Unilateral interlaminar fenestration on the convex side provides a reliable access for intrathecal administration of nusinersen in spinal muscular atrophy: a retrospective study.

BACKGROUND: As the first gene therapy for spinal muscular atrophy (SMA), nusinersen is supposed to be administrated via intrathecal injection regularly for a lifetime. However, for SMA patients with severe spinal deformities, bony fusion following posterior spinal instrumentation sets great obstacles for the application of nusinersen. Therefore, efforts have been devoted to the exploration of appropriate approach for nusinersen administration. This study aims to evaluate the safety and reliability of unilateral interlaminar fenestration on the convex side during spinal fusion surgery for intrathecal nusinersen injection in SMA. RESULTS: SMA patients receiving posterior spinal fusion and interlaminar fenestration in Peking Union Medical College Hospital from January 2020 to October 2021 were retrospectively analyzed. 13 patients were included. Of the 13 patients, 10 were classified into SMA type II and 3 into SMA type III. Distal fusion to pelvis was undertaken in 11 patients; while L5 was selected as the lowest instrumented vertebra in the other 2 patients. All patients received interlaminar fenestration on the convex side only with an area of about 15 mm × 20 mm. Fenestration at L2-L3 level was performed in 6 patients; while L3-L4 level was selected for windowing in the remaining 7 patients. 9 of the 13 patients received lumbar puncture and intrathecal nusinersen administration during the 1-year follow-up, with an accumulative total of 50 times. All injections were performed successfully under ultrasound guidance, with no one transferred to radiographic assistance. No severe complications occurred after injection. CONCLUSIONS: In SMA with severe scoliosis planning to receive posterior spinal fusion, unilateral lumbar interlaminar fenestration on the convex side provides a feasible and reliable access for intrathecal nusinersen administration after surgery.

Influence of Paraspinal Growth-Friendly Spinal Implants in Children with Spinal Muscular Atrophy on Parasol Deformity, Rib-Vertebral Angles, Thoracic, and Lung Volumes.

INTRODUCTION: Children with spinal muscular atrophy (SMA) and progressive neuromuscular scoliosis often require early growth-friendly spinal implant (GFSI) treatment for deformity correction with implant fixation either through pedicle screws or bilateral to the spine using ribto pelvis fixation. It has been proposed that the latter fixation may change the collapsing parasol deformity via changes in the rib-vertebral angle (RVA) with a positive effect on thoracic and lung volume. The purpose of this study was to analyze the effect of paraspinal GFSI with bilateral rib-to-pelvis fixation on the parasol deformity, RVA, thoracic, and lung volumes. METHODS: SMA children with (n = 19) and without (n = 18) GFSI treatment were included. Last follow-up was before definite spinal fusion at puberty. Scoliosis and kyphosis angles, parasol deformity, and index, as well as convex and concave RVA, were measured on radiographs, whereas computed tomography images were used to reconstruct thoracic and lung volumes. RESULTS: In all SMA children (n = 37; with or without GFSI), convex RVA was smaller than concave values at all times. GFSI did not crucially influence the RVA over the 4.6-year follow-up period. Comparing age- and disease-matched adolescents with and without prior GFSI, no effect of GFSI treatment could be detected on either RVA, thoracic, or lung volumes. Parasol deformity progressed over time despite GFSI. CONCLUSION: Despite different expectations, implantation of GFSI with bilateral rib-to-pelvis fixation did not positively influence parasol deformity, RVA and/or thoracic, and lung volumes in SMA children with spinal deformity directly and over time.

Scoliosis Treatment With Growth-Friendly Spinal Implants (GFSI) Relates to Low Bone Mineral Mass in Children With Spinal Muscular Atrophy.

