Xianming Xiao, Wei Feng, Jin Zhu, Linxiao Fan, Chenzhu Xiang, Zhili Wang, Jinping Hou, Wei Liu, Zhenhua Guo, Yi Wang
Anorectal malformation (ARM) and Hirschsprung disease (HSCR) are common congenital gastrointestinal defects, but their co-occurrence is rare. This retrospective study analyzed the clinical characteristics of patients with ARM associated with HSCR for early diagnosis and treatment guidance to reduce the occurrence of severe complications.A single-center retrospective cohort analysis from 2010 to 2024 identified 2,341 patients with ARM and 1,721 with HSCR. The histopathologic assessment included hematoxylin and eosin (H&E) staining and immunohistochemical staining.Seven patients (0.3%) out of 2,341 cases of ARM were diagnosed with concurrent HSCR, three males (42.9%) and four females (57.1%). Seven cases are rectoperineal fistula. All cases developed constipation with abdominal distension within 1 month to 1 year after anoplasty, even following aggressive bowel management. Barium enema showed obvious transition zones, and anorectal manometry revealed absent rectoanal inhibitory reflex in seven cases. All patients underwent the Swenson procedure. The mean duration of postoperative follow-up was 7.5 ± 2.8 years. Seven cases had no constipation, no soiling, voluntary bowel movements by Krickenbeck classification, and excellent continence by the Rintala scoring system in recent follow-up.The association between ARM and HSCR may be rarer than previously reported. Low-type ARM and short or rectosigmoid aganglionosis appeared more common in these cases. Persistent postoperative constipation and abdominal distension unresponsive to conservative treatment should raise suspicion for HSCR, prompting timely diagnostic evaluations. Postoperative bowel function needs to be interpreted carefully, and prospective studies are needed to confirm these findings and guide standardized care.
{"title":"Anorectal Malformations Associated with Hirschsprung Disease: Insights from a Large Cohort of 2,341 ARM Patients in a Single-Center Retrospective Study.","authors":"Xianming Xiao, Wei Feng, Jin Zhu, Linxiao Fan, Chenzhu Xiang, Zhili Wang, Jinping Hou, Wei Liu, Zhenhua Guo, Yi Wang","doi":"10.1055/a-2557-8073","DOIUrl":"10.1055/a-2557-8073","url":null,"abstract":"<p><p>Anorectal malformation (ARM) and Hirschsprung disease (HSCR) are common congenital gastrointestinal defects, but their co-occurrence is rare. This retrospective study analyzed the clinical characteristics of patients with ARM associated with HSCR for early diagnosis and treatment guidance to reduce the occurrence of severe complications.A single-center retrospective cohort analysis from 2010 to 2024 identified 2,341 patients with ARM and 1,721 with HSCR. The histopathologic assessment included hematoxylin and eosin (H&E) staining and immunohistochemical staining.Seven patients (0.3%) out of 2,341 cases of ARM were diagnosed with concurrent HSCR, three males (42.9%) and four females (57.1%). Seven cases are rectoperineal fistula. All cases developed constipation with abdominal distension within 1 month to 1 year after anoplasty, even following aggressive bowel management. Barium enema showed obvious transition zones, and anorectal manometry revealed absent rectoanal inhibitory reflex in seven cases. All patients underwent the Swenson procedure. The mean duration of postoperative follow-up was 7.5 ± 2.8 years. Seven cases had no constipation, no soiling, voluntary bowel movements by Krickenbeck classification, and excellent continence by the Rintala scoring system in recent follow-up.The association between ARM and HSCR may be rarer than previously reported. Low-type ARM and short or rectosigmoid aganglionosis appeared more common in these cases. Persistent postoperative constipation and abdominal distension unresponsive to conservative treatment should raise suspicion for HSCR, prompting timely diagnostic evaluations. Postoperative bowel function needs to be interpreted carefully, and prospective studies are needed to confirm these findings and guide standardized care.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143675066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ophelia Aubert, Marta Gazzaneo, Julio César Moreno-Alfonso, Hilmican Ulman, Hanna Garnier, Benno Ure, Martin Lacher
Objective: This study aims to assess publishing trends, motivations, preferences, and challenges among pediatric surgeons globally.
Methods: A cross-sectional survey was conducted among pediatric surgeons from multiple countries, distributed through the Trainees of European Pediatric Surgery (TEPS) network and social media. The anonymous questionnaire contained 26 items focusing on journal preferences, motivations for publishing, obstacles faced, peer-review experiences, open access publishing, and methods of research dissemination.
