Untethering

解除网络共享
  • 文章类型: Journal Article
    背景:末端紧纤维是一种以各种症状为特征的神经系统疾病,包括肌肉无力.值得注意的是缺乏有关肌肉无力的文献,特别是在青春期及以后出现的病例。由于缺乏放射学异常,诊断具有挑战性,以及有关其治疗的文献,尤其是解开束缚,成年人是有限的。这项研究旨在评估松绑在改善被诊断为终末紧的青春期后患者肌肉无力和其他症状方面的有效性。
    方法:对2018年1月至2022年8月期间在我们机构接受松绑的7名被诊断为终丝紧绷并表现出肌肉无力的青春期后患者进行了回顾性分析。监测患者的肌肉力量改善情况,腰椎和下肢疼痛,和肠和膀胱功能障碍(BBD)后解开。
    结果:松绑后,所有病例的肌无力均得到改善,改善的平均持续时间为9.1周。无法独立行走的患者平均在22.3周内恢复了活动能力。所有病例的腰椎和下肢疼痛在平均8.1周内得到改善,而BBD在平均1.9周内改善了7例中的6例。
    结论:我们的研究结果表明,对于诊断为终丝紧绷并表现为肌肉无力的青春期后患者,松脱是一种有效的手术干预措施。
    BACKGROUND: Tight filum terminale is a neurological condition marked by various symptoms, including muscle weakness. There is a notable lack of literature addressing muscle weakness, particularly in cases emerging during adolescence and beyond. The diagnosis is challenging due to a lack of radiological abnormalities, and the literature on its treatment, especially untethering, in adults is limited. This study aims to evaluate the effectiveness of untethering in improving muscle weakness and other symptoms in postadolescent patients diagnosed with tight filum terminale.
    METHODS: A retrospective analysis was conducted on seven postadolescent patients diagnosed with tight filum terminale and presenting muscle weakness who underwent untethering at our institution between January 2018 and August 2022. Patients were monitored for muscle strength improvement, lumbar and lower extremity pain, and bowel and bladder dysfunction (BBD) after untethering.
    RESULTS: Muscle weakness improved in all cases after untethering, with a mean duration of 9.1 weeks for the improvement. Patients unable to walk independently regained mobility in an average of 22.3 weeks. Lumbar and lower limb pain improved in all cases within an average of 8.1 weeks, while BBD improved in six of the seven cases within an average of 1.9 weeks.
    CONCLUSIONS: Our findings suggest that untethering is an effective surgical intervention for postadolescent patients diagnosed with tight filum terminale and presenting muscle weakness.
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  • 文章类型: Journal Article
    脊髓膜膨出(MMC)修复后,几乎所有患者都会出现继发性脊髓栓系。束缚的脊髓可能导致进行性神经系统恶化和行走障碍。这项回顾性队列研究旨在强调栓系脐带释放后一年的步行恢复及其与术前圆锥水平的关系。我们回顾了2014年1月至2022年12月在我们大学医院的病历。包括腰骶部MMC修复后进行脊髓松脱的患者。我们使用改良的Benzel量表评估了脐带释放后一年的步行恢复情况。37名患者符合我们的选择标准。有19个女孩(51.4%)和18个男孩(48.6%)。他们的平均年龄为8.6岁。术前圆锥椎体水平介于L4和S3之间。脊髓松解术后一年,37.8%的患者恢复了行走能力。所有术前圆锥水平为S2或S3的患者均恢复了步行能力。相比之下,所有术前圆锥水平为L4或L5的患者均未恢复行走能力.圆锥处于S1水平的患者中有三分之一(33.3%)在脐带释放一年后恢复了行走能力。系绳释放一年后,37.8%的患者恢复了行走能力。我们发现步行恢复与术前圆锥水平有统计学关联。需要多中心前瞻性研究来支持本研究的结果。
    After myelomeningocele (MMC) repair, a secondary tethered spinal cord occurs in almost all patients. The tethered spinal cord may result in progressive neurological deterioration and walking disability. This retrospective cohort study aimed to highlight the walking recovery one year after tethered cord release and its relation to the preoperative conus level. We reviewed the medical records at our university hospital from January 2014 to December 2022. The patients who underwent spinal cord untethering following lumbosacral MMC repair were included. We assessed the walking recovery one year after cord release using the modified Benzel scale. Thirty-seven patients met our selection criteria. There were 19 girls (51.4%) and 18 boys (48.6%). Their mean age at presentation was 8.6 years. The preoperative conus vertebral levels ranged between L4 and S3. One year after spinal cord release, 37.8% of the patients regained their walking ability. All the patients whose preoperative conus level was at S2 or S3 regained their walking ability. In contrast, all the patients with preoperative conus levels at L4 or L5 didn\'t regain their ability to walk. One-third (33.3%) of patients whose conus was at the S1 level regained their walking ability one year after cord release. One year after tethered cord release, 37.8% of the patients regained their walking ability. We found that the walking recovery was statistically associated with the preoperative conus level. A multicenter prospective study is required to support the results of this study.
