OBJECTIVE Malignant melanotic nerve sheath tumors are rare tumors characterized by neoplastic melanin-producing Schwann cells. In this study, the authors report their institution’s experience in treating spinal and peripheral malignant melanotic nerve sheath tumors and compare their results with the literature. METHODS Data were collected from 8 patients who underwent surgical treatment for malignant melanotic nerve sheath tumors between 1996 and 2023 at Mayo Clinic and 63 patients from the literature. Time-to-event analyses were performed for the combined group of 71 cases to evaluate the risk of recurrence, metastasis, and death based on tumor location and type of treatment received. Unpaired 2-sample t-tests and Fisher’s exact tests were used to determine statistical significance between groups. RESULTS Between 1996 and 2023, 8 patients with malignant melanotic nerve sheath tumors underwent surgery at the authors’ institution, while 63 patients were identified in the literature. The authors’ patients and those in the literature had the same mean age at diagnosis (43 years). At the authors’ institution, 5 patients (63%) experienced metastasis, 6 patients (75%) experienced long-term recurrence, and 5 patients (62.5%) died. In the literature, most patients (60.3%) were males, with a peak incidence between the 4th and 5th decades of life. Nineteen patients (31.1%) were diagnosed with Carney complex. Nerve root tumors accounted for most presentations (n = 39, 61.9%). Moreover, 24 patients (38.1%) had intradural lesions, with 54.2% (n = 13) being intramedullary and 45.8% (n = 11) extramedullary. Most patients underwent gross-total resection (GTR) (n = 41, 66.1%), followed by subtotal resection (STR) (n = 12, 19.4%), STR with radiation therapy (9.7%), and GTR with radiation therapy (4.8%). Sixteen patients (27.6%) experienced metastasis, 23 (39.7%) experienced recurrence, and 13 (22%) died. Kaplan-Meier analyses showed no significant differences among treatment approaches in terms of recurrence-free, metastasis-free, and overall survival (p > 0.05). Similar results were obtained when looking at the differences with respect to intradural versus nerve root location of the tumor (p > 0.05). CONCLUSIONS Malignant melanotic nerve sheath tumors are rare tumors with a high potential for malignancy. They carry a dismal prognosis, with a pooled local recurrence rate of 42%, distant metastasis rate of 27%, and mortality rate of 26%. The findings from this study suggest a trend favoring the use of GTR alone or STR with radiation therapy over STR alone. Mortality was similar regardless, which highlights the need for the development of effective treatment options to improve survival in patients with melanotic schwannomas.
Paraspinal Sarcopenia is Associated With Worse Patient-Reported Outcomes Following Laminoplasty for Degenerative Cervical Myelopathy
Study Design. Retrospective cohort study. Objective. The present study is the first to assess the impact of paraspinal sarcopenia on patient-reported outcome measures (PROMs) following cervical laminoplasty. Background. While the impact of sarcopenia on PROMs following lumbar spine surgery is well-established, the impact of sarcopenia on PROMs following laminoplasty has not been investigated. Methods. We performed a retrospective review of patients undergoing laminoplasty from C4-6 at a single institution between 2010 and 2021. Two independent reviewers utilized axial cuts of T2-weighted magnetic resonance imaging sequences to assess fatty infiltration of the bilateral transversospinales muscle group at the C5-6 level and classify patients according to the Fuchs Modification of the Goutalier grading system. PROMs were then compared between subgroups. Results. We identified 114 patients for inclusion in this study, including 35 patients with mild sarcopenia, 49 patients with moderate sarcopenia, and 30 patients with severe sarcopenia. There were no differences in preoperative PROMs between subgroups. Mean postoperative neck disability index scores were lower in the mild and moderate sarcopenia subgroups (6.2 and 9.1, respectively) than in the severe sarcopenia subgroup (12.9, P=0.01). Patients with mild sarcopenia were nearly twice as likely to achieve minimal clinically important difference (88.6 vs. 53.5%; P<0.001) and six times as likely to achieve SCB (82.9 vs. 13.3%; P=0.006) compared with patients with severe sarcopenia. A higher percentage of patients with severe sarcopenia reported postoperative worsening of their neck disability index (13 patients, 43.3%; P=0.002) and Visual Analog Scale Arm scores (10 patients, 33.3%; P=0.03). Conclusion. Patients with severe paraspinal sarcopenia demonstrate less improvement in neck disability and pain postoperatively and are more likely to report worsening PROMs following laminoplasty. Level of Evidence. 3
Is Severe Neck Pain a Contraindication to Performing Laminoplasty in Patients With Cervical Spondylotic Myelopathy?