BACKGROUND: Children with spinal muscular atrophy (SMA) frequently develop neuromuscular scoliosis at an early age, requiring surgical treatment with growth-friendly spinal implants (GFSI), such as magnetically controlled growing rods. This study investigated the effect of GFSI on the volumetric bone mineral density (vBMD) of the spine in SMA children. METHODS: Seventeen children (age 13.2±1.2 y) with SMA and GFSI-treated spinal deformity were compared with 25 scoliotic SMA children (age 12.9±1.7 y) without prior surgical treatment as well as age-matched healthy controls (n=29; age 13.3±2.0). Clinical, radiologic, and demographic data were analyzed. For the calculation of the vBMD Z-scores of the thoracic and lumbar vertebrae, phantom precalibrated spinal computed tomography scans were analyzed using quantitative computed tomography (QCT). RESULTS: Average vBMD was lower in SMA patients with GFSI (82.1±8.4 mg/cm 3) compared with those without prior treatment (108.0±6.8 mg/cm 3 ). The difference was more prominent in and around the thoracolumbar region. The vBMD of all SMA patients was significantly lower in comparison with healthy controls, especially in SMA patients with previous fragility fractures. CONCLUSIONS: The results of this study support the hypothesis of reduced vertebral bone mineral mass in SMA children with scoliosis at the end of GFSI treatment in comparison with SMA patients undergoing primary spinal fusion. Improving vBMD through pharmaceutical therapy in SMA patients could have a beneficial effect on the surgical outcome of scoliosis correction while reducing complications. LEVEL OF EVIDENCE: Therapeutic Level III.

Ultrasound-guided interlaminar approach for nusinersen administration in patients with spinal muscular atrophy with spinal fusion or severe scoliosis.

BACKGROUND: Intrathecal injection of medications can be challenging in spinal muscular atrophy (SMA) patients with severe scoliosis or after spine surgery. Here we report our experience with real-time ultrasound (US)-guided intrathecal administration of nusinersen in patients with SMA. METHODS: Seven patients (six children and one adult) with either spinal fusion or severe scoliosis were enrolled. We performed intrathecal injections of nusinersen under US guidance. The efficacy and safety of US-guided injection were explored. RESULTS: Five patients had undergone spinal fusion, while the other two presented severe scoliosis. Success was achieved in 19/20 lumbar punctures (95%), 15 of which were performed through the near-spinous process approach. The intervertebral space with a designated channel was selected for the five postoperative patients, while the interspaces with the smallest rotation angle were chosen for the other two patients with severe scoliosis. In 89.5% (17/19) of the punctures, the number of insertions was no more than two. No major adverse events were observed. CONCLUSION: Given its safety and efficacy, real-time US guidance is recommended for SMA patients with spine surgery or severe scoliosis, and the near-spinous process view can be used as a interlaminar puncture approach for US guidance.

Surgical treatment of spinal deformities in spinal muscular atrophy: a single-center experience from China.

PURPOSE: To report the clinical characteristics and surgical outcomes of scoliosis in patients with spinal muscular atrophy (SMA) from Mainland China. METHODS: Nineteen patients were retrospectively analyzed. Demographic, anthropometric and respiratory parameters were collected preoperatively. Surgical program was analyzed. Radiographic data were measured perioperatively. Motor status, ventilation support, sitting ability and respiratory symptoms were evaluated preoperatively and at final follow-up. RESULTS: Age at surgery was 17.08 (12.83, 20.08) years. More than 40% of patients were diagnosed with low weight. Pulmonary dysfunction was observed in all patients. All patients received posterior spinal fusion (PSF). Sacroiliac fixation with sacral-2 alar iliac technique was used in 16 patients. Major curve correction rate was 54.87 ± 16.14%. Pelvic obliquity correction rate was 63.84 ± 23.70%. T1-T12 height, space-available-for-lung ratio and thoracic transverse diameter were increased (p < 0.001). Percentage of patients capable of sitting independently increased from 26.32% preoperatively to 73.68% at final follow-up. Cumulative scores of sitting-related items in muscular dystrophy spine questionnaire improved from 19.11 ± 5.40 preoperatively to 26.21 ± 5.20 at final follow-up. Total scores of symptomatic domains in St. George's Respiratory Questionnaire decreased from 4 (2, 12) preoperatively to 1 (0, 3) at final follow-up. CONCLUSIONS: SMA patients in China always present severe scoliosis at late adolescence, accompanied with high proportion of low weight and pulmonary dysfunction. PSF is effective for the correction of scoliosis and pelvic obliquity and the improvement of thoracic morphology. Sitting ability and respiratory symptoms were improved postoperatively.

