外侧鼻突伴同侧鼻-嗅神经通路发育不全。

Proboscis lateralis with ipsilateral sinonasal and olfactory pathway aplasia.

机构信息

Department of Radiology and Imaging, Grant Medical Foundation, Ruby Hall Clinic, Pune-411001, Maharashtra, India.

出版信息

J Pediatr Surg. 2010 Feb;45(2):453-6. doi: 10.1016/j.jpedsurg.2009.10.044.

DOI:10.1016/j.jpedsurg.2009.10.044

PMID:20152374

Abstract

Proboscis lateralis is a rare craniofacial malformation characterized by absence of nasal cavity on one side with a trunk-like nasal appendage protruding from superomedial portion of the ipsilateral orbit. High-resolution computed tomography and magnetic resonance imaging are extremely useful in evaluating this congenital condition and the wide spectrum of associated anomalies occurring in the surrounding anatomical regions and brain. We present a case of proboscis lateralis in a 2-year-old girl with associated ipsilateral sinonasal aplasia, orbital cyst, absent olfactory bulb and olfactory tract. Absence of ipsilateral olfactory pathway in this rare disorder has been documented on high-resolution computed tomography and magnetic resonance imaging by us for the first time in English medical literature.

摘要

外侧鼻突是一种罕见的颅面畸形，其特征是一侧鼻腔缺失，而从同侧眼眶的内上部分突出一个类似躯干的鼻附属物。高分辨率计算机断层扫描和磁共振成像在评估这种先天性疾病以及周围解剖区域和大脑中发生的广泛相关异常方面非常有用。我们报告了一例 2 岁女孩的外侧鼻突，伴有同侧鼻窦发育不全、眼眶囊肿、嗅球和嗅束缺失。在这种罕见疾病中，我们首次在英文医学文献中通过高分辨率计算机断层扫描和磁共振成像记录到同侧嗅觉通路缺失。