Continuing professional development
Raising awareness of scoliosis among children’s nurses NCYP411 Honeyman C (2014) Raising awareness of scoliosis among children’s nurses. Nursing Children and Young People. 26, 5, 30-37. Date of submission: May 25 2013. Date of acceptance: August 28 2013.
Abstract Scoliosis affects up to 3% of adolescents, some babies and young children, and many children with existing neuromuscular and syndromic conditions. It is the most common spinal deformity. Not all children with scoliosis require active intervention, but for more significant, progressive curves, bracing and/or surgery may be required. Bracing studies have historically been of low methodological quality, but a recent randomised controlled trial (Weinstein et al 2013) has shown the efficacy of bracing in decreasing curve progression, thus reducing the necessity of surgery for some patients. Modern surgical techniques are effective in correcting scoliosis, but the surgery is major, with significant risks. Early identification of scoliosis is vital to maximise effective treatment, support the child and family, and optimise holistic health.
This article aims to raise awareness of scoliosis, outline how the condition can be diagnosed, identify which children are most likely to be affected, and explain what to do if a child has the condition. The primary concerns after identification of scoliosis are to establish whether it is likely to progress, causing further distortion in the normal curvature of the spine, and whether there is an underlying cause. After reading this article and completing the time out activities, you should be able to: ■■ Describe the different types of scoliosis, causes, prevalence and likely progression. ■■ Discuss how scoliosis can be identified and outline available treatments. ■■ Understand the effect scoliosis can have on children and their families. ■■ Consider other conditions that can be mistaken for scoliosis. ■■ Appropriately refer children with scoliosis to other healthcare professionals.
What is scoliosis? Scoliosis is a spinal deformity characterised by a lateral (sideways) curve of the spine of 10° or more as visualised on posteroanterior X-ray (Hresko 2013). The normal spine can be curved laterally by up to 10°, have a kyphosis (forward curve, or rounded back) in 30 June 2014 | Volume 26 | Number 5
the thoracic spine (20-45°), and lordosis (backward curve) of the cervical (35-45°) and lumbar (30-65°) spine. A person with scoliosis can have a single curve in one area of the spine or multiple curves along the length of their spine. Scoliosis affects the spine in all three planes: axial, coronal and sagittal (Figure 1). If the spine is curved and twisted in the thoracic region, the ribcage is pulled out of position causing a hump on the upper back. Curvature of the lumbar spine causes a hump on the lower back. Now do time out 1.
1 Time out
Aim and intended learning outcomes
Prevalence Consider the children you have worked with and estimate how common scoliosis is in your experience. Did any of the children you have encountered with scoliosis have other medical conditions?
Correspondence cheryl.honeyman@ stees.nhs.uk Cheryl Honeyman is specialist nurse, scoliosis and paediatric spine, James Cook University Hospital, Middlesbrough Conflict of interest
None declared
Keywords Adolescence, children, congenital conditions, idiopathic, scoliosis, screening, spinal deformity, spinal surgery This article has been subject to open peer review and checked using antiplagiarism software. For related articles visit our online archive and search using the keywords Author guidelines ncyp.rcnpublishing.com
Types of scoliosis Idiopathic Between 80% and 85% of scoliosis has no known cause, although there may be some genetic basis for the condition (Weinstein et al 2008). About 1% of cases are of infantile idiopathic scoliosis (0-3 years) (Konieczny et al 2013) and 10-15% are juveniles (4-10 years) (Coillard et al 2010); however, most (about 90%) NURSING CHILDREN AND YOUNG PEOPLE
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South Tees Hospitals NHS Foundation Trust
NURSING CHILDREN AND YOUNG PEOPLE
Peter Lamb
Coronal
Axial
Sagittal
Neuromuscular or syndromic scoliosis Scoliosis can be caused by nerve and muscle damage in the spine and surrounding tissue, for example due to cerebral palsy, spinal muscular atrophy or a traumatic brain injury. The severity of the angle of scoliosis curves increases if the child is non-ambulant. Children with two-limb cerebral palsy have a 25% chance of developing scoliosis, but the risk increases to 80% if all four limbs are affected (Scoliosis Research Society 2014). When young people with Duchenne muscular dystrophy
Figure 3 70° right thoracic and 66° left lumbar idiopathic scoliosis
South Tees Hospitals NHS Foundation Trust
Figure 2 110° right thoracic idiopathic scoliosis
Figure 1 Scoliosis can affect the spine in three planes
Figure 4 C-shaped neuromuscular scoliosis with tilting of the pelvis
South Tees Hospitals NHS Foundation Trust
are adolescents (Konieczny et al 2013), hence the term ‘adolescent idiopathic scoliosis’ (Wolpert 2006). Studies have found that up to 3% of adolescents will have some degree of scoliosis (Miyanji 2014); the prevalence of curve greater than 40° is only 0.1% (Miller 1999), so most adolescent cases will not require surgery (Lonstein et al 1982, Parent et al 2005). More than 40 years ago, Duval-Beaupère (1970) reported that scoliosis was most common in adolescent girls and worsened during growth spurts. Ten times more adolescent girls with scoliosis require treatment than adolescent boys with the condition (Arlet and Reddi 2007). In adolescence, the most common curve is the right thoracic (Figure 2) (Lenke et al 2002). In infantile scoliosis, however, more boys than girls are affected, and the left thoracic curve is the most frequent presentation (Rohmiller and Akbarnia 2007). However, the reasons for this change in gender predominance are not known. Some individuals may have a double scoliosis: Figure 3 shows a patient with the spine misaligned to the right in the thoracic region and to the left in the lumbar region. There is an underlying genetic basis of scoliosis, but the precise cause has not been identified (Gorman et al 2012). When both parents have scoliosis, the risk that their children will require treatment for the condition is 50 times higher than that in the general population (Lonstein 1994). Scoliosis can also be more prevalent in athletes, dancers and gymnasts, possibly due to loosening of the joints, stresses on the spine or delay in onset of puberty.
June 2014 | Volume 26 | Number 5 31
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Continuing professional development lose their ability to walk due to progressive muscular weakness, 90% develop scoliosis (Bushby et al 2010). Young people with neuromuscular scoliosis most commonly present with a long C-shaped curve with tilting of the pelvis (Figure 4, page 31) (Tolo 2003). Now do time out 2.
Time out
2
Association with other conditions Which of the conditions listed in Box 1 affect children that you care for? Consult textbooks and journal articles to help you understand the association between these conditions and scoliosis. Make brief notes to enable you to provide explanations to children and their parents or carers about the association between the condition and scoliosis.
Congenital scoliosis If the spine fails to develop properly in the first four to six weeks of pregnancy, the resulting spinal deformity can lead to scoliosis. The spine can fail to form normally, leading to hemivertebrae (wedge-shaped vertebra) and fused hemivertebrae, or it can fail to segment (Figure 5). Congenital scoliosis is slightly more common in girls than boys and is often not identified until the child reaches adolescence; it affects about one in 1,000 babies only (Sparrow et al 2012). Other causes These can be developmental (for example, achondroplasia, osteogenesis imperfecta or Chiari malformation), tumour associated (for example, astrocytoma or neurofibroma) or degenerative (for example, bone erosion or ruptured intervertebral disc).
