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Abstract：
Introduction The risk of skull base injury during choanal atresia repair can be mitigated via thorough understanding of skull base anatomy. There is a paucity of data describing differences in skull base anatomy between patients with coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear abnormalities (CHARGE) syndrome and those without. Objectives The aim of this study was to measure nasal and skull base anatomy in patients with isolated bilateral choanal atresia (BCA), CHARGE syndrome, and other syndromic congenital anomalies. Methods Retrospective chart review of patients with bilateral choanal atresia and computed tomography of the face between 2001 and 2019 were evaluated. Choanal width, height, mid-nasal height, and skull base slope were measured radiographically. Differences in anatomy between healthy patients, those with CHARGE syndrome, and those with other congenital anomalies were compared. Results Twenty-one patients with BCA and relevant imaging were identified: 7 with isolated BCA, 6 with CHARGE syndrome, and 8 with other congenital anomalies. A t -test indicated insignificant difference in skull base slope, choanal height, choanal width, or mid-nasal skull base height between isolate BCA cases and patients with any congenital anomaly. When comparing CHARGE to isolated BCA cases, mid-nasal height was shorter in CHARGE patients ( p = 0.03). There were no differences in measurements between patients with congenital anomalies excluding CHARGE ( p > 0.05). Two patients in the congenital anomaly group were found to have bony skull base defects preoperatively. Conclusion This study represents the largest description of skull base and nasal anatomy in patients with CHARGE syndrome and BCA. Surgeons should be aware of the lower skull base in CHARGE patients to avoid inadvertent skull base injury.
摘要：
简介通过彻底了解颅底解剖，可以减轻后鼻孔闭锁修复过程中颅底损伤的风险。缺乏描述结肠瘤患者颅底解剖结构差异的数据，心脏缺陷,锁骨闭锁,生长迟缓,生殖器异常,和耳朵异常（CHARGE）综合征和那些没有。目的本研究的目的是测量孤立的双侧后鼻孔闭锁（BCA）患者的鼻和颅底解剖，CHARGE综合征,和其他综合征性先天性异常。方法回顾性分析2001年至2019年双侧后鼻孔闭锁和面部计算机断层扫描的患者。Choanal宽度，高度,鼻中高,和颅底坡度进行了射线照相测量。健康患者之间的解剖学差异，那些患有CHARGE综合征的人，和其他先天性异常的人进行了比较。结果21例BCA和相关影像学检查结果：7例BCA孤立，6患有CHARGE综合征，和其他先天性异常8。t检验表明颅底斜率无显著性差异，后鼻孔高度,后鼻孔宽度，或孤立的BCA病例和任何先天性异常患者之间的鼻中颅底高度。当将费用与孤立的BCA病例进行比较时，CHARGE患者的鼻中高度较短(p=0.03).不包括CHARGE的先天性异常患者之间的测量值没有差异（p>0.05）。先天性异常组的两名患者术前发现骨性颅底缺损。结论这项研究代表了CHARGE综合征和BCA患者颅底和鼻解剖的最大描述。外科医生应注意CHARGE患者的下颅底，以避免意外的颅底损伤。
