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上颌鼻骨发育不全(宾德综合征)的临床与影像学表现

Clinical and radiologic aspects of maxillonasal dysostosis (Binder syndrome).

作者信息

Delaire J, Tessier P, Tulasne J F, Resche F

出版信息

Head Neck Surg. 1980 Nov-Dec;3(2):105-22. doi: 10.1002/hed.2890030205.

Abstract

Maxillonasal dysostosis, described by Binder in 1962, is probably more common than one would think from the small number of cases that have been published (fewer than 40). We have seen 37 cases of this syndrome over a period of 8 years. Diagnosis of the syndrome is easy because of its characteristic anomalies of the upper lip and nose: smallness of the nose, a groove under the columella, a "half-moon" appearance of the nasal apertures, absence of the nasal spine, and projection of the chin. In addition, we have observed several associated abnormalities: convergent strabismus (2 cases), mongolism (1 case), labiomaxillopalatine cleft (1 case), and, of particular note, numerous abnormalities of the cervical spine, frequently associated with mandibular prognathism. In view of the frequency of the latter abnormalities. Binder syndrome might well be called a "nasomaxillovertebral syndrome."

摘要

上颌鼻骨发育不全由宾德于1962年首次描述,其实际发病率可能比已发表的少数病例(不到40例)所显示的更为常见。在8年时间里,我们共诊治了37例该综合征患者。该综合征的诊断并不困难,因为其具有上唇和鼻部的特征性异常表现:鼻小、鼻小柱下方有沟、鼻孔呈“半月形”、鼻棘缺如以及下巴前突。此外,我们还观察到一些相关异常:内斜视(2例)、唐氏综合征(1例)、唇腭裂(1例),特别值得注意的是,颈椎存在众多异常,且常与下颌前突相关。鉴于后一种异常的发生频率,宾德综合征完全可以被称为“鼻上颌椎骨综合征”。

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