Single-stage Laparoscopic Proctocolectomy with Ileal pouch-anal Anastomosis for Familial Adenomatous Polyposis

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Kim, Kara Kennedy, William R. Johnston, and 2 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7677571/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 15 Nov, 2025 Read the published version in Pediatric Surgery International → Version 1 posted 7 You are reading this latest preprint version Abstract Purpose: Assess the safety of single-stage laparoscopic proctocolectomy with ileal pouch-anal anastomosis (LPC-IPAA) in children with familial adenomatous polyposis (FAP). Methods: Medical records for all patients with FAP who underwent LPC-IPAA between 1/1/2013 and 8/1/2024 at a pediatric center were reviewed. Baseline characteristics, postoperative complications, and functional outcomes were assessed. Results: Thirty-nine patients met inclusion criteria. Mean age was 14.9 years (range 9.4-21.5) and mean BMI was 23.0 (range 15.3-47.0). Follow-up averaged 2.8 years (SD 2.9). Mean operative time was 228.4 minutes (SD 44.5) in the stapled group (n = 32) and 316.1 minutes (SD 43.1) in the hand-sewn group (n = 7) (p < 0.001). There were no anastomotic leaks. One patient developed a postoperative abscess. Six (15%) developed presumed pouchitis, 4 of whom were in the hand-sewn group and all successfully treated with antibiotics. Six patients (15%) developed an anastomotic stricture – all in the stapled group – and responded well to anal dilation. No patient required treatment for fecal incontinence. Conclusion: LPC-IPAA in adolescents and young adults with FAP can safely be performed in a single operation and is associated with favorable functional outcomes. Familial Adenomatous Polyposis Ileal Pouch-anal Anastomosis Pediatrics Total Proctocolectomy INTRODUCTION Familial adenomatous polyposis (FAP) is characterized by the formation of intestinal – primarily colonic – adenomas, usually beginning within the first three decades of life [1]. It is caused by a germline mutation in the adenomatous polyposis coli ( APC ) tumor suppressor gene on chromosome 5q21 and is inherited in an autosomal dominant pattern [2]. The incidence of FAP is reported to be approximately 1 in 8,300 [1]. Without intervention, essentially all patients will develop colorectal cancer by age 40 [1,3]. Total proctocolectomy with ileal pouch-anal anastomosis (IPAA) is a commonly performed procedure to remove colonic and rectal epithelium at highest risk for malignant transformation in patients with FAP [4–6] and may be performed 1, 2 or 3 stages [7]. Single-stage LPC-IPAA avoids a temporary ileostomy and avoids the risks of having to undergo multiple operations [13]. The traditional two-stage procedure consists of total proctocolectomy with IPAA and diverting ileostomy, which is later closed at the second stage. The more contemporary two-stage approach, which we routinely offer patients with medically refractory ulcerative colitis (UC), consists of a laparoscopic colectomy and ileostomy, followed by completion proctectomy, IPAA, and ileostomy closure at the second operation. A three-stage approach includes total abdominal colectomy with end ileostomy, followed by completion proctectomy, IPAA, and diverting loop ileostomy at the second stage, and then closure of the diverting ileostomy at the third stage. Outcomes following total proctocolectomy with IPAA tend to be more favorable in patients with FAP compared to those with UC [10–12]. Laparoscopic proctocolectomy with IPAA has been consistently demonstrated to offer similar outcomes with lower morbidity compared to open approaches [15–17]. While LPC-IPAA for FAP via a two-stage approach has been established in previous studies as a safe and effective approach for patients who require total proctocolectomy, outcomes following a single-stage approach have not been thoroughly investigated. Although existing studies appear to support the safety and efficacy of single-stage total proctocolectomy with IPAA for select patients with inflammatory bowel disease and those with polyposis syndromes, conclusions are derived from limited sample sizes [14,18,19]. The primary goal of this study is to characterize the safety and efficacy of single-stage LPC-IPAA for the treatment of FAP. METHODS Operative Technique Our operative approach has evolved over many years but is fundamentally based on the technique originally published by Geiger et al. [9]. An abdominal colectomy is performed laparoscopically via 3 5mm ports and an advanced bipolar electrocautery device. A small low-transverse Pfannenstiel incision is made and the lateral avascular attachments of the small bowel mesentery are divided up to the level of the duodenum. After ensuring that adequate length has been mobilized for a tension-free IPAA, the bowel is packed away using warm moist laparotomy pads. The mesorectum is divided using an advanced bipolar device. An endorectal mucosectomy is started just below the peritoneal reflection and continued down to the level of the eventual anastomosis, which is in the mid-to-upper third of the anal canal, a few centimeters below the transition from rough rectal mucosa to smooth anoderm (the top of the crypts), generally appreciable by trans-anal palpation, and usually approximately 2-4cm above the dentate line. We do not evert the mucosa; instead, the mucosal tube is transected 1cm above the limit of the endorectal dissection (the eventual anastomotic site) using a 30mm stapling device. Stay sutures are placed in the muscle cuff to create a funnel shape. An 8-10cm J-pouch is made using a 75-80mm gastrointestinal linear stapler and reinforced as appropriate. In the past, we would routinely perform a hand-sewn anastomosis trans-anally using interrupted braided sutures, primarily due to surgeon preference, but this is almost never felt to be necessary anymore. We now perform all anastomoses with a 25-30mm end-to-end stapling device to create a tension-free J-pouch ileo-anal anastomosis. Tissue donuts are inspected and air insufflation under saline is used to confirm the absence of an air leak. Small leaks are repaired from above or below as indicated. The muscular cuff is tacked to the J-pouch with interrupted braided absorbable sutures. Although always potentially indicated in case of excessive tension or intraoperative misadventure, we rarely feel the need to perform a protective diverting ileostomy. Study Design We performed a retrospective cohort study of patients diagnosed with FAP who underwent single-stage LPC-IPAA between 1/1/2013 and 8/1/2024 at a single pediatric center. Diagnosis of FAP was confirmed by colonoscopy preoperatively. The need for written informed consent was waived by the Children’s Hospital of Philadelphia institutional review board (IRB #18-015795). Baseline characteristics, postoperative complications, and functional outcomes were examined and compared between hand-sewn and stapled anastomosis groups. Postoperative pouchitis and stricture were diagnosed clinically and confirmed with colonoscopy with biopsy. Normal stool quality was defined as Bristol stool type 4. Long-term outcomes were based on documentation from the last follow-up visit with either general surgery or GI, whichever was most recent. Descriptive statistics are presented as means for continuous data and frequencies for categorical data. Groups were compared using Fisher’s exact tests for categorical variables and Student’s t-tests for continuous variables. All variables with non-zero missingness are reported in Supplementary Table 1. Analyses were performed using Stata/BE 18.5 (StataCorp, College Station, TX). Statistical significance was defined as an alpha level of < 0.05. RESULTS Thirty-nine patients met the inclusion criteria for the study (Table 1 ). Mean age at surgery was 14.9 years (range 9.4–21.5) and mean BMI was 23.0 (range 15.3–47.0). There was a slight female preponderance, 24 (61.5%) versus 15 (38.5%). Thirty-five (89.7%) patients had a family history of FAP, 16 (41.0%) had a family history of colon cancer, and 8 (20.5%) had Gardner syndrome. Seven patients had additional medical comorbidities. No patient had been treated with corticosteroids. All cases at our institution are performed by a single surgeon. Mean overall follow-up was 2.8 years (SD 2.9). Mean overall operative time was 244.2 minutes (SD 55.4). There were no intraoperative complications. Table 1 Characteristics of FAP patients who underwent single-stage LPC-IPAA, by anastomosis type Total 39 (100) Stapled 32 (82.0) Hand-sewn 7 (18.0) p Demographics Age, y 14.9 (2.7) 14.7 (2.6) 15.8 (2.9) 0.35 BMI, kg/m 2 23.0 (6.5) 23.3 (6.9) 21.5 (4.4) 0.53 Male sex 15 (38.5) 13 (40.6) 2 (28.6) 0.69 Race 1.00 White 25 (64.1) 20 (64.5) 5 (71.4) Black 6 (15.4) 5 (16.1) 1 (14.3) Other 7 (18.0) 6 (19.4) 1 (14.3) Comorbidities a 7 (18.0) 5 (15.6) 2 (28.6) 0.62 Family history of FAP 35 (89.7) 28 (87.5) 7 (100.0) 1.00 Family history of colon cancer 16 (41.0) 12 (37.5) 4 (57.1) 0.42 Gardner syndrome 8 (20.5) 6 (18.8) 2 (28.6) 0.62 Categorical data expressed as n (%); continuous data expressed as mean (standard deviation). a Comorbidities included: Type 1 Diabetes (n = 1); Celiac Disease (n = 1); Asthma (n = 1); Psoriasis, GH Deficiency, ADHD (n = 1); Chromosomal abnormality, Hip Dysplasia (n = 1); Acquired Hypothyroidism (n = 1); Autism (n = 1) BMI = body mass index; FAP = familial adenomatous polyposis; LPC-IPAA = Laparoscopic Proctocolectomy, Ileal pouch-anal anastomosis The J-pouch ileoanal anastomosis was hand-sewn in 7 patients (17.9%) and stapled in 32 patients (82.1%) (Table 1 ). All anastomoses were initially hand-sewn, but after 2014, all but one patient underwent stapled IPAA. There were no differences in demographics between groups. Follow-up time in the stapled IPAA group averaged 2.2 years (SD 2.2) compared to 5.8 years (SD 3.7) in the hand-sewn group ( p = 0.03). Mean operative time was 228.4 minutes (SD 44.5) in the stapled group and 316.1 minutes (SD 43.1) in the hand-sewn group ( p < 0.001). Comparing patients in the stapled and hand-sewn groups, the mean post-operative length of stay was 7.2 days (SD 2.3) and 7.0 days (SD 1.7) respectively ( p = 0.83) (Table 2 ). Patients returned to a regular diet in a mean time of 4.0 days (SD 1.4) vs. 3.7 days (SD 2.7). Time to ambulation was on average 1.8 days (SD 1.1) vs. 2.1 days (SD 1.5) ( p = 0.55). Regarding surgical complications, a total of 8 patients (20.5%) experienced short-term complications, of which 7 were post-operative ileus and 1 developed an intraabdominal abscess. The patient with the intraabdominal abscess had a stapled anastomosis and was successfully treated with IV antibiotics (the collection was not amenable to drainage). There were no anastomotic leaks. One patient in the stapled anastomosis group returned to the operating room on postoperative day 6 for diagnostic laparoscopy due to concern for possible anastomotic leak, which revealed an ileus without evidence of leak or perforation. Six patients (15.4%) developed pouchitis, all of whom were successfully treated with antibiotics. There was a significantly greater rate of pouchitis in patients with a hand-sewn anastomosis compared to those with a stapled anastomosis (57.1% vs 6.3%, p = 0.01). Anastomotic stricture occurred in 6 patients (15.4%); all cases of stricture were in the stapled anastomosis group and were mild and resolved after only one dilation. There were no significant differences in other post-operative complications and long-term functional outcomes based on anastomosis type ( p > 0.05). Table 2 Post-operative outcomes following single-stage LPC- IPAA for FAP, by anastomosis type Variable Total 39 (100) Stapled 32 (82.0) Hand-sewn 7 (18.0) p Post-operative data Follow-up time, y 2.8 (2.9) 2.2 (2.2) 5.8 (3.7) 0.03* Length of stay, d 7.2 (2.2) 7.2 (2.3) 7.0 (1.7) 0.83 Time to regular diet, d 3.9 (1.6) 4.0 (1.4) 3.7 (2.7) 0.71 Time to ambulation, d 1.9 (1.2) 1.8 (1.1) 2.1 (1.5) 0.55 Complications a < 30d 8 (20.5) 6 (18.8) 2 (28.6) 0.62 Return to OR b < 30d 1 (2.6) 1 (3.1) 0 (0.0) 1.00 Stricture 6 (15.4) 6 (18.8) 0 (0.0) 0.57 Pouchitis 6 (15.4) 2 (6.3) 4 (57.1) 0.01* Long-term outcomes Presence of daily bowel movement 32 (100.0) 26 (100.0) 6 (100.0) 1.00 Average daily bowel movements 5.1 (3.1) 5.1 (3.4) 5.0 (1.7) 0.95 Normal stool consistency 25 (78.1) 21 (80.8) 4 (66.7) 0.59 Constipation 1 (3.1) 1 (3.9) 0 (0.0) 1.00 Abdominal pain 9 (28.1) 6 (23.1) 3 (50.0) 0.31 Nausea 6 (18.8) 4 (15.4) 2 (33.3) 0.31 Vomiting 1 (3.1) 1 (3.9) 0 (0.0) 1.00 Decreased appetite 6 (18.8) 5 (19.2) 1 (16.7) 1.00 Categorical data are expressed as n (%) and continuous data as mean (standard deviation). *Asterisk denotes statistical significance (p < 0.05). a postoperative ileus (n = 7) and intraabdominal abscess (n = 1, stapled anastomosis group) b Return to OR reason: peritoneal washout During the follow-up period, all patients had daily bowel movements after recovery from surgery, with 78.1% of patients reporting normal stool consistency. No patients developed incontinence requiring long-term management. One patient (3.1%) experienced constipation. Abdominal pain was reported in 28.1% of patients, while nausea and decreased appetite were reported by 18.8% of patients. Two patients developed desmoid tumors, one of whom underwent resection and was found to have recurrence 6 years after resection. This patient was subsequently treated with sorafenib for one year and remains under surveillance without evidence of disease progression. The other patient was primarily treated with sorafenib. DISCUSSION The primary aim of this study is to characterize the safety and efficacy of single-stage LPC-IPAA for the treatment of pediatric patients with FAP at our institution. We present a group of 39 consecutive children and adolescents with FAP who underwent single-stage LPC-IPAA. Cases of pouchitis and stricture were mild and successfully treated without complication. There were no anastomotic leaks. No patients experienced persistent incontinence. Patients with a stapled anastomosis had a shorter operative duration and a lower rate of pouchitis than those with a hand-sewn anastomosis. Other complications and long-term outcomes did not differ significantly. Overall postoperative complications in our cohort appear to be lower than other reports of outcomes following single-stage IPAA for FAP patients. Kennedy et al. compared one- and two-stage IPAA for FAP in pediatric patients at their institution and found that one-stage IPAA was associated with significantly higher rates of anastomotic leak (17.2% vs . 0%) and reoperation within 30 days of surgery (20.7% vs . 4.6%) [8]. In contrast, none of the patients in this study developed an anastomotic leak, and only one patient (2.6%) underwent reoperation within 30 days for a possible anastomotic leak that was ruled out. Pouchitis in FAP patients following IPAA may occur at a rate of up to 32% [8,20,21]. In the study performed by Kennedy et al., 17.2% of patients developed pouchitis after undergoing single-stage IPAA for FAP, which is similar to the rate of pouchitis detected in our study (15.4%). With regard to postoperative anal stricture, Kennedy et al. observed postoperative anal stricture in 24.1% of patients who underwent single-stage IPAA for FAP, higher than our observed anal stricture rate of 15.4%. Long-term postoperative bowel function in our cohort was favorable. All patients in our study had good bowel control without excessive urgency or incontinence. All patients had a manageable number of daily bowel movements with most reporting normal stool consistency. These findings again differ from those found by Kennedy et al., who report a 10.7% incontinence rate after single-stage IPAA, significantly lower than 36.0% in two-stage IPAA patients. Only one patient in our cohort developed a postoperative abscess, and there were no cases of postoperative obstruction. These complications occurred less often in our study compared to another study that reported pelvic abscess formation in 8% of patients and obstruction in 7%. [22]. While there was a higher rate of stricture in our stapled cohort, Konishi et al. found lower stricture rates in patients who underwent IPAA with a stapled anastomosis compared to those with a hand-sewn anastomosis [23]. A prior meta-analysis by Lovegrove et al. found no significant differences in postoperative complications based on anastomosis type but did find that stapled anastomoses were associated with lower incidence of nocturnal seepage and pad usage [24]. While findings from this study support single-stage proctocolectomy with IPAA for FAP, single-stage approaches may also be a safe and reasonable alternative to the traditional