Scheuermann's disease

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Scheuermann's disease
Other namesScheuermann's kyphosis, Calvé disease, idiopathic juvenile kyphosis of the spine
ScheuermannDiseaseT6to10.png
Scheuermann's disease on lateral Xray of the T spine
Pronunciation
Specialty Orthopaedics, Rheumatology Osteopathy, Chiropractic
Symptoms Curve of upper back, chronic pain
Complications Chronic pain, lower than average bone density hence greater risk for osteoporosis and osteopenia [1]
Usual onset Adolescence
DurationLifelong
CausesUnknown, Genetic factors
Diagnostic method X-ray, MRI
Treatment Physical therapy, avoiding excessive weight bearing exercises, back brace, surgery
Prognosis Self-limiting
PrevalenceIt has a prevalence of 0.4–8.3% in the population with a higher incidence in females
A pre-operative image of a 22-year-old male with a very extreme case of Scheuermann's disease Scheuermanns diseasebl.jpg
A pre-operative image of a 22-year-old male with a very extreme case of Scheuermann's disease

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. [2] [3] [4]

Contents

Signs and symptoms

Scheuermann's disease is considered to be a form of juvenile osteochondrosis of the spine. It is found mostly in teenagers and presents a significantly worse deformity than postural kyphosis. Patients suffering with Scheuermann’s kyphosis cannot consciously correct their posture. The apex of their curve, located in the thoracic vertebrae, is quite rigid.[ citation needed ]

Scheuermann's disease is notorious for causing lower and mid-level back and neck pain, which can be severe and disabling. The sufferer may feel pain at the apex of the curve, which is aggravated by physical activity and by periods of standing or sitting; this can have a significantly detrimental effect to their lives as their level of activity is curbed by their disability. The sufferer may feel isolated or uneasy amongst their peers if they are children, depending on the level of deformity.[ citation needed ]

In addition to the pain associated with Scheuermann's disease, many sufferers of the disorder have loss of vertebral height, and depending on where the apex of the curve is, may have a visual 'hunchback' or 'roundback'. It has been reported that curves in the lower thoracic region cause more pain, whereas curves in the upper region present a more visual deformity. Nevertheless, it is typically pain or cosmetic reasons that prompt sufferers to seek help for their condition. In studies, kyphosis is better characterized for the thoracic spine than for the lumbar spine. [5] [6]

The seventh and tenth thoracic vertebrae are most commonly affected. It causes backache and spinal curvature. In very serious cases it may cause internal problems and spinal cord damage, but these cases are extremely rare. The curvature of the back decreases height, thus putting pressure on internal organs, wearing them out more quickly than the natural aging process; surgical procedures are almost always recommended in this case.[ citation needed ]

Pain: Scheuermann’s patients had a higher risk for back pain and disabilities during activities of daily living than controls. However, the degree of thoracic kyphosis among Scheuermann’s patients was not related to back pain, quality of life, or general health. [7]

Associated conditions

Many with Scheuermann's disease often have an excessive lordotic curve in the lumbar spine; this is the body's natural way to compensate for the kyphotic curve above. Many with Scheuermann's disease have very large lung capacities and males often have broad, barrel chests. Most people have forced vital capacity (FVC) scores above average. It has been proposed that this is the body's natural way to compensate for a loss of breathing depth.[ citation needed ]

Often patients have tight hamstrings, which, again, is related to the body compensating for excessive spinal curvature, though this is also debated (for example, some suggest the tightness of ligament is the initial cause of the growth abnormality). In addition to the common lordosis, it has been suggested that between 20–30% of patients with Scheuermann's Disease also have scoliosis, though most cases are negligible. In more serious cases, however, the combination is classified as a separate condition known as kyphoscoliosis.[ citation needed ]

Patients with Scheurmann's disease are prone to having a lower than average bone density. They are hence at a statistical greater long term risk for osteopenia and osteoporosis, the reason for this is unknown. [1]

