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Scoliosis

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The most common deformity of the spine.

Scoliosis affects 2–3% of the United States population, about 5 to 9 million cases.

Defined as a lateral curvature of the spine that is 10 degrees or greater on a coronal x-ray image while the patient is standing.

Scoliosis spinal column’s curve of 10° or less affects 1.5% to 3% of individuals.

Age of onset is usually between 10 years and 15 years in children and adolescents, making up to 85% of those diagnosed.

Young age at onset due to rapid growth spurts occurring at puberty when spinal development is most related to genetic and environmental influences.

Viewed from the front, the spine appears to be straight, but when looked at from the side, the normal spine has two gentle S-curves.

Referred to as an abnormal curvature of the spine, where the spine curves to the side when viewed from the front, and each vertebra also twists on the next one in a corkscrew fashion.

Scoliosis affects girls twice as often. because female adolescents undergo growth spurts before postural musculoskeletal maturity, scoliosis is more prevalent among females.

About three to five of 1,000 people are affected.

Usually occurs in those older than 10 years, but can be seen in infants.

Categorized by its cause.

Congenital scoliosis is due to failure of formation or segmentation of the vertebral column, which with growth leads to progressive spinal deformity.

Neuromuscular scoliosis is caused by CNS dysfunction, dyfunction of the peripheral neuromuscular unit with muscular dystrophy, or spinal muscular atrophy, or combined sensory and motor dysfunctions.

Idioathic cause unknown, subclassified as infantile, juvenile, adolescent, or adult, according to when onset occurred.

Common in neurofibromatosis.

Common in connective tissue diseases including Marfans’s syndrome, and Ehlers-Danlos syndrome.

It is more common among females and may result from unequal growth of the two sides of one or more vertebrae, so that they do not fuse properly. 

It can also be caused by pulmonary atelectasis, as observed in asthma or pneumothorax.

Most cases are idiopathic.

65% of scoliosis cases are idiopathic, 15% are congenital and about 10% are secondary to a neuromuscular disease.

Adolescent idiopathic scoliosiis believed to be multifactorial in cause, with genetics playing a role.

CHD7 gene has been associated with the idiopathic form of scoliosis, and there is a linkage between idiopathic scoliosis and three microsatellite polymorphisms in the MATN1 gene.

The most common form of the condition, late-onset idiopathic scoliosis, is physiologically harmless and self-limiting, while the rarer forms pose risks of complications.

When patients reach skeletal maturity they are less likely to have a progressive deterioration.

Severe cases can lead to diminishing lung capacity, increasing the pressure on the heart, and restricted physical activities.

The signs of scoliosis can include: diminished musculature on one side of the spine, rib prominence and/or a prominent shoulder blade, caused by rotation of the ribcage, uneven hips, arms or leg lengths.

Associated conditions include: Charcot–Marie–Tooth disease, Prader–Willi syndrome, osteogenesis imperfecta, kyphosis, cerebral palsy, spinal muscular atrophy, muscular dystrophy, familial dysautonomia, CHARGE syndrome, Friedreich’s ataxia, fragile X syndrome, proteus syndrome, spina bifida, Marfan’s syndrome, nail–patella syndrome, neurofibromatosis, connective tissue disorders, congenital diaphragmatic hernia, hemihypertrophy, syringomyelia, mitral valve prolapse, and Arnold–Chiari malformation.

Congenital scoliosis occurs due to to a malformation of the spine during weeks three to six in utero.

Neuromuscular disease induced scoliosis may develop during adolescence, and often presents,, or worsens, during the adolescence growth spurt.

With the diagnosis of scoliosis the patient must be examined for additional findings to determine whether the deformity has an underlying cause: such findings include café au lait spots, indicative of neurofibromatosis, deformities of the feet, abnormal abdominal reflexes, and muscle spasticity.

Screening tests include the Adams forward bend test with individuals bending forward to detect abnormal prominences that may lead to x-ray confirmation of scoliosis.

