Skull Base Anatomy in Patients with Bilateral Choanal Atresia: A Radiographic Study

Abstract Background 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 背景：通过对颅底解剖结构的透彻了解，可以降低后鼻孔闭锁修复过程中颅底损伤的风险。描述眼缺损、心脏缺陷、后鼻孔闭锁、生长迟缓、生殖器异常和耳部异常（CHARGE）综合征患者与非综合征患者颅底解剖结构差异的数据很少。 目的：本研究旨在测量孤立性双侧后鼻孔闭锁（BCA）、CHARGE综合征以及其他先天性综合征患者的鼻腔和颅底解剖结构。 方法：对2001年至2019年间双侧后鼻孔闭锁患者的病历进行回顾性分析，并对其面部计算机断层扫描（CT）结果进行评估。通过影像学方法测量后鼻孔宽度、高度、鼻中部高度以及颅底斜率。比较健康患者、CHARGE综合征患者以及其他先天性异常患者之间的解剖结构差异。 结果：确定了21例具有BCA且有相关影像学资料的患者：7例为孤立性BCA，6例为CHARGE综合征，8例为其他先天性异常。t检验表明，孤立性BCA病例与任何先天性异常患者在颅底斜率、后鼻孔高度、后鼻孔宽度或鼻中部颅底高度方面差异无统计学意义。当比较CHARGE综合征患者与孤立性BCA病例时，CHARGE综合征患者的鼻中部高度较短（p = 0.03）。不包括CHARGE综合征的先天性异常患者之间的测量值无差异（p > 0.05）。在先天性异常组中有2例患者术前发现有骨性颅底缺陷。 结论：本研究是对CHARGE综合征和BCA患者颅底和鼻腔解剖结构的最全面描述。外科医生应了解CHARGE综合征患者的颅底较低这一情况，以避免意外的颅底损伤。