Results: A total of 172 responses were collected from pediatric surgeons in 33 countries. Most respondents worked in tertiary hospitals (88%) and were consultants or senior attendings (49%). Over half (65%) had published at least one scientific paper in the last 3 years. PubMed was the primary search engine (82%), and pediatric surgical journals were the preferred outlets for publication (87%). Key motivations for choosing a journal were impact factor (22%) and scope (19%), while publication costs (38%) and slow review processes (22%) were the primary deterrents. Open access publication options were used by more than half of respondents, with a third spending less than €2,500 on fees. Social media, particularly Instagram, emerged as a popular platform for research dissemination.
Conclusion: Pediatric surgeons prefer publishing in specialized journals, with impact factor and scope being key drivers of journal choice. Publication costs and the peer-review process are the most significant obstacles. Efforts to address these challenges, such as reducing fees and enhancing the review process, are crucial for facilitating research dissemination in pediatric surgery.
{"title":"Publishing Trends, Motivations, and Obstacles Among Pediatric Surgeons: An International Survey on Research Dissemination and Peer Review Challenges.","authors":"Ophelia Aubert, Marta Gazzaneo, Julio César Moreno-Alfonso, Hilmican Ulman, Hanna Garnier, Benno Ure, Martin Lacher","doi":"10.1055/a-2544-9739","DOIUrl":"https://doi.org/10.1055/a-2544-9739","url":null,"abstract":"<p><strong>Objective: </strong> This study aims to assess publishing trends, motivations, preferences, and challenges among pediatric surgeons globally.</p><p><strong>Methods: </strong> A cross-sectional survey was conducted among pediatric surgeons from multiple countries, distributed through the Trainees of European Pediatric Surgery (TEPS) network and social media. The anonymous questionnaire contained 26 items focusing on journal preferences, motivations for publishing, obstacles faced, peer-review experiences, open access publishing, and methods of research dissemination.</p><p><strong>Results: </strong> A total of 172 responses were collected from pediatric surgeons in 33 countries. Most respondents worked in tertiary hospitals (88%) and were consultants or senior attendings (49%). Over half (65%) had published at least one scientific paper in the last 3 years. PubMed was the primary search engine (82%), and pediatric surgical journals were the preferred outlets for publication (87%). Key motivations for choosing a journal were impact factor (22%) and scope (19%), while publication costs (38%) and slow review processes (22%) were the primary deterrents. Open access publication options were used by more than half of respondents, with a third spending less than €2,500 on fees. Social media, particularly Instagram, emerged as a popular platform for research dissemination.</p><p><strong>Conclusion: </strong> Pediatric surgeons prefer publishing in specialized journals, with impact factor and scope being key drivers of journal choice. Publication costs and the peer-review process are the most significant obstacles. Efforts to address these challenges, such as reducing fees and enhancing the review process, are crucial for facilitating research dissemination in pediatric surgery.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143789390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Richard Gnatzy, Martin Lacher, Michael Berger, Michael Boettcher, Oliver J Deffaa, Joachim Kübler, Omid Madadi-Sanjani, Illya Martynov, Steffi Mayer, Mikko P Pakarinen, Richard Wagner, Tomas Wester, Augusto Zani, Ophelia Aubert
The emergence of large language models (LLMs) has led to notable advancements across multiple sectors, including medicine. Yet, their effect in pediatric surgery remains largely unexplored. This study aims to assess the ability of the artificial intelligence (AI) models ChatGPT-4 and Microsoft Copilot to propose diagnostic procedures, primary and differential diagnoses, as well as answer clinical questions using complex clinical case vignettes of classic pediatric surgical diseases.We conducted the study in April 2024. We evaluated the performance of LLMs using 13 complex clinical case vignettes of pediatric surgical diseases and compared responses to a human cohort of experienced pediatric surgeons. Additionally, pediatric surgeons rated the diagnostic recommendations of LLMs for completeness and accuracy. To determine differences in performance, we performed statistical analyses.ChatGPT-4 achieved a higher test score (52.1%) compared to Copilot (47.9%) but less than pediatric surgeons (68.8%). Overall differences in performance between ChatGPT-4, Copilot, and pediatric surgeons were found to be statistically significant (p < 0.01). ChatGPT-4 demonstrated superior performance in generating differential diagnoses compared to Copilot (p < 0.05). No statistically significant differences were found between the AI models regarding suggestions for diagnostics and primary diagnosis. Overall, the recommendations of LLMs were rated as average by pediatric surgeons.This study reveals significant limitations in the performance of AI models in pediatric surgery. Although LLMs exhibit potential across various areas, their reliability and accuracy in handling clinical decision-making tasks is limited. Further research is needed to improve AI capabilities and establish its usefulness in the clinical setting.
