The VASCERN Pediatric and Primary Lymphedema Working Group (PPL WG) has published an important paper entitled ‘The VASCERN PPL working group patient pathway for primary and paediatric lymphoedema’, which outlines strategies for managing patients with primary and paediatric lymphoedema.
Lymphoedema is caused by an imbalance between fluid production and transport by the lymphatic system. This imbalance can be caused by either a reduced transport capacity of the lymphatic system or too much fluid production and leads to swelling associated with tissue changes (skin thickening, fat deposition). This article focuses on primary lymphoedema, the main common complication of which is the increased risk of developing cellulitis/erysipelas in the affected area, which can worsen lymphatic function and be the cause of increased patient morbidity if not treated correctly/urgently.
In this paper, the VASCERN PPL WG has presented a detailed patient pathway describing the clinical evaluation and investigations leading to clinical diagnosis, genetic testing, differential diagnosis, management and treatment options, and patient follow-up at expert and local centres. The pathway aims to improve patient care by reducing the time to diagnosis, defining the best management and follow-up strategies, and avoiding overuse of resources.
This publication is a collaborative effort between the experts and patient representatives of the VASCERN PPL WG. It is a valuable resource for healthcare professionals, patients and researchers alike. To read the full paper and gain a full understanding of this pathway, click here.
References:
Devoogdt N, Thomis S, Belva F, Dickinson-Blok J, Fourgeaud C, Giacalone G, Karlsmark T, Kavola H, Keeley V, Marques ML, Mansour S, Nissen CV, Nørregaard S, Oberlin M, Ručigaj TP, Somalo-Barranco G, Suominen S, Van Duinen K, Vignes S, Damstra R. The VASCERN PPL working group patient pathway for primary and paediatric lymphoedema. Eur J Med Genet. 2023 Dec 23;67:104905. doi: 10.1016/j.ejmg.2023.104905. Epub ahead of print. PMID: 38143023.