 cases revealed interosseous plexus hyperplasia, with reduced neuron counts or in size. Immunohistochemical results commonly showed positive staining for Calretinin, CgA, and NeuN, with S100 displaying empty bright areas(Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eSurgical prognosis:\u003c/p\u003e \u003cp\u003eOne type I CPC patient died one month post-colostomy due to complications from preexisting complex congenital heart disease combined with sepsis. Another type II CPC patient died due to dehydration at home one month postoperatively following repeated episodes of diarrhea and delayed medical attention. The outcomes for type IV CPC were more favorable.\u003c/p\u003e \u003cp\u003eFollow-up:\u003c/p\u003e \u003cp\u003eNine patients were followed for 12 to 84 months postoperatively. According to the Kelly score and Krickenbeck protocols, all demonstrated good defecation function without significant developmental delays compared to children of the same age. Imaging studies, including abdominal and urinary ultrasounds and barium enemas conducted six months postoperatively, showed no significant abnormalities. Short-term postoperative follow-up indicated the occurrence of perianal dermatitis in 1 /9 patients and rectal mucosal prolapse in 1/9 cases. One type II CPC patient required further surgery due to a spinal embolism. This patient initially showed poor bowel control and unshaped stools during the first postoperative year but showed improved bowel control over seven years of follow-up, though frequent fecal soiling persisted and achieved a poor Kelly score (1/9). Four other CPC patients followed for 7 years had regular bowel movements and excellent Kelly scores (4/9). Among patients followed for over three years, three showed good outcomes with good Kelly scores (3/9). Two patient experienced mild fecal impaction(2/9), another had anal laxity with occasional incontinence(1/9), and two had regular bowel movements with occasional constipation, which improved with conservative treatment(2/9). Seven patients (7/9) had no constipation. One patient was initially presented with anal stenosis during the first postoperative year, which improved after conservative treatment with anal dilatation with an excellent Kelly score (1/9)(Table\u0026nbsp;\u003cspan refid=\"Tab3\" class=\"InternalRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab3\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 3\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eAssociation of each bowel function scoring protocol.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"3\"\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=\"char\" char=\".\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eScoring system\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eOutcome standardization\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eNo. of patients (n)\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eKelly score\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eGood(6\u0026thinsp;\u0026minus;\u0026thinsp;5)\u003c/p\u003e \u003cp\u003eFair(4\u0026thinsp;\u0026minus;\u0026thinsp;3)\u003c/p\u003e \u003cp\u003ePoor(2\u0026thinsp;\u0026minus;\u0026thinsp;0)_\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e \u003cp\u003e4\u003c/p\u003e \u003cp\u003e3\u003c/p\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eKrickenbeck score\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eVBM yes, soiling grade 0,\u003c/p\u003e \u003cp\u003econstipation grade 0,\u003c/p\u003e \u003cp\u003eVBM yes, soiling grade 1\u0026ndash;2,\u003c/p\u003e \u003cp\u003econstipation grade 1\u0026ndash;2,\u003c/p\u003e \u003cp\u003eVBM no, soiling grade 2\u0026ndash;3,\u003c/p\u003e \u003cp\u003econstipation grade 2\u0026ndash;3,\u003c/p\u003e \u003cp\u003eVBM no, soiling grade 3,\u003c/p\u003e \u003cp\u003econstipation grade 3.\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e \u003cp\u003e5\u003c/p\u003e \u003cp\u003e6\u003c/p\u003e \u003cp\u003e2\u003c/p\u003e \u003cp\u003e2\u003c/p\u003e \u003cp\u003e0\u003c/p\u003e \u003cp\u003e0\u003c/p\u003e \u003cp\u003e1\u003c/p\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eOverall, type IV CPC cases demonstrated good long-term outcomes, with regular bowel movements in all patients during follow-up. Occasional complications, such as mild fecal soiling, anal laxity with incontinence, and frequent fecal soiling, were managed effectively with conservative interventions.