Surgery For Intramedullary Spinal Cord Tumors Research Articles

The application value and improved warning criterion of D-wave monitoring in intramedullary spinal cord tumor surgery

BACKGROUND CONTEXTThe primary treatment method for intramedullary spinal cord tumor (IMSCT) is surgical resection, but this procedure carries a significant risk of neurological damage. Intraoperative neurophysiological monitoring (IONM) has become a necessary adjunctive tool for IMSCT resection. PURPOSEThe current study aimed to explore the application value of D-wave monitoring in IMSCT surgery, and tried to investigate a tailored criterion for its early warning. STUDY DESIGNA retrospective clinical study. PATIENT SAMPLEA retrospective analysis was conducted based on the data of patients who underwent IMSCT surgeries performed by the same neurosurgical team at our hospital. IONM was applied in all surgeries. According to inclusion and exclusion criteria, ultimately 90 patients were enrolled in the study. OUTCOME MEASURESThe McCormick Scale (MMS) was applied to assess the functional outcome through outpatient visits or telephone follow-up at one month and six months postoperatively. Patients with an MMS grade over II one month after surgery were considered to have newly developed postoperative motor dysfunction (PMD). If the MMS grade could be restored to I or II six months after surgery, it was defined as a short-term PMD. Otherwise, it was defined as a long-term PMD. METHODSThe predictive value of different IONM modalities, including somatosensory evoked potential (SEP), muscle motor evoked potential (MEP), and D-wave for PMD, was assessed with sensitivity, specificity, positive predictive value, negative predictive value, and subsequent logistic regression analysis. At last, the cut-off value of the D-wave amplitude reduction ratio for predicting PMD was obtained through the receiver operating characteristic (ROC) curve analysis. RESULTSSEP showed the worst performance in predicting short-term and long-term PMD. Significant MEP changes were indicated as an independent predictive factor for short-term PMD (OR 5.062, 95% CI 1.947–13.166, p=.001), while D-wave changes were demonstrated as an independent predictor for long-term PMD (OR 339.433, 95% CI 11.337–10770.311, p=.001). The optimum cut-off value of the D-wave amplitude reduction ratio for predicting long-term PMD was 42.18%, with a sensitivity of 100% and a specificity of 93.8% (AUC=0.981, p<.001). CONCLUSIONSD-wave monitoring showed extremely high specificity in predicting PMD compared to SEP and MEP monitoring. Moreover, the authors suggested that a D-wave amplitude reduction of over 40% during IMSCT surgery generally indicates long-term PMD for patients.

Multimodal Intraoperative Neurophysiological Monitoring in Intramedullary Spinal Cord Tumors: A 10-Year Single Center Experience.

The study aimed at evaluating the efficacy and the ability of D-wave monitoring combined with somatosensory evoked potentials (SSEPs) and motor evoked potentials (MEPs) to predict functional outcomes in intramedullary spinal cord tumor (IMSCT) surgery. Between December 2011 and December 2020, all patients harboring IMSCT who underwent surgery at our institution were prospectively collected in a surgical spinal registry and retrospectively analyzed. Patient charts and surgical and histological reports were analyzed. The multimodal IONM included SSEPs, MEPs, and-whenever possible-D-waves. All patients were evaluated using the modified McCormick and Frankel grade at admission and 3, 6, and 12 months of follow-up. Sixty-four patients were enrolled in the study. SSEP and MEP monitoring was performed in all patients. The D-wave was not recordable in seven patients (11%). Significant IONM changes (at least one evoked potential modality) were registered in 26 (41%) of the 64 patients. In five cases (8%) where the SSEPs and MEPs lost and the D-wave permanently dropped by about 50%, patients experienced a permanent deterioration of their neurological status. Multimodal IONM (SSEP, MEP, and D-wave neuromonitoring) significantly predicted postoperative deficits (p = 0.0001), with a sensitivity of 100.00% and a specificity of 95.65%. However, D-waves demonstrated significantly higher sensitivity (100%) than MEPs (62.5%) and SSEPs (71.42%) alone. These tests' specificities were 85.10%, 13.89%, and 17.39%, respectively. Comparing the area under ROC curves (AUCs) of these evoked potentials in 53 patients (where all three modalities of IONM were registered) using the pairwise t-test, D-wave monitoring appeared to have higher accuracy and ability to predict postoperative deficits with strong statistical significance compared with MEP and SSEP alone (0.992 vs. 0.798 vs. 0.542; p = 0.018 and p < 0.001). The use of multimodal IONM showed a statistically significant greater ability to predict postoperative deficits compared with SSEP, MEP, and D-wave monitoring alone. D-wave recording significantly increased the accuracy and clinical value of neurophysiological monitoring in IMSCT tumor resection.

