Cervical kyphosis correction in Marfan syndrome: our experience and literature review.

BACKGROUND: Spinal deformities are common in Marfan syndrome (MFS). They usually involve the thoraco-lumbar spine but rarely involves the cervical spine. Kyphosis is the common spine deformity of the cervical spine and mandates surgical correction as they are at risk of neurological deterioration since they are refractory to conservative management. Few studies of surgical correction of spine deformity included cervical deformity. OBJECTIVES: To analyze the challenges faced during surgery, clinical and radiological outcome, and complications following surgical correction for cervical kyphosis in Marfan syndrome. METHODS: We identified that 5 patients with a diagnosis of MFS with cervical kyphosis who underwent fusion surgery between the years 2010 and 2022 were reviewed, retrospectively. We analyzed the demographic details, radiological parameters, operative variables (blood loss and nuances), perioperative complications, length of stay, clinical and radiological outcome, and complications following fusion surgery for cervical kyphosis in MFS. RESULTS: The mean age of patients was 16.6 ± 4.72 years (range, 12-23 years). The average kyphotic vertebra involved is 3 ± 0.7 bodies (range 2-4) with 2 patients with thoracic deformity. All patients underwent surgical deformity correction. All patients improved clinically with Nurick grade (pre vs. post: 3.4 vs. 2.2) and mJOA (pre vs. post: 8.2 vs. 12.6). There was significant deformity correction from 37.48° to 9.1°. Mean blood loss encountered was 900 ± 173.2 ml. Perioperative complications: wound complication with CSF leak (1). Late complications: ventilator dependence (1) and junctional kyphosis (1). Mean length of hospital stay was 103 ± 178.9 days. All patients were doing symptomatically better after mean follow-up of 58 ± 28.32 months. One patient is bedridden and hospitalized. CONCLUSION: Cervical kyphosis is a rare spine deformity in patients with MFS, and they usually present with neurological deterioration mandating surgical correction. Multidisciplinary approach (pediatrics, genetics and cardiology) is required for systematic evaluation of these patients. They should be evaluated with necessary imaging to rule out associated spinal deformity (atlanto-axial subluxation, scoliosis, and intraspinal pathology like ductal ectasia). Our results suggest better surgical outcome in terms of low operative complications with neurologic improvement in MFS patients. These patients require regular follow-up to identify late complications (instrument failure, non-union, and pseudarthrosis).

Correlation between the severity of myelopathy and cervical morphometric parameters on dynamic magnetic resonance imaging.

BACKGROUND: To compare morphometric factors of cervical spine in the cervical stenosis on dynamic and static magnetic resonance imaging. To analyse the relationship between the severity of myelopathy and morphometric parameters. METHODS: Patients with cervical canal stenosis scanned with MRI in neutral (N), flexion (F) and extension (E) positions were retrospectively reviewed. All cases were evaluated in Nurick and Muhle grades. Following parameters were measured: anterior and posterior length of the cervical cord (aLCC, pLCC), mid-cord distance (L value), cervical cord angle (CCA), cervical lordosis, spine/cord (S/C) angle ratio, spinal cord (SC) area, cerebrospinal fluid (CSF) area, and CSF reserve ratio (CSF/CSF plus SC). Univariate multiple regression for Nurick grade as dependent factor was used. RESULTS: Sixty-three patients and 34 men, with the mean age of 58.2 ± 11 years, were analysed. Significant differences were found for pLCC, SC area, C lordosis and CCA. The difference between F and E for C lordosis angle was 42.80° ± 14.4 and for CC angle 30.42° ± 9.6. The mean S/C angle ratio was calculated for 1.4 ± 1.3. Nurick grade positively correlated with age (p = 0.041) and S/C angle ratio (p = 0.011), negatively with SC area (p = 0.006) and flexion-extension difference of L value (0.004). CONCLUSIONS: Severity of myelopathy correlates with age, spinal cord area on extension and reduced mobility of spinal cord. An association between spine/cervical cord angle mismatch and Nurick grade was found.

