Pub Date : 2024-12-01DOI: 10.1016/j.sempedsurg.2025.151475
Isabelle Lane Curran, Robert A. Cowles
Biliary atresia can be a challenging diagnosis to make as there is no single definitive diagnostic laboratory or imaging study available and no single agreed upon diagnostic algorithm. The purpose of this article is to review the complex puzzle of clinical, laboratory, and imaging studies that aid in the evaluation of infants suspected of having biliary atresia. We have reviewed historical and current manuscripts and society guidelines, added our own experience in evaluating infants for biliary atresia, and then summarized the findings to provide a concise review of what we feel is the modern approach to diagnosis of biliary atresia.
{"title":"Preoperative evaluation of biliary atresia","authors":"Isabelle Lane Curran, Robert A. Cowles","doi":"10.1016/j.sempedsurg.2025.151475","DOIUrl":"10.1016/j.sempedsurg.2025.151475","url":null,"abstract":"<div><div>Biliary atresia can be a challenging diagnosis to make as there is no single definitive diagnostic laboratory or imaging study available and no single agreed upon diagnostic algorithm. The purpose of this article is to review the complex puzzle of clinical, laboratory, and imaging studies that aid in the evaluation of infants suspected of having biliary atresia. We have reviewed historical and current manuscripts and society guidelines, added our own experience in evaluating infants for biliary atresia, and then summarized the findings to provide a concise review of what we feel is the modern approach to diagnosis of biliary atresia.</div></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"Article 151475"},"PeriodicalIF":1.4,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143076232","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 : 2024-12-01DOI: 10.1016/j.sempedsurg.2025.151474
Phoebe N Miller , Suruthi Baskaran , Amar Nijagal
Biliary atresia is a progressive neonatal cholangiopathy that leads to liver failure. Characterized by inflammation-mediated liver injury, the immune system plays a critical role in the pathogenesis of this disease. Though several types of immune cells and mediators have been implicated in animal models of biliary atresia, emerging literature reflects the complex interplay of components of the immune response that contributes to disease progression in humans. Novel therapies targeting the immune system are needed to mitigate the devastating effects of biliary atresia. This review highlights the current literature on the components of the immune system that have been in implicated in biliary atresia and the rich interplay between the major arms of the immune system- innate and adaptive immunity- to cause the highly morbid consequences of this disease.
{"title":"Immunology of Biliary Atresia","authors":"Phoebe N Miller , Suruthi Baskaran , Amar Nijagal","doi":"10.1016/j.sempedsurg.2025.151474","DOIUrl":"10.1016/j.sempedsurg.2025.151474","url":null,"abstract":"<div><div>Biliary atresia is a progressive neonatal cholangiopathy that leads to liver failure. Characterized by inflammation-mediated liver injury, the immune system plays a critical role in the pathogenesis of this disease. Though several types of immune cells and mediators have been implicated in animal models of biliary atresia, emerging literature reflects the complex interplay of components of the immune response that contributes to disease progression in humans. Novel therapies targeting the immune system are needed to mitigate the devastating effects of biliary atresia. This review highlights the current literature on the components of the immune system that have been in implicated in biliary atresia and the rich interplay between the major arms of the immune system- innate and adaptive immunity- to cause the highly morbid consequences of this disease.</div></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"Article 151474"},"PeriodicalIF":1.4,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143043071","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-01DOI: 10.1016/j.sempedsurg.2025.151471
Ana M. Calinescu , Barbara E. Wildhaber
Cholangitis, defined as the inflammation of the bile ducts, is the most frequent complication after Kasai hepatoportoenterostomy in patients with biliary atresia (BA). This review seeks to provide a comprehensive synthesis of current knowledge on diagnosing and managing BA-associated cholangitis while identifying gaps in the existing literature. A scoping literature review was conducted to gather global insights into the definition, evaluation, and management of post-Kasai cholangitis, illustrated through data from the Swiss Biliary Atresia Registry (SBAR).