Causes

The cause is not currently known, and the condition appears to be multifactorial. [8] Several candidate genes (such as FBN1, which has been associated with Marfan) have been proposed and excluded. It has also been proposed that there may be an underlying, yet elusive, neurological disorder that plays a role in the eventual development of the disease. [9]

Diagnosis

A 20-year-old male with Scheuermann's disease, showing various measurement of kyphotic/lordotic degrees and their supplementary angles. Notice the signature 'wedging' shape of the four vertebrae in the lower thoracic area. The other vertebral bodies are otherwise normal. The measured kyphosis for this patient is ~70deg. Scheuermanns70.jpg
A 20-year-old male with Scheuermann's disease, showing various measurement of kyphotic/lordotic degrees and their supplementary angles. Notice the signature 'wedging' shape of the four vertebrae in the lower thoracic area. The other vertebral bodies are otherwise normal. The measured kyphosis for this patient is ~70°.
Scheuermann's disease on lateral CT of the T spine ScheuermannDiseaseT6to10CT.png
Scheuermann's disease on lateral CT of the T spine

Diagnosis is typically by medical imaging. The degree of kyphosis can be measured by Cobb's angle and sagittal balance.[ citation needed ]

Treatment

Conservative

Scheuermann's disease is self-limiting after growth is complete, meaning that it generally runs its course and never presents further complication. Typically, however, once the patient is fully grown, the bones will maintain the deformity. For this reason, there are many treatment methods and options available that aim to correct the kyphosis while the spine is still growing, and especially aim to prevent it from worsening.[ citation needed ]

While there is no explanation for what causes Scheuermann's disease, there are ways to treat it. For less extreme cases, manual medicine, physical therapy and/or back braces can help reverse or stop the kyphosis before it does become severe.[ citation needed ] Because the disease is often benign, and because back surgery includes many risks, surgery is usually considered a last resort for patients. In severe or extreme cases, patients may be treated through an extensive surgical procedure in an effort to prevent the disease from worsening or harming the body.[ citation needed ]

In Germany, a standard treatment for both Scheuermann's disease and lumbar kyphosis is the Schroth method, a system of specialized physical therapy for scoliosis and related spinal deformities. [10] The method has been shown to reduce pain and decrease kyphotic angle significantly during an inpatient treatment program. [11] [12]

Bracing can be utilized to help prevent progression and in some cases reduce the hyperkyphosis Cobb angle. Conservative treatment of Scheuermann's hyperkyphosis in international literature is generally regarded as an effective treatment approach. Physiotherapy and bracing are the first-line treatments for this condition. [13]

Braces such as the Scolibrace (kyphobrace) and Kyphologic brace systems have been shown to be effective in the treatment of Scheuermann's [13]

Surgery

A post-operative X-ray of a 22-year-old male with Scheuermann's disease. After a 13-level spinal fusion to correct the excessive curvature, the person now presents a normal degree of kyphosis, with a minimal loss of flexibility. CorrectedKyphosis.jpg
A post-operative X-ray of a 22-year-old male with Scheuermann's disease. After a 13-level spinal fusion to correct the excessive curvature, the person now presents a normal degree of kyphosis, with a minimal loss of flexibility.

Scheuermann's disease can be successfully corrected with surgical procedures, almost all of which include spinal fusion and hardware instrumentation, i.e., rods, pedicle screws, etc. While many patients are typically interested in getting surgery for their correction, it is important to realize the surgery aims to reduce pain, and not cosmetic defect. As always, surgical intervention should be used as a last resort once conservative treatment fails or the patient's health is in imminent danger as any surgical procedure is not without risk. However, the chances of complication are relatively low, and the surgeries are often successful.[ citation needed ]

There are two primary surgical techniques to correct kyphosis: posterior-only fusion and anterior/posterior fusion. While debate lingers over which surgical approach is optimal, several studies published since 2018 suggest treatment trends are favoring posterior-only fusion. [14] [15] [16]

