A Scoping Review of the Recent Clinical Practice Regarding the Evaluation of Bone Mineral Density in Children and Adolescents with Neuromuscular Diseases.

Introduction: Neuromuscular Diseases (NMD) are associated with decreased bone strength due to altered muscle-bone interaction. However, the evaluation of bone quality remains a certain challenge in these patients. The purpose of this scoping review is to investigate the recent literature regarding the assessment of Bone Mineral Density (BMD) in this population. Methods: An electronic search of the PubMed and Scopus database was performed considering studies published in the English literature after 2007 that evaluated BMD in pediatric and adolescent patients with NMD. We excluded studies that evaluated patients > 20 years, studies not involving humans, and studies investigating bone mineral density in various pediatric conditions, but without specific data on NMD. Results: Overall, 19 studies were included that evaluated BMD in 1983 patients with NMD. Duchenne Muscular Dystrophy was the most widely studied disease (n = 11 studies). Dual energy X-ray absorptiometry (DEXA) was the most common diagnostic modality for BMD evaluation, while the most frequent site for BMD measurement was the lumbar spine (89.4%, n = 17 studies), followed by total body BMD (68.4%, n = 13 studies). Low BMD in children with NMD was demonstrated in all studies, especially after loss of ambulation. Moreover, a positive correlation between lower BMD and older age was shown. Conclusions: BMD evaluation in NMD remains a clinical challenge, as indicated by the high heterogeneity regarding the optimal site and technique for the evaluation of bone quality in these patients. Although DXA is currently the diagnostic modality of choice, a consensus regarding the optimal site for BMD measurement, and the adjustment method for its obtained measurements for parameters such as age and height is needed.

Validity and reliability of the Greek version of the American Shoulder and Elbow Surgeons Standardized Shoulder Assessment Form.

BACKGROUND: The aim of our study is to prove the validity and reliability of the Greek translated version of the self-report section of the American Shoulder and Elbow Surgeons (ASES) questionnaire. METHODS: A total of 108 patients with various shoulder disorders were evaluated at two different orthopedic centers. All patients answered the Greek ASES questionnaire as well as the previously validated Greek version of the Disability Arm Shoulder and Hand score. Three days after the first evaluation, a subgroup of 40 individuals was randomly selected to complete again the Greek ASES text to prove its reliability, after the test-retest procedure. Reliability was tested with Cronbach's alpha, stability by calculating the intraclass correlation coefficient and by Blant Altman plot and structural validity with the confirmatory factor analysis. RESULTS: The internal consistency of the ASES functional score and the ASES total score was 0.925 and 0.750 respectively. The intraclass correlation coefficient between initial assessment and reassessment of the ASES functional and total score was 0.951 and 0.938 (P < .001), respectively. The correlation coefficients correlation between the ASES functional and total scores with DASH total score were -0.881 and -0.759 (P < .001), respectively. CONCLUSIONS: The Greek ASES version proved to be equivalent to the English original version in evaluating different shoulder disorders in the Greek population.

Retraction Note: Rare causes of scoliosis and spine deformity: experience and particular features.

[This retracts the article DOI: 10.1186/1748-7161-2-15.].

Reconstruction of a Neglected Tibial Plateau Fracture Malunion with an Open-Book Osteotomy: A Case Report.

CASE: Surgical management of tibial plateau fractures can be challenging, and complications can be devastating. We report the case of a lateral tibial plateau fracture malunion with an associated intra-articular depression, which we treated with an open-book osteotomy, obviating the need for an early corrective osteotomy or knee arthroplasty. CONCLUSION: A tibial plateau fracture malunion can be reconstructed. The surgical team should give priority to salvage options, which can obviate or delay the need for and the implications of an early total knee arthroplasty, especially in a young and active patient.

When and how to operate on spondylodiscitis: a report of 13 patients.

