SCOLIOSIS
Definition: Scoliosis as defined by the Scoliosis Research Society is a "lateral deviation of the spine by more than 10 degrees". It is more than this, it is a three dimensional deformity in which the spine is bent to the side as one looks from back to front, and twisted when viewed from above.
Description: Approximately 10% of all teens have some degree of scoliosis. Usually only 1% have severe enough curves to require some kind of medical attention beyond the normal 6 months or yearly observations. Scoliosis is found in both male and females but more often in females. Scoliosis in girls is more likely to progress than in boys. The reason for this is unknown.
TYPES OF SCOLIOSIS
Idiopathic
Infantile and Juvenile Idiopathic Scoliosis
Juvenile Scoliosis: Outcomes in Adulthood
Congenital
Neuromuscular
Infantile and Juvenile Idiopathic Scoliosis
Article written for BackTalk by
Robert B. Winter, M.D.
Orthopaedic Spine Surgeon
St. Paul, Minnesota
Introduction
Idiopathic scoliosis is sub-divided into three subgroups according to age: infantile (0-3 years), juvenile (4-10 years), and adolescent (11- maturity). The more common adolescent variety has been greatly discussed, but this article will deal with the less common infantile and juvenile forms. It is very important to realize that when the term "idiopathic" is used, it means that other causes of curvature have been excluded.
Infantile Idiopathic Scoliosis
This condition has always been more common in Great Britain and Northern Europe than in North America, but appears to be lessening in prevalence everywhere. Contrary to adolescent idiopathic scoliosis which is more common in girls, the infantile type is more common in boys. It is also different in that the curves are usually left thoracic rather than right thoracic.
The natural history is also quite interest-ing in that about 80% of the children have spontaneous resolution if the curve is below 30 degrees on the first visit. This occurs without any treatment whatsoever. A commonly used radiological sign is the rib vertebral angle difference, which if over 20 degrees indicates a high chance of progression. Frequent checkups are needed and if progression is seen, aggressive non-surgical treatment must be started.
There are thus two varieties of this condition, the self-resolving and the progressive. The progressive group is then subdivided into two subgroups, the benign progressive and the malignant progressive. The benign progressive requires vigorous non-surgical treatment with serial casting and Milwaukee bracing, but if this is done well, the curve will gradually disappear and treatment can finally be discontinued without recurrence.
The malignant progressive type is one of the most difficult challenges to the scoliosis surgeon. Despite the most vigorous and diligent casting and bracing program, the curve tends to progress at a young age. This is one of the most lethal of all scoliosis.
The only answer to such a problem is surgery. The big problem is what kind of surgery and when. In the old days the only available surgery was posterior spine fusion. This was better than nothing, but the "crankshaft" problem was very significant. Combined anterior and posterior fusion of the whole curve can greatly help with this problem, but of necessity this will stop all vertical growth in the fused area. There is obviously a desire to preserve some vertical torso growth while at the same time controlling the curve. This led to the development of instrumentation without fusion.
Distraction rodding is done and Milwaukee bracing is required to protect the rod from dislocation or breaking. Lengthening of the rod at four to six month intervals is necessary. Fusion is done when the curve begins to go "out of control: More recently surgeons have combined this program with convex anterior and posterior growth arrest and hemiarthrodesis (fusion of the convex side of the curve only).
Juvenile Idiopathic Scoliosis
The natural history of juvenile idiopathic scoliosis is quite different from either infan-tile or adolescent. About one-third of the patients have mild and non-progressive curves, but few of these are completely resolving like the majority of the infantiles. As stated previously, accurate diagnosis comes first, and most centers now routinely obtain an MRI.
For those patients with small (under 20 degrees) curves, periodic observation at four to six month intervals is all that is needed. For a curve showing progression, Milwaukee brace treatment is necessary. As in infantile curves, a Milwaukee brace is always needed rather than an underarm style of brace in order not to compress the soft ribs of the young child. Bracing should always begin with a "full-time" program in order to fully correct the curve, if possible. If the results are good, often the brace can gradually be removed long before the end of growth. Some children will require bracing until the end of growth, but never need surgery. Some will do well in the brace until they hit the pubertal growth spurt, and need surgery then. Others will fail even the best of brace programs and require surgery at a young age. Of all children started in a brace program, about one-third will follow each of these pathways. Bracing of juveniles can have more spectacular results than in adolescents. One of my most memorable patients was an eight year old girl who presented with a rapidly progressive 58 degree curve. She was immediately started on a Milwaukee brace which she had to wear for eight years. At brace removal her curve was 21 degrees, and seventeen years later it was 24 degrees!
