Cauda equina ependymomas: surgical treatment and long-term outcomes in a series of 125 patients.

OBJECTIVE: Cauda equina ependymoma (CEE) is a rare tumor for which little information is available on the oncological and clinical outcomes of patients. In this study the authors aimed to address functional, oncological, and quality-of-life (QOL) outcomes in a large series of consecutive patients operated on at their institution during the past 20 years. METHODS: The records of 125 patients who underwent surgery between January 1998 and September 2018 were reviewed. Analyzed variables included demographic, clinical, radiological, surgical, and histopathological features. Neurological outcomes were graded according to the McCormick and Kesselring scales. The QOL at follow-up was evaluated by administering the EQ-5DL questionnaire. RESULTS: On admission, 84% of patients had a McCormick grade of I and 76.8% had a Kesselring score of 0. At follow-up (clinical 8.13 years; radiological 5.87 years) most scores were unchanged. Sacral level involvement (p = 0.029) and tumor size (p = 0.002) were predictors of poor functional outcome at discharge. Tumor size (p = 0.019) and repeated surgery (p < 0.001) were predictors of poor outcome. A preoperative McCormick grade ≥ III and Kesselring grade ≥ 2 were associated with worse outcomes (p = 0.035 and p = 0.002, respectively). Myxopapillary ependymoma (MPE) was more frequent than grade II ependymoma (EII). The overall rate of gross-total resection (GTR) was 91.2% and rates were significantly higher for patients with EII (98%) than for those with MPE (84%) (p = 0.0074). On multivariate analysis, the only factor associated with GTR was the presence of a capsule (p = 0.011). Seventeen patients (13.7%) had recurrences (13 MPE, 4 EII; 76.4% vs 23.6%; p = 0.032). The extent of resection was the only factor associated with recurrence (p = 0.0023) and number of surgeries (p = 0.006). Differences in progression-free survival (PFS) were seen depending on the extent of resection at first operation (p < 0.001), subarachnoid seeding (p = 0.041), piecemeal resection (p = 0.004), and number of spine levels involved (3 [p = 0.016], 4 [p = 0.011], or ≥ 5 [p = 0.013]). At follow-up a higher proportion of EII than MPE patients were disease free (94.7% vs 77.7%; p = 0.007). The QOL results were inferior in almost all areas compared to a control group of subjects from the Italian general population. A McCormick grade ≥ 3 and repeated surgeries were associated with a worse QOL (p = 0.006 and p = 0.017). CONCLUSIONS: An early diagnosis of CEE is important because larger tumors are associated with recurrences and worse functional neurological outcomes. Surgery should be performed with the aim of achieving an en bloc GTR. The histological subtype was not directly associated with recurrences, but some of the features more commonly encountered in MPEs were. The outcomes are in most cases favorable, but the mean QOL perception is inferior to that of the general population.

Surgery for intramedullary spinal cord ependymomas in the neuromonitoring era: results from a consecutive series of 100 patients.

OBJECTIVE: The established treatment of intramedullary spinal cord ependymomas (ISCEs) is resection. Surgical series reporting treatment results often lack homogeneity, as these are collected over long time spans and their analysis is plagued by surgical learning curves and inconsistent use of intraoperative neurophysiological monitoring (IONM). The authors report the oncological and functional long-term outcomes in a modern series of 100 consecutive ISCEs that were resected between 2000 and 2015 by a surgically experienced team that consistently utilized IONM. METHODS: In this retrospective study, the authors tailored surgical strategy and multimodal IONM, including somatosensory evoked potentials, muscle motor evoked potentials (mMEPs), and D-waves, with the aim of gross-total resection (GTR). Preservation of the D-wave was the primary objective, and preservation of mMEPs was the second functional objective. Functional status was evaluated using the modified McCormick Scale (MMS) preoperatively, postoperatively, and at follow-up. RESULTS: Preoperatively, 89 patients were functionally independent (MMS grade I or II). A GTR was achieved in 89 patients, 10 patients had a stable residual, and 1 patient underwent reoperation for tumor progression. At a mean follow-up of 65.4 months, 82 patients were functionally independent, and 11 lost their functional independence after surgery (MMS grades III-V). Muscle MEP loss predicted short-term postoperative worsening (p < 0.0001) only, while the strongest predictors of a good functional long-term outcome were lower preoperative MMS grades (p < 0.0001) and D-wave preservation. D-wave monitorability was 67%; it was higher with lower preoperative MMS grades and predicted a better recovery (p = 0.01). CONCLUSIONS: In this large series of ISCEs, a high rate of GTR and long-term favorable functional outcome were achieved. Short- and long-term functional outcomes were best reflected by mMEPs and D-wave monitoring, respectively.