two-stage approach for patients with UC [7,25]. A meta-analysis by Weston-Petrides found an increased rate of anastomotic leak in FAP/UC patients who underwent restorative proctocolectomy without ostomy creation as well as an increased rate of anastomotic stricture in the group with ostomy [26]. Most recent studies comparing single- vs. two-stage approaches to total proctocolectomy with IPAA for UC are retrospective, thus raising concerns for selection bias. We still generally recommend a contemporary two-stage approach (laparoscopic colectomy/ileostomy followed 2–3 months later with mucosal proctectomy/J-pouch IPAA/ileostomy closure). The primary rationale is that most of our patients with medically refractory UC referred for consideration of surgical intervention are malnourished and anemic and have been exposed to high doses of corticosteroids or multiple biologic agents, making them less-than-ideal candidates for a big operation. In a recent review, Chen et al. suggested that patients with UC have fewer complications and secondary operations when diverted at the second stage, implying that perhaps we should consider going back to performing the operation in three stages [27]. However, they ultimately conclude that, despite the higher early complication rate, long-term outcomes are similar with or without routine diversion [27]. Rather than routinely subjecting every patient to diversion, we tell every patient that we might feel compelled for safety reasons to give them an ileostomy after proctectomy/J-pouch IPAA if we feel there is excessive tension on the pouch, the tissues are friable or grossly inflamed, or if the patient is still poorly nourished, cushingoid or chronically ill. Even Gray et al. recommend a period of nutritional rehabilitation for some weeks before considering a single-stage operation and also seem to suggest that concurrent steroid therapy might be a contraindication to a single-staged approach [25]. We have not adopted a single-staged approach for all patients, but for the rare patients with UC who are nutritionally sound and clinically well, we will sometimes offer this as an option. For the majority of patients, however, we continue to believe that the best way to help them improve their nutrition in preparation for proctectomy and J-pouch reconstruction is to remove their diseased colon as soon as possible, which almost always allows them fairly quickly to come off systemic therapy and rather dramatically increase their enteric caloric intake. In the end, prospective randomized studies will likely be needed to help elucidate whether single-staged proctocolectomy/IPAA should be the standard approach in patients with medically refractory UC. Our study has several limitations to take into consideration. We acknowledge that the retrospective nature of the study and unmeasured confounding variables could affect our results and interpretations. Moreover, the data originates from a single institution, which might limit generalizability. We also recognize that long-term complications may be under-detected if they occurred beyond the follow-up period in this study or if the patient presented to an outside institution with a complication. It is also important to note that hand-sewn IPAA was performed during an earlier time period than stapled IPAA. Correspondingly, the follow-up period in the hand-sewn IPAA group is longer than the stapled group and observed complication rates, such as pouchitis, must be interpreted with this in mind. Lastly, the small sample size, particularly in patients with a hand-sewn analysis, restricts the statistical power of our analyses. A large, multicenter, prospective study would help address the shortcomings of this study. In summary, single-stage LPC-IPAA for FAP in the pediatric population is safe and effective. Stapled and hand-sewn IPAA anastomosis techniques have comparable complication rates; however, stapled anastomosis takes less time to perform. Complications such as pouchitis and postoperative stricture respond well to conservative measures, and long-term functional outcomes are favorable overall. Declarations Disclosures: The authors have nothing to disclose. Conflicts of interest: The authors have no conflicts of interest to report. Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Author Contribution A.M and S.E.K wrote the main manuscript text. R.H. obtained and curated the data used in this work. S.E.K. performed the statistical analysis. A.M. and S.E.K prepared tables and figures. P.M. supervised and guided the work. All authors reviewed the manuscript. References Half E, Bercovich D, Rozen P. Familial adenomatous polyposis. Orphanet J Rare Dis 2009;4:22. https://doi.org/10.1186/1750-1172-4-22. Kinzler KW, Nilbert MC, Su L-K, et al. Identification of FAP Locus Genes from Chromosome 5q21. Science 1991;253:661–5. https://doi.org/10.1126/science.1651562. Vasen HFA, Moslein G, Alonso A, et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut 2008;57:704–13. https://doi.org/10.1136/gut.2007.136127. Aziz O, Athanasiou T, Fazio VW, et al. 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Additional Declarations No competing interests reported. Supplementary Files SupplementaryTable1.docx Cite Share Download PDF Status: Published Journal Publication published 15 Nov, 2025 Read the published version in Pediatric Surgery International → Version 1 posted Editorial decision: Revision requested 18 Oct, 2025 Reviews received at journal 13 Oct, 2025 Reviewers agreed at journal 30 Sep, 2025 Reviewers invited by journal 28 Sep, 2025 Editor assigned by journal 24 Sep, 2025 Submission checks completed at journal 23 Sep, 2025 First submitted to journal 22 Sep, 2025 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. 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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-7677571","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":524445821,"identity":"66e541fc-e374-4529-8712-ba239572aabf","order_by":0,"name":"Andrew Mudreac","email":"","orcid":"","institution":"Children’s Hospital of Philadelphia","correspondingAuthor":false,"prefix":"","firstName":"Andrew","middleName":"","lastName":"Mudreac","suffix":""},{"id":524445822,"identity":"2bc28977-c768-4ec7-904d-5c7699b25566","order_by":1,"name":"Spencer E. Kim","email":"","orcid":"","institution":"University of Pennsylvania","correspondingAuthor":false,"prefix":"","firstName":"Spencer","middleName":"E.","lastName":"Kim","suffix":""},{"id":524445823,"identity":"9ce1d789-fb03-42c3-8b95-58ef95ee2d70","order_by":2,"name":"Kara Kennedy","email":"","orcid":"","institution":"Children’s Hospital of Philadelphia","correspondingAuthor":false,"prefix":"","firstName":"Kara","middleName":"","lastName":"Kennedy","suffix":""},{"id":524445824,"identity":"a9fa0896-a27d-4624-a20c-86b3537e2767","order_by":3,"name":"William R. Johnston","email":"","orcid":"","institution":"Children’s Hospital of Philadelphia","correspondingAuthor":false,"prefix":"","firstName":"William","middleName":"R.","lastName":"Johnston","suffix":""},{"id":524445825,"identity":"bf5400a9-6f8b-4237-bbd3-ef0221540473","order_by":4,"name":"Rosa Hwang","email":"","orcid":"","institution":"Children’s Hospital of Philadelphia","correspondingAuthor":false,"prefix":"","firstName":"Rosa","middleName":"","lastName":"Hwang","suffix":""},{"id":524445826,"identity":"2c162491-e292-4b8b-9ad1-d31010326c46","order_by":5,"name":"Peter 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18:11:53","extension":"html","order_by":5,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":66683,"visible":true,"origin":"","legend":"","description":"","filename":"earlyproof.html","url":"https://assets-eu.researchsquare.com/files/rs-7677571/v1/9b46fb73aeb4581cd0c285d8.html"},{"id":96106156,"identity":"5a247866-28f6-4a12-99be-b1b9caf2e6bb","added_by":"auto","created_at":"2025-11-17 16:12:44","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":531058,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7677571/v1/0ea7ee52-edf9-4cb5-ac5e-527ad110e857.pdf"},{"id":93166284,"identity":"b2c50a52-53ee-49a1-abad-19d5af16e594","added_by":"auto","created_at":"2025-10-09 18:03:53","extension":"docx","order_by":0,"title":"","display":"","copyAsset":false,"role":"supplement","size":20403,"visible":true,"origin":"","legend":"","description":"","filename":"SupplementaryTable1.docx","url":"https://assets-eu.researchsquare.com/files/rs-7677571/v1/980334198e3997228ba3575f.docx"}],"financialInterests":"No competing interests reported.","formattedTitle":"Single-stage Laparoscopic Proctocolectomy with Ileal pouch-anal Anastomosis for Familial Adenomatous Polyposis","fulltext":[{"header":"INTRODUCTION","content":"\u003cp\u003eFamilial adenomatous polyposis (FAP) is characterized by the formation of intestinal \u0026ndash; primarily colonic \u0026ndash; adenomas, usually beginning within the first three decades of life [1]. It is caused by a germline mutation in the adenomatous polyposis coli (\u003cem\u003eAPC\u003c/em\u003e) tumor suppressor gene on chromosome 5q21 and is inherited in an autosomal dominant pattern [2]. The incidence of FAP is reported to be approximately 1 in 8,300 [1]. Without intervention, essentially all patients will develop colorectal cancer by age 40 [1,3].