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  • 文章类型: Case Reports
    据报道,腰椎管狭窄(LCS)是脊髓栓系的诱因,在儿童时期是无症状的,在成年期发展为脊髓栓系综合征(TCS)。然而,只有少数关于此类病例手术策略的报告.大约1年前,一名64岁的妇女在左臀部和大腿背侧出现难以忍受的疼痛。磁共振成像显示,由于L4-5椎骨水平的黄韧带增厚,脊髓与丝型脊髓脂肪瘤和LCS的束缚。行减压椎板切除术治疗LCS5个月后,在S4水平的硬脑膜死角处进行了解开手术。切断的纤维末端被向上抬高了7毫米,术后疼痛减轻。该病例研究表明,对于由LCS引发的成人发作的TCS,应进行两种病变的手术。
    Lumbar canal stenosis (LCS) has been reported as a precipitating factor by which a tethered spinal cord, which is asymptomatic during childhood, develops into tethered cord syndrome (TCS) in adulthood. However, only a few reports on surgical strategies for such cases are available. A 64-year-old woman presented with unbearable pain in the left buttock and dorsal aspect of the thigh approximately 1 year ago. Magnetic resonance imaging showed cord tethering with a filar-type spinal lipoma and LCS due to the thickening of the ligamentum flavum at the L4-5 vertebral level. Five months after the decompressive laminectomy for the treatment of LCS, an untethering surgery was performed at the dural cul-de-sac at the S4 level. The severed end of the filum was elevated rostrally by 7 mm, and the pain subsided postoperatively. This case study shows that surgeries for both lesions should be indicated for adult-onset TCS triggered by LCS.
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  • 文章类型: Journal Article
    目的:对于先天性腰骶部脊柱病变,复位并不是一种不寻常的手术。本研究旨在评估一种预防束缚的新手术技术。
    方法:解开脊髓后,圆锥延髓尾端的软脑膜或疤痕组织用8-0线松散地固定在腹侧硬脑膜上,硬脑膜直接闭合。这种技术被称为腹侧锚定。
    结果:在15例患者(年龄5至37岁,平均年龄:12.1岁),2014年至2021年。除一名患者外,所有患者均显示术前症状改善或稳定。未观察到与手术直接相关的并发症。术后MRI显示,14例患者的背侧蛛网膜下腔得以恢复,但在随访MRI中,3例患者无法检测到或缺失。在随访期间,没有患者出现脊髓栓系综合征的复发。
    结论:腹侧锚定对于解开脊髓后恢复背侧蛛网膜下腔是有效的。这项初步研究表明,腹侧锚固有可能预防先天性腰骶部脊柱病变患者的脊髓栓系术后影像学复发。
    Retethering is not an unusual operation for a congenital lumbosacral dysraphic spinal lesion. The present study aimed to assess a new surgical technique for preventing retethering.
    After untethering the spinal cord, the pia mater or scar tissue at the caudal end of the conus medullaris is anchored to the ventral dura mater loosely using 8-0 thread, and the dura mater is closed directly. This technique is called ventral anchoring.
    Ventral anchoring was performed in 15 patients (aged 5 to 37 years old, average age: 12.1 years old) between 2014 and 2021. All but one patient showed improvement or stabilization of the preoperative symptoms. No complication directly related to the procedure was observed. Postoperative MRI demonstrated that the dorsal subarachnoid space was restored in 14 patients but was undetectable or absent in three patients on follow-up MRI. No patients have experienced a recurrence of the tethered cord syndrome during the follow-up period.
