Dr Nick Jones, Academic Clinical Fellow, Nuffield Department of Primary, Care Health Sciences, Oxford
In the last issue of Backbone we reviewed the first six of the top priorities for future research in scoliosis. SAUK worked with patients, families, healthcare professionals and researchers to complete the first ever James Lind Alliance (JLA) Priority Setting
Partnership (PSP) in scoliosis. The idea of the PSP was to give as many people as possible their say about the direction future
scoliosis research should take. This involved a national survey, open to all the public. The results of the survey were reviewed by the JLA PSP team, as well as a patient panel to create a list of top questions that need to be answered. The hope is this final list will help guide and focus researchers and funding bodies to set up exciting and relevant new projects. Here we review the remaining six topics:
7. Which type of brace (eg, rigid or dynamic) is most effective in the treatment of (a) early onset scoliosis and (b) adolescent idiopathic scoliosis?
Bracing is a non-surgical treatment option and is usually recommended for children and adolescents who are still growing and have relatively small spinal curves. Broadly, the types of brace available can be divided into those made from rigid plastic or softer elastic. The
rigid braces are moulded to a patient’s body shape to ensure they fit comfortably and work effectively. They are worn for between 16 and 24 hours each day. Examples include the Boston and Milwaukee braces. The elastic equivalent, such as the SpineCor brace, are more flexible and use dynamic forces to stabilise the spine.
Compared to no treatment, both types of bracing appear to be effective at slowing or halting curve progression in adolescent idiopathic scoliosis (AIS). An American study comparing bracing to observation was stopped early because bracing was shown to be significantly more effective. Other studies have directly compared the rigid and flexible braces, and some suggest the rigid brace may be more effective at preventing curve progression. For example, among 22 people with AIS treated with the SpineCor brace, 68% did not show any curve progression over almost four years of follow-up, compared to 95% not showing any curve progression amongst the 21 people treated with the rigid brace.3 However, the number of participants included in the existing studies have not been enough to be certain that the rigid braces are better.
A 2005 review of non-surgical treatment options for scoliosis found only five small studies comparing different types of bracing.1 Across these studies there was no significant difference in the outcomes between the two types of brace. The elastic braces offer potential additional benefits in terms of comfort and flexibility so for some they may represent the best option based on the current evidence.
8. What forms of postural care are most effective (standing frames, seats, sleep suits and wheelchairs) for managing early onset, neuromuscular and syndromic scoliosis?
Spinal curvature in scoliosis typically has both a fixed and a flexible element. People who have a more flexible form of scoliosis may get most benefit from non-surgical treatment options that can either improve or slow curve progression. In addition to braces, these include a wide array of equipment that are designed to provide postural support, such as adjustable modular seating, specialised wheelchairs or standing frames.
There have been small studies which have reported that changes in the seat configuration and placement of pads in these specially designed chairs can successfully direct forces through the spine to reduce spinal curvature. These studies were conducted in people who had other neuromuscular conditions, such as cerebral palsy and were not able to walk. Similarly, other small studies suggest that supported standing frames to preserve muscle strength and tone are safe and well tolerated in children with Duchenne
Muscular Dystrophy. The longterm impact of these treatments on scoliosis progression and the relative merits in different forms of scoliosis is unclear, but further research is in progress.
9. If scoliosis is diagnosed earlier, does this change the choice of treatment and/or lead to better outcomes?
The causes of scoliosis are varied, including conditions that affect muscle strength (eg, Duchenne muscular dystrophy) or the neurological system (eg, cerebral palsy). In many cases, the cause of scoliosis is unknown, in which case it is defined as idiopathic scoliosis. The type of scoliosis and the age at which the curve begins to develop are crucial factors in determining what would be expected to happen to the curve of the spine if it were left untreated. In up to one third of people with idiopathic scoliosis, the curve will change little over time even without treatment. This means for many, the first step after diagnosis may be a period of observation, including regular review appointments with their specialist to monitor progress. In this scenario, earlier diagnosis would do little to change the choice of treatment or improve outcomes. For others, early intervention can be important in preventing the curve worsening. Certain factors can help identify those who are likely to need treatment, such as the degree of curve in the spine at the time of diagnosis. Nonsurgical treatment options, such as bracing and physiotherapy, can then be started when any spinal curve is relatively small to minimise curve progression and reduce the long-term impact of the condition.