The effect of scoliosis surgery on pulmonary function in spinal muscular atrophy patients: review of the literature and a meta-analysis.

PURPOSE: To determine the effect of surgical instrumentation on pulmonary function after surgery. METHODS: A literature review was performed to identify articles reporting on the effect of scoliosis surgery on the pulmonary function in patients with spinal muscular atrophy (SMA). Data for each patient were extracted from included studies, and a meta-analysis was performed using the values of forced vital capacity (FVC) before and after surgery. RESULTS: A total of 127 articles were reviewed, and ten articles were selected for data extraction according to inclusion criteria. The results of the meta-analysis showed no difference in respiratory function pre-operatively and at last follow-up. Four out of ten studies demonstrated similar results and five studies reported a decrease in pulmonary function after surgery. Only one study showed improvement in lung function. CONCLUSION: At the moment, there is insufficient evidence in the literature to support that spinal surgery can improve respiratory function. According to our meta-analysis study, vital capacity remains either unchanged or the rate of deterioration is decreased after surgery. Nevertheless, these are both considered favorable outcomes taking into account the natural course of the disease with progressive deterioration of pulmonary function over time.

Children with Spinal Muscular Atrophy Have Reduced Vertebral Body Height and Depth and Pedicle Size in Comparison to Age-Matched Healthy Controls.

BACKGROUND: Most children with spinal muscular atrophy (SMA) develop spinal deformity, which may require surgical intervention. In addition to poor bone stock, vertebral body shape may hinder the placement of spinal implants resulting in complications and poor outcome. The aim of this study was to analyze whether vertebral body morphology of children and adolescents with SMA is altered in comparison to healthy age-matched controls. METHODS: In this prospective cohort study, 17 children with SMA (mean age 8.7 ±1.0 years) and 13 adolescents with SMA (mean age 13.6 ±1.4 years), all with some degree of neuromuscular scoliosis, were analyzed by standardized radiographic measurements to evaluate vertebral body height and depth. Results were compared with age-matched healthy controls (n = 10 children; mean age 9.1 ± 1.6 years; n = 20 adolescents, mean age 13.1 ± 0.5 years). Computed tomography scans of 27 adolescents with SMA (13.5 ±1.2 years) and 25 healthy age-matched controls (13.8 ±2.0 years) were analyzed to define pedicle diameters. RESULTS: All children and adolescents with SMA had decreased vertebral height and depth in comparison to age-matched healthy controls. In adolescents, reduced depth was more pronounced than height in the thoracic spine. Pedicle size was significantly reduced in the lower thoracic and lumbar area. CONCLUSIONS: Reduced vertebral body height and depth and pedicle size in children and adolescents with SMA may influence surgical treatment of spinal deformity. Surgeons should be aware of anatomical differences and choose implant devices accordingly.

Protocol-driven early tracheal extubation in patients with flaccid neuromuscular scoliosis and pre-existing lung disease.