\u003c/p\u003e\u003cp\u003eTotal proctocolectomy with ileal pouch-anal anastomosis (IPAA) is a commonly performed procedure to remove colonic and rectal epithelium at highest risk for malignant transformation in patients with FAP [4\u0026ndash;6] and may be performed 1, 2 or 3 stages [7]. Single-stage LPC-IPAA avoids a temporary ileostomy and avoids the risks of having to undergo multiple operations [13]. The traditional two-stage procedure consists of total proctocolectomy with IPAA and diverting ileostomy, which is later closed at the second stage. The more contemporary two-stage approach, which we routinely offer patients with medically refractory ulcerative colitis (UC), consists of a laparoscopic colectomy and ileostomy, followed by completion proctectomy, IPAA, and ileostomy closure at the second operation. A three-stage approach includes total abdominal colectomy with end ileostomy, followed by completion proctectomy, IPAA, and diverting loop ileostomy at the second stage, and then closure of the diverting ileostomy at the third stage. Outcomes following total proctocolectomy with IPAA tend to be more favorable in patients with FAP compared to those with UC [10\u0026ndash;12].\u003c/p\u003e\u003cp\u003eLaparoscopic proctocolectomy with IPAA has been consistently demonstrated to offer similar outcomes with lower morbidity compared to open approaches [15\u0026ndash;17]. While LPC-IPAA for FAP via a two-stage approach has been established in previous studies as a safe and effective approach for patients who require total proctocolectomy, outcomes following a single-stage approach have not been thoroughly investigated. Although existing studies appear to support the safety and efficacy of single-stage total proctocolectomy with IPAA for select patients with inflammatory bowel disease and those with polyposis syndromes, conclusions are derived from limited sample sizes [14,18,19]. The primary goal of this study is to characterize the safety and efficacy of single-stage LPC-IPAA for the treatment of FAP.\u003c/p\u003e"},{"header":"METHODS","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\u003ch2\u003eOperative Technique\u003c/h2\u003e\u003cp\u003eOur operative approach has evolved over many years but is fundamentally based on the technique originally published by Geiger et al. [9]. An abdominal colectomy is performed laparoscopically via 3 5mm ports and an advanced bipolar electrocautery device. A small low-transverse Pfannenstiel incision is made and the lateral avascular attachments of the small bowel mesentery are divided up to the level of the duodenum. After ensuring that adequate length has been mobilized for a tension-free IPAA, the bowel is packed away using warm moist laparotomy pads. The mesorectum is divided using an advanced bipolar device. An endorectal mucosectomy is started just below the peritoneal reflection and continued down to the level of the eventual anastomosis, which is in the mid-to-upper third of the anal canal, a few centimeters below the transition from rough rectal mucosa to smooth anoderm (the top of the crypts), generally appreciable by trans-anal palpation, and usually approximately 2-4cm above the dentate line. We do not evert the mucosa; instead, the mucosal tube is transected 1cm above the limit of the endorectal dissection (the eventual anastomotic site) using a 30mm stapling device. Stay sutures are placed in the muscle cuff to create a funnel shape. An 8-10cm J-pouch is made using a 75-80mm gastrointestinal linear stapler and reinforced as appropriate. In the past, we would routinely perform a hand-sewn anastomosis trans-anally using interrupted braided sutures, primarily due to surgeon preference, but this is almost never felt to be necessary anymore. We now perform all anastomoses with a 25-30mm end-to-end stapling device to create a tension-free J-pouch ileo-anal anastomosis. Tissue donuts are inspected and air insufflation under saline is used to confirm the absence of an air leak. Small leaks are repaired from above or below as indicated. The muscular cuff is tacked to the J-pouch with interrupted braided absorbable sutures. Although always potentially indicated in case of excessive tension or intraoperative misadventure, we rarely feel the need to perform a protective diverting ileostomy.\u003c/p\u003e\u003c/div\u003e\n\u003ch3\u003eStudy Design\u003c/h3\u003e\n\u003cp\u003eWe performed a retrospective cohort study of patients diagnosed with FAP who underwent single-stage LPC-IPAA between 1/1/2013 and 8/1/2024 at a single pediatric center. Diagnosis of FAP was confirmed by colonoscopy preoperatively. The need for written informed consent was waived by the Children\u0026rsquo;s Hospital of Philadelphia institutional review board (IRB #18-015795).\u003c/p\u003e\u003cp\u003eBaseline characteristics, postoperative complications, and functional outcomes were examined and compared between hand-sewn and stapled anastomosis groups. Postoperative pouchitis and stricture were diagnosed clinically and confirmed with colonoscopy with biopsy. Normal stool quality was defined as Bristol stool type 4. Long-term outcomes were based on documentation from the last follow-up visit with either general surgery or GI, whichever was most recent.\u003c/p\u003e\u003cp\u003eDescriptive statistics are presented as means for continuous data and frequencies for categorical data. Groups were compared using Fisher\u0026rsquo;s exact tests for categorical variables and Student\u0026rsquo;s t-tests for continuous variables. All variables with non-zero missingness are reported in Supplementary Table\u0026nbsp;1. Analyses were performed using Stata/BE 18.5 (StataCorp, College Station, TX). Statistical significance was defined as an alpha level of \u0026lt;\u0026thinsp;0.05.\u003c/p\u003e"},{"header":"RESULTS","content":"\u003cp\u003eThirty-nine patients met the inclusion criteria for the study (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). Mean age at surgery was 14.9 years (range 9.4\u0026ndash;21.5) and mean BMI was 23.0 (range 15.3\u0026ndash;47.0). There was a slight female preponderance, 24 (61.5%) versus 15 (38.5%). Thirty-five (89.7%) patients had a family history of FAP, 16 (41.0%) had a family history of colon cancer, and 8 (20.5%) had Gardner syndrome. Seven patients had additional medical comorbidities. No patient had been treated with corticosteroids. All cases at our institution are performed by a single surgeon. Mean overall follow-up was 2.8 years (SD 2.9). Mean overall operative time was 244.2 minutes (SD 55.4). There were no intraoperative complications.\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\u003eCharacteristics of FAP patients who underwent single-stage LPC-IPAA, by anastomosis type\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=\"char\" char=\".\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTotal\u003c/p\u003e\u003cp\u003e39 (100)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003eStapled\u003c/p\u003e\u003cp\u003e32 (82.0)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003eHand-sewn\u003c/p\u003e\u003cp\u003e7 (18.0)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c5\"\u003e\u003cp\u003ep\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eDemographics\u003c/b\u003e\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\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAge, y\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e14.9 (2.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e14.7 (2.