Subluxation of the hip is common in patients with intermediate spinal muscular atrophy. This retrospective study aimed to investigate the influence of surgery on pain and function, as well as the natural history of subluxed hips which were treated conservatively. Thirty patients were assessed clinically and radiologically. Of the nine who underwent surgery only one reported satisfaction and four had recurrent subluxation. Of the 21 patients who had no surgery, 18 had subluxation at the latest follow-up, but only one reported pain in the hip. We conclude that surgery for subluxation of the hip in these patients is not justified

The majority of patients with chronic infantile and juvenile forms of spinal muscular atrophy survive to adult life. Forty-four patients have been reviewed at an average of 17 years after diagnosis. The subdivision of patients into four groups, based on the maximal physical function developed by the individual, correlates well with the onset and severity of secondary deformity of the limbs and spine. This information allows anticipation of the problems and plans for their treatment to be made from early childhood. After analysis of the orthotic and surgical treatment received by these patients, a specific programme of care is recommended for each of the functional groups

Background: The long term survival of patients with type II and III spinal muscular atrophy differs considerably from patients with Duchenne muscular dystrophy. Despite this, treatment of scoliosis in both groups is often reported together. 1. There are only sporadic reports, all with small numbers, of combined anterior and posterior (two stage) scoliosis surgery in patients with spinal muscular atrophy (SMA). 1. The aim of the current study was to document the peri-operative morbidity, length of stay and correction of deformity in patients with SMA that had two stage surgery and compare them with the patients that had single stage surgery. Methods: A retrospective analysis of data on our consecutive series of patients with SMA. We analysed the data of 31 patients with SMA (16M:15F) who underwent scoliosis surgery between 1996 and 2004. The data collected included SMA type, age at surgery, percentage predicted forced vital capacity(%PFVC), blood loss, duration of surgery, complications, type of surgery undertaken, pre-operative mean Cobb angle ± SD(including bending film Cobb angle ± SD), post-operative Cobb angle ± SD and length of hospital stay. The decision to do single or two stage surgery was based on the history of recurrent chest infection, %PFVC and the stiffness of the curve. Percentage correction of Cobb angle in patients that had two stage surgery compared with those that had single stage posterior surgery. Comparison of post-operative respiratory complications, estimated blood loss, total hours in theatre and mean length of stay between the two groups. Results: There were 27 SMA type II and 4 SMA type III’s with a mean age at surgery of12.5 years (range 7.8 – 17.4). The mean pre-operative Cobb angle of all 31 patients was 89.7° ± 19.7°, the mean bending preoperative Cobb angle was 54° ± 13.3° and the mean post-operative Cobb angle was 33.7° ± 17.3°. Eighteen patients had single stage surgery and 13 had two stage surgery. Twelve out of the thirteen two stage operations had either a thoracotomy or a thoracoabdominal approach. In the patients that had single stage posterior surgery, the mean bending preoperative Cobb angle was 54° ± 13.3° and the mean post-operative Cobb angle was 38.7° ± 19.2°. In the patients that had two stage surgery the mean pre-operative bending Cobb angle was 53.6° ± 11.6° and the post-operative Cobb angle was 25.5° ± 10.8°. The %PFVC in the patients that had single stage and two stage surgery was 39.2 ± 12.8 and 69.2 ± 12.2 respectively. There were 3 respiratory complications in the single stage group and 4 in the two stage group. The average total estimated blood loss (EBL) in the single stage and two stage groups (first and second stage EBL’s combined) were 2433ml and 1902ml respectively. The length of stay for the patients with single stage surgery and two stage surgery was 14.1 ± 4.1 and 18.5 ± 7.4 days respectively. The total surgical hours for the patients with single and two stage surgery were 2.9 ± .6 hrs and 4.8 ± 1.2 hrs respectively. Conclusion: The results of our series would suggest that in a selected group of SMA patients (no history of recurrent chest infection and an acceptable %PFVC) a better immediate deformity correction can be attained with two stage surgery. This has to be weighed up with a greater total EBL and mean length of stay for the patients that had two stage surgery

Introduction Musculoskeletal injuries, especially fractures, cause reduced limb mobilization. The diminished limb activity promotes muscular atrophy, leading to a slower return to function. Attempts to prevent this atrophy using electrical stimulation have been described after knee reconstruction. The Myospare percutaneous electrical stimulator has been developed to prevent immobilization related atrophy. We undertook this pilot study to assess feasibility, safety, and efficacy of applying electrical stimulation under a cast after ankle fractures. Patients and Methods Between May and December 2004, patients who sustained closed ankle fractures requiring surgery, were recruited to participate in this study. 24 patients took part in the study, sixteen male and eight female. Age range was 18 to 62 years (average 40). All patients underwent open reduction and internal fixation using standard AO technique. A short walking cast was applied after surgery. Patients were randomized into a treatment and a control group. The experimental device was applied in the treatment group for 6 weeks. Patients were examined at 2, 6 and 12 weeks. Evaluation included measurement of calf and ankle circumference, dorsiflexion and plantiflexion, and calculation of the ratio between the injured and uninjured side. At each visit pain intensity was assessed using a visual analog score, and patients filled out a function assessment questionnaire. Analysis was performed using chi square, t-test and repeated measures analysis. Results All patients tolerated the stimulator well. No adverse effects were encountered. There is a trend toward improvement in calf diameter, dorsiflexion and plantarflexion. However, with the small number of patients in this study, no significant difference was apparent. Functional recovery and VAS scores were borderline higher in the treatment group at 12 weeks (p=0.043 and p=.049) when compared to baseline. Discussion The use of the Myospare device under a cast in patients after surgical fixation of ankle fractures has been demonstrated as feasible and safe. In this pilot study a trend toward enhanced recovery was apparent in the treatment group

Aims

The gluteus minimus (GMin) and gluteus medius (GMed) have unique structural and functional segments that may be affected to varying degrees, by end-stage osteoarthritis (OA) and normal ageing. We used data from patients with end-stage OA and matched healthy controls to 1) quantify the atrophy of the GMin and GMed in the two groups and 2) describe the distinct patterns of the fatty infiltration in the different segments of the GMin and GMed in the two groups.

