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[鼻窝畸形与面部正中裂。新视角]

[Malformations of the nasal fossa and paramedian facial clefts. New perspectives].

作者信息

Garabedian E N, Ducroz V, Leperchey F, Roger G, Denoyelle F

机构信息

Service ORL et de Chirurgie Cervico-Faciale, Hôpital d'Enfants Armand Trousseau, Paris.

出版信息

Ann Otolaryngol Chir Cervicofac. 1996;113(7-8):373-8.

PMID:9207969
Abstract

Since choanal atresia may be associated with other cranio-facial malformations, including various degrees of nasal fossa malformation, and be a part of paramedian facial clefts, (as described by Tessier), they can be integrated into the larger group of neurocristopathies. We identified four such cases with combined clinical elements corresponding to Tessier's paramedian facial cleft, including eyelid coloboma, mild to severe choanal and nasal fossa anomalies, ethmoidal hypoplasia and anterior skull base malformation, sometimes with proboscis lateralis. These various malformations are due to abnormality of the olfactive placode and the adjacent mesenchyme. These discoveries incited us to elaborate a conception first of all on the pertinent embryology involved, second, to propose a new classification based on anatomical and pathogenic embryological considerations. And finally, since endonasal laser therapy is particularly dangerous in such cases, to propose the use of transpalatal approach to restore choanal permeability. Pediatric ENT surgeons should pay special attention to any small stigmatism of facial cleft when dealing with children affected by choanal atresia.

摘要

由于后鼻孔闭锁可能与其他颅面畸形相关,包括不同程度的鼻窝畸形,并且是正中旁面裂(如Tessier所描述)的一部分,因此它们可被纳入更大的神经嵴病组。我们确定了4例具有与Tessier正中旁面裂相对应的综合临床特征的病例,包括眼睑缺损、轻至重度后鼻孔和鼻窝异常、筛骨发育不全和前颅底畸形,有时伴有侧鼻吻。这些各种畸形是由于嗅基板及其相邻间充质的异常所致。这些发现促使我们首先阐述相关的胚胎学概念,其次,基于解剖学和致病胚胎学考虑提出一种新的分类方法。最后,由于鼻内激光治疗在这种情况下特别危险,建议采用经腭途径恢复后鼻孔通畅。小儿耳鼻喉科医生在处理患有后鼻孔闭锁的儿童时,应特别注意面部裂的任何微小体征。

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