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e15.8 (2.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.35\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eBMI, kg/m\u003csup\u003e2\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e23.0 (6.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e23.3 (6.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e21.5 (4.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.53\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eMale sex\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e15 (38.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e13 (40.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e2 (28.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.69\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eRace\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\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e1.00\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eWhite\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e25 (64.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e20 (64.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e5 (71.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eBlack\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e6 (15.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e5 (16.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e1 (14.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eOther\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e7 (18.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e6 (19.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e1 (14.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eComorbidities\u003csup\u003ea\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e7 (18.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e5 (15.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e2 (28.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.62\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eFamily history of FAP\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e35 (89.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e28 (87.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e7 (100.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e1.00\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eFamily history of colon cancer\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e16 (41.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e12 (37.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e4 (57.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.42\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eGardner syndrome\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e8 (20.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e6 (18.8)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e2 (28.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.62\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003ctfoot\u003e\u003ctr\u003e\u003ctd colspan=\"5\"\u003eCategorical data expressed as n (%); continuous data expressed as mean (standard deviation).\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd colspan=\"5\"\u003e\u003csup\u003ea\u003c/sup\u003e Comorbidities included: Type 1 Diabetes (n\u0026thinsp;=\u0026thinsp;1); Celiac Disease (n\u0026thinsp;=\u0026thinsp;1); Asthma (n\u0026thinsp;=\u0026thinsp;1); Psoriasis, GH Deficiency, ADHD (n\u0026thinsp;=\u0026thinsp;1); Chromosomal abnormality, Hip Dysplasia (n\u0026thinsp;=\u0026thinsp;1); Acquired Hypothyroidism (n\u0026thinsp;=\u0026thinsp;1); Autism (n\u0026thinsp;=\u0026thinsp;1)\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd colspan=\"5\"\u003eBMI\u0026thinsp;=\u0026thinsp;body mass index; FAP\u0026thinsp;=\u0026thinsp;familial adenomatous polyposis; LPC-IPAA\u0026thinsp;=\u0026thinsp;Laparoscopic Proctocolectomy, Ileal pouch-anal anastomosis\u003c/td\u003e\u003c/tr\u003e\u003c/tfoot\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e\u003cp\u003eThe J-pouch ileoanal anastomosis was hand-sewn in 7 patients (17.9%) and stapled in 32 patients (82.1%) (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). All anastomoses were initially hand-sewn, but after 2014, all but one patient underwent stapled IPAA. There were no differences in demographics between groups. Follow-up time in the stapled IPAA group averaged 2.2 years (SD 2.2) compared to 5.8 years (SD 3.7) in the hand-sewn group (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.03). Mean operative time was 228.4 minutes (SD 44.5) in the stapled group and 316.1 minutes (SD 43.1) in the hand-sewn group (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001).\u003c/p\u003e\u003cp\u003eComparing patients in the stapled and hand-sewn groups, the mean post-operative length of stay was 7.2 days (SD 2.3) and 7.0 days (SD 1.7) respectively (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.83) (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). Patients returned to a regular diet in a mean time of 4.0 days (SD 1.4) vs. 3.7 days (SD 2.7). Time to ambulation was on average 1.8 days (SD 1.1) vs. 2.1 days (SD 1.5) (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.55). Regarding surgical complications, a total of 8 patients (20.5%) experienced short-term complications, of which 7 were post-operative ileus and 1 developed an intraabdominal abscess. The patient with the intraabdominal abscess had a stapled anastomosis and was successfully treated with IV antibiotics (the collection was not amenable to drainage). There were no anastomotic leaks. One patient in the stapled anastomosis group returned to the operating room on postoperative day 6 for diagnostic laparoscopy due to concern for possible anastomotic leak, which revealed an ileus without evidence of leak or perforation. Six patients (15.4%) developed pouchitis, all of whom were successfully treated with antibiotics. There was a significantly greater rate of pouchitis in patients with a hand-sewn anastomosis compared to those with a stapled anastomosis (57.1% vs 6.3%, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.01). Anastomotic stricture occurred in 6 patients (15.4%); all cases of stricture were in the stapled anastomosis group and were mild and resolved after only one dilation. There were no significant differences in other post-operative complications and long-term functional outcomes based on anastomosis type (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026gt;\u0026thinsp;0.05).\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\u003ePost-operative outcomes following single-stage LPC- IPAA for FAP, by anastomosis type\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=\"char\" char=\".\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\"\u003e\u003cp\u003eVariable\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTotal\u003c/p\u003e\u003cp\u003e39 (100)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003eStapled\u003c/p\u003e\u003cp\u003e32 (82.0)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003eHand-sewn\u003c/p\u003e\u003cp\u003e7 (18.0)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c5\"\u003e\u003cp\u003ep\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003ePost-operative data\u003c/b\u003e\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\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eFollow-up time, y\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e2.8 (2.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e2.2 (2.2)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e5.8 (3.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e\u003cb\u003e0.03*\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLength of stay, d\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e7.2 (2.2)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e7.2 (2.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e7.0 (1.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.83\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eTime to regular diet, d\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e3.9 (1.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e4.0 (1.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e3.7 (2.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.71\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eTime to ambulation, d\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1.9 (1.2)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e1.8 (1.