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帕里-罗伯格综合征合并线性面硬化症:儿科人群的管理。

Parry Romberg Syndrome and linear facial scleroderma: management in pediatric population.

机构信息

Maxillofacial and Dental Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy.

Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy.

出版信息

J Biol Regul Homeost Agents. 2017 Apr-Jun;31(2 Suppl 1):131-138.

Abstract

Among all different types of cutaneous scleroderma, Parry Romberg syndrome and linear scleroderma “en coup de sabre” typically involve the visage. Gradual degeneration of the tissues, from the skin up to the bone, is the stigmata of the diseases and the range of clinical manifestations is wide. They typically start during childhood and slowly progress before stabilizing. Considering the gravity of the associated deformity and its impact on facial function and appearance, we strongly advocate a prompt intervention that however must be tailored on paediatric patients. There is not a general consensus on hemifacial atrophy treatment, but autologous fat grafting has been proved to be a suitable technique, due to its low morbidity, repeatability and efficacy in correcting volume defects and in improving skin texture. Following the same concept of safeness and non-invasiveness we propose 3D stereophotogrammetric acquisitions as a possible tool for the pre- and post-surgical follow up, indispensable to evaluate the surgical results. We present our case series composed of 8 paediatric patients, aged between 11 and 17 years, who underwent several lipofilling procedures, from 2012 to 2016. Starting from 2015, 3D stereophotgrammetric data has been obtained.

摘要

在所有不同类型的皮肤硬皮病中,Parry Romberg 综合征和线性硬皮病“en coup de sabre”通常涉及面部。组织从皮肤到骨骼逐渐退化是这些疾病的特征,临床表现范围广泛。它们通常在儿童时期开始,在稳定之前缓慢进展。考虑到相关畸形的严重性及其对面部功能和外观的影响,我们强烈主张及时干预,但必须针对儿科患者进行调整。对于半侧颜面萎缩的治疗尚未达成共识,但由于其发病率低、可重复性高以及在纠正体积缺陷和改善皮肤质地方面的有效性,自体脂肪移植已被证明是一种合适的技术。基于安全性和非侵入性的相同理念,我们提出 3D 立体摄影测量采集作为术前和术后随访的一种可能工具,对于评估手术结果是不可或缺的。我们介绍了我们的病例系列,由 8 名年龄在 11 至 17 岁之间的儿科患者组成,他们在 2012 年至 2016 年间接受了多次脂肪填充手术。从 2015 年开始,获得了 3D 立体摄影测量数据。

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