{"title":"Solving Complex Pediatric Surgical Case Studies: A Comparative Analysis of Copilot, ChatGPT-4, and Experienced Pediatric Surgeons' Performance.","authors":"Richard Gnatzy, Martin Lacher, Michael Berger, Michael Boettcher, Oliver J Deffaa, Joachim Kübler, Omid Madadi-Sanjani, Illya Martynov, Steffi Mayer, Mikko P Pakarinen, Richard Wagner, Tomas Wester, Augusto Zani, Ophelia Aubert","doi":"10.1055/a-2551-2131","DOIUrl":"10.1055/a-2551-2131","url":null,"abstract":"<p><p>The emergence of large language models (LLMs) has led to notable advancements across multiple sectors, including medicine. Yet, their effect in pediatric surgery remains largely unexplored. This study aims to assess the ability of the artificial intelligence (AI) models ChatGPT-4 and Microsoft Copilot to propose diagnostic procedures, primary and differential diagnoses, as well as answer clinical questions using complex clinical case vignettes of classic pediatric surgical diseases.We conducted the study in April 2024. We evaluated the performance of LLMs using 13 complex clinical case vignettes of pediatric surgical diseases and compared responses to a human cohort of experienced pediatric surgeons. Additionally, pediatric surgeons rated the diagnostic recommendations of LLMs for completeness and accuracy. To determine differences in performance, we performed statistical analyses.ChatGPT-4 achieved a higher test score (52.1%) compared to Copilot (47.9%) but less than pediatric surgeons (68.8%). Overall differences in performance between ChatGPT-4, Copilot, and pediatric surgeons were found to be statistically significant (<i>p</i> < 0.01). ChatGPT-4 demonstrated superior performance in generating differential diagnoses compared to Copilot (<i>p</i> < 0.05). No statistically significant differences were found between the AI models regarding suggestions for diagnostics and primary diagnosis. Overall, the recommendations of LLMs were rated as average by pediatric surgeons.This study reveals significant limitations in the performance of AI models in pediatric surgery. Although LLMs exhibit potential across various areas, their reliability and accuracy in handling clinical decision-making tasks is limited. Further research is needed to improve AI capabilities and establish its usefulness in the clinical setting.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143568929","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-01Epub Date: 2025-01-13DOI: 10.1055/a-2514-7244
Thomas O Xu, Inbal Samuk, Christina Feng, Richard J Wood, Andrea Badillo, Marc A Levitt
Objective: A novel modification of the cutback procedure, a posterior rectal advancement anoplasty (PRAA) for select male patients with an anorectal malformation and a rectoperineal fistula was recently described, which incised only within the limits of the sphincteric ellipse and eliminated an anterior rectal dissection and thus avoiding any possibility of a urethral injury. This report provides longer-term postoperative outcomes after PRAA.
Method: A retrospective, single-institution study was performed examining male patients with a rectoperineal fistula between January 2020 and December 2023. PRAA was done only if the rectoperineal fistula was located within the anterior extent of the sphincteric ellipse, which was true for all patients encountered during this study period. We assessed postoperative outcomes, length of stay, time to first feeding, and early stooling patterns.
Results: Eighteen patients underwent PRAA at a median age of 5.4 months (range 1 day-8 months) with a median follow-up of 14 months (range 4-40). Seven patients (39%) were repaired within the first month of life. Thirteen (72%) were repaired primarily and five (28%) had a diverting ostomy placed before referral. There were no instances of wound dehiscence, rectal prolapse, or urethral injury. Two (11%) patients developed an anal stricture requiring dilation or Heineke-Mikulicz anoplasty. All patients resumed feeds on postoperative day one. Median discharge was on postoperative day one (range 1-6). Fifteen (83%) were utilizing laxatives at their most recent follow-up.
Conclusion: The PRAA avoids any potential urethral injury or perineal dehiscence has a low rate of anal stenosis (11%) and appears to be applicable to all male patients with a rectoperineal fistula. The technique allows for early return to diet and discharge and can be safely done in the neonatal period or in a delayed fashion without the need for a stoma.