\u003c/p\u003e"},{"header":"4. Discussion","content":"\u003cp\u003eCPC is a rare form of high ARM that is frequently misdiagnosed and mistreated due to the lack of knowledge about the disease [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. With advancements in diagnostic technologies and increased clinical experience, the diagnosis and management of CPC have significantly improved. Abdominal radiographs (inverted position) are an important diagnostic tool and can often detect CPC during routine evaluations for ARM(Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e2\u003c/span\u003e). CPC typically appears as an isolated, broad, fluid-filled pouch containing air and meconium [\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. Without a diagnosis and treatment experience, it is easy to confuse the overlap of colon and stool shadow, and type I-II CPC is difficult to identify on enteroangiography due to the short colon, potentially leading to a missed diagnosis. In this study, all cases of CPC were associated with high ARM and genitourinary fistulae, often accompanied by other structural anomalies. The rate of concomitant malformations in CPC is high, and some anomalies can be fatal[\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. For instance, one male child with type I CPC was presented with appendiceal agenesis, Meckel's diverticulum, and rectovesical fistula.. He died due to complications from complex congenital heart disease one month after surgery. Other observed anomalies included genitourinary and gastrointestinal defects such as hydronephrosis, vesicoureteral reflux, appendiceal malformations, and spinal embolism. These findings highlight the need for thorough preoperative assessment to identify associated malformations.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eCPC must be differentiated from Congenital Segmental Dilation of the Colon (CSD) [\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e], a condition characterized by well-defined localized intestinal dilatation. Unlike CPC, CSD features visible migratory segments between proximal and distal intestinal tubes, no intestinal obstruction, and no abnormal innervation. Pathological examination in CSD may reveal classifications such as myxomatous hypertrophy, weakened myxomatous layers, or an absent mucosal appearance [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]. Our center managed a case of CSD in which resection of the dilated colon revealed no significant abnormalities in ganglion cell development. The patient had a good prognosis with no complications. They resumed normal school activities and daily life within two weeks post-surgery, and follow-up at six months showed normal outcomes.\u003c/p\u003e \u003cp\u003eSurgical intervention remains the only definitive treatment for CPC [\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e], and the choice of surgical approach depends on the patient's specific condition. The most widely recommended strategy is a staged surgical approach, adapted from protocols for congenital high ARM. This typically involves three phases: the first stage involves an ileostomy or colostomy, the second stage includes spherical colectomy anoplasty with spherical colectomy (resection of the entire dilated colon), or bulbous colioplasty.With advances in laparoscopic technology, laparoscopic-assisted anoplasty has become the preferred surgical approach for treating congenital high ARM [\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e]. This technique offers unique advantages, including easier separation of fistulae between the rectum and the rectourethra or rectovagina, more precise fistula management, and reduced risk of urethral injury. In patients with high ARM combined with CPC, laparoscopic spherical colectomy is particularly advantageous for achieving clearer dissection and complete resection under direct laparoscopic visualization. At this center, laparoscopic spherical colectomy was performed in all cases. In recent years, the application of the da Vinci robotic surgical system for ARM has also increased [\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e], and one case of robotic-assisted surgery for CPC was also performed here.In this study, all CPC patients underwent radical spherical colectomy. For patients with high ARM, the staged approach involved enterostomy in the first stage, thus enterostomy is recommended for patients with type I-II CPC. The shortness of the colon presents difficulties in fistula removal, and after ileostomy, it is convenient to pull out the ileum during the second operation with anastomosis with the anus. Both CPC patients who