In the literature many different decision algorithms exist for BA-associated cholangitis underlining the clear need for standardizing diagnostic criteria. As an illustration, in 2015 the Swiss center introduced structured guidelines to diagnose BA-associated cholangitis. Upon retrospective analysis of SBAR data (2010–2022) we noted significant differences in disease management before and after implementation: The number of multiple diagnosed cholangitis episodes was reduced and we observed a reduced transplantation rate. Building on this, the Biliary Atresia and Related Diseases consensus has developed key diagnostic and management guidelines with objective criteria that have yet to undergo prospective validation. Indeed, prospective multicenter assessment is clearly needed to enhance acceptance and improve outcomes. Further, rigorous evaluation of prophylactic and therapeutic interventions, including antibiotics and immunomodulatory treatments, seems essential, with randomized trials required to establish their effectiveness and explore microbiota modulation in cholangitis management.
The BA-community should strive to establish and adopt a widely accepted protocol for the diagnosis, management and prevention of BA-associated cholangitis. The implementation of such protocols is expected to significantly improve clinical outcomes for these patients.
{"title":"Post-Kasai cholangitis evaluation and management strategies: Review of the literature with insights from the Swiss Biliary Atresia Registry","authors":"Ana M. Calinescu , Barbara E. Wildhaber","doi":"10.1016/j.sempedsurg.2025.151471","DOIUrl":"10.1016/j.sempedsurg.2025.151471","url":null,"abstract":"<div><div>Cholangitis, defined as the inflammation of the bile ducts, is the most frequent complication after Kasai hepatoportoenterostomy in patients with biliary atresia (BA). This review seeks to provide a comprehensive synthesis of current knowledge on diagnosing and managing BA-associated cholangitis while identifying gaps in the existing literature. A scoping literature review was conducted to gather global insights into the definition, evaluation, and management of post-Kasai cholangitis, illustrated through data from the Swiss Biliary Atresia Registry (SBAR).</div><div>In the literature many different decision algorithms exist for BA-associated cholangitis underlining the clear need for standardizing diagnostic criteria. As an illustration, in 2015 the Swiss center introduced structured guidelines to diagnose BA-associated cholangitis. Upon retrospective analysis of SBAR data (2010–2022) we noted significant differences in disease management before and after implementation: The number of multiple diagnosed cholangitis episodes was reduced and we observed a reduced transplantation rate. Building on this, the Biliary Atresia and Related Diseases consensus has developed key diagnostic and management guidelines with objective criteria that have yet to undergo prospective validation. Indeed, prospective multicenter assessment is clearly needed to enhance acceptance and improve outcomes. Further, rigorous evaluation of prophylactic and therapeutic interventions, including antibiotics and immunomodulatory treatments, seems essential, with randomized trials required to establish their effectiveness and explore microbiota modulation in cholangitis management.</div><div>The BA-community should strive to establish and adopt a widely accepted protocol for the diagnosis, management and prevention of BA-associated cholangitis. The implementation of such protocols is expected to significantly improve clinical outcomes for these patients.</div></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"Article 151471"},"PeriodicalIF":1.4,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143043072","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-01DOI: 10.1053/S1055-8586(25)00016-2
{"title":"fm i -- Contents","authors":"","doi":"10.1053/S1055-8586(25)00016-2","DOIUrl":"10.1053/S1055-8586(25)00016-2","url":null,"abstract":"","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"Article 151485"},"PeriodicalIF":1.4,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143654511","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 : 2024-12-01DOI: 10.1016/j.sempedsurg.2025.151481
Joel Cazares, Hiroyuki Koga, Atsuyuki Yamataka
Biliary atresia is a rare, degenerative, and obliterative inflammatory disease of the bile ducts in neonates, affecting both the intra- and extrahepatic biliary tract. With an unknown etiology and no parallel condition in adults or older children, biliary atresia affects 1 in 10,000–18,000 live births and, if untreated, progresses to liver cirrhosis and death by the age of two. The Kasai portoenterostomy, introduced in 1950, remains the primary palliative procedure to restore bile drainage and delay the need for liver transplantation, which is required in cases of biliary flow failure. Biliary atresia continues to be the leading cause of pediatric liver transplants, accounting for up to 75 % of procedures in children under two years. While the Kasai technique has undergone numerous modifications; surgeons at Juntendo University Hospital, Japan, have modernized the procedure while preserving Dr. Kasai's original portoenterostomy principles. Since 2009, the hospital has been a pioneer in laparoscopic Kasai portoenterostomy; this article presents a detailed description of the state-of-the-art approaches and postoperative management, along with analysis of the latest outcomes. These advancements highlight the hospital's role as a leader in treatment of biliary atresia.
