The VASCERN-VASCA working group diagnostic and management pathways for lymphatic malformations
Por:
Ghaffarpour N, Baselga E, Boon LM, Diociaiuti A, Dompmartin A, Dvorakova V, El Hachem M, Gasparella P, Haxhija E, Kyrklund K, Irvine AD, Kapp FG, Rößler J, Salminen P, van den Bosch C, van der Vleuten C, Kool LS and Vikkula M
Publicada:
1 dic 2022
Ahead of Print:
1 oct 2022
Resumen:
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.
Filiaciones:
Ghaffarpour N:
Department of Reconstructive Plastic Surgery, Karolinska University Hospital, Stockholm, Sweden
Baselga E:
Pediatric Dermatology, Hospital Sant Joan de Deu, Barcelona, Spain
Boon LM:
Center for Vascular Anomalies, Division of Plastic Surgery, University Clinics Saint-Luc, University of Louvain, Brussels, Belgium
VASCERN VASCA European Reference Centre University of Louvain, Brussels, Belgium
Diociaiuti A:
Dermatology Unit and Genodermatosis Unit, Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, Piazza Sant'Onofrio 4, 00165, Rome, Italy
Dompmartin A:
Department of Dermatology, Université de Caen Basse Normandie, CHU Caen, Caen, France
Dvorakova V:
Paediatric Dermatology, Children's Health Ireland, (Y)Clinical Medicine, Trinity College Dublin, Ireland
El Hachem M:
Dermatology Unit and Genodermatosis Unit, Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, Piazza Sant'Onofrio 4, 00165, Rome, Italy
Gasparella P:
Department of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria
Haxhija E:
Department of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria
Kyrklund K:
Department of Pediatric Surgery, HUS Rare Disease Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
Irvine AD:
Paediatric Dermatology, Children's Health Ireland, (Y)Clinical Medicine, Trinity College Dublin, Ireland
Kapp FG:
Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg, Germany
Rößler J:
Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg, Germany
Division of Pediatric Hematology and Oncology, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Switzerland
Salminen P:
Department of Pediatric Surgery, HUS Rare Disease Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
van den Bosch C:
Hevas, Patient Organisation for Vascular Anomalies, the Netherlands
van der Vleuten C:
Department of Dermatology, Radboudumc Expertise Center for Haemangiomas and Congenital Vascular Malformations Nijmegen (Hecovan), Radboud University Medical Center, Nijmegen, the Netherlands
Kool LS:
Department of Radiology, Radboudumc Expertise Center for Haemangiomas and Congenital Vascular Malformations Nijmegen (Hecovan), Radboud University Medical Center, Nijmegen, the Netherlands
Vikkula M:
Center for Vascular Anomalies, Division of Plastic Surgery, University Clinics Saint-Luc, University of Louvain, Brussels, Belgium
VASCERN VASCA European Reference Centre University of Louvain, Brussels, Belgium
Human Molecular Genetics, de Duve Institute, University of Louvain, Brussels, Belgium
Department of Reconstructive Plastic Surgery, Karolinska University Hospital, Stockholm, Sweden.
Green Submitted, hybrid
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