Edit concept Question Editor Create issue ticket

Scoliosis

Scolioses

Scoliosis is the lateral curvature and three dimensional rotation of the spinal column.


Presentation

Scoliosis has characteristic physical findings. It presents as an abnormal shape and symmetry of the back, sometimes with either a very prominent rib or a bulging shoulder blade. Sometimes there may be a hump at the back. On examination, posture appears to be distorted and limb length may appear unequal. Ramirez and his coworkers from the Texas Scottish Rite Hospital studied more than 2400 patients with scoliosis and found that a full 23% (560 of 2442 patients) had back pain at the time of presentation [7]. Some patients may also suffer from lung hypoplasia and compressed respiratory tree or other thoraco-abdominal organs.

Cerebral Palsy
  • A reassessment of spinal stabilization in severe cerebral palsy. Journal of Pediatric Orthopedics 1994; 14( 6): 731-9. Comstock CP, Leach J, Wenger DR. Scoliosis in total-body-involvement cerebral palsy.[web.archive.org]
  • OBJECTIVE: Scoliosis is commonly found in children with cerebral palsy. Many patients with cerebral palsy and scoliosis undergo intrathecal baclofen (ITB) pump placement.[ncbi.nlm.nih.gov]
  • palsy, we reviewed retrospectively the cases of fifty-one patients.[doi.org]
  • We present the case of a 17-year-old female patient with cerebral palsy who had ITB therapy from the age of 11 years. During this period, she developed a severe scoliosis measuring 86 from T11 to L4, with pain due to costo-pelvic impingement.[ncbi.nlm.nih.gov]
  • To study the natural history of scoliosis in institutionalized adults who have cerebral palsy, we reviewed retrospectively the cases of fifty-one patients.[ncbi.nlm.nih.gov]
Back Pain
  • PATIENT CONCERNS: A 54-year-old woman just complained about sever back pain without any radiculopathy and neurodeficit of low limb for 2 years, Visual Analogue Scale (VAS) for back pain was 9 points and x-ray showed adult LDS with lordosis angle of 10[ncbi.nlm.nih.gov]
  • Untreated LIS causes little physical impairment other than back pain and cosmetic concerns.[ncbi.nlm.nih.gov]
  • KEYWORDS: Braces; Case report; Low back pain; Outcome assessment; Rehabilitation; Scoliosis[ncbi.nlm.nih.gov]
  • Current literature does not support the recent trend to save motion segments (selective fusion), and no significant association was found between the distal level of fusion and the incidence of low back pain.[ncbi.nlm.nih.gov]
  • I disagree, however, with their conclusion that there is "little physical impairment other than back pain and cosmetic concern."[jama.ama-assn.org]
Torticollis
  • The manifestations of the WS in this patient are severe: she has torticollis, and an extremely severe scoliosis. In addition, she is short (-3 SD; parental target height 0.8SD) and has mixed sensorineural and conductive deafness.[ncbi.nlm.nih.gov]
Leg Length Inequality
  • This may cause crowding of the ribs on one side. scoliosis S-shaped spinal curvature in frontal plane, causing muscle/soft-tissue compensation, e.g. flexible (soft-tissue contracture) or fixed (bony ankylosis) deformity; causes apparent leg length inequality[medical-dictionary.thefreedictionary.com]
Hip Pain
  • Pain in the areas surrounding the spine, including the shoulder, pelvis, and hip. Pain with specific movement or activity.[moveforwardpt.com]
Dystonia
  • Abstract Segawa's syndrome or dopa-responsive dystonia is a rare hereditary disorder characterized by progressive dystonia of childhood onset, diurnal fluctuation of symptoms and complete or near complete alleviation of symptoms with administration of[ncbi.nlm.nih.gov]
  • A workup revealed generalized primary torsion dystonia. The condition was refractory to medical treatment and necessitated implantation of a deep brain stimulator.[ncbi.nlm.nih.gov]
Guillain-Barré Syndrome
  • Abstract Guillain-Barré syndrome (GBS) is an acute autoimmune inflammatory demyelinating polyneuropathy that mostly affects the peripheral nervous system. Little is reported about spinal deformity associated with GBS.[ncbi.nlm.nih.gov]
Retrograde Ejaculation
  • ejaculation in men – where sperm travels backwards into the bladder when you ejaculate rather than out of your penis; this happens as a result of nerve damage and occurs in an estimated 1 in 100 cases More serious nerve damage can result in the loss[web.archive.org]

Workup

Laboratory tests

A scoliometer may be used to diagnose the condition. Genetic testing may also be conducted, if needed. Tests may also include hematocrit and haemoglobin levels. Serum alkaline phosphatase levels and vitamin D levels may also be assessed.