胆道闭锁是一种罕见的新生儿胆管退行性闭塞性炎症性疾病，影响肝内和肝外胆道。胆道闭锁病因不明，成人或年龄较大的儿童中没有类似的疾病，每10,000-18,000个活产婴儿中就有1人患有胆道闭锁，如果不治疗，会在两岁前发展为肝硬化和死亡。1950年引入的Kasai门肠造口术仍然是恢复胆汁引流和延迟肝移植需要的主要姑息性手术，肝移植是胆流衰竭的情况下所必需的。胆道闭锁仍然是儿童肝脏移植的主要原因，占两岁以下儿童手术的75%。虽然开赛技术经历了许多修改；日本顺天道大学医院（Juntendo University Hospital）的外科医生在保留Kasai博士最初的门静脉肠造口术原则的同时，使该手术现代化。自2009年以来，该院一直是腹腔镜开赛门静脉肠造口术的先驱；本文详细介绍了最先进的方法和术后管理，以及对最新结果的分析。这些进步突出了医院在治疗胆道闭锁方面的领导作用。
{"title":"Advances in the surgical technique of Kasai portoenterostomy","authors":"Joel Cazares, Hiroyuki Koga, Atsuyuki Yamataka","doi":"10.1016/j.sempedsurg.2025.151481","DOIUrl":"10.1016/j.sempedsurg.2025.151481","url":null,"abstract":"<div><div>Biliary atresia is a rare, degenerative, and obliterative inflammatory disease of the bile ducts in neonates, affecting both the intra- and extrahepatic biliary tract. With an unknown etiology and no parallel condition in adults or older children, biliary atresia affects 1 in 10,000–18,000 live births and, if untreated, progresses to liver cirrhosis and death by the age of two. The Kasai portoenterostomy, introduced in 1950, remains the primary palliative procedure to restore bile drainage and delay the need for liver transplantation, which is required in cases of biliary flow failure. Biliary atresia continues to be the leading cause of pediatric liver transplants, accounting for up to 75 % of procedures in children under two years. While the Kasai technique has undergone numerous modifications; surgeons at Juntendo University Hospital, Japan, have modernized the procedure while preserving Dr. Kasai's original portoenterostomy principles. Since 2009, the hospital has been a pioneer in laparoscopic Kasai portoenterostomy; this article presents a detailed description of the state-of-the-art approaches and postoperative management, along with analysis of the latest outcomes. These advancements highlight the hospital's role as a leader in treatment of biliary atresia.</div></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"Article 151481"},"PeriodicalIF":1.4,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143549934","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 : 2024-12-01DOI: 10.1016/j.sempedsurg.2025.151477
Qiongfen Lin , Paul Kwong-hang Tam , Clara Sze-man Tang
Biliary atresia (BA) is a severe neonatal cholestatic disorder marked by fibro-obliteration of the extrahepatic and intrahepatic bile ducts. It is the most common cause of pediatric end-stage liver disease and the leading indication for liver transplantation in children. There is significant heterogeneity in the etiology, involving various genetic and environmental factors such as viral infection, immune dysregulation and genetic predisposition to defective hepatobiliary development. In this review, we discuss the strategies to uncover the genetic factors underlying BA and highlight their associated molecular and pathological mechanisms, as well as the challenges faced in this area of research.