The classic surgical procedure entails entering two titanium rods, each roughly 1.5 feet (0.46 m) long (depending on the size of the kyphosis) into the back on either side of the spine. Eight titanium screws and hardware are drilled through the bone to secure the rods onto either side of the spine. On the internal-facing side of the spine, ligaments (which can be too short, pulling the spine into its abnormal shape) must be surgically cut or released, not only stopping part of the cause of the kyphosis, but also allowing the titanium rods to pull the spine into a more natural position. The damaged discs between the troubled vertebrae (wedged vertebrae) are normally removed and replaced with bone grafting from the hip or other parts of the vertebrae, which once healed or "fused" will solidify. The titanium instrumentation holds everything in place during healing. The patient can expect to remain in hospital for minimum of a week, and possibly longer. They may then be required to wear a brace for several months more to ensure the spine heals appropriately. The titanium instrumentation may stay in the body permanently, or be removed years later. Patients who have undergone such surgery may need physical therapy to manage pain and mobility. Recovery can be prolonged: typically patients are not allowed to lift anything above 5–10 pounds (2.3–4.5 kg) for 6 months to 1 year, and many are out of work for at least six months. However, once the fusion is solidified, most patients can return to their usual lifestyle within 1–2 years.[ citation needed ]

Prognosis

Spinal fusion for kyphosis and scoliosis is an extremely invasive surgery. The risk of complications is estimated to be about 10%. Possible complications may be inflammation of the soft tissue or deep inflammatory processes, breathing impairments, bleeding and nerve injuries, or infection. As early as five years after surgery around 5% require reoperation and long-term issues remain unclear. [17] [18] Taking into account that some of the symptoms of the spinal deformity cannot be changed by surgical intervention, surgery remains a cosmetic indication, [17] [19] though the cosmetic effects of surgery are not necessarily stable. [17]

Notable cases

See also

Related Research Articles

<span class="mw-page-title-main">Scoliosis</span> Medical condition of the spine

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 impact a person's life and hence, can also be considered a disability.

<span class="mw-page-title-main">Kyphosis</span> Medical condition

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.

<span class="mw-page-title-main">Lumbar spinal stenosis</span> Medical condition of the spine

Lumbar spinal stenosis (LSS) is a medical condition in which the spinal canal narrows and compresses the nerves and blood vessels at the level of the lumbar vertebrae. Spinal stenosis may also affect the cervical or thoracic region, in which case it is known as cervical spinal stenosis or thoracic spinal stenosis. Lumbar spinal stenosis can cause pain in the low back or buttocks, abnormal sensations, and the absence of sensation (numbness) in the legs, thighs, feet, or buttocks, or loss of bladder and bowel control.

<span class="mw-page-title-main">Laminectomy</span> Surgical removal of a lamina

A laminectomy is a surgical procedure that removes a portion of a vertebra called the lamina, which is the roof of the spinal canal. It is a major spine operation with residual scar tissue and may result in postlaminectomy syndrome. Depending on the problem, more conservative treatments may be viable.

<span class="mw-page-title-main">Spondylosis</span> Degeneration of the vertebral column

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.

<span class="mw-page-title-main">Lordosis</span> Medical condition

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.

<span class="mw-page-title-main">Degenerative disc disease</span> Medical condition

Degenerative disc disease (DDD) is a medical condition typically brought on by the normal aging process in which there are anatomic changes and possibly a loss of function of one or more intervertebral discs of the spine. DDD can take place with or without symptoms, but is typically identified once symptoms arise. The root cause is thought to be loss of soluble proteins within the fluid contained in the disc with resultant reduction of the oncotic pressure, which in turn causes loss of fluid volume. Normal downward forces cause the affected disc to lose height, and the distance between vertebrae is reduced. The anulus fibrosus, the tough outer layers of a disc, also weakens. This loss of height causes laxity of the longitudinal ligaments, which may allow anterior, posterior, or lateral shifting of the vertebral bodies, causing facet joint malalignment and arthritis; scoliosis; cervical hyperlordosis; thoracic hyperkyphosis; lumbar hyperlordosis; narrowing of the space available for the spinal tract within the vertebra ; or narrowing of the space through which a spinal nerve exits with resultant inflammation and impingement of a spinal nerve, causing a radiculopathy.