Surgery is needed in about one-third of juveniles. If a brace program can control the curve until a Risser 2 status, then a posterior fusion alone is adequate. If however, the Risser stage is zero, and secondary sexual development has not begun, then combined anterior and posterior fusion are needed. If surgery is needed at age six or so, then rodding without fusion can be a good choice.
Conclusions
Infantile and juvenile idiopathic scoliosis are quite different from the more common adolescent type. Establishing the correct diagnosis is the most important first step. This may require an MRI. Controlled observation, bracing, and surgery all have roles to play. All other form of non-surgical treatment (massage, chiropractic, acupuncture, medications, biofeedback, etc.) have never shown any benefit. Left untreated, these curves can be fatal.
Article written for BackTalk by
Peter D. Masso, M.D.
Ellen Meeropol, M.S., R.N., P.N.P.
Elise Lennon, M.P.H., R.N.
Juvenile Scoliosis: Outcomes in Adulthood
Juvenile onset scoliosis, defined as spinal curves diagnosed between the third and tenth birthdays, is less common than adolescent scoliosis but not rare, comprising up to one-fifth of scoliosis cases. Evidence suggests that compared to adolescent scoliosis, juvenile curves are less likely to respond to brace treatment and more likely to progress to surgical intervention. Juvenile scoliosis differs from the adolescent condition in two other ways: there is a 18-26% rate of intraspinal defects such as Chiari I malformation and syringomyelia in children with juvenile onset scoliosis, and if untreated, severe juvenile onset curves are associated with increased mortality rates in midlife.
Juvenile scoliosis is a distinct entity with its own challenges in evaluation and management. There is scant published research describing the condition, factors predicting curve progression, effectiveness of treatment, or functional and psychosocial impact of the condition. The existing studies of juvenile scoliosis report 27% to 87% of patients progress to surgical treatment. Higher risk of progression is reported in juvenile curves with apical vertebra at T8, T9, or T10, with initial rib-vertebra angle difference (RVAD) of greater than 10 degrees, or with increasing serial RVAD measurements. In adolescent idiopathic scoliosis there is no evidence of increased depression or poor self-esteem. Adolescents treated surgically are reported to have better self-esteem than those treated with braces.
We were unable to locate studies of functional outcome or self esteem in children with juvenile scoliosis. In order to better understand the clinical and psychosocial impact of having scoliosis as a child, we reviewed the medical records and radiographs, and collected outcome questionnaire data from 52 patients treated for juvenile onset scoliosis. We included only those patients who had a Cobb angle of 10 degrees or greater measured after the third birthday, but before the tenth birthday, and who were at least two years after completion of surgical or orthotic treatment and at least two years after skeletal maturity (defined as Risser IV for females, Risser V for males).
In evaluating functional outcomes of a condition treated largely in childhood, we felt it was important to elicit reports from both patients and parents. Patients completed the Child Health Questionnaire (CHQ) which addresses 12 health concepts of physical and psychosocial functioning. Parents filled out the Pediatric Outcomes Data Collection Instrument (PODCI) developed by POSNA, AAOS, AAP, ? and Shriners Hospitals for use in the assessment of pediatric musculoskeletal disorders.
Study patients fell into three groups based on the treatment received: observation, bracing or surgery. Children with curves of less than 20 degrees were observed. Children with curves of 20 degrees or greater were usually braced and a screening MRI of the entire spine was obtained. Surgery was generally recommended for children with curves of greater than 50 degrees. Medical record, radiograph and outcome questionnaire data were examined and compared between these three groups.
Nine children (17%) were followed with observation. Seventeen children (33%) received orthotic treatment only. Another seventeen children were braced but had curves which progressed and subsequently required spinal instrumentation and fusion. Brace treatment at our hospital was initiated at a mean age of 10.1 years for an average of 3.9 years. Ninety-four percent of patients were treated in underarm braces. Half were prescribed full-time bracing and half part-time (18 hrs/day). Patient reports revealed that only 43% of children wore their brace 18 or more hours, while 39% wore them 12-17 hours, and 18% had less than 12 hours of daily wear. Twenty-six of the children studied (50%), including the 17 children with prior bracing, required spinal instrumentation and fusion. At surgery, their average age was 14.0 years and mean curve magnitude was 68 degrees.
