Scheuermann's disease
Encyclopedia
Scheuermann’s disease is a self-limiting skeletal
disorder of childhood. It is also known as Sherman's Disease, Scheuermann’s kyphosis (since it results in kyphosis
), Calvé disease and Juvenile Osteochondrosis of the Spine. It is named after Holger Werfel Scheuermann
. Scheuermann's disease describes a condition where the vertebrae grow unevenly with respect to the Sagittal plane
; that is, the anterior angle is often greater than the posterior. This uneven growth results the signature "wedging" shape of the vertebrae, causing kyphosis
.
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.
Scheuermann's disease is notorious for causing lower and mid-level back pain, which can be severe and disabling. The sufferer may feel pain at the apex of the curve, which can be aggravated by physical activity and by long periods of standing or sitting; this can have a significantly detrimental effect to their lives as their level of activity is curbed by their condition and they may feel isolated or uneasy amongst their peers if they are children, depending on the level of deformity.
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 get help for their condition. In studies, kyphosis is better characterized for the thoracic spine than for the lumbar spine.
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.
curve in the lumbar spine; this is the body's natural way to compensate for the kyphotic curve above. Interestingly, many with Scheuermann's disease have very large lung capacities and males often have broad, barrel chest
s. Most people have FVC scores above average. It has been proposed that this is the body's natural way to compensate for a loss of breathing depth.
Often patients have tight hamstrings, which, again, is related to the body compensating for excessive spinal curative, 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
.
, which has been associated with Marfan syndrome
) have been proposed and excluded.
While there is no explanation for what causes Scheuermann's Disease, there are ways to treat it. For decades there has been a lot of controversy surrounding treatment options. For less extreme cases, manual medicine, physical therapy and/or back braces can help reverse or stop the kyphosis before it does become severe. Because the disease is often benign, and because back surgery includes many risks, surgery is usually considered a last resort for patients. However in severe or extreme cases sometimes patients may be treated through an extensive surgical procedure, in an effort to prevent the disease from worsening or harming the body.
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. The method has been shown to reduce pain and decrease kyphotic angle significantly during an inpatient treatment program.
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.
One of the largest debates surrounding Scheuermann's disease correction is the use of very different correction procedures. There are different techniques to correct kyphosis; usually the differences being posterior/anterior entry or posterior entry (rear) only. The classic surgical procedure partially entails entering two titanium rods, each roughly one and a half feet long, into the back on either side of the spine. Eight titanium screws and hardware are drilled through the bone and secures them in place onto either side of the spine. On the internal-facing side of the spine, ligaments (which can be too short, pulling the spine into the general shape of kyphosis) 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. Normally, the damaged discs between the troubled vertebrae (wedged vertebrae) are removed and replaced with cartilage 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 and is not necessary once fusion completes. Recovery begins in the hospital and depending on whether the operation is one or two stage the patient can expect to be in hospital for minimum of a week possibly longer depending on recovery.
They will then often be required to wear a brace for several months to ensure the spine heals correctly leaving the patient with the correct posture. The titanium instrumentation can stay in the body permanently, or be removed years later. Patients undergoing surgery for Scheuermann's disease often need physical therapy to manage pain and mobility, however their range of motion is generally not limited very much. Recovery from kyphosis correction surgery can be very long; typically patients are not allowed to lift anything above 5 of 10 pounds for 6 months to a year. Many are out of work for at least 6 months. However, once the fusion is solidified, most patients can return to their usual lifestyle within one to two years.
Complications
Spinal fusion for kyphosis and scoliosis is extremely invasive surgery. The risk of undergoing spinal fusion surgery for kyphosis, like in scoliosis is estimated to be 5 % . Possible complications may be inflammation of the soft tissue or deep inflammatory processes, breathing impairments, bleeding and nerve injuries. However, according to the latest evidence the rate of complications is beyond that rate. As early as five years after surgery another 5% require reoperation and today it is not yet clear what to expect from spine surgery in the long-term. Taking into account that signs and symptoms of spinal deformity cannot be changed by surgical intervention, surgery remains to be a cosmetic indication. Unfortunately the cosmetic effects of surgery are not necessarily stable.
In case one decides to undergo surgery a specialized centre should be preferred.
