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Kyphoscoliosis | |
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Kyphoscoliosis in a 10-year-old girl with HSANIII. | |
Specialty | Orthopedic |
Kyphoscoliosis describes an abnormal curvature of the spine in both the coronal and sagittal planes. It is a combination of kyphosis and scoliosis. This musculoskeletal disorder often leads to other issues in patients, such as under-ventilation of lungs, pulmonary hypertension, difficulty in performing day-to-day activities, psychological issues emanating from anxiety about acceptance among peers, especially in young patients. It can also be seen in syringomyelia, Friedreich's ataxia, spina bifida, kyphoscoliotic Ehlers–Danlos syndrome (kEDS), and Duchenne muscular dystrophy due to asymmetric weakening of the paraspinal muscles.
A person with kyphoscoliosis may exhibit an abnormal hunch along with the presence of an S or C-like shape, the presence of associated disorders like hypertension and neurological disorders, or an abnormal gait.[ citation needed ]
Kyphosis by itself refers to an excessive convex curvature of the spine occurring in the thoracic and sacral regions. A normal thoracic curvature from the 1st to the 12th vertebra has a naturally occurring convex shape with angles ranging from 20 degrees to 45 degrees. When the curvature exceeds 45 degrees, the condition is termed kyphosis.[ citation needed ]
Kyphosis, depending on the extent of curvature, can result in several symptoms including breathing and digestion difficulties, cardiovascular issues, and neurological issues.[ citation needed ]
There are several forms of kyphosis, including postural (related to slouching), Scheuermann's kyphosis (found mostly in teenagers), congenital, nutritional, osteoporosis-induced and post-traumatic.[ citation needed ][ clarification needed ]
Depending on the type of kyphosis, the extent of curvature, the age of the patient, different treatments may be recommended, including the provision for posture correction, braces, physiotherapy, and surgery. Surgery can be most useful in patients who have an extensive deformity. The rate of post-surgery complications may be higher in elderly patients.[ citation needed ]
Scoliosis refers to a form of abnormal spinal curvature in which the person's spine takes an S or C shape. Scoliosis has forms of treatments available similar to those for kyphosis including bracing, physical therapy and various types of surgeries. Typically, a human spine is straight laterally, but in scoliosis patients, there may be a curve of ten degrees in either direction, left or right.[ citation needed ]
Kyphoscoliosis may manifest in an individual at different stages of life and for various causes. When present at a young age ranging from childhood to teenage, kyphoscoliosis may be present from birth due to congenital abnormalities including spina bifida.[ citation needed ]
Certain infections can also lead to the development of kyphoscoliosis such as vertebral or general tuberculosis. Osteochondrodysplasia, a disorder related to the development of bone and cartilage, can also cause this disease.[ citation needed ]
At later ages, kyphoscoliosis can occur in patients with chronic degenerative diseases like osteoporosis and osteoarthritis. This type of incidence is usually seen in patients above 50 years of age and is mainly attributed to structural changes in the spine and adjoining tissues. Sometimes, a traumatic injury can also lead to its development.[ citation needed ]
Further, there are many idiopathic occurrences of kyphoscoliosis where the exact cause is not very well known but is suspected to be caused by genetic factors.[ citation needed ]
Kyphoscoliosis is one of the main criteria in kyphoscoliotic Ehlers–Danlos syndrome. It is caused by a mutation in the PLOD1 gene or FKBP14 [1] gene. The diagnosis is confirmed by molecular testing [2] and suggested when a patient meets criteria 1 and criteria 2: congenital muscle hypotonia and congenital or early onset kyphoscoliosis (progressive or non-progressive), respectively. [3]
The decision to choose a particular treatment approach depends on multiple factors such as the age of the patient, understanding the root cause of the condition (i.e. postural, congenital, etc.), and risks involved in surgery for the patient. In addition to the below treatments, oxygen may be necessary in the long term for patients with significant hypoxemia. [4]
When the extent of curvature is mild and the underlying cause is bad posture, then physical therapy becomes the first line of treatment. Physical therapy may also be advised for other causes as well, to delay the development of abnormal curvatures, such as in the case of elderly patients with a degenerative disease of bones and cartilage. The primary objective of physical therapy is to strengthen the spinal tissues for either correcting the curvature to the maximum extent possible or limiting further damage.