Of the 26 children studied by MRI, four (15%) were found to have intraspinal anomalies. The findings included syringomyelia in four patients and Chiari I malformation in three. Neurosurgical intervention was required in three of these children. Abnormal MRI findings were equally divided between the bracing and surgical groups and were not correlated with back pain or abnormal reflexes.
Children in the three treatment groups were similar in age at diagnosis, amount of thoracic hypokyphosis, and amount of decompensation measured on standing x-rays. Differences between groups were observed in curve magnitude, curve progression, median rib vertebral angle difference (RVAD) and mean apical vertebra, as well as in the psychosocial and functional outcome data from the self-report questionnaires.
Curve magnitude at diagnosis was 15 degrees in the observation group compared to 23 degrees in the orthotics group and 24 degrees in the surgical group. A similar difference was seen between the three groups at final follow-up (17 degrees, 30 degrees, and 33 degrees). For patients evaluated at this facility before their tenth birthday, median RVAD was 5 degrees in the observation group, 11 degrees in the brace group, and 15 degrees in patients requiring surgery. Apical vertebra also differed by treatment group, with mean level of 111 in the observation group, T10 in the brace only group, and T8-9 in the children requiring surgery.
On the Child Health Questionnaire the children in the observation group reported the least pain, and the best Physical Function and Health Perception scores. The children who had been braced reported more pain than other groups. The surgical patients had the lowest Physical Function and Health Perception scores, but less pain than the patients treated in orthoses. All treatment groups reported similar scores on the Self Esteem scale.
On the PODCI questionnaire, the Satisfaction scale measures the parent's feelings if the child were to spend the rest of his/her life with the condition as it was at the time of the study. Parents of the braced children had the lowest scores and parents of surgical patients were most satisfied. On the Happiness scale, which measures the parent's perception of the child's happiness with his/her appearance, body image and ability, the parents of children in the observation group had the highest scores and parents of surgical patients the lowest. Since normative data is just now becoming available, we do not know whether these groups differ significantly from "normal" populations.
This preliminary study is limited by the small sample, lack of normative data, and retrospective design. Despite these limitations, these results support the impressions of the existing literature that juvenile onset scoliosis is likely to progress and require treatment, is less responsive to bracing and more likely to require surgery than adolescent scoliosis. These data also suggest that the RVAD and apex of curve may be helpful in predicting which curves will progress and that the treatment of scoliosis in children does not affect the child's self-esteem.
This article is based on the study reported in Masso, RD., Meeropol, E., Lennon, E. (2002). Juvenile onset scoliosis followed up to adulthood: Orthopaedic and functional outcomes. Journal of Pediatric Orthopaedics, 22,279-84.
Bibliography
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2. Evans SC, Edgar MA, IIall-Craggs MA, Powell MP, Taylor BA, Noordeen HH. MRI of "idiopathic" juvenile scol-iosis. I Bone Joint Surg [Br] 1996; 78:314-7.
3. Figueiredo UM, James JIP. Juvenile idiopathic scoliosis. I Bone Joint Surg [Br] 1981; 63:61-66.
4. Landgraf JM, Abetz L, Ware JE. The CHQ user's manual. First Edition. Boston, MA: The Health Institute, New England Medical Center, 1996:30.
5. Mannherz RE, Betz RR, Clancy M, Steel IIH. Juvenile idiopathic scoliosis fol ?lowed to skeletal maturity. Spine 1988; 13:1087-90.
6. Pehrsson K, Larsson 5, Oden A, Nachemson A. Long-term follow-up of patients with untreated scoliosis. A study of mortality, causes of death, and symptoms. Spine 1992; 17:1091-6.
7. Robinson CM, McMaster MJ. Juvenile idiopathic scoliosis. Curve patterns and prognosis in one hundred and nine patients. JBone Joint SurgAm 1996; 78:1140-8.
8. Tolo VT, Gifiespie R. The characteristics of juvenile idiopathic scoliosis and results of its treatment. J Bone Joint Surg [Br] 1978; 60: 181-8.
ADOLESCENT IDIOPATHIC SCOLIOSIS
From an article in BackTalk by,
Robert S. Pashman, M.D.
