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Scoliosis (from Greek: skoliōsis meaning "crooked condition," from skolios, "crooked") is a medical condition in which a person's spine is curved from side to side and may also be rotated. On an x-ray, viewed from the rear, the spine of an individual with a typical scoliosis may look more like an "S" or a "C" than a straight line. It is typically classified as either congenital (caused by vertebral anomalies present at birth), idiopathic (cause unknown, sub-classified as infantile, juvenile, adolescent, or adult according to when onset occurred) or neuromuscular (having developed as a secondary symptom of another condition, such as spina bifida, cerebral palsy, spinal muscular atrophy or physical trauma).

Cause

In the case of the most common form of scoliosis, adolescent idiopathic scoliosis, there is no clear causal agent . Various causes have been implicated, but none have consensus among scientists as the cause of scoliosis. The role of genetic factors in the development of this condition is widely accepted. Scoliosis is more often diagnosed in females and is often seen in patients with cerebral palsy or spina bifida,which is a birth defect that involves the incomplete development of the spinal cord and its coverings , although this form of scoliosis is different from that seen in children without these conditions. In some cases, scoliosis exists at birth due to a congenital vertebral anomaly. Occasionally, development of scoliosis during adolescence is due to an underlying anomaly such as a tethered spinal cord, but most often the cause is unknown or idiopathic, having been inherited through genetics . Some therapists like the referenced Hanna Somatic therapist believe that trauma to an adult can cause, not just asymmetry but an actual curve to the spine visible on x-ray, although no documentation is offered in her article. Scoliosis often presents itself, or worsens, during the adolescence growth spurt. During adolescence, due to rapid growth of the body, hip and leg proportions in the leg and thigh may become misaligned, causing temporary acute scoliosis.

In April 2007, researchers at Texas Scottish Rite Hospital for Children identified the first gene associated with idiopathic scoliosis, CHD7. The medical breakthrough was the result of a 10-year study and is outlined in the May 2007 issue of the American Journal of Human Genetics.

Prevalence

Scoliotic curves of 10° or less affect 3-5 out of every 1,000 people. The prevalence of curves less than 20° is about equal in males and females. 2% of women and 0.5% of men are affected by Scoliosis.

Symptoms

Patients who have reached skeletal maturity are less likely to have a worsening case. Some of the severe cases of scoliosis can lead to diminishing lung capacity, putting pressure on the heart, and restricting physical activities.The symptoms of scoliosis can include:
  • Uneven musculature on one side of the spine
  • A rib "hump" (Pectus carinatum) and/or a prominent shoulder blade, caused by rotation of the ribcage in thoracic scoliosis
  • Uneven hip, rib cage, and shoulder levels
  • Asymmetric size or location of breast in females
  • Unequal distance between arms and body
  • Slow nerve action (in some cases)


Associated conditions

Scoliosis is sometimes associated with other conditions such as Ehler-Danlos Syndrome (hyperflexibility, 'floppy baby' syndrome, and other variants of the condition), Charcot-Marie-Tooth, kyphosis, cerebral palsy, spinal muscular atrophy, muscular dystrophy, familial dysautonomia, CHARGE syndrome, Friedreich's ataxia, proteus syndrome, Spina bifida, Marfan's syndrome, neurofibromatosis, connective tissue disorders, congenital diaphragmatic hernia, and craniospinal axis disorders (e.g., syringomyelia, mitral valve prolapse, Arnold-Chiari malformation).

Investigation

who initially present with scoliosis are examined to determine whether there is an underlying cause of the deformity. During a physical examination, the following is assessed:

During the exam, the patient is asked to remove his shirt and bend forward (this is known as the Adam's Bend Test and is often performed on school students). If a hump is noted, then scoliosis is a possibility and the patient should be sent for an x-ray to confirm the diagnosis. Alternatively, a scoliometer may be used to diagnose the condition.The patient's gait is assessed, and there is an exam for sign of other abnormalities (e.g., Spina bifida as evidenced by a dimple, hairy patch, lipoma, or hemangioma).A thorough neurological examination is also performed.