    Ventral anchoring is effective for restoring the dorsal subarachnoid space after untethering the spinal cord. This preliminary study suggested that ventral anchoring has the potential to prevent the postoperative radiographic recurrence of tethered spinal cord in patients with a congenital lumbosacral dysraphic spinal lesion.
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  • 文章类型: Journal Article
    目的:在最初的松脱手术后可发生脐带的复位。在儿科患者中通常难以确定指示脐带束缚的典型神经系统表现。接受过原发性松绑手术的患者可能会由于先前的栓系事件而出现一定程度的神经功能缺损。尿动力学检查(UDS)和脊柱图像经常异常。因此,需要更客观的工具来检测网络共享。本研究试图描述系链EDS的特点,因此,可以支持再系的诊断。
    方法:在692名进行了松绑手术的受试者中,我们回顾性地提取了93例临床上怀疑重新连接的受试者的数据.受试者分为两组,一个束缚的团体,和一个非进展组,根据是否进行了手术干预。两个连续的EDS,临床发现,脊柱磁共振成像扫描,回顾并比较新的系留症状发展之前的UDS。
    结果:在肌电图(EMG)研究中,在再系组中,新肌肉异常自发活动(ASA)的出现明显(p<0.01)。非进展组的ASA丢失更为明显(p<0.01)。肌电图的特异性和灵敏度分别为80.4和56.5%,分别。在神经传导研究中,两组没有差异.两组之间的纤颤电位大小没有差异。
    结论:为了为临床医生关于重新连接的决定提供支持,当结果与先前的EDS结果相比时,EDS可以是具有高特异性的有利工具。建议将术后常规随访EDS作为基线,以进行临床怀疑重新连接时的比较。
    Retethering of the cord can occur after the initial untethering surgery. Typical neurological manifestations indicative of cord tethering are often difficult to determine in pediatric patients. Patients who had a primary untethering operation are likely to present with some degree of neurological deficits from a previous tethering event, and urodynamic studies (UDSs) and spine images are frequently abnormal. Therefore, more objective tools to detect retethering are needed. This study sought to delineate the characteristics of EDS of retethering, and therefore, could support the diagnosis of retethering.
    Among 692 subjects who had an untethering operation, data from 93 subjects who had been suspected of retethering clinically were retrospectively extracted. The subjects were divided into two groups, a retethered group, and a non-progression group, according to whether or not surgical interventions had been performed. Two consecutive EDSs, clinical findings, spine magnetic resonance imaging scans, and UDSs before the development of new tethering symptoms were reviewed and compared.
    In the electromyography (EMG) study, the appearance of abnormal spontaneous activity (ASA) in new muscles was prominent in the retethered group (p < 0.01). The loss of ASA was more pronounced in the non-progression group (p < 0.01). Specificity and sensitivity of EMG for retethering were 80.4 and 56.5%, respectively. In the nerve conduction study, the two groups did not show differences. The size of fibrillation potential was not different between the groups.
    To provide support for a clinician\'s decision on retethering, EDS could be an advantageous tool with high specificity when the results are compared to previous EDS results. Routine follow-up EDS post-operatively is recommended as a baseline for comparison at the time when retethering is clinically suspected.
