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

E Segna, V Pucciarelli,G A Beltramini,C Sforza, F J Silvestre,A B Giannì,A Baj

JOURNAL OF BIOLOGICAL REGULATORS AND HOMEOSTATIC AGENTS(2017)

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摘要
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.
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关键词
linear scleroderma,three dimensional imaging,facial hemiatrophy,fat graft
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