The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients’ quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction

Introduction and Objective. Dislocation of a hip hemiarthroplasty is a significant complication with a high mortality rate in elderly patients. Previous studies have shown a higher risk of dislocation in patients with neuromuscular conditions. In this study, we reviewed our larger cohort of patients to identify if there is a link between neuromuscular disorders and dislocation of hip hemiarthroplasty in patients with neuromuscular conditions. Materials and Methods. We have retrospectively analysed a single-centre data that was collected over 34 years for patients with intracapsular neck of femur fracture who underwent hip hemiarthroplasty. The study population was composed of four groups: patients with no neuromuscular disorders, patients with Parkinson's disease, patients with previous stroke, and patients with mental impairment. Results. A total of 3827 patients were included in the analysis. 3371 patients had no neuromuscular condition (Group I) with a dislocation rate of 1.1%. 219 patients had Parkinsonism (Group II) with a dislocation rate of 3.2%, 104 patients had a previous stroke with weakness on the fracture side with a dislocation rate of 1.0% (Group III), and 984 patients had severe mental impairment with a dislocation rate of 1.8% (Group IV). The increased dislocation rate for those with Parkinson's disease was statistically significant (p=0.02) while none of the other neuromuscular conditions were statistically significant. Conclusions. Our study has shown an increased risk of dislocation of hemiarthroplasty in patients with Parkinson's disease in comparison to other groups. No increase was apparent for patients with mental impairment or weakness from a previous stroke

Abstract. Background. Dislocation of a hip hemiarthroplasty is a devastating complication with a high mortality rate in elderly patients. Previous studies have suggested a higher dislocation rate in patients with neuromuscular conditions. In this study, we have reviewed our larger cohort of patients to identify whether there is any association between neuromuscular disorders and prosthetic dislocation in patients treated with hip hemiarthroplasty for femoral neck fractures. Patients and Methods. Our study is a retrospective analysis of data collected over 34 years for patients with intracapsular neck of femur fracture who underwent hip hemiarthroplasty. The study population is composed of four groups: patients with no neuromuscular disorders, patients with Parkinson's disease, patients with previous stroke, and patients with dementia. Results. A total of 3827 patients were treated with hip hemiarthroplasty. For the 3371 patients with no neuromuscular condition (Group I) the dislocation rate was 1.1%. 219 patients had Parkinsonism (Group II) with a dislocation rate of 3.2%, 104 patients had a previous stroke with weakness on the fracture side with a dislocation rate of 1.0% (Group III), and 984 patients had severe dementia with a dislocation rate of 1.8% (Group IV). The increased dislocation rate for those with Parkinson's disease was statistically significant (p=0.02) while none of the other neuromuscular conditions were statistically significant. Conclusion. Our study demonstrates an increased risk of dislocation after hemiarthroplasty for patients with Parkinson's disease in comparison to other groups. No increase was apparent for those with dementia or weakness from a previous stroke

Neuromuscular scoliosis patients face rates of major complications of up to 49%. Along with pre-operative risk reduction strategies (including nutritional and bone health optimization), intra-operative strategies to decrease blood loss and decrease surgical time may help mitigate these risks. A major contributor to blood loss and surgical time is the insertion of instrumentation which is challenging in neuromuscular patient given their abnormal vertebral and pelvic anatomy. Standard pre-operative radiographs provide minimal information regarding pedicle diameter, length, blocks to pedicle entry (e.g. iliac crest overhang), or iliac crest orientation. To minimize blood loss and surgical time, we developed an “ultra-low dose” CT protocol without sedation for neuromuscular patients. Our prospective quality improvement study aimed to determine: if ultra-low dose CT without sedation was feasible given the movement disorders in this population; what the radiation exposure was compared to standard pre-operative imaging; whether the images allowed accurate assessment of the anatomy and intra-operative navigation given the ultra-low dose and potential movement during the scan. Fifteen non-ambulatory surgical patients with neuromuscular scoliosis received the standard spine XR and an ultra-low dose CT scan. Charts were reviewed for etiology of neuromuscular scoliosis and medical co-morbidities. The CT protocol was a high-speed, high-pitch, tube-current modulated acquisition at a fixed tube voltage. Adaptive statistical iterative reconstruction was applied to soft-tissue and bone kernels to mitigate noise. Radiation dose was quantified using reported dose indices (computed tomography dose index (CTDIvol) and dose-length product (DLP)) and effective dose (E), calculated through Monte-Carlo simulation. Statistical analysis was completed using a paired student's T-test (α = 0.05). CT image quality was assessed for its use in preoperative planning and intraoperative navigation using 7D Surgical System Spine Module (7D Surgical, Toronto, Canada). Eight males and seven females were included in the study. Their average age (14±2 years old), preoperative Cobb angle (95±21 degrees), and kyphosis (60±18 degrees) were recorded. One patient was unable to undergo the ultra-low dose CT protocol without sedation due to a co-diagnosis of severe autism. The average XR radiation dose was 0.5±0.3 mSv. Variability in radiographic dose was due to a wide range in patient size, positioning (supine, sitting), number of views, imaging technique and body habitus. Associated CT radiation metrics were CTDIvol = 0.46±0.14 mGy, DLP = 26.2±8.1 mGy.cm and E = 0.6±0.2 mSv. CT radiation variability was due to body habitus and arm orientation. The radiation dose differences between radiographic and CT imaging were not statistically significant. All CT scans had adequate quality for preoperative assessment of pedicle diameter and orientation, obstacles impeding pedicle entry, S2-Alar screw orientation, and intra-operative navigation. “Ultra-low dose” CT scans without sedation were feasible in paediatric patients with neuromuscular scoliosis. The effective dose was similar between the standard preoperative spinal XR and “ultra-low dose” CT scans. The “ultra-low dose” CT scan allowed accurate assessment of the anatomy, aided in pre-operative planning, and allowed intra-operative navigation despite the movement disorders in this patient population

