This study summaries the current management of scoliosis in patients with Duchenne Muscular Dystrophy.A literature review of Medline was performed and the collected articles critically appraised. This literature is discussed to give an overview of the current management of scoliosis within Duchenne Muscular Dystrophy.Importantly, improvements in respiratory care, the use of steroids and improving surgical techniques have allowed patients to maintain quality of life and improved life expectancy in this patient group.
Children who suffer with cerebral palsy (CP) have a significant chance of developing scoliosis during their early years and adolescence. The behavior of this scoliosis is closely associated with the severity of the CP disability and unlike idiopathic scoliosis, it continues to progress beyond skeletal maturity. Conservative measures may slow the progression of the curve, however, surgery remains the only definitive management option. Advances in surgical technique over the last 50 years have provided methods to effectively treat the deformity while also reducing complication rates. The increased risk of surgical complications with these complex patients make decisions about treatment challenging, however with careful pre-operative optimization and post-operative care, surgery can offer a significant improvement in quality of life. This review discusses the development of scoliosis in CP patient, evaluates conservative and surgical treatment options and assesses post-operative outcome.
This study investigated changes in body composition in relation to training load determined using RPE and duration (sRPE), and its relationship with physical qualities over a preseason period. Sixteen professional academy players (age = 17.2 ± 0.7 years; stature = 179.9 ± 4.9 cm; body mass = 88.5 ± 10.1 kg) participated in the study. Body composition was assessed before and after each training phase and physical qualities assessed at the start and end of preseason. Across the whole preseason period, skinfold thickness, body fat percentage and fat mass were most likely lower (ES = -0.73 to -1.00), and fat free mass and lean mass were likely to most likely higher (ES = 0.31 to 0.40). Results indicated that the magnitude of change appeared phase-dependent (ES = -0.05 to -0.85) and demonstrated large individual variability. Changes in physical qualities ranged from unclear to most likely (ES = -0.50 to 0.64). Small to moderate correlations were observed between changes in body composition, and TL with changes in physical qualities. This study suggests training phase and TL can influence a player's body composition; that large inter-participant variability exists; and that body composition and TL are related to the change in physical qualities.
The Haller index is a ratio of thoracic width and height, measured from an axial CT image and used to describe the internal dimensions of the thoracic cage. Although the Haller index for a normal thorax has been established (Haller et al. 1987; Daunt et al. 2004), this is only at one undefined vertebral level in the thorax. What is not clear is how the Haller index describes the thorax at every vertebral level in the absence of sternal deformity, or how this is affected by age. This paper documents the shape of the thorax using the Haller index calculated from the thoracic width and height at all vertebral levels of the thorax between 8 and 18 years of age. The Haller Index changes with vertebral level, with the largest ratio seen in the most cranial levels of the thorax. Increasing age alters the shape of the thorax, with the most cranial vertebral levels having a greater Haller index over the mid thorax, which does not change. A slight increase is seen in the more caudal vertebral levels. These data highlight that a 'one size fits all' rule for chest width and depth ratio at all ages and all thoracic levels is not appropriate. The normal range for width to height ratio should be based on a patient's age and vertebral level.
The clinical assessment of scoliosis is based on the recognition of asymmetry. It is not clear what the degree of asymmetry is in a population without scoliosis, which could make the differentiation between abnormal and normal uncertain. This study defines the range of normality in certain parameters of torso shape that are also associated with the clinical assessment of scoliosis. This was done by analysing the surface topography of a group of 195 children serially measured over a 5-year period. The analysis considered both the spinal curvature and the relative position of shoulders, axillae and waist on each side. The bivariate relationships were examined using 95% confidence interval data ellipses. Our results showed that a degree of spinal curvature was seen, either as a main thoracic or main thoracolumbar curve. The distribution of the data about a mean point is illustrated by 95% confidence interval (CI) data ellipses with shoulder, axilla and waist data plotted against spinal curvature. The mean values were close to zero (exact symmetry) for all of the measured parameters, with the ellipses showing little differences in the distributions. We conclude that mild asymmetry of the measured torso parameters is normal. These results define what is normal and beyond what point asymmetry becomes abnormal. This information is of use for those managing and counselling patients with scoliosis both before and after surgery.
Study DesignRetrospective observational study of a continuous series of 28 children.PurposeTo determine the mechanical failure rate in our cohort of children treated with magnetically controlled growth rods (MCGRs).Overview of LiteraturePrevious studies report a MCGR mechanical failure rate of 0%–75%.MethodsAll patients with MCGR implantation between 2012 and 2015 were examined and followed up for a minimum of 2 years. A retrospective evaluation of contemporaneously documented clinical findings was conducted, and radiographs were retrospectively examined for mechanical failure. The external remote controller (ERC)-specified length achieved in the clinic was compared to the length measured on subsequent radiographs.ResultsFourteen mechanical failures were identified in 28 children (50%) across a total of 52 rods (24 pairs and four single constructs). Mechanical failures were due to: failure to lengthen under general anesthesia (seven children), actuator pin fracture (four), rod fracture (one), foundation screw failure (one), and ran out of rod length (one). Of the 14 mechanical failures, six were treated with final fusion operations (reflecting limited further growth potential), and eight patients were treated with the intention for further lengthening. We therefore consider these eight patients to represent the true incidence of mechanical failure in our cohort (29%). The difference between the ERC length and radiographic length was found to be identical in 11% cases; 35% were overestimates, and 54% were underestimates. The median underestimate was 2.45 mm whereas the median overestimate was 3.1 mm per distraction episode. In total, 95% of all ERC distractions were within ±10 mm of the radiographic length achieved over a median of nine distraction episodes.ConclusionsOur series is the most comprehensive MCGR series published to date, and we present a mechanical failure rate of 29%. Clinicians should be mindful of the discrepancies between ERC length and radiographic measurements of rod length; other modalities may be more helpful in this regard.
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