Fitzpatrick Nicholas S, Bartley Anthony C, Bekhit Elhamy, Berkowitz Robert G
Department of Otolaryngology, Royal Children's Hospital Melbourne, Parkville, Victoria, Australia.
Medical Imaging Department, Royal Children's Hospital Melbourne, Parkville, Victoria, Australia.
Int J Pediatr Otorhinolaryngol. 2018 Dec;115:61-64. doi: 10.1016/j.ijporl.2018.09.009. Epub 2018 Sep 14.
Bilateral choanal atresia (BCA) is associated with a high incidence of congenital abnormalities that include skull base anomalies and defects. Surgical repair of BCA is necessary in the early neonatal period and any altered anatomy of the adjacent skull base will heighten the risk of intracranial injury. This risk may be further increased in patients with CHARGE syndrome.
To measure surgically relevant nasal and skull base dimensions in neonates with BCA in order to determine whether any difference exists between isolated and CHARGE syndrome associated subgroups, thereby optimizing the safety of surgical repair.
A retrospective review of medical charts and computed tomography was undertaken at a tertiary pediatric hospital of all neonates diagnosed with BCA between 2004 and 2016. Isolated and CHARGE syndrome subgroups of BCA were identified from clinical records and CT data was analyzed and compared between the two. The skull base parameters measured were choanal width, choanal height, mid-nasal skull base height and skull base slope.
Of the 13 patients included, 3 had CHARGE syndrome and 10 had isolated BCA. Whilst the difference in mid-nasal height approached significance for the two groups, numbers were too small for a statistical difference to be identified. The mean value for choanal width in the isolated BCA group was significantly less the largest series of normative data available in the literature for comparison (p < 0.001). No skull base anomalies were noted in either group.
While this is a small study with limited numbers, it is the first that has attempted to identify and measure the posterior nasal and skull base anatomy most pertinent to avoiding skull base injury in the surgical management of BCA.
双侧后鼻孔闭锁（BCA）与包括颅底异常和缺损在内的先天性异常的高发生率相关。BCA的手术修复在新生儿早期是必要的，相邻颅底的任何解剖结构改变都会增加颅内损伤的风险。患有CHARGE综合征的患者这种风险可能会进一步增加。
测量患有BCA的新生儿的手术相关鼻腔和颅底尺寸，以确定孤立性和CHARGE综合征相关亚组之间是否存在差异，从而优化手术修复的安全性。
在一家三级儿科医院对2004年至2016年间所有诊断为BCA的新生儿的病历和计算机断层扫描进行回顾性研究。从临床记录中识别出BCA的孤立性和CHARGE综合征亚组，并对两者的CT数据进行分析和比较。测量的颅底参数包括后鼻孔宽度、后鼻孔高度、鼻中部颅底高度和颅底斜率。
纳入的13例患者中，3例患有CHARGE综合征，10例患有孤立性BCA。虽然两组鼻中部高度的差异接近显著，但由于样本数量太少，无法确定统计学差异。孤立性BCA组的后鼻孔宽度平均值明显小于文献中可获得的最大系列规范数据（p<0.001）。两组均未发现颅底异常。
虽然这是一项样本量有限的小型研究，但它是首次尝试识别和测量在BCA手术管理中与避免颅底损伤最相关的后鼻腔和颅底解剖结构的研究。
