Scoliosis comes in idiopathic, congenital, neuromuscular, and degenerative forms and can affect different age groups from newborns to adults. These types of scoliosis are found in either cervical (neck), thoracic (mid back), or lumbar (low back) spinal regions and can affect more than one region at a time, which is especially common with thoracolumbar scoliosis.
Idiopathic scoliosis, meaning that the cause is unknown, is by far the most common type of scoliosis. There is some thought that it may have a genetic or familial part. This type of scoliosis is further broken down into infantile (onset before age 3), juvenile (onset between ages of 3 and 10), adolescent (onset after age 10), and adult (onset after age 18). Idiopathic scoliosis is more frequently found in females and severe curves can continue to progress through adulthood. The curve can worsen with a large growth spurt and in these cases usually slows or stops, once growth has ceased.
Congenital scoliosis is present at birth though there has been no genetic or hereditary component found. The abnormal formation of the spine is formed in the womb; the spine forms early in development and is usually completed by the sixth week of pregnancy. Congenital scoliosis branches further into two major types: a hemivertebrae and unilateral bar. Both can affect one or multiple vertebrae.
Hemivertebrae are vertebrae that are not completely formed and are the most common type of congenital malformation. Since spinal vertebra are stacked on each other, the hemivertebrae acts as a ‘wedge’ which causes a curvature of the spine.
Unilateral bar, also called block vertebrae, is a failure of the vertebral bodies to separate into single, distinct vertebra during formation which results in a spinal fusion. Inadequate separation of the vertebral bodies can cause only one side to grow, leading to an abnormal curvature.
Both congenital abnormalities can occur together and lead to a more serious condition. Congenital scoliosis is typically detected during a new born exam after birth but can often go unnoticed until adolescence, since the spine may not curve at birth even though the malformation is present.
Neuromuscular scoliosis is a result of congenital (i.e. present at birth) neurological or muscular conditions, such as cerebral palsy, muscular dystrophy, and spina bifida: the curvature develops, as the muscles of the body are too weak to support the spine. This type of scoliosis has a larger curve and is more severe in patients who are unable to ambulate, or walk and the curve progresses rapidly.
Degenerative scoliosis, also referred to as adult scoliosis, occurs in people greater than age 18. This type of scoliosis occurs as the elements of the spinal column (e.g. intervertebral discs and facet joints) deteriorate from wear due to repetitive movements, weight gain, and arthritis. It can also be seen in people that have osteoporosis, a loss of bone density. This deterioration can cause one side of the spine to weaken, which causes a shift of the vertebral bodies and the formation of a scoliosis curve. The lumbar spine is the most commonly affected site for degenerative scoliosis, as this region of the spine bears the most body weight.
The cause in majority of scoliosis cases is idiopathic, or unknown. More rare causes include malformation of the spinal column during formation in the womb when a specific vertebra does not fully form or when multiple vertebrae fuse rather than separating. Other causes of scoliosis include neuromuscular disease such as cerebral palsy, spina bifida, and muscular dystrophy. These conditions cause weakness in the back muscles, which allows curvature of the spine. Lastly, degenerative changes can lead to formation of a scoliosis curve. Degenerative causes include arthritis, osteophyte (bone spurs) formation, disc dehydration (drying out of the intervertebral disc), failure of facet joints to form properly in the spinal column, and osteopenia or osteoporosis. These conditions can cause weakening on one side of the spinal column, leading to scoliotic curvature.
Certain modifiable and non-modifiable risk factors can increase the likelihood of developing scoliosis, especially degenerative scoliosis. Non-modifiable risk factors include gender, family history, and age. Scoliosis is more frequently diagnosed in females than in males and adolescent idiopathic scoliosis is most commonly found in children from ages 10-12. Though idiopathic scoliosis has no known cause, there are studies that have supported genetics as playing a role in its development.
Modifiable risk factors include weight as increased body weight places more stress on the spine. Over time, the added stress causes spinal joints to wear out and intervertebral discs dry, leading to degeneration of the disc and thereby degenerative scoliosis. Decreased estrogen has a negative effect on bone density and is directly related to osteopenia and osteoporosis, both of which increase risk for fracture which can cause the spine to curve. Smoking can also lead to scoliosis, as it may accelerate degenerative changes in the spine and can decrease bone density, leading to osteoporosis. In addition, smoking reduces oxygen in the blood stream, which can prevent nutrients from reaching tissues for proper healing. Smoking may also lead to breakdown of the intervertebral discs, a condition known as degenerative disc disease, which can lead to degenerative scoliosis. Lastly, high impact movement and repetitive motions from sporting activities can increase wear and tear on spinal column or lead to a vertebral body fracture which may lead to scoliosis.