Treatment Treatment for scoliosis is one or a combination of three approaches – monitoring, bracing and surgery – depending on severity of curvature and patient preference (Lenke 2007). For mild curvatures, regular clinic appointments with a spinal surgeon, or local paediatric orthopaedic surgeon, are required to monitor curve progression. Bracing is usually considered
Figure 5 Formation failure (left) and segmentation failure (right) Hemivertebra
Block vertebra
Bar with hemivertebra
Box 1 Syndromes or conditions associated with scoliosis ■■ Arthrogryposis (or arthrogryposis multiplex congenita). ■■ Charcot-Marie-Tooth disease. ■■ Costello syndrome (or faciocutaneoskeletal syndrome). ■■ Cri du chat syndrome (or Lejeune’s syndrome). ■■ Cushing’s syndrome. ■■ DiGeorge syndrome. ■■ Ehlers-Danlos syndrome. ■■ Fragile X syndrome. ■■ Friedreich’s ataxia. ■■ Hypothyroidism. ■■ Klippel-Feil syndrome. ■■ Larsen syndrome. ■■ Marfan’s syndrome. ■■ Morquio syndrome. ■■ Neurofibromatosis. ■■ Noonan syndrome. when the spine is curved by 20° to 50°, the patient is skeletally immature and the curve is becoming more pronounced (Bono 2007). When the spinal curvature is progressing and measures over 45°, the trunk is shifting to one side and the young person is unhappy with their shape, surgery is offered to straighten and fuse the spine (Hresko 2013, Weinstein et al 2008). Figure 6 shows X-rays before and after surgical correction. The patient was 15 years old and had a moderate idiopathic right thoracic scoliosis measuring 58°. She was otherwise fit and well. Her spine was fixed and fused from the second thoracic vertebra (T2) to the first lumbar vertebra (L1) in single-stage surgery. She recovered well and grew 6.5cm on the day of surgery, due to the straightening of her spine. If the child is skeletally immature, growing rods can be used that are extended as the child grows, before the final fusion surgery is done. For children with neuromuscular conditions who are wheelchair bound, the effect of the scoliosis on their seated position, skin integrity and lung function are the primary considerations, with the risks and benefits of bracing and surgery being of paramount importance. Now do time out 3.
Fused hemivertebra 32 June 2014 | Volume 26 | Number 5
Peter Lamb
Bar
Time out
3
Effect on daily activities Imagine that you have just been told you have scoliosis, that you need to wear a spinal brace and may need major surgery in future. How do you feel? How will it affect your life? What situations might you find especially difficult?
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Most adolescents with scoliosis are otherwise healthy. Cosmesis is usually the main concern for adolescents with a spinal deformity (Hart et al 2010), rather than back pain or long-term consequences. Adolescence is a sensitive time associated with rapid physical development, corresponding concerns over body image and the scrutiny of peers (Ahuja et al 2010). The egocentrism of the pre-school child returns in adolescence and, as part of this, adolescents find it difficult to differentiate between their preoccupations and those of others, leading to acute self-consciousness (Greene et al 1996). Self-esteem is unstable during early adolescence and low self-esteem in particular has been linked to stress and major physical and psychological changes associated with puberty (Bee and Lloyd 2013). The need for peer conformity in adolescence predominates, and the physical differences of scoliosis can result in stigmatisation, devaluation and social exclusion. Reaction to this stigma can result in concealment of the deformity by wearing very loose clothing or drawing attention to another part of the body, such as changing hair colour frequently or having tattoos. However, some adolescents might cope with their scoliosis by celebrating their differences, wearing fitted clothing and sharing their experiences with others with the condition to offer support. Readers wishing to explore stigma and physical deformity are encouraged to read the seminal work of Erving Goffman (1963). Adolescence is also a difficult time for parents, because they have to ‘let go’ and separation anxiety can apply in reverse (Rogers 2008). Parents may become overprotective, which can be difficult for adolescents seeking their independence. Families, particularly mothers, often report feeling guilty that they have not identified their child’s deformity sooner. Reassurance and supportive information needs to be given to young people and their families because positive attitudes from peers, families and healthcare professionals can help people with scoliosis to come to terms with their condition (Ahuja et al 2010). On a practical level, scoliosis requires regular clinic appointments, even if no active treatment is given. This can have financial consequences because specialist scoliosis centres can be some distance from home, so the necessary extra journeys will increase transport costs. Attending clinics also results in time away from school for the young person, often during the years when they need to take exams, and days off work for parents and carers. For families of children with existing medical conditions this can be especially problematic because it increases costs and commitments further. Parents and carers may also be worried about how the scoliosis will affect their child’s condition; NURSING CHILDREN AND YOUNG PEOPLE
Figure 6 A patient’s spine before surgical correction (left) and after surgery (right)
South Tees Hospitals NHS Foundation Trust
Consequences
for example, if lung function is already restricted by an existing condition, there may be concern that this symptom will be exacerbated with worsening scoliosis. For children diagnosed in infancy, families may be concerned about the effect of scoliosis on physical development, especially walking. It is difficult to cope with being diagnosed with a spinal deformity that could worsen, asked to wear a brace for at least 18 hours a day or having to face major spinal surgery, and the child and family need to be supported throughout. Most scoliosis centres in the UK have a designated specialist nurse whose primary role is to work with families to ensure their experience is as positive as possible. However, it is important that the wider healthcare team is aware of scoliosis and appreciates the effect it can have on young people and their families for it to work effectively alongside scoliosis teams for maximum benefit.
Progression Spinal surgeons use two primary factors – skeletal maturity and magnitude of the curvature – to predict whether scoliosis is likely to progress. The prediction methods presented in tables 1 and 2 (page 34) are based on long-term research studies (Nachemson et al 1982, Lonstein and Carlson 1984) and are used widely. Table 1 shows prediction of curve progression based on the Risser sign, which is a measure of ossification of the iliac crest as identified on X-ray to determine the stage of skeletal maturity. Other factors that affect progression of scoliosis include: ■■ Gender. Scoliosis in females is more likely to progress than in males (Hresko 2013). ■■ Menarche. The peak pubertal growth spurt is June 2014 | Volume 26 | Number 5 33
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Continuing professional development Table 1 Prediction of curve progression in untreated scoliosis based on the Risser sign and curve magnitude at initial detection Percentage of curvatures that progress Risser sign
Curve 5-19°
Curve 20-29°
0-1
22.0%
68.0%
2-4
1.6%
23.0%
(Adapted from Lonstein and Carlson 1984)
Table 2 Prediction of curve progression based on curve magnitude at initial detection and patient age Percentage of curvatures that progress Curve at detection
10-12 years
13-15 years
16 years
60°
100%
90%
70%
(Adapted from Nachemson et al 1982)
associated with initiation and progression of adolescent idiopathic scoliosis (Grivas et al 2006). Peak growth occurs one year before menarche and continues for two years after (Hresko 2013). ■■ Curve type. Thoracic curves are more likely to progress than lumbar curves, and double curves are more likely to progress than single curves (Lonstein and Carlson 1984, Bunnell 1986, Soucacos et al 1998). Congenital scoliosis has a worse prognosis when the anomaly is in the lower spine (Loder 2003). ■■ Subtype of scoliosis. Neuromuscular scoliosis tends to progress even after skeletal maturity (Thometz and Simon 1988); 90% of infantile scoliosis will be self-resolving (Lloyd-Roberts and Pilcher 1965).
