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- W2982446183 abstract "Les anomalies vasculaires superficielles constituent un large groupe de pathologies tumorales ou malformatives, développées aux dépens des vaisseaux de tous types. Les tumeurs vasculaires résultent d’une hyperplasie cellulaire ; les malformations vasculaires (MVs) sont faites de vaisseaux dysplasiques. Cette différence physiopathologique est la base de la classification de l’ISSVA (International Society for the Study of Vascular Anomalies). Les tumeurs sont dominées par l’hémangiome infantile de loin le plus fréquent, dont le traitement, si nécessaire, repose sur le propranolol. Les hémangiomes congénitaux et les tumeurs associées au phénomène de Kasabach–Merritt, c’est-à-dire à la survenue d’une thrombopénie profonde, sont des tumeurs beaucoup plus rares. La prise en charge du phénomène de Kasabach–Merritt est transformée par l’utilisation du sirolimus. Les MVs à flux lent sont les malformations capillaires, veineuses et lymphatiques ; celles à flux rapide sont les malformations artério-veineuses. Ces différents types de MVs peuvent être combinés. Les MVs font l’objet actuellement d’un démembrement génétique croissant. Leur prise en charge reste symptomatique, adaptée au cas par cas, décidée lors de consultations pluridisciplinaires. Les traitements interventionnels, chirurgie et radiologie interventionnelle, sont d’efficacité partielle ; la prise en charge de ces patients est de plus en plus médicale. Le traitement des complications liées à la coagulopathie dans les malformations veineuses repose en 1ère intention sur les héparines de bas poids moléculaire ; le sirolimus semble très efficace pour les complications hémorragiques résistantes au traitement habituel. Le sirolimus devient le traitement de fond des manifestations inflammatoires douloureuses des malformations lymphatiques mixtes et/ou complexes. Superficial vascular anomalies constitute a large group of malformations and tumoral conditions developed from all types of vessels. Vascular tumors are the result of cellular hyperplasia, whereas vascular malformations (VMs) are composed of dysplastic vessels. The classification by the ISSVA (International Society for the Study of Vascular Anomalies) is based on this pathogenic difference. The most common vascular tumor is infantile hemangioma, whose treatment, when necessary, is propranolol. Congenital hemangiomas and tumors that might be complicated with Kasabach–Merritt phenomenon, i.e., deep thrombocytopenia, are much rarer. Management of Kasabach–Merritt phenomenon is now largely based on sirolimus. Low-flow VMs include capillary, venous, and lymphatic malformations; arteriovenous malformations are high-flow malformations. These different types of VMs might be combined. Currently, there is increasing research in delineating the different entities based on molecular findings. Treatment of VMs depends on the associated impairment, and is decided on a case-by-case basis in multidisciplinary consultations. Interventional treatments, especially surgery and sclerotherapy, are usually partially effective, and management of patients with VMs increasingly involves medical drugs. First-line treatment of coagulation disorders associated with venous malformations is based on low-molecular-weight heparin; sirolimus seems efficient in hemorrhagic complications refractory to usual treatment. Sirolimus is set to become the standard treatment in painful inflammatory manifestations of mixed and/or complicated lymphatic malformations." @default.
- W2982446183 created "2019-11-08" @default.
- W2982446183 creator A5053839055 @default.
- W2982446183 date "2019-12-01" @default.
- W2982446183 modified "2023-10-17" @default.
- W2982446183 title "Anomalies vasculaires superficielles de l’enfant" @default.
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- W2982446183 doi "https://doi.org/10.1016/j.perped.2019.10.005" @default.
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