Do Intraoperative Neurophysiological Changes During Decompressive Surgery for Cervical Myeloradiculopathy Affect Functional Outcome? A Prospective Study.

STUDY DESIGN: Prospective cohort. OBJECTIVE: To investigate whether intraoperative neuromonitoring (IONM) positive changes affect functional outcome after surgical intervention for myeloradiculopathy secondary to cervical compressive pathology (cervical compressive myelopathy). METHODS: Twenty-eight patients who underwent cervical spine surgery with IONM for compressive myeloradiculopathy were enrolled. During surgery motor-evoked potential (MEP) and somatosensory evoked potential (SSEP) at baseline and before and after decompression were documented. A decrease in latency >10% or an increase in amplitude >50% was regarded as a "positive changes." Patients were divided into subgroups based on IONM changes: group A (those with positive changes) and group B (those with no change or deterioration). Nurick grade and modified Japanese Orthopaedic Association (mJOA) score were evaluated before and after surgery. RESULTS: Nine patients (32.1%) showed improvement in MEP. The mean preoperative Nurick grade and mJOA score of group A and B were (2.55 ± 0.83 and 11.11 ± 1.65) and (2.47 ± 0.7 and 11.32 ± 1.24), respectively. The mean postoperative Nurick grade of groups A and B at 6 months was 1.55 ± 0.74 and 1.63 ± 0.46, respectively, and this difference was not significant. The mean postoperative mJOA score of groups A and B at 6 months was 14.3 ± 1.03 and 12.9 ± 0.98, respectively, and this difference was statistically significant (P = .011). Spearman correlation coefficient showed significant positive correlation between the IONM change and the mJOA score at 6 months postoperatively (r = 0.47; P = .01). CONCLUSION: Our study shows that impact of positive changes in MEP during IONM reflect in functional improvement at 6 months postoperatively in cervical compressive myelopathy patients.

AIIMS Cervical Myelopathy Score - A New Comprehensive and Objective Patient-Reported Modification of mJOA: Results of an Internal Validation Study.

Background: Several scoring systems have been developed for assessment of patients with compressive cervical myelopathy. However, all of these have some shortcomings. We proposed a new modification of the modified Japanese Orthopedic Association (mJOA) score-the AIIMS cervical myelopathy score (ACMS). Objective: The aim of this study was to compare the ACMS with mJOA score and Nurick score. Methods: We prospectively studied patients with cervical compressive myelopathy. The new ACMS, mJOA, Nurick proposed by Benzel, and Nurick scores were recorded preoperatively and at three months postoperatively in patients. Results: Sixty-two patients completed the 3-month follow-up and were included in the final analysis. The mean preoperative and postoperative Nurick, mJOA, and ACMS scores were 3.3 and 3.0, 12.3 and 13.8, and 15.1 and 17.7, respectively. High correlation (Pearson's r > 0.8, 95% CI: 0.94 to 0.97, P < 0.005) was observed between ACMS and mJOA scores for all the individual components of both scales, both in pre- and postoperative assessments. A negative correlation was observed between the occupational ability scores ACMS and the Nurick scale (r = -0.76, 95% CI: -0.83 to - 0.68). No correlation was found with cord/canal ratio on magnetic resonance imaging (MRI) with any of the three scoring systems (preoperative, postoperative, or recovery rates). Conclusions: The ACMS score showed a good correlation with the mJOA score for evaluation of functional disability in the setting of cervical myelopathy. The patients could themselves report the scores using the ACMS scoring chart. The occupational component of the ACMS also correlated well with the Nurick score.

Functional Outcomes and Temporal Profile of Recovery in Patients with Intradural Extramedullary Spinal Cord Tumors with Poor Nurick Grade.