Director; Scoliosis and Spinal Deformity Service
Cedars-Sinai Institute for Spinal Disorders Los Angeles, California
Scoliosis is defined as curvature of the spine in the coronal {front view) plane. Idiopathic scoliosis should be 
conceptualized as a three dimensional deformity though; twisting of the spine is coupled with curvature producing deformity in both coronal and sagittal {side view) planes. As its name implies, adolescent idiopathic scoliosis occurs between the ages of 10 and 18 and to date, has no known cause. The magnitude of the curve is determined using the Cobb method {Figure 1) and conveyed in degrees. Curves measuring more than 10 degrees occur with an approximate worldwide incidence of 3% and are distributed equally between males and females. Curves greater than 20 degrees occur with an incidence of 0.3% and have a distribution of five females to every male. Because larger curves are both more likely to require treatment and occur more frequently among females, there is a common misconception that adolescent idiopathic scoliosis, in general, is more common in girls.
The exact etiology of idiopathic scoliosis is yet to be determined, but it is thought to be due to multiple factors. Although the exact genetics is uncl
ear, the observation that idiopathic scoliosis is more common within families suggests the presence of an inherited trait.
Research focusing on changes in muscles, the spinal column, rib cage and the chemistry of cartilage in discs suggests that these abnormalities are most likely secondary to the primary scoliosis and not a causative factor. The fact that most curves occur in common patterns, such as right thoracic or left lumbar, raises the possibility that other anatomical asymmetries such as the pulsatile beating of a leftsided heart, might have an influence on curve production and progression. Here at the Cedars Sinai Institute for Spinal Disorders, we are currently investigating with MRI the relationship between observed turbulent CSF (fluid surrounding the spinal cord) flow at curve apex, differential pressure on the spinal cord, and the influence of these factors on curve progression. For the reader who is interested in more information on the current state of knowledge and research into the etiology of idiopathic scoliosis, a recent Current Concepts Review (Journal of Bone and Joint Surgery, Volume 82A, No.8, August 2000) will be helpful.
Adolescent idiopathic scoliosis curves are classified by their location in the spine. Curves can occur in the cervical, thoracic and lumbar spine in various combinations (figure 2). Structural curves are defined as those curves that incompletely straighten on side-bending. Compensatory curves straighten significantly on side-bending and function to produce spinal balance. The location of the structural curve determines the classification of the scoliosis. For example, a structural curve occurring in the thoracic spine with a lumbar compensatory curve is called thoracic adolescent idiopathic scoliosis. (Figure 3) Single curves, curves whose apex is at T12 or 11 may be defined as a thoracolumbar curve (Figure 4), and curves with apices at 12 or 13 are defined as lumbar curves. Structural curves in both the thoracic and lumbar spine are called double major curves. (Figure 5) The exact definition of the curve has implications for determining progression and treatment.
I 
n California, as in many other states, law mandates middle school screening for scoliosis. Scoliosis is detected by observation of a rib prominence during a forward bending test (Figure 6). Girls and boys are most often screened in the 7th and 8th grades, respectively. School screening has effectively reduced both the number of patients requiring surgery and the magnitude of those curves at the time of surgery.
Once scoliosis is detected, the patient should be referred to an orthopaedic surgeon who specializes in spinal deformities. 36 x 14 inch scoliosis x-rays in the frontal and sagittal planes are obtained and measured according to the Cobb method. Idiopathic scoliosis rarely produces neurological findings, such as numbness or weakness in the extremities. Neurologic findings should suggest a non-idiopathic scoliosis and, in most cases, only then should be investigated with imaging modalities such as MRI. Studies have shown that routine MRI of patients with idiopathic scoliosis rarely uncovers intraspinal pathology and, therefore, is not cost effective.
The treatment of patients with idiopathic scoliosis begins with an estimation of the probability for curve progression. The two major determinants of curve progression are the patient's age (both chronological and bone development) and size of the curve. Secondary determinants include sex of the patient and rotation of the spine. Once these determinants are assessed, the treating physician can estimate the likelihood of curve progression and formulate a treatment strategy.
In general, the current treatment of adolescent idiopathic scoliosis is guided by certain general principles. First, small curves in older patients have less chance of progression than larger curves in you 
nger patients. Second, adolescent curves greater than 40 degrees are difficult to control mechanically with braces. Third, adult curves greater than 50 degrees will continue to progress at an average rate of 1 degree per year. Taken together, the goal of treatment is to keep adolescent curves less than 40 degrees during growth, and at the end of growth to keep all curves less than 50 degrees. Finally, cosmetic considerations should only be a rare, primary indication for surgery.