<span class="mw-page-title-main">Klippel–Feil syndrome</span> Congenital condition characterised by fusion of two or more vertebrae in the neck

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 results 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.

<span class="mw-page-title-main">Harrington rod</span>

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.

<span class="mw-page-title-main">Back brace</span> Corrective medical device worn around a patients back

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.

<span class="mw-page-title-main">Spinal fusion</span> Immobilization or ankylosis of two or more vertebrae by fusion of the vertebral bodies

Spinal fusion, also called spondylodesis or spondylosyndesis, is a surgery performed by orthopaedic surgeons or neurosurgeons that joins two or more vertebrae. This procedure can be performed at any level in the spine and prevents any movement between the fused vertebrae. There are many types of spinal fusion and each technique involves using bone grafting—either from the patient (autograft), donor (allograft), or artificial bone substitutes—to help the bones heal together. Additional hardware is often used to hold the bones in place while the graft fuses the two vertebrae together. The placement of hardware can be guided by fluoroscopy, navigation systems, or robotics.

A burst fracture is a type of traumatic spinal injury in which a vertebra breaks from a high-energy axial load, with shards of vertebra penetrating surrounding tissues and sometimes the spinal canal. The burst fracture is categorized by the "severity of the deformity, the severity of (spinal) canal compromise, the degree of loss of vertebral body height, and the degree of neurologic deficit." Burst fractures are considered more severe than compression fractures because long-term neurological damage can follow. The neurologic deficits can reach their full extent immediately, or can progress for a prolonged time.

<span class="mw-page-title-main">Kyphoscoliosis</span> Medical condition

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.

<span class="mw-page-title-main">Gibbus deformity</span>

Gibbus deformity is a form of structural kyphosis typically found in the upper lumbar and lower thoracic vertebrae, where one or more adjacent vertebrae become wedged. Gibbus deformity most often develops in young children as a result of spinal tuberculosis and is the result of collapse of vertebral bodies. This can in turn lead to spinal cord compression causing paraplegia.

<span class="mw-page-title-main">Spinal disease</span> Diseases involving the vertebral column

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, lumbar spinal stenosis, spina bifida, spinal tumors, osteoporosis and cauda equina syndrome.

<span class="mw-page-title-main">Spinal stenosis</span> Disease of the bony spine that results in narrowing of the spinal canal

Spinal stenosis is an abnormal narrowing of the spinal canal or neural foramen that results in pressure on the spinal cord or nerve roots. Symptoms may include pain, numbness, or weakness in the arms or legs. Symptoms are typically gradual in onset and improve with leaning forward. Severe symptoms may include loss of bladder control, loss of bowel control, or sexual dysfunction.

<span class="mw-page-title-main">Vertebral column</span> Bony structure found in vertebrates

The vertebral column, also known as the backbone or spine, is the core part of the axial skeleton in vertebrate animals. The vertebral column is the defining characteristic of vertebrate endoskeleton in which the notochord found in all chordates has been replaced by a segmented series of mineralized irregular bones called vertebrae, separated by fibrocartilaginous intervertebral discs. The dorsal portion of the vertebral column houses the spinal canal, a cavity formed by alignment of the neural arches that encloses and protects the spinal cord.

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:

  1. Observation
  2. Bracing
  3. Specialized physical therapy
  4. Surgery

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.

<span class="mw-page-title-main">Halo-gravity traction device</span> Device used to treat spinal deformities

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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