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  • 文章类型: Multicenter Study
    目的:脊髓栓系综合征(TCS)可导致严重的下游神经功能缺损,包括步态恶化,失禁,和经常无法解释的慢性腰痛。手术干预可以缓解症状,但没有明确的与手术结果和功能状态相关的放射学参数.作者旨在定义评估TCS手术结果的术前和术后放射学参数。
    方法:作者对2016年至2021年接受TCS治疗的所有儿科患者进行了单中心回顾性研究。患者基线特征和手术指标包括年龄,性别,圆锥的水平,程序级别,束缚病理学,出现时的症状,并发症,改善症状,和再手术率。MRI测量包括术前和术后前管距离(ACD)和弯曲角度(BA)。
    结果:确定了33例儿科患者,他们在2016年至2021年之间进行了脊髓松脱,并进行了术前和术后MRI检查。患者平均年龄为5.64±5.33岁。20例(60.60%)为女性。关于松绑的地点,在腰骶部区域进行了31次手术(93.93%),在胸腰椎区域进行了2次手术(6.06%)。21.21%的患者发现延髓圆锥高于L3。术后,18.18%的患者出现并发症,48.48%的人症状有所改善,48.48%的患者模棱两可或有持续性症状。术前平均ACD0(从椎体后缘[中]到延髓前缘测量)为6.15±3.18mm,术后ACD0为2.25±2.72mm,ACD0的平均变化为-0.90±1.31mm。术前平均BA为26.00°±11.56°,术后平均BA为15.92°±9.81°,BA的平均变化为-10.08°±8.80°。术前BA预测接受手术的复杂TCS患儿再次手术的最佳临界值为≥31.70°(曲线下面积=0.83)。
    结论:在手术治疗的TCS患者中,某些术前放射学参数对于预测术后手术结局可能很重要;可以对这些参数进行评估和报告,以提示并发症高危患者.有必要进行进一步的前瞻性多中心研究,以提供可靠的证据证明TCS患者预后与术前放射学参数之间的关联。
    Tethered cord syndrome (TCS) can lead to significant downstream neurological deficits including gait deterioration, incontinence, and often unexplained chronic low-back pain. Surgical intervention may relieve symptoms, but there are no defined radiological parameters associated with surgical outcomes and functional status. The authors aimed to define pre- and postoperative radiological parameters for assessing surgical outcomes in TCS.
    The authors performed a single-center retrospective review of all pediatric patients treated for TCS between 2016 and 2021. Patient baseline characteristics and operative metrics included age, sex, level of conus, level of procedure, tethering pathology, symptoms at presentation, complications, improvement of symptoms, and reoperation rate. MRI measurements included pre- and postoperative anterior canal distance (ACD) and bending angle (BA).
    Thirty-three pediatric patients were identified who underwent untethering of the spinal cord and had pre- and postoperative MRI between 2016 and 2021. The mean patient age was 5.64 ± 5.33 years. Twenty patients (60.60%) were female. Regarding the site of untethering, 31 procedures (93.93%) were performed at the lumbosacral region and 2 (6.06%) were performed at the thoracolumbar region. The conus medullaris was found above L3 in 21.21% of patients. Postoperatively, 18.18% of patients experienced complications, 48.48% showed improvement in their symptoms, and 48.48% were equivocal or had persistent symptoms. The mean preoperative ACD0 (measured from the posterior vertebral body margin [middle] to the anterior margin of the conus medullaris) was 6.15 ± 3.18 mm, the postoperative ACD0 was 2.25 ± 2.72 mm, and the average change in ACD0 was -0.90 ± 1.31 mm. The mean preoperative BA was 26.00° ± 11.56°, the mean postoperative BA was 15.92° ± 9.81°, and the average change in BA was -10.08° ± 8.80°. An optimal cutoff value for preoperative BA to predict reoperation in pediatric patients with complex TCS undergoing surgery was ≥ 31.70° (area under the curve = 0.83).
    In surgically treated patients with TCS, certain preoperative radiological parameters may be important in predicting postoperative surgical outcomes; these parameters can be evaluated and reported to indicate patients at high risk for complications. Further prospective multicenter research is warranted to offer robust evidence of association of patient outcomes with preoperative radiological parameters in TCS.