Study Design: Retrospective review. Objective: The purpose of this study was to investigate the surgical outcomes in a cohort of patients with severe preoperative axial neck pain undergoing laminoplasty for cervical spondylotic myelopathy (CSM). Summary of Background Data: No study has investigated whether patients with severe axial symptoms may achieve satisfactory neck pain and disability outcomes after laminoplasty. Methods: We performed a retrospective review of 91 patients undergoing C4-6 laminoplasty for CSM at a single academic institution between 2010 and 2021. Patient-reported outcome measures (PROMs), including Neck Disability Index (NDI), visual analog scale (VAS) Neck, and VAS Arm, were recorded preoperatively and at 6 months and 1 year postoperatively. Patients were stratified as having mild pain if VAS neck was 0–3, moderate pain if 4–6, and severe pain if 7–10. PROMs were then compared between subgroups at all the perioperative time points. Results: Both the moderate and severe neck pain subgroups demonstrated a substantial improvement in VAS neck from preoperative to 6 months postoperatively (−3.1±2.2 vs. −5.6±2.8, respectively; P<0.001), and these improvements were maintained at 1 year postoperatively. There was no difference in VAS neck between subgroups at either the 6-month or 1-year postoperative time points. Despite the substantially higher mean NDI in the moderate and severe neck pain subgroups preoperatively, there was no difference in NDI at 6 months or 1 year postoperatively (P=0.99). There were no differences between subgroups in the degree of cord compression, severity of multifidus sarcopenia, sagittal alignment, or complications. Conclusions: Patients with moderate and severe preoperative neck pain undergoing laminoplasty achieved equivalent PROMs at 6 months and 1 year as patients with mild preoperative neck pain. The results of this study highlight the multifactorial nature of neck pain in these patients and indicate that severe axial symptoms are not an absolute contraindication to performing laminoplasty in well-aligned patients with CSM.
En bloc resection of a high cervical chordoma followed by reconstruction with a free vascularized fibular graft: illustrative case
BACKGROUND Wide excision of chordoma provides better local control than intralesional resection or definitive radiotherapy. The en bloc excision of high cervical chordomas is a challenging endeavor because of the complex anatomy of this region and limited reconstructive options. OBSERVATIONS This is the first case report to describe reconstruction with a free vascularized fibular graft following the en bloc excision of a chordoma involving C1–3. LESSONS This report demonstrates the durability of this construct at 10-year follow-up and is the first case report demonstrating satisfactory long-term oncological outcomes after a true margin-negative resection of a high cervical chordoma.
At Mean 30-Year Follow-Up, Cervical Spine Disease Is Common and Associated with Thoracic Hypokyphosis after Pediatric Treatment of Adolescent Idiopathic Scoliosis
Patients with adolescent idiopathic scoliosis (AIS) often have reduced sagittal thoracic kyphosis (hypokyphosis) and cervical lordosis causing an uneven distribution of physiologic load. However, the long-term consequences of hypokyphosis in AIS patients have not been previously documented. To evaluate whether uneven load distribution leads to future complications in patients with AIS, we conducted a retrospective chart review and subsequently surveyed 180 patients treated for idiopathic scoliosis between 1975 and 1992. These patients all had a minimum follow-up time of 20 years since their treatment. We observed a ten-fold increase in the incidence of anterior cervical discectomy and fusion (ACDF) compared to reported rates in the non-pathologic population. Out of the 180 patients, 33 patients met the criteria and returned for follow-up radiographs. This population demonstrated a statistically significant increased rate of cervical osteoarthritis and disc degeneration. Overall, our study suggests that hypokyphosis in patients with AIS presents with increased rates of cervical spine degeneration and dysfunction, suggesting that these patients may require additional follow-up and treatment.
