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梅尼克-尼德尔斯综合征的正颌外科手术:文献综述及两例同胞病例报告

Orthognathic surgery in Melnick-Needles syndrome: a review of the literature and report of two siblings.

作者信息

O'Connell J E, Bourke B, Kearns G J

机构信息

Department of Oral and Maxillofacial Surgery, St James's Hospital, Dublin, Ireland.

St. Columcille's Hospital, Loughlinstown, Co. Dublin, Ireland.

出版信息

Int J Oral Maxillofac Surg. 2018 Jun;47(6):738-742. doi: 10.1016/j.ijom.2017.12.001. Epub 2018 Jan 12.

Abstract

Melnick-Needles syndrome (MNS) is a rare congenital X-linked dominant skeletal dysplasia, characterized by exophthalmos, a prominent forehead, and mandibular hypoplasia and retrognathism. Dental features may include anodontia, hypodontia, or oligodontia. Increased collagen content, unpredictable collagen synthesis, and abnormal bony architecture have raised concerns regarding bone splitting intraoperatively and bone healing postoperatively. This report describes the cases of two sisters with MNS, who successfully underwent orthognathic surgery consisting of bilateral mandibular ramus osteotomies combined with advancement genioplasty and iliac crest bone grafting, to correct the classical MNS facial deformity of mandibular retrognathia.

摘要

梅尔尼克-尼德尔斯综合征(MNS)是一种罕见的先天性X连锁显性骨骼发育不良,其特征为眼球突出、前额突出、下颌发育不全和下颌后缩。牙齿特征可能包括无牙症、少牙症或牙数减少。胶原蛋白含量增加、不可预测的胶原蛋白合成以及异常的骨结构引发了对术中骨劈开和术后骨愈合的担忧。本报告描述了两名患有MNS的姐妹的病例,她们成功接受了正颌手术,包括双侧下颌升支截骨术联合颏成形术和髂嵴骨移植,以矫正下颌后缩这一典型的MNS面部畸形。

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