It is usual when scoliosis is suspected to arrange for weight-bearing full-spine AP/coronal (front-back view) and lateral/sagittal (side view) xrays to be taken, to assess both the scoliosis curves and also the kyphosis and lordosis, as these can also be affected in individuals with scoliosis. Full-length standing spine X rays are the standard method for evaluating the severity and progression of the scoliosis, and whether it is congenital or idiopathic in nature. In growing individuals, serial radiographs are obtained at 3-12 month intervals to follow curve progression. In some instances, MRI investigation is warranted.

The standard method for assessing the curvature quantitatively is measurement of the Cobb angle, which is the angle between two lines, drawn perpendicular to the upper endplate of the uppermost vertebrae involved and the lower endplate of the lowest vertebrae involved. For patients who have two curves, Cobb angles are followed for both curves. In some patients, lateral bending xrays are obtained to assess the flexibility of the curves or the primary and compensatory curves.

Genetic testing for AIS, which has become available in 2009, greatly improves the ability of physicians to accurately predict the likelihood of curve progression.

Mass-screening for scoliosis using posture photos
It has been suggested that entire populations be examined, for early detection. For example, in the 1940s, American psychologist William Sheldon proposed mandatory physical examinations that included nude photographs of each person being examined. One purpose of these photographs was the detection of rickets, scoliosis, and lordosis. His approach was implemented at a number of ivy league schools in which all freshmen were examined (Ivy League nude posture photos). A similar program was implemented in Boston's prison system.

Prognosis

The prognosis of scoliosis depends on the likelihood of progression. The general rules of progression are that larger curves carry a higher risk of progression than smaller curves, and that thoracic and double primary curves carry a higher risk of progression than single lumbar or thoracolumbar curves. In addition, patients who have not yet reached skeletal maturity have a higher likelihood of progression.

Genetic Testing for Adolescent Idiopathic Scoliosis
Through a genome-wide association study, geneticists have identified single nucleotide polymorphism markers in the DNA that are significantly associated with Adolescent Idiopathic Scoliosis. Genetic testing for AIS now allows many AIS patients to find out, with great accuracy, their own likelihood of progression to a severe curve.

Management

The traditional medical management of scoliosis is complex and is determined by the severity of the curvature and skeletal maturity, which together help predict the likelihood of progression.

The conventional options are, in order:
  1. Observation
  2. Bracing
  3. Surgery


Bracing is normally done when the patient has bone growth remaining and is generally implemented to hold the curve and prevent it from progressing to the point where surgery is indicated. Braces are sometimes prescribed for adults to relieve pain. Bracing involves fitting the patient with a device that covers the torso; in some cases it extends to the neck. The most commonly used brace is a TLSO, a corset-like appliance that fits from armpits to hips and is custom-made from fiberglass or plastic. It is usually worn 22–23 hours a day and applies pressure on the curves in the spine. The effectiveness of the brace depends not only on brace design and orthotist skill, but on patient compliance and amount of wear per day. Typically, braces are used for idiopathic curves that are not grave enough to warrant surgery, but they may also be used to prevent the progression of more severe curves in young children, to buy the child time to grow before performing surgery, which would prevent further growth in the part of the spine affected. Bracing may cause emotional and physical discomfort. Physical activity may become more difficult because the brace presses against the stomach, making it difficult to breathe. Children may lose weight from the brace, due to increased pressure on the abdominal area.

In infantile and sometimes juvenile scoliosis, a plaster jacket applied early may be used instead of a brace. It has been proven possible to permanently correct cases of infantile idiopathic scoliosis by applying a series of plaster casts (EDF: elongation, derotation, flexion) applied on a specialized frame under corrective traction, which helps to "mould" the infant's soft bones and work with their growth spurts. This method was pioneered by UK scoliosis specialist Min Mehta.