Is There a Role for Intraoperative Neuromonitoring in Intradural Extramedullary Spine Tumors? Results and Indications from an Institutional Series.

While intraoperative neurophysiological monitoring (IONM) is considered a standard for intramedullary spinal cord tumor surgery, the effective role of IONM in intradural extramedullary (IDEM) tumors is still debated. We present the results of 60 patients affected by IDEM tumors undergoing surgery with the aid of IONM. Each patient was evaluated according to the modified McCormick scale (MMS) at admission, discharge and at follow-up. During surgery, motor evoked potentials (MEPs) and somatosensory evoked potentials (SEPs) were studied using the Medtronic NIM-eclipse® 32-channel system (Medtronic Xomed, Inc. 6743 Southpoint Drive North Jacksonville FL USA). Patients' age, gender and tumor location did not affect MMS modifications. Tumors involving more than three levels had an increased likelihood of MMS worsening, while meningioma pathology was associated with worse preoperative and 1-year follow-up MMS. No MEP amplitude ratio was able to predict clinical variations, while intraoperative SEP worsening was associated with 100% risk of poor MMS at discharge and with 50% risk of poor MMS at long-term follow-up. In our opinion, SEP monitoring is a valid tool that may contribute to the preservation of the patient's neurological status. MEP monitoring is not mandatory in IDEM surgery while more studies are required to explore the feasibility and the role of D-wave in this kind of surgery.

Outcomes of Intramedullary Spinal Cord Tumor Surgery in Older Versus Younger Adults: A Multicenter Subanalysis Study by the Neurospinal Society of Japan.

Intramedullary spinal cord tumors (IMSCTs) are uncommon and difficult to treat. Studies examining the efficacy of rare IMSCT surgery in the elderly are limited. We conducted a subanalysis using multicenter retrospective-historical data provided by the Japan Neurospinal Society to compare surgical outcomes between older and younger adults with IMSCTs. We classified patients with IMSCTs into younger (aged 18-64 years) or older ( ≥ 65 years) groups. The primary outcomes of "improved" or "worsened" from the preoperative period to 6 months after surgery were evaluated using the modified McCormick scale (mMCs). A favorable outcome was defined as an mMCs grade of I/II at 6 months. Among 841 patients registered, there were 658 younger (78.2%) and 183 older patients (21.8%) evaluated using mMCs at 6 months. Median preoperative mMCs grades were significantly worse in older patients than in younger patients. Neither the "improved" nor "worsened" rate differed significantly between the groups (28.1% vs. 25.1%; crude odds ratio [cOR], 0.86; 95% confidence interval [CI], 0.59-1.25; adjusted OR [aOR], 0.84; 95% CI, 0.55-1.28; 16.9% vs. 23.0%; cOR, 1.47; 95% CI, 0.98-2.20; aOR, 1.28; 95% CI, 0.83-1.97). Favorable outcomes were significantly less common among older adults in the univariate analysis but were not significant in the multivariate analysis (66.4% vs. 53.0%; cOR, 0.57; 95% CI, 0.41-0.80; aOR, 0.77; 95% CI, 0.50-1.19). In both younger and older patients, preoperative mMCs accurately predicted favorable outcomes. Age alone is not a sufficient reason to prohibit surgery for IMSCTs.

Chapter 13 - Intraoperative neurophysiology in intramedullary spinal cord tumor surgery

