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Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_1 | Pages 29 - 29
1 Jan 2022
Awadallah M Ong J Kumar N Rajata P Parker M
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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


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_13 | Pages 48 - 48
1 Dec 2022
Yee N Iorio C Shkumat N Rocos B Ertl-Wagner B Green A Lebel D Camp M
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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


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_12 | Pages 68 - 68
1 Dec 2022
Yee N Lorio C Shkumat N Rocos B Ertl-Wagner B Green A Lebel D Camp M
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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


Orthopaedic Proceedings
Vol. 100-B, Issue SUPP_10 | Pages 30 - 30
1 Jun 2018
Taunton M
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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


Orthopaedic Proceedings
Vol. 98-B, Issue SUPP_22 | Pages 31 - 31
1 Dec 2016
Kraay M
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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


Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_22 | Pages 33 - 33
1 May 2013
Kraay M
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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


Orthopaedic Proceedings
Vol. 99-B, Issue SUPP_7 | Pages 32 - 32
1 Apr 2017
Duncan C
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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


Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_1 | Pages 146 - 146
1 Jan 2013
Ul Islam S Henry A Khan T Davis N Zenios M
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Introduction. Through the paediatric LCP Hip plating system, the highly successful technique of the locking compression plate used in adult surgery, has been incorporated in a system dedicated to paediatrics. The purpose of this study was to review the outcome of the paediatric LCP Hip plate use in children, both with and without neuromuscular disease, for fixation of proximal femoral osteotomy for a variety of indications. Materials and methods. We retrospectively reviewed the notes and radiographs of all those children who have had Paediatric LCP Hip Plate for the fixation of proximal femoral osteotomy and proximal femur fractures in our institution, between October 2007 and July 2010, for their clinical progress, mobilization status, radiological healing and any complications. Results. Forty-three Paediatric LCP hip plates were used in forty patients for the fixation of proximal femoral osteotomies (n=40) and proximal femur fractures (n=3). The osteotomies were performed for a variety of indications including Perthes disease, DDH, Cerebral Palsy, Down's syndrome, coxa vara, Leg length discrepancy and previous failed treatment of SUFE. Twenty-five children were allowed touch to full weight bearing post operatively. Two were kept non-weight bearing for 6 weeks. The remaining 13 children were treated in hip spica due to simultaneous pelvic osteotomy or multilevel surgery for cerebral palsy. All osteotomies and fractures radiologically healed within 6 months (majority [n=40] within 3 months). There was no statistically significant difference (p = 0.45) in the neck shaft angle between the immediately postoperative and final x-rays after completion of bone healing. There were no implant related complications. Conclusion. The Paediatric LCP Hip Plate provides a stable and reliable fixation of the proximal femoral osteotomy performed for a variety of paediatric hip conditions in children with and without neuromuscular disease


Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_14 | Pages 29 - 29
1 Mar 2013
Puddu A Dunn R
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Purpose of the study. The aim of the study was to review the surgical management of Neuromuscular Scoliosis (NMS) and its outcome. Method. NMS patients who underwent surgery between 2001 and 2012 were identified. Case notes, surgical data, complications and radiographic results were reviewed retrospectively. The families were contacted telephonically. Sitting posture status and whether the parents and patients would opt to have the surgery again if given the opportunity, was assessed. Summary of Results. 96 NMS patients had corrective spinal surgery performed by a single surgeon in 4 institutions. Cerebral Palsy was the most common underlying cause (18) followed by Myelomeningocoele (15), SMA (12) and post spinal cord injury scoliosis (10). 52 patients were female and 44 male with a median age of 13.6 years at surgery. The delay to surgery after presentation was 20.6 months. Seventy patients received posterior only based surgery and 12 patients had anterior only fusions. Another 12 patients had a 2-stage procedure with a delay of 12 days between operations. The average blood loss was 1600ml and duration of surgery 222 minutes. Forty seven patients had pedicle screw only constructs and 46 patients' constructs included the pelvis. An average of 14.2 levels were included in the construct. The primary curve was corrected from 84.4° to 32.7° which was mostly maintained throughout the follow-up period. 55 patients had a follow-up of greater or equal to 2 years. Only one patient reported not having improved his sitting posture. 3 parents and 5 patients would opt not to have the surgery again. Conclusion. Spine fusion in NMS is effective in correcting and maintaining correction of associated scoliosis despite the risk of major complications. Most patients and family value the clinical improvement and would agree to surgery again. NO DISCLOSURES