Importance of early detection The primary goals of treatment for scoliosis are to prevent progression of the curve and to preserve pulmonary and cardiac function, with improvement of body shape – and, therefore, of self-esteem – being an important adjunct. Early detection, monitoring and treatment of the condition are essential to minimise its effects and provide patients with the best treatment options. Significant untreated scoliosis can result in psychological issues such as anxiety, depression, lack of 34 June 2014 | Volume 26 | Number 5
self-esteem and altered body image. Lack of treatment can also lead to increased risk of back pain in adulthood, inability to work, and higher morbidity and mortality (Nachemson 1968, Nilsonne and Lundgren 1968, Weinstein et al 2003). Children with scoliosis caused by neuromuscular conditions can benefit from bracing and wheelchair modifications for postural support to try to slow down the progression of the curve; this approach is especially important in those for whom the risks of surgery outweigh the benefits (Kotwicki et al 2008). Large curvatures can restrict lung functioning and, as children with neuromuscular conditions often have respiratory problems, it is vital that their scoliosis is treated. School screening for scoliosis is undertaken in Japan and some parts of the United States, Italy and Greece (Grivas et al 2007). The UK National Screening Committee (2011) reviewed all the available evidence and, despite agreeing that scoliosis is a significant health problem in children, decided there was insufficient good quality evidence to support national screening. Specific reasons given against the case for screening were that: ■■ Most cases identified at an early stage do not progress enough to require treatment. ■■ False positives may result in unnecessary X-rays and hospital appointments. ■■ Cases needing treatment are likely to be detected without screening. In a UK study, Ali Fazal and Edgar (2006) found that 56% of 100 consecutive adolescent idiopathic scoliosis patients referred to a London hospital clinic had a primary curve of more than 40°. Adobor et al (2012) compared outcomes before and after scoliosis screening was abolished in Norway; although the study had acknowledged limitations, the authors suggest that delays in referrals reduce the chances of slowing or halting curve progression by bracing, leaving surgery as the only treatment option for some patients. In the author’s practice, adolescents have presented for the first time in clinic with curves measuring 90° that require surgery. Adolescents often hide their deformity under loose clothing, are self-conscious and can keep any concerns they have about body shape to themselves in their quest for independence. The current trend for end-of-school proms has been a notable factor in identifying scoliosis, as dress fittings have highlighted physical asymmetry. Scoliosis is also commonly identified on holiday, when the child or young person is in swimwear, or at gym or dance class. Children with neuromuscular scoliosis can have so many comorbidities and associated problems that parents and carers can mistake the scoliosis as another part of their existing condition, not realising that it may benefit from treatment. In the absence of scoliosis screening programmes, it is important for all healthcare professionals who work with NURSING CHILDREN AND YOUNG PEOPLE
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Figure 7 Common features of scoliosis Peter Lamb
Peter Lamb
Figure 8 The Adams forward bend test: normal spine (left) versus scoliosis (right)
1
3 4
6 2
5
5
7 6 (Adapted from Adams 1882)
Identification The most common features of scoliosis are (Figure 7): 1. One shoulder is higher than the other. 2. The body tilts to one side. 3. One scapula is higher than the other. 4. One scapula is more prominent than the other. 5. The waist is uneven. 6. The presence of rib or loin humps (more evident on forward bending). 7. One hip appears higher than the other. In addition, clothes may not hang properly, girls may find that one breast appears more prominent than the other, Box 2 Defects associated with the Vacterl association V A C TE R L
Vertebral anomalies Anal atresia Cardiovascular anomalies Tracheoesophageal fistula Renal anomalies Limb defects
NURSING CHILDREN AND YOUNG PEOPLE
and children in wheelchairs may become uncomfortable in the seated position. If a child or young person is suspected of having scoliosis, they should be asked to put the palms of their hands together, place their feet together, and to bend forward with hands towards the toes (the Adams forward bend test (1882). Any rib or loin humps caused by scoliosis should then be more evident (Figure 8). Now do time out 4.
4 Time out
children and young people to be aware of the condition, how to identify it and to whom to refer the child. Reamy and Slakey (2001) suggest that those at low risk of progression should not be referred to spinal surgeons, as this can cause anxiety, absence from school and unnecessary radiation exposure. However, unless you are confident that you are able to monitor the child, it is safest to refer to a specialist for expert advice.
Examination Using a willing young volunteer, practise an examination for signs of scoliosis. If any anomalies are discovered, consider the differential diagnoses below before considering referral.
If congenital scoliosis is suspected, the Vacterl association (or VATER syndrome, Box 2) should be considered because up to 80% of patients with the Vacterl association have vertebral anomalies (Solomon 2011). Any child with congenital scoliosis should be investigated for these defects. The kidney and bladder are formed at the same time as the spine, so there is a 25% chance these organs will also be affected. There is a 10% chance of heart defects, and 2-5% chance of lung and oesophageal problems (Scoliosis Association (UK) 2012). June 2014 | Volume 26 | Number 5 35
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Continuing professional development Leg length discrepancy can be mistaken for scoliosis because one side of the pelvis is higher than the other. If the spine is examined in the sitting position and the curve is due to a leg length discrepancy, the curve will disappear. Unequal leg lengths are found in 50% of the population, but this proportion increases to 75% in people with low back pain (Friberg 1983). Leg length discrepancy can be identified by laying the patient supine, measuring the distance between the anterior superior iliac spine and medial malleolus on each side of the body, and then comparing the two figures. More precise measurements can be made using a computed tomography scanogram. If leg length discrepancy is found to be over 1.5cm, a referral should be made to an orthopaedic surgeon. Most cases of leg length discrepancy can be corrected by using a shoe insert under the shorter leg. For larger and troublesome leg length discrepancies, an orthopaedic surgeon may consider arresting growth of the longer leg by epiphysiodesis – surgical interruption of the epiphyseal plate (Staheli 2008). Sprengel’s deformity of the shoulder, or high scapula, is a congenital skeletal abnormality where one scapula fails to descend fully during early foetal development. This painless deformity is also often noticed only in adolescence and the asymmetry can be mistaken for scoliosis. Treatment is only required if the condition affects movement of the arm and can be in the form of physiotherapy or, in severe cases, surgery. Non-structural (postural) curves can be caused by muscle spasms, and resolve when the underlying cause is treated. Occasionally, there may be a psychological
reason for presenting with a posture indicating a spinal curve. Curves due to non-structural causes will disappear on forward bending. Now do time out 5.
5 Time out
Differential diagnosis
Referral A 14-year-old girl tells you her hips are uneven. You examine her and identify features suggestive of scoliosis. Think about what you would do in your role as a children’s nurse and write a mock referral to a spinal surgeon.
What to do when scoliosis is identified If scoliosis is suspected, the child should be referred to a spinal surgeon for assessment, monitoring and treatment, if necessary. Children and their families will be understandably concerned and many will search for further information online. The quality of such information varies, so it would be helpful if healthcare professionals had information about their local services to enable them to guide children and families towards these, to ensure information is relevant and accurate. Like many regional scoliosis centres, the Paediatric Scoliosis and Spinal Service of the South Tees Hospitals NHS Foundation Trust has a website with information leaflets available for patients and families (www. southtees.nhs.uk/services/orthopaedics/paediatric-spinal). Families could also be given the contact information of the Scoliosis Association UK (www.sauk.org.uk). Emphasis should be made on how scoliosis cannot be
References Adams W (1882) Lectures on the Pathology and Treatment of Lateral and Other Forms of Curvature of the Spine. Churchill, London. Adobor RD et al (2012) Scoliosis detection, patient characteristics, referral patterns and treatment in the absence of a screening programme in Norway. Scoliosis. 7, 1, 18. Ahuja A et al (2010) Living With a Spinal Deformity: Perception of Deformity and Expectations from Treatment. Lambert Academic Publishing, Saarbrücken, Germany. Ali Fazal M, Edgar M (2006) Detection of adolescent idiopathic scoliosis. Acta Orthopaedica Belgica. 72, 2, 184-186. Arlet V, Reddi V (2007) Adolescent idiopathic scoliosis. Neurosurgery Clinics of North America. 18, 2, 255-259. Bee HL, Lloyd DG (2013) The Developing Child. Thirteenth edition. Pearson Education, Harlow. Bono CM (2007) Bracing and non-operative treatment of spinal deformity. In Heary RF, Albert TJ (Eds) Spinal Deformities:
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The Essentials. Thieme Medical Publishers, New York NY. Bunnell WP (1986) The natural history of idiopathic scoliosis before skeletal maturity. Spine. 11, 8, 773-776. Bushby K et al (2010) Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurology. 9, 1, 77-93.
Goffman E (1963) Stigma: Notes on the Management of Spoiled Identity. Prentice-Hall, New Jersey NY.
Konieczny et al (2013) Epidemiology of adolescent idiopathic scoliosis. Journal of Children’s Orthopaedics. 7, 1, 3-9.
Gorman KF et al (2012) The genetic epidemiology of idiopathic scoliosis. European Spine Journal. 21, 10, 1905-1919.
Kotwicki T et al (2008) Bracing for neuromuscular scoliosis: orthosis construction to improve the patient’s function. Disability and Rehabilitation. Assistive Technology. 3, 3, 161-169.
Greene K et al (1996) The utility of understanding adolescent egocentrism in designing health promotion messages. Health Communication. 8, 2, 131-152.
Lenke LG (2007) The Lenke classification system of operative adolescent idiopathic scoliosis. Neurosurgery Clinics of North America. 18, 2, 199-206.
