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单侧唇腭裂患者的额鼻及纤维性发育异常

Frontonasal and fibrous dysplasia in a patient with unilateral cleft lip and palate.

作者信息

Weathers William M, Wolfswinkel Erik M, Albright Steven B, Hollier Larry H, Buchanan Edward P

机构信息

Division of Plastic Surgery, Baylor College of Medicine Houston, TX 77030-2399, USA.

出版信息

J Craniofac Surg. 2013 Jul;24(4):e422-4. doi: 10.1097/SCS.0b013e3182942d27.

Abstract

Frontonasal dysplasia is a rare entity. It has characteristic physical deformities: hypertelorism, broad nasal root, median facial cleft of the upper lip or palate, clefting of the nasal alae, poorly formed nasal tip, cranium bifidum occultum, and a widow's peak hairline. Fibrous dysplasia is a benign bone tumor in which normal bone is replaced by fibrous, poorly formed osseus tissues. We present a patient with frontonasal dysplasia who desired correction of her hypertelorism. Incidentally, fibrous dysplasia was found in her left orbit complicating surgical correction. In addition, the patient has velopharyngeal insufficiency and a class III malocclusion. The interplay of all these craniofacial defects makes the sequencing and timing of surgery important in this unique patient.

摘要

额鼻发育异常是一种罕见病症。它具有特征性的身体畸形:眼距过宽、鼻根宽阔、上唇或腭部的正中面部裂、鼻翼裂、鼻尖发育不良、隐性颅裂以及寡妇峰发际线。骨纤维发育不良是一种良性骨肿瘤,其中正常骨被纤维性、发育不良的骨组织所取代。我们报告一名患有额鼻发育异常的患者,她希望矫正眼距过宽。偶然发现她的左侧眼眶存在骨纤维发育不良,这给手术矫正带来了复杂性。此外,该患者存在腭咽闭合不全和III类错牙合。所有这些颅面缺陷之间的相互作用使得在这位独特患者中手术的顺序和时机变得至关重要。

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