died, died one month after the second surgery, one case possibly related to concurrent severe cardiac malformations. In another case, the colon was removed completely, resulting in difficulties in stool storage and water absorption by the intestine. This led to diarrhea, electrolyte disorders, severe dehydration, and death in the absence of timely medical treatment. Singh et al. [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e] proposed the use of spherical colonoplasty to avoid the complications of ileal dragging, with the patients having good prognosis and follow-up outcomes. At present, there is insufficient high-quality evidence from large-sample multicenter randomized controlled trials to reach a conclusion regarding the safety and effectiveness of colonoplasty. It remains controversial whether spherical colioplasty can mitigate postoperative intestinal complications in children. In this group of cases, intraoperative assessment of frozen pathological sections indicated the presence of reduced numbers and sizes of ganglion cells in the colon. To avoid the development of constipation caused by poor residual bowel peristalsis, laparoscopic spherical colectomy\u0026thinsp;+\u0026thinsp;anal plasty was performed in the second stage.\u003c/p\u003e \u003cp\u003eBowel function was evaluated postoperatively using clinical history, Kelly scores, and Krickenbeck protocols [\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e]. Barium enema imaging was performed six months after surgery to assess the terminal bowel and its anatomical location. Routine abdominal and urinary ultrasounds were conducted to identify postoperative abnormalities, and voiding cystourethrography was performed in cases of urinary dysfunction. As CPC is also complicated with a high likelihood of rectal malformation, postoperative complications are mostly associated with anal plasty surgery. Improvements were seen after short-term follow-up, with the development of rectal mucosal prolapse which was removed 3 months later. One case with a loss of internal control one year after treatment for type II CPC was associated with spinal cord tethering; the condition improved with age. Cases with Type I-II CPC, because of more complex malformations, disease severity, and poor prognosis, should have increased follow-up, together with improved education about the condition, so that in the event of fever or diarrhea, treatment should be sought immediately.\u003c/p\u003e \u003cp\u003eHowever, pelvic computed tomography (CT) or magnetic resonance imaging (MRI), as well as a detailed evaluation of pelvic skeletal and muscular anatomy, were not included in this study's follow-up protocol. The sample size was small, and the study was retrospective. The follow-up time was relatively short and associated with very different outcomes, and functional assessment of postoperative defecation was not uniform. In the next step, more high-quality multicenter prospective randomized controlled studies, as well as long-term follow-up studies, should be performed. It is important to consider the prognosis of CPC and conduct long-term follow-up assessments.\u003c/p\u003e"},{"header":"5. Conclusion","content":"\u003cp\u003eThe prognosis for type I-II CPC remains poor due to complications such as dehydration and severe associated malformations. For children with type IV CPC, individualized treatment strategies should be implemented. Complete resection of the dilated colon during anoplasty is recommended, with laparoscopic-assisted resection of dilated intestinal segments being the preferred surgical approach. It is important to improve the management of rare diseases such as CPC by establishing robust long-term follow-up mechanisms.\u003c/p\u003e "},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eHuman Ethics and Consent to Participate declarations:\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was conducted in accordance with the Declaration of Helsinki and was approved by the Ethics Review Committee of the Affiliated Hospital of Zunyi Medical University (approval number:KLL-2024-507). Informed consent was obtained from all participants.