The June 2024 Children’s orthopaedics Roundup. 360. looks at: Proximal femoral unicameral bone cysts: is ESIN the answer?; Hybrid-mesh casts in the conservative management of paediatric supracondylar humeral fractures: a randomized controlled trial; Rate and risk factors for contralateral slippage in adolescents treated for slipped capital femoral epiphysis; CRP predicts the need to escalate care after initial debridement for musculoskeletal infection; Genu valgum in paediatric patients presenting with patellofemoral instability; Nusinersen therapy changed the natural course of spinal muscular atrophy type 1: what about spine and hip?; The necessity of ulnar nerve exploration and translocation in open reduction of medial humeral epicondyle fractures in children

Aims. To report the mid-term results of a modified self-growing rod (SGR) technique for the treatment of idiopathic and neuromuscular early-onset scoliosis (EOS). Methods. We carried out a retrospective analysis of 16 consecutive patients with EOS treated with an SGR construct at a single hospital between September 2008 and December 2014. General demographics and deformity variables (i.e. major Cobb angle, T1 to T12 length, T1 to S1 length, pelvic obliquity, shoulder obliquity, and C7 plumb line) were recorded preoperatively, and postoperatively at yearly follow-up. Complications and revision procedures were also recorded. Only patients with a minimum follow-up of five years after surgery were included. Results. A total of 16 patients were included. Six patients had an idiopathic EOS while ten patients had a neuromuscular or syndromic EOS (seven spinal muscular atrophy (SMA) and three with cerebral palsy or a syndrome). Their mean ages at surgery were 7.1 years (SD 2.2) and 13.3 years (SD 2.6) respectively at final follow-up. The mean preoperative Cobb angle of the major curve was 66.1° (SD 8.5°) and had improved to 25.5° (SD 9.9°) at final follow-up. The T1 to S1 length increased from 289.7 mm (SD 24.9) before surgery to 330.6 mm (SD 30.4) immediately after surgery. The mean T1 to S1 and T1 to T12 growth after surgery were 64.1 mm (SD 19.9) and 47.4 mm (SD 18.8), respectively, thus accounting for a mean T1 to S1 and T1 to T12 spinal growth after surgery of 10.5 mm/year (SD 3.7) and 7.8 mm/year (SD 3.3), respectively. A total of six patients (five idiopathic EOS, one cerebral palsy EOS) had broken rods during their growth spurt but were uneventfully revised with a fusion procedure. No other complications were noted. Conclusion. Our data show that SGR is a safe and effective technique for the treatment of EOS in nonambulatory hypotonic patients with a neuromuscular condition. Significant spinal growth can be expected after surgery and is comparable to other published techniques for EOS. While satisfactory correction of the deformity can be achieved and maintained with this technique, a high rate of rod breakage was seen in patients with an idiopathic or cerebral palsy EOS. Cite this article: Bone Joint J 2020;102-B(11):1560–1566

Disturbed muscular architecture, fatty infiltration and muscular atrophy remain irreversible in chronic rotator cuff tears (RCT) even after repair. Poly-[ADP-ribose]-polymerase 1 (PARP-1), a nuclear factor involved in DNA damage repair, has shown to be a key element in the up-regulation of early muscle inflammation, atrophy and fat deposition. We therefore hypothesized that the absence of PARP-1 would lead to a reduction in muscular architectural damage, early inflammation, atrophy and fatty infiltration subsequent to combined tenotomy and neurectomy in a PARP-1 knock-out mouse model. PARP-1 knock-out (KO group) and standard wild type C57BL/6 (WT group) mice were randomly allocated into three different time points (1, 6 and 12 weeks, total n=72). In all mice the supraspinatus (SSP) and infraspinatus (ISP) tendons of the left shoulder were detached and the SSP muscle was denervated according to a recently established model. Macroscopic muscle weight analysis, retraction documentation using macroscopic suture, magnetic resonance imaging, immunohistochemistry gene expression analysis using real time qPCR (RTqPCR) and histology were used to assess the differences in muscle architecture, early inflammation, fatty infiltration and atrophy between knock out and wild type mice in the supraspinatus muscle. The SSP did retract in both groups, however; the KO muscles and tendons retracted less than the WT muscles (2.1±21mm vs 3.4±0.41mm; p=0.02). Further assessment of muscle architecture demonstrated that the pennation angle was significantly higher in the KO groups at 6 and 12 weeks (28±5 vs 36±5 and 29±4 vs 34±3; p<0.0001). Combined Tenotomy and neurectomy resulted in a significant loss of muscle mass in both groups compared to the contralateral unoperated side (KO group 62±11% and WT group 52±11%, p=0.04) at 6 weeks. But at 12 weeks postoperatively, there was a significant increase in muscle mass to near normal levels in KO group compared to the WT group (14±6% and 42±7% lower muscle mass respectively; p<0.0001) and less fatty infiltration (12.5 ± 1.82% and 19.6 ± 1.96%, p=0.027). Immunohistochemistry revealed a significant decrease in the expression of inflammatory, apoptotic, adipogenic and muscular atrophy genes at both the 1 week and 6 weeks time points, but not at 12 weeks in the KO group compared to the WT group. This was confirmed by histology. Our study is the first to show that knocking out PARP-1 leads to decreased loss of muscle architecture, early inflammation, fatty infiltration and atrophy after combined tenotomy and neurectomy of the rotator cuff muscle. Although the macroscopic muscles reaction to injury is similar in the first 6 weeks, its ability to regenerate is much greater in the PARP-1 group leading to a near normalization of the muscle substance and muscle weight, less retraction, and less fatty infiltration after 12 weeks