Conventional chiropractic and physical therapy have some degree of anecdotal success in treating scoliosis that is primarily neuromuscular in nature. Non-surgical approaches will not address severe bone deformities associated with some cases of scoliosis. Chiropractors and physical therapists use joint mobilization techniques and therapeutic exercise to increase a scoliosis patient's flexibility and strength, theorizing that this better enables the brace to influence the curvature of the spine. Electrical muscle stimulation (EMS) is another therapeutic modality commonly used by chiropractors and physical therapists to reduce muscle spasms and strengthen atrophied muscles.

A growing body of scientific research testifies to the efficacy of specialized treatment programs of physical therapy, which may include bracing. Debate in the scientific community about whether chiropractic and physical therapy can influence scoliotic curvature is partly complicated by the variety of methods proposed and employed: Some are supported by more research than others.

The Schroth Method is one non-invasive, physiotherapeutic treatment for scoliosis which has been used successfully in Europe since the 1920s. Originally developed in Germany by scoliosis sufferer Katharina Schroth, this method is now taught to scoliosis patients in clinics specifically devoted to Schroth therapy in Germany, Spain, England and, most recently, the United States. The method is based upon the concept of scoliosis as resulting from a complex of muscular asymmetries (especially strength imbalances in the back) that can be at least partially corrected by targeted exercises.

Surgery

Surgery is usually indicated for curves that have a high likelihood of progression, curves that cause a significant amount of pain with some regularity, curves that would be cosmetically unacceptable as an adult, curves in patients with spina bifida and cerebral palsy that interfere with sitting and care, and curves that affect physiological functions such as breathing.

Surgery for scoliosis is usually done by a surgeon who specializes in spine surgery. For various reasons it is usually impossible to completely straighten a scoliotic spine, but in most cases very good corrections are achieved.

Surgery is usually required with a curve of 40 to 50 degrees.

Spinal fusion with instrumentation

Spinal fusion is the most widely performed surgery for scoliosis. In this procedure, bone (either harvested from elsewhere in the body autograft, or donor bone allograft) is grafted to the vertebrae so that when it heals, they will form one solid bone mass and the vertebral column becomes rigid. This prevents worsening of the curve at the expense of spinal movement. This can be performed from the anterior (front) aspect of the spine by entering the thoracic or abdominal cavity, or performed from the back (posterior). A combination of both is used in more severe cases.

Originally, spinal fusions were done without metal implants. A cast was applied after the surgery, usually under traction to pull the curve as straight as possible and then hold it there while fusion took place. Unfortunately, there was a relatively high risk of pseudarthrosis (fusion failure) at one or more levels and significant correction could not always be achieved. In 1962, Paul Harrington introduced a metal spinal system of instrumentation which assisted with straightening the spine, as well as holding it rigid while fusion took place. The original, now obsolete Harrington rod operated on a ratchet system, attached by hooks to the spine at the top and bottom of the curvature that when cranked would distract, or straighten, the curve. A major shortcoming of the Harrington method was that it failed to produce a posture where the skull would be in proper alignment with the pelvis and it didn't address rotational deformity. As a result, unfused parts of the spine would try to compensate for this in the effort to stand up straight. As the person aged, there would be increased wear and tear, early onset arthritis, disc degeneration, muscular stiffness and pain with eventual reliance on painkillers, further surgery, inability to work full-time and disability. "Flatback" became the medical name for a related complication, especially for those who had lumbar scoliosis. Modern spinal systems are attempting to address sagittal imbalance and rotational defects unresolved by the Harrington rod system. They involve a combination of rods, screws, hooks and wires fixing the spine and can apply stronger, safer forces to the spine than the Harrington rod. This technique is known as the Cotrel-Dubousset instrumentation, currently the most common technique for the procedure.