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e2.1 (1.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.55\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eComplications\u003csup\u003ea\u003c/sup\u003e \u0026lt; 30d\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e8 (20.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e6 (18.8)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e2 (28.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.62\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eReturn to OR\u003csup\u003eb\u003c/sup\u003e \u0026lt; 30d\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1 (2.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e1 (3.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0 (0.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e1.00\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eStricture\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e6 (15.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e6 (18.8)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0 (0.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.57\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePouchitis\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e6 (15.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e2 (6.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e4 (57.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e\u003cb\u003e0.01*\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eLong-term outcomes\u003c/b\u003e\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\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePresence of daily bowel movement\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e32 (100.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e26 (100.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e6 (100.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e1.00\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAverage daily bowel movements\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e5.1 (3.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e5.1 (3.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e5.0 (1.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.95\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eNormal stool consistency\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e25 (78.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e21 (80.8)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e4 (66.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.59\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eConstipation\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1 (3.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e1 (3.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0 (0.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e1.00\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAbdominal pain\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e9 (28.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e6 (23.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e3 (50.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.31\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eNausea\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e6 (18.8)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e4 (15.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e2 (33.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.31\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eVomiting\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1 (3.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e1 (3.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0 (0.0)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e1.00\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eDecreased appetite\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e6 (18.8)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e5 (19.2)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e1 (16.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e1.00\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003ctfoot\u003e\u003ctr\u003e\u003ctd colspan=\"5\"\u003eCategorical data are expressed as n (%) and continuous data as mean (standard deviation).\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd colspan=\"5\"\u003e*Asterisk denotes statistical significance (p\u0026thinsp;\u0026lt;\u0026thinsp;0.05).\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd colspan=\"5\"\u003e\u003csup\u003ea\u003c/sup\u003e postoperative ileus (n\u0026thinsp;=\u0026thinsp;7) and intraabdominal abscess (n\u0026thinsp;=\u0026thinsp;1, stapled anastomosis group)\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd colspan=\"5\"\u003e\u003csup\u003eb\u003c/sup\u003e Return to OR reason: peritoneal washout\u003c/td\u003e\u003c/tr\u003e\u003c/tfoot\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e\u003cp\u003eDuring the follow-up period, all patients had daily bowel movements after recovery from surgery, with 78.1% of patients reporting normal stool consistency. No patients developed incontinence requiring long-term management. One patient (3.1%) experienced constipation. Abdominal pain was reported in 28.1% of patients, while nausea and decreased appetite were reported by 18.8% of patients. Two patients developed desmoid tumors, one of whom underwent resection and was found to have recurrence 6 years after resection. This patient was subsequently treated with sorafenib for one year and remains under surveillance without evidence of disease progression. The other patient was primarily treated with sorafenib.\u003c/p\u003e"},{"header":"DISCUSSION","content":"\u003cp\u003eThe primary aim of this study is to characterize the safety and efficacy of single-stage LPC-IPAA for the treatment of pediatric patients with FAP at our institution. We present a group of 39 consecutive children and adolescents with FAP who underwent single-stage LPC-IPAA. Cases of pouchitis and stricture were mild and successfully treated without complication. There were no anastomotic leaks. No patients experienced persistent incontinence. Patients with a stapled anastomosis had a shorter operative duration and a lower rate of pouchitis than those with a hand-sewn anastomosis. Other complications and long-term outcomes did not differ significantly.\u003c/p\u003e\u003cp\u003eOverall postoperative complications in our cohort appear to be lower than other reports of outcomes following single-stage IPAA for FAP patients. Kennedy et al. compared one- and two-stage IPAA for FAP in pediatric patients at their institution and found that one-stage IPAA was associated with significantly higher rates of anastomotic leak (17.2% \u003cem\u003evs\u003c/em\u003e. 0%) and reoperation within 30 days of surgery (20.7% \u003cem\u003evs\u003c/em\u003e. 4.6%) [8]. In contrast, none of the patients in this study developed an anastomotic leak, and only one patient (2.6%) underwent reoperation within 30 days for a possible anastomotic leak that was ruled out. Pouchitis in FAP patients following IPAA may occur at a rate of up to 32% [8,20,21]. In the study performed by Kennedy et al., 17.2% of patients developed pouchitis after undergoing single-stage IPAA for FAP, which is similar to the rate of pouchitis detected in our study (15.4%). With regard to postoperative anal stricture, Kennedy et al. observed postoperative anal stricture in 24.1% of patients who underwent single-stage IPAA for FAP, higher than our observed anal stricture rate of 15.4%. Long-term postoperative bowel function in our cohort was favorable. All patients in our study had good bowel control without excessive urgency or incontinence. All patients had a manageable number of daily bowel movements with most reporting normal stool consistency. These findings again differ from those found by Kennedy et al., who report a 10.7% incontinence rate after single-stage IPAA, significantly lower than 36.0% in two-stage IPAA patients.