Trans-thoracic versus retropleural approach for symptomatic thoracic disc herniations: comparative analysis of 94 consecutive cases.

PURPOSE: The authors illustrate their results in the surgical treatment of symptomatic thoracic disc herniations (TDHs) by comparing the traditional open to the less invasive retropleural lateral approaches. METHODS: Retrospective review of 94 consecutive cases treated at a single Institution between 1988 and 2014. Fifty-two patients were males, 42 females, mean age was 53.9 years. Mean follow-up was 46.9 months (12-79 months). 33 patients were diagnosed with a giant thoracic disc herniation (GTDH). Upon admission, the most common symptoms were: motor impairment (91.4%, n = 86), neuropathic radicular pain with VAS > 4 (50%), bladder and bowel dysfunction (57.4% and 41.4% respectively) and sensory disturbances (29.7%). The surgical approach was based upon level, laterality and presence or absence of calcified lesions. RESULTS: Decompression was performed in 7 cases via a thoraco-laparo-phrenotomy and in 87 cases via an antero-lateral thoracotomy. Out of the latter cases, 49 (56%) were trans-thoracic trans-pleural approaches (TTA) and 38 (44%) were less invasive retropleural approaches (MIRA). At follow-up, there were 59.5% neurologically intact patients according to the McCormick Scale, while 64.8% and 67% had no bladder or bowel dysfunction respectively. Complications occurred in 24 patients (25.5%). Pulmonary complications were the commonest (12.7%) with pleural effusion being significantly more common in patients treated with TTA compared to MIRA (20% vs 5.2%: X2 4.13 P:0.042). Severe post-operative neuralgia (VAS 7-10) was also significantly more frequent in the TTA group (22.4% vs 2.6% X2 7.07 p 0.0078). CONCLUSIONS: MIRA is a safe and effective technique to obtain adequate TDH decompression and is associated with lower morbidity compared to TTA.

Tolerability and efficacy of erythropoietin (EPO) treatment in traumatic spinal cord injury: a preliminary randomized comparative trial vs. methylprednisolone (MP).

The only available treatment of traumatic spinal cord injury (TSCI) is high-dose methylprednisolone (MP) administered acutely after injury. However, as the efficacy of MP is controversial, we assessed the superiority of erythropoietin (EPO) versus MP in improving clinical outcome of acute TSCI. Patients aged 18 to 65 years after C5-T12 injury, and grade A or B of the ASIA Impairment Scale (AIS), admitted within 8 h, hemodynamically stable, were randomized to MP according to the NASCIS III protocol or EPO iv (500 UI/kg, repeated at 24 and 48 h). Patients were assessed by an investigator blind to treatment assignment at baseline and at day 3, 7, 14, 30, 60 and 90. Primary end point: number of responders (reduction of at least one AIS grade). Secondary end points: treatment safety and the effects of drugs on a number of disability measures. Frequentistic and post hoc Bayesian analyses were performed. Eight patients were randomized to MP and 11 to EPO. Three patients (27.3 %) on EPO and no patients on MP reached the primary end point (p = 0.17). No significant differences were found for the other disability measures. No adverse events or serious adverse events were reported in both groups. The Bayesian analysis detected a 91.8 % chance of achieving higher success rates on the primary end point with EPO in the intention-to-treat population with a 95 % chance the difference between EPO and MP falling in the range (-0.10, 0.51) and a median value of 0.2. The results of Bayesian analysis favored the experimental treatment.

Intraoperative neurophysiology in tethered cord surgery: techniques and results.

