Dompmartin Anne, Baselga Eulalia, Boon Laurence M, Diociaiuti Andrea, Dvorakova Veronika, El Hachem May, Gasparella Paolo, Haxhija Emir, Ghaffarpour Nader, Kyrklund Kristiina, Irvine Alan D, Kapp Friedrich G, Rößler Jochen, Salminen Päivi, van den Bosch Caroline, van der Vleuten Carine, Schultze Kool Leo, Vikkula Miikka
Dermatology Department CHU Caen Université Caen Normandie CHU Caen Côte nacre 14033 Caen Cedex, France.
Pediatric Dermatology, Hospital Sant Joan de Deu, Barcelona, Spain; VASCERN VASCA European Reference Centre.
J Vasc Anom (Phila). 2023 Mar 23;4(2):e064. doi: 10.1097/JOVA.0000000000000064. eCollection 2023 Jun.
To elaborate expert consensus patient pathways to guide patients and physicians toward efficient diagnostics and management of patients with venous malformations.
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.
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.
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/).
详细阐述专家共识患者路径,以指导患者和医生对静脉畸形患者进行高效诊断和管理。
该路径从对静脉型畸形(VM)的临床怀疑开始,列出支持该怀疑所需寻找的临床特征。建议了后续成像和组织病理学的策略。这些旨在为诊断提供信息,并将患者分为4个亚型:(1)散发性单发性VM或(2)多灶性,(3)家族性、多灶性,以及(4)合并和/或综合征性VM。路径的后续页面详细介绍了每种类型的管理,通过颜色编码来识别关于(1)临床评估、(2)检查、(3)治疗和(4)相关基因的部分。与所有类型相关的行动在单独的框中标记,包括建议进行成像时。当达成明确诊断时,路径还指向针对特定疾病的额外检查和随访建议。讨论了每种亚型的管理选项,包括保守和侵入性治疗以及新型分子疗法。
由9个专家中心组成的网络VASCERN-VASCA的共同努力,产生了VM的共识诊断和管理路径,以协助临床医生和患者。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。