Intramedullary spinal cord tumor (ISCT) surgery is challenged by a significant risk of neurological injury. Indeed, while most ISCT patients arrive to surgery in good neurological condition due to early diagnosis, many experience some degree of postoperative sensorimotor deficit. Thus, intraoperative neuromonitoring (IONM) is invaluable for providing functional information that helps neurosurgeons tailor the surgical strategy to maximize resection while minimizing morbidity. Somatosensory evoked potential (SEP), muscle motor evoked potential (mMEP), and D-wave monitoring are routinely used to continuously assess the functional integrity of the long pathways within the spinal cord. More recently, mapping techniques have been introduced to identify the dorsal columns and the corticospinal tracts. Intraoperative SEP decline is not a sufficient reason to abandon surgery, since SEPs are very sensitive to anesthesia and surgical maneuvers. Yet, a severe proprioceptive deficit may adversely impact daily life, and the value of SEPs should be reconsidered. While mMEPs are good predictors of short-term motor outcome, the D-wave is the strongest predictor of long-term motor outcome, and its preservation during surgery is essential. Mapping techniques are promising but still need validation in large cohorts of patients to determine their impact on clinical outcome. The therapeutic rather than merely diagnostic value of IONM in spine surgery is still debated, but there is emerging evidence that IONM provides an essential adjunct in ISCT surgery.

척수 종양 수술 중 척수 지도화

The main treatment for most patients with intramedullary spinal cord tumors is surgical resection. Intraoperative neurophysiological monitoring can be applied in this case to reduce the risk for the occurrence of postoperative neurological complications. In addition to motor-evoked, somatosensory-evoked potentials and the bulbocavernosus reflex, which are applied in various spinal surgery types, ‘spinal cord mapping (SCM)’ can be used for intramedullary spinal cord tumor surgery. Two examples are the dorsal column mapping before myelotomy to determine the electrophysiological midline, to attempt minimizing the postoperative dorsal column damage, and motor tract mapping to reduce the risk of motor-pathway damage during tumor removal. Our review explores the research published overseas regarding these two mapping methods, including technical aspects and key points in the mapping application. Although SCM has not yet been established in Korea, its application is expected to increase soon.

P2-10-03. Motor evoked potential monitoring in intramedullary spinal cord tumor surgery with inhalation or intravenous anesthesia

P2-10-03. Motor evoked potential monitoring in intramedullary spinal cord tumor surgery with inhalation or intravenous anesthesia

Preoperative steroids do not improve outcomes for intramedullary spinal tumors: a NSQIP analysis of 30-day reoperation and readmission rates.

Intramedullary spinal cord tumors (IMSCTs) account for 8-10% of all spinal cord tumors and affect patients of all ages. Although uncommon, IMSCTs carry risk of neurological morbidity and mortality, with 5-year survival rates ranging from 50% to 80%. In this study, we utilize the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) database to determine the effect of steroid administration on 30-day outcomes following surgery for IMSCTs. ACS-NSQIP data for patients undergoing surgery for intramedullary tumors from 2005 to 2015 was reviewed. Patients were selected based on current procedural terminology (CPT) codes 63285 (Laminectomy, intradural, intramedullary, cervical), 63286 (Laminectomy, intradural, intramedullary, thoracic), and 63287 (Laminectomy, intradural, intramedullary, thoracolumbar). ICD-9 and ICD-10 codes were chosen based on the diagnosis of a tumor. The 30-day clinical outcome data, including reoperations and readmission rates, were collected and compared. A total of 259 patients were reviewed. One hundred eighty-one patients had benign intramedullary tumors and 78 had malignant intramedullary tumors. The majority of IMSCTs were at the thoracic level (n=100), followed by the cervical (n=99), and thoracolumbar (n=39) levels. Thirty-one patients were on corticosteroid therapy prior to surgery. Patients with preoperative steroid administration had no significant difference in reoperation and readmission rates. No significant differences were noted between steroid vs. non-steroid therapy for discharge destination, length of hospital stay, or other postoperative complications. Contrary to previous reports, corticosteroid use prior to surgery for IMSCTs does not have a significant impact on 30-day risk of readmission, reoperation, and risk of postoperative complications.