INTRODUCTION: Intraoperative neurophysiologic monitoring (IOM) is nowadays extensively used to minimize neurological morbidity in tethered cord surgery. Our goal is to describe and discuss the standard IOM techniques used during these surgical procedures and to summarize our clinical experience using a multimodal IOM approach. MATERIAL AND METHODS: Neurophysiological mapping of the conus-cauda is performed through direct stimulation of these structures and bilateral recording from segmental target muscles. While mapping identifies ambiguous neural structures, their functional integrity during surgery can be assessed by monitoring techniques only, such as somatosensory evoked potentials (SEPs), transcranial motor-evoked potentials (MEPs) from the limb muscles and anal sphincters, and the bulbocavernosus reflex (BCR). RESULTS: Between 2002 and 2012, we performed 48 surgical procedures in 47 patients with a tethered cord secondary to a variety of spinal dysraphisms. The monitorability rate was 84 % for SEPs, 97 % for limb muscle MEPs, 74 % for the anal sphincter MEPs, and 59 % for the BCR. In all patients but one, SEP, MEP, and BCR remained stable during surgery. Postoperatively, two out of 47 patients presented a significant-though transient-neurological worsening. In six patients, an unexpected muscle response was evoked by stimulating tissue macroscopically considered as not functional. CONCLUSIONS: Mapping techniques allow identifying and sparing functional neural tissue and vice versa to cut nonfunctional structures that may contribute to cord tethering. Monitoring techniques, MEP and BCR in particular, improve the reliability of intraoperative neurophysiology, though these may require a higher degree of neuromonitoring expertise. IOM minimizes neurological morbidity in tethered cord surgery.

Cystic dilation of the ventriculus terminalis.

OBJECT: The ventriculus terminalis, an embryological remnant consisting of the ependymal-lined space of the conus medullaris, can occasionally become symptomatic after cystic dilation. In the existing literature, consisting of 32 cases, the preferred type of management (conservative vs surgical) is still debated. The object of this study was to report the surgical results in a consecutive series of 10 adult patients with cystic dilation of the ventriculus terminalis (CDVT), to match them with data retrieved from the relevant literature, and specifically to validate a new recent clinical classification. METHODS: The authors reported 13 new cases of CDVT treated in the Department of Neurosurgery at University Hospital in Verona, Italy. Treatment modalities and clinical and radiological outcomes, both early and at follow-up, were analyzed and compared with a preoperative classification of clinical presentation, as established by de Moura Batista and colleagues (2008). RESULTS: Surgical treatment seemed to guarantee the resolution of CDVT. Dorsolumbar laminotomy, myelotomy, and cystic drainage were performed in 10 patients. Patients with Type I symptoms (nonspecific complaints) often presented with comorbidities (herniated disc or facet hypertrophy) confusing their clinical status. The surgical treatment of patients with Type I symptoms promoted good results only if the diagnosis of CDVT was definitive and symptoms had rapidly evolved. In patients with Type II (focal neurological deficits) and III (sphincter disturbances) symptoms, surgical treatment sustained improvement even at the late follow-up. CONCLUSIONS: While confirming the usefulness of de Moura Batista and colleagues' classification in its impact on prognosis, the authors propose a revision of the classification with subgroups Type Ia (nonspecific symptoms without clear relation to CDVT), which is best treated conservatively, and Type Ib (rapid onset and invalidating unspecific complaints without comorbidities), which may benefit from surgical evacuation.

Late deterioration of neurologic function in patients surgically treated for spinal dural arteriovenous fistulas.

BACKGROUND: A limited number of reports on the long-term neurologic outcome of patients with SDAVFs treated by surgery and/or embolization are available in the literature. The aim of our study is to neurologically evaluate these patients at 2 different follow-up stages, after surgery, to demonstrate a possible late neurologic deterioration after an initial improvement. METHODS: Between January 1987 and May 2002, 29 patients with SDAVFs were operated on at the Verona Department of Neurosurgery. In this group we retrospectively identified 16 patients who had 2 different clinical follow-ups, at a mean of 4.5 and 9.2 years, respectively. We compared their neurologic status using the ALS. All these data were obtained from clinical charts and phone interviews. RESULTS: The epidemiologic, clinical, and radiologic features of our group of patients are very similar to those previously described in the literature. Comparing the global clinical status between the 2 different follow-up stages, we observed a late deterioration in 8 cases (50%). A worsening of the mean G and M values of the ALS was also noted. Spinal angiography and contrast-enhanced MRI did not show any signs of recurrence of the fistula. CONCLUSIONS: Our study confirms the possible occurrence of a late clinical deterioration in as many as 50% of patients surgically treated for a SDAVF. We deem that the main pathophysiologic mechanism underlining this phenomenon is a gradual and irreversible decline in spinal function related to those hemodynamic modifications induced by the fistula and to the persistence of a state of anatomofunctional deficiency of the spinal venous drainage.

Intraneural cavernous malformation of the cauda equina.