In cases where the underlying reason for deformity is postural and is detected before the bones stop growing, back braces can be used. Braces provide support to the muscles and bones, as well as apply corrective pressure to reduce the curvature. Braces for kyphoscoliosis have to be designed such that they become effective with abnormal curvatures in both the coronal and sagittal planes. The latest in brace design involves the use of CAD/CAM, which is available only in a few developed countries such as Germany. The efficacy of braces for correcting curvature issues has been analyzed by a few studies. In one such study, it was found that bracing led to successful outcomes in 72% of patients and correlated strongly with hours of braces worn. [5] However, compliance with wearing braces for a period up to 18 hours or more can be physiologically and psychologically limiting, especially when one considers the teenage period.
Surgery is usually indicated when the extent of deformity is large, causes issues in physiological functions such as breathing, interferes with the daily activities or is cosmetically unacceptable to the adult. It is also advised when the patient has passed the age where other therapies such as braces are no longer effective. Kyphoscoliosis implies that the patient has both types of curvature deformity. [6]
The decision to undergo surgery is usually complex, but may become inevitable when the deformity begins to interfere with crucial physiological functions like breathing or makes everyday activities extremely painful.[ citation needed ] Sometimes, patients in their early adulthood may choose to have surgery because the presence of such deformity causes social issues such as rejection from their peers, or their disability prevents them from working. In the case of elderly patients who have such deformity triggered at a later age, other factors are to be considered such as the presence of underlying disease that caused it, whether a progressive decline is expected, and if conservative treatments using physiotherapy or drugs have failed to give relief from debilitating pain. [7]
Surgery is attempted to correct the spine and arrest progression of the deformity. [8] Surgery may include the use of spinal implants like the Harrington Rod, or the VEPTR.
A study which focused on elderly patients found that the rate of complications was much higher for a sample population of 72 cases with a mean age of 60.7 years. The rate of complications was as high as 22% in the sample. The study points out that in the case of elderly patients, surgery should only be considered when there is no other option left, the disease is in progression stage, and the quality of life has degraded to an extent where conservative treatments can no longer help with pain. [9]
While there are many surgical approaches for spinal deformity correction, including anterior only, posterior only, and anterior-posterior. The technique that is most popular nowadays[ when? ] is posterior vertebral column resection, or pVCR. [10] One of the studies which analyzed pVCR also noted the benefit of using a technique called neurogenic motor evoked potential (NMEP) monitoring in assisting the surgeon to avoid any neurological complications while performing a spine surgery. [11]
Scoliosis is a condition in which a person's spine has an abnormal curve. The curve is usually S- or C-shaped over three dimensions. In some, the degree of curve is stable, while in others, it increases over time. Mild scoliosis does not typically cause problems, but more severe cases can affect breathing and movement. Pain is usually present in adults, and can worsen with age. As the condition progresses, it may alter a person's life, and hence can also be considered a disability.
Ehlers–Danlos syndromes (EDS) are a group of 13 genetic connective-tissue disorders. Symptoms often include loose joints, joint pain, stretchy velvety skin, and abnormal scar formation. These may be noticed at birth or in early childhood. Complications may include aortic dissection, joint dislocations, scoliosis, chronic pain, or early osteoarthritis. The current classification was last updated in 2017, when a number of rarer forms of EDS were added.
Kyphosis is an abnormally excessive convex curvature of the spine as it occurs in the thoracic and sacral regions. Abnormal inward concave lordotic curving of the cervical and lumbar regions of the spine is called lordosis. It can result from degenerative disc disease; developmental abnormalities, most commonly Scheuermann's disease; Copenhagen disease, osteoporosis with compression fractures of the vertebra; multiple myeloma; or trauma. A normal thoracic spine extends from the 1st thoracic to the 12th thoracic vertebra and should have a slight kyphotic angle, ranging from 20° to 45°. When the "roundness" of the upper spine increases past 45° it is called kyphosis or "hyperkyphosis". Scheuermann's kyphosis is the most classic form of hyperkyphosis and is the result of wedged vertebrae that develop during adolescence. The cause is not currently known and the condition appears to be multifactorial and is seen more frequently in males than females.