PURPOSE: Conflicting reports exist regarding the surgical indications, timing, approach, staged or not operation, and spinal instrumentation for patients with spondylodiscitis. Therefore, we performed this study to evaluate the outcome of a series of patients with spondylodiscitis aiming to answer when and how to operate on these patients. MATERIALS AND METHODS: We retrospectively studied the files of 153 patients with spondylodiscitis treated at our institution from 2002 to 2012. The approach included MR imaging of the infected spine, isolation of the pathogen with blood cultures and/or biopsy, and further conservative or surgical treatment. The mean follow-up was 6 years (range 1-13 years). We evaluated the indications, timing (when), and methods (how) for surgical treatment, and the clinical outcome of these patients. RESULTS: Orthopedic surgical treatment was necessary for 13 of the 153 patients (8.5 %). These were patients with low access to healthcare systems because of low socioeconomic status, third-country migrants, prisoners or intravenous drug use, patients in whom a bacterial isolate documentation was necessary, and patients with previous spinal operations. The most common pathogen was Mycobacterium tuberculosis. The surgical indications included deterioration of the neurological status (11 patients), need for bacterial isolate (10 patients), septicemia due to no response to antibiotics (five patients), and/or spinal instability (three patients). An anterior vertebral approach was more commonly used. Nine of the 13 patients had spinal instrumentation in the same setting. Improvement or recovery of the neurological status was observed postoperatively in all patients with preoperative neurological deficits. Postoperatively, two patients deceased from pulmonary infection and septicemia, and heart infarction. At the last follow-up, patients who were alive were asymptomatic; ten patients were neurologically intact, and one patient experienced paraparesis. Imaging showed spinal fusion, without evidence of recurrent spondylodiscitis. Complications related to the spinal instrumentation were not observed in the respective patients. CONCLUSIONS: Conservative treatment is the standard for spondylodiscitis. Physicians should be alert for Mycobacterium tuberculosis spondylitis because of the low access to healthcare systems of patients with low social and economic status. Surgical indications include obtaining tissue sample for diagnosis, occurrence or progression of neurological symptoms, failure of conservative treatment, large anterior abscesses, and very extensive disease. Thorough debridement of infected tissue and spinal stability is paramount. The anterior approach provides direct access and improved exposure to the most commonly affected part of the spine. Spinal instrumentation is generally recommended for optimum spinal stability and fusion, without any implant-related complications.

Rib hump deformity assessment using the rib index in adolescent idiopathic scoliotics treated with full screw or hybrid constructs: aetiological implications.

BACKGROUND: Review of literature reveals that in Idiopathic Scoliosis (IS) children, the post-operative rib hump (RH) correction using full transpedicular screw construct has never been compared to hybrid constructs, applying the Rib-Index (RI) method. Therefore the aim of this report is to study which of the above two constructs offers better postoperative Rib Hump Deformity (RHD) correction. METHODS: Twenty five patients with Adolescent Idiopathic Scoliosis (AIS) were operated using full pedicle screw construct or hybrid construct. Sixteen underwent full screw instrumentation (group A) and nine an hybrid one (group B). The median age for group A was 15 years and for group B 17.2 years. The RHD was assessed on the lateral spinal radiographs using the RI. The RI was calculated by the ratio of spine distances d1/d2, where d1 is the distance between the most extended point of the most extending rib contour and the posterior margin of the corresponding vertebra on the lateral scoliosis films and d2 is the distance from the least projected rib contour and the posterior margin of the same vertebra. Moreover the amount of RI correction was calculated by subtracting the post-operative RI from the pre-operative RI. RESULTS: Although within group A the RI correction was statistical significant (the pre-op RI was 1.93 and the post-op 1.37; p<0.001) and similarly in group B (the mean pre-op RI was 2.06 while the mean post-op 1.51; p=0.008), between group A and B the post-operative RI correction mean values were found to be no statistically significant, (p=0.803). CONCLUSION: Although the pre- and post-operative RI correction was statistically significant within each group, this did not happen post-operatively between the two groups. It appears that the RHD correction is not different, no matter what the spinal construct type was used. Provided that the full screw construct is powerful, the post-operative derotation and RHD correction was expected to be better than when an hybrid construct is applied, which is not the case in this study. It is therefore implied that the RHD results more likely from the asymmetric rib growth rather than from vertebral rotation, as it has been widely believed up to now. In 2013 Lykissas et al, reported that costoplasty combined with pedicle screws and vertebral derotation significantly improved RH deformity as opposed to pedicle screws and vertebral derotation alone. Another interesting implication is that the spinal deformity is the result of the thoracic asymmetry, implication in line with the late Prof. John Sevastikoglou's (Sevastik's) thoracospinal concept.

The rib index is not practically affected by the distance between the radiation source and the examined child.