PURPOSE: To review the results of a postoperative respiratory pathway for patients with muscular dystrophy (MD) and spinal muscular atrophy (SMA) undergoing spinal surgery. METHODS: With IRB approval, a retrospective review was done on all patients with SMA and MD undergoing spinal surgery on a neuromuscular protocol. Baseline demographics, perioperative results, and long-term outcomes were collected. Per the protocol, patients remained intubated after surgery and were transported to the intensive care unit (ICU) for extubation. We present the results of protocol implementation and compare patients with MD to those with SMA. RESULTS: Twenty-four patients were treated using the protocol. Average age was 13.1 years. Severe restrictive lung disease was present in 75% of patients. Nocturnal BiPAP was required in 68% of patients. Average number of instrumented levels was 17. All patients were immediately extubated upon entering the ICU. There were three respiratory complications and only was patient was re-intubated. Average ICU stay was 1.8 days and average hospital length of stay was 6.7 days. No differences in postoperative inspiratory or expiratory positive airway pressures were observed between the MD and SMA groups. CONCLUSION: Through a multidisciplinary neuromuscular protocol, excellent clinical outcomes were achieved in patients with neuromuscular scoliosis and restrictive lung disease, with complication rates and length of stay significantly lower than previously published data. LEVEL OF EVIDENCE: IV.

Anaesthetic considerations in posterior instrumentation of scoliosis due to spinal muscular atrophy: Case series of 56 operated patients.

BACKGROUND: Spinal muscular atrophy (SMA) is a rare illness that often leads to severe kyphoscoliosis. This case series adds to the heretofore sparse information as regards the anaesthetic management of SMA scoliosis patients. METHODS: This retrospective study reviewed the charts of 79 SMA patients (type II n = 34 and type III n = 45) presenting for possible scoliosis surgery during the time period 2007-2019. Special attention focused on preoperative assessment and clearance requirements, anaesthesia protocol and postoperative handling. RESULTS: Out of 79 patients, 17 did not receive clearance for the procedure mostly due to grave respiratory insufficiency. Out of 62 patients with clearance for both surgery and anaesthesia, 56 patients [44 females, 12 males; age mean ± SD (range) 22 ± 7.3 (10-40) years] underwent the procedure. Their forced vital capacity and forced expiratory volume in 1 s were mean ± SD (range) 1.41 ± 0.53 (0.61-2.65) L and 1.26 ± 0.47 (0.52-2.27) L, respectively. Intubation difficulties and their resolution, e.g. with the help of fibreoptic technique and video laryngoscopy, are described. All 56 patients were extubated in the operating room postoperativley. Patients stayed at the postanaesthesia care unit for one (n = 48) or two (n = 8) nights. A considerable amount of the patients (19/56) developed hypokalaemia postoperatively. CONCLUSION: This analysis is one of the bigger series of its kind and adds insight into the preoperative clearance process, the anaesthetic protocol and some of the postoperative complications, e.g. the tendency for developing postoperative hypokalaemia which has not been reported previously.

Hybrid versus total sublaminar wires in patients with spinal muscular atrophy undergoing scoliosis surgery.

BACKGROUND: Early versions of spinal muscular atrophy (SMA) scoliosis correction surgery often involved sublaminar devices. Recently, the utilization of pedicle screws has gained much popularity. Pedicle screws are generally believed to provide additional deformity correction, but pedicle size and rotational deformity limit their application in the thoracic spine, resulting in a hybrid construct involving pedicle screws and sublaminar wire. Studies of the efficacy of hybrid instrumentation in SMA scoliosis are often limited by the scarcity of the disease itself. In this study, we aimed to compare the surgical outcomes between hybrid constructs involving pedicle screws and sublaminar wire and sublaminar wire alone in patients with SMA scoliosis. METHODS: We retrospectively reviewed the clinical records and radiographic assessments of patients with SMA scoliosis who underwent corrective surgery between 1993 and 2017. The radiographic assessments included deformity correction and progressive changes in the major curve angle, pelvic tilt (PT) and coronal balance (CB). The correction of deformities was observed postoperatively and at the patient's 2-year follow-up to test the efficacy of each type of construct. RESULTS: Thirty-three patients were included in this study. There were 14 and 19 patients in the wiring and hybrid construct groups, respectively. The hybrid construct group demonstrated a higher major curve angle correction (50.5° ± 11.2° vs. 36.4° ± 8.4°, p < 0.001), a higher apical vertebral rotation correction (10.6° ± 3.9° vs. 4.8° ± 2.6°, p < 0.001), and a reduced progression of the major curve angle at the 2-year follow-up (5.1° ± 2.9° vs. 8.7° ± 4.8°, p < 0.001). A moderate correlation was observed between the magnitude of correction of the apical vertebral rotation angle and the major curve (r = 0.528, p = 0.002). CONCLUSION: This study demonstrated that hybrid instrumentation can provide a greater magnitude of correction in major curve and apical rotation as well as less major curve progression than sublaminar wire instrumentation alone in patients with SMA scoliosis. Level of evidence III.

