背景:先天性巨结肠病是一种以缺乏肠神经节细胞为特征的先天性肠动力障碍。全结肠神经节病和近全或全肠神经节病,定义为整个结肠中不存在神经节细胞,并且涉及不同长度的小肠,是危及生命的疾病,影响不到10%的所有Hirschsprung病患者。该项目的目的是在ERNICA内部制定临床共识声明,欧洲罕见的先天性消化系统疾病参考网络,关于四个主要主题:全结肠神经节病的外科治疗,全肠神经节病的外科治疗,全结肠和/或肠神经节病肠功能不良的管理以及全结肠和/或肠神经节病肠功能不良的长期管理。
方法:来自ERNICA中心的多学科代表小组应邀参加。文献被搜索,使用指定的搜索条件,在Medline(ALL)中,Embase和谷歌学者。筛选摘要并选择全文出版物。小组分为四组,从全文出版物中提取数据,并就每个主要主题提出陈述草案。使用修改后的Delphi过程来完善和商定声明。
结果:由来自10个欧洲国家的24名参与者组成的多学科小组进行了共识声明,45项声明经过3轮德尔菲达成共识。高质量临床证据的可用性有限,大多数陈述都是基于专家意见。另有25项声明没有达成共识。
结论:全结肠和全肠神经节病是Hirschsprung病的罕见变种,高质量临床证据的可用性非常有限。这份共识声明提供了关于手术治疗的声明,这些罕见患者的肠功能不良管理和长期管理。专家小组一致认为,患者受益于多学科和个性化护理,最好是在专家中心。
方法:临床共识声明。
方法:3a。
BACKGROUND: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical
consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis.
METHODS: A multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements.
RESULTS: The
consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached
consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach
consensus.
CONCLUSIONS: Total colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center.
METHODS: Clinical
consensus statement.
METHODS: 3a.