Spondylosis is the degeneration of the vertebral column from any cause. In the more narrow sense it refers to spinal osteoarthritis, the age-related degeneration of the spinal column, which is the most common cause of spondylosis. The degenerative process in osteoarthritis chiefly affects the vertebral bodies, the neural foramina and the facet joints. If severe, it may cause pressure on the spinal cord or nerve roots with subsequent sensory or motor disturbances, such as pain, paresthesia, imbalance, and muscle weakness in the limbs.
Lordosis is historically defined as an abnormal inward curvature of the lumbar spine. However, the terms lordosis and lordotic are also used to refer to the normal inward curvature of the lumbar and cervical regions of the human spine. Similarly, kyphosis historically refers to abnormal convex curvature of the spine. The normal outward (convex) curvature in the thoracic and sacral regions is also termed kyphosis or kyphotic. The term comes from the Greek lordōsis, from lordos.
Pectus carinatum, also called pigeon chest, is a malformation of the chest characterized by a protrusion of the sternum and ribs. It is distinct from the related malformation pectus excavatum.
Klippel–Feil syndrome (KFS), also known as cervical vertebral fusion syndrome, is a rare congenital condition characterized by the abnormal fusion of any two of the seven bones in the neck. It can result in a limited ability to move the neck and shortness of the neck, resulting in the appearance of a low hairline. Most people only have one or two of those symptoms so it may not be noticeable without medical imaging.
The Harrington rod is a stainless steel surgical device. Historically, this rod was implanted along the spinal column to treat, among other conditions, a lateral or coronal-plane curvature of the spine, or scoliosis. Up to one million people had Harrington rods implanted for scoliosis between the early 1960s and the late 1990s.
A back brace is a device designed to limit the motion of the spine in cases of bone fracture or in post-operative spinal fusiona, as well as a preventative measure against some progressive conditions or to correct patient posture.
Scheuermann's disease is a self-limiting skeletal disorder of childhood. Scheuermann's disease describes a condition where the vertebrae grow unevenly with respect to the sagittal plane; that is, the posterior angle is often greater than the anterior. This uneven growth results in the signature "wedging" shape of the vertebrae, causing kyphosis. It is named after Danish surgeon Holger Scheuermann.
The Milwaukee brace, also known as a cervico-thoraco-lumbo-sacral orthosis or CTLSO, is a back brace most often used in the treatment of spinal curvatures in children but also, more rarely, in adults to prevent collapse of the spine and associated pain and deformity. It is a full-torso brace that extends from the pelvis to the base of the skull. It was originally designed by Blount and Schmidt in 1946 for postoperative care when surgery required long periods of immobilization.
Tethered cord syndrome (TCS) refers to a group of neurological disorders that relate to malformations of the spinal cord. Various forms include tight filum terminale, lipomeningomyelocele, split cord malformations (diastematomyelia), occult, dermal sinus tracts, and dermoids. All forms involve the pulling of the spinal cord at the base of the spinal canal, literally a tethered cord. The spinal cord normally hangs loose in the canal, free to move up and down with growth, and with bending and stretching. A tethered cord, however, is held taut at the end or at some point in the spinal canal. In children, a tethered cord can force the spinal cord to stretch as they grow. In adults the spinal cord stretches in the course of normal activity, usually leading to progressive spinal cord damage if untreated. TCS is often associated with the closure of a spina bifida. It can be congenital, such as in tight filum terminale, or the result of injury later in life.
Diastematomyelia is a congenital disorder in which a part of the spinal cord is split, usually at the level of the upper lumbar vertebra in the longitudinal (sagittal) direction. Females are affected much more commonly than males. This condition occurs in the presence of an osseous, cartilaginous or fibrous septum in the central portion of the spinal canal which then produces a complete or incomplete sagittal division of the spinal cord into two hemicords. When the split does not reunite distally to the spur, the condition is referred to as diplomyelia, which is true duplication of the spinal cord.
