OBJECT Lipomyelomeningocele (LMM) is a congenital spinal cord anomaly. While patients with LMM may initially be asymptomatic, neurological sequelae secondary to LMM may become apparent as the patient ages. Consequently, some pediatric neurosurgeons have advocated for upfront neurosurgical interventions irrespective of the presence of symptoms at diagnosis. By contrast, others pursue a conservative approach when overt neurological symptoms are not yet evident. In light of the various practice styles to the heterogeneous anatomical locations, symptoms, and ages associated with LMM, we have conducted a multi-center survey of Canadian pediatric neurosurgeons using clinical vignettes representative of LMM patients. METHODS An online survey of the opinions of Canadian pediatric neurosurgeons was conducted using 5 separate cases with magnetic resonance imaging (MRI) scans of the lumbar spine. Each case was accompanied with the same three clinical vignettes, which varied in severity at time of presentation: asymptomatic, progressive somatic motor deficit, or longstanding overflow incontinence. Participants were asked the question, "Would you offer surgical management?" after each clinical vignette. After the five cases and their corresponding 3 clinical vignettes, participants were asked, "If you answered yes to any of the preceding questions, what type of surgery would you perform?". Options for surgical goals and techniques included complete removal, near-total removal, debulking, detethering, and expansile duroplasty. Surgical adjuncts included CUSA, LASER, and neurophysiologic monitoring. RESULTS Twenty-three responses were received from the 38 questionnaires sent out to all staff pediatric neurosurgeons across academic medical centers in Canada. This represented a response rate of 61%. Canadian pediatric neurosurgeons generally maintain a conservative approach to the surgical management of LMM as only 13% (n = 3) of surgeons indicated that they would operate in all scenarios. By contrast, 43% (n = 10) indicated surgical management in only those cases presenting with symptoms, and another 43% (n = 10) displayed a variable surgical approach. Nine percent (n = 2) of participants would not perform surgery for incontinence. The greatest level of disagreement among participants pertained to the management of asymptomatic sacral LMM where 43% of participants favored prophylactic surgery, while 57% of participants preferred conservative management. CONCLUSIONS The current study highlights the differences in management of LMM among Canadian pediatric neurosurgeons and provides further support for future prospective cohort studies to develop appropriate expert opinions and guidelines such that the care of LMM patients may be according to evidence-based best practice. This is especially true for the treatment of asymptomatic patients, a patient group that would benefit from a randomized controlled trial to assess the long-term outcomes of conservative and surgical management.

中文翻译：

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加拿大圆锥脂肪瘤的神经外科治疗：一项多中心调查。

对象 脊髓脊膜膨出 (LMM) 是一种先天性脊髓异常。虽然 LMM 患者最初可能无症状，但随着患者年龄的增长，继发于 LMM 的神经系统后遗症可能会变得明显。因此，一些儿科神经外科医生主张无论诊断时是否存在症状，都应进行前期神经外科干预。相比之下，当明显的神经系统症状尚不明显时，其他人则采用保守的方法。鉴于与 LMM 相关的异质解剖位置、症状和年龄的各种实践方式，我们使用代表 LMM 患者的临床小插曲对加拿大儿科神经外科医生进行了一项多中心调查。方法 对加拿大儿科神经外科医生的意见进行在线调查，使用 5 个独立的病例进行腰椎磁共振成像 (MRI) 扫描。每个病例都伴随着相同的三个临床小插曲，其在就诊时的严重程度各不相同：无症状、进行性躯体运动障碍或长期溢出性尿失禁。参与者被问到一个问题，“你会提供手术治疗吗？” 在每个临床小插曲之后。在 5 个病例及其对应的 3 个临床小插曲之后，参与者被问到：“如果您对上述任何一个问题的回答是肯定的，您会进行哪种类型的手术？”。手术目标和技术的选择包括完全切除、几乎完全切除、减瘤、去束缚和可膨胀硬脑膜成形术。手术辅助包括 CUSA、激光和神经生理监测。结果 从向加拿大学术医疗中心的所有儿科神经外科医生发出的 38 份问卷中收到了 23 份答复。这表示响应率为 61%。加拿大儿科神经外科医生通常对 LMM 的手术治疗持保守态度，因为只有 13% (n = 3) 的外科医生表示他们将在所有情况下进行手术。相比之下，43% (n = 10) 仅对出现症状的病例表示手术治疗，另有 43% (n = 10) 表示手术方法不一。百分之九（n = 2）的参与者不会因失禁而进行手术。参与者之间最大程度的分歧与无症状骶骨 LMM 的管理有关，其中 43% 的参与者赞成预防性手术，而 57% 的参与者倾向于保守治疗。结论 目前的研究突出了加拿大儿科神经外科医生在 LMM 管理方面的差异，并为未来的前瞻性队列研究提供了进一步的支持，以制定适当的专家意见和指南，以便 LMM 患者的护理可以根据循证最佳实践进行。对于无症状患者的治疗尤其如此，该患者组将从随机对照试验中受益，以评估保守和手术治疗的长期结果。结论 目前的研究突出了加拿大儿科神经外科医生在 LMM 管理方面的差异，并为未来的前瞻性队列研究提供了进一步的支持，以制定适当的专家意见和指南，以便 LMM 患者的护理可以根据循证最佳实践进行。对于无症状患者的治疗尤其如此，该患者组将从随机对照试验中受益，以评估保守和手术治疗的长期结果。结论 目前的研究突出了加拿大儿科神经外科医生在 LMM 管理方面的差异，并为未来的前瞻性队列研究提供了进一步的支持，以制定适当的专家意见和指南，以便 LMM 患者的护理可以根据循证最佳实践进行。对于无症状患者的治疗尤其如此，该患者组将从随机对照试验中受益，以评估保守和手术治疗的长期结果。