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XVII | Pages 38 - 38
1 May 2012
Bruce-Brand R O'Byrne J Moyna N
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Quadriceps femoris muscle weakness has long been associated with disuse atrophy in symptomatic knee osteoarthritis but more recently implicated in the aetiology of this condition. The purpose of this study was to assess the benefits of two interventions aimed at increasing quadriceps strength in subjects with moderate to severe knee osteoarthritis.

Twenty-eight patients, aged fifty-five to seventy-five, were recruited and randomised to either a six-week home resistance-training exercise program or a six-week neuromuscular electrical stimulation (NMES) program. Eleven patients matched for age, gender and osteoarthritis severity formed a control group, receiving standard care. The resistance-training group performed six exercises three times per week, while the NMES group used the garment stimulator for twenty minutes five times per week Outcome measures included isometric and isokinetic quadriceps strength, functional capacity, quadriceps cross-sectional area, and validated health survey scores. These measures were assessed at baseline, post-intervention and at 6-weeks post-intervention.

Both intervention groups showed significant improvements in all functional tests, in the global health survey, and in quadriceps cross-sectional area immediately post-intervention. An increase in isokinetic strength was seen in the exercise group only. With the exception of isokinetic strength, all benefits were maintained six weeks post-intervention.

Both a six-week home resistance-training program and a six-week NMES program produce significant improvements in functional performance as well as physical and mental health for patients with moderate to severe knee osteoarthritis. Home-based NMES is an acceptable alternative to physical therapy, and is especially appropriate for patients who have difficulty complying with an exercise program.


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_1 | Pages 1 - 1
1 Jan 2022
Srinivasan SH Murthy SN Bishnoi AJ Swamy G
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Abstract. In the pediatric population, scoliosis is classified into congenital, syndromic, idiopathic, and neuromuscular in aetiology. Syndromic scoliosis represents a wide range of systemic anomalies associated with scoliosis. The primary challenge for a clinician is to think beyond the scoliotic curve, as the underlying pathology is multisystemic. The aim of this review is to identify the systemic anomalies, associated with syndromic scoliosis. MEDLINE, EMBASE, and CINAHL databases were searched, dating from 1990–2020, relevant to the purpose of our study. Keywords used: “scoliosis”+ “syndrome” + “genetic”. Retrospective, prospective studies were included. Case reports that had fewer than 4 patients were not included. Delineating 60 articles, we found a total of 41 syndromes to be associated with scoliosis. Thoracic region was the most common level of scoliosis curve, being noted in 28 syndromes. Mental retardation, seizures, and ataxia were the commonly noticed CNS anomalies. VSD, ASD, and TGA were the anomalies associated with CVS; Hypotonia, rib and vertebral malformations were the most identified neuromuscular anomalies; pulmonary hypoplasia, renal agenesis, and strabismus were other associations. A multidisciplinary approach, involving spinal surgeons, paediatricians, geneticists, anesthesiologists, and allied health professionals, is vital for the best care of patients with syndromic scoliosis. The location of the scoliotic curve reflects the associated anomalies, as thoracic curvature is more closely linked with cardiac anomalies, while lumbosacral curvature is seen to be often linked with genitourinary anomalies. We hope that this article provides a clear overview of the systemic associations in syndromic scoliosis and thus, facilitates and streamlines the management protocol


