Introduction: The reported prevalence of tethered spinal cord in patients with anorectal malformations (ARMs) ranges from 9% to 64%. Practice patterns surrounding the diagnosis and management of tethered cord (TC) are suspected to vary, with consideration to the type of spine imaging, adjunct imaging modalities, what patients are offered surgical intervention, and how patients are followed after detethering. We sought to determine what consensus, if any, exists among pediatric neurosurgeons in the USA in terms of diagnosis and management of TC and, specifically, patients with TC and ARM. Methods: A survey was sent to members of the American Society of Pediatric Neurosurgeons (ASPN). Members of the ASPN received an email with a link to an anonymous REDCap survey that asked about their experience with detethering procedures, indications for surgery, diagnostic tools used, and follow-up protocols. Results: The survey was completed by 93 of the 192 ASPN members (48%). When respondents were asked about the total number of all simple filum detetherings they performed annually, 61% (N = 57) indicated they perform less than 10 for all TC patients. Ninety-three percent (N = 87) of neurosurgeons performed these procedures in patients with simple filum TC and ARM patients (TC + ARM) specifically. When asked about prophylactic detethering in those with a confirmed diagnosis of low-lying conus and with a filum fatty terminale, 59.1% (N = 55) indicated they would offer this to TC + ARM patients regardless of their age. Regarding preoperative workup for simple filum detethering, all respondents indicated they would order an MRI in both TC and TC + ARM patients, with a minority also requiring additional testing such as urodynamics, neurodevelopmental assessments, and anorectal manometry for both groups. When following patients postoperatively, almost all respondents indicated they would require clinical neurosurgical follow-up with a clinic visit (100% in all simple filum TC patients, 98.9% in fatty filum/low-lying conus TC + ARM patients), but there was wide variation in the use of other tools such as urological testing, neurodevelopmental assessment, and anorectal manometry. Discussion/Conclusions: A wide variety of diagnostic criteria and indication for procedural intervention exists for management of TC patients with and without ARM. Further studies are needed to determine outcomes. Prospective protocols need to be developed and evaluated to standardize care for this patient population and determine best practices.


中文翻译：

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肛门直肠畸形中脐带栓系的检测和处理：美国小儿神经外科医生的调查

简介：据报道，肛门直肠畸形 (ARM) 患者脊髓栓系的发生率为 9% 至 64%。围绕脊髓栓系 (TC) 的诊断和管理的实践模式可能会有所不同，考虑到脊柱成像的类型、辅助成像方式、向哪些患者提供手术干预，以及患者在松解后的随访方式。我们试图确定美国儿科神经外科医生在 TC 尤其是 TC 和 ARM 患者的诊断和管理方面是否存在共识（如果有的话）。方法：向美国儿科神经外科医生协会 (ASPN) 的成员发送了一项调查。ASPN 的成员收到了一封电子邮件，其中包含指向匿名 REDCap 调查的链接，该调查询问了他们在松解程序、手术适应症、使用的诊断工具和后续协议方面的经验。结果：调查由 192 名 ASPN 成员中的 93 名 (48%) 完成。当受访者被问及他们每年执行的所有简单终丝松解术的总数时，61% ( N = 57) 表示他们对所有 TC 患者执行的次数少于 10 次。百分之九十三（N= 87) 的神经外科医生专门对患有单纯丝状肌 TC 和 ARM 患者 (TC + ARM) 的患者进行了这些手术。当被问及对确诊为低位圆锥和脂肪终丝的患者进行预防性松解时，59.1% ( N= 55) 表示他们会向 TC + ARM 患者提供此服务，无论他们的年龄如何。关于简单的纵丝松解术的术前检查，所有受访者都表示他们会为 TC 和 TC + ARM 患者安排 MRI，少数人还需要额外的测试，例如两组的尿动力学、神经发育评估和肛门直肠测压。在术后随访患者时，几乎所有受访者都表示他们需要进行临床神经外科随访并进行门诊就诊（所有单纯丝状 TC 患者为 100%，脂肪丝/低位圆锥 TC + ARM 患者为 98.9%），但有其他工具的使用差异很大，例如泌尿科测试、神经发育评估和肛门直肠测压。讨论/结论：对于伴有和不伴有 ARM 的 TC 患者的管理，存在多种诊断标准和程序干预指征。需要进一步的研究来确定结果。需要制定和评估前瞻性协议，以标准化对这一患者群体的护理并确定最佳实践。