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Anorectal Malformations Associated with Hirschsprung Disease: Insights from a Large Cohort of 2,341 ARM Patients in a Single-Center Retrospective Study. 与赫氏病相关的肛门直肠畸形:单中心回顾性研究中 2341 名 ARM 患者组成的大型队列的启示。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-07 DOI: 10.1055/a-2557-8073
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.

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引用次数: 0
Publishing Trends, Motivations, and Obstacles Among Pediatric Surgeons: An International Survey on Research Dissemination and Peer Review Challenges.
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-04 DOI: 10.1055/a-2544-9739
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.

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引用次数: 0
Solving Complex Pediatric Surgical Case Studies: A Comparative Analysis of Copilot, ChatGPT-4, and Experienced Pediatric Surgeons' Performance.
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-02 DOI: 10.1055/a-2551-2131
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}
引用次数: 0
Anorectal Malformation with Rectoperineal Fistula in Males Treated with a Posterior Rectal Advancement Anoplasty: Report of Early Outcomes. 直肠直肠畸形伴直肠会阴瘘的男性经直肠后进肛门成形术(PRAA)治疗——早期结果报告。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-01 Epub Date: 2025-01-13 DOI: 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.

Level of evidence:  Level III.

目的:对切除手术的一种新颖的改进,对有肛门直肠畸形和直肠会阴瘘的男性患者进行后直肠推进肛门成形术(PRAA),该手术仅在括约肌椭圆的范围内切开,消除了直肠前夹层,从而避免了尿道损伤的任何可能性。本报告提供了PRAA术后较长期的结果。方法回顾性分析2020年1月- 2023年12月男性直肠会阴瘘患者的单机构研究。只有当直肠会阴瘘管位于括约肌椭圆的前段时才进行PRAA,在本研究期间遇到的所有患者都是如此。我们评估了术后结果、住院时间、首次进食时间和早期大便模式。结果18例患者接受PRAA治疗,中位年龄5.4个月(1 ~ 8个月),中位随访14个月(4 ~ 40个月)。7例患者(39%)在出生后1个月内修复。13例(72%)进行了初步修复,5例(28%)在转诊前进行了转移造口术。没有伤口裂开、直肠脱垂或尿道损伤的病例。2例(11%)患者出现肛门狭窄,需要扩张或Heineke-Mikulicz肛门成形术。所有患者在术后第一天恢复进食。中位出院时间为术后第1天(范围1-6)。15人(83%)在最近的随访中使用泻药。结论PRAA可避免尿道损伤或会阴开裂,肛门狭窄发生率低(11%),适用于所有男性直肠会阴瘘患者。该技术允许早期恢复饮食和出院,可以安全地在新生儿期或以延迟的方式进行,而不需要造口。
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引用次数: 0
Development of European Consensus Guidelines and Evolution of Innovative Surgical Strategies for the Treatment of Anorectal Malformations.
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-01 Epub Date: 2025-03-24 DOI: 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}
引用次数: 0
Evaluation of 4DryField® as an Adhesion Prophylaxis in Pediatric Patients: A Propensity-Score Matched Study. 评估 4DryField® 作为儿科患者的粘连预防疗法:倾向分数匹配研究。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-01 Epub Date: 2024-06-07 DOI: 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.

简介手术后腹腔粘连可导致肠梗阻和盆腔疼痛等并发症。虽然目前还没有针对粘连形成过程的分子疗法,但存在各种屏障剂。4DryField® 已显示出减少成人出血和粘连的前景。本研究旨在评估其对儿童的有效性:本研究对 2018 年 1 月至 2022 年 2 月期间接受开腹手术的所有儿童患者进行了检查。研究比较了接受 4DryField® 治疗的患者和对照组的治疗效果。关键终点包括手术翻修、粘连复发、感染、不足、发热、CRP水平和胃肠道通过时间:共有 233 名儿童接受了肠粘连手术。经过倾向评分匹配后,82 名患者被纳入分析范围:对照组 39 人,4DryField® 组 43 人。4DryField® 并未影响再粘连率。治疗组患儿的并发症明显增多(47% 对 15%,P=0.002),发烧更频繁,CRP 水平更高:结论:4DryField® 在减少粘连形成方面没有显示出潜力,但在儿童患者中与明显更多的并发症有关。因此,未来的前瞻性研究将评估 4DryField® 在儿童中的安全性和有效性。
{"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}
引用次数: 0
The PPP - Perineal Body Preserving PSARP (Posterior Sagittal Anorectoplasty) for Anorectal Malformation with Rectovestibular Fistula in Females-Report of Early Outcomes. 针对女性肛门直肠畸形伴直肠前庭瘘的会阴体保留整形术(PPP)--早期疗效报告。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-01 Epub Date: 2024-11-08 DOI: 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.