A scoliometer may be used to diagnose the condition.

With scoliosis weight-bearing full-spine AP/coronal and lateral/sagittal X-rays are usually taken to assess the scoliosis curves and the kyphosis and lordosis.

Full-length standing spine X-rays are standard for evaluating the severity and progression of the disease.

In growing patients serial radiographs are obtained at three- to 12-month intervals to follow curve progression.

The 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 vertebra involved and the lower endplate of the lowest vertebra involved.

Management of scoliosis is determined by the severity of the curvature and skeletal maturity, and the likelihood of progression of disease.

The conventional options for children and adolescents are:

Observation

Bracing

Surgery

For adults, treatment usually focuses on relieving any pain.

Bracing

Surgery

Mild scoliosis with less than 30° deviation may simply be monitored and treated with exercise.

Moderately severe scoliosis of 30–45° in a child who is still growing may require bracing.

Severe curvatures may be treated surgically with spinal rod placement.

Bracing may prevent a progressive curvature.

Bracing evidence for this is not very strong, and evidence to support use of exercises or physiotherapy is weak.

Bracing is normally performed when the person has bone growth remaining.

Bracing is implemented to hold the curve and prevent it from progressing to the point where surgery is recommended.

In juveniles, some reports that bracing has reduced curves significantly, going from a 40° out of the brace to 18° in it.

Braces, for adults, may be offered to relieve pain related to scoliosis.

The most commonly used brace such as a Boston brace, is a corset-like appliance that fits from armpits to hips and is custom-made from fiberglass or plastic.

Braces can be worn more than 20hours a day, applying pressure on the curves in the spine.

The effectiveness of the brace depends on brace design, orthotist skill, and patient compliance.

Braces typically are used curves that are not grave enough to warrant surgery, but they may also be used to prevent the progression of disease.

Indications for bracing:

individuals who are still growing who present with Cobb angles less than 20° should be closely monitored.

Patients who are still growing who present with Cobb angles of 20 to 29° should be braced according to the risk of progression by considering age.

With Cobb angles greater than 30° and still growing, patients should be braced.

Bracing prevents worsening of disease.

Whether bracing changes quality of life, appearance, or back pain is unclear.

Surgery is usually recommended for curves with a high likelihood of progression to greater than 45 to 50° of magnitude, for. 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.

It is impossible to surgically completely straighten a scoliotic spine

The two main types of surgery are: Anterior or Posterior fusion.

The anterior surgical fusion approach is through an incision at the side of the chest wall.

The posterior fusion surgical approach is through an incision on the back.

The posterior fusion surgical approach involves the use of metal instrumentation to correct the spine curve.

At time both of these surgical procedures approaches may be needed.

A 50-year follow-up study found physical health, cardiopulmonary, neurological functions, and mental health of idiopathic scoliosis patients are comparable to those of the general population.

It is rare for scoliosis to interfere with normal systemic functions.

In a University of Iowa follow-up study, 91 percent of idiopathic scoliosis patients displayed normal pulmonary function, and their life expectancy was 2% longer than that of the general population.

Prognosis depends on the likelihood of progression.

It is generally held that: larger curves carry a higher risk of progression than smaller curves, and thoracic and double primary curves carry a higher risk of progression than single lumbar or thoracolumbar curves.

Patients not having yet reached skeletal maturity have a higher likelihood of progression.

Respiratory deficiencies may also arise from thoracic deformities and cause abnormal breathing, affecting exercise and work capacity, and decreases the overall quality of life.

The ‘Harrington rod’ technique was the first significant surgical procedure to manipulate spinal positioning.

Surgery may straighten the spine, it does not necessarily eradicate the pain.

Scoliosis may be a result of a bipedal stance, as a highly mobile, elongated lower spine is very beneficial.

The spine takes on an S-shaped curve with lumbar lordosis, which allows for better balance and support of an upright trunk.

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