A multi-step approach to overcome challenges in the management of head and neck lymphatic malformations, and response to treatment.

IF 3.4 2区 医学 Q2 GENETICS & HEREDITY
Valentina Trevisan, Eugenio De Corso, Germana Viscogliosi, Roberta Onesimo, Alessandro Cina, Marco Panfili, Lucrezia Perri, Cristiana Agazzi, Valentina Giorgio, Donato Rigante, Giovanni Vento, Patrizia Papacci, Filomena Valentina Paradiso, Sara Silvaroli, Lorenzo Nanni, Nicoletta Resta, Marco Castori, Jacopo Galli, Gaetano Paludetti, Giuseppe Zampino, Chiara Leoni
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Abstract

Background: Lymphatic malformations are vascular developmental anomalies varying from local superficial masses to diffuse infiltrating lesions, resulting in disfigurement. Patients' outcomes range from spontaneous regression to severe sequelae notwithstanding appropriate treatment. The current classification guides, in part, clinicians through the decision-making process, prognosis prediction and choice of therapeutic strategies. Even though the understanding of molecular basis of the disease has been recently improved, a standardized management algorithm has not been reached yet.

Results: Here, we report our experience on five children with different lymphatic anomalies of the head and neck region treated by applying a multidisciplinary approach reaching a consensus among specialists on problem-solving and setting priorities.

Conclusions: Although restitutio ad integrum was rarely achieved and the burden of care is challenging for patients, caregivers and healthcare providers, this study demonstrates how the referral to expert centres can significantly improve outcomes by alleviating parental stress and ameliorating patients' quality of life. A flow-chart is proposed to guide the multidisciplinary care of children with LMs and to encourage multidisciplinary collaborative initiatives to implement dedicated patients' pathways.

采用多步骤方法克服头颈部淋巴畸形管理中的挑战,以及对治疗的反应。
背景:淋巴管畸形是一种血管发育异常,从局部浅表肿块到弥漫浸润性病变,导致毁容。患者的预后各不相同,有的会自发消退,有的虽经适当治疗仍会留下严重后遗症。目前的分类在一定程度上指导着临床医生的决策过程、预后预测和治疗策略的选择。尽管最近对该疾病分子基础的认识有所提高,但尚未形成标准化的管理算法:在此,我们报告了五名头颈部淋巴异常患儿的治疗经验,这些患儿的治疗采用了多学科方法,专家们在解决问题和确定优先次序方面达成了共识:结论:虽然很少能实现综合治疗,患者、护理人员和医疗服务提供者的护理负担也很重,但本研究表明,转诊到专家中心可以减轻家长的压力,改善患者的生活质量,从而显著提高治疗效果。本研究提出了一个流程图,用于指导 LMs 患儿的多学科护理,并鼓励多学科合作实施患者专用路径。
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来源期刊
Orphanet Journal of Rare Diseases
Orphanet Journal of Rare Diseases 医学-医学:研究与实验
CiteScore
6.30
自引率
8.10%
发文量
418
审稿时长
4-8 weeks
期刊介绍: Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.
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