Coillard et al (2010) SpineCor treatment for Juvenile Idiopathic Scoliosis: SOSORT award 2010 winner. Scoliosis. 5, 25. doi: 10.1186/1748-7161-5-25.
Grivas TB et al (2006) Association between adolescent idiopathic scoliosis prevalence and age at menarche in different geographic latitudes. Scoliosis. 1, 9.
Duval-Beaupère G (1970) Les reperes maturation dans la surveillance des scoliosis [Maturation indices in the surveillance of scoliosis]. Revue de Chirurgie Orthopedique et Reparatrice de L’Appareil Moteur. 56, 1, 59-76.
Grivas TB et al (2007) SOSORT consensus paper: school screening for scoliosis. Where are we today? Scoliosis. 2, 1, 17.
Friberg O (1983) Clinical symptoms and biomechanics of lumbar spine and hip joint in leg length inequality. Spine. 8, 6, 643-651.
Hart ES et al (2010) Scheuermann’s thoracic kyphosis in the adolescent patient. Orthopaedic Nursing. 29, 6, 365-371. Hresko MT (2013) Idiopathic scoliosis in adolescents. New England Journal of Medicine. 368, 9, 834-841.
Lenke LG et al (2002) Curve prevalence of a new classification of operative adolescent idiopathic scoliosis: does classification correlate with treatment? Spine. 27, 6, 604-611. Lloyd-Roberts GC, Pilcher MF (1965) Structural idiopathic scoliosis in infancy: a study of the natural history of 100 patients. Journal of Bone and Joint Surgery. 47, 3, 520-3. Loder RT (2003) Congenital scoliosis and kyphosis. In DeWald RL (Ed) Spinal Deformities: The Comprehensive Text. Thieme Medical Publishers, New York NY.
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Time out
6
Information and support Look at the Scoliosis Association UK website (www.sauk.org.uk) and browse the information available. In addition, find out who is in your local scoliosis team, so that you know to whom to refer patients.
Although scoliosis is essentially a painless deformity, mild back pain is often reported (Adobor 2012), possibly due to muscle imbalance. To determine urgency of treatment, and therefore referral, a history of severe back pain, radiating limb pain, motor or sensory changes, and any bladder or bowel problems should be obtained. These symptoms warrant a more urgent referral as they could indicate a spinal cord tumour, tethered spinal cord, spondylolisthesis, spondylolysis or Chiari malformation with a syrinx (Hresko 2013).
Conclusion Scoliosis is the most common spinal deformity and usually affects adolescents, although younger children
Lonstein JE (1994) Adolescent idiopathic scoliosis. The Lancet. 344, 8934, 1407-1412. Lonstein JE, Carlson JM (1984) The Prediction of curve progression in untreated idiopathic scoliosis during growth. Journal of Bone and Joint Surgery. 66, 7, 1061-1071. Lonstein JE et al (1982) Voluntary school screening for scoliosis in Minnesota. Journal of Bone and Joint Surgery. 64, 4, 481-488. Miller NH (1999) Cause and natural history of adolescent idiopathic scoliosis. Orthopedic Clinics of North America. 30, 3, 343-352. Miyanji F (2014) Adolescent idiopathic scoliosis: current perspectives. Orthopedic Research and Reviews. 6, 17-26. doi: http://dx.doi.org/10.2147/ORR.S37321 Nachemson A (1968) A long term follow-up study of non-treated scoliosis. Acta Orthopaedica Scandinavica. 39, 4, 466-476.
and those with pre-existing neuromuscular or syndromic conditions can also be affected. Treatment is in the form of monitoring, bracing or surgery, or any combination of these approaches. Progression varies and depends on various factors, such as skeletal maturity, gender and type of curve. Early identification of scoliosis can enable prompt, effective treatment, which may reduce the need for major surgery for some children. Raising awareness and sharing knowledge of scoliosis is an important task for children’s nurses working in children’s spinal services. Now do time out 7.
7 Time out
prevented and has no known cause, as many parents feel guilt because they failed to recognise the deformity earlier. The spinal surgeon will request a standing posteroanterior and lateral X-ray of the whole spine, including the hips, so it would be helpful if this can be organised before the referral. A referral should include a brief medical history, information about any pain experienced, medications prescribed, relevant family history and features suggestive of scoliosis. Now do time out 6.
Self-assessment questionnaire Now that you have completed the article you might like to complete the questionnaire on page 38. To write a practice profile, go to ncyp.rcnpublishing.com
Further information ■■ What is scoliosis? Scoliosis Association (UK): www.sauk.org.uk/about-scoliosis/what-is-scoliosis. html ■■ Paediatric scoliosis and spinal service, South Tees Hospitals NHS Foundation Trust: www.southtees. nhs.uk/services/orthopaedics/paediatric-spinal ■■ Neuromuscular Scoliosis, Scoliosis Research Society: www.srs.org/patient_and_family/scoliosis/ neuromuscular_scoliosis
Nachemson A et al (1982) Report of the Scoliosis Research Society Prevalence and Natural History Committee. Scoliosis Research Society, Milwaukee WI.
Scoliosis Association (UK) (2012) Congenital Scoliosis. www.sauk.org.uk/ about-scoliosis/congenital-scoliosis. html (Last accessed: May 9 2014.)
Nilsonne U, Lundgren KD (1968) Longterm prognosis in idiopathic scoliosis. Acta Orthopaedica Scandinavica. 39, 4, 456-465.
Scoliosis Research Society (2014) Neuromuscular Scoliosis. tinyurl.com/srs-nmscoliosis (Last accessed: May 8 2014.)
Parent S et al (2005) Adolescent idiopathic scoliosis: etiology, anatomy, natural history, and bracing. Instructional Course Lectures. 54, 529-536.
Solomon BD (2011) Vacterl/Vater Association. Orphanet Journal of Rare Diseases. 6, 56.
Reamy BV, Slakey JB (2001) Adolescent idiopathic scoliosis: review and current concepts. American Family Physician. 64, 1, 111-116. Rogers R (2008) Managing Persistent Pain in Adolescents – A Handbook for Therapists. Radcliffe, Oxford. Rohmiller MT, Akbarnia BA (2007) Infantile scoliosis. In Heary RF, Albert TJ (Eds) Spinal Deformities: The Essentials. Thieme Medical Publishers, New York NY.
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Soucacos PN et al (1998) Assessment of curve progression in idiopathic scoliosis. European Spine Journal. 7, 4, 270-277. Sparrow DB et al (2012) A mechanism for gene-environment interaction in the etiology of congenital scoliosis. Cell. 149, 2, 295-306. Staheli LT (2008) Fundamentals of Pediatric Orthopedics. Fourth edition. Lippincott Williams & Wilkins, Philadelphia PA. Thometz JG, Simon SR (1988) Progression of scoliosis after skeletal maturity in institutionalized adults who have cerebral palsy. Journal of Bone and Joint Surgery. 70, 9, 1290-1296.
Acknowledgement Thank you to Raman Kalyan, Waleed Hekal and Charles Greenough, consultant spine surgeons, James Cook University Hospital, for sharing their expert knowledge and experience Conflict of interest None declared
Tolo VT (2003) Clinical evaluation for neuromuscular scoliosis and kyphosis. In DeWald RL (Ed) Spinal Deformities: The Comprehensive Text. Thieme Medical Publishers, New York NY. UK National Screening Committee (2011) Screening for Adolescent Idiopathic Scoliosis. External Review Against Programme Appraisal Criteria for the UNSC (UK NSC). tinyurl.com/ nsc-adol-idio-scol (Last accessed: May 8 2014.) Weinstein SL et al (2003) Health and function of patients with untreated idiopathic scoliosis: A 50 year natural history study. Journal of the American Medical Association. 289, 5, 559-57. Weinstein SL et al (2008) Adolescent idiopathic scoliosis. The Lancet. 371, 9623, 1527-1537. Weinstein S et al (2013) Effects of bracing in adolescents with idiopathic scoliosis. New England Journal of Medicine. 369, 16, 1512-1521. Wolpert DK (2006) Scoliosis Surgery: The Definitive Patient’s Reference. Third edition. Swordfish Communications, Austin TX.