The practice of rotator cuff repair rapidly moves towards arthroscopic techniques especially for single tendon tears. Although the clinical results are relatively well established, the structural results of open rotator cuff repair and their correlation with the clinical outcome are poorly known. In order to provide a baseline for future studies using other repair techniques, this study was carried out to assess the structural changes in the repaired musculotendinous unit and to correlate these findings with the clinical outcome after rupture and repair of an isolated full thickness single tendon tear of the rotator cuff. To prospectively assess the quality of an open rotator cuff tendon repair technique, to determine how structural changes of rotator cuff muscles are affected by tendon repair, and to correlate the structural results of MRI studies with the clinical outcome. Direct open repair of a complete, isolated single tendon tear of the rotator cuff resulted in significant improvement in function and objective evidence of tendon healing on MRI. Successful direct repair was not associated with recovery of preoperative muscular atrophy or fatty infiltration when comparing Goutallier stage fatty infiltration between pre and postoperative patients. The age and gender-adjusted Constant score increased significantly from an average of 63.9% preoperatively to 94.5% postoperatively (p< 0.0001). Pain improved significantly from 6.8 points to 13.2 points on a visual analogue scale. (p< 0.0001). Activities of daily living increased from 11.2 points preoperatively to 17.9 points postoperatively (p< 0.0001). Anterior elevation, abduction and internal rotation improved significantly for subscapularis and supraspinatus repairs, but there was no significant change in external rotation. The overall rerupture rate was 12.5%. There was no significant improvement or recovery of muscular atrophy or fatty infiltration after tendon repair. Thirty-two consecutive open repairs of a single tendon tear of the rotator cuff were analysed. The supraspinatus tendon was involved in twenty-two patients and the subscapularis tendon in ten patients. All tendons were repaired using an open technique, a modified Mason-Allen suturing technique, and transosseus fixation with non-absorbable suture material knotted over a bone augmentation device

Objectives. Our objectives were to describe the therapeutic aspects and assess the prognosis of chronic osteomyelitis in children. Materials and methods. We made a retrospective study from January 2007 to December 2016. The study concerned children from 0 to15 years, treated for chronic osteomyelitis and monitored in the pediatric surgery department of the teaching hospital Gabriel Toure, Bamako (Mali). The other types of bone infections, osteitis and bone tumors were not included in the study. In 10 years we received and treated 215 children with chronic osteomyelitis. This represented 3.56% of all the hospitalizations. The mean age was 8.8 (± 6.67) years with extremes of 28 days and 15 years. The patients were first seen by the traditional healer in 165 (76.7%) cases. The sex ratio was 1.26. The major clinical feature was local swelling associated with pain in 110 cases (51.2%). In 135 cases (62.8%) the staphylococcus aureus was found in direct examination or culture. After a year we performed a functional and morphological assessment according to the method of DIMEGLIO. Results. Surgical treatment was performed in all patients. The average delay of stay in hospital was 4.95 ± 4.57 weeks, with extremes of 2 and 12 weeks. The means follow-up was 13 months with extremes of 3 and 20 months. Good results were found in 115 patients, fair in 60 (40 in keloid knee valgus to 11 ° in 10, muscular atrophy 10), bad in 40 (shortening member in 25 non-union in 10 valgus to 18 ° in 5) According Dimeglio score. There was no significant association between the time of consultation, prior treatment received, the surgical technique and the occurrence of complications (p> 0.05). Conclusion. The management of the chronic osteomyelitis is well codified. The functional prognosis is dependent on an early care and sequels can be dramatic in children of school age. Keywords. Chronic osteomyelitis, Treatment, Prognosis, Children

Subscapularis tenotomy (SST) has been the preferred approach for shoulder arthroplasty for decades but recent controversy has propelled lesser tuberosity osteotomy (LTO) as a potential alternative. Early work by Gerber suggested improved healing and better outcomes with LTO although subscapularis muscular atrophy occurred in this group as well with unknown long-term implications. However, we previously performed a biomechanical study showing that some of the poor results following tenotomy may have been due to historic non-anatomic repair techniques. Surgical technique is critical to allow anatomic healing – this is true of both SST or LTO techniques. A recent meta-analysis of biomechanical cadaveric studies showed that LTO was stronger to SST at “time-zero” with respect to load to failure but there were no significant differences in cyclic displacement. A recent study evaluated neurodiagnostic, functional, and radiographic outcomes in 30 patients with shoulder arthroplasty who had SST. The authors found that the EMG findings were normal in 15 patients but abnormal in the other 15 and that these abnormalities occurred in 5 muscle groups (not just the subscapularis). In another study, patient outcomes were inferior in those patients who had documented subscapularis dysfunction following SST compared to patients who had LTO (none of whom had subscap dysfunction). The literature is not clear, however, on ultimate outcomes based on subscapularis dysfunction post-arthroplasty with some studies showing no difference and others showing significant differences

