Pool Christopher, Slonimsky Einat, Nayak Roshan, Engle Lisa, Zhu Junjia, Wilson Meghan
Department of Otolaryngology - Head and Neck Surgery, Penn State Hershey Medical Center, Hershey, Pennsylvania, United States.
Department of Radiology, Penn State Hershey Medical Center, Hershey, Pennsylvania, United States.
J Neurol Surg B Skull Base. 2021 Feb 4;83(3):223-227. doi: 10.1055/s-0040-1722230. eCollection 2022 Jun.
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. 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. 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. 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 -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 ( = 0.03). There were no differences in measurements between patients with congenital anomalies excluding CHARGE ( > 0.05). Two patients in the congenital anomaly group were found to have bony skull base defects preoperatively. 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.
通过全面了解颅底解剖结构，可降低后鼻孔闭锁修复术中颅底损伤的风险。目前关于患有眼组织缺损、心脏缺陷、后鼻孔闭锁、生长发育迟缓、生殖器畸形和耳部畸形（CHARGE）综合征的患者与未患该综合征的患者之间颅底解剖结构差异的数据较少。 本研究的目的是测量孤立性双侧后鼻孔闭锁（BCA）、CHARGE综合征和其他综合征性先天性异常患者的鼻腔和颅底解剖结构。 对2001年至2019年间双侧后鼻孔闭锁患者的回顾性病历审查及面部计算机断层扫描进行了评估。通过影像学测量后鼻孔宽度、高度、鼻中高度和颅底斜率。比较了健康患者、CHARGE综合征患者和其他先天性异常患者之间的解剖结构差异。 确定了21例患有BCA并进行了相关影像学检查的患者：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之间在颅底斜率、后鼻孔高度、后鼻孔宽度或鼻中颅底高度方面无显著差异。将CHARGE综合征患者与孤立性BCA病例进行比较时，CHARGE综合征患者的鼻中高度较短（P = 0.03）。排除CHARGE综合征的先天性异常患者之间的测量结果无差异（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损。 本研究是对CHARGE综合征和BCA患者颅底及鼻腔解剖结构的最大规模描述。外科医生应注意CHARGE综合征患者的较低颅底，以避免意外的颅底损伤。