Symptoms of scoliosis can range from nonexistent to debilitating symptoms of pain. The severity and underlying cause has a direct effect on the symptoms that a patient may experience. The main symptoms, across all types of scoliosis, are typically asymmetry throughout the body. One hip, rib, or shoulder may sit higher on one side of the body to compensate for the curve. This may be minimal, but is readily visible in those that have a moderate to severe curve. Unevenness, particularly in the hips, can cause abnormalities in walking and those with severe scoliosis in the thoracic (midback region) may have twisted ribs that put pressure on the lungs and heart. This can lead to difficulty breathing or pumping blood.
Idiopathic scoliosis is typically diagnosed in adolescents and is referred to as adolescent idiopathic scoliosis (AIS). This type of scoliosis is usually found during a screening examination by a pediatrician or when the parent/teacher notices unevenness in hips, ribs, or shoulders, which prompts evaluation by a medical professional. Scoliosis, in and of itself, does not cause any pain. Back pain in children is usually more related to weak core and back muscles strained over increasing participation in exercise, aerobic, and sports activities.
Congenital scoliosis may lead to unevenness of the hips, ribs, or shoulders, which may give a leaning appearance. This unevenness is not always apparent at birth even if the defect is present. Scoliosis itself does not cause pain and since the curve is not always present at birth, the diagnosis may happen later in life. Congenital scoliosis occurs due to spinal malformation early in pregnancy and development; these individuals often have other medical conditions related to the heart, kidneys, or digestive system, as proper development in these may have been disrupted as well.
Neuromuscular scoliosis is only present in those that have been diagnosed with certain conditions, such as Muscular Dystrophy, Cerebral Palsy, Spina Bifida. These lead to weakness in the musculature of the back, allowing the curvature of scoliosis to form. The symptoms rarely cause pain, but can lead to a more rapidly progressive scoliosis. The main symptoms these patients experience are difficulty controlling the trunk of their body and difficulty sitting; majority are confined to wheelchairs or other assistive devices because their disease weakens muscles in other areas of the body as well. The severity of the scoliosis may also lead to thoracic insufficiency syndrome, which can limit lung growth and cause difficulty breathing. When the lung capacity is decreased due to the scoliosis curve, the ability for respirations to occur is limited, leading to shortness of breath which necessitate more rapid breathing and thereby fatigue from breathing.
Individuals with degenerative scoliosis commonly suffer from mild to severe pain which typically starts as a lower back ache and gradually increases. The pain is commonly caused by degenerative changes, such as spinal stenosis, disc degeneration, and disc herniations from wear and tear on the spine. Disc degeneration causes the spine to curve and puts pressure on the foramen, vertebral spaces through which nerves branch from the spinal cord. Degenerative scoliosis most frequently affects the lower back and therefore common symptoms of lumbar disease, such as pain and stiffness or radiating symptoms in the buttocks, hips, legs, and feet, commonly occur. Pain tends to be worse with physical activities and better with rest. With idiopathic adolescent scoliosis, many patient may have no pain, but later in adulthood, with worsening of their curve and added degenerative changes, may experience pain.
Children are routinely screened for adolescent idiopathic scoliosis at their yearly physical exam, most commonly with the Adam’s Forward Bend Test, a technique that is easily performed at a doctor’s office or school setting. The patient stands with both feet together and the toes even. With either the shirt lifted or removed, the patient bends forward at the waist as far as they can with palms held together, as if they are going to touch their toes. The examiner then inspects the patient from behind, looking for abnormalities or differences between the left and right side of the body. A patient with scoliosis may have a shoulder, scapula, or ribs that sit higher on one side and there may be a visible curve of the spine. If the Adam Bend Test reveals abnormalities, further evaluation would be prompted.