{"title":"Anorectal Malformation with Rectoperineal Fistula in Males Treated with a Posterior Rectal Advancement Anoplasty: Report of Early Outcomes.","authors":"Thomas O Xu, Inbal Samuk, Christina Feng, Richard J Wood, Andrea Badillo, Marc A Levitt","doi":"10.1055/a-2514-7244","DOIUrl":"10.1055/a-2514-7244","url":null,"abstract":"<p><strong>Objective: </strong> A novel modification of the cutback procedure, a posterior rectal advancement anoplasty (PRAA) for select male patients with an anorectal malformation and a rectoperineal fistula was recently described, which incised only within the limits of the sphincteric ellipse and eliminated an anterior rectal dissection and thus avoiding any possibility of a urethral injury. This report provides longer-term postoperative outcomes after PRAA.</p><p><strong>Method: </strong> A retrospective, single-institution study was performed examining male patients with a rectoperineal fistula between January 2020 and December 2023. PRAA was done only if the rectoperineal fistula was located within the anterior extent of the sphincteric ellipse, which was true for all patients encountered during this study period. We assessed postoperative outcomes, length of stay, time to first feeding, and early stooling patterns.</p><p><strong>Results: </strong> Eighteen patients underwent PRAA at a median age of 5.4 months (range 1 day-8 months) with a median follow-up of 14 months (range 4-40). Seven patients (39%) were repaired within the first month of life. Thirteen (72%) were repaired primarily and five (28%) had a diverting ostomy placed before referral. There were no instances of wound dehiscence, rectal prolapse, or urethral injury. Two (11%) patients developed an anal stricture requiring dilation or Heineke-Mikulicz anoplasty. All patients resumed feeds on postoperative day one. Median discharge was on postoperative day one (range 1-6). Fifteen (83%) were utilizing laxatives at their most recent follow-up.</p><p><strong>Conclusion: </strong> The PRAA avoids any potential urethral injury or perineal dehiscence has a low rate of anal stenosis (11%) and appears to be applicable to all male patients with a rectoperineal fistula. The technique allows for early return to diet and discharge and can be safely done in the neonatal period or in a delayed fashion without the need for a stoma.</p><p><strong>Level of evidence: </strong> Level III.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":" ","pages":"141-146"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142980890","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-01Epub Date: 2025-03-24DOI: 10.1055/a-2528-5324
Marijke E B Kremer, Ramon R Gorter, Jen Tidman, Jan-Hendrick Gosemann, Ivo de Blaauw
{"title":"Development of European Consensus Guidelines and Evolution of Innovative Surgical Strategies for the Treatment of Anorectal Malformations.","authors":"Marijke E B Kremer, Ramon R Gorter, Jen Tidman, Jan-Hendrick Gosemann, Ivo de Blaauw","doi":"10.1055/a-2528-5324","DOIUrl":"https://doi.org/10.1055/a-2528-5324","url":null,"abstract":"","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":"35 2","pages":"77-78"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143702304","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-01Epub Date: 2024-06-07DOI: 10.1055/a-2340-9373
Michaela Klinke, Nina Dietze, Tina Trautmann, Marietta Jank, Richard Martel, Julia Elrod, Michael Boettcher
Introduction: Abdominal adhesions following surgery can lead to complications like intestinal obstruction and pelvic pain. While no molecular therapies currently target the underlying adhesion formation process, various barrier agents exist. 4DryField® has shown promise in reducing bleeding and adhesions in adults. This study aimed to assess its effectiveness in children.
Methods: The study examined all pediatric patients who underwent laparotomy between January 2018 and February 2022. It compared outcomes between those treated with 4DryField® and a control group. Key endpoints included surgical revision, adhesion recurrence, infections, insufficiencies, fever, C-reactive protein (CRP) levels, and time to gastrointestinal passage.
Results: In total, 233 children had surgery for bowel adhesions. After propensity score matching, 82 patients were included in the analysis: 39 in the control and 43 in the 4DryField® group. 4DryField® did not affect the readhesion rate. Children in the treatment group had significantly more complications (47 vs. 15%, p = 0.002), more often fever, and higher CRP levels.
Conclusions: 4DryField® did not show potential in reducing adhesion formation, but it was associated with significantly more complications in pediatric patients. Thus, future prospective studies are needed to evaluate the safety and effectiveness of 4DryField® in children.