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Raising awareness of scoliosis among children’s nurses NCYP411 Honeyman C (2014) Raising awareness of scoliosis among children’s nurses. Nursing Children and Young People. 26, 5, 30-37. Date of submission: May 25 2013. Date of acceptance: August 28 2013.
Abstract Scoliosis affects up to 3% of adolescents, some babies and young children, and many children with existing neuromuscular and syndromic conditions. It is the most common spinal deformity. Not all children with scoliosis require active intervention, but for more significant, progressive curves, bracing and/or surgery may be required. Bracing studies have historically been of low methodological quality, but a recent randomised controlled trial (Weinstein et al 2013) has shown the efficacy of bracing in decreasing curve progression, thus reducing the necessity of surgery for some patients. Modern surgical techniques are effective in correcting scoliosis, but the surgery is major, with significant risks. Early identification of scoliosis is vital to maximise effective treatment, support the child and family, and optimise holistic health.
This article aims to raise awareness of scoliosis, outline how the condition can be diagnosed, identify which children are most likely to be affected, and explain what to do if a child has the condition. The primary concerns after identification of scoliosis are to establish whether it is likely to progress, causing further distortion in the normal curvature of the spine, and whether there is an underlying cause. After reading this article and completing the time out activities, you should be able to: ■■ Describe the different types of scoliosis, causes, prevalence and likely progression. ■■ Discuss how scoliosis can be identified and outline available treatments. ■■ Understand the effect scoliosis can have on children and their families. ■■ Consider other conditions that can be mistaken for scoliosis. ■■ Appropriately refer children with scoliosis to other healthcare professionals.
What is scoliosis? Scoliosis is a spinal deformity characterised by a lateral (sideways) curve of the spine of 10° or more as visualised on posteroanterior X-ray (Hresko 2013). The normal spine can be curved laterally by up to 10°, have a kyphosis (forward curve, or rounded back) in 30 June 2014 | Volume 26 | Number 5
the thoracic spine (20-45°), and lordosis (backward curve) of the cervical (35-45°) and lumbar (30-65°) spine. A person with scoliosis can have a single curve in one area of the spine or multiple curves along the length of their spine. Scoliosis affects the spine in all three planes: axial, coronal and sagittal (Figure 1). If the spine is curved and twisted in the thoracic region, the ribcage is pulled out of position causing a hump on the upper back. Curvature of the lumbar spine causes a hump on the lower back. Now do time out 1.
1 Time out
Aim and intended learning outcomes
Prevalence Consider the children you have worked with and estimate how common scoliosis is in your experience. Did any of the children you have encountered with scoliosis have other medical conditions?
Correspondence cheryl.honeyman@ stees.nhs.uk Cheryl Honeyman is specialist nurse, scoliosis and paediatric spine, James Cook University Hospital, Middlesbrough Conflict of interest
None declared
Keywords Adolescence, children, congenital conditions, idiopathic, scoliosis, screening, spinal deformity, spinal surgery This article has been subject to open peer review and checked using antiplagiarism software. For related articles visit our online archive and search using the keywords Author guidelines ncyp.rcnpublishing.com
Types of scoliosis Idiopathic Between 80% and 85% of scoliosis has no known cause, although there may be some genetic basis for the condition (Weinstein et al 2008). About 1% of cases are of infantile idiopathic scoliosis (0-3 years) (Konieczny et al 2013) and 10-15% are juveniles (4-10 years) (Coillard et al 2010); however, most (about 90%) NURSING CHILDREN AND YOUNG PEOPLE
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South Tees Hospitals NHS Foundation Trust
NURSING CHILDREN AND YOUNG PEOPLE
Peter Lamb
Coronal
Axial
Sagittal
Neuromuscular or syndromic scoliosis Scoliosis can be caused by nerve and muscle damage in the spine and surrounding tissue, for example due to cerebral palsy, spinal muscular atrophy or a traumatic brain injury. The severity of the angle of scoliosis curves increases if the child is non-ambulant. Children with two-limb cerebral palsy have a 25% chance of developing scoliosis, but the risk increases to 80% if all four limbs are affected (Scoliosis Research Society 2014). When young people with Duchenne muscular dystrophy
Figure 3 70° right thoracic and 66° left lumbar idiopathic scoliosis
South Tees Hospitals NHS Foundation Trust
Figure 2 110° right thoracic idiopathic scoliosis
Figure 1 Scoliosis can affect the spine in three planes
Figure 4 C-shaped neuromuscular scoliosis with tilting of the pelvis
South Tees Hospitals NHS Foundation Trust
are adolescents (Konieczny et al 2013), hence the term ‘adolescent idiopathic scoliosis’ (Wolpert 2006). Studies have found that up to 3% of adolescents will have some degree of scoliosis (Miyanji 2014); the prevalence of curve greater than 40° is only 0.1% (Miller 1999), so most adolescent cases will not require surgery (Lonstein et al 1982, Parent et al 2005). More than 40 years ago, Duval-Beaupère (1970) reported that scoliosis was most common in adolescent girls and worsened during growth spurts. Ten times more adolescent girls with scoliosis require treatment than adolescent boys with the condition (Arlet and Reddi 2007). In adolescence, the most common curve is the right thoracic (Figure 2) (Lenke et al 2002). In infantile scoliosis, however, more boys than girls are affected, and the left thoracic curve is the most frequent presentation (Rohmiller and Akbarnia 2007). However, the reasons for this change in gender predominance are not known. Some individuals may have a double scoliosis: Figure 3 shows a patient with the spine misaligned to the right in the thoracic region and to the left in the lumbar region. There is an underlying genetic basis of scoliosis, but the precise cause has not been identified (Gorman et al 2012). When both parents have scoliosis, the risk that their children will require treatment for the condition is 50 times higher than that in the general population (Lonstein 1994). Scoliosis can also be more prevalent in athletes, dancers and gymnasts, possibly due to loosening of the joints, stresses on the spine or delay in onset of puberty.
June 2014 | Volume 26 | Number 5 31
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Continuing professional development lose their ability to walk due to progressive muscular weakness, 90% develop scoliosis (Bushby et al 2010). Young people with neuromuscular scoliosis most commonly present with a long C-shaped curve with tilting of the pelvis (Figure 4, page 31) (Tolo 2003). Now do time out 2.
Time out
2
Association with other conditions Which of the conditions listed in Box 1 affect children that you care for? Consult textbooks and journal articles to help you understand the association between these conditions and scoliosis. Make brief notes to enable you to provide explanations to children and their parents or carers about the association between the condition and scoliosis.
Congenital scoliosis If the spine fails to develop properly in the first four to six weeks of pregnancy, the resulting spinal deformity can lead to scoliosis. The spine can fail to form normally, leading to hemivertebrae (wedge-shaped vertebra) and fused hemivertebrae, or it can fail to segment (Figure 5). Congenital scoliosis is slightly more common in girls than boys and is often not identified until the child reaches adolescence; it affects about one in 1,000 babies only (Sparrow et al 2012). Other causes These can be developmental (for example, achondroplasia, osteogenesis imperfecta or Chiari malformation), tumour associated (for example, astrocytoma or neurofibroma) or degenerative (for example, bone erosion or ruptured intervertebral disc).