Neuromuscular scoliosis patients face rates of major complications of up to 49%. Along with pre-operative risk reduction strategies (including nutritional and bone health optimization), intra-operative strategies to decrease blood loss and decrease surgical time may help mitigate these risks. A major contributor to blood loss and surgical time is the insertion of instrumentation which is challenging in neuromuscular patient given their abnormal vertebral and pelvic anatomy. Standard pre-operative radiographs provide minimal information regarding pedicle diameter, length, blocks to pedicle entry (e.g. iliac crest overhang), or iliac crest orientation. To minimize blood loss and surgical time, we developed an “ultra-low dose” CT protocol without sedation for neuromuscular patients. Our prospective quality improvement study aimed to determine:. if ultra-low dose CT without sedation was feasible given the movement disorders in this population;. what the radiation exposure was compared to standard pre-operative imaging;. whether the images allowed accurate assessment of the anatomy and intra-operative navigation given the ultra-low dose and potential movement during the scan. Fifteen non-ambulatory surgical patients with neuromuscular scoliosis received the standard spine XR and an ultra-low dose CT scan. Charts were reviewed for etiology of neuromuscular scoliosis and medical co-morbidities. The CT protocol was a high-speed, high-pitch, tube-current modulated acquisition at a fixed tube voltage. Adaptive statistical iterative reconstruction was applied to soft-tissue and bone kernels to mitigate noise. Radiation dose was quantified using reported dose indices (computed tomography dose index (CTDIvol) and dose-length product (DLP)) and effective dose (E), calculated through Monte-Carlo simulation. Statistical analysis was completed using a paired student's T-test (α= 0.05). CT image quality was assessed for its use in preoperative planning and intraoperative navigation using 7D Surgical System Spine Module (7D Surgical, Toronto, Canada). Eight males and seven females were included in the study. Their average age (14±2 years old), preoperative Cobb angle (95±21 degrees), and kyphosis (60±18 degrees) were recorded. One patient was unable to undergo the ultra-low dose CT protocol without sedation due to a co-diagnosis of severe autism. The average XR radiation dose was 0.5±0.3 mSv. Variability in radiographic dose was due to a wide range in patient size, positioning (supine, sitting), number of views, imaging technique and body habitus. Associated CT radiation metrics were CTDIvol = 0.46±0.14 mGy, DLP = 26.2±8.1 mGy.cm and E = 0.6±0.2 mSv. CT radiation variability was due to body habitus and arm orientation. The radiation dose differences between radiographic and CT imaging were not statistically significant. All CT scans had adequate quality for preoperative assessment of pedicle diameter and orientation, obstacles impeding pedicle entry, S2-Alar screw orientation, and intra-operative navigation. “Ultra-low dose” CT scans without sedation were feasible in paediatric patients with neuromuscular scoliosis. The effective dose was similar between the standard preoperative spinal XR and “ultra-low dose” CT scans. The “ultra-low dose” CT scan allowed accurate assessment of the anatomy, aided in pre-operative planning, and allowed intra-operative navigation despite the movement disorders in this patient population

