先天性部分无鼻畸形：一例报告

Congenital partial arhinia: a case report.

作者信息

Akkuzu Guzin, Akkuzu Babur, Aydin Erdinc, Derbent Murat, Ozluoglu Levent

机构信息

Department of Otolaryngology, Başkent University, Ankara, Turkey.

出版信息

J Med Case Rep. 2007 Sep 20;1:97. doi: 10.1186/1752-1947-1-97.

DOI:10.1186/1752-1947-1-97

PMID:17883831

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2064923/

Abstract

Congenital arhinia is an extremely rare anomaly consisting of an absence of external nasal structures and nasal passages. Fewer than 30 cases have been reported. Patients with a familial absence of the nose have been reported, but the effects of genetic and maternal factors are unknown. Midface hypoplasia may accompany arhinia. Accompanying malformations are thought to be caused by an absent or rudimentary nose. A patient with partial congenital arhinia is presented and the embryology and literature review are discussed.

摘要

先天性无鼻是一种极为罕见的畸形，表现为外部鼻结构和鼻道缺失。报告的病例少于30例。有家族性鼻缺失的患者已有报道，但遗传因素和母体因素的影响尚不清楚。面中部发育不全可能与先天性无鼻伴发。人们认为，伴随的畸形是由鼻缺失或发育不全引起的。本文报告了一名部分先天性无鼻患者，并对胚胎学及相关文献进行了讨论。