BACKGROUND: All lateral spinal radiographs in idiopathic scoliosis (IS) show a Double Rib Contour Sign (DRCS) of the thoracic cage, a radiographic expression of the rib hump. The outline of the convex overlies the contour of the concave ribs. The rib index (RI) method was extracted from the DRCS to evaluate rib hump deformity in IS patients. The RI was calculated by the ratio of spine distances d1/d2 where d1 is the distance between the most extended point of the most extending rib contour and the posterior margin of the corresponding vertebra on the lateral scoliosis films, while d2 is the distance from the least projection rib contour and the posterior margin of the same vertebra, (Grivas et al 2002). In a symmetric thorax the "rib index" is 1. This report is the validity study of DRCS, ie how the rib index is affected by the distance between the radiation source and the irradiated child. METHODS: The American College of Radiology's (2009) guidelines for obtaining radiographs for scoliosis in children recommends for the scoliotic - films distance to be 1,80 meters. Normal values used for the transverse diameter of the ribcage in children aged 6-12 years were those reported by Grivas in 1988. RESULTS: Using the Euclidean geometry, it is shown that in a normal 12-year old child d1/d2 = 1.073 provided that the distance ΔZ ≈ 12cm (11,84) and EA = 180cm, with transverse ribcage diameter of the child 22 cm. CONCLUSIONS: This validity study demonstrates that the DRCS is substantially true and the RI is not practically affected by the distance between the radiation source and the irradiated child. The RI is valid and may be used to evaluate the effect of surgical or conservative treatment on the rib cage deformity (hump) in children with IS. It is noted that RI is a simple method and a safe reproducible way to assess the rib hump deformity based on lateral radiographs, without the need for any other special radiographs and exposure to additional radiation.

Reliability study for the Rib Index in chest radiographs of a control group.

BACKGROUND: The Rib Index, (RI), extracted from the double rib contour sign (DRCS) on lateral spinal radiographs to evaluate rib hump deformity, (RHD), in idiopathic scoliosis, (IS), patients, has been previously introduced. Although various papers using the RI have been published, no study on its reproducibility has been reported. The aim of this report is to estimate the variations of the RI in a number of a pair set of lateral chest radiographs (LCRs). The hypothesis was that the RI should have minimal variability for each subject having successive LCRs. METHODS: Seventy randomized patients who were treated in the hospital for lung diseases (mainly pneumonia or other communicable lung diseases), were initially included in the study. Each of these patients had two successive LCRs (named A and B group of radiographs) at the radiological department of the hospital, by the same technician, during the course of their treatment. The radiation source - patient distance was constant. LCRs obtained at an incorrect patient's position, or from patients who underwent a thoracic intervention and all LCRs with symmetric hemi-thoraces were excluded from the study. The LCRs of 49 patients were deemed suitable for inclusion in the study. The RI was calculated in both (A and B) LCRs of each patient. The statistical analysis included the following techniques: paired t-test, Pearson correlation coefficient and intra- and inter-observer error using the formula (SD/√2)/2, where SD is this of the differences of the two sets of measurement (As-Bs). The SPSS v16 statistical package was used. RESULTS: In the 49 pairs of LCRs there was no statistical difference of the RI, (paired t-test p< 0.314). The RI in the A and B group of LCRs was perfectly correlated (correlation coefficient = 0,924, p < 0.0001). The intra-observer error was 0.0080 while the inter-observer error 0.0213 in terms of 95% CI. CONCLUSION: The RI proves to be a reliable method to evaluate the thoracic deformity and the effect of surgical or non-operative treatment on the IS RHD. RI is a simple method, a safe reproducible way to assess the RHD based on lateral radiographs, without the need for any further special radiographs and exposure to additional radiation.

When and how to operate on thoracic and lumbar spine fractures?

PURPOSE: To discuss when and how to operate on thoracic and lumbar spine fractures. PATIENTS AND METHODS: We retrospectively studied 77 consecutive patients with thoracic and lumbar spine fractures treated from 2000 to 2011; 28 patients experienced high-energy spinal trauma and 49 low-energy spinal trauma. Mean follow-up was 5 years (1-11 years). Surgical treatment was done in 15 patients with neurological deficits, and in 16 neurologically intact patients with fractures-dislocations, burst fractures and fractures with marked deformity. Non-surgical treatment was done in 46 neurologically intact patients with simple fracture configurations. Clinical and imaging examination and the Oswestry Disability Index (O.D.I.) questionnaire were obtained. RESULTS: All patients treated surgically maintained spinal alignment; patients with long fusion maintained the best alignment; however, they experienced back stiffness and moderate low back pain. Patients with combined posterior fusion and kyphoplasty experienced earlier recovery and improved sagittal correction. Mean O.D.I. was 22.4 and 14.2% at 3 and 12 months postoperatively. Thirty six (78%) patients treated non-surgically were asymptomatic, 22 (48%) experienced mild residual kyphosis, 10 (22 %) developed marked deformity during their follow-up and were finally operated; mean O.D.I. was 28.6 and 12.1% at 3 and 12 months. No difference in O.D.I. was observed between patients who had surgical and non-surgical treatment. CONCLUSIONS: Progressive neurological deficits and/or mechanical instability of the spine are absolute indications for early surgical treatment. Younger patients with high-energy spinal trauma, unstable fractures and neurological deficits should be treated surgically in order to provide optimum conditions for neurologic recovery, early mobilization and possibly ambulation. Most cases can be adequately operated through a posterior only surgical approach; an anterior or combined approach is usually indicated for burst and thoracic spine fractures. Postoperative complications, more common infection and neurological deterioration may occur. Elderly, neurologically intact patients with low-energy, stable spinal fractures without marked spinal deformity may be successfully treated conservatively. Most of these patients will do well; however, follow-up for progressive posttraumatic deformity is required.