We have treated 101 patients with scoliosis secondary to muscular dystrophy over a 13-year period; 64 had Duchenne’s muscular dystrophy, 33 spinal muscular atrophy and four congenital muscular dystrophy. The patients underwent a modified Luque (87) or Harrington-Luque instrumentation (14) combined with a limited Moe fusion in all except 27 cases. A mean of 13 levels was instrumented. The mean preoperative sitting Cobb angle was 84° (10 to 150) and the mean postoperative angle 40° (52% correction). Most patients (96%) were able to discard their braces and there was a high level of patient satisfaction (89.6%). Less correction was seen for severe curves, and there was a greater recurrence of postoperative pelvic tilt in those patients not instrumented to the sacrum. Although the incidence of minor or temporary complications was high, these occurred chiefly in the early high-risk patients with very severe curves and considerable pre-existing immobility

Objective: This study evaluates the use of the Isola Growing Rod System for scoliosis in a group of skeletally immature patients with predominantly neuromuscular disorders. Our series of patients was unsuitable for definitive anterior and posterior fusion due to poor respiratory function. Design: We reviewed the case notes and x-rays of eighteen patients who had undergone instrumentation with the Isola Growing Rod System. The Cobb angles on the pre-operative and subsequent post-operative X-rays were measured. Complications were noted. Subjects: Eighteen children with scoliosis. Outcome measures: Curve correction and complications. Results: Two patients had idiopathic scoliosis, four patients had spinal muscular atrophy with the remainder having varied diagnoses. Eight patients were fully ambulant, the remainder being wheelchair bound pre-operatively. The average age at rod insertion was eight years, three months. The average number of lengthenings was four. The mean pre-operative Cobb angle was 76 degrees, the mean most recent Cobb angle 28 degrees. One patient required definitive fusion. Two patients required revision procedures due to implant failure. Conclusion: Our study shows that progressive scoliosis associated with neuromuscular disorder can be controlled with the growing rod system, thus avoiding both anterior release and the ‘crankshaft’ phenomenon, until the optimal time for definitive spinal fusion

Aims: To find a long-term effect of posterolateral fusion for isthmic spondylolysis and spondylolisthesis on lumbar spine. Methods: A posterolateral fusion was performed on 56 patients (30 females, 26 males) with an average age of 16 (range 11 to 20) years. A clinical and MRI examination was performed on these patients on average 18 years later (range 11 to 25 years). The size of the spinal canal were assessed. Disc space, degeneration and protrusions were evaluated. Bone marrow changes (Modic I & II), facet joint degeneration and the state of the spinal muscles were assessed. Results: In MR images, none of the patients had lumbar spinal stenosis. In contrast, the spinal canal was wide in the level spondylolysis and spondylolisthesis. Narrowing of the neural foramina was noted in 13 (23%) patients. This was associated usually in severe slip (> 50%) and was noted always at the L5-S1 level. Of the studied 332 intervertebral discs 56 (17%) were speckled and 57 (17%) were black and 76 (23%) narrowed. Most commonly speckled/black and narrowed disc was found in the two lowest lumbar levels. Only one patient, 41-year-old female, had prolapse. Modic I and II changes were noted in 7 (2%) and 9 (3%) intervertebral disc levels, respectively. Degenerative-like facet joint hypertrophy was noted in 47 (48%) of the studied levels. Of the patients, seven (12%) had muscular atrophy. Conclusions: Stenosis of neural foramina may be associated to severe spondyolisthesis. Degenerative changes were found most commonly found in the level of the spondylolysis and spondylolisthesis and above fusion level. Bone marrow changes associated with disc degeneration were rare

Objective: To evaluate per-operative and postoperative complications following surgical correction of neuromuscular scoliosis and assess the amount of radiological correction of Cobb’s angle and pelvic obliquity angle. Design: Retrospective study of 25 consecutive patients with neuromuscular scoliosis (10 Duchenne Muscular Dystrophy, 8 Cerebral palsy, 2 Neurofibromatosis, and 1 each of Spinal Muscular Atrophy, Friedrich’s Ataxia, Spina Bifida, Rett’s Syndrome and incarcerated hemi-vertibrae) who had surgical correction for their spinal disorders between 1999 and 2002. Background: Aims of surgical correction in neuromuscular scoliosis include improving sitting ability, cardio-respiratory function and cosmesis. Patients studied underwent a combination of anterior release, posterior spinal fusion and pelvic fixation. Due to the length and complexity of procedures many of these patients need two stage surgical procedures on two separate occasions to achieve this goal. However our practice is performing these two stage procedures in one sitting with post operative ITU support. Results: Patients were aged between 4 and 16 years of age with mean preoperative Cobb angles of 75.9° and pelvic obliquity of 18°. A mean correction of 55.8° of Cobb angle and 12° of Pelvic obliquity were achieved. We had mean operating time of 413 minutes with a mean blood loss of 50.2 ml/ Kg body weight. All patients were admitted to ITU post operatively with a mean stay in ITU of 5.8 days. All patients achieved clinical and radiological spinal fusion. Instrumentation had to be removed from one patient after fusion because of persistent infection. Conclusion: Surgery for Neuromuscular Scoliosis is time consuming and complicated. With anticipation of blood loss, complications and ITU support, we can successfully perform the two-stage procedure in one sitting