Minimally Invasive Fusionless Surgery for Scoliosis in Spinal Muscular Atrophy: Long-term Follow-up Results in a Series of 59 Patients.

BACKGROUND: Treatment of spinal muscular atrophy (SMA) scoliosis has evolved in the last decade, with the emergence of fusionless surgical techniques that allow correction of the deformity before the end of growth spurt. These techniques are expected to delay definitive spine fusion and preserve trunk growth. PURPOSE: The aim was to evaluate long-term clinical, radiologic, and respiratory outcomes of a minimally invasive fusionless surgery (MIFLS) in SMA scoliosis. METHODS: All children affected with SMA scoliosis who underwent MIFLS in our department from 2011 to 2019 were included. The instrumentation consisted in a bilateral sliding rod construct from T1 to the sacrum, anchored proximally by double-hook claws and distally by iliosacral screws. Clinical, genetic, respiratory and radiographic data were retrospectively reviewed. A patient's satisfaction survey was performed. RESULTS: A total of 59 children with genetic confirmation of SMA (9SMA1c, 47SMA2, and 3SMA3) underwent MIFLS at a mean age of 11±1.9 years. All of them were nonwalker at the time of surgery. Twenty-six were treated with intrathecal Nusinersen. Mean follow-up was 5.2 years (2 to 9.6 y). Mean major coronal curve improved from 79±15 to 41±16 degrees and pelvic obliquity decreased from 24±11 to 5.9±4 degrees. Mean space available for lung improved from 77% to 93%. Mechanical or infectious complications occurred in 9 patients, with removal of the implant in 1. 6 children required unplanned surgeries. Postoperative bracing was needed in 13 children. Mean gain weight 3 years after the first surgery was 6 kg. 91.5% of patients had a positive satisfaction of the surgery. There was no significant impact in respiratory function postoperatively. Only 30 children required rod lengthening procedures, with a mean interval between procedures of 1.9 years (0.5 to 3.7 y). No arthrodesis was required at last follow-up in any patient. CONCLUSION: Bipolar MIFLS in SMA preserves spinal and thoracic growth without interference with respiratory function. It provides a significant correction of spinal deformity and pelvic obliquity, having a reduced rate of complications. The correction of spinal deformity was maintained at long term, not requiring definitive fusion at the end of growth. LEVEL OF EVIDENCE: Level IV.

Evolving Surgical Management for Early-Onset Scoliosis in Spinal Muscular Atrophy Type 1 Given Improvements in Survival.

CASE: We present a case of an 18-month-old child with early-onset scoliosis in the setting of spinal muscular atrophy (SMA) type 1 whose rapidly progressive scoliosis is successfully managed with magnetic growing rods, the youngest age of implantation in a patient with SMA we are currently aware of. Technical challenges, complications, and outcome are described in this case presentation. CONCLUSION: Patients with SMA type 1 and early-onset scoliosis can be managed with growing-rod constructs given dramatic improvements in medical care that have expanded life expectancy.

Transforaminal intrathecal delivery of nusinersen for older children and adults with spinal muscular atrophy and complex spinal anatomy: an analysis of 200 consecutive injections.

