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Familial Chiari malformation: a systematic review and illustrative cases

Alaina Dhawan, Jillian Dhawan, Ajay N. Sharma, Daniel B. Azzam, Ahmed Cherry, and Michael G. Fehlings

OBJECTIVE

Chiari malformations (CMs) are a group of congenital or acquired disorders characterized by hindbrain overcrowding into an underdeveloped posterior cranial fossa. CM is considered largely sporadic—however, there exists growing evidence of transmissible genetic underpinnings. The purpose of this systematic review of all familial studies of CM was to investigate the existence of an inherited component and provide recommendations to manage and monitor at-risk family members.

METHODS

This paper includes the following: 1) a unique case report of dizygotic twins who presented at the Toronto Western Hospital Spinal Cord Clinic with symptomatic CM type 1 (CM-1) and syringomyelia; and 2) a systematic review of familial CM. The EMBASE and MEDLINE databases were searched on June 27, 2023, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Only articles in the English language concerning the diagnosis of CM in > 1 human family member presented as a case study, case series, or literature review were included.

RESULTS

Among the 29 articles included in the final analysis, a total of 34 families with CM were analyzed. An average of 3 cases of CM were found per family among all generations. Eighty-one cases (88%) reported CM-1, whereas the other 11 (12%) cases reported either CM-0, CM-1.5, or tonsillar ectopia. A syrinx was present in 37 (54%) cases, with 14 (38%) of these patients also reporting a skeletal abnormality, the most common comorbidity. Most family members diagnosed with CM were siblings (18; 35%), followed by monozygotic twins/triplets (12; 23%).

CONCLUSIONS

Patients most often presented with headaches, sensory disturbances, or generalized symptoms. Overall, there exists mounting evidence for a hereditary component of CM. It is unlikely to be explained by a classic mendelian inheritance pattern, but is rather a polygenic architecture influenced by variable penetrance, cosegregation, and entirely nongenetic factors. For first-degree relatives of those affected by CM, the authors’ findings may influence clinicians to conduct closer clinical and radiographic monitoring, promote patient education, and consider earlier genetic testing.

Free access

Letter to the Editor. Methylprednisolone following acute traumatic spinal cord injury

Mark S. Nash, James V. Boddu, and Barth A. Green

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Adverse effects of frailty on the outcomes of surgery for degenerative cervical myelopathy: results from a prospective multicenter international data set of 757 patients

Jamie R. F. Wilson, Jetan H. Badhiwala, Ali Moghaddamjou, Alexander R. Vaccaro, Paul M. Arnold, Ronald H. M. A. Bartels, Giuseppe Barbagallo, and Michael G. Fehlings

OBJECTIVE

The goal of this study was to determine the effect of the degree of frailty on long-term neurological and functional outcomes after surgery for degenerative cervical myelopathy (DCM).

METHODS

A combined database of patients enrolled in the Cervical Spondylotic Myelopathy–North America and Cervical Spondylotic Myelopathy–International prospective international multicenter observational studies who underwent surgery for DCM was used as the source data. All patients underwent baseline and follow-up assessment at 2 years after surgery for functional, disability, and quality of life measurements (modified Japanese Orthopaedic Association [mJOA] scale, Neck Disability Index, SF-36 physical and mental component summary scores). Patients were separated into 4 groups according to their baseline modified frailty index 5-point scale score: not frail, pre-frail, frail, and severely frail. Differences among groups were analyzed at baseline and at 2 years after surgery, including change in scores (delta values) and the odds ratio of achieving the minimum clinically important difference (MCID) through univariate and multivariable logistic regression adjusting for age, approach, number of levels treated, and sex.