Semispinalis Cervicis Sarcopenia is Associated With Worsening Cervical Sagittal Balance and Junctional Alignment Following Posterior Cervical Fusion for Myelopathy
Study Design: This was a retrospective cohort study. Objective: The present study is the first to investigate whether cervical paraspinal sarcopenia is associated with cervicothoracic sagittal alignment parameters after posterior cervical fusion (PCF). Summary of Background Data: Few studies have investigated the association between sarcopenia and postoperative outcomes after cervical spine surgery. Methods: We retrospectively reviewed patients undergoing PCF from C2–T2 at a single institution between the years 2017–2020. Two independent reviewers utilized axial cuts of T2-weighted magnetic resonance imaging sequences to perform Goutallier classification of the bilateral semispinalis cervicis (SSC) muscles. Cervical sagittal alignment parameters were compared between subgroups based upon severity of SSC sarcopenia. Results: We identified 61 patients for inclusion in this study, including 19 patients with mild SSC sarcopenia and 42 patients with moderate or severe SSC sarcopenia. The moderate-severe sarcopenia subgroup demonstrated a significantly larger change in C2–C7 sagittal vertical axis (+6.8 mm) from the 3-month to 1-year postoperative follow-up in comparison to the mild sarcopenia subgroup (−2.0 mm; P=0.02). The subgroup of patients with moderate-severe sarcopenia also demonstrated an increase in T1–T4 kyphosis (10.9–14.2, P=0.007), T1 slope (28.2–32.4, P=0.003), and C2 slope (24.1–27.3, P=0.05) from 3-month to 1-year postoperatively and a significant decrease in C1-occiput distance (6.3–4.1, P=0.002) during this same interval. Conclusions: In a uniform cohort of patients undergoing PCF from C2–T2, SSC sarcopenia was associated with worsening cervicothoracic alignment from 3-month to 1-year postoperatively.
Multifidus Sarcopenia Is Associated With Worse Patient-reported Outcomes Following Posterior Cervical Decompression and Fusion
Study Design. Retrospective cohort study. Objective. The present study is the first to assess the impact of paraspinal sarcopenia on patient-reported outcome measures (PROMs) following posterior cervical decompression and fusion (PCDF). Summary of Background Data. While the impact of sarcopenia on PROMs following lumbar spine surgery is well-established, the impact of sarcopenia on PROMs following PCDF has not been investigated. Materials and Methods. We performed a retrospective review of patients undergoing PCDF from C2 to T2 at a single institution between the years 2017 and 2020. Two independent reviewers who were blinded to the clinical outcome scores utilized axial cuts of T2-weighted magnetic resonance imaging sequences to assess fatty infiltration of the bilateral multifidus muscles at the C5–C6 level and classify patients according to the Fuchs Modification of the Goutalier grading system. PROMs were then compared between subgroups. Results. We identified 99 patients for inclusion in this study, including 28 patients with mild sarcopenia, 45 patients with moderate sarcopenia, and 26 patients with severe sarcopenia. There was no difference in any preoperative PROM between the subgroups. Mean postoperative Neck Disability Index scores were lower in the mild and moderate sarcopenia subgroups (12.8 and 13.4, respectively) than in the severe sarcopenia subgroup (21.0, P <0.001). A higher percentage of patients with severe multifidus sarcopenia reported postoperative worsening of their Neck Disability Index (10 patients, 38.5%; P =0.003), Visual Analog Scale Neck scores (7 patients, 26.9%; P =0.02), Patient-Reported Outcome Measurement Information System Physical Component Scores (10 patients, 38.5%; P =0.02), and Patient-Reported Outcome Measurement Information System Mental Component Scores (14 patients, 53.8%; P =0.02). Conclusion. Patients with more severe paraspinal sarcopenia demonstrate less improvement in neck disability and physical function postoperatively and are substantially more likely to report worsening PROMs postoperatively. Level of Evidence. 3.