Overview on Criteria for MEP Monitoring

Intraoperative motor evoked potentials include the D-wave as a surrogate for long-term motor outcome and muscle motor evoked potentials as a surrogate for early outcome. Their efficacy depends on excluding confounding factors and on warning criteria; insufficiently sensitive criteria could result in unpredicted deficits, whereas excessively sensitive ones could cause false alarms deterring surgical treatment and jading surgeons to alerts, eventually leading to deficits through failure to intervene. Although D-waves have few indications, they are nonsynaptic, linear, and stable-properties that support amplitude reduction criteria: >50% for intramedullary spinal cord tumor surgery and >30% to 40% for peri-Rolandic brain surgery. Muscle motor evoked potentials have many indications but are polysynaptic, nonlinear, and unstable-properties that challenge warning criteria and make them unusually capricious and sensitive. Disappearance is a remarkably frequent pathologic sign compared with other evoked potentials and is always a major criterion. Marked (>80%) amplitude reduction may be a minor or moderate spinal cord criterion, depending on the surgical circumstance. Modest (>50%) reduction may be a major criterion for brain, brainstem, and facial nerve monitoring, if justified by sufficient preceding stability. Acute ≥100-V threshold elevation may be a minor or moderate spinal cord criterion, depending on the surgical circumstance and on adherence to reported methodology. Morphology criteria lack support. Tailoring warning criteria to different monitoring situations based on anatomy, surgical goals, and published evidence seems advisable.

Differences in Multimodality Intraoperative Neurophysiological Monitoring Changes Between Spinal Intramedullary Ependymoma and Hemangioblastoma

Intraoperative neurophysiological monitoring using transcranial muscle motor evoked potentials (MEPs) and somatosensory evoked potentials is an established method for intramedullary spinal cord tumor surgery. Ependymomas and hemangioblastomas arise in different anatomic locations and require different surgical techniques. The aim of our study was to assess differences in intraoperative neurophysiological monitoring findings between ependymoma and hemangioblastoma. Fifty-six limbs from 16 patients diagnosed with ependymoma and 18 limbs from six patients with hemangioblastoma were included. The alarm criterion for MEPs was a 50% decrease in amplitude, whereas for somatosensory evoked potentials, it was a 50% decrease in amplitude and/or a 10% delay in latency. We found that 14 of the 56 ependymoma limbs (25.9%) and 8 of the 18 hemangioblastoma limbs (44.4%) showed MEP decrement during surgery. Eight limbs of patients with ependymoma (57.1%) and one limb of a patient with hemangioblastoma (12.5%) did not show recovery of MEPs at the end of surgery. Among those who showed recovery of MEPs, six ependymoma (10.7%) and six hemangioblastoma (33.3%) limbs did not show postoperative motor deficits (P = 0.04). Finally, 11 limbs of patients with ependymoma and one limb of a patient with hemangioblastoma showed postoperative weakness. In our study, the incidence of transient changes in MEPs was higher in hemangioblastoma than in ependymoma. Our data suggest that it may be necessary to consider tumor features and the type of surgical technique used, particularly when interpreting intraoperative neurophysiologic monitoring profiles of intramedullary spinal cord tumors such as ependymomas and hemangioblastomas.

Management and outcomes of intramedullary spinal cord tumors: A single center experience from a developing country.

Background:Intraoperative neurophysiology, high magnification microscopes, and ultrasonic aspirators are considered essential aid for the safe resection of intramedullary spinal cord tumors (IMSCTs). Most centers in developing countries such as Pakistan still lack these facilities. The purpose of this study was to review the management of IMSCTs at our hospital and to determine factors associated with the outcomes of surgery.Methods:This was a retrospective review of medical records of adult patients undergoing surgery for IMSCT over 12 years. The institutional ethical review committee approved this study. Data were collected regarding demographics, clinical and radiological features, and surgical details. Modified McCormick Scale was used to grade patients’ neurological status at admission, discharge, and follow-up. Statistical analysis was performed using the Statistical Package for Social Sciences version 22.Results:Forty three cases were reviewed. Mean age was 33.8 ± 15.1 years whereas median follow-up was 5 months (range: 0.25–96 months). Most patients had ependymoma (n = 16; 73%). Cervical region was the most commonly involved (n = 15; 34.9%). Gross total resection (GTR) was achieved in 30 cases (69.8%). The preoperative McCormick grade was significantly associated with follow-up McCormick grade (P value = 0.002). Eight patients (18.6%) underwent intraoperative electrophysiological monitoring, out of which GTR was achieved in all cases, and none had disease progression or recurrence. Ten patients received postoperative radiotherapy. Thirty five patients (81.4%) had progression free survival at last follow-up.Conclusions:We achieved a GTR rate of 68.9% for IMSCTs with limited resources. In few cases, where intraoperative electrophysiology was used, the rate of GTR was 100%. Preoperative neurological status was associated with better postoperative McCormick score.

Multimodal intraoperative monitoring during intramedullary spinal cord tumor surgery.