RESULTS

A total of 757 patients (63% male) with a mean age of 56 (95% CI 55.5–57.2) years were included: 470 patients underwent an anterior approach, 310 had a posterior approach, and 23 had a combined anterior/posterior approach. A total of 50% (n = 378) of patients were classified as not frail, with 33% (n = 250) pre-frail, 13% (n = 101) frail, and 4% (n = 28) severely frail. The baseline mJOA score was significantly lower with increasing frailty (14.00 [95% CI 13.75–14.19] for not frail vs 9.71 [95% CI 9.01–10.42] for severely frail patients; p < 0.05), but the change at 2 years was not significantly different among all groups (2.43 [95% CI 2.16–2.71] for not frail vs 2.56 [95% CI 1.10–4.02] for severely frail). The SF-36 delta values were also not different among groups, but significantly worse at baseline with increasing frailty. The odds ratio of achieving MCID for mJOA was significantly higher in the not frail group (1.89 [95% CI 1.36–2.61]; p < 0.05) compared to the other frailty cohorts, which remained after adjusting for age, approach, levels treated, and sex. The odds ratio of achieving MCID for the SF-36 domains was similar among all frailty groups.

CONCLUSIONS

Increasing frailty is associated with worse baseline functional and quality of life measures in patients undergoing surgery for DCM. Frailty does not affect the magnitude of improvement in outcome measures after surgery, but reduces the chance of achieving the MCID for functional impairment significantly. Preoperative frailty assessment can therefore help guide clinicians in managing expectations after surgery for DCM. Potentially modifiable factors should be optimized in frail patients preoperatively to enhance functional outcomes.

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Analysis of recovery trajectories in degenerative cervical myelopathy to facilitate improved patient counseling and individualized treatment recommendations

Blessing N. R. Jaja, Christopher D. Witiw, Erin M. Harrington, Yingshi He, Ali Moghaddamjou, Michael G. Fehlings, and Jefferson R. Wilson

OBJECTIVE

There is a need to better understand and predict postsurgical outcomes for degenerative cervical myelopathy (DCM) patients, particularly to support treatment decisions for patients with mild DCM. The goal of this study was to identify and predict outcome trajectories for DCM patients up to 2 years postsurgery.

METHODS

The authors analyzed two North American multicenter prospective DCM studies (n = 757). Functional recovery and physical health component quality of life were assessed in DCM patients at baseline, 6 months, and 1 and 2 years postoperatively using the modified Japanese Orthopaedic Association (mJOA) score and Physical Component Summary (PCS) of the SF-36, respectively. Group-based trajectory modeling was used to identify recovery trajectories for mild, moderate, and severe DCM. Prediction models for recovery trajectories were developed and validated in bootstrap resamples.

RESULTS

Two recovery trajectories were identified for the functional and physical components of quality of life: good recovery and marginal recovery. Depending on outcome and myelopathy severity, one-half to three-fourths of the study patients followed the good recovery trajectory characterized by improvement in mJOA and PCS scores over time. The remaining one-half to one-fourth of patients followed the marginal recovery trajectory, experiencing little improvement and, in certain cases, worsening postoperatively. The prediction model for mild DCM had an area under the curve of 0.72 (95% CI 0.65–0.80), with preoperative neck pain, smoking, and posterior surgical approach noted as dominant predictors of marginal recovery.

CONCLUSIONS

Surgically treated DCM patients follow distinct recovery trajectories in the first 2 years postoperatively. While most patients experience substantial improvement, a significant minority experience little improvement or worsening. The ability to predict DCM patient recovery trajectories in the preoperative setting facilitates the formulation of individualized treatment recommendations for patients with mild symptoms.

Open access

Perception of frailty in spinal metastatic disease: international survey of the AO Spine community

Mark A. MacLean, Miltiadis Georgiopoulos, Raphaële Charest-Morin, C. Rory Goodwin, Ilya Laufer, Nicolas Dea, John H. Shin, Ziya L. Gokaslan, Laurence D. Rhines, John E. O’Toole, Daniel M. Sciubba, Michael G. Fehlings, Byron F. Stephens, Chetan Bettegowda, Sten Myrehaug, Alexander C. Disch, Cordula Netzer, Naresh Kumar, Arjun Sahgal, Niccole M. Germscheid, Michael H. Weber, and on behalf of the AO Spine Knowledge Forum Tumor

OBJECTIVE

Frailty has not been clearly defined in the context of spinal metastatic disease (SMD). Given this, the objective of this study was to better understand how members of the international AO Spine community conceptualize, define, and assess frailty in SMD.

METHODS

The AO Spine Knowledge Forum Tumor conducted an international cross-sectional survey of the AO Spine community. The survey was developed using a modified Delphi technique and was designed to capture preoperative surrogate markers of frailty and relevant postoperative clinical outcomes in the context of SMD. Responses were ranked using weighted averages. Consensus was defined as ≥ 70% agreement among respondents.