BACKGROUND: Patients with spinal intradural extramedullary (IDEM) tumors usually have a good functional outcome after tumor excision. However, the literature is sparse on the functional outcome in patients with poor Nurick grade (NG 4 and 5). METHODS: A retrospective review of 81 patients with IDEM tumors presenting with a poor NG was performed to determine postoperative functional outcome and the temporal pattern of recovery. The following risk factors were analyzed: preoperative NG, duration of symptoms, tumor location, peritumoral edema, presence of syrinx, and tumor type. RESULTS: Neurologic function started recovering soon after surgery, with >80% of the patients improving by ≥1 grade at the end of 1 week after surgery. Of the 66 patients available for follow-up of >6 months after surgery, 63 (95.5%) improved to NG 0-2 and 51 (77.2%) became asymptomatic (NG 0 or 1). Three patients had a poor functional outcome on follow-up of >31 months; 2 had improved from NG 5 to NG 4, whereas 1 patient continued to be in NG 4. Factors associated with a poor outcome were an upper thoracic location (P = 0.025) and presence of a syrinx (P = 0.004). None of the patients had bladder dysfunction at follow-up of >6 months. CONCLUSIONS: After excision of spinal IDEM tumors, in patients who present with a poor neurologic function (NG 4 and 5), good functional outcome (NG 0-2) can be expected in >95% of patients. No recovery can be anticipated beyond 1 year after surgery.

Clinicopathological Study of Intradural Extramedullary Spinal Tumors and Its Correlation With Functional Outcome.

Introduction Intradural extramedullary (IDEM) spinal cord tumors account for approximately two-thirds of largely benign intraspinal neoplasms. These are amenable to gross total excision and usually carry a good functional outcome. Methods In this study, we reviewed the surgical outcomes of 35 patients who underwent excision of intradural extramedullary tumors. Patient demographics, severity and duration of symptoms, and tumor characteristics (anatomical and pathological) in all operated spinal IDEM tumors were collected. The neurological findings obtained during the preoperative stage and the postoperative follow-up were evaluated according to the Frankel and Nurick grading. The back pain was assessed with help of the Denis pain scale (DPS). Results The histopathological outcomes of the study were as follows: six patients of neurofibroma, 12 cases of schwannoma, nine cases of meningiomas, three cases of ependymoma, one case of dorsal neurenteric cyst, two cases of epidermoid cyst, one case of cauda equina paraganglioma, and one case of filum terminale dermoid cyst. Paresthesia/numbness were the commonest symptoms (88.6%), weakness of limbs in (80%), sphincter dysfunction in 15 patients (42.9%), and paraplegia was seen in three patients (8.57%). The complications encountered were - one case each of cerebrospinal fluid (CSF) leak, surgical site infection, and pseudomeningocele. The percentage of spinal canal occupied ranged from 71-94%. The mean percentage of the spinal canal occupied by the tumor was 81.8%. In our series, 77.14% of patients (p<0.0001) had good functional outcomes as per improvement in Frankel score. The DPS and Nurick score mean values showed a significant decrease over the follow-up duration as compared to preoperative mean values. Significant functional improvement was noted at the one-week, one-month, and one-year follow-up, with a p-value of <0.0001. Conclusions The IDEM tumors are usually benign and are readily detected by contrast-enhanced MRI scans. These have excellent surgical outcomes with some exceptions. Greater canal occupancy and a longer duration of symptoms are usually seen to correspond with suboptimal functional outcomes.

Prevalence of anemia and its relationship with neurological status in patients undergoing surgery for degenerative cervical myelopathy and radiculopathy: A retrospective study of 2 spine centers.

