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浅表动静脉畸形的诊断与管理:法国医疗保健网络的建议。

Diagnosis and management of superficial arteriovenous malformations: French healthcare network's recommendations.

作者信息

Boccara Olivia, Salvan Didier, Laurian Claude, Degrugillier-Chopinet Caroline, Degardin Nathalie, Dillinger Jean-Guillaume, Malloizel-Delaunay Julie, Mouton Stéphane, Munck Stéphane, Maruani Annabel, Bisdorff-Bresson Annouk

机构信息

French Coordinator Reference Center for Superficial Vascular Anomalies in Children and Adults of FAVA-Multi Network, Paris, France.

Department of Dermatology, University Hospital Necker-Enfants Malades, Paris, France.

出版信息

Orphanet J Rare Dis. 2025 Jan 30;20(1):45. doi: 10.1186/s13023-024-03413-5.

Abstract

Superficial arteriovenous malformations are rare fast-flow lesions. They consist of arteriovenous shunts, without cellular hyperplasia or proliferation, which develop in the surrounding tissues (cutaneous, subcutaneous, muscular, bone). Although benign, they are among the most severe of superficial malformations. Their evolution can be life-threatening in exceptional cases. With the aim of optimizing diagnosis and management worldwide, this  protocol offers a state of the art for the diagnosis and management of these diseases. To this end, the French healthcare network specialized in these diseases have drawn on literature data and experience. Developed from the French National Diagnosis and Care Protocol, it presents the patient journeys for initial and differential diagnoses, and personalized therapeutic strategies. This requires a multidisciplinary team, with specialized professionals in handling genetic, treatment and psychosocial issues.

摘要

浅表动静脉畸形是罕见的快速血流病变。它们由动静脉分流组成,无细胞增生或增殖,发生于周围组织(皮肤、皮下、肌肉、骨骼)。尽管是良性的,但它们是最严重的浅表畸形之一。在特殊情况下,其进展可能危及生命。为了在全球范围内优化诊断和管理,本方案提供了这些疾病诊断和管理的最新技术。为此,法国专门治疗这些疾病的医疗网络借鉴了文献数据和经验。它源自法国国家诊断和护理方案,介绍了初始诊断和鉴别诊断的患者流程以及个性化治疗策略。这需要一个多学科团队,其中包括处理遗传、治疗和心理社会问题的专业人员。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f53/11783702/c7235b1221f2/13023_2024_3413_Fig1_HTML.jpg

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