RESULTS

Results were analyzed for 359 respondents, with an 87% completion rate. Study participants represented 71 countries. In the clinical setting, most respondents informally assess frailty and cognition in patients with SMD by forming a general perception based on clinical condition and patient history. Consensus was attained among respondents regarding the association between 14 preoperative clinical variables and frailty. Severe comorbidities, extensive systemic disease burden, and poor performance status were most associated with frailty. Severe comorbidities associated with frailty included high-risk cardiopulmonary disease, renal failure, liver failure, and malnutrition. The most clinically relevant outcomes were major complications, neurological recovery, and change in performance status.

CONCLUSIONS

The respondents recognized that frailty is important, but they most commonly evaluate it based on general clinical impressions rather than using existing frailty tools. The authors identified numerous preoperative surrogate markers of frailty and postoperative clinical outcomes that spine surgeons perceived as most relevant in this population.

Open access

Methylprednisolone in acute traumatic spinal cord injury: case-matched outcomes from the NASCIS2 and Sygen historical spinal cord injury studies with contemporary statistical analysis

Fred H. Geisler, Ali Moghaddamjou, Jamie R. F. Wilson, and Michael G. Fehlings

OBJECTIVE

Methylprednisolone (MP) to treat acute traumatic spinal cord injury (ATSCI) remains controversial since the release of the second National Acute Spinal Cord Injury Study (NASCIS2) in 1990. As two historical studies, NASCIS2 and Sygen in ATSCI, used identical MP dosages, it was possible to construct a new case-level pooled ATSCI data set satisfying contemporary criteria and able to clarify the effect of MP.

METHODS

The new pooled data set was first modernized by excluding patients with injury levels caudal to T10, lower-extremity American Spinal Injury Association (ASIA) motor scores (LEMSs) ≥ 46, Glasgow Coma Scale scores ≤ 11, and age < 15 or > 75 years, and then standardized to the ASIA grading and scoring format. A new updated NASCIS2 data set from this pooled data set contained 31.6% fewer patients than the 1990 NASCIS2 data set.

RESULTS

In the new pooled data set, recovery of LEMSs from baseline to 26 weeks, the primary outcome variable, was separated statistically into five different injury severity cohorts (p < 0.0001). The severity cohorts contained groups with severe floor (62.9%) and ceiling (10.7%) effects, which do not contribute to drug effects. The new NASCIS2 data set duplicated the p value for MP versus placebo in the sub-subgroup analysis of MP initiated ≤ 8 hours (the subgroup) and recovery of motor function on only the right side of the body (a further subgroup within the ≤ 8-hour subgroup), presented as the positive MP effect in the original NASCIS2 reporting. However, current statistical interpretation considers results seen only in post hoc sub-subgroups, without multi-test corrections, to be random effects without clinical significance. The combined case-level pooled data set from the NASCIS2 and Sygen studies increased the MP group from 106 to 431 patients, creating a new MP combined group. This new data set served as a surrogate for a contemporary MP study and found that administration of MP did not enhance ASIA motor score improvement in the lower extremities at 26 weeks. Secondary analysis of descending ASIA motor and sensory cervical neurological levels in cervical ATSCI patients at 26 weeks also found no MP drug effect.

CONCLUSIONS

Analysis of both the new updated NASCIS2 data set and the new case-matched pooled data set from two historical ATSCI studies revealed that administration of MP after spinal cord injury did not demonstrate any enhancement in neurological recovery at 26 weeks. The results of this analysis warrant review by clinical guideline groups.

Free access

Spinal manifestations of Ehlers-Danlos syndrome: a scoping review

Nandan Marathe, Laura-Nanna Lohkamp, and Michael G. Fehlings

OBJECTIVE

Since its initial description, the definition of Ehlers-Danlos syndrome (EDS) has notably changed. At present, it broadly refers to disorders of the connective tissue that are heritable and have similar features including joint hypermobility, dermal dysplasia, and vascular as well as internal organ fragility. There has been no comprehensive review of spinal manifestations of EDS in the recent literature. That has led to controversies in management protocols of this so-called orphan disease.