The aim of this work is to evaluate the utility of multimodal intraoperative monitoring (IOM) during intramedullary spinal cord tumor (IMSCT) surgery in our institution, and to investigate which IOM events are likely to be encountered during critical surgical phases. Twenty-five patients who underwent IMSCT surgery with IOM were included in this study. Our multimodal IOM assessment included SSEP, mMEP, and fEMG monitoring. Positive predictive value (PPV), negative predictive value (NPV), sensitivity, and specificity were assessed 24 h and 1 month after surgery. The IOM events during three main surgical phases were also investigated. For mMEP assessment, two warning criteria (>50 % decrease in mMEP amplitude and all-or-none mMEP amplitude presence) were employed. Long-term outcome prediction was better when the all-or-none criterion was applied than when the >50 % amplitude decrease criterion was applied. Based on the all-or-none criterion, the PPV, NPV, sensitivity, and specificity were 60, 100, 100, and 91 %. Frequent IOM events were observed during the three major main surgical phases. Seven (29 %) patients showed SSEP events during opening of the spinal cord. During tumor removal, 21 of 25 patients (84 %) had IOM events, and 13 of 18 (72 %) of the fEMG events occurred prior to the mMEP events. Based on the association of fEMG events with upcoming mMEP events during tumor removal, we recommend inclusion of fEMG monitoring in IOM. Multimodal IOM provides useful electrophysiological information during IMSCT surgery, especially during the main surgical phases.

Intraoperative spinal cord monitoring using combined motor and sensory evoked potentials recorded from the spinal cord during surgery for intramedullary spinal cord tumor

The risk of postoperative neurological impairment mandates the use of intraoperative spinal cord monitoring (IOM) during intramedullary spinal cord tumor (IMSCT) surgery. We have used spinal cord evoked potential after electrical stimulation of the cord(Sp-SCEP) to monitor the sensory tract, and SCEP after electrical stimulation to the brain (Br-SCEP) to monitor the motor tract. Both Sp-SCEP and Br-SCEP are stable under general anesthesia. We assessed the clinical utility of these multimodal spinal cord monitoring methods in a retrospective study of a cohort of patients undergoing IMSCT surgery. Thirteen patients with IMSCTs underwent tumor resection using Sp-SCEP and/or Br-SCEP. Four patients underwent surgery using only Sp-SCEP monitoring, resulting in two false negatives. Nine patients underwent surgery monitored by Br-SCEP and Sp-SCEP. In three of the nine cases, Br-SCEP amplitude fell by 50% from control levels, despite there being no change in Sp-SCEP. In one of the nine cases, Sp-SCEP amplitude fell to 50% of control levels, but Br-SCEP amplitude was stable. During IMSCT surgery, localized regions of the cord may be damaged. Multimodal monitoring should be used to monitor.Combining Sp-SCEP and Br-SCEP is a useful means of monitoring the sensory and motor tracts.

Intraoperative neurophysiological monitoring during resection of intradural extramedullary spinal cord tumors: experience with 100 cases.

Intradural-extramedullary spinal cord tumor surgery is common. Unlike intramedullary spinal cord tumor surgery, where intraoperative neurophysiological monitoring (IONM) has been described extensively, the application of IONM has not been described in this context, and its relevance has not been investigated. From 2001 to 2012, 100 patients underwent intradural-extramedullary spinal cord tumor resection with IONM. Preoperative and postoperative clinical evaluations were completed retrospectively, using a modified McCormick grading scale and correlated with IONM monitorability and dynamics. IONM consisted of transcranial motor evoked potentials (tcMEP), spinal (D wave) and muscle generators, somatosensory evoked potentials (SSEP), and electromyography (EMG). Both short-term and long-term clinical evaluations were performed. Patient demographics, tumor type, span, location, and morphologic complexity were analyzed. Surgeries were performed for resection of schwannomas (33 %), meningiomas (22 %), ependymomas (12 %), and other pathologies (20 %); pathology was unknown in 13 % of patients. Tumor locations were cervical in 21 %, thoracic in 46 %, thoracolumbar in 7 %, lumbar 20 %, and not specified in 6 %. Tumors spanned an average of 2.2 spinal levels. Monitorability was 97 and 67 % with tcMEP and SSEP modalities respectively. D waves were monitorable in 73 % of attempts. Intraoperative tcMEP changes were reported in 29 cases with 14 resolved intraoperatively, There were one false-negative outcome and five true-positive outcomes. For SSEP, 13 changes were noted and three resolved; there were three false-negative results and one true-positive result. For D wave monitoring there were two intraoperative changes with none resolved leading to one false negative and one true positive result. With a multimodality approach incorporating any change in evoked potential, IONM demonstrated sensitivity of 0.82, specificity of 0.95, positive predictive value of 0.82, and a negative predictive value of 0.95. IONM is feasible and useful in the context of intradural-extramedullary spinal cord surgery for identifying iatrogenic injury to the spinal cord.