{"title":"Evaluation of 4DryField® as an Adhesion Prophylaxis in Pediatric Patients: A Propensity-Score Matched Study.","authors":"Michaela Klinke, Nina Dietze, Tina Trautmann, Marietta Jank, Richard Martel, Julia Elrod, Michael Boettcher","doi":"10.1055/a-2340-9373","DOIUrl":"10.1055/a-2340-9373","url":null,"abstract":"<p><strong>Introduction: </strong> Abdominal adhesions following surgery can lead to complications like intestinal obstruction and pelvic pain. While no molecular therapies currently target the underlying adhesion formation process, various barrier agents exist. 4DryField® has shown promise in reducing bleeding and adhesions in adults. This study aimed to assess its effectiveness in children.</p><p><strong>Methods: </strong> The study examined all pediatric patients who underwent laparotomy between January 2018 and February 2022. It compared outcomes between those treated with 4DryField® and a control group. Key endpoints included surgical revision, adhesion recurrence, infections, insufficiencies, fever, C-reactive protein (CRP) levels, and time to gastrointestinal passage.</p><p><strong>Results: </strong> In total, 233 children had surgery for bowel adhesions. After propensity score matching, 82 patients were included in the analysis: 39 in the control and 43 in the 4DryField® group. 4DryField® did not affect the readhesion rate. Children in the treatment group had significantly more complications (47 vs. 15%, <i>p</i> = 0.002), more often fever, and higher CRP levels.</p><p><strong>Conclusions: </strong> 4DryField® did not show potential in reducing adhesion formation, but it was associated with significantly more complications in pediatric patients. Thus, future prospective studies are needed to evaluate the safety and effectiveness of 4DryField® in children.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":" ","pages":"159-164"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141288964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-01Epub Date: 2024-11-08DOI: 10.1055/a-2464-2686
Thomas O Xu, Julia Ann Ryan, Christina Feng, Andrea Badillo, Anthony Sandler, Marc A Levitt
Introduction: The perineal body preserving posterior sagittal anorectoplasty (PSARP) (PPP) is a novel modification of the original PSARP for female patients with rectovestibular fistulas designed to eliminate the risk of perineal body dehiscence. This study aims to examine the outcomes following PPP.
Methods: A retrospective, single-institution study was performed examining female patients with rectovestibular fistula who underwent PPP between January /2020 and December 2023. Exposure was done through the intended anoplasty only. Perineal body or posterior sagittal incision was not utilized. No routine postoperative dilations were performed. The postoperative outcomes, day of discharge, time to first feeding, and early stooling patterns were assessed.
Results: A total of 15 patients underwent a PPP at a median age of 6 months (range 2 days to 19 months) with median follow-up of 11 months (range 1-36). Three (20%) patients underwent repair within the first 3 months of life. Five (33%) had diverting ostomies prior to referral to our team. There was no incidence of dehiscence or rectal prolapse. Two (13%) patients developed an anal stricture which required revision. Fourteen (93%) patients resumed normal feeds on postoperative day 1. Eleven (73%) were discharged on postoperative day 1. All patients were stooling spontaneously at their most recent clinical encounter with 11 (73%) utilizing laxatives.
Conclusions: PPP eliminates the risk of perineal body dehiscence and has a quick return to regular diet and home. There is a 13% stricture rate which could relate to a difference in the distal rectal mobilization compared with the traditional PSARP.
{"title":"The PPP - Perineal Body Preserving PSARP (Posterior Sagittal Anorectoplasty) for Anorectal Malformation with Rectovestibular Fistula in Females-Report of Early Outcomes.","authors":"Thomas O Xu, Julia Ann Ryan, Christina Feng, Andrea Badillo, Anthony Sandler, Marc A Levitt","doi":"10.1055/a-2464-2686","DOIUrl":"10.1055/a-2464-2686","url":null,"abstract":"<p><strong>Introduction: </strong> The perineal body preserving posterior sagittal anorectoplasty (PSARP) (PPP) is a novel modification of the original PSARP for female patients with rectovestibular fistulas designed to eliminate the risk of perineal body dehiscence. This study aims to examine the outcomes following PPP.</p><p><strong>Methods: </strong> A retrospective, single-institution study was performed examining female patients with rectovestibular fistula who underwent PPP between January /2020 and December 2023. Exposure was done through the intended anoplasty only. Perineal body or posterior sagittal incision was not utilized. No routine postoperative dilations were performed. The postoperative outcomes, day of discharge, time to first feeding, and early stooling patterns were assessed.</p><p><strong>Results: </strong> A total of 15 patients underwent a PPP at a median age of 6 months (range 2 days to 19 months) with median follow-up of 11 months (range 1-36). Three (20%) patients underwent repair within the first 3 months of life. Five (33%) had diverting ostomies prior to referral to our team. There was no incidence of dehiscence or rectal prolapse. Two (13%) patients developed an anal stricture which required revision. Fourteen (93%) patients resumed normal feeds on postoperative day 1. Eleven (73%) were discharged on postoperative day 1. All patients were stooling spontaneously at their most recent clinical encounter with 11 (73%) utilizing laxatives.</p><p><strong>Conclusions: </strong> PPP eliminates the risk of perineal body dehiscence and has a quick return to regular diet and home. There is a 13% stricture rate which could relate to a difference in the distal rectal mobilization compared with the traditional PSARP.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":" ","pages":"135-140"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142633333","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-01Epub Date: 2024-10-11DOI: 10.1055/s-0044-1791250
