Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
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.
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.
BackgroundThe feasibility of conducting a definitive randomised controlled trial (RCT) evaluating the clinical effectiveness and cost-effectiveness of scoliosis-specific exercises (SSEs) for adolescent idiopathic scoliosis (AIS) is uncertain.ObjectivesThe aim of this study was to assess the feasibility of conducting a large, multicentre trial of SSE treatment for patients with AIS, in comparison with standard care, and to refine elements of the study design. The objectives were to (1) update a systematic review of controlled trials evaluating the efficacy of SSE in AIS; (2) survey UK orthopaedic surgeons and physiotherapists to determine current practice, patient populations and equipoise; (3) randomise 50 adolescents to a feasibility trial of either usual care or SSE interventions across a range of sites; (4) develop, document and assess acceptability and adherence of interventions; (5) assess and describe training requirements of physiotherapists; and (6) gain user input in all relevant stages of treatment and protocol design.DesignMulticomponent feasibility study including UK clinician survey, systematic literature review and a randomised feasibility trial.SettingThe randomised feasibility study involved four secondary care NHS trusts providing specialist care for patients with AIS.ParticipantsThe randomised feasibility study recruited people aged 10–16 years with mild AIS (Cobb angle of < 50°).InterventionsThe randomised study allocated participants to standard practice of advice and education or a physiotherapy SSE programme supported by a home exercise plan. Our choice of intervention was informed by a systematic review of exercise interventions for AIS.Main outcome measuresThe main outcome was feasibility of recruitment to the randomised study. Other elements were to inform choice of outcomes for a definitive trial and included curve severity, quality of life, requirement for surgery/brace, adverse events, psychological symptoms, costs and health utilities.ResultsA UK survey of orthopaedic consultants and physiotherapists indicated a wide variation in current provision of exercise therapy through physiotherapy services. It also found that clinicians from at least 15 centres would be willing to have their patients involved in a full study. A systematic review update found five new studies that were generally of low quality but showed some promise of effectiveness of SSE. The randomised study recruited 58 patients from four NHS trusts over 11 months and exceeded the pre-specified target recruitment rate of 1.4 participants per centre per month, with acceptable 6-month follow-up (currently 73%). Adherence to treatment was variable (56% of participants completed treatment offered). The qualitative study found the exercise programme to be highly acceptable. We learnt important lessons from patient and public involvement during the study in terms of study and intervention presentation, as well as practical elements such as scheduling of intervention sessions.ConclusionsA definitive RCT evaluating clinical effectiveness and cost-effectiveness of SSE for idiopathic scoliosis is warranted and feasible. Such a RCT is a priority for future work in the area. There is a sufficiently large patient base, combined with willingness to be randomised within specialist UK centres. Interventions developed during the feasibility study were acceptable to patients, families and physiotherapists and can be given within the affordability envelope of current levels of physiotherapy commissioning.Trial registrationCurrent Controlled Trials ISRCTN90480705.FundingThis project was funded by the NIHR Health Technology Assessment programme and will be published in full inHealth Technology Assessment; Vol. 19, No. 55. See the NIHR Journals Library website for further project information.
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