Kyphoscoliosis

Kyphoscoliosis is a three dimensional deformity of the spine defined as a curvature of the spine in the coronal and axial planes. This may present as an ‘s-shaped’ curvature with a combination of lateral deformity, increased kyphosis and a rotational element (Aebi, 2005).

Cause

Kyphoscoliosis can be categorised into three main causes – congenital, syndromic and idiopathic.

Congenital scoliosis (10%) refers to spinal deformity caused by abnormally formed vertebrae and is commonly associated with genitourinary anomalies.

Syndromic scoliosis (15%) is associated with a disorder of the neuromuscular, skeletal, or connective tissue systems such as:

• Neurofibromatosis
• Marfan’s syndrome
• Cerebral palsy
• Spina Bifida
• Poliomyelitis
• Osteogenesis Imperfecta
• Hunter’s syndrome

Idiopathic scoliosis (80%) has no known cause and can be subdivided based on age of onset:

• Infantile: 0 – 3 years
• Juvenile: 4 – 10 years
• Adolescent: over 10 years

Adolescent idiopathic scoliosis (AIS) is the most common spinal deformity seen (Altaf et al, 2013).

Other Causes

Adult degenerative scoliosis

Adult degenerative scoliosis may arise as a progression of any of the above (congenital, syndromic or idiopathic) or as a compensatory spinal deformity due to degenerative changes, Tuberculosis or fractures due to osteoporosis, trauma or tumour.

With a pre-existing idiopathic scoliosis further progression of the curvature may continue after skeletal maturity at about 1° per year.

Prevalence

AIS occurs in around 2 – 3% of the general population (Negrini et al, 2012) with a predominance in girls with a ratio of 9 to 1. The most common presentation is a right thoracic scoliosis (thoracic spine convex to the right).

Presentation

The presentation of the kyphoscoliosis varies in terms of the extent of the deformity and associated pain. In mild cases the deformity might not be obvious and may appear to be a mild lateral shift position due to pain and in more severe cases may present with shoulder and waistline asymmetry or rib prominence. Mild disease is usually painless but as the deformity grows the pain may increase (Negrini et al, 2012).

Cardiopulmonary problems may develop if the angle exceeds 60 - 65° and symptoms of myelopathy may develop if the angle exceeds 90°.

Physical examination of a patient presenting with kyphoscoliosis should include a baseline assessment of posture and body contour.

Inspect the back from behind with the patient standing upright with the whole back bared and the patient wearing no shoes.

• Note any curvature and difference in muscle mass between the two sides
• Shoulder asymmetry and protruding scapulae on the convex side is common
• In the case of a right thoracic scoliosis the right shoulder is rotated forward and the medial border of the right scapula protrudes posteriorly
• The hip normally protrudes on the concave side
• Often it is helpful to run a finger down the dorsal spines of the vertebral column, as it is easier to feel than to see a curve
• Adam’s forward bend test assesses the presence of a structural deformity. When positive the deformity becomes more obvious in flexion. This test can be performed in both standing and sitting

The diagnosis of kyphoscoliosis is made through imaging primarily postero-anterior and lateral x-rays of the spine in standing. A commonly used parameter is the Cobb angle which gives a measurement of the curvature (Cobb angle). As a general rule, a Cobb angle of 10° is regarded as the minimum angulation to define the presence of a scoliosis. A Cobb angle of over 40° may have surgical implications.

CT and MRI may be used to assess the spinal canal, the structure of the vertebral column and threat to the neural structures.

Society of Scoliosis Orthopaedic and rehabilitation Treatment 

First, consider supported self management (SSM) and review conservative treatment options

Link to exit/redirection and health improvement.

Long term

Timeframes/Natural History

Mild curvature in skeletally mature patients has a low risk of progression at about 2%.

More significant curvature in skeletally immature patients has a much higher risk of progression around 70% (Fusco et al, 2014; Negrini et al, 2012).

Treatment Options

Early diagnosis and intervention is beneficial.

The aims of physiotherapy treatment of AIS are:

• To prevent or treat spinal pain
• To improve aesthetics via postural correction (Negrini et al, 2012)
• To stop or prevent further curve progression at puberty
• To prevent or treat respiratory dysfunction

Exercises

High-quality evidence is lacking regarding the benefit of specific exercises (Romano et al, 2012)

One study has shown, however, that active self-correction and task-orientated exercises are superior to traditional exercises in reducing spinal deformities and improving the health-related quality of life in patients with mild AIS (Monticone et al, 2014)

Aerobic exercise, postural correction and control are also very important (SOSORT)

Surgery

Surgery may be indicated in adolescents with a curvature that has a Cobb angle greater than 45 - 50° (Altaf et al, 2013). The aims of surgery are to arrest curve progression by achieving a solid fusion to correct the deformity and to improve cosmetic appearance.

Progressing as expected (up to 3 Rxs) before discharge or onward referral.

Consider general progression/escalation advice.

Onward Referral

Onward referral to local orthopaedics departments may be considered if:

• The patient is under skeletal maturity age with undiagnosed scoliosis and signs of deformity
• There are associated symptoms of radiculopathy or neurogenic claudication (stenotic-type symptoms) not resolving with physiotherapy
• There is severe pain and significant functional restriction as a direct result of the deformity
• A tertiary referral to the National Spinal Deformity Centre, Edinburgh may occur following orthopaedic assessment.

Romano, M., Minozzi, S., Bettany-Saltikov, J., Zaina, F., Chockalingam, N., Kotwicki, T., Maier-Hennes, A. and Negrini. S. 2012. Exercises for adolescent idiopathic scoliosis. Cochrane Database Systematic Review. 2012 Aug15;8:CD007837. doi: 10.1002/14651858.CD007837.pub2. Link Here for abstract (link correct as of 13/12/19). NHS Scotland Athens username and password may be required.

Aebi, M. 2005. The adult scoliosis. European Spine,14(10): 925-48 Link Here (link correct as of 13/12/19). NHS Scotland Athens username and password may be required.

Altaf, F., Gibson, A., Dannawi, Z. And Noordeen, H. 2013. Adolescent idiopathic scoliosis. BMJ, 364: 1 -7 Link Here (link correct as of 13/12/19). NHS Scotland Athens username and password may be required.

Monticone, M., Ambrosini, E., Cazzaniga, D., Rocca, B. and Ferrante, S. 2014. Active self-correction and task-oriented exercises reduce spinal deformity and improve quality of life in subjects with mild adolescent idiopathic scoliosis. Results of a randomised controlled trial. European Spine Journal 23(6):1204-14. Link Here (link correct as of 13/12/19). NHS Scotland Athens username and password may be required.

Negrini, S., Aulisa, A.G., Aulisa, L., Circo, A.B., de Mauroy, J.C,, Durmala, J., Grivas, T.B., Knott, P., Kotwicki, T., Maruyama, T., Minozzi, S., O'Brien, J.P,, Papadopoulos, D., Rigo, M., Rivard, C.H., Romano, M., Wynne, J.H.,

Villagrasa, M., Weiss, H.R. and Zaina, F. 2012. SOSORT guidelines: Orthopaedic and Rehabilitation treatment of idiopathic scoliosis during growth. Scoliosis. 2012 Jan 20;7(1) doi: 10.1186/1748-7161-7-3 Link Here (link correct as of 13/12/19). NHS Scotland Athens username and password may be required.

Links 

Society of Orthopaedic and Rehabilitation Treatment