Aims. It is uncertain whether instrumented spinal fixation in nonambulatory children with neuromuscular scoliosis should finish at L5 or be extended to the pelvis. Pelvic fixation has been shown to be associated with up to 30% complication rates, but is regarded by some as the standard for correction of deformity in these conditions. The incidence of failure when comparing the most caudal level of instrumentation, either L5 or the pelvis, using all-pedicle screw instrumentation has not previously been reported. In this retrospective study, we compared nonambulatory patients undergoing surgery at two centres: one that routinely instrumented to L5 and the other to the pelvis. Methods. In all, 91 nonambulatory patients with neuromuscular scoliosis were included. All underwent surgery using bilateral, segmental, pedicle screw instrumentation. A total of 40 patients underwent fusion to L5 and 51 had their fixation extended to the pelvis. The two groups were assessed for differences in terms of clinical and radiological findings, as well as complications. Results. The main curve (MC) was a mean of 90° (40° to 141°) preoperatively and 46° (15° to 82°) at two-year follow-up in the L5 group, and 82° (33° to 116°) and 19° (1° to 60°) in the pelvic group (p < 0.001 at follow-up). Correction of MC and pelvic obliquity (POB) were statistically greater in the pelvic group (p < 0.001). There was no statistically significant difference in the operating time, blood loss, or complications. Loss of MC correction (> 10°) was more common in patients fixated to the pelvis (23% vs 3%; p = 0.032), while loss of pelvic obliquity correction was more frequent in the L5 group (25% vs 0%; p = 0.007). Risk factors for loss of correction (either POB or MC) included preoperative coronal imbalance (> 50 mm, odds ratio (OR) 11.5, 95%confidence interval (CI) 2.0 to 65; p = 0.006) and postoperative sagittal imbalance (> 25 mm, OR 11.0, 95% CI1.9 to 65; p = 0.008). Conclusion. We found that patients undergoing pelvic fixation had a greater correction of MC and POB. The rate of complications was not different. Preoperative coronal and postoperative sagittal imbalance were associated with increased risks of loss of correction, regardless of extent of fixation. Therefore, we recommend pelvic fixation in all nonambulatory children with neuromuscular scoliosis where coronal or sagittal imbalance are present preoperatively. Cite this article: Bone Joint J 2020;102-B(2):261–267

Patients with neuromuscular disease and imbalance present a particularly challenging clinical situation for the orthopaedic hip surgeon. The cause of the neuromuscular imbalance may be intrinsic or extrinsic. Intrinsic disorders include those in which the hip is in development, such as cerebral palsy, polio, CVA, and other spinal cord injuries and disease. This can result in subluxation and dislocation of the hip in growing children, and subsequent pain, and difficulty in sitting and perineal care. Extrinsic factors involve previously stable hips and play a secondary role in the development of osteoarthritis and contractures in later life. Examples of extrinsic factors are Parkinson's disease, dyskinesis, athetosis, and multiple sclerosis. Goals of treatment in adults with pain and dysfunction in the setting of neuromuscular imbalance are to treat contractures and to perform salvage procedures to improve function and eliminate pain. Treatment of patients with neuromuscular imbalance may include resection arthroplasty (Girdlestone), arthrodesis, or total hip arthroplasty. Resection arthroplasty is typically reserved for patients that are non-ambulatory, or hips that are felt to be so unstable that arthroplasty would definitely fail due to instability. In modern times arthrodesis has limited use as it negatively impacts function and self-care in patients with neuromuscular disorders. Total hip arthroplasty has the ability to treat pain, relieve contractures, and provide improved function. Due to the increased risk of instability, special considerations must be made during primary total hip arthroplasty in this patient cohort. Risk of instability may be addressed by surgical approach, head size, or use of alternative bearing constructs. Posterior approach may have increased risk of posterior dislocation in this patient group, particularly if a posterior capsular repair is not possible due to the flexion contractures and sitting position in many patients. Surgeons familiar with the approaches may utilise the anterolateral or direct anterior approach judicially. Release of the adductors may be performed in conjunction with primary total hip arthroplasty to help with post-operative range of motion and to decrease risk of instability. In a standard bearing, the selected head size should be the largest that can be utilised for the particular cup size. Rigorous testing of intra-operative impingement, component rotation, and instability is required. If instability cannot be adequately addressed by a standard bearing, the next option is a dual mobility bearing. Multiple studies have shown improved stability with the use of these bearings, but they are also at risk for instability, intraprosthetic dislocation, and fretting and corrosion of the modular connections. Another option is a constrained liner. However, this results in reduced range of motion, and an increased risk for mechanical complications of the construct. The use of a constrained liner in a primary situation should be limited to the most severe instability cases, and the patient should be counseled with the associated risks. If total hip arthroplasty results in repeated instability, revision surgery or Girdlestone arthroplasty may be considered