The best way to diagnosis scoliosis is with x-ray imaging because this can uncover underlying causes including partially formed vertebral bodies, fused vertebral bodies, or lack of skeletal maturity and it can determine the degree or progression of spinal curvature. Special scoliosis x-rays show the entire spine with the shoulders and pelvis which emphasizes unevenness of the hips or shoulders in comparison to the curvature of the spine. X-rays are completed while the patient is weight bearing (standing), as this position allows gravity to act on the spine and gives the most accurate representation. The severity of scoliosis is determined by a specific angle, called the Cobb angle.
X-rays are mostly used for the diagnosis of scoliosis; 3D imaging with CT (Computed Tomography Scan) or MRI (Magnetic Resonance Imaging) scans is often needed to assess the cause. CT scans are mainly used to assess the bony anatomy of the spine; it would be used to look for any partially formed vertebral bodies, or fused vertebrae. MRI scans are primarily used to look at the soft tissue of the body, such as intervertebral discs and nerves. These scans reveal degenerative changes that could lead to scoliosis, such as facet joint disease or degenerative disc disease, and can also reveal conditions caused by scoliosis, such as disc herniation or spinal stenosis.
Conservative options are intended to relieve the pain and discomfort associated with scoliosis as well as prevent worsening curvature. The severity of the curvature is determined with x-ray imaging and the Cobb Angle measurement. Severity determines appropriate treatment options, initial management, and follow up considerations. In mild scoliosis typically no treatment is necessary and a follow up at the yearly physical is the only recommendation. For moderate scoliosis it is recommended that the patient have a follow up with x-rays every 4-6 months. In other cases conservative treatment options include bracing, physical therapy, exercise, manual therapy, medications, and steroid injections.
Traditional surgical options for scoliosis included a large, open fusion. This required a large, long incision on the back, tissue destruction, lots of blood loss, and a long operation. Recovery is slow due to the size of the incision and risk for infection increases due to the size of the incision. Severe curves with a Cobb angle greater than 40 degrees in idiopathic adolescent scoliosis, may still have a traditional surgery, as specialized hardware is placed to allow for continued growth; however, there are minimally invasive surgical options available, such as a transforaminal lumbar interbody fusion (TLIF) and an extreme lateral lumbar interbody fusion (XLIF).
A rigid brace in considered in patients if there has been a five degree Cobb angle increase in the scoliosis curve after 4-6 months or if the curve is already close to 25 degrees and the patient has a significant amount of growth left. The rigid brace does not improve the curve, but aims to prevent further curving of the spine because the scoliosis curve in patients with a Cobb angle of 40-50 degrees will continue to worsen throughout adulthood.
A rigid plastic brace, called a TLSO brace because it targets thoracic, lumbar, and sacral orthosis, is fitted from the hips to the underarms and surrounds the entire body. Daytime and nighttime use is recommended because the time spent in the brace is correlated with its ability to prevent progression. Length of treatment is typically over two years but is dependent on how much more the patient is expected to grow. Once the growth plates are closed and the patient stops growing taller, the brace is discontinued. Bracing in an adult with degenerative scoliosis cannot reverse the curve or prevent further progression of the curve, as the bones are no longer growing. Bracing in adults is not recommended because it causes core muscles of the abdomen and back to weaken. The brace prevents motion within the spine and without continued motion, the muscles begin to weaken.
Manual therapy for the spine includes both chiropractic treatment and massage. Massage can reduce pain in the muscles surrounding the spine which is useful because the malposition of the spine can create tension and frequent spasms in the muscles. Chiropractic care can aid with stretching, manipulations, and traction to provide pain relief.
Physical therapy focuses on changing body mechanics to improve posture and walking as well as to strengthen and retrain muscles, particularly those in the core. The initial step is meeting with a Physical Therapist for a consultation to assess posture, gait, and muscle weakness and to design a personalized exercise program. Other techniques that physical therapists may use are manual treatments such as massage, ultrasound, and electro-stimulation through a TENS (transcutaneous electrical nerve stimulation) unit. A combination of these methods can provide symptom relief.
A home exercise program may be used in conjunction with or instead of a formal physical therapy regimen. A daily low impact exercise regimen is important for strengthening muscles, especially those in the core. Low impact cardiovascular activities such as walking, swimming, elliptical, and biking are also important for increasing blood flow and strengthening muscles without adding stress on the spine. Home exercise can also lead to weight loss which may reduce pressure on the spine and reduce symptoms. Home exercise routines should be paired with stretches and physician supervision to avoid harm.