Spinal cavernous angiomas are rare vascular malformations occurring mainly in the vertebral body with or without an extradural extension. Only 3-5% of these lesions are entirely located in the spinal canal where they can occupy an extradural, intradural-extramedullary or intramedullary position. We present a 75-year-old woman with signs and symptoms of multiple lumbar radiculopathy. The lumbosacral MRI showed an intradural cauda equina lesion with heterogeneous contrast enhancement that was subsequently radically removed through an L3-L4 laminectomy. The microscopic appearance was suggestive of cavernous angioma with intraneural growth. Clinical, radiological, and surgical features of this unusual lesion (to date, only 12 cases are reported) are discussed.

Surgery for intramedullary spinal cord tumors: the role of intraoperative (neurophysiological) monitoring.

In spite of advancements in neuro-imaging and microsurgical techniques, surgery for intramedullary spinal cord tumors (ISCT) remains a challenging task. The rationale for using intraoperative neurophysiological monitoring (IOM) is in keeping with the goal of maximizing tumor resection and minimizing neurological morbidity. For many years, before the advent of motor evoked potentials (MEPs), only somatosensory evoked potentials (SEPs) were monitored. However, SEPs are not aimed to reflect the functional integrity of motor pathways and, nowadays, the combined used of SEPs and MEPs in ISCT surgery is almost mandatory because of the possibility to selectively injury either the somatosensory or the motor pathways. This paper is aimed to review our perspective in the field of IOM during ISCT surgery and to discuss it in the light of other intraoperative neurophysiologic strategies that have recently appeared in the literature with regards to ISCT surgery. Besides standard cortical SEP monitoring after peripheral stimulation, both muscle (mMEPs) and epidural MEPs (D-wave) are monitored after transcranial electrical stimulation (TES). Given the dorsal approach to the spinal cord, SEPs must be monitored continuously during the incision of the dorsal midline. When the surgeon starts to work on the cleavage plane between tumor and spinal cord, attention must be paid to MEPs. During tumor removal, we alternatively monitor D-wave and mMEPs, sustaining the stimulation during the most critical steps of the procedure. D-waves, obtained through a single pulse TES technique, allow a semi-quantitative assessment of the functional integrity of the cortico-spinal tracts and represent the strongest predictor of motor outcome. Whenever evoked potentials deteriorate, temporarily stop surgery, warm saline irrigation and improved blood perfusion have proved useful for promoting recovery, Most of intraoperative neurophysiological derangements are reversible and therefore IOM is able to prevent more than merely predict neurological injury. In our opinion combining mMEPs and D-wave monitoring, when available, is the gold standard for ISCT surgery because it supports a more aggressive surgery in the attempt to achieve a complete tumor removal. If quantitative (threshold or waveform dependent) mMEPs criteria only are used to stop surgery, this likely impacts unfavorably on the rate of tumor removal.

Motor evoked potential monitoring improves outcome after surgery for intramedullary spinal cord tumors: a historical control study.

OBJECTIVE: The value of intraoperative neurophysiological monitoring (INM) during intramedullary spinal cord tumor surgery remains debated. This historical control study tests the hypothesis that INM monitoring improves neurological outcome. METHODS: In 50 patients operated on after September 2000, we monitored somatosensory evoked potentials and transcranially elicited epidural (D-wave) and muscle motor evoked potentials (INM group). The historical control group consisted of 50 patients selected from among 301 patients who underwent intramedullary spinal cord tumor surgery, previously operated on by the same team without INM. Matching by preoperative neurological status (McCormick scale), histological findings, tumor location, and extent of removal were blind to outcome. A more than 50% somatosensory evoked potential amplitude decrement influenced only myelotomy. Muscle motor evoked potential disappearance modified surgery, but more than 50% D-wave amplitude decrement was the major indication to stop surgery. The postoperative to preoperative McCormick grade variation at discharge and at a follow-up of at least 3 months was compared between the two groups (Student's t tests). RESULTS: Follow-up McCormick grade variation in the INM group (mean, +0.28) was significantly better (P = 0.0016) than that of the historical control group (mean, -0.16). At discharge, there was a trend (P = 0.1224) toward better McCormick grade variation in the INM group (mean, -0.26) than in the historical control group (mean, -0.5). CONCLUSION: The applied motor evoked potential methods seem to improve long-term motor outcome significantly. Early motor outcome is similar because of transient motor deficits in the INM group, which can be predicted at the end of surgery by the neurophysiological profile of patients.