Ophelia Aubert, Willemijn F E Irvine, Dalia Aminoff, Ivo de Blaauw, Salvatore Cascio, Célia Cretolle, Barbara Daniela Iacobelli, Konstantinos Mantzios, Paola Midrio, Marc Miserez, Sabine Sarnacki, Eberhard Schmiedeke, Nicole Schwarzer, Cornelius Sloots, Pernilla Stenström, Martin Lacher, Jan-Hendrik Gosemann
Introduction: Anorectal malformations (ARMs) are rare congenital anomalies that involve the anus, rectum, and oftentimes the genitourinary tract. The management of ARM patients is complex, and many controversies exist. To address this issue, the European Reference Network eUROGEN for rare and complex urogenital conditions aimed to develop comprehensive guidelines for the management of ARM.
Methods: The Dutch Quality Standard for ARM served as the basis for the development of guidelines applicable on a European level. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from 7 European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected, and recommendations were formed considering current evidence and/or expert consensus.
Results: Prenatal and neonatal diagnostic workup as well as postsurgical follow-up of anorectal, genitourinary tract, and neurologic system were reviewed. Seven new studies were identified. The panel adapted 13 recommendations, adopted 7, and developed 8 de novo. The availability of high-quality evidence was limited, and most recommendations were based on retrospective studies, case series, or expert opinion.
Conclusion: Patients with ARM and their families require highly specialized and comprehensive care from the prenatal period to adulthood. This guideline provides recommendations for a comprehensive diagnostic workup of children with ARM throughout their life that is applicable on a European level.
{"title":"ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part I: Diagnostics.","authors":"Ophelia Aubert, Willemijn F E Irvine, Dalia Aminoff, Ivo de Blaauw, Salvatore Cascio, Célia Cretolle, Barbara Daniela Iacobelli, Konstantinos Mantzios, Paola Midrio, Marc Miserez, Sabine Sarnacki, Eberhard Schmiedeke, Nicole Schwarzer, Cornelius Sloots, Pernilla Stenström, Martin Lacher, Jan-Hendrik Gosemann","doi":"10.1055/s-0044-1791250","DOIUrl":"10.1055/s-0044-1791250","url":null,"abstract":"<p><strong>Introduction: </strong> Anorectal malformations (ARMs) are rare congenital anomalies that involve the anus, rectum, and oftentimes the genitourinary tract. The management of ARM patients is complex, and many controversies exist. To address this issue, the European Reference Network eUROGEN for rare and complex urogenital conditions aimed to develop comprehensive guidelines for the management of ARM.</p><p><strong>Methods: </strong> The Dutch Quality Standard for ARM served as the basis for the development of guidelines applicable on a European level. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from 7 European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected, and recommendations were formed considering current evidence and/or expert consensus.</p><p><strong>Results: </strong> Prenatal and neonatal diagnostic workup as well as postsurgical follow-up of anorectal, genitourinary tract, and neurologic system were reviewed. Seven new studies were identified. The panel adapted 13 recommendations, adopted 7, and developed 8 de novo. The availability of high-quality evidence was limited, and most recommendations were based on retrospective studies, case series, or expert opinion.</p><p><strong>Conclusion: </strong> Patients with ARM and their families require highly specialized and comprehensive care from the prenatal period to adulthood. This guideline provides recommendations for a comprehensive diagnostic workup of children with ARM throughout their life that is applicable on a European level.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":" ","pages":"104-111"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142407294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-01Epub Date: 2024-09-19DOI: 10.1055/s-0044-1791257
Ophelia Aubert, Willemijn F E Irvine, Dalia Aminoff, Ivo de Blaauw, Salvatore Cascio, Célia Cretolle, Barbara Daniela Iacobelli, Konstantinos Mantzios, Paola Midrio, Marc Miserez, Sabine Sarnacki, Eberhard Schmiedeke, Nicole Schwarzer, Cornelius Sloots, Pernilla Stenström, Martin Lacher, Jan-Hendrik Gosemann
Introduction: Anorectal malformations (ARMs) are rare birth defects affecting the anorectum and oftentimes the genitourinary region. The management of ARM patients is complex and requires highly specialized surgical and medical care. The European Reference Network eUROGEN for rare and complex urogenital conditions aimed to develop comprehensive guidelines for the management of ARM applicable on a European level.