Degenerative problems of the hip in patients with childhood and adult onset neuromuscular disorders can be challenging to treat. Many orthopaedic surgeons are reluctant to recommend total hip replacement (THR) for patients with underlying neuromuscular disorders due to the perceived increased risks of dislocation, implant loosening, and lack of information about the functional outcomes and potential benefits of these procedures in these patients. Modular femoral components and alternative bearings which facilitate the use of large femoral heads, constrained acetabular components and perhaps more importantly, a better understanding about the complications and outcomes of THR in the patient with neuromuscular disorders, make this option viable. This paper will review the current literature and our experience with THR in the more frequently encountered neuromuscular disorders. Cite this article: Bone Joint J 2014;96-B(11 Suppl A):27–31

Introduction: The study was to evaluate the effectiveness using a new type of instrumentation, a U-rod, in the treatment of neuromuscular scoliosis. This technique provides a method of secure fixation and excellent correction in neuromuscular curves, including correction of pelvic obliquity by terminating the rod construct in pedicle screws at Lumbar 5 without crossing the lumbosacral joint. The need for surgery for progressive neuromuscular scoliosis is not controversial. However, often the type of instrumentation to be used is. Initially, Luque rods provided strong segmental fixation and the advent of the unit rod allowed strong segmental fixation with excellent fixation to the pelvis. However, there are cases where instrumentation to the pelvis is neither feasible nor necessary. The U-rod offers the structural stability of a unit rod, being one continuous rod, avoiding the instability often seen with linked Luque rods, but without the need to invade the pelvis. The U-rod terminates in pedicle screws at Lumbar 4 or 5, is fixed segmentally to the remainder of the spine, and connects pelvic obliquity through the pull of the iliolumbar ligaments. Methods and Results: 11 patients have been treated with the U-rod, all for neuromuscular curves. Minimum follow-up is two years. Primary indications for use of the U-rod are: 1) ambulatory neuromuscular patient, 2) a lumbar curve with less than 15° tilt of Lumbar 5 on Sacral 1, despite the degree of pelvic obliquity, 3) a non-ambulatory neuromuscular patient meeting the above criteria for lumbar tilt/and/or pelvic obliquity. Correction of curves has been excellent, accomplished either by posterior instrumentation alone or posterior instrumentation following anterior discectomy. The greater the degree of correction of the lumbar curve, the greater the correction of the pelvic obliquity Pelvic obliquity of up to 45° has been corrected with instrumentation to Lumbar 5 and the correction has been maintained. Conclusions: In selected patients, the U-rod offers the ability to correct neuromuscular curves, including those with significant pelvic obliquity , without the necessity to invade the pelvis or cross the Lumbar 5 Sacral 1 joint. This is important in ambulatory neuromuscular patients. In non-ambulatory patients the unit rod offers convenience, decreased operative time, blood loss, and preserving the iliac crest for bone grafting

Arthritis of the hip is a relatively common problem in patients with neuromuscular disorders due to muscle imbalance around the hip from weakness, paralysis, contractures and spasticity. Neuromuscular disorders such as cerebral palsy, Parkinson's disease, poliomyelitis, previous cerebrovascular accident (CVA) and Charcot arthropathy have been considered by many to be contraindications to total hip arthroplasty (THA). The presence of certain anatomic abnormalities (excessive femoral anteversion, acetabular dysplasia, leg length discrepancy (LLD) and coax valga) and significant soft tissue contractures, muscle imbalance, and muscular weakness make THA a challenging surgical procedure in this patient population, and can predispose to dislocation and poor functional outcome following surgery. THA can, however, result in substantial pain relief and functional improvement, and can be safely performed, provided certain technical considerations are addressed. The patient's motor strength and functional status (ambulatory vs. “sitter”) should be carefully assessed preoperatively, since both of these factors may affect the choice of surgical approach and component position. Significant soft tissue contractures should be released at the time of surgery. Although these can be frequently performed “open”, percutaneous adductor tenotomy is occasionally necessary for patients with significant adduction contractures. Patients requiring significant soft tissue releases may benefit from 6 weeks of bracing to allow soft tissues to heal in appropriately and minimise risk of dislocation during this period of time. Use of modular femoral components that allow for correction of excessive femoral anteversion, should be considered in patients with coexistent dysplasia and neuromuscular disease (i.e., CP or polio). Large femoral head components should also be considered in patients with increased risk factors for dislocation. Despite their obvious theoretical advantages, the use of large head metal on metal THAs should be used with extreme caution in view of growing concerns about these devices. Although constrained acetabular liners are associated with an increased risk of mechanical failure, their use should be strongly considered in patients with significant motor weakness or major soft tissue deficiencies. Meticulous soft tissue closure of the capsule of the hip is recommended, especially when performing THA through a posterior approach. Patients with neuromuscular disorders associated with spasticity and involuntary movements need to be optimally treated medically prior to and indefinitely after THA. There are limited reports of outcomes following THA in patients with neuromuscular disorders, however, some generalizations based on underlying diagnosis can be made. Patients with cerebral palsy and polio frequently have acetabular dysplasia, excessive femoral anteversion and LLD, and although durability does not seem to be a major concern, dislocation and instability is relatively common and needs to be addressed. Durability and instability do not appear to be major concerns in patients with Parkinson's disease, however, these patients have frequent medical complications perioperatively and have deterioration in function over time due to the progressive nature of their underlying disorder. Patients with previous CVA also appear to have acceptable durability and dislocation risk, but are at high risk of developing heterotopic ossification postoperatively. Patients with Charcot arthropathy or myelodysplasia are at high risk of instability and appear to have limited functional improvement following THA. As a result, the consensus of opinion is that THA is contraindicated in these patients