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding Declaration:\u0026nbsp;\u003c/strong\u003eNo funding was received for this study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eClinical trial number:\u0026nbsp;\u003c/strong\u003enot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData Availability Declaration:\u0026nbsp;\u003c/strong\u003eThe datasets used and analyzed during the current study are available from the corresponding author upon reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting Interest declaration:\u0026nbsp;\u003c/strong\u003eThe authors declare no competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor Contribution declaration: \u0026nbsp; \u0026nbsp; \u0026nbsp;\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eHuang Lu:Writing,Original Draft,validation.Xia Xing Rong: Conceptualization. Tang ChengYan:Data Curation,Visualization.Jin Zhu:Project administration Funding Acquisition,Writing-Review\u0026amp; Editing.Liu YuanMei:Formal analysis.Zheng ZeBing :Investigation.Du Qin:Methodology.Zhou WanKang: Resources.Zhu DaiWei: Software. Li ZePing: Supervision.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eNah SA, Ong CC, Lakshmi NK, Yap TL, Jacobsen AS, Low Y. Anomalies associated with anorectal malformations according to the Krickenbeck anatomic classification. J Pediatr Surg. 2012;47(12):2273-2278. doi:10.1016/j.jpedsurg.2012.09.017\u003c/li\u003e\n\u003cli\u003eChadha R, Bagga D, Mahajan JK, Gupta S. Congenital pouch colon revisited. J Pediatr Surg. 1998;33(10):1510-1515. doi:10.1016/s0022-3468(98)90487-0\u003c/li\u003e\n\u003cli\u003eHolschneider A, Hutson J, Pe\u0026ntilde;a A, Beket E, Chatterjee S, Coran A, et al. Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Pediatr Surg. 2005;40(10):1521-1526. doi:10.1016/j.jpedsurg.2005.08.002\u003c/li\u003e\n\u003cli\u003ede Blaauw I, Stenstr\u0026ouml;m P, Yamataka A, Miyake Y, Reutter H, Midrio P, et al. Anorectal malformations. Nat Rev Dis Primers. 2024;10(1):88. doi:10.1038/s41572-024-00574-2\u003c/li\u003e\n\u003cli\u003eGupta S, Gupta N, Tiwari P, Menon S, Mathur P, Kothari SL, et al. Lnc-EPB41-Protein Interactions Associated with Congenital Pouch Colon. Biomolecules. 2018;8(3):95. doi:10.3390/biom8030095\u003c/li\u003e\n\u003cli\u003eGupta S, Mathur P, Mishra AK, Medicherla KM, Bandapalli OR, Suravajhala P. Whole Exome-Trio Analysis Reveals Rare Variants Associated with Congenital Pouch Colon. Children (Basel). 2023;10(5):902. doi:10.3390/children10050902\u003c/li\u003e\n\u003cli\u003eMathur P, Nunia V, Sharma R, Simlot A, Medicherla KM. Congenital Pouch Colon: Role of Genetics or Environmental Influence? Pathobiology. 2018;85(5 - 6):332-341. doi:10.1159/000492432\u003c/li\u003e\n\u003cli\u003ePuri A, Chadha R, Choudhury SR, et al. Congenital pouch colon: follow-up and functional results after definitive surgery. J Pediatr Surg. 2006 Aug;41(8):1413-1419. doi:10.1016/j.jpedsurg.2006.04.017\u003c/li\u003e\n\u003cli\u003eWakhlu A, Wakhlu AK. Technique and long-term results of coloplasty for congenital short colon. Pediatr Surg Int. 2009;25(1):47-52. doi:10.1007/s00383-008-2281-y\u003c/li\u003e\n\u003cli\u003eSingh S, Rawat JD, Kumar P. Congenital pouch colon: Our experience with coloplasty. Afr J Paediatr Surg. 2018;15(1):16-21. doi:10.4103/ajps\u003c/li\u003e\n\u003cli\u003eMathur P, Saxena AK, Simlot A. Management of congenital pouch colon based on the Saxena-Mathur classification. J Pediatr Surg. 2009;44(5):962-966. doi:10.1016/j.jpedsurg.2009.01.035\u003c/li\u003e\n\u003cli\u003eKelly JH. The clinical and radiological assessment of anal continence in childhood. Aust NZ J Surg. 1972;42(1):62-63\u003c/li\u003e\n\u003cli\u003eDemirogullari B, Ozen IO, Karabulut R, Turkyilmaz Z, Sonmez K, Kale N, et al. Colonic motility and functional assessment of the patients with anorectal malformations according to Krickenbeck consensus. J Pediatr Surg. 2008;43(10):1839-1843. doi:10.1016/j.jpedsurg.2008.01.055\u003c/li\u003e\n\u003cli\u003eGangopadhyay AN, Pandey A, Rastogi N, Mandal MB, Gopal SC, Gupta DK, et al. A study of the functional aberration of the pouch in anorectal malformation associated with congenital pouch colon. Colorectal Dis. 2010;12(3):226-231. doi:10.1111/j.1463-1318.2009.01780.x\u003c/li\u003e\n\u003cli\u003eMathur P, Saxena AK, Bajaj M, Chandra T, Sharma NC, Simlot A, et al. Role of plain abdominal radiographs in predicting type of congenital pouch colon. Pediatr Radiol. 2010;40(10):1603-1608. doi:10.1007/s00247-010-1786-4\u003c/li\u003e\n\u003cli\u003eUdawat H, Nunia V, Mathur P, Udawat HP, Gaur KL, Saxena AK, et al. Histopathological and Immunohistochemical Findings in Congenital Pouch Colon: A Prospective Study. Pathobiology. 