The VASCERN-VASCA working group diagnostic and management pathways for severe and/or rare infantile hemangiomas
| Autor: | Andrea Diociaiuti, Eulalia Baselga, Laurence M. Boon, Anne Dompmartin, 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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| Přispěvatelé: | UCL - SSS/DDUV/GEHU - Génétique, UCL - (SLuc) Centre de malformations vasculaires congénitales, UCL - (SLuc) Service de chirurgie plastique, HUS Children and Adolescents, Children's Hospital, University of Helsinki, Lastenkirurgian yksikkö |
| Rok vydání: | 2022 |
| Předmět: |
Skin Neoplasms
Vascular damage Radboud Institute for Health Sciences [Radboudumc 16] Pelvis All institutes and research themes of the Radboud University Medical Center Lumbar Diagnosis Genetics Humans LUMBAR/PELVIS/SACRAL syndrome Vascular Diseases Multifocal hemangiomas 610 Medicine & health Genetics (clinical) 1184 Genetics developmental biology physiology SACRAL syndrome Infant Large segmental hemangiomas General Medicine Syndrome PHACES syndrome Infantile hemangioma Management Europe Algorithm Hemangioma |
| Zdroj: | European journal of medical genetics, Vol. 65, no.6, p. 104517 (2022) European Journal of Medical Genetics, 65, 6 European Journal of Medical Genetics, 65 European Journal of Medical Genetics, Vol. 65, no.6, p. 104517 (2022) |
| ISSN: | 1769-7212 |
| DOI: | 10.48350/169635 |
| Popis: | The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN), is dedicated to gathering the best expertise in Europe and provide accessible cross-border healthcare to patients with rare vascular diseases. Infantile Hemangiomas (IH) are benign vascular tumors of infancy that rapidly growth in the first weeks of life, followed by stabilization and spontaneous regression. In rare cases the extent, the localization or the number of lesions may cause severe complications that need specific and careful management. Severe IH may be life-threatening due to airway obstruction, liver or cardiac failure or may harbor a risk of functional impairment, severe pain, and/or significant and permanent disfigurement. Rare IHs include syndromic variants associated with extracutaneous abnormalities (PHACE and LUMBAR syndromes), and large segmental hemangiomas. There are publications that focus on evidence-based medicine on propranolol treatment for IH and consensus statements on the management of rare infantile hemangiomas mostly focused on PHACES syndrome. The Vascular Anomalies Working Group (VASCA-WG) decided to develop a diagnostic and management pathway for severe and rare IHs 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 two face-to-face meetings and in 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 severe and rare IH in a practical manner; we present an algorithmic view of the results of our work. |
| Databáze: | OpenAIRE |
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