Orthopaedic Proceedings
Vol. 106-B, Issue SUPP_5 | Pages 26 - 26
23 Apr 2024
Aithie J Herman J Holt K Gaston M Messner J
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Introduction. Limb deformity is usually assessed clinically assisted by long leg alignment radiographs and further imaging modalities (MRI and CT). Often decisions are made based on static imaging and simple gait interpretation in clinic. We have assessed the value of gait lab analysis in surgical decision making comparing surgical planning pre and post gait lab assessment. Materials & Methods. Patients were identified from the local limb reconstruction database. Patients were reviewed in the outpatient clinic and long leg alignment radiographs and a CT rotational limb profile were performed. A surgical plan was formulated and documented. All patients then underwent a formal gait lab analysis. The gait lab recommendations were then compared to the initial plan. Results. Twelve patients (8 female) with mean age of 14 (range 12–16) were identified. Nine were developmental torsional malalignments, one arthrogryposis, one hemiparesis secondary to spinal tumour resection and one syndromic limb deficiency. The gait lab recommended conservative management in four patients and agreed with eight surgical plans with one osteotomy level changing. Five patients are post-operative: two bilateral distal tibial osteotomies, two de-rotational femoral osteotomy with de-rotational tibial osteotomies and one bilateral femoral de-rotational osteotomies. Conclusions. Limb deformity correction is major surgery with long rehabilitation and recovery period. Gait lab analysis can identify who would benefit from conservative management rather than surgery with our study showing changes to surgical planning in one third of patients. The gait lab analysis helps to identify patients with functional and neuromuscular imbalances where correcting the bony anatomy may not actually benefit the patient


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_13 | Pages 22 - 22
1 Dec 2022
Parker E AlAnazi M Hurry J El-Hawary R
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Clinically significant proximal junctional kyphosis (PJK) occurs in 20% of children treated with posterior distraction-based growth friendly surgery. In an effort to identify modifiable risk factors, it has been theorized biomechanically that low radius of curvature (ROC) implants (i.e., more curved rods) may increase post-operative thoracic kyphosis, and thus may pose a higher risk of developing PJK. We sought to test the hypothesis that EOS patients treated with low ROC (more curved rods) distraction-based treatment will have a greater risk of developing PJK as compared to those treated with high ROC (straighter) implants. This is a retrospective review of prospectively collected data obtained from a multi-centre EOS database on children treated with rib-based distraction with minimum 2-year follow-up. Variables of interest included: implant ROC at index (220 mm or 500 mm), patient age, pre-operative scoliosis, pre-operative kyphosis, and scoliosis etiology. In the literature, PJK has been defined as clinically significant if revision surgery with superior extension of the upper instrumented vertebrae was performed. In 148 scoliosis patients, there was a higher risk of clinically significant PJK with low ROC (more curved) rods (OR: 2.6 (95%CI 1.09-5.99), χ2 (1, n=148) = 4.8, p = 0.03). Patients had a mean pre-operative age of 5.3 years (4.6y 220 mm vs 6.2y 500 mm, p = 0.002). A logistic regression model was created with age as a confounding variable, but it was determined to be not significant (p = 0.6). Scoliosis etiologies included 52 neuromuscular, 52 congenital, 27 idiopathic, 17 syndromic with no significant differences in PJK risk between etiologies (p = 0.07). Overall, patients had pre-op scoliosis of 69° (67° 220mm vs 72° 500mm, p = 0.2), and kyphosis of 48° (45° 220mm vs 51° 500mm, p = 0.1). The change in thoracic kyphosis pre-operatively to final follow up (mean 4.0 ± 0.2 years) was higher in patients treated with 220 mm implants compared to 500 mm implants (220 mm: 7.5 ± 2.6° vs 500 mm: −4.0 ± 3.0°, p = 0.004). Use of low ROC (more curved) posterior distraction implants is associated with a significantly greater increase in thoracic kyphosis which likely led to a higher risk of developing clinically-significant PJK in EOS patients


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_8 | Pages 36 - 36
1 May 2014
Kraay M
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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 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 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 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 patients with Charcot arthropathy and myelodysplasia


