Shah Gopi B, Ordemann Allison, Daram Shiva, Roman Emily, Booth Tim, Johnson Romaine, Xi Yin, Mitchell Ron
Department of Otolaryngology, University of Texas Southwestern Medical Center, 2001 Inwood Road, 6th & 7th Floors, Dallas, TX, 75390, USA.
Department of Otolaryngology, University of Texas Southwestern Medical Center, 2001 Inwood Road, 6th & 7th Floors, Dallas, TX, 75390, USA.
Int J Pediatr Otorhinolaryngol. 2019 Nov;126:109608. doi: 10.1016/j.ijporl.2019.109608. Epub 2019 Jul 26.
Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal respiratory distress that is difficult to treat. The primary objective of this study was to identify factors that predict the need for initial and revision surgery for CNAPS. The secondary objective is to identify risk factors in maternal history associated with the development of CNPAS.
Infants with CNPAS between 2010 and 2017 were identified by ICD- 9 and 10 codes. Demographics, maternal history, anatomic features on imaging and medical and/or surgical management were reviewed. Frequencies, means and standard deviations were calculated. A p-value <.05 was considered significant.
Twenty infants were included. All underwent flexible nasal endoscopy with inability to pass the scope in either nostril in 65% of infants. Nineteen had a CT scan and 13 had a MRI with midline defects in 76.3% and 53.8%, respectively. Solitary central mega-incisor was present in 65%. Half underwent surgical intervention at a mean age of 74.8 days, with 90% requiring revision surgery. There was no difference in pyriform aperture distance in the surgical and non-surgical patient subgroups (5.4 mm and 5.2 mm, p = .6 respectively). No specific variables were predictive of need for initial or revision surgery. Maternal diabetes mellitus (MDM) was found in 55% of mothers of infants with CNPAS.
Pyriform aperture distance was not a predictor of surgical intervention. MRI should be considered in all infants with CNPAS as the rate of intracranial complications is high. MDM may be a risk factor for CNPAS.
先天性鼻梨状孔狭窄（CNPAS）是新生儿呼吸窘迫的罕见病因，治疗困难。本研究的主要目的是确定预测CNAPS初次手术和翻修手术需求的因素。次要目的是确定母亲病史中与CNPAS发生相关的危险因素。
通过国际疾病分类第9版和第10版编码确定2010年至2017年间患有CNPAS的婴儿。回顾人口统计学、母亲病史、影像学上的解剖特征以及医疗和/或手术管理情况。计算频率、均值和标准差。p值<0.05被认为具有统计学意义。
纳入20例婴儿。所有婴儿均接受了鼻内镜检查，65%的婴儿双侧鼻孔均无法通过内镜。19例进行了CT扫描，13例进行了MRI检查，分别有76.3%和53.8%存在中线缺损。65%的婴儿有单独的中央巨切牙。半数婴儿在平均74.8日龄时接受了手术干预，其中90%需要翻修手术。手术组和非手术组患者的梨状孔距离无差异（分别为5.4毫米和5.2毫米，p = 0.6）。没有特定变量可预测初次手术或翻修手术的需求。在患有CNPAS婴儿的母亲中，55%患有妊娠糖尿病（MDM）。
梨状孔距离不是手术干预的预测因素。由于颅内并发症发生率高，所有患有CNPAS的婴儿均应考虑进行MRI检查。MDM可能是CNPAS的一个危险因素。