Aims: Muscle fat degeneration and fibrosis following long time denervation is today the main cause of poor functional recovery after peripheral nerve surgery especially for reconstruction of proximally located lesions of median and ulnar nerves such as those at brachial plexus level. External electro-stimulation is actually one possible way to avoid muscular atrophy and degeneration and is frequently used in the post-operative of patients with neurological palsy. A new approach that has been proposed to prevent denervation-related muscle atrophy is sensory protection performed by direct neurotization of a denervated muscle with a branch of a sensitive nerve passing nearby. The aim of this study was to study the effectiveness of sensory protection on denervated muscles as a technique to avoid their atrophic process. Methods: In four groups of rats (A,B,C and D), the median nerves were transected at right and left forearm. In group A,B and C, denervated muscles were “reinnervated” with a sensory nerve (sensory protection). Animals of group A was sacrificed after six months for a morphologic study of muscles. Animals of groups B and C were reinnervated after six months either keeping sensory protection (group B) or removing it (group C) and sacrificed after one year. Group D (without sensory protection) was reinnervated after six months and sacrificed after one year (control group). Muscle histology was performed on all samples. Functional comparison of different group was perfor-mend by means of the grasping test. Results: Histological analysis showed that sensory protection led to a better muscular trophism in all experimental groups. Also the functional testing showed better performances in sensory protected animals and especially in group C (de-protected before re-innervation) compared with group B (not de-protected before re-innervation) and D (control). Conclusions: Initial data analisys obtained in this study showed that sensory protection is effective in reducing denervation-related muscle atrophy. De-protection of the muscle before its reinnervation is also important to ameliorate post-surgical functional recovery. A new trial will be carried out with a larger number of cases in order to confirm these results which could have important applications in the clinical perspective

Purpose of the study: We noted that four postoperative compartment syndromes observed in our department occurred in short stature patients with micromely secondary to chondrodysplasia. This complication occurred in young subjects with significant muscular development (two with intensive sports practice) whose muscle hypertrophy appeared exaggerated by the short length of the limbs. Material and methods: The first case concerned a young man with pycnodysostosis who developed a tibial compartment syndrome after prolonged ventral decubitus subsequent to spinal surgery. No surgical procedure had been performed on the lower limb. The complication was probably triggered by simple compression of the proximal portion of the limb leading to elevated venous pressure and rapid self-aggravating muscular atrophy in a small aponeurotic compartment. The second case was a female patient with metaphyseal dysplasia who during adolescence had undergone corrective bilateral tibial osteotomy to align the lower limbs. Preventive aponeurotomy had been performed which left on one side a disgraceful muscle herniation. Aponeurotic plasty had been performed in adulthood to reconstitute the tibial compartment. A compartment syndrome developed in the immediate postoperative period. The third case was a young adult with hypochondroplasia who developed bilateral crural compartment syndrome 24 hours after femoral osteotomy with implantation of an external fixator for lower limb lengthening. Results: Sequelae were observed in only one of these four cases of compartment syndrome (patient n° 1). Extensive necrosis of the tibialis anterior was observed due to late diagnosis, leading to legal implications and a court suit. Emergency treatment was given in the three other cases and no sequelae were observed. Discussion: These patients with chondrodysplasia and micromely appeared to be predisposed to postoperative compartment syndrome because of the relative muscle hypertrophy and the narrow muscle compartments. The risk of this complication in small stature patients warrants the use of preventive aponeurotomy during orthopedic procedures involving the limbs and implies rigorous postoperative surveillance for early detection. Systematic aponeurotomies should be extended over the entire length of the muscle compartment involved in order to achieve effective decompression and avoid disgraceful localized muscle herniation

Aim: The aim of the study is to evaluate the results of the use of the Rotating Knee Endo Model not only as a revision implant, but as a primary choice too. Method: It is a retrospective study of 73 patients who underwent primary total knee replacement (TKR) in 96 knees, during 1990–2001. The mean follow up is 5.6 years. The average age of the patients was 70.6 years (38 – 87 years), and 79.6 % were female. The indications for surgery were osteoarthritis in 66 knees, rheumatoid arthritis (R.A.) in 10 knees and aseptic osteonecrosis of femoral condyles in 3 knees. Seventy-nine prosthesis were examined both clinically and with plain films. Fifteen patients died in the mean time and 2 others could not participate in the follow up. The pre- and post-operative evaluation based on the «The Hospital for Special Surgery (HSS) knee rating scale». Results: Fifty-eight knees were rated as excellent, 13 knees were rated as good and 8 knees were rated as fair. Deep venous thrombosis occured in 3 patients, non-fatal pulmonary embolism in 2, and 3 patients demonstrated superficial wound infection cured with oral antibiotic administration. One patient suffered dislocation of the apparatus, which required revision of the femoral component. The tibial insertion of the patellar tendon of one patient detached, which was reattached. Ectopic ossification was present in another patient 4 months after surgery and excised in a second stage, deep infection in 1 patient with history of osteomyelitis silent for 10 years, who eventually underwent an above-knee amputation. Conclusions: The Rotating Knee Endo Model allows axial correction of the extremity, stabilization of the joint, useful range of motion and pain relief while the infection rate is considered low. So the prosthesis could be a good alternative not only in revision procedures but in primary TKA in cases of serious axial deformity and in rheumatoid knees with instability and muscular atrophy as well