Skeleton
The skeleton is the body part that forms the supporting structure of an organism. There are two different skeletal types: the exoskeleton, which is the stable outer shell of an organism, and the endoskeleton, which forms the support structure inside the body.In a figurative sense, skeleton can...
disorder of childhood. It is also known as Sherman's Disease, Scheuermann’s kyphosis (since it results in kyphosis
Kyphosis
Kyphosis , also called roundback or Kelso's hunchback, is a condition of over-curvature of the thoracic vertebrae...
), Calvé disease and Juvenile Osteochondrosis of the Spine. It is named after Holger Werfel Scheuermann
Holger Werfel Scheuermann
Holger Werfel Scheuermann was a Danish surgeon after whom Scheuermann's disease is named.- Biography :Holger Werfel Scheuermann was born into a medical family in Hørsholm, a small town between Copenhagen and Øresund. He began his studies in 1895 and graduated in medicine at the University of...
. Scheuermann's disease describes a condition where the vertebrae grow unevenly with respect to the Sagittal plane
Sagittal plane
Sagittal plane is a vertical plane which passes from front to rear dividing the body into right and left sections.-Variations:Examples include:...
; that is, the anterior angle is often greater than the posterior. This uneven growth results the signature "wedging" shape of the vertebrae, causing kyphosis
Kyphosis
Kyphosis , also called roundback or Kelso's hunchback, is a condition of over-curvature of the thoracic vertebrae...
.
Presentation
Scheuermann's disease is considered to be a a form of juvenile osteochondrosisOsteochondrosis
Osteochondrosis is a family of orthopedic diseases of the joint that occur in children and adolescents and in rapidly growing animals, particularly pigs, horses, and dogs. They are characterized by interruption of the blood supply of a bone, in particular to the epiphysis, followed by localized...
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.
Scheuermann's disease is notorious for causing lower and mid-level back pain, which can be severe and disabling. The sufferer may feel pain at the apex of the curve, which can be aggravated by physical activity and by long periods of standing or sitting; this can have a significantly detrimental effect to their lives as their level of activity is curbed by their condition and they may feel isolated or uneasy amongst their peers if they are children, depending on the level of deformity.
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 get help for their condition. In studies, kyphosis is better characterized for the thoracic spine than for the lumbar spine.
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.
Comorbid Conditions
Many with Scheuermann's disease often have an excessive lordoticLordosis
Lordosis is a medical term used to describe an inward curvature of a portion of the lumbar and cervical vertebral column. Two segments of the vertebral column, namely cervical and lumbar, are normally lordotic, that is, they are set in a curve that has its convexity anteriorly and concavity...
curve in the lumbar spine; this is the body's natural way to compensate for the kyphotic curve above. Interestingly, many with Scheuermann's disease have very large lung capacities and males often have broad, barrel chest
Barrel chest
Barrel chest generally refers to a broad, deep chest found on a man. A man described as barrel chested will usually have a naturally large ribcage, very round torso, large lung capacity, and will potentially have lots of upper body strength...
s. Most people have FVC scores above average. It has been proposed that this is the body's natural way to compensate for a loss of breathing depth.
Often patients have tight hamstrings, which, again, is related to the body compensating for excessive spinal curative, 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
Scoliosis
Scoliosis is a medical condition in which a person's spine is curved from side to side. Although it is a complex three-dimensional deformity, on an X-ray, viewed from the rear, the spine of an individual with scoliosis may look more like an "S" or a "C" than a straight line...
, though most cases are negligible. In more serious cases, however, the combination is classified as a separate condition known as kyphoscoliosis
Kyphoscoliosis
Kyphoscoliosis describes an abnormal curvature of the spine in both a coronal and sagittal plane. It is a combination of kyphosis and scoliosis. Kyphoscoliosis is a musculoskeletal disorder causing chronic underventilation of the lungs and may be one of the major causes of pulmonary hypertension...
.
Causes
The cause is not currently known, and the condition appears to be multifactorial. Several candidate genes (such as COL1A2COL1A2
Collagen alpha-2 chain is a protein that in humans is encoded by the COL1A2 gene.-External links:* -Further reading:...
, which has been associated with Marfan syndrome
Marfan syndrome
Marfan syndrome is a genetic disorder of the connective tissue. People with Marfan's tend to be unusually tall, with long limbs and long, thin fingers....
) have been proposed and excluded.
Conservative treatment
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 getting any worse.While there is no explanation for what causes Scheuermann's Disease, there are ways to treat it. For decades there has been a lot of controversy surrounding treatment options. For less extreme cases, manual medicine, physical therapy and/or back braces can help reverse or stop the kyphosis before it does become severe. Because the disease is often benign, and because back surgery includes many risks, surgery is usually considered a last resort for patients. However in severe or extreme cases sometimes patients may be treated through an extensive surgical procedure, in an effort to prevent the disease from worsening or harming the body.
