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上颌鼻发育不全(宾德综合征)的临床和病理特征:鼻前窝在病因学上的意义

Clinical and pathologic features of maxillonasal dysplasia (Binder's syndrome): significance of the prenasal fossa on etiology.

作者信息

Holmström H

出版信息

Plast Reconstr Surg. 1986 Nov;78(5):559-67. doi: 10.1097/00006534-198611000-00001.

DOI:10.1097/00006534-198611000-00001
PMID:3763743
Abstract

Clinical and pathologic anatomic parameters were studied in 50 patients with maxillonasal dysplasia (Binder's syndrome). The skeletal deformity causing the flat and low-set nose was in typical patients a palpable depression in the anterior nasal floor (fossa prenasalis) and a localized maxillary hypoplasia in the alar base region. Class III malocclusion was found in 54 percent. In 6 percent of the patients a slope (sulcus prenasalis) was found instead of a fossa in the anterior nasal floor, and in one patient a rudimentary fossa was found. Concomitant malformations were noted in 18 percent, and a hereditary connection was seen in 16 percent. The etiology is discussed in relation to the development of the premaxilla and the appearance of a secondary external trabecular network of bone in the canine region. An inhibition of the latter ossification center would explain the localized hypoplasia in the floor and walls of the piriform aperture in maxillonasal dysplasia.

摘要

对50例上颌鼻发育不全(宾德综合征)患者的临床和病理解剖参数进行了研究。导致鼻扁平及位置低的骨骼畸形,在典型患者中表现为鼻前底(鼻前窝)可触及的凹陷以及鼻翼基部区域局限性上颌骨发育不全。54%的患者存在Ⅲ类错牙合。6%的患者在前鼻底发现的是斜坡(鼻前沟)而非凹陷,1例患者发现有残留的凹陷。18%的患者伴有其他畸形,16%的患者有遗传关联。结合上颌前部的发育以及尖牙区继发性骨外小梁网络的出现对病因进行了讨论。后一个骨化中心的抑制可以解释上颌鼻发育不全中梨状孔底部和壁的局限性发育不全。

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