Modern spinal fusions generally have good outcomes with high degrees of correction and low rates of failure and infection. Patients with fused spines and permanent implants tend to have normal lives with unrestricted activities when they are younger, it remains to be seen whether those that have been treated with the newer surgical techniques will develop problems as they age.A notable limitation of spinal fusions is that patients who have undergone surgery for scoliosis are ineligible for service in the military of countries such as the United Kingdommarker, Swedenmarker and the United Statesmarker.

In cases where scoliosis has caused a significant deformity resulting in a rib hump, it is often possible to perform a surgery called a costoplasty (also called a thorocoplasty) in order to achieve a more pleasing cosmetic result. This procedure may be performed at any time after a fusion surgery, whether as part of the same operation or several years afterwards. It is usually impossible to completely straighten and untwist a scoliotic spine, and it should be noted that the level of cosmetic success will depend on the extent to which the fused spine still rotates out into the ribcage. A rib hump is evidence that there is still some rotational deformity to the spine. Specific weight training techniques can be used to influence this rotational deformity in the unfused parts of the spine. This leads to a marked decrease in pain and to some improvement in organ function depending on the person's particular case and is to be recommended over any cosmetic surgical procedure .

Surgery without fusion

New implants have been developed that aim to delay spinal fusion and to allow more spinal growth in young children. For the youngest patients, whose thoracic insufficiency compromises their ability to breathe and applies significant cardiac pressure, ribcage implants that push the ribs apart on the concave side of the curve may be especially useful. These Vertical Expandable Prosthetic Titanium Ribs (VEPTR) provide the benefit of expanding the thoracic cavity and straightening the spine in all three dimensions while allowing the spine to grow. Although these methods are novel and promising, these treatments are only suitable for growing patients. Spinal fusion remains the "gold standard" of surgical treatment for scoliosis.Surgery is usually required if the spine has a curve of 40 to 50 degrees.

Alternatives

In children with immature skeletons and remaining growth potential, Schroth-method physical therapy is used in combination with the Rigo System-Cheneau brace, not only to prevent progression of (and often reduce) the abnormal curvature, but also to train and strengthen patients in holding their bodies in a corrected position after completion of the bracing treatment (i.e., when the skeleton has reached maturity). A patient’s consistent practicing of an individualized Schroth program has been clinically shown to inhibit the mechanical forces, exacerbated by poor postural habits and gravity, that otherwise perpetuate the progression of the curvature over time (the so-called “vicious cycle”), even after the cessation of physical growth..

See also



References

  1. Online Etymology Dictionary. Douglas Harper, Historian. Accessed 27 December 2008. Dictionary.com http://dictionary.reference.com/browse/scoliosis
  2. Kouwenhoven, J & Castelein, R, 2008, 'The Pathogenesis of Adolescent Idiopathic Scoliosis', Spine, vol. 33, no. 26, pp. 2898-2908. 10.1097/BRS.0b013e3181891751
  3. Ogilvie JW, Braun J, Argyle V, Nelson L, Meade M, Ward K, 2006, 'The Search for Idiopathic Genes', Spine, vol. 31, no. 6, pp. 679-81. March 2006
  4. [1]
  5. [2]
  6. Texas Scottish Rite Hospital for Children: [3]
  7. [4]
  8. Nelson L, Ward K, Ogilvie J "Adolescent Idiopathic Scoliosis and Genetic Testing" Current Opinion in Pediatrics, August 2009 (submitted)
  9. All about Criminal Motivation, by Mark Gado - Crime Library on truTV.com
  10. Nelson L, Ward K, Ogilvie J "Adolescent Idiopathic Scoliosis and Genetic Testing" Current Opinion in Pediatrics, August 2009 (submitted)
  11. Lehnert-Schroth, Christa (2007). Three-Dimensional Treatment for Scoliosis: A Physiotherapeutic Method for Deformities of the Spine. (Palo Alto, CA: The Martindale Press): pp. 1-6.
  12. Lehnert-Schroth, Christa (2007). Three-Dimensional Treatment for Scoliosis: A Physiotherapeutic Method for Deformities of the Spine. (Palo Alto, CA: The Martindale Press): passim.



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