Arthritis of the hip is a relatively common problem in patients with neuromuscular disorders due to muscle imbalance around the hip from weakness, paralysis, contractures and spasticity. Neuromuscular disorders such as cerebral palsy, Parkinson's disease, poliomyelitis, previous cerebrovascular accident (CVA) and Charcot arthropathy have been considered by many to be relative contraindications to total hip arthroplasty (THA). The presence of certain anatomic abnormalities (excessive femoral anteversion, acetabular dysplasia, leg length discrepancy (LLD) and coax valga) and significant soft tissue contractures, muscle imbalance, and muscular weakness make THA a challenging surgical procedure in this patient population, and can predispose to dislocation and poor functional outcome following surgery. THA can, however, result in substantial pain relief and functional improvement in patients with significant hip arthritis and neuromuscular disorders, and can be safely performed, provided certain technical considerations are addressed. The patient's motor strength and functional status (ambulatory vs. “sitter”) should be carefully assessed pre-operatively, since both of these factors may affect the choice of surgical approach and component position. Significant soft tissue contractures should be released at the time of surgery. Although these can be frequently performed “open,” percutaneous adductor tenotomy is occasionally necessary for patients with significant adduction contractures. Patients requiring significant soft tissue releases may benefit from 6 weeks of bracing to allow soft tissues to heal in appropriately and minimize risk of dislocation during this period of time. Use of modular femoral components that allow for correction of excessive femoral anteversion, should be considered in patients with coexistent dysplasia and neuromuscular disease (i.e. CP or polio). Large femoral head components should also be considered in patients with increased risk factors for dislocation. Despite their obvious theoretical advantages, the use of large head metal on metal THAs should be used with extreme caution in view of growing concerns about these devices. Although constrained acetabular liners are associated with an increased risk of mechanical failure, their use should be strongly considered in patients with significant motor weakness or major soft tissue deficiencies. Meticulous soft tissue closure of the capsule of the hip is recommended, especially when performing THA through a posterior approach. Patients with neuromuscular disorders associated with spasticity and involuntary movements need to be optimally treated medically prior to and indefinitely after THA. There are limited reports of outcomes following THA in patients with neuromuscular disorders, however some generalisations based on underlying diagnosis can be made. Patients with cerebral palsy and polio frequently have acetabular dysplasia, excessive femoral anteversion and LLD, and although durability does not seem to be a major concern, dislocation and instability is relatively common and needs to be addressed. Durability and instability do not appear to be major concerns in patients with Parkinsons disease, however, these patients have frequent medical complications perioperatively and have deterioration in function over time due to the progressive nature of their underlying disorder. Patients with previous CVA also appear to have acceptable durability and dislocation risk, but are at high risk of developing heterotopic ossification post-operatively. Patients with Charcot arthropathy or myelodysplasia are at high risk of instability and appear to have limited functional improvement following THA. As a result, the consensus of opinion is that THA is contraindicated in patients with Charcot arthropathy and myelodysplasia

Neuromuscular scoliosis is a difficult condition to treat. Curve severity, secondary pelvic obliquity and poor respiratory function can make operative treatment and post operative care challenging. The benefits to the child in terms of improved sitting position and trunk posture can be considerable. We present a large consecutive series of patients with neuromuscular scoliosis treated surgically at our institution. The aim of this work was to study the clinical and radiographic impact of surgery for neuromuscular scoliosis. Data was gathered from patient records and radiographs for all cases of neuromuscular scoliosis treated surgically between April 2002 and Feb 2005. 52 cases were identified. They fell into 2 surgical groups: single stage posterior correction and two stage anterior and posterior correction. All posterior instrumentation was transpedicular. Complications, length of stay, and change in severity of sagittal and coronal plane deformity were recorded. Average pre-operative Cobb angle was 85°. There were 16 patients with additional sagittal plane deformity. Average percentage improvement of Cobb angle was 59%. The correction was better in two stage procedures. Pelvic obliquity was improved in those who were obligatory sitters. Fusion rate was 83% for those followed up more than 1 year. ITU stay was longer in single stage procedures. Complication rate was 58%. We have shown that with appropriate patient selection the correction of neuromuscular scoliosis can achieve good results with high fusion rates. Two stage correction confers correctional advantage on those who have sufficient respiratory reserve to tolerate it