Intraoperative Neurological Deterioration Secondary to Spinal Kyphotic Change After Cervicothoracic Junction Laminectomy

Case report. We report a case in which intraoperative spinal kyphosis after laminectomy led to neurological deterioration during intramedullary spinal cord tumor surgery at the cervicothoracic junction (CTJ). CTJ is a transitional zone between the cervical and thoracic spine; this region can be easily affected by mechanical stress. Although postoperative spinal instability or kyphosis after laminectomy at CTJ has been reported, no reports of intraoperative neurological deterioration after laminectomy exist. The patient was a 40-year-old female with an intramedullary spinal cord tumor at T2-T4, which was suspected to be an ependymoma. She had no neurological deficit before the surgery. Although tumor removal surgery using a posterior approach was planned, the waves observed on intraoperative neurophysiological monitoring in the bilateral lower extremity disappeared soon after laminectomy. The waveform reappeared when we manually corrected the kyphosis at CTJ by pushing the spine. However, when we stopped the manual correction, the waveform disappeared again. The cause of neurological deterioration was strongly suspected to be a spinal kyphosis secondary to laminectomy. We removed the tumor after posterior instrumentation, and her neurological status was restored in 3 months postoperatively. A minimal change in spinal alignment secondary to laminectomy could be a cause of intraoperative neurological deterioration during surgery at CTJ. Spinal fixation using spinal instrumentation should be performed before laminectomy, particularly in patients with preoperative neurological damage or severe spinal cord compression at CTJ. 4.

The cutoff amplitude of transcranial motor evoked potentials for transient postoperative motor deficits in intramedullary spinal cord tumor surgery.

Retrospective clinical study of intraoperative transcranial motor evoked potential (TcMEP) amplitudes and postoperative motor deficits (PMDs). To determine the quantifiable cutoff amplitude of TcMEP for predicting transient PMDs in intramedullary spinal cord tumor (IMSCT) surgery. The "presence or absence" criterion is reliable and widely used the alarm criterion for preventing permanent PMDs in IMSCT surgery. However, we wanted to prevent PMDs even if it is transient. The cutoff amplitude for transient PMDs should be identified. We conducted a retrospective study to identify the cutoff amplitude for predicting transient PMDs in IMSCT surgery. Thirty-seven patients were included in the study. We examined intraoperative electrophysiological changes and perioperative motor status in these patients. Receiver operating characteristic analyses were performed to identify the cutoff amplitudes for predicting transient PMDs in IMSCT surgery. The incidence of PMDs and cutoff TcMEP amplitude in cervical and thoracic lesions were compared. Thirteen cases demonstrated transient PMDs. Among 280 monitorable muscles in 37 cases, 51 muscles in 13 patients showed PMDs. Through receiver operating characteristic analysis, the relative and the absolute cutoff amplitudes at the intraoperative point of deterioration were identified to be 12% residual of baseline amplitude and 3.2 μV, respectively. Sensitivity/specificity for those cutoff points are 86%/74% and 88%/78%, respectively. The incidence of PMD was significantly higher, and the cutoff amplitude was lower in the thoracic lesions than in the cervical lesions. We determined the cutoff TcMEP amplitude for predicting transient PMDs in IMSCT surgery. The cutoff amplitude for the cervical lesions was higher than that for the thoracic lesions. The results suggest the need for setting different alarm criteria in different level of spine. 3.

Long-term incidence and risk factors for development of spinal deformity following resection of pediatric intramedullary spinal cord tumors.