Imaging

  • Full spine AP/Coronal CT scan
  • Lateral/saggital plane X-rays
  • MRI

Test results

On the basis of physical examination and imaging studies, scoliosis can be diagnosed and the extent of rotation can be assessed.

Treatment

Conservative management

It mainly constitutes the use of braces, casts and analgesics. In a prospective, multicenter study from the Scoliosis Research Society, Nachemson and his coworkers found brace treatment (an underarm plastic brace worn for at least 16 h/d) to be successful 74% of the time (95% confidence interval [CI], 52-84%) [8]. The results of a study conducted for infantile scoliosis patients suggest that body casting is useful in cases of smaller, flexible spinal curves, and vertical expandable prosthetic titanium rib
(VEPTR) is a viable alternative for larger curves [9].

Surgery

Spinal fusion is the most commonly used surgical treatment. For a thoracic curve with adequate flexibility, posterior spinal fusion with instrumentation is used. If the curve is large and stiff, an anterior release including diskectomy and bone grafting is performed first. Large bone screws, pedicle screws and hooks are used to surgically derotate the spine. Endoscopic spinal instrumentation techniques have also been introduced and continue to evolve [10].

Prognosis

Scoliosis begins insidiously and persists for life unless surgically corrected. It is a chronic progressive disease and may prove to be fatal. Clinical outcomes following treatment of idiopathic scoliosis are strongly linked to curve magnitude [6]. Mortality rate is associated with ‘curve effects’, meaning the distorted posture and curved spine may lead to damage of respiratory and cardiovascular organs. With appropriate treatment, mortality rate can be significantly reduced and quality of life can be substantially improved.

Etiology

Idiopathic scoliosis is the most common type of spinal deformity confronting orthopedic surgeons [2]. It has multifactorial causes, including genetics among which CHD7 gene is known to be involved. Congenital scoliosis is due to spinal malformation of the fetus between the 3rd and 6th week of development. Several neuromuscular diseases like poliomyelitis, cerebral palsy, spinomuscular atrophy, Ehlers Danlos syndrome, Marfan syndrome or neurofibromatosis may be the primary cause or associating disease in secondary or neuromuscular scoliosis.

Epidemiology

Incidence

Infantile scoliosis is a very rare condition with only 1% cases in North America. Idiopathic scoliosis on the other hand is a relatively common condition affecting 2-3% of the United States population, which is equivalent to about 5 to 9 million cases [3].

Age

The prevalence of scoliosis was highest (1.2%) in patients aged 12-14 years [4].

Sex

In early-onset infantile scoliosis, 60% males are affected. In late-onset infantile scoliosis, 90% females are affected. In idiopathic scoliosis, females are at a greater risk.

Sex distribution
Age distribution

Pathophysiology

The pathophysiology of scoliosis is still unclear. The spinal curvature that characterizes scoliosis may be due to malformation occurring during intrauterine development, resulting in infantile scoliosis. This type includes scoliosis appearing either at birth or within the first 3 years of life. Infantile scoliosis, along with genetic factors, is also associated with nursing posture and the side of deviation of the curve.

Neuromuscular scoliosis may be a result of other preexisting neuromuscular diseases such as Ehlers-Danlos syndrome, Marfan syndrome, neurofibromatosis, etc. The location is defined by the vertebra that is most deviated and rotated from midline, called the apical vertebra [5].

Whatever the type of scoliosis it may be, it is known that there are segments of the spinal column that are misaligned and these fusion defects result in the development of curvature of the spine to one side, which then progresses to three dimensional rotation and the resultant ‘S’ shaped spinal column.

