Hou Yikang, Jin Yunbo, Lin Xiaoxi, Chai Gang, Zhang Yan, Qi Zuoliang
From the *Department of Plastic and Cosmetic Surgery, Gansu Provincial Hospital, Lanzhou; †Department of Plastic & Reconstructive Surgery, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai; and ‡Plastic Surgery Hospital (Institute), Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, P.R. China.
Ann Plast Surg. 2017 Jun;78(6):684-691. doi: 10.1097/SAP.0000000000000933.
Ectodermal dysplasia syndrome is a hereditary disease of ectodermal origin. Appearances of nail dystrophy, alopecia or hypotrichosis, saddle nose deformity, and palmoplantar hyperkeratosis are usually associated with a lack of sweat glands as well as partial or complete absence of teeth. These manifestations are usually corrected only with oral rehabilitation by mounting dentures. In this study, plastic rehabilitation was developed to correct the special features of patients with ectodermal dysplasia. Four men and 1 woman with ectodermal dysplasia syndrome were treated. Four patients showed dysostosis of the midface, and rhinoplasty with costal bone was performed, whereas cosmetic operation aiming to repair soft tissue defects was adopted for the last patient. After plastic corrections, all 5 patients were satisfied with the results and had no social embarrassment.
外胚层发育不全综合征是一种外胚层起源的遗传性疾病。甲营养不良、脱发或毛发稀少、鞍鼻畸形以及掌跖角化过度等表现通常与汗腺缺乏以及部分或完全无牙有关。这些表现通常仅通过佩戴假牙进行口腔修复来矫正。在本研究中，开展了整形修复以矫正外胚层发育不全患者的特殊面容。对4名男性和1名患有外胚层发育不全综合征的女性进行了治疗。4例患者表现为面中部骨发育不全，实施了肋软骨隆鼻术，而最后1例患者则采用了旨在修复软组织缺损的美容手术。经过整形矫正后，所有5例患者对结果均满意，且没有社交方面的困扰。