Methods: The Dutch Quality Standard for ARM served as the basis for the development of guidelines. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from seven European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected and recommendations were formed considering the current evidence, expert opinion, and the European context.
Results: Surgical and medical treatment of ARM, postoperative instructions, toilet training, and management of fecal and urinary incontinence were addressed. Seven new studies were identified. The panel adapted 23 recommendations, adopted 3, and developed 8 de novo. The overall level of newly found evidence was considered low.
Conclusion: Treatment of ARM patients requires a multidisciplinary team and expertise about anatomical and surgical aspects of the disease, as well as long-term follow-up. This guideline offers recommendations for surgical and medical treatment of ARM and associated complications, according to the best available evidence and applicable on a European level.
导言:肛门直肠畸形(ARM)是一种罕见的先天性缺陷,会影响肛门直肠,有时还会影响泌尿生殖系统。肛门直肠畸形患者的治疗非常复杂,需要高度专业化的手术和医疗护理。欧洲罕见复杂泌尿生殖系统疾病参考网络 eUROGEN 的目标是制定适用于欧洲水平的 ARM 综合管理指南:方法:荷兰 ARM 质量标准是制定指南的基础。在 Medline、Embase 和 Cochrane 中进行了文献检索。采用 ADAPTE 方法纳入最新的可用证据。一个由来自七个欧洲国家的 15 位专家组成的小组对建议的时效性、可接受性和适用性进行了评估。对荷兰质量标准中的建议进行了调整、采纳或否决,并在考虑当前证据、专家意见和欧洲背景的基础上形成了建议:结果:研究涉及 ARM 的手术和药物治疗、术后指导、如厕训练以及大小便失禁的处理。其中发现了 7 项新研究。专家小组调整了 23 项建议,采纳了 3 项建议,并重新制定了 8 项建议。新发现的证据总体水平较低:ARM患者的治疗需要多学科团队、疾病解剖和手术方面的专业知识以及长期随访。本指南根据现有的最佳证据,为 ARM 及相关并发症的手术和药物治疗提供了建议,适用于欧洲范围。
{"title":"European Reference Network eUROGEN Guidelines on the Management of Anorectal Malformations, Part II: Treatment.","authors":"Ophelia Aubert, Willemijn F E Irvine, Dalia Aminoff, Ivo de Blaauw, Salvatore Cascio, Célia Cretolle, Barbara Daniela Iacobelli, Konstantinos Mantzios, Paola Midrio, Marc Miserez, Sabine Sarnacki, Eberhard Schmiedeke, Nicole Schwarzer, Cornelius Sloots, Pernilla Stenström, Martin Lacher, Jan-Hendrik Gosemann","doi":"10.1055/s-0044-1791257","DOIUrl":"10.1055/s-0044-1791257","url":null,"abstract":"<p><strong>Introduction: </strong> Anorectal malformations (ARMs) are rare birth defects affecting the anorectum and oftentimes the genitourinary region. The management of ARM patients is complex and requires highly specialized surgical and medical care. The European Reference Network eUROGEN for rare and complex urogenital conditions aimed to develop comprehensive guidelines for the management of ARM applicable on a European level.</p><p><strong>Methods: </strong> The Dutch Quality Standard for ARM served as the basis for the development of guidelines. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from seven European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected and recommendations were formed considering the current evidence, expert opinion, and the European context.</p><p><strong>Results: </strong> Surgical and medical treatment of ARM, postoperative instructions, toilet training, and management of fecal and urinary incontinence were addressed. Seven new studies were identified. The panel adapted 23 recommendations, adopted 3, and developed 8 de novo. The overall level of newly found evidence was considered low.</p><p><strong>Conclusion: </strong> Treatment of ARM patients requires a multidisciplinary team and expertise about anatomical and surgical aspects of the disease, as well as long-term follow-up. This guideline offers recommendations for surgical and medical treatment of ARM and associated complications, according to the best available evidence and applicable on a European level.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":" ","pages":"112-119"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142302089","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-01Epub Date: 2024-09-19DOI: 10.1055/s-0044-1791249
Ophelia Aubert, Willemijn F E Irvine, Dalia Aminoff, Ivo de Blaauw, Salvatore Cascio, Célia Cretolle, Barbara Daniela Iacobelli, Martin Lacher, Konstantinos Mantzios, Marc Miserez, Sabine Sarnacki, Eberhard Schmiedeke, Nicole Schwarzer, Cornelius Sloots, Pernilla Stenström, Paola Midrio, Jan-Hendrik Gosemann
Introduction: Anorectal malformations (ARMs) are complex congenital anomalies of the anorectal region, oftentimes also affecting the genitourinary system. Although successful surgical correction can often be achieved in the neonatal period, many children will experience functional problems in the long term. The European Reference Network for rare and complex urogenital conditions (eUROGEN) assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM.