BACKGROUND: Nusinersen is the only approved treatment for all spinal muscular atrophy (SMA) subtypes and is delivered intrathecally. Distorted spinal anatomy and instrumentation preclude standard approaches for intrathecal access, necessitating alternative techniques for delivery. The purpose of this study is to report technical success and adverse events of transforaminal intrathecal delivery of nusinersen. METHODS: 28 patients, mean age 24.1±9.8 years (range 10.0-51.0 years), with intermediate or late onset SMA, underwent a combined 200 transforaminal nusinersen injections. All patients had osseous fusion or spinal instrumentation precluding standard posterior access routes. Patients who underwent nusinersen injections using a technique other than transforaminal lumbar puncture (n=113) were excluded. Technical success, adverse events (AEs) and radiation exposure were recorded. RESULTS: 200 (100%) procedures were technically successful; 6 (3%) required a second level of attempt for access. 187 (93.5%) interventions were completed using cone beam computed tomography (CBCT) with two-axis fluoroscopic navigational overlay. 13 (6.5%) procedures were performed with fluoroscopic-guidance only at subsequent sessions. There were 8 (4.0%) mild AEs and 2 (0.5%) severe AEs; one patient received antibiotics for possible traversal of the large bowel but did not develop meningitis, and one patient developed aseptic meningitis. Mean air kerma was 74.5±161.3 mGy (range 5.2-1693.0 mGy). CONCLUSION: Transforaminal intrathecal delivery of nusinersen is feasible and safe for gaining access in patients with distorted spinal anatomy. The use of CBCT delineates anatomy and optimizes needle trajectory during the initial encounter, and may be used selectively for subsequent procedures.

Correlation of Pulmonary Function to Novel Radiographic Parameters of Collapsing Parasol Deformity in Spinal Muscular Atrophy.

Spinal muscular atrophy (SMA) is a neuromuscular disease with manifestations of scoliosis, pulmonary function decline, and, uniquely, collapse of the ribs. Methods to quantify rib deformity and its impact on pulmonary function are sparse. The authors propose new radiographic measurements to quantify the aspect of SMA known as collapsing parasol deformity and correlate these measurements with pulmonary function. Twenty-eight full-spine radiographs of pediatric SMA patients were measured twice by 3 independent investigators, with 2 weeks separating each measurement. Radiographic measurements, demographics, spirometry results, and assisted ventilation rating were obtained. Twenty-one patients with spirometry metrics were assessed to correlate pulmonary function and spinal measurements. The intrarater intraclass correlation coefficient (ICC) for the measurements ranged from 0.706 to 0.99, and the interrater ICC ranged from 0.64 to 0.97. Eighteen of 19 variables had ICC values greater than 0.75 for inter- and intrarater reliability. Twenty-one patients with forced expiratory volume in 1 second and forced vital capacity were assessed in terms of these measurements. Ratio of the concave hemithoracic width at T6/convex hemithoracic width at T6 (P=.004) and ratio of convex vertical rib displacement at the apical rib/concave vertical rib displacement (P=.021) were both significantly correlated with decreased pulmonary function. No significant correlation was found examining the average vertical rib displacement at the apical rib. High inter-and intrarater reliability can be obtained in a variety of spinal measurements of SMA patients. Various measurements are correlated to diminished pulmonary function, specifically variables showing asymmetric changes in the chest cavity. [Orthopedics. 2021;44(2):e287-e293.].

Continuous lengthening potential after four years of magnetically controlled spinal deformity correction in children with spinal muscular atrophy.