Introduction: Pathological conditions, which determine human atrophy, are numerouses and heterogeneous. Experimental studies prove that these different pathological conditions use common enzymatic pathways leading muscle atrophy. In every catabolic conditions where there is proteolyses’s increase, this one happens in association with up-regulation of two specific genes of skeletal muscle atrophy. These genes, MuRf1 (muscle ring finger-1) and MAFbx (muscle atrophy F-box), encode ubiquitin ligases. These ligases bind and mediate ubiquitination of myofibrillar proteins for subsequent degradation during muscle atrophy. The aim of our study is to obtain a better understanding of human muscle physiopathology in atrophy by use of histochemistry and immunolocalisation of MuRF-1 and MAFbx. Patients and Methods: 15 patients, amputated at third distal or proximal leg because of different acute or chronic pathology, were divided in two group. Group A: 12 elderly patients (mean age 72 years) amputated for vascular diseases (8) and complication of a diabetic foot (4). Group B: 3 young patients involved in car accident (mean age 25) amputated for limb’s acute arterial insufficiency. Gastrocnemius muscle biopsy specimens were obtained for all the patients, after that the informed consent was obtain, for histochemical (haematossilineosin), and immunohistochemical (anti- MuRf1, anti- MAFbx) analysis. Results: Histochemistry: Group A: skeletal muscle showed a decrease in fiber size in cross-sectional area and fiber length with adipose tissue. Group B: light skeletal muscle structural alteration. Immuno-histochemistry: in group A, in muscular drawings, polyclonal antibodies direct against MuRf1 and MAFbx had stained muscle biopsy specimens. Muscle fiber cells showed MuRf1 and MAFbx subsarcolemmatic immunoreactivity and weakly immunoreactivity of the extracellular matrix. We noticed no positivity to anti- MuRf1 and anti- MAFbx less in sections from group B muscle biopsy specimens and in sections in which were present tissue muscle degeneration with replacement of adipose tissue. Conclusion: The pathological results supported the concept that MuRf1 and MAFbx appeared to be regulatory peptide in cellular pathology that conduce to muscular atrophy. Our data support the hypothesis that different pathological conditions use common enzymatic pathways leading muscle atrophy. The demonstration that the muscle-specific proteins MAFbx and MuRF1 are upregulated in multiple pathological conditions of skeletal muscle atrophy it is critical to continue studying the cellular pathways to discover promising targets for the development of effective new treatments for skeletal muscle disease

Aims

Rotator cuff (RC) injuries are characterized by tendon rupture, muscle atrophy, retraction, and fatty infiltration, which increase injury severity and jeopardize adequate tendon repair. Epigenetic drugs, such as histone deacetylase inhibitors (HDACis), possess the capacity to redefine the molecular signature of cells, and they may have the potential to inhibit the transformation of the fibro-adipogenic progenitors (FAPs) within the skeletal muscle into adipocyte-like cells, concurrently enhancing the myogenic potential of the satellite cells.

Introduction and Aims: Surgical correction of pelvic obliquity is an important component of spinal instrumentation for neuromuscular scoliosis, though instrumentation to the pelvis has high reported complication rates. This study evaluates the results of pelvic fixation during surgical correction of neuromuscular scoliosis in a consecutive series of 62 children and adolescents. Method: A retrospective chart and radiographic review of 62 consecutive patients treated with spinal fusions to the pelvis as treatment for neuromuscular scoliosis was performed. Follow-up ranged from two to seven years. Diagnoses included cerebral palsy (36 patients), muscular dystrophy (16 patients), myelomeningocele (three patients), spinal muscular atrophy (three patients) and other disorders (four patients). Mean age at surgery was 13.5 years. Pelvic fixation techniques used included Luque-Galveston or iliosacral screw fixation. Correction of deformity in each patient was assessed with Cobb angle measurements of scoliosis, thoracic kyphosis, and lumbar lordosis. Pelvic obliquity and coronal decompensation was also assessed. Results: The Luque-Galveston spinal instrumentation technique was used in 54 patients and iliosacral screw fixation was used in eight patients. Seventeen patients had an additional anterior release and fusion without instrumentation. The mean Cobb angle measured 73 degrees pre-operatively and 31 degrees (mean correction 59%) post-operatively. The mean Cobb angle on latest follow-up was 33 degrees (loss of correction 12%). Thoracic kyphosis remained essentially unchanged, as did lumbar lordosis (56 pre-op and 61 on follow-up). Pelvic obliquity corrected from a mean of 16 degrees pre-operatively to eight degrees on most recent follow-up. Mean pre-operative coronal decompensation measured 135mm, and follow-up decompensation measured 46mm. Eleven patients with Galveston fixation exhibited the ‘windshield-wiper’ sign, with a radiolucency of 2mm or more, though most were asymptomatic. Wound infection was observed in 6% (3/54) of the patients who underwent Galveston instrumentation and 50% (4/8) who had iliosacral screws. In patients treated with Galveston fixation, three had symptomatic prominant hardware and one had hardware breakage for an overall mechanical failure rate of 7% (4/54). In contrast, two patients with iliosacral screws had construct breakage and pseudoarthrosis for a mechanical failure rate of 25% (2/8), though the numbers in the iliosacral screw group are small. Conclusions: In this series, Galveston pelvic fixation during spinal instrumentation treatment of neuromuscular scoliosis was associated with satisfactory results and with less complications than generally reported in the literature. This technique is recommended as the preferred method for pelvic fixation in severe neuromuscular scoliosis associated with pelvic obliquity