Treatment Treatment for scoliosis is one or a combination of three approaches – monitoring, bracing and surgery – depending on severity of curvature and patient preference (Lenke 2007). For mild curvatures, regular clinic appointments with a spinal surgeon, or local paediatric orthopaedic surgeon, are required to monitor curve progression. Bracing is usually considered
Figure 5 Formation failure (left) and segmentation failure (right) Hemivertebra
Block vertebra
Bar with hemivertebra
Box 1 Syndromes or conditions associated with scoliosis ■■ Arthrogryposis (or arthrogryposis multiplex congenita). ■■ Charcot-Marie-Tooth disease. ■■ Costello syndrome (or faciocutaneoskeletal syndrome). ■■ Cri du chat syndrome (or Lejeune’s syndrome). ■■ Cushing’s syndrome. ■■ DiGeorge syndrome. ■■ Ehlers-Danlos syndrome. ■■ Fragile X syndrome. ■■ Friedreich’s ataxia. ■■ Hypothyroidism. ■■ Klippel-Feil syndrome. ■■ Larsen syndrome. ■■ Marfan’s syndrome. ■■ Morquio syndrome. ■■ Neurofibromatosis. ■■ Noonan syndrome. when the spine is curved by 20° to 50°, the patient is skeletally immature and the curve is becoming more pronounced (Bono 2007). When the spinal curvature is progressing and measures over 45°, the trunk is shifting to one side and the young person is unhappy with their shape, surgery is offered to straighten and fuse the spine (Hresko 2013, Weinstein et al 2008). Figure 6 shows X-rays before and after surgical correction. The patient was 15 years old and had a moderate idiopathic right thoracic scoliosis measuring 58°. She was otherwise fit and well. Her spine was fixed and fused from the second thoracic vertebra (T2) to the first lumbar vertebra (L1) in single-stage surgery. She recovered well and grew 6.5cm on the day of surgery, due to the straightening of her spine. If the child is skeletally immature, growing rods can be used that are extended as the child grows, before the final fusion surgery is done. For children with neuromuscular conditions who are wheelchair bound, the effect of the scoliosis on their seated position, skin integrity and lung function are the primary considerations, with the risks and benefits of bracing and surgery being of paramount importance. Now do time out 3.
Fused hemivertebra 32 June 2014 | Volume 26 | Number 5
Peter Lamb
Bar
Time out
3
Effect on daily activities Imagine that you have just been told you have scoliosis, that you need to wear a spinal brace and may need major surgery in future. How do you feel? How will it affect your life? What situations might you find especially difficult?
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Most adolescents with scoliosis are otherwise healthy. Cosmesis is usually the main concern for adolescents with a spinal deformity (Hart et al 2010), rather than back pain or long-term consequences. Adolescence is a sensitive time associated with rapid physical development, corresponding concerns over body image and the scrutiny of peers (Ahuja et al 2010). The egocentrism of the pre-school child returns in adolescence and, as part of this, adolescents find it difficult to differentiate between their preoccupations and those of others, leading to acute self-consciousness (Greene et al 1996). Self-esteem is unstable during early adolescence and low self-esteem in particular has been linked to stress and major physical and psychological changes associated with puberty (Bee and Lloyd 2013). The need for peer conformity in adolescence predominates, and the physical differences of scoliosis can result in stigmatisation, devaluation and social exclusion. Reaction to this stigma can result in concealment of the deformity by wearing very loose clothing or drawing attention to another part of the body, such as changing hair colour frequently or having tattoos. However, some adolescents might cope with their scoliosis by celebrating their differences, wearing fitted clothing and sharing their experiences with others with the condition to offer support. Readers wishing to explore stigma and physical deformity are encouraged to read the seminal work of Erving Goffman (1963). Adolescence is also a difficult time for parents, because they have to ‘let go’ and separation anxiety can apply in reverse (Rogers 2008). Parents may become overprotective, which can be difficult for adolescents seeking their independence. Families, particularly mothers, often report feeling guilty that they have not identified their child’s deformity sooner. Reassurance and supportive information needs to be given to young people and their families because positive attitudes from peers, families and healthcare professionals can help people with scoliosis to come to terms with their condition (Ahuja et al 2010). On a practical level, scoliosis requires regular clinic appointments, even if no active treatment is given. This can have financial consequences because specialist scoliosis centres can be some distance from home, so the necessary extra journeys will increase transport costs. Attending clinics also results in time away from school for the young person, often during the years when they need to take exams, and days off work for parents and carers. For families of children with existing medical conditions this can be especially problematic because it increases costs and commitments further. Parents and carers may also be worried about how the scoliosis will affect their child’s condition; NURSING CHILDREN AND YOUNG PEOPLE
Figure 6 A patient’s spine before surgical correction (left) and after surgery (right)
South Tees Hospitals NHS Foundation Trust
Consequences
for example, if lung function is already restricted by an existing condition, there may be concern that this symptom will be exacerbated with worsening scoliosis. For children diagnosed in infancy, families may be concerned about the effect of scoliosis on physical development, especially walking. It is difficult to cope with being diagnosed with a spinal deformity that could worsen, asked to wear a brace for at least 18 hours a day or having to face major spinal surgery, and the child and family need to be supported throughout. Most scoliosis centres in the UK have a designated specialist nurse whose primary role is to work with families to ensure their experience is as positive as possible. However, it is important that the wider healthcare team is aware of scoliosis and appreciates the effect it can have on young people and their families for it to work effectively alongside scoliosis teams for maximum benefit.
Progression Spinal surgeons use two primary factors – skeletal maturity and magnitude of the curvature – to predict whether scoliosis is likely to progress. The prediction methods presented in tables 1 and 2 (page 34) are based on long-term research studies (Nachemson et al 1982, Lonstein and Carlson 1984) and are used widely. Table 1 shows prediction of curve progression based on the Risser sign, which is a measure of ossification of the iliac crest as identified on X-ray to determine the stage of skeletal maturity. Other factors that affect progression of scoliosis include: ■■ Gender. Scoliosis in females is more likely to progress than in males (Hresko 2013). ■■ Menarche. The peak pubertal growth spurt is June 2014 | Volume 26 | Number 5 33
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Continuing professional development Table 1 Prediction of curve progression in untreated scoliosis based on the Risser sign and curve magnitude at initial detection Percentage of curvatures that progress Risser sign
Curve 5-19°
Curve 20-29°
0-1
22.0%
68.0%
2-4
1.6%
23.0%
(Adapted from Lonstein and Carlson 1984)
Table 2 Prediction of curve progression based on curve magnitude at initial detection and patient age Percentage of curvatures that progress Curve at detection
10-12 years
13-15 years
16 years
60°
100%
90%
70%
(Adapted from Nachemson et al 1982)
associated with initiation and progression of adolescent idiopathic scoliosis (Grivas et al 2006). Peak growth occurs one year before menarche and continues for two years after (Hresko 2013). ■■ Curve type. Thoracic curves are more likely to progress than lumbar curves, and double curves are more likely to progress than single curves (Lonstein and Carlson 1984, Bunnell 1986, Soucacos et al 1998). Congenital scoliosis has a worse prognosis when the anomaly is in the lower spine (Loder 2003). ■■ Subtype of scoliosis. Neuromuscular scoliosis tends to progress even after skeletal maturity (Thometz and Simon 1988); 90% of infantile scoliosis will be self-resolving (Lloyd-Roberts and Pilcher 1965).