The objective of this study was to determine if abnormal neuromuscular patterns exist in individuals with knee Osteoarthritis compared to those with healthy knees. We collected surface electromyographic signals during preferred speed and fast walk conditions from seven muscles crossing the knee joint. We found differences between the two groups that could lead to differences in joint loading, with the OA group having higher coactivity between hamstrings and quadriceps during initial loading. Further investigating these differences is warranted in particular given the trend for lower external extensor moments for the OA group at the fast walking speed. The purpose of this study was to compare neuromuscular control of knee joint motion during walking between those with moderate Osteoarthritis (OA) and those with healthy knees (CON). Moderate OA neuromuscular control patterns differed from those with healthy knees. Detecting neuromuscular alteration associated with mild to moderate knee OA is important to direct therapeutic strategies aimed to slow down or possibly reverse disease progression. Surface electromyographic (EMG) recordings were collected from seven muscles crossing the knee joint of CON (n=7) and those with moderate OA (n=4) during preferred speed and a fast-paced walks. A pattern recognition technique was applied to the EMG profiles. No differences (> 0.05) were reported between the two groups for spatial and temporal gait parameters or knee joint kinematics. Statistical differences were found (p< 0.05) in muscle activation patterns between the two groups and the differences were more prominent at the faster walking speed. The two vasti muscles had double peaks during stance and higher amplitudes at heel strike for the OA group. There was higher activity in the two hamstring muscles at heel contact and a burst of activity during late stance for the OA group. The disproportionately higher knee flexor coactivity at heel strike may reflect a guarded response to pain, whereas the burst during weight transfer may reflect a stabilizing response as the knee moment changes from a flexor to an extensor moment. At normal walking speeds the neuromuscular control patterns were similar between groups, but differences were exaggerated when the system was stressed at higher speed

Scoliosis correction surgery is one of the longest and most complex procedures of all orthopedic surgery. The complication rate is therefore not negligible and is particularly high when the surgery is performed in patients with neuromuscular or connective tissue disease or complex genetic syndromes. In fact, these patients have various comorbidities and organ deficits (respiratory capacity, swallowing / nutrition, heart function, etc.), which can compromise the outcome of the surgery. In these cases, an accurate assessment and preparation for surgery is essential, also making use of external consultants. To make this phase simpler, more effective and homogeneous, a multidisciplinary path of peri-operative optimization is being developed in our Institute, which also includes the possibility of post-operative hospitalization for rehabilitation and recovery. The goal is to improve the basic functional status as much as possible, in order to ensure faster functional recovery and minimize the incidence of peri-operative complications, to be assessed by clinical audit. The path model and the preliminary results on the first patients managed according to the new modality are presented here. The multidisciplinary path involves the execution of the following assessments / interventions: • Pediatric visit with particular attention to the state of the upper airways and the evaluation of chronic or frequent inflammatory states • Cardiological Consultation with Echocardiogram. • Respiratory Function Tests, Blood Gas Analysis and Pneumological Consultation to evaluate indications for preoperative respiratory physiotherapy cycles, Non-Invasive Ventilation (NIV) cycles, Cough Machine. Possible Polysomnography. • Nutrition consultancy to assess the need for nutritional preparation in order to improve muscle trophism. • Consultation of the speech therapist in cases of dysphagia for liquids and / or solids. • Electroencephalogram and Neurological Consultation in epileptic patients. • Physiological consultation in patients already being treated with a cough machine and / or NIV. • Availability of postoperative hospitalization in the rehabilitation center (with skills in respiratory and neurological rehabilitation) for the most complex cases. When all the appropriate assessments have been completed, the anesthetist in charge at our Institute examines the clinical documentation and establishes whether the path can be considered complete and whether the patient is ready for surgery. At the end of the surgery, the patient is admitted to the Post-operative Intensive Care Unit of the Institute. If necessary, a new program of postoperative rehabilitation (respiratory, neuromotor, etc.) is programmed in a specialist reference center. To date, two patients have been referred to the preoperative optimization path: one with Ullrich Congenital Muscular Dystrophy, and one with 6q25 Microdeletion Syndrome. In the first case, the surgery was performed successfully, and the patient was discharged at home. In the second case, after completing the optimization process, the surgery was postponed due to the finding of urethral malformation with the impossibility of bladder catheterization, which made it necessary to proceed with urological surgery first. The preliminary case series presented here is still very limited and does not allow evaluations on the impact of the program on the clinical practice and the complication rate. However, these first experiences made it possible to demonstrate the feasibility of this complex multidisciplinary path in which a network of specialists takes part

