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进行性半侧面部萎缩——病例报告。

Progressive Hemifacial Atrophy--case report.

作者信息

Pinheiro Thiago Pastor da Silva, Silva Camila Camarinha da, Silveira Carolina Souza Limeira da, Botelho Patrícia Cristina Ereno, Pinheiro Maria das Graças Rodriguez, Pinheiro João de Jesus Viana

机构信息

Department of Estomatology, School of Dentistry, University Center of Pará, Brazil.

出版信息

Med Oral Patol Oral Cir Bucal. 2006 Mar 1;11(2):E112-4.

Abstract

Progressive Hemifacial Atrophy, also known as Parry-Romberg Syndrome, is an uncommon degenerative and poorly understood condition. It is characterized by a slow and progressive atrophy affecting one side of the face. The incidence and the cause of this alteration is unknown. A cerebral disturbance of fat metabolism has been proposed as a primary cause. This can be the result of a trophic malformation of Cervical Sympathetic Nervous System. Possible factors that are involved in the pathogenesis include trauma, viral infections, heredity, endocrine disturbances and auto-immunity, among others. The most common complications that appear in association to this health disorder are: trigeminal neuritis, facial paresthesia, severe headache and epilepsy, being this last one the most frequent complication of the Central Nervous System. Characteristically, the atrophy progresses slowly for several years and, soon after, it become stable. Now, plastic surgery with graft of autogenous fat can be performed, after stabilization of the disease, to correct the deformity. Orthodontic treatment can help in the correction of any associated malformation. The objective of this work is, through the presentation of a clinical case, to accomplish a literature review concerning general characteristics, etiology, physiopathology, differential diagnosis and treatment of progressive hemifacial atrophy.

摘要

进行性半侧颜面萎缩症,又称帕里-罗姆伯格综合征,是一种罕见的、人们了解甚少的退行性疾病。其特征是一侧面部出现缓慢进展的萎缩。这种病变的发病率和病因尚不清楚。有人提出脂肪代谢的脑部紊乱是主要病因。这可能是颈交感神经系统营养性畸形的结果。发病机制中可能涉及的因素包括创伤、病毒感染、遗传、内分泌紊乱和自身免疫等。与这种健康障碍相关出现的最常见并发症有:三叉神经炎、面部感觉异常、严重头痛和癫痫,其中癫痫是中枢神经系统最常见的并发症。其特点是萎缩会缓慢进展数年,之后很快会稳定下来。疾病稳定后,可通过自体脂肪移植进行整形手术来矫正畸形。正畸治疗有助于矫正任何相关的畸形。本文通过一个临床病例,对进行性半侧颜面萎缩症的一般特征、病因、病理生理学、鉴别诊断及治疗进行文献综述。

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