Previous studies in animal models of limb lengthening have shown a wide spectrum of histopathological changes during distraction phase. Much less is known about the structural response of muscle during the consolidation phase. This study aimed to observe and score changes in morphology, weight, length and maximal perimeter of gastrocnemius during the distraction and consolidation phases. Thirty two immature New Zealand white rabbits were divided into two equal groups: lengthening and sham. In each group, half of the rabbits were killed at the end of lengthening and half 5 weeks later. A bilateral external fixator was applied to tibia and a mid-diaphysis osteotomy performed. The lengthening rate was 0.4 mm twice daily with an initial delay of 7 days. 30% lengthening was achieved in 4 to 5 weeks. After sacrifice, the whole gastrocnemius was taken from its attachments. Its weight, length and maximal perimeter were measured. At the middle of belly, a specimen 0.5cm in length was taken from the medial gastrocnemius for H& E and Masson trichrome staining. A scoring system was used to achieve a semi-quantitative analysis of the histopathological changes in gastrocnemius. No abnormal changes were observed in the sham side. Degeneration, atrophy and endomysial fibrosis were all found in the lengthened side. The scores of histopathological changes between the end of lengthening and 5 weeks later showed a decreasing trend, but no significant difference. The weight and perimeter decreased and length increased in the lengthening side. The weight, perimeter and length of gastrocnemius in both lengthening and control sides increased at 5 weeks after the end of lengthening. Muscular atrophy, as shown by a decrease in weight, perimeter and muscle fibre size, occurred and might be due to the combined effect of continuous muscle stretching and inactivity. Continuous stretching of muscles beyond a certain point produced damage. Some studies reported that damage to muscle fibres, which has been shown as degeneration and fibrosis in this study, can release and activate satellite cells. As myoblast precursors, satellite cells become myoblasts, which proliferate and fuse into the microlesioned areas, regenerating and repairing myofibrils. Also, the immature muscles have more active abilities of proliferation, regeneration, growth and healing. In this study gastrocnemius growth shown by an increase in weight, perimeter and length occurred during the consolidation phase of 5 weeks. The mean scores of histopathological changes in gastrocnemius decreased during consolidation period, indicating some recovery of damage to muscle. It is not clear whether this reflects a normal response, which would have been seen in other studies had samples been taken later or whether it is a unique response of the immature animal

Aims

The first aim of this study was to evaluate whether preoperative renal function is associated with postoperative changes in whole blood levels of metal ions in patients who have undergone a Birmingham Hip Resurfacing (BHR) arthroplasty with a metal-on-metal bearing. The second aim was to evaluate whether exposure to increased cobalt (Co) and chromium (Cr) levels for ten years adversely affected renal function.

Aims

The aim of this study was to develop and internally validate a prognostic nomogram to predict the probability of gaining a functional range of motion (ROM ≥ 120°) after open arthrolysis of the elbow in patients with post-traumatic stiffness of the elbow.

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.

Aims

The aim of this study was to describe a quantitative 3D CT method to measure rotator cuff muscle volume, atrophy, and balance in healthy controls and in three pathological shoulder cohorts.

Aims

Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.

Aims

Postoperative rehabilitation regimens following ankle arthrodesis vary considerably. A systematic review was conducted to determine the evidence for weightbearing recommendations following ankle arthrodesis, and to compare outcomes between different regimens.

Aims

The aim of this study was to compare the outcomes of surgery using growing rods in patients with severe versus moderate early-onset scoliosis (EOS).

Objectives

Osteoporosis is a chronic disease. The aim of this study was to identify key genes in osteoporosis.

Our aim was to review the efficacy of the wound vacuum-assisted closure (VAC) system in the treatment of deep infection after extensive instrumentation and fusion for spinal deformity in children and adolescents.

A total of 14 patients with early deep spinal infection were treated using this technique. Of these, 12 had neuromuscular or syndromic problems. Clinical and laboratory data were reviewed. The mean follow-up was 44 months (24 to 72). All wounds healed. Two patients required plastic surgery to speed up the process. In no patient was the hardware removed and there was no loss of correction or recurrent infection.

We believe that the wound VAC system is a useful tool in the armamentarium of the spinal surgeon dealing with patients susceptible to wound infections, especially those with neuromuscular diseases. It allows for the retention of the instrumentation and the maintenance of spinal correction. It is reliable and easy to use.

In this case study, we describe the clinical presentation and treatment of 36 patients with periosteal chondrosarcoma collected over a 59-year period by the archive of the Netherlands Committee on Bone Tumours. The demographics, clinical presentation, radiological features, treatment and follow-up are presented with the size, location, the histological grading of the tumour and the survival.

We found a slight predominance of men (61%), and a predilection for the distal femur (33%) and proximal humerus (33%). The metaphysis was the most common site (47%) and the most common presentation was with pain (44%). Half the tumours were classified histologically as grade 1. Pulmonary metastases were reported in one patient after an intra-lesional resection. A second patient died from local recurrence and possible pulmonary and skin metastases after an incomplete resection.

It is clearly important to make the diagnosis appropriately because an incomplete resection may result in local recurrence and metastatic spread. Staging for metastatic disease is recommended in grade II or III lesions.

These patients should be managed with a contrast-enhanced MRI of the tumour and histological confirmation by biopsy, followed by en-bloc excision.

Cite this article: Bone Joint J 2014;96-B:823–8.