Spinal disease refers to a condition impairing the backbone. These include various diseases of the back or spine ("dorso-"), such as kyphosis. Dorsalgia refers to back pain. Some other spinal diseases include spinal muscular atrophy, ankylosing spondylitis, scoliosis, lumbar spinal stenosis, spina bifida, spinal tumors, osteoporosis and cauda equina syndrome.
The neuromechanics of idiopathic scoliosis is about the changes in the bones, muscles and joints in cases of spinal deformity consisting of a lateral curvature scoliosis and a rotation of the vertebrae within the curve, that is not explained by either congenital vertebral abnormalities, or neuromuscular disorders such as muscular dystrophy. The idiopathic scoliosis accounts for 80–90% of scoliosis cases. Its pathogenesis is unknown. However, changes in the vestibular system, a lateral shift of the hand representation and abnormal variability of erector spinae motor map location in the motor cortex may be involved in this disease. A short spinal cord and associated nerve tensions has been proposed as a cause and model for idiopathic scoliosis. Besides idiopathic scoliosis being more frequent in certain families, it is suspected to be transmitted via autosomal dominant inheritance. Estrogens could also play a crucial part in the progression of idiopathic scoliosis through their roles in bone formation, growth, maturation and turnover. Finally, collagen, intervertebral disc and muscle abnormalities have been suggested as the cause in idiopathic scoliosis, although these are perhaps results rather than causes.
The management of scoliosis is complex and is determined primarily by the type of scoliosis encountered: syndromic, congenital, neuromuscular, or idiopathic. Treatment options for idiopathic scoliosis are determined in part by the severity of the curvature and skeletal maturity, which together help predict the likelihood of progression. Non-surgical treatment should be pro-active with intervention performed early as "Best results were obtained in 10-25 degrees scoliosis which is a good indication to start therapy before more structural changes within the spine establish." Treatment options have historically been categorized under the following types:
Iliocostal friction syndrome, also known as costoiliac impingement syndrome, is a condition in which the costal margin comes in contact with the iliac crest. The condition presents as low back pain which may radiate to other surrounding areas as a result of irritated nerve, tendon, and muscle structures. It may occur unilaterally due to conditions such as scoliosis, or bilaterally due to conditions such as osteoporosis and hyperkyphosis.
Spinal posture is the position of the spine in the human body. It is debated what the optimal spinal posture is, and whether poor spinal posture causes lower back pain. Good spinal posture may help develop balance, strength and flexibility.
Rounded shoulder posture (RSP), also known as “mom posture”, is a common postural problem in which the resting position of the shoulders leans forward from the body’s ideal alignment. Patients usually feel slouched and hunched, with the situation deteriorating if left untreated. A 1992 study concluded that 73% of workers aged 20 to 50 years have a right rounded shoulder, and 66% of them have a left rounded shoulder. It is commonly believed that digitalisation combined with the improper use of digital devices have resulted in the prevalence of sedentary lifestyles, which contribute to bad posture. Symptoms of RSP will lead to upper back stiffness, neck stiffness and shoulder stiffness. It can be diagnosed by several tests, including physical tests and imaging tests. To prevent RSP from worsening, maintaining a proper posture, doing regular exercise, and undergoing therapeutic treatments could be effective. If the situation worsens, patients should seek help from medical practitioners for treatments. If RSP is left untreated, chronic pain, reduction in lung capacity and worsened psychosocial health are likely to be resulted.
Halo-gravity traction (HGT) is a type of traction device utilized to treat spinal deformities such as scoliosis, congenital spine deformities, cervical instability, basilar invagination, and kyphosis. It is used prior to surgical treatment to reduce the difficulty of the following surgery and the need for a more dangerous surgery. The device works by applying weight to the spine in order to stretch and straighten it. Patients are capable of remaining somewhat active using a wheelchair or a walker whilst undergoing treatment. Most of the research suggests that HGT is a safe treatment, and it can even improve patients' nutrition or respiratory functioning. However, some patients may experience side effects such as headaches or neurological complications. The halo device itself was invented in the 1960s by doctors working at the Riancho Los Amigos hospital. Their work was published in a paper entitled "The Halo: A Spinal Skeletal Traction Fixation Device." The clinician Pierre Stagnara utilized the device to develop Halo-Gravity traction.
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