The VASCERN-VASCA Working Group Diagnostic and Management Pathways for Venous Malformations.

Anne Dompmartin, Eulalia Baselga, Laurence M Boon, Andrea Diociaiuti, Veronika Dvorakova, May El Hachem, Paolo Gasparella, Emir Haxhija, Nader Ghaffarpour, Kristiina Kyrklund, Alan D Irvine, Friedrich G Kapp, Jochen Rößler, Päivi Salminen, Caroline van den Bosch, Carine van der Vleuten, Leo Schultze Kool, Miikka Vikkula
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引用次数: 0

Abstract

To elaborate expert consensus patient pathways to guide patients and physicians toward efficient diagnostics and management of patients with venous malformations.

Methods: VASCERN-VASCA (https://vascern.eu/) is a European network of multidisciplinary centers for Vascular Anomalies. The Nominal Group Technique was used to establish the pathways. Two facilitators were identified: one to propose initial discussion points and draw the pathways, and another to chair the discussion. A dermatologist (AD) was chosen as first facilitator due to her specific clinical and research experience. The draft was subsequently discussed within VASCERN-VASCA monthly virtual meetings and annual face-to-face meetings.

Results: The Pathway starts from the clinical suspicion of a venous type malformation (VM) and lists the clinical characteristics to look for to support this suspicion. Strategies for subsequent imaging and histopathology are suggested. These aim to inform on the diagnosis and to separate the patients into 4 subtypes: (1) sporadic single VMs or (2) multifocal, (3) familial, multifocal, and (4) combined and/or syndromic VMs. The management of each type is detailed in subsequent pages of the pathway, which are color coded to identify sections on (1) clinical evaluations, (2) investigations, (3) treatments, and (4) associated genes. Actions relevant to all types are marked in separate boxes, including when imaging is recommended. When definite diagnoses have been reached, the pathway also points toward disease-specific additional investigations and recommendations for follow up. Options for management are discussed for each subtype, including conservative and invasive treatments, as well as novel molecular therapies.

Conclusion: The collaborative efforts of VASCERN-VASCA, a network of the 9 Expert Centers, has led to a consensus Diagnostic and Management Pathways for VMs to assist clinicians and patients. It also emphasizes the role of multidisciplinary expert centers in the management of VM patients. This pathway will become available on the VASCERN website (http://vascern.eu/).

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VASCERN-VASCA工作组静脉畸形的诊断和管理途径。
制定专家共识的患者路径,指导患者和医生对静脉畸形患者进行有效的诊断和管理。方法:VASCERN-VASCA (https://vascern.eu/)是欧洲血管异常多学科中心网络。采用标称组技术建立神经通路。确定了两位主持人:一位提出初步讨论点并绘制路径,另一位主持讨论。一位皮肤科医生(AD)因其特殊的临床和研究经验而被选为第一位主持人。该草案随后在VASCERN-VASCA每月虚拟会议和年度面对面会议上进行了讨论。结果:该路径从临床怀疑静脉型畸形(VM)开始,列出临床特征来寻找支持这一怀疑。建议后续影像学和组织病理学的策略。这些目的是为诊断提供信息,并将患者分为4种亚型:(1)散发的单个vm或(2)多灶性vm,(3)家族性,多灶性vm和(4)合并和/或综合征性vm。每种类型的管理在路径的后续页面中详细介绍,这些页面用颜色编码来识别(1)临床评估,(2)调查,(3)治疗和(4)相关基因。与所有类型相关的操作在单独的框中进行了标记,包括建议进行成像的操作。当获得明确的诊断时,该途径还指向特定疾病的额外调查和随访建议。讨论了每个亚型的治疗选择,包括保守和侵入性治疗,以及新的分子治疗。结论:VASCERN-VASCA(一个由9个专家中心组成的网络)的共同努力,已经形成了共识的vm诊断和管理途径,以帮助临床医生和患者。它还强调了多学科专家中心在VM患者管理中的作用。该路径将在VASCERN网站(http://vascern.eu/)上提供。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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