There has been a reluctance, until relatively recently, to consider replacement of the hip in patients with substantial neuromuscular imbalance. This relates to many factors, including the young age of many (such as cerebral palsy in the older teen and young adult), developmental anatomic abnormality, oft-present poor bone health, neuromuscular imbalance, and the risk of complication; especially dislocation. Mental retardation also introduces challenges with rehabilitation and an increased burden on the family and societal support systems if the outcome is to be maximised. With the development of newer techniques and technology, and the emergence of encouraging outcome studies, these patients can be more easily offered predictable relief of pain, a reasonable chance of improved function, longevity of the reconstruction, and an acceptable risk of complication. A large number of background neurological diagnoses can lead to hip degeneration, or can introduce increased complexity during management of hip degeneration unrelated to that background. Be that as it may, a short list of fundamental questions is common to all and will help guide management:. Important questions to be addressed include:. 1. Did the NV imbalance precede skeletal development? This relates to the dependence of skeletal shape and size on the loads being placed upon it: hence “Form Follows Function”. The shape and size of the hip, and location of the femoral head, will be much different in the young adult with spastic dislocation due to cerebral palsy, when compared with the elderly adult with a late onset CVA-related spasticity superimposed on hip degeneration. 2. Is the muscle tone which will support the hip arthroplasty predominantly spastic or flaccid? In each there is a risk of dislocation, which needs to be addressed at the index procedure, but in spasticity there is the added question as to what tissues need to be released or de-functioned so as to alter the magnitude and direction of the joint reaction forces. 3. Is pain the main reason for consultation? Because pain relief is the most predictable outcome that we can offer, it should guide the indications and timing of intervention. Replacement of the NM hip to improve function, in the absence of pain, should be approached with great caution

Abstract. Source of Study: London, United Kingdom. This intervention study was conducted to assess two developing protocols for quadriceps and hamstring rehabilitation: Blood Flow Restriction (BFR) and Neuromuscular Electrical Stimulation Training (NMES). BFR involves the application of an external compression cuff to the proximal thigh. In NMES training a portable electrical stimulation unit is connected to the limb via 4 electrodes. In both training modalities, following device application, a standardised set of exercises were performed by all participants. BFR and NMES have been developed to assist with rehabilitation following lower limb trauma and surgery. They offer an alternative for individuals who are unable to tolerate the high mechanical stresses associated with traditional rehabilitation programmes. The use of BFR and NMES in this study was compared across a total of 20 participants. Following allocation into one of the training programmes, the individuals completed training programmes across a 4-week period. Post-intervention outcomes were assessed using Surface Electromyography (EMG) which recorded EMG amplitude values for the following muscles: Vastus Medialis, Vastus Lateralis, Rectus Femoris and Semitendinosus. Increased Semitendinosus muscle activation was observed post intervention in both BFR and NMES training groups. Statistically significant differences between the two groups was not identified. Larger scale randomised-controlled trials are recommended to further assess for possible treatment effects in these promising training modalities

Spinal stabilization through appropriate neuromuscular responses to external perturbations is important in the prevention and rehabilitation of low back pain (LBP). Muscle synergism, coordination and imbalances are terms used to describe the neuromuscular strategies considered important to actively maintain spinal stability. We recorded surface electromyographic (EMG) recordings from healthy controls (CON) and those with chronic, mechanical low back pain during performance of an exercise model that dynamically challenged lumbar-pelvic stability. Those with LBP showed greater variation in amplitude in response to the perturbations imposed by the exercise model, and demonstrated a lack of synergistic and antagonistic coactivation compared to the CON group. The purpose of this study was to compare the neuromuscular control strategies used by those with LBP and those without to complete a standardized task aimed at dynamically challenging stability of the lumbar spine and pelvis. Those with LBP activated their muscles in a more asynchronous manner than normal controls, illustrating an alteration in neuromuscular control that should be a focus of therapeutic intervention strategies aimed at prevention and rehabilitation of LBP. These data illustrate a need for neuromuscular retraining, focusing on muscle coactivation in response to dynamic perturbations rather than a single perturbation. Surface EMG recordings from two trunk extensor and five abdominal muscle sites were recorded from twenty-four men without LBP and fourteen men with chronic LBP while they performed a task that dynamically challenged lumbar spine and pelvis stability. The EMG amplitudes recorded from the upper and lower rectus abdominus sites were significantly (p< 0.05) lower for the LBP compared to the CON group. The temporal EMG profiles were compared using a statistical pattern recognition technique. This analysis showed that the LBP group used different patterns of synergistic muscle coactivity compared to the highly coordinated manner in which all seven muscles were recruited for the CON. These results quantify the neuromuscular control differences between the two groups providing a foundation for developing an objective classifier of neuromuscular control impairments associated with LBP. In future this approach could assist in directing therapeutic interventions in particular those aimed at muscle reeducation