Rheumatoid Arthritis in Spine Surgery: A Systematic Review and Meta-Analysis
Study Design Systematic Review and Meta-analysis Objective The purpose of this study is to synthesize recommendations for perioperative medical management of RA patients and quantify outcomes after spine surgery when compared to patients without RA. Methods A search of available literature on patients with RA and spine surgery was performed. Studies were included if they provided a direct comparison of outcomes between patients undergoing spine surgery with or without RA diagnosis. Meta-analysis was performed on operative time, estimated blood loss, hospital length of stay, overall complications, implant-related complications, reoperation, infection, pseudarthrosis, and adjacent segment disease. Results Included in the analysis were 9 studies with 703 patients with RA undergoing spine surgery and 2569 patients without RA. In RA patients compared to non-RA patients undergoing spine surgery, the relative risk of infection was 2.29 times higher (P = .036), overall complications 1.61 times higher (P < .0001), implant-related complications 3.93 times higher (P = .009), and risk of reoperation 2.45 times higher (P < .0001). Hospital length of stay was 4.6 days longer in RA patients (P < .0001). Conclusions Treatment of spinal pathology in patients with RA carries an increased risk of infection and implant-related complications. Spine-specific guidelines for perioperative management of antirheumatic medication deserve further exploration. All RA patients should be perioperatively co-managed by a rheumatologist. This review helps identify risk profiles in RA specific to spine surgery and may guide future studies seeking to medically optimize RA patients perioperatively.
Cervical Alignment and Proximal and Distal Junctional Failure in Posterior Cervical Fusion
Study Design: This was a multicenter retrospective cohort study. Objective: The purpose of this study was to compare the surgical and radiographic outcomes of patients undergoing posterior cervical fusion (PCF) with constructs extending from C2 to T2 to patients with constructs extending from C3 to T1. Summary of Background Data: Limited evidence exists regarding the appropriate level of proximal and distal extension of PCF constructs. Methods: A multicenter retrospective cohort study of patients who underwent PCF between 2012 and 2020 was performed. Surgical and radiographic outcomes were compared between those who had C3–T1 or C2–T2 constructs. Results: A total of 155 patients were included in the study (C2–T2: 106 patients, C3–T1: 49 patients). There were no significant differences in demographics or preoperative symptoms between cohorts. Fusion rates were significantly higher in the C2–T2 (93%) than the C3–T1 (80%, P=0.040) cohort. When comparing the C2–T2 to the C3–T1 cohort, the C3–T1 cohort had a significantly greater rate of proximal junctional failure (2% vs. 10%, P=0.006), distal junctional failure (1% vs. 20%, P<0.001) and distal screw loosening (4% vs. 15%, P=0.02). Although ∆C2–C7 sagittal vertical axis increased significantly in both cohorts (C2–T2: 6.2 mm, P=0.04; C3–T1: 8.4 mm, P<0.001), correction did not significantly differ between groups (P=0.32). The C3–T1 cohort had a significantly greater increase in ∆C2 slope (8.0 vs. 3.1 degrees, P=0.03) and ∆C0–C2 Cobb angle (6.4 vs. 1.2 degrees, P=0.04). Conclusion: In patients undergoing PCF, a C2–T2 construct demonstrated lower rates of pseudarthrosis, distal junctional failure, proximal junctional failure, and compensatory upper cervical hyperextension compared with a C3–T1 construct.
Study Design Retrospective cohort study Objective Substantial variability in both the measurement and classification of subsidence limits the strength of conclusions that can be drawn from previous studies. The purpose of this study was to precisely characterize patterns of cervical cage subsidence utilizing computed tomography (CT) scans, determine risk factors for cervical cage subsidence, and investigate the impact of subsidence on pseudarthrosis rates. Methods We performed a retrospective review of patients who underwent one- to three-levels of anterior cervical discectomy and fusion (ACDF) utilizing titanium interbodies with anterior plating between the years 2018 and 2020. Subsidence measurements were performed by two independent reviewers on CT scans obtained 6 months postoperatively. Subsidence was then classified as mild if subsidence into the inferior and superior endplate were both ≤2 mm, moderate if the worst subsidence into the inferior or superior endplate was between 2 to 4 mm, or severe if the worst subsidence into the inferior or superior endplate was ≥4 mm. Results A total of 51 patients (100 levels) were included in this study. A total of 48 levels demonstrated mild subsidence (≤2 mm), 38 demonstrated moderate subsidence (2-4 mm), and 14 demonstrated severe subsidence (≥4 mm). Risk factors for severe subsidence included male gender, multilevel constructs, greater mean vertebral height loss, increased cage height, lower Taillard index, and lower screw tip to vertebral body height ratio. Severe subsidence was not associated with an increased rate of pseudarthrosis. Conclusion Following ACDF with titanium cervical cages, subsidence is an anticipated postoperative occurrence and is not associated with an increased risk of pseudarthrosis.