Both degenerative cervical myelopathy (DCM) and anemia are common among older patients, however insufficient data exists evaluating their co-occurrence and the influence of anemia on baseline neurological status. To address this, we examined a retrospective multicenter series of patients treated for DCM or radiculopathy. Myelopathy was graded using the Nurick scale. Established criteria for diagnosing abnormalities were used to identify blood abnormalities, including macrocytic and microcytic anemia. Multivariable regression was used to determine the impact of hematological anomalies on Nurick grades. In our analysis, we included 725 patients (age of 57.1 ± 11.7), of whom 398 presented with myelopathy and 327 presented with radiculopathy alone. Twenty six percent of all patients were anemic at baseline and the mean preoperative Nurick grade across all patients was 2.09 ± 1.29; mean Nurick grade amongst those with DCM was 2.98 ± 1.12. Compared to those with myelopathy, patients with radiculopathy were significantly younger (53.8 ± 11.0 vs 59.8 ± 11.6, p < 0.001) and less likely to be anemic (16.8% vs 33.7%, p < 0.0001). Nurick grading was significantly higher in myelopathy patients with anemia (3.13 ± 1.19 vs 2.91 ± 1.07, p = 0.05) and macrocytic anemia (4.00 ± 1.41 vs 2.97 ± 1.11, p = 0.04). Multivariate regression demonstrated that anemia (p < 0.001), age (p < 0.0001), and posterior surgical approach (p < 0.0001) were related to worse preoperative Nurick grade. In sum, these data suggest that anemia and degenerative cervical spine pathologies commonly co-occur. Anemia, and macrocytic anemia specifically, is associated with poorer neurological status in myelopathic patients. These data suggest anemia may influence baseline neurological status and impact surgical recovery in patients treated for DCM or radiculopathy.

Role of Decompressive Laminectomy without Instrumentation in the Management of Nurick Grade 4 and 5 Cervical Compressive Myelopathy.

INTRODUCTION: Cervical laminectomy is a very well-known posterior decompressive procedure for cervical compressive myelopathy (CCM). Our objective is to evaluate the functional effect of posterior decompressive laminectomy for poor grade CCM. METHODS: This study was an observational retrospective study carried out on patients with poor-grade CCM who underwent decompressive laminectomy from January 2010 to December 2015. Patients with Nurick Grades 4 and 5 (walking with support or bedbound) were included in the study. Clinical data and radiological information were collected from medical records, and objective scales were applied to compare the surgical outcome between preoperative score and postoperative score. RESULTS: A total of 69 patients who underwent decompressive laminectomy for poor grade CCM were included. The mean age was 54.9 years, and the male-to-female ratio was 5.3:1. Ossified posterior longitudinal ligament comprised 52.6% cases. The follow-up data of at least 6 months' duration after surgery was available for 57 (82.6%) cases. On comparing with preoperative Nurick grade at follow-up, 40 of the 57 patients (70.2%) were found to have improvement following surgery by at least one grade. The remaining 17 (29.8%) had either remained the same or had deteriorated further. The mean preoperative modified Japanese Orthopedic Association score was 8.4 ± 2.8, and the mean follow-up score was 11.8±0.3 (P = 0.0001). On multivariate analysis, the number of levels of laminectomy, postoperative deterioration, and anesthesia grade were predictors of outcome. CONCLUSION: Decompressive laminectomy for poor grade myelopathy is effective in improving functional outcome.

Cervical Spine and Cord Angle Mismatch in the Pathogenesis of Myelopathy.

BACKGROUND: Cervical myelopathy is a complex pathology and dynamic compression of the tethered cervical cord, which may be responsible for clinical symptoms. METHODS: Patients with cervical canal stenosis who had magnetic resonance imaging in flexion and extension positions were retrospectively reviewed. All cases were evaluated in Nurick grade. The cervical parameters-cervical cord (CC) angle, cervical lordosis, and spine/cord (S/C) angle ratio-were measured on the magnetic resonance imaging. Mean values of these parameters were compared between nonmyelopathic (Nurick grade 0) and myelopathic groups (Nurick grades 1-5). A multinomial ordinal logistic regression was used to predict outcome for Nurick grade using the CC angle, the cervical lordosis angle, and the S/C angle ratio as independent variables. RESULTS: A total of 65 patients (35 men) with the mean age of 58.6 ± 11.4 years were analyzed. A comparison of means between Nurick grade 0 against each of myelopathic grades 1-5 revealed significant differences only for the S/C angle ratio. A cumulative comparison between nonmyelopathic and myelopathic grades for the S/C angle ratio showed significant difference of 0.29 (1.16 ± 0.5 vs. 1.45 ± 0.6, respectively; P < 0.05). Cumulative comparison for the CC angle difference in flexion and extension lordosis did not show substantial differences. The S/C angle ratio was the only significant parameter in the prediction of the Nurick grade with an odds ratio of 2.63 (95% confidence interval 2.11-2.79). CONCLUSIONS: A positive correlation between Nurick grade and cervical spine and cord angle mismatch was found.

