Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, India.
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, India.
World Neurosurg. 2019 Jun;126:346-353. doi: 10.1016/j.wneu.2019.02.143. Epub 2019 Mar 6.
Idiopathic spinal cord herniation (ISCH) is a treatable spinal disease. It is rare and often misdiagnosed, causing a delay in management. The etiology is multifactorial, with one of the causes being duplication of the ventral dura. The spinal cord herniates within this defect and becomes strangulated, causing neurological deficits. We report the duplication of a ventral spinal cord as an important cause of ISCH in our review.
We present 2 cases of ISCH with duplication of the dura, including their relevant clinical and imaging features. The patients underwent surgical reduction of the herniated spinal cord with enlargement of the defect and placement of a dural substitute ventral to the cord. We have also reported the outcomes of the 2 patients, with an emphasis on the factors predictive of poor outcomes (i.e., long-standing symptoms, a delay in intervention, poor neurological status at presentation, and a thinned out atrophic spinal cord found during surgery). We also reviewed the available data for duplication of the dura with ISCH.
Very few asymptomatic patients can be treated conservatively. The surgical outcomes have been favorable for symptomatic patients. Proper exposure, gentle manipulation while reducing the herniated spinal cord, enlargement of the defect, and the use of intraoperative monitoring will help limit the postoperative deficits. Duplication of the ventral dura is an important cause of ISCH. It prevents the formation of an anterior pseudomeningocele after surgery. Owing to the rarity of the disease and the lack of follow-up data with recurrence rates, it has not been possible to form clear guidelines for management.
特发性脊髓疝（ISCH）是一种可治疗的脊髓疾病。它很少见，经常被误诊，导致治疗延误。病因是多因素的，其中一个原因是腹侧硬脑膜的重复。脊髓在这个缺陷内疝出并被绞窄，导致神经功能缺损。我们在综述中报告了硬脑膜重复是 ISCH 的一个重要原因。
我们介绍了 2 例 ISCH 伴硬脑膜重复的病例，包括其相关的临床和影像学特征。患者接受了手术复位疝出的脊髓，扩大了缺陷，并在脊髓腹侧放置了硬脑膜替代物。我们还报告了这 2 例患者的结局，重点介绍了预测不良结局的因素（即，症状持续时间长、干预延迟、就诊时神经状态差、手术中发现脊髓变薄和萎缩）。我们还回顾了硬脑膜重复伴 ISCH 的可用数据。
极少数无症状患者可以保守治疗。有症状的患者的手术结果是有利的。适当的暴露、在复位疝出的脊髓时轻柔操作、扩大缺陷以及使用术中监测将有助于减少术后缺损。腹侧硬脑膜重复是 ISCH 的一个重要原因。它可防止术后形成前假性脑膜膨出。由于疾病的罕见性和缺乏复发率的随访数据，尚不可能为管理制定明确的指南。