\u003c/p\u003e\u003cp\u003eOnly one patient in our cohort developed a postoperative abscess, and there were no cases of postoperative obstruction. These complications occurred less often in our study compared to another study that reported pelvic abscess formation in 8% of patients and obstruction in 7%. [22]. While there was a higher rate of stricture in our stapled cohort, Konishi et al. found lower stricture rates in patients who underwent IPAA with a stapled anastomosis compared to those with a hand-sewn anastomosis [23]. A prior meta-analysis by Lovegrove et al. found no significant differences in postoperative complications based on anastomosis type but did find that stapled anastomoses were associated with lower incidence of nocturnal seepage and pad usage [24].\u003c/p\u003e\u003cp\u003eWhile findings from this study support single-stage proctocolectomy with IPAA for FAP, single-stage approaches may also be a safe and reasonable alternative to the traditional two-stage approach for patients with UC [7,25]. A meta-analysis by Weston-Petrides found an increased rate of anastomotic leak in FAP/UC patients who underwent restorative proctocolectomy without ostomy creation as well as an increased rate of anastomotic stricture in the group with ostomy [26]. Most recent studies comparing single- vs. two-stage approaches to total proctocolectomy with IPAA for UC are retrospective, thus raising concerns for selection bias. We still generally recommend a contemporary two-stage approach (laparoscopic colectomy/ileostomy followed 2\u0026ndash;3 months later with mucosal proctectomy/J-pouch IPAA/ileostomy closure). The primary rationale is that most of our patients with medically refractory UC referred for consideration of surgical intervention are malnourished and anemic and have been exposed to high doses of corticosteroids or multiple biologic agents, making them less-than-ideal candidates for a big operation. In a recent review, Chen et al. suggested that patients with UC have fewer complications and secondary operations when diverted at the second stage, implying that perhaps we should consider going back to performing the operation in three stages [27]. However, they ultimately conclude that, despite the higher early complication rate, long-term outcomes are similar with or without routine diversion [27]. Rather than routinely subjecting every patient to diversion, we tell every patient that we might feel compelled for safety reasons to give them an ileostomy after proctectomy/J-pouch IPAA if we feel there is excessive tension on the pouch, the tissues are friable or grossly inflamed, or if the patient is still poorly nourished, cushingoid or chronically ill. Even Gray et al. recommend a period of nutritional rehabilitation for some weeks before considering a single-stage operation and also seem to suggest that concurrent steroid therapy might be a contraindication to a single-staged approach [25]. We have not adopted a single-staged approach for all patients, but for the rare patients with UC who are nutritionally sound and clinically well, we will sometimes offer this as an option. For the majority of patients, however, we continue to believe that the best way to help them improve their nutrition in preparation for proctectomy and J-pouch reconstruction is to remove their diseased colon as soon as possible, which almost always allows them fairly quickly to come off systemic therapy and rather dramatically increase their enteric caloric intake. In the end, prospective randomized studies will likely be needed to help elucidate whether single-staged proctocolectomy/IPAA should be the standard approach in patients with medically refractory UC.\u003c/p\u003e\u003cp\u003eOur study has several limitations to take into consideration. We acknowledge that the retrospective nature of the study and unmeasured confounding variables could affect our results and interpretations. Moreover, the data originates from a single institution, which might limit generalizability. We also recognize that long-term complications may be under-detected if they occurred beyond the follow-up period in this study or if the patient presented to an outside institution with a complication. It is also important to note that hand-sewn IPAA was performed during an earlier time period than stapled IPAA. Correspondingly, the follow-up period in the hand-sewn IPAA group is longer than the stapled group and observed complication rates, such as pouchitis, must be interpreted with this in mind. Lastly, the small sample size, particularly in patients with a hand-sewn analysis, restricts the statistical power of our analyses. A large, multicenter, prospective study would help address the shortcomings of this study.\u003c/p\u003e\u003cp\u003eIn summary, single-stage LPC-IPAA for FAP in the pediatric population is safe and effective. Stapled and hand-sewn IPAA anastomosis techniques have comparable complication rates; however, stapled anastomosis takes less time to perform. Complications such as pouchitis and postoperative stricture respond well to conservative measures, and long-term functional outcomes are favorable overall.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eDisclosures:\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors have nothing to disclose.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConflicts of interest:\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors have no conflicts of interest to report.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding:\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor Contribution\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eA.M and S.E.K wrote the main manuscript text. R.H. obtained and curated the data used in this work. S.E.K. performed the statistical analysis. A.M. and S.E.K prepared tables and figures. P.M. supervised and guided the work. All authors reviewed the manuscript.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eHalf E, Bercovich D, Rozen P. Familial adenomatous polyposis. Orphanet J Rare Dis 2009;4:22. https://doi.org/10.1186/1750-1172-4-22.\u003c/li\u003e\n\u003cli\u003eKinzler KW, Nilbert MC, Su L-K, et al. Identification of FAP Locus Genes from Chromosome 5q21. Science 1991;253:661\u0026ndash;5. https://doi.org/10.1126/science.1651562.\u003c/li\u003e\n\u003cli\u003eVasen HFA, Moslein G, Alonso A, et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut 2008;57:704\u0026ndash;13. https://doi.org/10.1136/gut.2007.136127.\u003c/li\u003e\n\u003cli\u003eAziz O, Athanasiou T, Fazio VW, et al. Meta-analysis of observational studies of ileorectal \u003cem\u003eversus\u003c/em\u003e ileal pouch\u0026ndash;anal anastomosis for familial adenomatous polyposis. British Journal of Surgery 2006;93:407\u0026ndash;17. https://doi.org/10.1002/bjs.5276.\u003c/li\u003e\n\u003cli\u003eFlahive CB, Onwuka A, Bass LM, et al. Characterizing Pediatric Familial Adenomatous Polyposis in Patients Undergoing Colectomy in the United States. The Journal of Pediatrics 2022;245:117\u0026ndash;22. https://doi.org/10.1016/j.jpeds.2021.09.021.\u003c/li\u003e\n\u003cli\u003eHerzig D, Hardiman K, Weiser M, et al. The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Management of Inherited Polyposis Syndromes. Dis Colon Rectum 2017;60:881\u0026ndash;94. https://doi.org/10.1097/DCR.0000000000000912.\u003c/li\u003e\n\u003cli\u003eRubalcava NS, Gadepalli SK, Criss CN, et al. Single-stage restorative proctocolectomy for ulcerative colitis in pediatric patients: a safe alternative. Pediatr Surg Int 2021;37:1453\u0026ndash;9. https://doi.org/10.1007/s00383-021-04943-w.\u003c/li\u003e\n\u003cli\u003eAbdalkoddus M, Franklyn J, Balasubramanya S, et al. Long-term mental and physical quality of life outcomes following ileal pouch anal anastomosis surgery. Annals 2025;107:18\u0026ndash;24. https://doi.org/10.1308/rcsann.2023.0075.\u003c/li\u003e\n\u003cli\u003eLavryk O, Maspero M, Holubar SD, et al. Postoperative outcomes of a pelvic pouch procedure: Lessons learned over 40 years among 5070 patients. Journal of Gastrointestinal Surgery 2025;29:101938. https://doi.org/10.1016/j.gassur.2024.101938.\u003c/li\u003e\n\u003cli\u003eHuang CC, Rescorla FJ, Landman MP. Clinical Outcomes After Ileal Pouch-Anal Anastomosis in Pediatric Patients. Journal of Surgical Research 2019;234:72\u0026ndash;6. https://doi.org/10.1016/j.jss.2018.09.011.\u003c/li\u003e\n\u003cli\u003eDrews JD, Onwuka EA, Fisher JG, et al. Complications after proctocolectomy and ileal pouch-anal anastomosis in pediatric patients: A systematic review. Journal of Pediatric Surgery 2019;54:1331\u0026ndash;9. https://doi.org/10.1016/j.jpedsurg.2018.08.047.