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  • 文章类型: Journal Article
    背景:脑膜脊髓囊肿原发性闭合后的TCS是脊柱裂疾病的已知并发症。SSCU手术后的结果数据是异质的,缺乏标准化。因此,我们旨在找到一种可靠的系统来评估SSCU手术前后的膀胱功能并记录术后结果。
    方法:对一组诊断为脊柱裂的患者进行回顾性研究。总的来说,130名患者接受了182例SSCU手术,其中56个符合我们的纳入标准。一个分类系统,包括两种不同的方法,被使用。AC系统使用基线压力和逼尿肌超过活动来定义膀胱功能障碍的三个水平。第二种方法通过将膀胱容量从0到2分授予膀胱功能障碍的严重程度,自主收缩期间逼尿肌的最大压力,漏点压力和膀胱输尿管反流得分高与严重膀胱功能障碍相关。
    结果:性别分布相同(男性:n=29;51.8%;女性:n=27;48.2%)。SSCU的中位年龄为902岁(范围为0.5-22.8岁)。在SSCU之后,11例(19.6%)患者的分期有所改善,11例(19.6%)患者恶化,干预后34例(60.7%)患者保持不变(AC评分)。在总共45例(80.4%)中观察到非恶化(p<0.001)。MHS评分(n=27,48.2%)提高,保持不变(n=12,21.4%),17例患者恶化(30.4%)。在所有病例中,有39例(69.6%)的术后膀胱功能结果未恶化(p<0.005)。无论膀胱功能是按AC还是MHS分类,当进行SSCU时,由于单独的运动功能恶化增加,术后结局显著恶化(p<0.05).在24例以NOD为适应症的病例中,22(91.7%)的情况没有变化(n=10;41.7%)或改善(n=12;50.0%),意味着积极的神经骨科结果,只有2(8.3%)恶化(p<0.001)。
    结论:我们的研究为脊柱裂患者的膀胱功能提供了可靠的评估系统。由于SSCU手术的适应症不同,了解手术后对膀胱功能的可能影响是很重要的。即使轻度膀胱功能受损也有在SSCU手术后恶化的风险。特别有趣的是,随着脑膜脊髓囊肿的产前闭合数量的增加,TCS的患病率可能会变得更加频繁。
    BACKGROUND: A TCS after primary closure of meningomyeloceles is a known complication of the spina bifida disease. Data on the outcome after SSCU surgery is heterogeneous and lacking standardization. Thus we aimed to find a reliable system for assessment of the bladder function before and after SSCU surgery and document postoperative outcome.
    METHODS: A retrospective study was performed on a cohort of patients with spina bifida diagnosis. In total, 130 patients underwent 182 SSCU surgeries, 56 of those met our inclusion criteria. A classification system, including two different methods, was used. The AC system used baseline pressure and detrusor over activity to define three levels of bladder dysfunction, the second method ranked the severity of bladder dysfunction by awarding points from 0 to 2 for bladder capacity, maximal detrusor pressure during autonomous contractions, leak point pressure and vesicoureteral reflux A high score is correlated with a severe bladder dysfunction.
    RESULTS: Gender distribution was equally (male: n = 29; 51.8%; female: n = 27; 48.2%). The median age at SSCU was 902 years (range 0.5-22.8 years). After SSCU, the stage improved in 11 patients (19.6%), worsened in 11 (19.6%) patients and remained the same in 34 patients (60.7%) after intervention (AC score). Non-worsening was observed in a total of 45 cases (80.4%) (p < 0.001). MHS score (n = 27, 48.2%) improved, remained unchanged (n = 12, 21.4%), 17 patients worsened (30.4%). Non-worsening in postoperative bladder functional outcome was demonstrated in 39 cases (69.6%) over all (p < 0.005). Regardless of whether bladder function is categorized by AC or MHS, postoperative outcome worsened significantly when SSCU was performed due to increasing deterioration in motor function alone (p < 0.05). Of the 24 cases with NOD as indication, 22 (91.7%) had an unchanged (n = 10; 41.7%) or improved (n = 12; 50.0%), meaning positive neuro-orthopedic outcome, only 2 (8.3%) deteriorated (p < 0.001).
    CONCLUSIONS: Our study presents reliable evaluation systems for bladder function in spina bifida patients. Since indications for SSCU surgery differ, it is important to know the possible effects on bladder function after this surgical procedure. Even a mild impairment of bladder function has a risk to deteriorate after SSCU surgery. Particularly interesting becomes this with regard to the fact that the prevalence of TCS might become more frequent with the rising numbers of prenatal closures of meningomyeloceles.