We determined the frequency, rate and extent of development of scoliosis (coronal plane deformity) in wheelchair-dependent patients with Duchenne muscular dystrophy (DMD) who were not receiving steroid treatment. We also assessed kyphosis and lordosis (sagittal plane deformity). The extent of scoliosis was assessed on sitting anteroposterior (AP) spinal radiographs in 88 consecutive non-ambulatory patients with DMD. Radiographs were studied from the time the patients became wheelchair-dependent until the time of spinal fusion, or the latest assessment if surgery was not undertaken. Progression was estimated using a longitudinal mixed-model regression analysis to handle repeated measurements.

Scoliosis ≥ 10° occurred in 85 of 88 patients (97%), ≥ 20° in 78 of 88 (89%) and ≥ 30° in 66 of 88 patients (75%). The fitted longitudinal model revealed that time in a wheelchair was a highly significant predictor of the magnitude of the curve, independent of the age of the patient (p <  0.001). Scoliosis developed in virtually all DMD patients not receiving steroids once they became wheelchair-dependent, and the degree of deformity deteriorated over time.

In general, scoliosis increased at a constant rate, beginning at the time of wheelchair-dependency (p < 0.001). In some there was no scoliosis for as long as three years after dependency, but scoliosis then developed and increased at a constant rate. Some patients showed a rapid increase in the rate of progression of the curve after a few years – the clinical phenomenon of a rapidly collapsing curve over a few months.

A sagittal plane kyphotic deformity was seen in 37 of 60 patients (62%) with appropriate radiographs, with 23 (38%) showing lumbar lordosis (16 (27%) abnormal and seven (11%) normal).

This study provides a baseline to assess the effects of steroids and other forms of treatment on the natural history of scoliosis in patients with DMD, and an approach to assessing spinal deformity in the coronal and sagittal planes in wheelchair-dependent patients with other neuromuscular disorders.

Cite this article: Bone Joint J 2014;96-B:100–5.

Haematogenous osteomyelitis in newborns and infants usually occurs in the long bones and is rare in the short or flat bones. We present two neonates with osteomyelitis of the upper cervical spine affecting the second to fourth cervical vertebrae and the first and second cervical vertebrae, respectively. Despite some delay in diagnosis, both responded successfully to conservative treatment with antibiotics, a cervical collar and needle puncture. The latest follow-up at six and seven years, respectively, showed no persistent neurological deficit and a normal diameter of the cervical spinal canal on MRI.

Scapulothoracic fusion (STF) for painful winging of the scapula in neuromuscular disorders can provide effective pain relief and functional improvement, but there is little information comparing outcomes between patients with dystrophic and non-dystrophic conditions. We performed a retrospective review of 42 STFs in 34 patients with dystrophic and non-dystrophic conditions using a multifilament trans-scapular, subcostal cable technique supported by a dorsal one-third semi-tubular plate. There were 16 males and 18 females with a mean age of 30 years (15 to 75) and a mean follow-up of 5.0 years (2.0 to 10.6). The mean Oxford shoulder score improved from 20 (4 to 39) to 31 (4 to 48). Patients with non-dystrophic conditions had lower overall functional scores but achieved greater improvements following STF. The mean active forward elevation increased from 59° (20° to 90°) to 97° (30° to 150°), and abduction from 51° (10° to 90°) to 83° (30° to 130°) with a greater range of movement achieved in the dystrophic group. Revision fusion for nonunion was undertaken in five patients at a mean time of 17 months (7 to 31) and two required revision for fracture. There were three pneumothoraces, two rib fractures, three pleural effusions and six nonunions. The main risk factors for nonunion were smoking, age and previous shoulder girdle surgery.

STF is a salvage procedure that can provide good patient satisfaction in 82% of patients with both dystrophic and non-dystrophic pathologies, but there was a relatively high failure rate (26%) when poor outcomes were analysed. Overall function was better in patients with dystrophic conditions which correlated with better range of movement; however, patients with non-dystrophic conditions achieved greater functional improvement.

We retrospectively examined the prevalence and natural history of asymptomatic lumbar canal stenosis in patients treated surgically for cervical compressive myelopathy in order to assess the influence of latent lumbar canal stenosis on the recovery after surgery. Of 214 patients who had undergone cervical laminoplasty for cervical myelopathy, we identified 69 (32%) with myelographically documented lumbar canal stenosis. Of these, 28 (13%) patients with symptomatic lumbar canal stenosis underwent simultaneous cervical and lumbar decompression. Of the remaining 41 (19%) patients with asymptomatic lumbar canal stenosis who underwent only cervical surgery, 39 were followed up for ≥ 1 year (mean 4.9 years (1 to 12)) and were included in the analysis (study group). Patients without myelographic evidence of lumbar canal stenosis, who had been followed up for ≥ 1 year after the cervical surgery, served as controls (135 patients; mean follow-up period 6.5 years (1 to 17)). Among the 39 patients with asymptomatic lumbar canal stenosis, seven had lumbar-related leg symptoms after the cervical surgery.

Kaplan–Meier analysis showed that 89.6% (95% confidence interval (CI) 75.3 to 96.0) and 76.7% (95% CI 53.7 to 90.3) of the patients with asymptomatic lumbar canal stenosis were free from leg symptoms for three and five years, respectively. There were no significant differences between the study and control groups in the recovery rate measured by the Japanese Orthopaedic Association score or improvement in the Nurick score at one year after surgery or at the final follow-up.

These results suggest that latent lumbar canal stenosis does not influence recovery following surgery for cervical myelopathy; moreover, prophylactic lumbar decompression does not appear to be warranted as a routine procedure for coexistent asymptomatic lumbar canal stenosis in patients with cervical myelopathy, when planning cervical surgery.