Introduction: The Dynamic Imaging Grade of Swallowing Toxicity (DIGEST) scale was developed to evaluate the safety, efficiency, and overall pharyngeal swallowing performance in patients with dysphagia (DIGESTs, DIGESTe, and DIGESTt, respectively). Although various types of swallowing dysfunction are encountered in children with esophageal atresia (EA), oropharyngeal dysphagia poses risk for aspiration. Therefore, a retrospective study was performed to evaluate the safety and efficacy of swallowing by using DIGEST score in children with EA.
Patients and methods: Thirty-nine EA patients were included. The demographic features, respiratory problems, results, and outcomes of surgical treatment were evaluated from medical records. The videofluoroscopic swallowing evaluation investigated for both airway protection and bolus residuals at the level of vallecula, posterior pharyngeal wall, and pyriform sinus at liquid and pudding consistencies. The penetration and aspiration scale (PAS) was used to define penetration and aspiration severity, and DIGEST was used to evaluate DIGESTs, DIGESTe, and DIGESTt.
Results: The median age of the patients were 13 months (7-39 months), and male-to-female ratio was 25:14. Sixty-seven percent of patients were type-C EA and 61% of them has associated anomalies; 38% of patients had aspiration (PAS = 6-8) in liquids and 10% in pudding consistency. Life-threatening/profound swallowing dysfunction in DIGESTe (DIGEST = 4) was seen in 13% (n = 5) of patients; 40% of EA patients showed severe problems in DIGESTt.
Conclusion: DIGEST is a valid and reliable tool to define the efficacy and safety of swallowing in children with EA.
{"title":"Dynamic Imaging Grade of Swallowing Toxicity in Children with Esophageal Atresia.","authors":"Numan Demir, Begüm Pişiren, Selen Serel Arslan, Ozlem Boybeyı-Turer, Tutku Soyer","doi":"10.1055/a-2181-2293","DOIUrl":"10.1055/a-2181-2293","url":null,"abstract":"<p><strong>Introduction: </strong> The Dynamic Imaging Grade of Swallowing Toxicity (DIGEST) scale was developed to evaluate the safety, efficiency, and overall pharyngeal swallowing performance in patients with dysphagia (DIGESTs, DIGESTe, and DIGESTt, respectively). Although various types of swallowing dysfunction are encountered in children with esophageal atresia (EA), oropharyngeal dysphagia poses risk for aspiration. Therefore, a retrospective study was performed to evaluate the safety and efficacy of swallowing by using DIGEST score in children with EA.</p><p><strong>Patients and methods: </strong> Thirty-nine EA patients were included. The demographic features, respiratory problems, results, and outcomes of surgical treatment were evaluated from medical records. The videofluoroscopic swallowing evaluation investigated for both airway protection and bolus residuals at the level of vallecula, posterior pharyngeal wall, and pyriform sinus at liquid and pudding consistencies. The penetration and aspiration scale (PAS) was used to define penetration and aspiration severity, and DIGEST was used to evaluate DIGESTs, DIGESTe, and DIGESTt.</p><p><strong>Results: </strong> The median age of the patients were 13 months (7-39 months), and male-to-female ratio was 25:14. Sixty-seven percent of patients were type-C EA and 61% of them has associated anomalies; 38% of patients had aspiration (PAS = 6-8) in liquids and 10% in pudding consistency. Life-threatening/profound swallowing dysfunction in DIGESTe (DIGEST = 4) was seen in 13% (<i>n</i> = 5) of patients; 40% of EA patients showed severe problems in DIGESTt.</p><p><strong>Conclusion: </strong> DIGEST is a valid and reliable tool to define the efficacy and safety of swallowing in children with EA.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41162500","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}
Luisa Maria Frankenbach, Anne-Sophie Holler, Christina Oetzmann von Sochaczewski, Lucas Wessel, Oliver J Muensterer, Jens Dingemann, Anke Widenmann, Paul Helm, Jannos Siaplaouras, Ulrike Bauer, Christian Apitz, Claudia Niessner, Tatjana Tamara König
Aim of the study: Esophageal atresia (EA) is associated with impaired motor development, cardiopulmonary function and physical activity (PA). Despite missing scientific evidence, this fact is often attributed to associated congenital heart disease (CHD). The aim of this study is to investigate PA in EA patients without CHD compared to CHD patients and healthy controls.
Methods: In this multicenter study, EA patients aged 6 through 17 years were included. Moderate-to-vigorous physical activity (MVPA, minutes per week) was assessed using the standardized and validated questionnaire MoMo-PAQ. EA patients were randomly matched 1:4 for gender and age with patients with CHD (n=1262) and healthy controls (n=6233). Patients born with both EA and CHD were excluded. Means and 95%-confidence intervals (95%-CI) were calculated. To identify associated factors, Spearman correlation was performed.