Spinal deformity in pediatric patients with intramedullary spinal cord tumors (IMSCTs) may be either due to neurogenic disability or due to secondary effects of spinal decompression. It is associated with functional decline and impairment in health-related quality-of-life measures. The authors sought to identify the long-term incidence of spinal deformity in individuals who had undergone surgery for IMSCTs as pediatric patients and the risk factors and overall outcomes in this population. Treatment records for pediatric patients (age < 21 years) who underwent surgical treatment for histology-proven primary IMSCTs between 1975 and 2010 were reviewed. All patients were evaluated in consultation with the pediatric orthopedics service. Clinical records were reviewed for baseline and follow-up imaging studies, surgical fusion treatment, and long-term skeletal and disease outcomes. The authors identified 55 patients (30 males and 25 females) who were treated for pediatric IMSCTs between January 1975 and January 2010. The mean duration of follow-up (± SEM) was 11.4 ± 1.3 years (median 9.3 years, range 0.2-37.2 years). Preoperative skeletal deformity was diagnosed in 11 (20%) of the 55 patients, and new-onset postoperative deformity was noted in 9 (16%). Conservative management with observation or external bracing was sufficient in 8 (40%) of these 20 cases. Surgical fusion was necessary in 11 (55%). Posterior surgical fusion was sufficient in 6 (55%) of these 11 cases, while combined anterior and posterior fusion was undertaken in 5 (45%). Univariate and multivariate analysis of clinical and surgical treatment variables indicated that preoperative kyphoscoliosis (p = 0.0032) and laminectomy/laminoplasty at more than 4 levels (p = 0.05) were independently associated with development of spinal deformity that necessitated surgical fusion. Functional scores and 10-year disease survival outcomes were similar between the 2 groups. Long-term follow-up is essential to monitor for delayed development of spinal deformity, and regular surveillance imaging is recommended for patients with underlying deformity. The authors' extended follow-up highlights the risk factors associated with development of spinal deformity in patients treated for pediatric IMSCTs. Surgical fusion allows patients who develop progressive deformity to achieve long-term functional and survival outcomes comparable to those of patients who do not develop progressive deformity.

Long-term disease and neurological outcomes in patients with pediatric intramedullary spinal cord tumors.

Radical resection is recommended as the first-line treatment for pediatric intramedullary spinal cord tumors (IMSCTs), but it is associated with morbidity, including risk of neurological decline and development of postoperative spinal deformity. The authors report long-term data on clinical and treatment determinants affecting disease survival and neurological outcomes. Case records for pediatric patients (< 21 years of age at presentation) who underwent surgery for IMSCTs at the authors' institution between January 1975 and January 2010 were analyzed. The patients' demographic and clinical characteristics (including baseline neurological condition), the treatment they received, and their disease course were reviewed. Long-term disease survival and functional outcome measures were analyzed. A total of 55 patients (30 male and 25 female) were identified. The mean duration of follow-up (± SEM) was 11.4 ± 1.3 years (median 9.3 years, range 0.2-37.2 years). Astrocytomas were the most common tumor subtype (29 tumors [53%]). Gross-total resection (GTR) was achieved in 21 (38%) of the 55 patients. At the most recent follow-up, 30 patients (55%) showed neurological improvement, 17 (31%) showed neurological decline, and 8 (15%) remained neurologically stable. Patients presenting with McCormick Grade I were more likely to show functional improvement by final follow-up (p = 0.01) than patients who presented with Grades II-V. Kaplan-Meier actuarial tumor progression-free survival rates at 5, 10, and 20 years were 61%, 54%, and 44%, respectively; the overall survival rates were 85% at 5 years, 74% at 10 years, and 64% at 20 years. On multivariate analysis, GTR (p = 0.04) and tumor histological grade (p = 0.02) were predictive of long-term survival; GTR was also associated with improved 5-year progression-free survival (p = 0.01). The prognosis for pediatric IMSCTs is favorable with sustained functional improvement expected in a significant proportion of patients on long-term follow-up. Long-term survival at 10 years (75%) and 20 years (64%) is associated with aggressive resection. Gross-total resection was also associated with improved 5-year progression-free survival (86%). Hence, the treatment benefits of GTR are sustained on extended follow-up.