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. The method has been shown to reduce pain and decrease kyphotic angle significantly during an inpatient treatment program.
Surgery for Scheuermann's Disease
Scheuermann's disease can be successfully corrected with surgical procedures, almost all of which include spinal fusionSpinal fusion
Spinal fusion, also known as spondylodesis or spondylosyndesis, is a surgical technique used to join two or more vertebrae. Supplementary bone tissue, either from the patient or a donor , is used in conjunction with the body's natural bone growth processes to fuse the vertebrae.Fusing of the...
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.
One of the largest debates surrounding Scheuermann's disease correction is the use of very different correction procedures. There are different techniques to correct kyphosis; usually the differences being posterior/anterior entry or posterior entry (rear) only. The classic surgical procedure partially entails entering two titanium rods, each roughly one and a half feet long, into the back on either side of the spine. Eight titanium screws and hardware are drilled through the bone and secures them in place onto either side of the spine. On the internal-facing side of the spine, ligaments (which can be too short, pulling the spine into the general shape of kyphosis) 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. Normally, the damaged discs between the troubled vertebrae (wedged vertebrae) are removed and replaced with cartilage 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 and is not necessary once fusion completes. Recovery begins in the hospital and depending on whether the operation is one or two stage the patient can expect to be in hospital for minimum of a week possibly longer depending on recovery.
They will then often be required to wear a brace for several months to ensure the spine heals correctly leaving the patient with the correct posture. The titanium instrumentation can stay in the body permanently, or be removed years later. Patients undergoing surgery for Scheuermann's disease often need physical therapy to manage pain and mobility, however their range of motion is generally not limited very much. Recovery from kyphosis correction surgery can be very long; typically patients are not allowed to lift anything above 5 of 10 pounds for 6 months to a year. Many are out of work for at least 6 months. However, once the fusion is solidified, most patients can return to their usual lifestyle within one to two years.
Complications
Spinal fusion for kyphosis and scoliosis is extremely invasive surgery. The risk of undergoing spinal fusion surgery for kyphosis, like in scoliosis is estimated to be 5 % . Possible complications may be inflammation of the soft tissue or deep inflammatory processes, breathing impairments, bleeding and nerve injuries. However, according to the latest evidence the rate of complications is beyond that rate. As early as five years after surgery another 5% require reoperation and today it is not yet clear what to expect from spine surgery in the long-term. Taking into account that signs and symptoms of spinal deformity cannot be changed by surgical intervention, surgery remains to be a cosmetic indication. Unfortunately the cosmetic effects of surgery are not necessarily stable.
In case one decides to undergo surgery a specialized centre should be preferred.
Prominent Sufferers
- Milan LucicMilan LucicMilan Lucic is a Canadian professional ice hockey left winger of Serbian origin currently playing for the Boston Bruins of the National Hockey League . He played major junior with the Vancouver Giants in the Western Hockey League for three seasons and captured a Memorial Cup, while being named...
, NHL hockey player for the Boston BruinsBoston BruinsThe Boston Bruins are a professional ice hockey team based in Boston, Massachusetts, USA. They are members of the Northeast Division of the Eastern Conference of the National Hockey League . The team has been in existence since 1924, and is the league's third-oldest team and its oldest in the... - George SampsonGeorge SampsonGeorge Jennifer Sampson is a street dancer and actor from Warrington, England. He won the second series of Britain's Got Talent on 31 May 2008 at the age of 14.-2007 series:...
, winner of the second series of Britain's Got TalentBritain's Got TalentBritain's Got Talent is a British television talent show competition which started in June 2007 and originated from the Got Talent series. The show is produced by FremantleMedia's TalkbackThames and Simon Cowell's production company SYCOtv. The show is broadcast on ITV in Britain and TV3 in Ireland...
External links
- http://orthoinfo.aaos.org/fact/thr_report.cfm?Thread_ID=247&topcategory=Spine
- http://www.back.com/causes-developmental-scheuermann.html http://www.emedicine.com/pmr/topic129.htm]
- Scheuermann's Kyphosis (Scheuermann's Disease): Abnormal Curvature of the Spine
- http://www.allaboutbackandneckpain.com/html/spinesub.asp?id=27