Orthopaedic Proceedings
Vol. 103-B, Issue SUPP_6 | Pages 43 - 43
1 May 2021
Hutchinson R Ferguson D
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Introduction. Retention and removal of children's orthopaedic metalwork is a contentious issue that has implications for current resource allocation, health economics, complication risks and can impact on future treatments. Understanding how to guide families make informed choices requires an overview of all the relevant evidence to date, and knowledge of where the evidence is lacking. Our aim was to systematically review the literature and provide a meta-analysis where possible, recommending either retention or removal. Materials and Methods. A search of the literature yielded 2420 articles, of which 22 papers were selected for the study analysis. Inclusion criteria: Any paper (evidence level I-IV) assessing the risks or benefits of retaining or removing orthopaedic metalwork in children. Exclusion criteria: Spinal implants; implant number < 40; < 75% recorded follow up; papers including implants in their analysis that always require removal; patients aged >18 years. Results. In total, 4988 patients (6412 implants) were included across all 22 studies. There was a significant amount of heterogenicity between studies. Overall the short term risks of metalwork retention and removal are low, with a few exceptions. In forearm plating re-fracture rates following removal were lower than those seen in studies looking at retained metalwork, provided removal occurred later than 12 months from the initial operation. Forearm re-fracture rates after removal of flexible nails significantly increased if removal was performed before 6 months. Major complications following routine metalwork removal from the proximal femur are relatively rare, with re-fracture rates of 1–5%. The majority of these re-fractures are seen in neuromuscular patients or in patients where removal occurs earlier than 6 months. Routine metalwork removal following SUFE had a complication rate of 30–60%. Conclusions. We found that there are certain subgroups of children that benefit from retention of metalwork and some that benefit from removal. There are several subgroups that we have identified which do not yet have sufficient long term evidence to make a balanced recommendation. We advise that families are made aware of what is known and what is unknown in order to allow for shared decision making


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXIII | Pages 233 - 233
1 May 2012
Hohmann E Tay M Tetsworth K Bryant A
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Given their role in reducing anterior tibial translation, the recruitment patterns and viscoelastic properties of the hamstring muscles have been implicated as neuromuscular factors contributing to the ACL gender bias. Nevertheless, it is uncertain whether patterns of aberration displayed by the female neuromuscular system significantly alters the antagonist moments generated by the hamstrings during maximal effort knee extension. The purpose of the current study was to examine the effect of gender on hamstring antagonist moments in order to explain the higher ACL injury rates in females. Eleven females (age 30.6 ± 10.1 years, mass 62.1± 6.9 kg, height 165.9 ± 4.6) and 11 males (age 29.0 ± 8.2 years, mass 78.6± 14.4 kg, height 178.5± 6.2) were recruited as subjects. Surface electrodes were placed over the semitendinosus (ST) and biceps femoris (BF) muscles of the dominant and non-dominant limbs. Each subject performed two sets of five maximal extension and flexion repetitions at 180-1. EMG, isokinetic torque and knee displacement data were sampled at 1000Hz using an AMLAB data acquisition system. Average hamstring antagonist torque data across the range of knee flexion for female subjects was significantly higher (%Diff=24%) than for the male control subject. Statistical analyses revealed a significant main effect of gender (F = 4.802; p = 0.036). Given that females possess a more compliant ACL and hamstring musculature, compared with their male counterparts, an augmented hamstring antagonist may represent a compensatory neuromuscular strategy to increase knee stiffness to control tibial translation and ACL strain. The results of this project suggest that it is unlikely that gender-related differences in hamstring antagonist torque is one of the predisposing factors contributing to the higher ACL injury rates in females