Importance of early detection The primary goals of treatment for scoliosis are to prevent progression of the curve and to preserve pulmonary and cardiac function, with improvement of body shape – and, therefore, of self-esteem – being an important adjunct. Early detection, monitoring and treatment of the condition are essential to minimise its effects and provide patients with the best treatment options. Significant untreated scoliosis can result in psychological issues such as anxiety, depression, lack of 34 June 2014 | Volume 26 | Number 5
self-esteem and altered body image. Lack of treatment can also lead to increased risk of back pain in adulthood, inability to work, and higher morbidity and mortality (Nachemson 1968, Nilsonne and Lundgren 1968, Weinstein et al 2003). Children with scoliosis caused by neuromuscular conditions can benefit from bracing and wheelchair modifications for postural support to try to slow down the progression of the curve; this approach is especially important in those for whom the risks of surgery outweigh the benefits (Kotwicki et al 2008). Large curvatures can restrict lung functioning and, as children with neuromuscular conditions often have respiratory problems, it is vital that their scoliosis is treated. School screening for scoliosis is undertaken in Japan and some parts of the United States, Italy and Greece (Grivas et al 2007). The UK National Screening Committee (2011) reviewed all the available evidence and, despite agreeing that scoliosis is a significant health problem in children, decided there was insufficient good quality evidence to support national screening. Specific reasons given against the case for screening were that: ■■ Most cases identified at an early stage do not progress enough to require treatment. ■■ False positives may result in unnecessary X-rays and hospital appointments. ■■ Cases needing treatment are likely to be detected without screening. In a UK study, Ali Fazal and Edgar (2006) found that 56% of 100 consecutive adolescent idiopathic scoliosis patients referred to a London hospital clinic had a primary curve of more than 40°. Adobor et al (2012) compared outcomes before and after scoliosis screening was abolished in Norway; although the study had acknowledged limitations, the authors suggest that delays in referrals reduce the chances of slowing or halting curve progression by bracing, leaving surgery as the only treatment option for some patients. In the author’s practice, adolescents have presented for the first time in clinic with curves measuring 90° that require surgery. Adolescents often hide their deformity under loose clothing, are self-conscious and can keep any concerns they have about body shape to themselves in their quest for independence. The current trend for end-of-school proms has been a notable factor in identifying scoliosis, as dress fittings have highlighted physical asymmetry. Scoliosis is also commonly identified on holiday, when the child or young person is in swimwear, or at gym or dance class. Children with neuromuscular scoliosis can have so many comorbidities and associated problems that parents and carers can mistake the scoliosis as another part of their existing condition, not realising that it may benefit from treatment. In the absence of scoliosis screening programmes, it is important for all healthcare professionals who work with NURSING CHILDREN AND YOUNG PEOPLE
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Figure 7 Common features of scoliosis Peter Lamb
Peter Lamb
Figure 8 The Adams forward bend test: normal spine (left) versus scoliosis (right)
1
3 4
6 2
5
5
7 6 (Adapted from Adams 1882)
Identification The most common features of scoliosis are (Figure 7): 1. One shoulder is higher than the other. 2. The body tilts to one side. 3. One scapula is higher than the other. 4. One scapula is more prominent than the other. 5. The waist is uneven. 6. The presence of rib or loin humps (more evident on forward bending). 7. One hip appears higher than the other. In addition, clothes may not hang properly, girls may find that one breast appears more prominent than the other, Box 2 Defects associated with the Vacterl association V A C TE R L
Vertebral anomalies Anal atresia Cardiovascular anomalies Tracheoesophageal fistula Renal anomalies Limb defects
NURSING CHILDREN AND YOUNG PEOPLE
and children in wheelchairs may become uncomfortable in the seated position. If a child or young person is suspected of having scoliosis, they should be asked to put the palms of their hands together, place their feet together, and to bend forward with hands towards the toes (the Adams forward bend test (1882). Any rib or loin humps caused by scoliosis should then be more evident (Figure 8). Now do time out 4.
4 Time out
children and young people to be aware of the condition, how to identify it and to whom to refer the child. Reamy and Slakey (2001) suggest that those at low risk of progression should not be referred to spinal surgeons, as this can cause anxiety, absence from school and unnecessary radiation exposure. However, unless you are confident that you are able to monitor the child, it is safest to refer to a specialist for expert advice.
Examination Using a willing young volunteer, practise an examination for signs of scoliosis. If any anomalies are discovered, consider the differential diagnoses below before considering referral.
If congenital scoliosis is suspected, the Vacterl association (or VATER syndrome, Box 2) should be considered because up to 80% of patients with the Vacterl association have vertebral anomalies (Solomon 2011). Any child with congenital scoliosis should be investigated for these defects. The kidney and bladder are formed at the same time as the spine, so there is a 25% chance these organs will also be affected. There is a 10% chance of heart defects, and 2-5% chance of lung and oesophageal problems (Scoliosis Association (UK) 2012). June 2014 | Volume 26 | Number 5 35
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Continuing professional development Leg length discrepancy can be mistaken for scoliosis because one side of the pelvis is higher than the other. If the spine is examined in the sitting position and the curve is due to a leg length discrepancy, the curve will disappear. Unequal leg lengths are found in 50% of the population, but this proportion increases to 75% in people with low back pain (Friberg 1983). Leg length discrepancy can be identified by laying the patient supine, measuring the distance between the anterior superior iliac spine and medial malleolus on each side of the body, and then comparing the two figures. More precise measurements can be made using a computed tomography scanogram. If leg length discrepancy is found to be over 1.5cm, a referral should be made to an orthopaedic surgeon. Most cases of leg length discrepancy can be corrected by using a shoe insert under the shorter leg. For larger and troublesome leg length discrepancies, an orthopaedic surgeon may consider arresting growth of the longer leg by epiphysiodesis – surgical interruption of the epiphyseal plate (Staheli 2008). Sprengel’s deformity of the shoulder, or high scapula, is a congenital skeletal abnormality where one scapula fails to descend fully during early foetal development. This painless deformity is also often noticed only in adolescence and the asymmetry can be mistaken for scoliosis. Treatment is only required if the condition affects movement of the arm and can be in the form of physiotherapy or, in severe cases, surgery. Non-structural (postural) curves can be caused by muscle spasms, and resolve when the underlying cause is treated. Occasionally, there may be a psychological
reason for presenting with a posture indicating a spinal curve. Curves due to non-structural causes will disappear on forward bending. Now do time out 5.
5 Time out
Differential diagnosis
Referral A 14-year-old girl tells you her hips are uneven. You examine her and identify features suggestive of scoliosis. Think about what you would do in your role as a children’s nurse and write a mock referral to a spinal surgeon.
What to do when scoliosis is identified If scoliosis is suspected, the child should be referred to a spinal surgeon for assessment, monitoring and treatment, if necessary. Children and their families will be understandably concerned and many will search for further information online. The quality of such information varies, so it would be helpful if healthcare professionals had information about their local services to enable them to guide children and families towards these, to ensure information is relevant and accurate. Like many regional scoliosis centres, the Paediatric Scoliosis and Spinal Service of the South Tees Hospitals NHS Foundation Trust has a website with information leaflets available for patients and families (www. southtees.nhs.uk/services/orthopaedics/paediatric-spinal). Families could also be given the contact information of the Scoliosis Association UK (www.sauk.org.uk). Emphasis should be made on how scoliosis cannot be
References Adams W (1882) Lectures on the Pathology and Treatment of Lateral and Other Forms of Curvature of the Spine. Churchill, London. Adobor RD et al (2012) Scoliosis detection, patient characteristics, referral patterns and treatment in the absence of a screening programme in Norway. Scoliosis. 7, 1, 18. Ahuja A et al (2010) Living With a Spinal Deformity: Perception of Deformity and Expectations from Treatment. Lambert Academic Publishing, Saarbrücken, Germany. Ali Fazal M, Edgar M (2006) Detection of adolescent idiopathic scoliosis. Acta Orthopaedica Belgica. 72, 2, 184-186. Arlet V, Reddi V (2007) Adolescent idiopathic scoliosis. Neurosurgery Clinics of North America. 18, 2, 255-259. Bee HL, Lloyd DG (2013) The Developing Child. Thirteenth edition. Pearson Education, Harlow. Bono CM (2007) Bracing and non-operative treatment of spinal deformity. In Heary RF, Albert TJ (Eds) Spinal Deformities:
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The Essentials. Thieme Medical Publishers, New York NY. Bunnell WP (1986) The natural history of idiopathic scoliosis before skeletal maturity. Spine. 11, 8, 773-776. Bushby K et al (2010) Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurology. 9, 1, 77-93.
Goffman E (1963) Stigma: Notes on the Management of Spoiled Identity. Prentice-Hall, New Jersey NY.
Konieczny et al (2013) Epidemiology of adolescent idiopathic scoliosis. Journal of Children’s Orthopaedics. 7, 1, 3-9.
Gorman KF et al (2012) The genetic epidemiology of idiopathic scoliosis. European Spine Journal. 21, 10, 1905-1919.
Kotwicki T et al (2008) Bracing for neuromuscular scoliosis: orthosis construction to improve the patient’s function. Disability and Rehabilitation. Assistive Technology. 3, 3, 161-169.
Greene K et al (1996) The utility of understanding adolescent egocentrism in designing health promotion messages. Health Communication. 8, 2, 131-152.
Lenke LG (2007) The Lenke classification system of operative adolescent idiopathic scoliosis. Neurosurgery Clinics of North America. 18, 2, 199-206.
Coillard et al (2010) SpineCor treatment for Juvenile Idiopathic Scoliosis: SOSORT award 2010 winner. Scoliosis. 5, 25. doi: 10.1186/1748-7161-5-25.