2017;84(4):202-209. doi:10.1159/000460821\u003c/li\u003e\n\u003cli\u003eMathur P, Medicherla KM, Chaudhary S, Patel M, Bagali P, Suravajhala P. Whole exome sequencing reveals rare variants linked to congenital pouch colon. Sci Rep. 2018;8(1):6646. doi:10.1038/s41598-018-24967-y. Erratum in: Sci Rep. 2018;8(1):13867. doi:10.1038/s41598-018-32119-5\u003c/li\u003e\n\u003cli\u003eHan YJ, Huang CJ, Qin Q, Lv CJ, Gu SS, Zhao XX, et al. Clinical features of congenital pouch colon and its diagnosis and treatment experience. J Third Mil Med Univ. 2018;40(23):2131-2135. DOI:10.16016/j.1000-5404.201806077\u003c/li\u003e\n\u003cli\u003eZeng FTA, Makaba SM, Hager J, Sergi CM. Congenital segmental dilatation of the intestine: an in-depth review. J Matern Fetal Neonatal Med. 2023;36(2):2259047. doi:10.1080/14767058.2023.2259047\u003c/li\u003e\n\u003cli\u003eSharma S, Gupta DK. Management options of congenital pouch colon--a rare variant of anorectal malformation. Pediatr Surg Int. 2015;31(8):753-758. doi:10.1007/s00383-015-3739-3\u003c/li\u003e\n\u003cli\u003eMitani Y, Kubota A, Goda T, Takifuji K, Iwamoto R, Kawai M. Laparoscopic-Assisted Total Resection and Endorectal Pull-Through Technique for Congenital Megarectum with Anorectal Malformation. J Pediatr Surg. 2023;58(7):1269-1273. doi:10.1016/j.jpedsurg.2023.01.061\u003c/li\u003e\n\u003cli\u003eAlbassam A, Gado A, Mallick MS, Alnaami M, Al-Shenawy W. Robotic-assisted anorectal pull-through for anorectal malformations. J Pediatr Surg. 2011;46(9):1794-1797. doi:10.1016/j.jpedsurg.2011.04.019\u003c/li\u003e\n\u003cli\u003eShaari MK, Tan YW, Abdullah MY, Sharudin MF, Osman M, Teoh THQ, et al. Comparing Consistency and Usability of Common Bowel Function Scoring Systems in Anorectal Malformation Patients. J Pediatr Surg. 2024;59(4):571-576. doi:10.1016/j.jpedsurg.2023.12.002 \u003cstrong\u003e\u003c/strong\u003e\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":"congenital pouch colon, anorectal malformation, clinical features, surgical treatments, follow-up outcomes","lastPublishedDoi":"10.21203/rs.3.rs-6160098/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6160098/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eObjective\u003c/h2\u003e \u003cp\u003eThis study aimed to summarize the clinical features, surgical treatments, and follow-up outcomes of 11 cases of congenital pouch colon (CPC) to provide a reference for the clinical diagnosis and treatment of similar diseases.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e \u003cp\u003eA retrospective analysis was conducted on the general data, surgical methods, prognosis ,and follow-up of 11 CPC patients admitted to the Pediatric Surgery Department of the Affiliated Hospital of Zunyi Medical University from 2017 to 2023. CPC classification was performed using the Saxena-Mathur system.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e \u003cp\u003e \u003cb\u003eGeneral Information\u003c/b\u003e: Of the 11 cases, 7 were male and 4 female, with 1 being type I,3 being type II and 7 being type IV CPC cases. \u003cb\u003eSurgical Procedures\u003c/b\u003e: eleven patients underwent laparoscopic spherical colectomy and anoplasty,. \u003cb\u003ePostoperative Pathology\u003c/b\u003e: Histopathology revealed proliferation of the interosseous plexus with reduced neuronal numbers and sizes. \u003cb\u003ePrognosis\u003c/b\u003e: One patient with type I CPC and one with type II CPC died one month after surgery. The median follow-up duration of patients with CPC was 48 (12\u0026ndash;84) months. The Kelly score was poor in 11.1% of the patients, good in 11.0% of patients, and excellent in 55.6%of patients. In terms of Krickenbeck's score, 66.7% of patients had regular bowel movements, 11.1% had frequent fouling, 22.2% had mild fouling, 22.2% had occasional constipation which was treated conservatively, and 77.8% had no constipation.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eType I-II CPC is associated with a poor prognosis and higher postoperative complications and mortality. Individualized treatment of CPC with complete resection of the dilated colon and laparoscopic-assisted procedures is recommended.\u003c/p\u003e","manuscriptTitle":"Management of Congenital Pouch Colon: Surgical Outcomes and Long-Term Follow-Up","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-04-14 23:24:56","doi":"10.21203/rs.3.rs-6160098/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":"50464ad6-46b8-45a9-a671-36740e8d7a80","owner":[],"postedDate":"April 14th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-09-21T10:55:58+00:00","versionOfRecord":[],"versionCreatedAt":"2025-04-14 23:24:56","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-6160098","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6160098","identity":"rs-6160098","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}