Screening for scoliosis is generally not recommended, with agencies such as the United States Preventive Task Force suggesting the harms related to over diagnosis are likely to outweigh the benefits of early detection. This advice is based on the high ‘false negative’ rate demonstrated in previous screening studies, meaning many children were referred for further assessment who were found not to have scoliosis.
10. How are the psychological impacts (including on body image) of diagnosis and treatment best managed?
Scoliosis can have a significant impact on people’s psychological well-being. The diagnosis itself may lead to a period of adjustment and there may be fear or uncertainty as to what the future holds. Physical changes can restrict usual activities or may make people feel uncomfortable or embarrassed about their body image. Long-term health problems can also redefine relationships with other family members, sometimes causing strain but at other times bringing families closer together.
Providing the right level of support and psychological treatment will inevitably be linked to the needs of each individual, their family circumstances, type of scoliosis and planned treatment. Generally, psychological support seems to be welcomed by children and adolescents with scoliosis, as well as by their parents. Some suggest that scoliosis peer-groups can help in creating an environment where people feel comfortable and able to share their feelings. This may be supplemented by family therapy or individual sessions where needed. Others support higher intensity support and treatment at times of particular stress, such as in the run-up to spinal surgery. Further research is needed to provide more specific treatment advice on managing body image and the psychological impact of scoliosis. Key areas identified in the JLA survey included the benefit of early clinical psychologist input for scoliosis, how to support young adults facing treatment and what play techniques can help children prepare for surgery.
11. Can the instrumentation (rods/implants, etc) cause harm in the short or long term?
Rates of complication related to surgery depend in part upon the type of scoliosis. People who have idiopathic or congenital scoliosis tend to be at lower risk compared to those with neuromuscular conditions or adult onset scoliosis. Complication rates also vary between different types of surgery. For spinal fusion, there are immediate risks associated with the surgery, including a risk of death of less than 1% during the operation and a risk of 5-10% of significant infection. Some people develop chronic pain as a result of the surgery and this is the most common reason for a repeat operation being required. There is reduced range of movement in the part of the spine that has been fused, which can place stress on other parts of the spine, increasing the future risk of a fracture.
In recent years, there has been a move to using magnetic extendable spinal rods, such as the MAGEC system for treating early onset scoliosis. These reduce the number of operations needed and offer the possibility of greater flexibility in the spine. In the United Kingdom, the MAGEC system has been approved for use in children aged over two years old who need surgery. As this is a relatively new treatment option, the long-term risks remain unclear. A recent study has found there is a risk of damage to the MAGEC rods over time, which can also result in titanium debris around the rod itself.1 This debris may increase the risk of the rods failing and needing removal and repeat surgery. Surgery remains the gold standard treatment for many people with scoliosis with proven benefit in terms of reduction in spinal curve and improved function. However, the potential harms of new and existing devices needs continued evaluation to ensure patient safety in both the short and long-term.
12. How is scoliosis affected by hormonal changes in women (puberty, pregnancy and the menopause) and does this have implications for treatment using hormone replacement therapy (HRT)?
The difference between the number of men and women affected by scoliosis has led to interest in the possible role of sex hormones in the development and progression of scoliosis. Oestrogen is one such hormone that’s known to be important for bone strength. Changes in the levels of oestrogen and other sex hormones after the menopause increase the risk of women developing other problems with their bone, such as osteoporosis. Osteoporosis occurs when bones become weaker and more prone to breaking. Hormone replacement therapy can reduce the risk of osteoporosis developing and therefore may improve the strength of the skeletal system for women in the years following the menopause. How this relates to risk of scoliosis in the post-menopausal years is unclear. During puberty the levels of oestrogen rise along with other key hormone levels, such as growth hormone. Given puberty and adolescent years tends to coincide with a worsening of the spinal curve in scoliosis, growth hormone levels are also of interest in better understanding the factors responsible for scoliosis progression. Some studies have reported increased rates of scoliosis in those treated with growth hormone for other medical conditions, although these results have not been replicated in subsequent later trials. There is very limited information around hormone changes during pregnancy and how this may relate to scoliosis.
Summary
The JLA process has helped highlight key questions that need addressing in the field of scoliosis research. Many existing studies have recruited small numbers of participants and therefore lack certainty in answering their research question. There is a need for larger scale trials, particularly focussed on minimising the risks of treatment to patients, understanding the relative merits of existing treatment options and improving the holistic care for people with scoliosis.
Funding
This Scoliosis Priority Setting Partnership was funded by the British Scoliosis Research Foundation (BSRF) and supported by the National Institute for Health Research (NIHR) Oxford Biomedical Research Centre (BRC).
