The VASCERN-VASCA working group diagnostic and management pathways for lymphatic malformations

Abstract: Lymphatic malformations (LMs) are developmental defects of lymphatic vessels. LMs are histologically benign lesions, however, due to localization, size, and unexpected swelling, they may cause serious complications that threaten vital functions such as compression of the airways. A large swelling of the face or neck may also be disfiguring and thus constitute a psychological strain for patients and their families. LMs are also highly immunologically reactive, and are prone to recurrent infections and inflammation causing pain as well as chronic oozing wounds.

The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) is dedicated to gathering the best expertise in Europe. There are only few available guidelines on management and follow up of LMs, which commonly focus on very specific situations, such as head and neck LM (Zhou et al., 2011). It is still unclear, what constitutes an indication for treatment of LMs and how to follow up the patients. The Vascular Anomalies Working Group (VASCA-WG) of VASCERN decided to develop a diagnostic and management pathway for the management of LMs with a Nominal Group Technique (NGT), a well-established, structured, multistep, facilitated group meeting technique used to generate consensus statements. The pathway was drawn following 2 face-to-face meetings and multiple web meetings to facilitate discussion, and by mail to avoid the influence of most authoritative members.

The VASCA-WG has produced this opinion statement reflecting strategies developed by experts and patient representatives on how to approach patients with lymphatic malformations in a practical manner; we present an algorithmic view of the results of our work

Location
Deutsche Nationalbibliothek Frankfurt am Main
Extent
Online-Ressource
Language
Englisch
Notes
European journal of medical genetics. - 65, 12 (2022) , 104637, ISSN: 1878-0849

Event
Veröffentlichung
(where)
Freiburg
(who)
Universität
(when)
2022
Creator
Ghaffarpour, Nader
Baselga Torres, Eulalia
Boon, Laurence M.
Diociaiuti, Andrea
Dompmartin, Anne
Dvorakova, Veronika
El Hachem, Maya
Gasparella, Paolo
Haxhija, Emir Q.
Kyrklund, Kristiina
Irvine, Alan
Kapp, Friedrich
Rößler, Jochen
Salminen, Päivi
van den Bosch, Caroline
van der Vleuten, Carine
Kool, Leo Schultze
Vikkula, Miikka

DOI
10.1016/j.ejmg.2022.104637
URN
urn:nbn:de:bsz:25-freidok-2302889
Rights
Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
Last update
25.03.2025, 1:46 PM CET

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Associated

  • Ghaffarpour, Nader
  • Baselga Torres, Eulalia
  • Boon, Laurence M.
  • Diociaiuti, Andrea
  • Dompmartin, Anne
  • Dvorakova, Veronika
  • El Hachem, Maya
  • Gasparella, Paolo
  • Haxhija, Emir Q.
  • Kyrklund, Kristiina
  • Irvine, Alan
  • Kapp, Friedrich
  • Rößler, Jochen
  • Salminen, Päivi
  • van den Bosch, Caroline
  • van der Vleuten, Carine
  • Kool, Leo Schultze
  • Vikkula, Miikka
  • Universität

Time of origin

  • 2022

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