Elbow contracture is a well recognised sequel of neuromuscular disorders and can be a rather debilitating condition. Non operative treatment, such as physiotherapy and splinting, results in an improved range of motion, but since musculoskeletal pathology in neuromuscular diseases is progressive, an open surgical release of the elbow is often required. Therefore, the purpose of the present study was to assess the results of surgical treatment of elbow stiffness in patients suffering from neuromuscular disorders. Between January 2000 and October 2008, 11 patients with neuromuscular diseases underwent surgical treatment of elbow contracture. The mean age of the patients was 21 years. Eight patients had cerebral palsy, 2 arthrogryposis and 1 brachial plexus palsy. Pre-operatively the lag of elbow flexion and extension was 45° and 38° respectively. In 6 patients releases were performed through a lateral approach, while 3 required an additional medial incision. In 2 patients the pathology was addressed through a posterior approach. The mean follow up was 26 months. Postoperatively one patient developed skin necrosis which was treated conservatively. Furthermore, another patient developed transient ulnar neuritis, and finally one more presented with medial collateral ligament insufficiency. All patients had an improved functional arc of motion. Namely, the lag of elbow flexion and extension was reduced to 22° and 10° respectively. At the final follow up the patients maintained 90% of the range of motion that was achieved immediately postoperatively. Open release of the elbow contracture in neuromuscular diseases yield satisfactory results. Therefore, it can be expected that patients will obtain a functional range of motion

Aim: This study measures the outcomes of surgery for neuromuscular scoliosis using patient/ carer goal setting techniques. Method and Results: In neuromuscular scoliosis, the objective of surgery is to maintain or improve sitting ability and to improve overall function. Measuring the outcome of surgery by patient/carer grading of patient/ carer set goals has not been used in this patient group. A group of 20 children who underwent spinal fusion for neuromuscular scoliosis were assessed using a postural and functional measure pre-op, post-op, and at 3 and 12 months post-op. In addition, each patient was asked to record three goals for undergoing the surgery. At one year post op, patient/carers were asked to grade on a scale of 0 – 10, how satisfied they were that the goals had been achieved. Nineteen patients had clear pre-op goals for the surgery relating to functional activities. The most frequent goals stated for the non-ambulant children were- sitting for longer periods (7/46), making dressing easier (7/46) and sitting more upright (6/38). There were 15 other functional goals stated. The ambulant children stated- appearing straighter (3/12), increase in confidence (2/12), reducing pain (2/12) and maintaining respiratory function (2/12). There were 3 other functional goals stated. Seventeen patients completed the study, 2 were lost to follow up, 1 died. The average satisfaction rate from goals achieved 1 year post-op was 7.9/10. Conclusion: Establishing goals that are realistic and contribute positively to the functional ability or practical management of the child with neuromuscular scoliosis undergoing spinal surgery, encourages the family to be central in the decision making process. It also allows unrealistic expectations to be discussed pre-op and represents the most patient centred method of outcome assessment

Introduction: Surgery in patients with neuromuscular scoliosis is aimed at improving truncal balance, facilitating sitting, prevention of progression of the curve and preservation of respiratory function. Patients and Methods: This was a retrospective study of surgical results in a group of 24 patients with minimum follow up of 2 years an average post-operative follow up of 5 years (2–9 years) with neuromuscular scoliosis due to varying aetiologies. The aetiologies included SMA (7), CP (6), Duchenne (5), Congenital Myopathies (3), Spina Bifida (2) and Paraspinal Neuroblastoma (1). Results: The average age at surgery was 10.6 years, the average duration was 4hrs 25mins with an average blood loss pf 1.1 liters. An average ICU stay was 1–2 days and stay in hospital is 11 days. The curves ranged from 25–103° (average 75.6°) pre-operative and we were able to obtain a correction of 56%. In all but 2 of our patients we were successful in preventing deterioration of respiratory function and all our patients could at least sit without support post-operatively. Our complications included only one deep infection (necessitating implant removal), 1 rod breakage and 2 patients with UTI. There were no significant respiratory complications in post-operation. Conclusion: Spinal surgery in patients with neuromuscular scoliosis is safe and prevents deterioration of respiratory and improve truncal balance and hence quality of life