Main results: Overall, 69 EA patients were matched with 276 CHD patients and 276 controls (57% male, 43% female, mean age 10,3 years, 95%-CI: 9,5-11,1). Mean MVPA was reduced in EA (492 minutes, 95%-CI: 387-598) and CHD patients (463 minutes, 95%-CI: 416-511) compared to controls (613 minutes, 95%-CI: 548-679). In subgroup analysis, MVPA was reduced further in females and older age groups with EA and CHD. For EA patients, there was no statistical association between Gross type, current symptoms, surgical approaches and MVPA.
Conclusions: Isolated EA and CHD were associated with reduced PA, especially in females and teenagers. To avoid additional morbidity associated with sedentary behaviour, PA should be promoted during follow-up.
研究目的食道闭锁(EA)与运动发育、心肺功能和体力活动(PA)受损有关。尽管缺乏科学证据,但这一事实往往被归因于相关的先天性心脏病(CHD)。本研究旨在调查无先天性心脏病的 EA 患者与先天性心脏病患者和健康对照组的体力活动情况:在这项多中心研究中,纳入了 6 至 17 岁的 EA 患者。采用标准化的有效问卷 MoMo-PAQ 评估中度至高强度体力活动(MVPA,每周分钟数)。EA患者与先天性心脏病患者(1262 人)和健康对照组(6233 人)按性别和年龄 1:4 随机配对。同时患有EA和先天性心脏病的患者被排除在外。计算了平均值和 95% 置信区间 (95%-CI)。为确定相关因素,进行了斯皮尔曼相关分析:总体而言,69 名 EA 患者与 276 名 CHD 患者和 276 名对照组(57% 为男性,43% 为女性,平均年龄为 10.3 岁,95%-CI:9.5-11.1 岁)进行了配对。与对照组(613 分钟,95%-CI:548-679)相比,EA(492 分钟,95%-CI:387-598)和 CHD 患者(463 分钟,95%-CI:416-511)的平均 MVPA 有所减少。在亚组分析中,女性和年龄较大的 EA 和心脏病患者的 MVPA 进一步减少。对于EA患者,毛发类型、当前症状、手术方法与MVPA之间没有统计学关联:结论:孤立性 EA 和心脏病与运动量减少有关,尤其是女性和青少年。为避免久坐导致额外的发病率,应在随访期间促进运动量。
{"title":"Physical Activity Levels in Children with Esophageal Atresia and Congenital Heart Disease: A Comparative Multicenter Study.","authors":"Luisa Maria Frankenbach, Anne-Sophie Holler, Christina Oetzmann von Sochaczewski, Lucas Wessel, Oliver J Muensterer, Jens Dingemann, Anke Widenmann, Paul Helm, Jannos Siaplaouras, Ulrike Bauer, Christian Apitz, Claudia Niessner, Tatjana Tamara König","doi":"10.1055/a-2420-0202","DOIUrl":"https://doi.org/10.1055/a-2420-0202","url":null,"abstract":"<p><strong>Aim of the study: </strong>Esophageal atresia (EA) is associated with impaired motor development, cardiopulmonary function and physical activity (PA). Despite missing scientific evidence, this fact is often attributed to associated congenital heart disease (CHD). The aim of this study is to investigate PA in EA patients without CHD compared to CHD patients and healthy controls.</p><p><strong>Methods: </strong>In this multicenter study, EA patients aged 6 through 17 years were included. Moderate-to-vigorous physical activity (MVPA, minutes per week) was assessed using the standardized and validated questionnaire MoMo-PAQ. EA patients were randomly matched 1:4 for gender and age with patients with CHD (n=1262) and healthy controls (n=6233). Patients born with both EA and CHD were excluded. Means and 95%-confidence intervals (95%-CI) were calculated. To identify associated factors, Spearman correlation was performed.</p><p><strong>Main results: </strong>Overall, 69 EA patients were matched with 276 CHD patients and 276 controls (57% male, 43% female, mean age 10,3 years, 95%-CI: 9,5-11,1). Mean MVPA was reduced in EA (492 minutes, 95%-CI: 387-598) and CHD patients (463 minutes, 95%-CI: 416-511) compared to controls (613 minutes, 95%-CI: 548-679). In subgroup analysis, MVPA was reduced further in females and older age groups with EA and CHD. For EA patients, there was no statistical association between Gross type, current symptoms, surgical approaches and MVPA.</p><p><strong>Conclusions: </strong>Isolated EA and CHD were associated with reduced PA, especially in females and teenagers. To avoid additional morbidity associated with sedentary behaviour, PA should be promoted during follow-up.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142309220","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, 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":"https://doi.org/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":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-19","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}
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":"https://doi.org/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":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-19","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}