Magnetically controlled growing rods (MCGR) are commonly implanted for the treatment of early-onset scoliosis. While most authors report favorable short-term results, little is known about long-term deformity correction. This prospective cohort study assesses spinal deformity control in a homogeneous spinal muscular atrophy (SMA) patient group treated with MCGR implants, a standardized lengthening protocol and a minimum follow-up of four years. 17 SMA patients with progressive scoliosis were treated with MCGR implanted parallel to the spine with rib-to-pelvis fixation. Radiologic measurements were performed before and after MCGR implantation and during external lengthening procedures. These included measurements of the scoliotic curve, kyphosis, lordosis, pelvic obliquity and the spinal length. Additional clinical data of the complications were also analyzed. 17 children (mean age 7.4 years) were surgically treated and underwent a total of 376 lengthenings. Complication rates were 3.5% in respect to all interventions or 41% of the patients had complications during 3.5% of the lengthening sessions. The initial implantation significantly reduced the main scoliotic curve by 59%, with the correction remaining constant throughout the follow-up. Pelvic obliquity was also significantly and permanently corrected by 72%, whereas kyphosis and lordosis were not influenced. The spinal length could be significantly increased mostly during the first year of treatment. Bilateral implantation of MCGRs for correction of spinal deformity in children with SMA showed no decrease of the lengthening potential during a four-year follow-up. Therefore, the previously described 'law of diminishing returns' could not be applied to this patient population.Level of Evidence/Clinical relevance: Therapeutic Level IV.

Scoliosis in spinal muscular atrophy: is the preoperative magnetic resonance imaging necessary?

PURPOSE: To determine the prevalence of intraspinal alterations in scoliosis due to Spinal Muscular Atrophy (SMA). METHODS: Cross-sectional, observational, descriptive study. Fifty-six patients with SMA diagnosis required surgical treatment due to scoliosis. INCLUSION CRITERIA: scoliosis/kyphoscoliosis > 50 degrees in the coronal plane, clinical characteristics of Spinal Muscular Atrophy, accurate diagnosis by means of molecular or genetic study. Prior to the spinal surgery, and to find related intraspinal alterations, MRI of the spine and posterior cranial fossa was performed. RESULTS: Forty females, 16 males, mean age 11 years (range 6-14 years). 94% of the patients had Spinal Muscular Atrophy type 2. The mean angle value was 81 degrees (range 53-122 degrees) in the coronal plane and 62 degrees (range 35-80 degrees) in the sagittal plane. The prevalence of intraspinal alterations was 1.78%. One patient with cervical hydromyelia and no neurological surgical procedure prior to the spinal deformity surgery was reported. CONCLUSIONS: In the context of preoperative planning and strategy of patients with scoliosis due to Spinal Muscular Atrophy, MRI may have not to be requested.

Evaluation of Anterior Decompression Surgical Outcomes of Proximal-Type Cervical Spondylotic Amyotrophy: A Retrospective Study.

OBJECTIVE: To investigate the efficiency of anterior decompression on the proximal-type cervical spondylotic amyotrophy patients. METHODS: This was a retrospective analysis. From January 2014 to November 2017, 21 patients with proximal-type cervical spondylotic amyotrophy (CSA) underwent anterior decompression. There were 15 males and 6 females, aged 35-73 years with an average of 51.62 years. All the patients underwent surgery of anterior decompression (ACDF or ACCF). Among them, 12 patients underwent C4/5 single level ACDF, eight patients underwent C4/5 and C5/6 double level ACDF, and one patient underwent C5 anterior cervical corpectomy decompression and fusion surgery. Preoperative and postoperative clinical and radiologic parameters were assessed. The clinical examinations were reviewed, including muscle strength, neck disability index (NDI) score, cervical Japanese Orthopaedic Association (JOA) score, and improvement rate of manual muscle test (MMT) at the last follow-up. Preoperative spinal cord or nerve impingement was assessed by magnetic resonance imaging (MRI) or computed tomography (CT) myelography. Postoperative lateral X-ray radiographs were performed every 3 months after the surgery. RESULTS: Severe preoperative muscle atrophy of the deltoid or biceps muscles occurred in 21 patients included in the study. All of them involve impingements of the ventral nerve root and/or the anterior horn according to MRI and CT myelography. The preoperative duration of symptoms averaged 8.4 months. The average follow-up for all patients was 13.2 months. At the final follow-up, all patients showed statistically significant improvements in muscle strength and NDI scores (P < 0.05, P < 0.05). For the deltoid muscles force and C-JOA scores, the average improvement rates were 66.49% ± 10.04% and 62.23% ± 9.23%, respectively. With respect to MMT, 12 proximal-type patients were graded excellent, six were good, and three were fair, and the overall improvement rate was 85.7%. CONCLUSIONS: For proximal-type CSA patients with cervical radiculopathy, earlier anterior decompression surgery can achieve satisfactory results by significantly improving a patient's muscle strength and relieving compression symptoms.