There are multiple types of prescribed and over the counter medications that may be used to treat the symptoms of scoliosis. Some of these medications include Tylenol (acetaminophen), NSAIDs (nonsteroidal anti-inflammatory drugs), muscle relaxants, nerve membrane stabilizers, topical medications, and off label medications.
Common over the counter medications are acetaminophen and NSAIDs. Acetaminophen is considered an analgesic, meaning it relieves pain but does not reduce inflammation. NSAIDs are effective at both relieving pain and reducing inflammation; they can be found in brand and generic forms over the counter. These medications are best taken with food to avoid an upset stomach. They can be taken for longer durations, as long as there is no history of kidney disease. The mechanism of both NSAIDs and Acetaminophen differ, so they are taken together often to combat pain.
Muscle relaxants are used to provide relief from muscle spasms and increase range of motion; they are best used in combination with physical therapy or an exercise/stretching regimen. Drowsiness is a common side effect of muscle relaxants and they are not recommended while driving or working. Common muscle relaxants include Flexeril, Soma, Baclofen, Robaxin, and Tizanidine.
Nerve membrane stabilizers are another class of medications often used to treat the numbness, tingling, shooting, stabbing, or radiating pain associated with scoliosis. Gabapentin (Neurontin) and Lyrica (Pregabalin) are commonly prescribed nerve membrane stabilizers and work by reducing pain signals released by nerves. Both medications, Gabapentin especially, can be taken in higher dosages multiple times per day, but this class of medication may cause dizziness and drowsiness. Nerve membrane stabilizers are typically started on a low and infrequent dose; the dosage is gradually increased until the pain is controlled and then gradually decreased prior to discontinuation.
There are other medications that may be used ‘off-label,’ meaning not for the use listed on the label. Some common medications used off label for pain are anti-depressants, such as Cymbalta and Amitriptyline. Similar to the nerve membrane stabilizers, these medications are slowly increased in dosage and they also target nerve-type pain, such as burning, numbness, and tingling associated with nerve pain. They may also cause drowsiness and are usually used at night for pain control.
Epidural Steroid Injections
Epidural steroid injections reduce pain by injecting steroid, or anti-inflammatory, medication at a specific level/levels in the spine. As the herniation places pressure on a nerve, this causes inflammation of the nerve and surrounding tissue which causes pain. A specialized needle is used to deliver medication to the correct area, past the muscles of the back and as close to the spinal nerves as possible. As steroids are a natural anti-inflammatory, this may reduce the inflammation around the nerve, diminishing pain caused by the disc herniation. Steroid injections have the ability to provide pain relief for up to several months, and provide better pain relief for radiating symptoms, such as leg pain, than treatments designed for back pain alone. Steroid injections are only safe to have completed 3 times in one year and must be at least one month apart between sequential injections. This is due to the muscle and tendon breakdown with exposure to the steroid medication too often or too soon.
A transverse lumbar interbody fusion (TLIF) is a minimally invasive stabilization procedure that uses a specialized implant to fix the appropriate disc level. This procedure is performed through a small incision on the midline of the lumbar spine. Once the spine is reached, the backside portion of the vertebrae is removed, the laminae, until the nerve is reached and the compression on the nerve is removed. The disc material is removed from the disc space and a special implant with bone graft is placed into the disc space with screws placed into the vertebral body above and below the disc space, that are connected via rods. Once the procedure is complete the spinal level is fused.
An extreme lateral lumbar interbody fusion, or XLIF, is a minimally invasive surgery that is performed on the lumbar spine. A small incision is made on the left side, between the hip and the ribs. A thin wire, called a guide wire, is placed and sequentially cylindrical tubes, called dilators are placed through the psoas muscle to reach the left side of the spine. Once the spine is reached, the disc is then completely removed. A special implant is sized, filled with bone graft and placed in the empty disc space. The implant is then screwed in place, with screws placed into the upper and lower vertebral body to provide structure. This will fuse and stabilize the level to prevent any further motion. The XLIF can only be performed on certain levels in the lumbar spine, as the space between the hip and lowest rib is limited, even with specialized positioning of the body on the surgical table.
All procedures used to alleviate the symptoms and structural problems from scoliosis are minimally invasive decompressive and stabilization procedures. The spinal nerve roots are decompressed properly any mal-aligned vertebral body is stabilized to prevent further motion. Multiple factors are taken into consideration to plan an appropriate surgical option to treat the structural problems associated with scoliosis.
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