{"title":"Genetics of biliary atresia: Approaches, pathological insights and challenges","authors":"Qiongfen Lin , Paul Kwong-hang Tam , Clara Sze-man Tang","doi":"10.1016/j.sempedsurg.2025.151477","DOIUrl":"10.1016/j.sempedsurg.2025.151477","url":null,"abstract":"<div><div>Biliary atresia (BA) is a severe neonatal cholestatic disorder marked by fibro-obliteration of the extrahepatic and intrahepatic bile ducts. It is the most common cause of pediatric end-stage liver disease and the leading indication for liver transplantation in children. There is significant heterogeneity in the etiology, involving various genetic and environmental factors such as viral infection, immune dysregulation and genetic predisposition to defective hepatobiliary development. In this review, we discuss the strategies to uncover the genetic factors underlying BA and highlight their associated molecular and pathological mechanisms, as well as the challenges faced in this area of research.</div></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"Article 151477"},"PeriodicalIF":1.4,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143043070","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 : 2024-10-01DOI: 10.1016/j.sempedsurg.2024.151453
Brynn FitzGerald , Krishna Jagannathan , Nicholas Burjek , Matthew Rowland
{"title":"The development and benefits of a pediatric airway response team in a children's hospital","authors":"Brynn FitzGerald , Krishna Jagannathan , Nicholas Burjek , Matthew Rowland","doi":"10.1016/j.sempedsurg.2024.151453","DOIUrl":"10.1016/j.sempedsurg.2024.151453","url":null,"abstract":"","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 5","pages":"Article 151453"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142441509","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 : 2024-10-01DOI: 10.1016/j.sempedsurg.2024.151454
Eric C. Cheon , Mehul V. Raval , Alexander Froyshteter , Hubert A. Benzon
{"title":"Utilizing national surgical quality improvement program-pediatric for assessing anesthesia outcomes","authors":"Eric C. Cheon , Mehul V. Raval , Alexander Froyshteter , Hubert A. Benzon","doi":"10.1016/j.sempedsurg.2024.151454","DOIUrl":"10.1016/j.sempedsurg.2024.151454","url":null,"abstract":"","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 5","pages":"Article 151454"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142434371","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 : 2024-10-01DOI: 10.1016/j.sempedsurg.2024.151459
Aaron R. Brussels , Michelle S. Kim
Pectus excavatum (PE) is a common anterior chest wall deformity that affects the heart and lungs depending on the severity of compression. The Nuss procedure, a minimally invasive repair of pectus excavatum, has evolved over the years with thoracoscopic assistance and minimal incision. Despite improved surgical techniques, pain and nausea are often the most common factors determining hospital length of stay. This review will explore the preoperative, intraoperative, and postoperative anesthetic considerations necessary for improving patient outcomes, reducing surgical stress, and shortening hospital stays for patients undergoing the Nuss procedure.
{"title":"Perioperative considerations in anesthesia for minimally invasive repair of pectus excavatum, Nuss procedure","authors":"Aaron R. Brussels , Michelle S. Kim","doi":"10.1016/j.sempedsurg.2024.151459","DOIUrl":"10.1016/j.sempedsurg.2024.151459","url":null,"abstract":"<div><div>Pectus excavatum (PE) is a common anterior chest wall deformity that affects the heart and lungs depending on the severity of compression. The Nuss procedure, a minimally invasive repair of pectus excavatum, has evolved over the years with thoracoscopic assistance and minimal incision. Despite improved surgical techniques, pain and nausea are often the most common factors determining hospital length of stay. This review will explore the preoperative, intraoperative, and postoperative anesthetic considerations necessary for improving patient outcomes, reducing surgical stress, and shortening hospital stays for patients undergoing the Nuss procedure.</div></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 5","pages":"Article 151459"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142444603","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}
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