导言:会阴体保留 PSARP(后矢状肛门成形术)(PPP)是针对女性直肠前庭瘘患者的原始 PSARP 的新型改良术式,旨在消除会阴体开裂的风险。本研究旨在探讨 PPP 术后的效果。方法 对 2020 年 1 月 1 日至 2023 年 12 月 12 日期间接受 PPP 的直肠前庭瘘女性患者进行了一项回顾性单机构研究。仅通过预定的肛门成形术进行暴露,未使用会阴体或后矢状切口。术后未进行常规扩张。对术后效果、出院日、首次进食时间和早期排便模式进行了评估。结果 15 名患者接受了 PPP 手术,中位年龄为 6 个月(2 天-19 个月),中位随访时间为 11 个月(1-36 个月)。三名患者(20%)在出生后三个月内接受了修复手术。五名患者(33%)在转诊至我们的团队之前已经进行了分流造口。没有发生开裂或直肠脱垂。两名(13%)患者出现肛门狭窄,需要进行修补。14名患者(93%)在术后第一天就恢复了正常进食。11名患者(73%)在术后第一天出院。所有患者在最近一次临床就诊时都能自行排便,只有 11 名患者(73%)使用了泻药。结论 PPP 消除了会阴体开裂的风险,并能迅速恢复正常饮食和居家生活。狭窄率为 13%,这可能与直肠远端移动与传统 PSARP 相比有所不同有关。
{"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}
引用次数: 0
ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part I: Diagnostics. ERN eUROGEN《肛门直肠畸形管理指南》第一部分:诊断。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-01 Epub Date: 2024-10-11 DOI: 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.

导言:肛门直肠畸形(ARM)是一种罕见的先天性畸形,涉及肛门、直肠,有时还包括泌尿生殖道。肛门直肠畸形患者的治疗非常复杂,存在许多争议。为解决这一问题,欧洲罕见复杂泌尿生殖系统疾病参考网络 eUROGEN 旨在为 ARM 的治疗制定全面的指南:方法:以荷兰 ARM 质量标准为基础,制定适用于欧洲的指南。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,并根据当前证据和/或专家共识形成了建议:结果:对产前和新生儿诊断工作以及肛门直肠、泌尿生殖道和神经系统的术后随访进行了回顾。发现了 7 项新研究。专家小组调整了 13 项建议,采纳了 7 项建议,并重新制定了 8 项建议。高质量证据有限,大多数建议都是基于回顾性研究、系列病例或专家意见:结论:ARM 患者及其家庭需要从产前到成年的高度专业化和全面的护理。本指南为ARM患儿一生中的全面诊断工作提供了适用于欧洲水平的建议。
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引用次数: 0
European Reference Network eUROGEN Guidelines on the Management of Anorectal Malformations, Part II: Treatment. 欧洲肛门直肠畸形管理参考网络 eUROGEN 指南,第二部分:治疗。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-01 Epub Date: 2024-09-19 DOI: 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。
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引用次数: 0
ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part III: Lifelong Follow-up and Transition of Care. ERN eUROGEN《肛门直肠畸形管理指南》第三部分:终身随访和护理过渡。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2025-04-01 Epub Date: 2024-09-19 DOI: 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。
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引用次数: 0
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European Journal of Pediatric Surgery
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