Skip constructs in spinal muscular atrophy: outcomes of a novel approach for posterior spinal instrumentation and fusion.

PURPOSE: Many children with spinal muscular atrophy (SMA) develop progressive spinal deformity, worsening already compromised pulmonary function and global spinal balance. Early results demonstrate that intrathecal administration of nusinersen, a recent FDA-approved drug, improves motor function and ventilator-free survival, necessitating preservation of intrathecal access when considering PSIF. The purpose of this study is to assess medium-term outcomes of a specialized approach for posterior spinal instrumentation and fusion (PSIF) to preserve intrathecal access in patients with SMA. METHODS: A retrospective review of patients with SMA undergoing PSIF at a single tertiary academic medical center during a 3-year period was completed. To facilitate intrathecal drug administration, the traditional approach to PSIF was modified to "skip" one or more intervertebral levels at the thoracolumbar junction. Clinical notes and radiographs were reviewed for postoperative outcomes including major coronal curve correction and complications, including loss of correction, hardware failure and surgical revision. RESULTS: Eight patients were identified, with a mean age of 12.7 ± 1.6 years and follow-up of 4 years. These patients had a mean preoperative major coronal curve of 56.4°, with mean curve correction of 35.2°. At follow-up, no patients experienced rod breakage, loss of correction, or postoperative chronic pain. Only one patient required revision surgery due to bony overgrowth at the skipped level after three and a half years. CONCLUSION: Implementing the skip construct approach for PSIF in patients with SMA allows for scoliosis correction without compromising intrathecal drug delivery. Follow-up at 4 years reveals no adverse clinical events, hardware failure or loss of correction. LEVEL OF EVIDENCE: IV.

Is prophylactic formal fusion with implant revision necessary in non-ambulatory children with spinal muscular atrophy and growing rods who are no longer lengthened?

STUDY DESIGN: Single center, retrospective chart review. OBJECTIVES: To determine if routine posterior spinal fusion (PSF) is unnecessary in non-ambulatory growing rod graduates with SMA. Most non-ambulatory children with SMA develop early-onset scoliosis (EOS). Posterior growing rods (GR) have been shown safe and effective in managing spinal deformities in these children. The best management of these children, once graduated from their GR, is currently unknown. In this study, we report the clinical results of managing these children without routine definitive fusion following a course of GR treatment. METHODS: A single-center, retrospective chart and radiographic review was performed on children with SMA treated with posterior distraction GR, with a two-year minimum follow-up since final lengthening. Electronic medical records and radiographs were reviewed for demographic variables, Cobb measurements, implant revisions, occult radiographic implant failure, symptomatic failure, and/or conversion to PSF. RESULT: 12 patients (2 type 1, 9 type 2, 1 type 1/2) met inclusion criteria. Mean age at growing rod insertion was 6.2 years of age (range 4.1-8.2) and age at final lengthening 10.3 years of age (range 9.3-11.9). The mean time between last lengthening and latest clinical or radiographic review was 5.5 (range 2.1-9.0) years. Average mean pre, post, final Cobb angles were 71°, 27° (p < 0.001), 25°. Following final lengthening, only one patient required hardware revision and conversion to definitive fusion in attempts to alleviate chronic hip pain, which was unsuccessful. One additional patient was found to have an occult rod failure that has not required treatment. CONCLUSION: While limited by sample size, this single-center cohort of non-ambulatory SMA patients with EOS treated with similar constructs suggests that routine, definitive fusion in SMA GR graduates may be unnecessary. LEVEL OF EVIDENCE: Level IV.