Prognostic Value of Preoperative Nurick Grade and Time with Symptoms in Patients with Cervical Myelopathy and Gait Impairment.

OBJECTIVE: To investigate the prognostic value of preoperative Nurick grade and time with symptoms for gait improvement and recovery in patients with ataxia secondary to cervical myelopathy. METHODS: A retrospective chart review of all adult patients who underwent surgical decompression for cervical myelopathy between 1996 and 2013 was performed. Only adults with a Nurick grade of at least 2 or worse were included. Outcome measures included gait improvement and recovery. RESULTS: A total of 170 patients were identified. Gait improvement and gait recovery occurred in 57.7% and 45.9% of patients, respectively. Time to improvement occurred as early as 1 month up to 24 months postoperatively. A greater preoperative Nurick grade was associated with lower odds of gait improvement (odds ratio 0.74; 95% confidence interval 0.53-0.99, P = 0.048) and gait recovery (odds ratio 0.27; 95% confidence interval 0.17-0.43, P < 0.001). The proportion of patients with symptoms for 12 months or less that experienced gait improvement was 71.2%, compared with 36.4% for patients with symptoms for over 12 months (P < 0.001). Patients with symptoms for 12 months or less had a 59.6% gait recovery rate, compared to 24.2% in patients with symptoms for over 12 months (P < 0.001). Having symptoms for over 12 months was independently associated with lower odds of improvement and recovery. CONCLUSIONS: Patients with a greater preoperative Nurick grade and symptoms for more than 12 months may have significantly lower odds of experiencing gait improvement or gait recovery after surgery for cervical myelopathy. This study's conclusion favors early intervention in patients with cervical myelopathy.

Surgical outcome in patients with cervical ossified posterior longitudinal ligament: A single institutional experience.

OBJECTIVE: Ossification of the posterior longitudinal ligament (OPLL) is a complex multi-factorial disease process having both metabolic and biomechanical factors. The role of surgical intervention as well as the choice of approach weather anterior or posterior is ambiguous. The objective of this study was to assess the surgical out come and post operative functional improvement in patients with cervical OPLL at a tertiary care centre. PATIENTS AND METHODS: This prospective study included 63 patients of cervical OPLL who underwent either anterior and/or posterior surgeries in Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad between June 2009 to May 2011. Patient's data including age, sex, pre and post operative functional status, radiographic findings and OPLL subtypes were recorded and analyzed over a follow up ranging up to minimum two years. RESULTS: The mean age of the patients was 51.1 (range 30-80 years) involving 14 women and 49 men. Out of 63 patients, 14 patients underwent surgery by anterior approach (corpectomy and fusion) and all of them improved (P = 0.52). 49 patients underwent surgery by posterior approach where decompressive laminectomy was performed in 40, laminectomy with instrumentation was done in 5, laminoplasty was done in 3 and 1 patient underwent both anterior and posterior surgeries. Of those who underwent posterior surgery, 40 patients improved, 7 remained the same as their preoperative status (who were having signal intensity changes on T2W MRI) and 2 patients deteriorated in the immediate post operative period and then showed gradual improvement. All the patients were followed up for 24 months. The mean pre-operative Nurick grade was 2.82 which later on improved to 2.03 post surgery (P < 0.05). Minor complications included wound infections in two patients (1.26%). CONCLUSIONS: Anterior cervical decompression and reconstruction is a safe and appropriate treatment for cervical spondylitic myelopathy in the setting of single or two level OPLL. Laminectomy or laminoplasty is indicated in patients with preserved cervical lordosis having three or more levels of involvement. Younger patients with good pre operative functional status and less than 2 levels of involvement have better outcome following anterior surgery.