METHODS

The authors used the latest version of the EDS classification from 2017, in which 13 subtypes were recognized. EDS has 19 different causal genes, mainly associated with collagen synthesis. Of these, 5 subtypes have associated spinal manifestations.

RESULTS

Some of the spinal pathologies associated with EDS include Chiari malformation, craniocervical instability, kyphoscoliosis, segmental instability and kyphosis, spontaneous CSF leaks, Tarlov cyst syndrome, tethered cord, and problems associated with wound healing. Here, the authors briefly discuss the demographics, etiology, pathophysiology, clinical features, management strategies, and directions for further research for each of these manifestations.

CONCLUSIONS

EDS belongs to the group of orphan diseases, with the total patient population being below 200,000. Further research on spinal manifestations of EDS is the need of the hour to establish clinical practice guidelines and close the significant knowledge gaps that currently exist.

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Long-term functional outcome of surgical treatment for degenerative cervical myelopathy

Mark D. Dijkman, Martine W. T. van Bilsen, Michael G. Fehlings, and Ronald H. M. A. Bartels

OBJECTIVE

Degenerative cervical myelopathy (DCM) is a major global cause of spinal cord dysfunction. Surgical treatment is considered a safe and effective way to improve functional outcome, although information about long-term functional outcome remains scarce despite increasing longevity. The objective of this study was to describe functional outcome 10 years after surgery for DCM.

METHODS

A prospective observational cohort study was undertaken in a university-affiliated neurosurgery department. All patients who underwent surgery for DCM between 2008 and 2010 as part of the multicenter Cervical Spondylotic Myelopathy International trial were included. Participants were approached for additional virtual assessment 10 years after surgery. Functional outcome was assessed according to the modified Japanese Orthopaedic Association (mJOA; scores 0–18) score at baseline and 1, 2, and 10 years after surgery. The minimal clinically important difference was defined as 1-, 2-, or 3-point improvement for mild, moderate, and severe myelopathy, respectively. Outcome was considered durable when stabilization or improvement after 2 years was maintained at 10 years. Self-evaluated effect of surgery was assessed using a 4-point Likert-like scale. Demographic, clinical, and surgical data were compared between groups that worsened and improved or remained stable using descriptive statistics. Functional outcome was compared between various time points during follow-up with linear mixed models.

RESULTS

Of the 42 originally included patients, 37 participated at follow-up (11.9% loss to follow-up, 100% response rate). The mean patient age was 56.1 years, and 42.9% of patients were female. Surgical approaches were anterior (76.2%), posterior (21.4%), or posterior with fusion (2.4%). The mean follow-up was 10.8 years (range 10–12 years). The mean mJOA score increased significantly from 13.1 (SD 2.3) at baseline to 14.2 (SD 3.3) at 10 years (p = 0.01). A minimal clinically important difference was achieved in 54.1%, and stabilization of functional status was maintained in 75.0% in the long term. Patients who worsened were older (median 63 vs 52 years, p < 0.01) and had more comorbidities (70.0% vs 25.9%, p < 0.01). A beneficial effect of surgery was self-reported by 78.3% of patients.

CONCLUSIONS

Surgical treatment for DCM results in satisfactory improvement of functional outcome that is maintained at 10-year follow-up.

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Clinical outcomes of nonoperatively managed degenerative cervical myelopathy: an ambispective longitudinal cohort study in 117 patients

Allan R. Martin, Sukhvinder Kalsi-Ryan, Muhammad A. Akbar, Anna C. Rienmueller, Jetan H. Badhiwala, Jefferson R. Wilson, Lindsay A. Tetreault, Aria Nouri, Eric M. Massicotte, and Michael G. Fehlings

OBJECTIVE

Degenerative cervical myelopathy (DCM) is among the most common pathologies affecting the spinal cord but its natural history is poorly characterized. The purpose of this study was to investigate functional outcomes in patients with DCM who were managed nonoperatively as well as the utility of quantitative clinical measures and MRI to detect deterioration.