Grivas TB et al (2006) Association between adolescent idiopathic scoliosis prevalence and age at menarche in different geographic latitudes. Scoliosis. 1, 9.
Duval-Beaupère G (1970) Les reperes maturation dans la surveillance des scoliosis [Maturation indices in the surveillance of scoliosis]. Revue de Chirurgie Orthopedique et Reparatrice de L’Appareil Moteur. 56, 1, 59-76.
Grivas TB et al (2007) SOSORT consensus paper: school screening for scoliosis. Where are we today? Scoliosis. 2, 1, 17.
Friberg O (1983) Clinical symptoms and biomechanics of lumbar spine and hip joint in leg length inequality. Spine. 8, 6, 643-651.
Hart ES et al (2010) Scheuermann’s thoracic kyphosis in the adolescent patient. Orthopaedic Nursing. 29, 6, 365-371. Hresko MT (2013) Idiopathic scoliosis in adolescents. New England Journal of Medicine. 368, 9, 834-841.
Lenke LG et al (2002) Curve prevalence of a new classification of operative adolescent idiopathic scoliosis: does classification correlate with treatment? Spine. 27, 6, 604-611. Lloyd-Roberts GC, Pilcher MF (1965) Structural idiopathic scoliosis in infancy: a study of the natural history of 100 patients. Journal of Bone and Joint Surgery. 47, 3, 520-3. Loder RT (2003) Congenital scoliosis and kyphosis. In DeWald RL (Ed) Spinal Deformities: The Comprehensive Text. Thieme Medical Publishers, New York NY.
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Time out
6
Information and support Look at the Scoliosis Association UK website (www.sauk.org.uk) and browse the information available. In addition, find out who is in your local scoliosis team, so that you know to whom to refer patients.
Although scoliosis is essentially a painless deformity, mild back pain is often reported (Adobor 2012), possibly due to muscle imbalance. To determine urgency of treatment, and therefore referral, a history of severe back pain, radiating limb pain, motor or sensory changes, and any bladder or bowel problems should be obtained. These symptoms warrant a more urgent referral as they could indicate a spinal cord tumour, tethered spinal cord, spondylolisthesis, spondylolysis or Chiari malformation with a syrinx (Hresko 2013).
Conclusion Scoliosis is the most common spinal deformity and usually affects adolescents, although younger children
Lonstein JE (1994) Adolescent idiopathic scoliosis. The Lancet. 344, 8934, 1407-1412. Lonstein JE, Carlson JM (1984) The Prediction of curve progression in untreated idiopathic scoliosis during growth. Journal of Bone and Joint Surgery. 66, 7, 1061-1071. Lonstein JE et al (1982) Voluntary school screening for scoliosis in Minnesota. Journal of Bone and Joint Surgery. 64, 4, 481-488. Miller NH (1999) Cause and natural history of adolescent idiopathic scoliosis. Orthopedic Clinics of North America. 30, 3, 343-352. Miyanji F (2014) Adolescent idiopathic scoliosis: current perspectives. Orthopedic Research and Reviews. 6, 17-26. doi: http://dx.doi.org/10.2147/ORR.S37321 Nachemson A (1968) A long term follow-up study of non-treated scoliosis. Acta Orthopaedica Scandinavica. 39, 4, 466-476.
and those with pre-existing neuromuscular or syndromic conditions can also be affected. Treatment is in the form of monitoring, bracing or surgery, or any combination of these approaches. Progression varies and depends on various factors, such as skeletal maturity, gender and type of curve. Early identification of scoliosis can enable prompt, effective treatment, which may reduce the need for major surgery for some children. Raising awareness and sharing knowledge of scoliosis is an important task for children’s nurses working in children’s spinal services. Now do time out 7.
7 Time out
prevented and has no known cause, as many parents feel guilt because they failed to recognise the deformity earlier. The spinal surgeon will request a standing posteroanterior and lateral X-ray of the whole spine, including the hips, so it would be helpful if this can be organised before the referral. A referral should include a brief medical history, information about any pain experienced, medications prescribed, relevant family history and features suggestive of scoliosis. Now do time out 6.
Self-assessment questionnaire Now that you have completed the article you might like to complete the questionnaire on page 38. To write a practice profile, go to ncyp.rcnpublishing.com
Further information ■■ What is scoliosis? Scoliosis Association (UK): www.sauk.org.uk/about-scoliosis/what-is-scoliosis. html ■■ Paediatric scoliosis and spinal service, South Tees Hospitals NHS Foundation Trust: www.southtees. nhs.uk/services/orthopaedics/paediatric-spinal ■■ Neuromuscular Scoliosis, Scoliosis Research Society: www.srs.org/patient_and_family/scoliosis/ neuromuscular_scoliosis
Nachemson A et al (1982) Report of the Scoliosis Research Society Prevalence and Natural History Committee. Scoliosis Research Society, Milwaukee WI.
Scoliosis Association (UK) (2012) Congenital Scoliosis. www.sauk.org.uk/ about-scoliosis/congenital-scoliosis. html (Last accessed: May 9 2014.)
Nilsonne U, Lundgren KD (1968) Longterm prognosis in idiopathic scoliosis. Acta Orthopaedica Scandinavica. 39, 4, 456-465.
Scoliosis Research Society (2014) Neuromuscular Scoliosis. tinyurl.com/srs-nmscoliosis (Last accessed: May 8 2014.)
Parent S et al (2005) Adolescent idiopathic scoliosis: etiology, anatomy, natural history, and bracing. Instructional Course Lectures. 54, 529-536.
Solomon BD (2011) Vacterl/Vater Association. Orphanet Journal of Rare Diseases. 6, 56.
Reamy BV, Slakey JB (2001) Adolescent idiopathic scoliosis: review and current concepts. American Family Physician. 64, 1, 111-116. Rogers R (2008) Managing Persistent Pain in Adolescents – A Handbook for Therapists. Radcliffe, Oxford. Rohmiller MT, Akbarnia BA (2007) Infantile scoliosis. In Heary RF, Albert TJ (Eds) Spinal Deformities: The Essentials. Thieme Medical Publishers, New York NY.
NURSING CHILDREN AND YOUNG PEOPLE
Soucacos PN et al (1998) Assessment of curve progression in idiopathic scoliosis. European Spine Journal. 7, 4, 270-277. Sparrow DB et al (2012) A mechanism for gene-environment interaction in the etiology of congenital scoliosis. Cell. 149, 2, 295-306. Staheli LT (2008) Fundamentals of Pediatric Orthopedics. Fourth edition. Lippincott Williams & Wilkins, Philadelphia PA. Thometz JG, Simon SR (1988) Progression of scoliosis after skeletal maturity in institutionalized adults who have cerebral palsy. Journal of Bone and Joint Surgery. 70, 9, 1290-1296.
Acknowledgement Thank you to Raman Kalyan, Waleed Hekal and Charles Greenough, consultant spine surgeons, James Cook University Hospital, for sharing their expert knowledge and experience Conflict of interest None declared
Tolo VT (2003) Clinical evaluation for neuromuscular scoliosis and kyphosis. In DeWald RL (Ed) Spinal Deformities: The Comprehensive Text. Thieme Medical Publishers, New York NY. UK National Screening Committee (2011) Screening for Adolescent Idiopathic Scoliosis. External Review Against Programme Appraisal Criteria for the UNSC (UK NSC). tinyurl.com/ nsc-adol-idio-scol (Last accessed: May 8 2014.) Weinstein SL et al (2003) Health and function of patients with untreated idiopathic scoliosis: A 50 year natural history study. Journal of the American Medical Association. 289, 5, 559-57. Weinstein SL et al (2008) Adolescent idiopathic scoliosis. The Lancet. 371, 9623, 1527-1537. Weinstein S et al (2013) Effects of bracing in adolescents with idiopathic scoliosis. New England Journal of Medicine. 369, 16, 1512-1521. Wolpert DK (2006) Scoliosis Surgery: The Definitive Patient’s Reference. Third edition. Swordfish Communications, Austin TX.
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