Orthopaedic Proceedings
Vol. 102-B, Issue SUPP_6 | Pages 114 - 114
1 Jul 2020
Thompson G Hardesty C Son-Hing J Ina J Tripi P Poe-Kochert C
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Intrathecal morphine (IM) is a common adjunct in paediatric spinal deformity surgery. We previously demonstrated with idiopathic scoliosis it provides safe and effective analgesia in the immediate postoperative period. This study represents our 25 year experience with IM in all diagnostic groups. Our prospective Pediatric Orthopaedic Spine Database (1993–2018) was reviewed to identify all patients undergoing spinal deformity surgery who received IM and who did not. Patients 21 years of age or less who had a posterior spinal fusion (PSF) with segmental spinal instrumentation (SSI), and received 9–19 mcg/kg (up to 1 mg) of IM were included. Early onset scoliosis surgical patients were excluded. We assessed demographics, pain scores, time to first dose of opioids, diagnoses, surgical time, paediatric intensive care unit (PICU) admission and IM complications (respiratory depression, pruritus, nausea/ vomiting). There were 986 patients who met inclusion criteria. This included 760 patients who received IM and 226 who did not. IM was not used for short procedures (< 3 hrs), respiratory concerns, unsuccessful access of intrathecal space, paraplegia, and anesthesiologist decision. Both groups followed the same strict perioperative care path. The patients were divided into 5 diagnostic groups (IM / non IM patients): idiopathic (578/28), neuromuscular (100/151), syndromic (36/17), and congenital scoliosis (32/21) and kyphosis (14/9). Females predominated over males (697/289). The first dose of opioids after surgery was delayed for a mean of 10.6 hrs in IM group compared to 2.3 hrs in the non-IM group (p=0.001). The postoperative pain scores were significantly lower in the IM groups in the Post Anesthesia Care Unite (p=0.001). Only 17 IM patients (2%) were admitted to the PICU for observation secondary to respiratory depression, none required re-intubation. None of the IM group were re-intubated. Forty-nine patients (6%) experienced pruritus in the IM group compared to 4 of 226 patients (2%) in the non IM group. There were 169 patients (22%) of the IM patients and 21 patients (9%) of the non IM had nausea and vomiting postoperatively. Three patients (0.39%) had a dural leak from the administration of IM but did not require surgical repair. There were no other perioperative complications related to the use of IM. There were no significant group differences. Pre-incision IM is a safe and effective adjunct for pain management in all diagnostic groups undergoing spinal surgery. The IM patients had lower pain scores and a longer time to first administration of post-operative opioids. Although there is an increased frequency of respiratory depression, pruritus, and nausea/vomiting in the IM group, there were no serious complications


Orthopaedic Proceedings
Vol. 102-B, Issue SUPP_7 | Pages 42 - 42
1 Jul 2020
Rollick N Helfet D Bear J Diamond O Wellman D
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Malreduction of the syndesmosis is a poor prognosticator following ankle fracture and has been documented in as many as 52% of patients following fracture fixation. The current standard for assessment of reduction of the syndesmosis is bilateral computed tomography (CT) scan of the ankle. Multiple radiographic parameters are utilized to define malreduction, however, there has been limited investigation into the accuracy of these measurements to differentiate malreduction from inherent anatomical asymmetry. The purpose of this study was to identify the prevalence of positive malreduction standards within the syndesmosis of native, uninjured ankles. Bilateral lower extremity CT scans including ankles were screened. Studies were excluded if the patient was skeletally immature, had pathology below the knee or if they had congenital neuromuscular syndromes. The resulting cohort consisted of 207 patients. The indication for bilateral CT scan was femoral acetabular impingement in 110 patients (53%), rotation assessment following arthroplasty in 32 patients (15%), rotation assessment following femoral fracture in 30 patients (14%), rotational assessment for patellar instability in 30 patients (14%) and five miscellaneous indications (2%). Fifty patients were reviewed by three observers independently and to determine inter-observer reliability. A single observer repeated the measurements within the same cohort four weeks later to evaluate intra-observer reliability. Three observers then measured the anterior syndesmotic distance, posterior syndesmotic distance, central syndesmotic distance, fibular rotation and sagittal fibular translation at 1cm from the distal tibial articular surface. Overall side to side variability between the left and right ankle were assessed. Previously studied malreduction standards were evaluated. These included: anterior to posterior syndesmotic distance > 2mm, central syndesmotic difference > 1.5mm, average syndesmotic distance > 2mm, fibular rotational difference > 10o and sagittal translational difference > 2mm. The inter- and intra-observer reliability was good to excellent for anterior, posterior and central syndesmotic distance, and fibular rotation measurements. Sagittal fibular translation had an ICC of 0.583, and thus was only of fair reliability. Side to side comparison revealed statistically significant difference in only anterior syndesmotic difference (p=0.038). A difference of anterior to posterior syndesmotic distance of greater than 2mm was observed in 43 patients (20.2%). Thirty eight patients (17.8%) had a central syndesmotic difference of greater than 1.5mm. A fibular rotational difference of greater than 10o was observed in 49 patients (23%). The average difference between the anterior and posterior syndesmosis was greater than 2mm in 17 patients (8.2%). Nine patients (4.2%) had sagittal translation of greater than 2mm. Eighty one patients (39%) demonstrated at least one parameter beyond previously set standards for malreduction. Only one parameters was anomalous in 54 patients (26%), 18 patients (8%) had two positive parameters, while eight patients (4%) had three. One patient was asymmetrical in all measured parameters. In this study there was no statistically significant asymmetry between ankles. However, 39% of native syndesmoses would be classified as malreduced on CT scan using previously studied malreduction limits. Current radiographic parameters are not sufficient to differentiate mild inherent anatomical asymmetry from malreduction of the syndesmosis


