Scoliosis
What is scoliosis?
Scoliosis is a sideways curvature of the vertebral column (the spine). The severity of scoliosis is determined on the basis of the angle of the curvature (the Cobb angle). Scoliosis also results in the rotation of the spine around its axis, causing the ribs to protrude at the back at the level of the chest: a 'gibbus deformity' or 'hunch'.
What are the complaints associated with scoliosis?
Scoliosis – certainly in children – does not in itself result in pain but it will impair lung function. Later in life, scoliosis can lead to pain and fatigue. If the scoliosis develops later, it will often cause severe pain and restricted movement. Very severe scoliosis may result in pressure on the lungs and abdominal organs.
People with intellectual disabilities sometimes find it impossible to communicate these symptoms. The people around them may then notice only a change in behaviour.
How common is scoliosis in the general population?
Idiopathic adolescent scoliosis with a Cobb angle of 10 degrees or more is seen in 3%. However, only 10% of these people need treatment.
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How common is scoliosis in people with intellectual disabilities?
Scoliosis is seen in 20-25% of people with cerebral palsy (spasticity). In 50% of them, it presents before the tenth year of life.
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In people with severe multiple disabilities, prevalence is even higher at about 60%.
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Scoliosis is also seen more often in certain syndromes, such as Rett syndrome (prevalence of 36% to 100%, increasing with age
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,
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), Prader Willi syndrome (15-43% in children
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,
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rising to 78% in adults
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) and Williams syndrome (34%)
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.
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275Scherl SA, Phillips W, Torchia MM. Clinical features, evaluation and diagnosis of adolescent idiopathic scoliosis. UpToDate, 14 April 2016.
Scherl SA, Phillips W, Torchia MM. Clinical features, evaluation and diagnosis of adolescent idiopathic scoliosis. UpToDate, 14 April 2016.
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554Braam W., Duinen-Maas M.J. van, Festen D.A.M., Gelderen, I. van, Huisman, S.A., Tonino, M.A.M. Medische zorg voor patiënten met een verstandelijke beperking. Uitgeverij Prelum, 2014.
Braam W., Duinen-Maas M.J. van, Festen D.A.M., Gelderen, I. van, Huisman, S.A., Tonino, M.A.M. Medische zorg voor patiënten met een verstandelijke beperking. Uitgeverij Prelum, 2014.
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555Rousseau MC, Mathieu S, Brisse C, Motawaj M, Grimont E, Auquier P, Billette de Villemeur T. Aetiologies, comorbidities and causes of death in a population of 133 patients with polyhandicaps cared for at specialist rehabilitation centres. Brain Inj. 2015;29(7-8):837-42.
Rousseau MC, Mathieu S, Brisse C, Motawaj M, Grimont E, Auquier P, Billette de Villemeur T. Aetiologies, comorbidities and causes of death in a population of 133 patients with polyhandicaps cared for at specialist rehabilitation centres. Brain Inj. 2015;29(7-8):837-42.
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276Huang TJ, Lubicky JP, Hammerberg KW. Scoliosis in Rett syndrome. Orthop Rev. 1994 Dec;23(12):931-7.
Huang TJ, Lubicky JP, Hammerberg KW. Scoliosis in Rett syndrome. Orthop Rev. 1994 Dec;23(12):931-7.
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556Anderson A, Wong K, Jacoby P, Downs J, Leonard H. Twenty years of surveillance in Rett syndrome: what does this tell us? Orphanet J Rare Dis. 2014 Jun 19;9:87.
Anderson A, Wong K, Jacoby P, Downs J, Leonard H. Twenty years of surveillance in Rett syndrome: what does this tell us? Orphanet J Rare Dis. 2014 Jun 19;9:87.
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277Butler JV, Whittington JE, Holland AJ, Boer H, Clarke D, Webb T. Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study. Dev Med Child Neurol. 2002 Apr;44(4):248-55.
Butler JV, Whittington JE, Holland AJ, Boer H, Clarke D, Webb T. Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study. Dev Med Child Neurol. 2002 Apr;44(4):248-55.
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278Vogels A, Fryns JP. Age at diagnosis, body mass index and physical morbidity in children and adults with the Prader-Willi syndrome. Genet Couns. 2004;15(4):397-404.
Vogels A, Fryns JP. Age at diagnosis, body mass index and physical morbidity in children and adults with the Prader-Willi syndrome. Genet Couns. 2004;15(4):397-404.
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279De Lind van Wijngaarden RFA, de Klerk LW, Festen DA, Hokken-Koelega AC. Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype. Arch Dis Child. 2008 Dec;93(12):1012-6.
De Lind van Wijngaarden RFA, de Klerk LW, Festen DA, Hokken-Koelega AC. Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype. Arch Dis Child. 2008 Dec;93(12):1012-6.
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280Odent T, Accadbled F, Koureas G, Cournot M, Moine A, Diene G, Molinas C, Pinto G, Tauber M, Gomes B, de Gauzy JS, Glorion C. Scoliosis in patients with Prader-Willi Syndrome. Pediatrics. 2008 Aug;122(2):e499-503.
Odent T, Accadbled F, Koureas G, Cournot M, Moine A, Diene G, Molinas C, Pinto G, Tauber M, Gomes B, de Gauzy JS, Glorion C. Scoliosis in patients with Prader-Willi Syndrome. Pediatrics. 2008 Aug;122(2):e499-503.
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557Laurier V, Lapeyrade A, Copet P, Demeer G, Silvie M, Bieth E, Coupaye M, Poitou C, Lorenzini F, Labrousse F, Molinas C, Tauber M, Thuilleaux D, Jauregi J. Medical, psychological and social features in a large cohort of adults with Prader-Willi syndrome: experience from a dedicated centre in France. J Intellect Disabil Res. 2015 May;59(5):411-21.
Laurier V, Lapeyrade A, Copet P, Demeer G, Silvie M, Bieth E, Coupaye M, Poitou C, Lorenzini F, Labrousse F, Molinas C, Tauber M, Thuilleaux D, Jauregi J. Medical, psychological and social features in a large cohort of adults with Prader-Willi syndrome: experience from a dedicated centre in France. J Intellect Disabil Res. 2015 May;59(5):411-21.
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558Damasceno ML, Cristante AF, Marcon RM, Barros Filho TE. Prevalence of scoliosis in Williams-Beuren syndrome patients treated at a regional reference center. Clinics (Sao Paulo). 2014 Jul;69(7):452-6.
Damasceno ML, Cristante AF, Marcon RM, Barros Filho TE. Prevalence of scoliosis in Williams-Beuren syndrome patients treated at a regional reference center. Clinics (Sao Paulo). 2014 Jul;69(7):452-6.