\u003c/li\u003e\n\u003cli\u003eKeleidari B, Mahmoudieh M, Shiasi M. Laparoscopic Restorative Total Proctocolectomy with Ileal Pouch-Anal Anastomosis for Familial Adenomatous Polyposis and Ulcerative Colitis. Advanced Biomedical Research 2023;12. https://doi.org/10.4103/abr.abr_249_21.\u003c/li\u003e\n\u003cli\u003eNozawa H, Hata K, Sasaki K, et al. Laparoscopic vs open restorative proctectomy after total abdominal colectomy for ulcerative colitis or familial adenomatous polyposis. Langenbecks Arch Surg 2022;407:1605\u0026ndash;12. https://doi.org/10.1007/s00423-022-02492-x.\u003c/li\u003e\n\u003cli\u003eHabermann A, Gassie H, Rustom S, et al. Trends in surgical outcomes for Ileal pouch\u0026ndash;anal anastomosis construction using a large nationwide database. Colorectal Disease 2024;26:1950\u0026ndash;8. https://doi.org/10.1111/codi.17188.\u003c/li\u003e\n\u003cli\u003eRyan DP, Doody DP. Restorative proctocolectomy with and without protective ileostomy in a pediatric population. Journal of Pediatric Surgery 2011;46:200\u0026ndash;3. https://doi.org/10.1016/j.jpedsurg.2010.09.085.\u003c/li\u003e\n\u003cli\u003ePotter DD, Tung J, Faubion WA, et al. Single-Incision Laparoscopic Colon and Rectal Surgery for Pediatric Inflammatory Bowel Disease and Polyposis Syndromes. Journal of Laparoendoscopic \u0026amp; Advanced Surgical Techniques 2012;22:203\u0026ndash;7. https://doi.org/10.1089/lap.2011.0117.\u003c/li\u003e\n\u003cli\u003eHor T, Zalinski S, Lefevre JH, et al. Feasibility of laparoscopic restorative proctocolectomy without diverting stoma. Digestive and Liver Disease 2012;44:118\u0026ndash;22. https://doi.org/10.1016/j.dld.2011.09.007.\u003c/li\u003e\n\u003cli\u003eGeiger JD, Teitelbaum DH, Hirschl RB, et al. A new operative technique for restorative proctocolectomy: the endorectal pull-through combined with a double-stapled ileo-anal anastomosis. Surgery 2003;134:492\u0026ndash;5. https://doi.org/10.1067/S0039-6060(03)00087-4.\u003c/li\u003e\n\u003cli\u003eKennedy RD, Zarroug AE, Moir CR, et al. Ileal pouch anal anastomosis in pediatric familial adenomatous polyposis: A 24-year review of operative technique and patient outcomes. Journal of Pediatric Surgery 2014;49:1409\u0026ndash;12. https://doi.org/10.1016/j.jpedsurg.2014.03.003.\u003c/li\u003e\n\u003cli\u003eGilad O, Gluck N, Brazowski E, et al. Determinants of Pouch-Related Symptoms, a Common Outcome of Patients With Adenomatous Polyposis Undergoing Ileoanal Pouch Surgery. Clin Transl Gastroenterol 2020;11:e00245. https://doi.org/10.14309/ctg.0000000000000245.\u003c/li\u003e\n\u003cli\u003eQuinn KP, Lightner AL, Pendegraft RS, et al. Pouchitis Is a Common Complication in Patients With Familial Adenomatous Polyposis Following Ileal Pouch\u0026ndash;Anal Anastomosis. Clinical Gastroenterology and Hepatology 2016;14:1296\u0026ndash;301. https://doi.org/10.1016/j.cgh.2016.04.010.\u003c/li\u003e\n\u003cli\u003eDuff SE, Sagar PM, Rao M, et al. Laparoscopic restorative proctocolectomy: safety and critical level of the ileal pouch anal anastomosis. Colorectal Disease 2012;14:883\u0026ndash;6. https://doi.org/10.1111/j.1463-1318.2011.02810.x.\u003c/li\u003e\n\u003cli\u003eKonishi T, Ishida H, Ueno H, et al. Postoperative complications after stapled and hand-sewn ileal pouch-anal anastomosis for familial adenomatous polyposis: A multicenter study. Ann Gastroenterol Surg 2017;1:143\u0026ndash;9. https://doi.org/10.1002/ags3.12019.\u003c/li\u003e\n\u003cli\u003eLovegrove RE, Constantinides VA, Heriot AG, et al. A Comparison of Hand-Sewn Versus Stapled Ileal Pouch Anal Anastomosis (IPAA) Following Proctocolectomy: A Meta-Analysis of 4183 Patients. Annals of Surgery 2006;244:18\u0026ndash;26. https://doi.org/10.1097/01.sla.0000225031.15405.a3.\u003c/li\u003e\n\u003cli\u003eGray BW, Drongowski RA, Hirschl RB, et al. Restorative proctocolectomy without diverting ileostomy in children with ulcerative colitis. Journal of Pediatric Surgery 2012;47:204\u0026ndash;8. https://doi.org/10.1016/j.jpedsurg.2011.10.041.\u003c/li\u003e\n\u003cli\u003eWeston-Petrides GK. Comparison of Outcomes After Restorative Proctocolectomy With or Without Defunctioning Ileostomy. Arch Surg 2008;143:406. https://doi.org/10.1001/archsurg.143.4.406.\u003c/li\u003e\n\u003cli\u003eChen YJ, Grant R, Lindholm E, et al. Is fecal diversion necessary during ileal pouch creation after initial subtotal colectomy in pediatric ulcerative colitis? Pediatr Surg Int 2019;35:443\u0026ndash;8. https://doi.org/10.1007/s00383-019-04440-1.\u003cbr\u003e \u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-surgery-international","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pesi","sideBox":"Learn more about [Pediatric Surgery International](http://link.springer.com/journal/383)","snPcode":"383","submissionUrl":"https://submission.nature.com/new-submission/383/3","title":"Pediatric Surgery International","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Familial Adenomatous Polyposis, Ileal Pouch-anal Anastomosis, Pediatrics, Total Proctocolectomy","lastPublishedDoi":"10.21203/rs.3.rs-7677571/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7677571/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003ePurpose: \u003c/strong\u003eAssess the safety of single-stage laparoscopic proctocolectomy with ileal pouch-anal anastomosis (LPC-IPAA) in children with familial adenomatous polyposis (FAP).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eMethods: \u003c/strong\u003eMedical records for all patients with FAP who underwent LPC-IPAA between 1/1/2013 and 8/1/2024 at a pediatric center were reviewed. Baseline characteristics, postoperative complications, and functional outcomes were assessed.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eResults: \u003c/strong\u003eThirty-nine patients met inclusion criteria.\u003cstrong\u003e \u003c/strong\u003eMean age was 14.9 years (range 9.4-21.5) and mean BMI was 23.0 (range 15.3-47.0). Follow-up averaged 2.8 years (SD 2.9). Mean operative time was 228.4 minutes (SD 44.5) in the stapled group (n = 32) and 316.1 minutes (SD 43.1) in the hand-sewn group (n = 7) (p \u0026lt; 0.001). There were no anastomotic leaks. One patient developed a postoperative abscess. Six (15%) developed presumed pouchitis, 4 of whom were in the hand-sewn group and all successfully treated with antibiotics. Six patients (15%) developed an anastomotic stricture – all in the stapled group – and responded well to anal dilation. No patient required treatment for fecal incontinence.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion: \u003c/strong\u003eLPC-IPAA in adolescents and young adults with FAP can safely be performed in a single operation and is associated with favorable functional outcomes.\u003c/p\u003e","manuscriptTitle":"Single-stage Laparoscopic Proctocolectomy with Ileal pouch-anal Anastomosis for Familial Adenomatous Polyposis","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-10-09 17:55:48","doi":"10.21203/rs.3.rs-7677571/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-10-18T19:52:16+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-10-13T10:36:20+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"217642215541158204764840201001153911545","date":"2025-10-01T03:09:52+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-09-28T20:54:56+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-09-24T18:06:14+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-09-23T04:22:47+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Surgery International","date":"2025-09-22T10:29:03+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":"ea4a2fe9-0143-4b1a-a35d-b6c0260d6a9a","owner":[],"postedDate":"October 9th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-11-17T16:11:36+00:00","versionOfRecord":{"articleIdentity":"rs-7677571","link":"https://doi.org/10.1007/s00383-025-06244-y","journal":{"identity":"pediatric-surgery-international","isVorOnly":false,"title":"Pediatric Surgery International"},"publishedOn":"2025-11-15 15:58:15","publishedOnDateReadable":"November 15th, 2025"},"versionCreatedAt":"2025-10-09 17:55:48","video":"","vorDoi":"10.1007/s00383-025-06244-y","vorDoiUrl":"https://doi.org/10.1007/s00383-025-06244-y","workflowStages":[]},"version":"v1","identity":"rs-7677571","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7677571","identity":"rs-7677571","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}