Methods: The Dutch Quality Standard for ARM served as the foundation for the development of guidelines applicable on a European level. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from 7 European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected and recommendations were formed considering the current evidence and/or expert consensus.
Results: Lifelong follow-up, integration, and transition of care were assessed. A total of eight new studies were identified. The panel adapted 18 recommendations, adopted 6, and developed 6 de novo. Overall, the level of evidence was considered low.
Conclusion: Successful lifelong follow-up and transition of care require a dedicated team of pediatric and adult specialist and an individually tailored patient-centered approach. This guideline summarizes the best available evidence on follow-up of ARM patients and provides guidance for the development of structured transition programs.
导言:肛门直肠畸形(ARM)是肛门直肠部位复杂的先天性畸形,有时还会影响泌尿生殖系统。虽然在新生儿期通常可以通过手术成功矫正,但许多患儿长期会出现功能问题。欧洲罕见复杂泌尿生殖系统疾病参考网络(eUROGEN)组建了一个专家小组,以应对这些挑战,并制定全面的 ARM 管理指南:方法:荷兰 ARM 质量标准是制定适用于欧洲的指南的基础。在 Medline、Embase 和 Cochrane 中进行了文献检索。利用 ADAPTE 方法纳入了最新的可用证据。一个由来自 7 个欧洲国家的 15 位专家组成的小组对建议的时效性、可接受性和适用性进行了评估。对荷兰质量标准中的建议进行了调整、采纳或否决,并根据现有证据和/或专家共识形成了建议:结果:对终生随访、整合和护理过渡进行了评估。共确定了 8 项新研究。专家小组调整了 18 项建议,采纳了 6 项建议,并重新制定了 6 项建议。总体而言,证据水平较低:成功的终身随访和护理过渡需要一个由儿科和成人专科医生组成的专业团队,以及以患者为中心的个性化定制方法。本指南总结了有关 ARM 患者随访的现有最佳证据,并为制定结构化过渡计划提供了指导。
{"title":"ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part III: Lifelong Follow-up and Transition of Care.","authors":"Ophelia Aubert, Willemijn F E Irvine, Dalia Aminoff, Ivo de Blaauw, Salvatore Cascio, Célia Cretolle, Barbara Daniela Iacobelli, Martin Lacher, Konstantinos Mantzios, Marc Miserez, Sabine Sarnacki, Eberhard Schmiedeke, Nicole Schwarzer, Cornelius Sloots, Pernilla Stenström, Paola Midrio, Jan-Hendrik Gosemann","doi":"10.1055/s-0044-1791249","DOIUrl":"10.1055/s-0044-1791249","url":null,"abstract":"<p><strong>Introduction: </strong> Anorectal malformations (ARMs) are complex congenital anomalies of the anorectal region, oftentimes also affecting the genitourinary system. Although successful surgical correction can often be achieved in the neonatal period, many children will experience functional problems in the long term. The European Reference Network for rare and complex urogenital conditions (eUROGEN) assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM.</p><p><strong>Methods: </strong> The Dutch Quality Standard for ARM served as the foundation for the development of guidelines applicable on a European level. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from 7 European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected and recommendations were formed considering the current evidence and/or expert consensus.</p><p><strong>Results: </strong> Lifelong follow-up, integration, and transition of care were assessed. A total of eight new studies were identified. The panel adapted 18 recommendations, adopted 6, and developed 6 de novo. Overall, the level of evidence was considered low.</p><p><strong>Conclusion: </strong> Successful lifelong follow-up and transition of care require a dedicated team of pediatric and adult specialist and an individually tailored patient-centered approach. This guideline summarizes the best available evidence on follow-up of ARM patients and provides guidance for the development of structured transition programs.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":" ","pages":"120-127"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142302088","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}