Quality of life after surgical treatment of primary intramedullary spinal cord tumors in children

Presently, the best available treatment for intramedullary spinal cord tumors (IMSCTs) in children is microsurgery with the objective of maximal tumor removal and minimal neurological morbidity. The latter has become manageable with the development and standard use of intraoperative neurophysiological monitoring. Traditionally, the perioperative neurological evaluation is based on surgical or spinal cord injury scores focusing on sensorimotor function. Little is known about the quality of life after such operations; therefore, this study was designed to investigate the impact of surgery for IMSCTs on the quality of life in children. Twelve consecutive pediatric patients treated for IMSCT were included in this retrospective fixed cohort study. A multidimensional questionnaire-based quality of life instrument, the Pediatric Quality of Life Questionnaire version 4 (PedsQL 4.0), was chosen to analyze follow-up data. This validated instrument particularly allows for a comparison between a patient cohort and a healthy pediatric sample population. Of 11 mailed questionnaires (1 patient had died of progressive disease), 10 were returned, resulting in a response rate of 91%. There were 8 low-grade lesions (5 pilocytic astrocytomas, 1 ganglioglioma, 1 hemangioblastoma, and 1 cavernoma) and 4 high-grade lesions (2 anaplastic gangliogliomas, 1 glioblastoma, and 1 glioneuronal tumor). The mean age at diagnosis was 7.5 years, the mean follow-up was 4.2 years, and 83% of the patients were male. Total resection was achieved in 5 patients and subtotal resection in 7. Four patients had undergone 2 or more resections. The 4 patients with high-grade tumors and 2 with incompletely resected low-grade tumors underwent adjuvant treatment (2 chemotherapy and 4 both radiotherapy and chemotherapy). The mean modified McCormick Scale score at the time of diagnosis was 1.7; at the time of follow-up, 1.5. The mean PedsQL 4.0 total score in the low-grade group was 78.5; in the high-grade group, 82.6. There was no significant difference in PedsQL 4.0 scores between the patient cohort and the normal population. In a small cohort of children who had undergone surgery for IMSCTs with a mean follow-up of 4.2 years, quality of life scores according to the PedsQL 4.0 instrument were not different from those in a normal sample population.

Massive hematomyelia following intramedullary spinal cord tumor surgery

Dear Editor, We would like to present an interesting case of postoperative hematomyelia following resection of an intramedullary spinal cord tumor (IMSCT) and discuss its clinical implications. Hematomyelia is a relatively rare phenomenon. Hematomyelia is mostly associated with traumatic insult, spinal vascular malformations such as intramedullary cavernous malformations [3, 10], or anticoagulation therapy [1, 9]. Clinical presentation varies from acute onset of spinal neurological deficits, accompanied by severe back or radicular pain, to chronic, slowly progressing neurological decline. A slow clinical course is usually associated with better prognosis [7]. Hematomyelia is a known presenting sign of spinal cord tumors [8]. It is also a rarely described complication of IMSCT surgery [2, 5]. Our patient was a 32-year-old male with a history of lung sarcoidosis presented with progressive sensorymotor disturbances of the lower limbs. MRI demonstrated multifocal pial lesions with a large intramedullary lesion at T6-7. Following rapid worsening of motor functions, he was operated upon. A subtotal resection of the lesion was performed. Pathology was consistent with low-grade glial tumor with pilocytic features. Following the first surgery, weakness on the right side improved. Eighteen months after surgery, a further decline in motor function was noticed. MRI demonstrated marked growth of the remnant tumor with homogenous enhancement and subependymal spread evident up to C6-7. The patient experienced rapid motor deterioration and underwent a second operation. A very generous subtotal re-resection was performed. Throughout the surgery, MEP and SSEP potentials monitoring remained uneventful. At the conclusion of surgery, the spinal cord was free of pressure with absolute hemostasis, and displayed pulsation and free CSF flow. Immediately following surgery the patient felt well, with evident motor improvement. However, on postoperative day 2 the patient attempted to get up from his bed unassisted and collapsed. A family member lifted the patient back to his bed. A few hours later the patient began suffering from severe back pain. In the following hours, the patient developed almost complete quadriplegia with minimal distal left hand movements. An emergency MRI revealed massive hematomyelia from the tumor site at T7 ascending all the way up to the foramen magnum (Fig. 1). A decision not to operate was made. The patient was intubated and transferred to the NICU. He underwent a tracheostomy, and over the next few weeks regained motor function in the upper limbs. The patient remains paraplegic however. B. Shofty : J. Roth :A. Korn : S. Constantini (*) Department of Pediatric Neurosurgery, Tel-Aviv Medical Center, 6th Weizman St., Tel-Aviv, Israel e-mail: sconsts@netvision.net.il