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  • 文章类型: Journal Article
    背景:Currarino综合征是一种罕见的疾病,通常表现为骶前肿块的三联征,肛门直肠畸形,和脊髓发育不良。骶前肿块通常是良性的,虽然恶性转化是可能的。对于诊断和症状缓解,需要对肿块进行手术治疗,并对相关的发育不良进行探索和修复。以前没有雄激素不敏感患者的Currarino综合征的报道。
    方法:一名17岁女性患者出现月经初潮缺乏。体格检查和实验室调查确定了完全的雄激素不敏感。影像学分析显示骶骨前肿块,患者被送往手术切除肿块和脊髓脱离。术中超声显示纤维茎连接鞘囊和骶前肿块,它是断开的,不需要鞘内探查。然后切除了骶前肿块,病理分析显示为成熟的囊性畸胎瘤。术后,患者康复,无神经或胃肠道后遗症。
    结论:不完全性Currarino综合征的诊断可能很困难,但可以通过检查其他疾病来确定,如雄激素不敏感。术中超声可用于手术决策,并且可以避免在Currarino综合征的情况下修复发育不良期间进行鞘内探查。
    BACKGROUND: Currarino syndrome is a rare disorder that classically presents with the triad of presacral mass, anorectal malformation, and spinal dysraphism. The presacral mass is typically benign, although malignant transformation is possible. Surgical treatment of the mass and exploration and repair of associated dysraphism are indicated for diagnosis and symptom relief. There are no previous reports of Currarino syndrome in an androgen-insensitive patient.
    METHODS: A 17-year-old female patient presented with lack of menarche. Physical examination and laboratory investigation identified complete androgen insensitivity. Imaging analysis revealed a presacral mass lesion, and the patient was taken to surgery for resection of the mass and spinal cord untethering. Intraoperative ultrasound revealed a fibrous stalk connecting the thecal sac to the presacral mass, which was disconnected without the need for intrathecal exploration. The presacral mass was then resected, and pathological analysis revealed a mature cystic teratoma. Postoperatively, the patient recovered without neurological or gastrointestinal sequelae.
    CONCLUSIONS: Diagnosis of incomplete Currarino syndrome may be difficult but can be identified via work-up of other disorders, such as androgen insensitivity. Intraoperative ultrasound is useful for surgical decision making and may obviate the need for intrathecal exploration during repair of dysraphism in the setting of Currarino syndrome.
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  • 文章类型: Journal Article
    脂肪纤维终末是脊柱发育不良的一种形式,三分之一的患者会出现感觉症状,电机,和泌尿障碍。6个月的早期手术具有骨密度仍然柔软的优点,病人还不能走动,促进更快的愈合。
    我们提出了我们的最小侵入性手术技术的FFT解链。
    由于低并发症发生率和手术潜在的高获益,建议预防性解开。
    Fatty filum terminale is a form of spinal dysraphism and a third of all patients develop symptoms such as sensory, motor, and urinary impairment. Early surgery at 6 months has the advantage that the bone density is still soft, and the patients are not ambulatory yet, promoting faster healing.
    We present our minimal invasive surgical technique for FFT untethering.
    Due to the low complication rate and the potentially high benefit of surgery, prophylactic untethering is recommended.
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  • 文章类型: Case Reports
    UNASSIGNED: Although the optimal timing of prophylactic untethering surgery for limited dorsal myeloschisis (LDM) with intact or subtle neurological findings diagnosed at birth remains undetermined, intentional delayed surgery is commonly used for flat and tail-like LDM. Conversely, for saccular LDM, early surgery is indicated during the postnatal period because it prevents rupture of the sac. We treated a saccular LDM patient, in whom intentional delayed surgery was selected because the sac was thickly covered with normal skin. We describe the clinical course of the case and discuss the optimal timing of the surgery.
    UNASSIGNED: The patient had a dorsal midline sac in the upper lumbar region. Initial magnetic resonance imaging (MRI) after birth revealed a tethering tract that began at the dome of the sac and joined the lumbar cord. Dorsal bending of the cord at the stalk-cord union and invagination of the cord into the sac were noted. At 2 months, he was neurologically normal; however, the second MRI examination revealed that the cord tethering was aggravated. The cord was markedly displaced dorsally and to the left, with deviation of the cord to the sac out of the spinal canal. Following untethering surgery, the spinal cord deformity markedly improved.
    UNASSIGNED: Early surgery may be recommended for saccular LDM when tethering is present, including dorsal bending of the cord at the stalk-cord union and invagination of the cord into the sac observed on detailed MRI examination, even if the sac has no risk of rupture.
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