Orthopaedic Proceedings
Vol. 98-B, Issue SUPP_3 | Pages 26 - 26
1 Jan 2016
Matsuda S
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Introduction. Genu recurvatum deformities are unusual before total knee arthroplasty (TKA), occurring in less than 1% of patients. The purpose of this study is to evaluate the clinical and radiographic results of primary TKA in patients that had recurvatum deformities before surgery. Patients and Methods. The inclusion criteria was to have recurvatum deformity over 10 degrees on lateral standing X-ray view. We retrospectively reviewed 22 knees with pre-operative recurvatum deformities, and the incident was 1.0% of all TKAs at our hospital. The etiology of the arthritis was osteoarthritis in 21 knees, of which 3 knees were neuropathic disease, and rheumatoid arthritis in 1 knee. There were 6 men and 16 women, and the average age was 73.3 years (range, 53 to 83 years) at the time of operation. The average follow-up period was 15 months (range, 3 to 81 months). We performed to use medial parapatellar approach and bone cutting was done by measured resection technique. The surgical knacks were resection of less distal femur and proximal tibia bone to make extension gap tightly, additionally decrease the tibial posterior slope. Posterior-stabilizer (PS) implants (NexGen LPS: Zimmer, Bisurface KU4+: JMM) were used in 20 knees and constrained implants (NexGen RH knee: Zimmer, Endo-Model Hinge Knee: Link) were in 2 knees with neuropathic joints. Results. The averaged Knee Society Knee and Function score improved from 33.1 points to 94.1 points, and 28.0 points to 60.5 points at the time of the last follow-up. The femorotibial angle changed from averaged 183.4 degrees (range, 162 to 195 degrees) preoperatively to averaged 173.3 degrees (range, 170 to 177 degrees). Preoperative hyperextension was averaged 18.2 degrees (range, 10 to 40 degrees). Intraoperatively, the hyperextension deformity was corrected in all cases. The tibial posterior slope was averaged 1.7 degrees (range, −2.7 to 6 degrees). The final hyperextension improved averaged 6.9 degrees (range, −1.7 to 26.6 degrees), all but one knee were corrected. One case treated with a standard PS type, who was associated with neuropathic disease, had a recurrence of recurvatum deformity and required revision surgery. Discussion. Recurvatum may be associated with a severe osseous deformity, capsular or ligamentous laxity, and neuromuscular disease. Surgical solutions may be to use a standard PS prosthesis with a slight underresection of the bone edge, and decrease tibial posterior slope. An alternative solution is to use a rotating-hinge prosthesis with less than antigravity quadriceps strength for neuromuscular disease