Severe anaemia and subcapital femur fracture in a patient with Left Ventricular Assist Device Heart Mate II: the cardiologist's management of this rare patient.

The use of Left Ventricular Assist Devices (LVADs) has increased over the last decade because of the lack of healthy donor hearts. In this report we describe for the first time a patient with an LVAD Heart Mate II (HM II) implanted 6 months before admission, who initially suffered from severe anaemia and later on underwent a successful bipolar hip replacement owing to subcapital fracture of the right femur. The patient was managed successfully by a team approach, which included a cardiologist, anaesthesiologist, orthopaedic surgeon, and LVAD technician.

Functional neuroanatomy of proprioception.

Proprioception is the sense of body position that is perceived both at the conscious and unconscious levels. Typically, it refers to two kinds of sensations: that of static limb position and of kinesthesia. Static position reflects the recognition of the orientation of the different body parts, whereas kinesthesia is the recognition of rates of movement. Proprioception is based on a multicomponent sensory system. There are various peripheral receptors that detect specific signals and major sensory afferent pathways that carry the information from the spinal cord up to the cortex. There are parallel pathways, some of which serve conscious proprioception, and others that serve subconscious proprioception. Conscious proprioception is relayed mostly by the dorsal column and in part by the spinocervical tract. Finally, the organ of perception for position sense is the sensory cortex of the brain. This review outlines the current understanding of these complex neural pathways, starting from the peripheral receptors and working up toward the center of perception, the brain.

Late postoperative infection following spinal instrumentation: stainless steel versus titanium implants.

Late postoperative infection following instrumented spinal surgery is a clinical entity that has emerged in recent years. The extended surface of the spinal instrumentation in combination with hematogenous seeding or intraoperative inoculation is the main predisposing factor. In order to investigate the contribution of the instrumentation material (stainless steel versus titanium implants) and mechanical loosening, two groups of patients are presented. The first group includes 50 idiopathic scoliotic patients who were treated with first-generation posterior stainless steel spinal segmental multihook instrumentation [Texas Scottish Rite Hospital (TSRH) instrumentation system], and the second group includes 45 similar patients who were treated with newer titanium implants (MOSS MIAMI, XIA, and CD). Follow-up ranged from 3 to 13 years. Six patients from the first group and one patient from the second group presented with late infections 1 to 7 years postoperatively. Common intraoperative findings were excessive inflammatory tissue and some degree of instrumentation loosening and corrosion (stainless steel). Removal of instrumentation in combination with appropriate antibiotics was an effective treatment. Further study with long-term follow-up is necessary in order to understand the exact incidence and pathology of such infections.

Rare causes of scoliosis and spine deformity: experience and particular features.

BACKGROUND: Spine deformity can be idiopathic (more than 80% of cases), neuromuscular, congenital or neurofibromatosis-related. However, there are many disorders that may also be involved. We present our experience treating patients with scoliosis or other spine deformities related to rare clinical entities. METHODS: A retrospective study of the records of a school-screening study in North-West Greece was performed, covering a 10-year period (1992-2002). The records were searched for patients with deformities related to rare disorders. These patients were reviewed as regards to characteristics of underlying disorder and spine deformity, treatment and results, complications, intraoperative and anaesthesiologic difficulties particular to each case. RESULTS: In 13 cases, the spine deformity presented in relation to rare disorders. The underlying disorder was rare neurological disease in 2 cases (Rett syndrome, progressive hemidystonia), muscular disorders (facioscapulohumeral muscular dystrophy, arthrogryposis) in 2 patients, osteogenesis imperfecta in 2 cases, Marfan syndrome, osteopetrosis tarda, spondyloepiphyseal dysplasia congenita, cleidocranial dysplasia and Noonan syndrome in 1 case each. In 2 cases scoliosis was related to other congenital anomalies (phocomelia, blindness). Nine of these patients were surgically treated. Surgery was avoided in 3 patients. CONCLUSION: This study illustrates the fact that different disorders are related with curves with different characteristics, different accompanying problems and possible complications. Investigation and understanding of the underlying pathology is an essential part of the clinical evaluation and preoperative work-up, as clinical experience at any specific center is limited.