Prevention

Scoliosis may be prevented by maintaining correct posture and engaging in daily exercise and a healthy diet. Congenital scoliosis, however, has no known prevention but its progress can be halted or at least somewhat managed with appropriate treatment. Neuromuscular scoliosis can be prevented from occurring by adequately managing the underlying cause or associating disease with medication and physiotherapy.

Summary

Scoliosis is defined as a lateral curvature of the spine, most often than not accompanied by rotation. By convention, 10 degree of curvature (as measured by the Cobb angle) defines a scoliosis [1]. Spinal curvatures that are of less than 10 degree are simply referred to as spinal asymmetry. On imaging, the spinal deformity appears to be of an ‘S’ shape. Scoliosis may be broadly classified into 3 types: Congenital, idiopathic and neuromuscular. All three are described below.

Patient Information

Definition

Scoliosis is defined as lateral or sideways curving and rotation of the spine. The curvature needs to be equal to or more than 10 degrees for the deformity to be classified as scoliosis. It may be present since birth, called infantile scoliosis or it may develop later on in life. The late-onset scoliosis is often a result of predisposing conditions.

Cause

The most common type of scoliosis is idiopathic, meaning it has no known cause. Neuromuscular scoliosis may be due to other diseases affecting the body’s structure. Infantile scoliosis is due to genetic factors and in some rare cases, it may be due to trauma during pregnancy.

Signs and symptoms

Scoliosis presents as abnormal shape and symmetry of the spine which may be accompanied with a hump and distorted posture. There may be protrusion of a rib or shoulder blade and unequal length of arms. Patients experience back pain which is exacerbated with movement and their physical activity becomes somewhat restricted.

Treatment

Scoliosis can be conservatively managed with the help of casts and braces and painkillers. In severe cases, the curved spine may have to be corrected with the help of surgery which includes insertion of screws and rods to straighten the spine. Surgery is followed with physiotherapy to help relax and strengthen the muscles.

References

Article

  1. Kane WJ. Scoliosis prevalence: a call for a statement of terms. Clin Orthop Relat Res. 1977;126:43-46.
  2. Lonstein JE. Idiopathic scoliosis. In: Lonstein JE, Bradfordn DS, Winter RB, Ogilvie J, eds. Moe's Textbook of Scoliosis and Other Spinal Deformities. 3rd ed. Philadelphia, PA: WB Saunders Co; 1995:219-256.
  3. Negrini S, Aulisa AG, Aulisa L, et al. 2011 SOSORT guidelines: Orthopaedic and Rehabilitation treatment of idiopathic scoliosis during growth. Scoliosis. 2012;7(3):1-35.
  4. Stirling AJ, Howel D, Millner PA, et al. Late-onset idiopathic scoliosis in children six to fourteen years old. A cross-sectional prevalence study. J Bone Joint Surg Am. 1996;78(9):1330-1336.
  5. Tachdijan MO. The Spine. In: Clinical Pediatric Orthopedics: The Art of Diagnosis and Principles of Management. Stanford, CA: Appleton & Lange; 1997:p325.
  6. Tsutsui S, Pawelek J, Bastrom T, et al. Dissecting the effects of spinal fusion and deformity magnitude on quality of life in patients with adolescent idiopathic scoliosis. Spine. 2009;34(18):E653-E658.
  7. Ramirez N, Johnston CE, Browne RH. The prevalence of back pain in children who have idiopathic scoliosis. J Bone Joint Surg Am. 1997;79(3):364-368.
  8. Nachemson AL, Peterson LE. Effectiveness of treatment with a brace in girls who have adolescent idiopathic scoliosis. A prospective, controlled study based on data from the Brace Study of the Scoliosis Research Society. J Bone Joint Surg Am. 1995;77(6):815-822.
  9. Smith JR, Samdani AF, Pahys J, et al. The role of bracing, casting, and vertical expandable prosthetic titanium rib for the treatment of infantile idiopathic scoliosis: a single-institution experience with 31 consecutive patients. J Neurosurg Spine. 2009;11(1):3-8.
  10. Picetti G 3rd, Blackman RG, O'Neal K, Luque E. Anterior endoscopic correction and fusion of scoliosis. Orthopedics. 1998;21(12):1285-1287.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2017-10-31 15:12