METHODS

Patients with newly diagnosed DCM or recurrent myelopathic symptoms after previous surgery who were initially managed nonoperatively were included. Retrospective chart reviews were performed to analyze clinical outcomes and anatomical MRI scans for worsening compression or increased signal change. Quantitative neurological assessments were collected prospectively, including modified Japanese Orthopaedic Association (mJOA) score; Quick-DASH; graded redefined assessment of strength, sensation, and prehension–myelopathy version (GRASSP–M: motor, sensory, and dexterity); grip dynamometer; Berg balance scale score; gait stability ratio; and gait variability index. A deterioration of 10% was considered significant (e.g., a 2-point decrease in mJOA score).

RESULTS

A total of 117 patients were included (95 newly diagnosed, 22 recurrent myelopathy), including 74 mild, 28 moderate, and 15 severe cases. Over a mean follow-up of 2.5 years, 57% (95% CI 46%–67%) of newly diagnosed patients and 73% (95% CI 50%–88%) of patients with recurrent DCM deteriorated neurologically. Deterioration was best detected with grip strength (60%), GRASSP dexterity (60%), and gait stability ratio (50%), whereas the mJOA score had low sensitivity (33%) in 50 patients. A composite score had a sensitivity of 81% and a specificity of 82%. The sensitivity of anatomical MRI was 28% (83 patients).

CONCLUSIONS

DCM appears to have a poor natural history; however, prospective studies are needed for validation. Serial assessments should include mJOA score, grip strength, dexterity, balance, and gait analysis. The absence of worsening on anatomical MRI or in mJOA scores is not sufficient to determine clinical stability.

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A comparison of the perioperative outcomes of anterior surgical techniques for the treatment of multilevel degenerative cervical myelopathy

Jetan H. Badhiwala, Sean N. Leung, Yosef Ellenbogen, Muhammad A. Akbar, Allan R. Martin, Fan Jiang, Jamie R. F. Wilson, Farshad Nassiri, Christopher D. Witiw, Jefferson R. Wilson, and Michael G. Fehlings

OBJECTIVE

Degenerative cervical myelopathy (DCM) is the most common cause of spinal cord dysfunction in adults. Multilevel ventral compressive pathology is routinely managed through anterior decompression and reconstruction, but there remains uncertainty regarding the relative safety and efficacy of multiple discectomies, multiple corpectomies, or hybrid corpectomy-discectomy. To that end, using a large national administrative healthcare data set, the authors sought to compare the perioperative outcomes of anterior cervical discectomy and fusion (ACDF), anterior cervical corpectomy and fusion (ACCF), and hybrid corpectomy-discectomy for multilevel DCM.

METHODS

Patients with a primary diagnosis of DCM who underwent an elective anterior cervical decompression and reconstruction operation over 3 cervical spinal segments were identified from the 2012–2017 National Surgical Quality Improvement Program database. Patients were separated into those undergoing 3-level discectomy, 2-level corpectomy, or a hybrid procedure (single-level corpectomy plus additional single-level discectomy). Outcomes included 30-day mortality, major complication, reoperation, and readmission, as well as operative duration, length of stay (LOS), and routine discharge home. Outcomes were compared between treatment groups by multivariable regression, adjusting for age and comorbidities (modified Frailty Index). Effect sizes were reported by adjusted odds ratio (aOR) or mean difference (aMD) and associated 95% confidence interval.

RESULTS

The study cohort consisted of 1298 patients; of these, 713 underwent 3-level ACDF, 314 2-level ACCF, and 271 hybrid corpectomy-discectomy. There was no difference in 30-day mortality, reoperation, or readmission among the 3 procedures. However, on both univariate and adjusted analyses, compared to 3-level ACDF, 2-level ACCF was associated with significantly greater risk of major complication (aOR 2.82, p = 0.005), longer hospital LOS (aMD 0.8 days, p = 0.002), and less frequent discharge home (aOR 0.59, p = 0.046). In contrast, hybrid corpectomy-discectomy had comparable outcomes to 3-level ACDF but was associated with significantly shorter operative duration (aMD −16.9 minutes, p = 0.002).

CONCLUSIONS

The authors found multiple discectomies and hybrid corpectomy-discectomy to have a comparable safety profile in treating multilevel DCM. In contrast, multiple corpectomies were associated with a higher complication rate, longer hospital LOS, and lower likelihood of being discharged directly home from the hospital, and may therefore be a higher-risk operation.