Based on these considerations, the attached table summarizes current treatment of adolescent idiopathic scoliosis (Figure 7). Small curves measuring less than 20-25 degrees that do not require brace treatment should be observed during periodic examinations of four to six months or one year 
intervals, based on their size. Observation remains a form of treatment because any 5 degree increase in the size of the curve may change the course of treatment.
Although there is little controversy, whether patients who meet certain criteria should be braced, the exact choice of brace type and duration of brace wear generates some debate. An excellent discussion of brace effectiveness was summarized in Winter's article in Backtalk, April 1999 (Volume 22/ Number I). Three points emerge from his review. First, bracing alters the natural history of curve progression. Second, bracing is "dose dependent", the more the brace is worn, the greater chance for it to be efficacious. Third, for the brace to be effective, it should be worn until impending skeletal maturity and then weaned slowly.
On the other hand, it is uncommon for braces to induce permanent correction and curves generally return to their pre-braced Cobb angle quickly after being discontinued. Moreover, for unknown reasons, certain curves progress despite brace treatment. Currently, we are unable to predict which curves will progress during brace treatment.
Those patients who have curves greater than 40 degrees at presentation and have progressive curves despite bracing should be considered for surgery. Curves between 40 and 50 degrees fall into a relative grey area for surgical indications, but any curve above 50 degrees in a growing child should be surgically stabilized.
The basic principle of surgery is to stop progression of the curve and to leave the patient balanced in a frontal and sagittal plane. Cessation of curve progression is achieved with bony fusion between the affected vertebrae while the correction is held and supported by spinal instrumentation until healing is complete. All structural curves need to be fused. Depending on the type of instrumentation, a brace may or may not be necessary subsequent to surgery.
Although much attention has been focused on the various approaches to stabilizing curves in adolescent idiopathic scoliosis, certain principles are applicable to all of them. A minimum number of vertebrae should be fused to achieve a balanced spine. Secondly, the extent of the fusion into the lumbar spine may negatively impact the future occurrence of low back pain in the patient and, therefore, the fusion should attempt to preserve as many free lumbar segments as possible.
In general, either anterior or posterior spinal fusions are employed, based on surgeon preference and curve location. Only in very large curves is it necessary to operate on both sides of the spine. Some approaches clearly make more sense than others. For example, in this author's opinion a thoracolumbar curve fused through the front generally has a greater chance of p 
reserving distal lumbar fusion levels than a similarly efficacious posterior fusion. (Figure 8)
Spinal instrumentation has revolutionized the surgical treatment of progressive curves in adolescent idiopathic scoliosis. Instrumentation serves to correct the curve while holding it stable until bone applied to the spine heals {the fusion). Once the bony fusion occurs, the instrumentation has no function, although it rarely needs to be removed.
In the past, Harrington rods provided two points of fixation in the spine and therefore needed to be supplemented with a cast to hold the spine. Contemporary instrumentation techniques utilize segmental fixation which provides attachment to the spine at multiple points. Unlike the Harrington rod, segmental fixation techniques allow better correction of the curve in both the frontal and sagittal planes {Figure 9). {See Sagittal Balance of the Spine and Flat Back Deformity article by Robert S. Pashman, M.D. in Backtalk, June/July 1996.) New instrumentation techniques have, in many instances, proven to be so rigid that postoperative bracing is sometimes not necessary {Figure 10). The type of instrumentation, approach, and the use of postoperative braces are based on the surgeon's experience.
One exciting, potential advance in the surgical treatment of adolescent idiopathic scoliosis is the use of less invasive techniques, utilizing multiple small incisions for the placement of cameras to view and instruments to correct the scoliosis from the front of the thoracic or thoracolumbar spine. Currently, multi-center studies are under way to establish the safety and efficacy of this type of approach.
From an article written in BackTalk by
Dennis G. Crandall, M.D.
Medical Director
Sonoran Spine Center
Phoenix, Arizona
SCOLIOSIS IN ADULTS
Adult scoliosis is a condition where rotation of the spinal vertebrae leads to curvature of the spine in a person who has finished growing (usually by 18-21 years old). In order to be classified as scoliosis, the curve must exceed 10 degrees. Curves can occur in the chest area (thoracic spine) or the lower back area (lumbar spine). Occasionally, curves involve areas of the spine which lie in between, such as thoracolumbar. The neck or cervical spine is rarely involved.
CAUSES
Adult Idiopathic Scoliosis - When curvature of the spine starts in adolescence in an otherwise healthy person, it is most commonly diagnosed as "idiopathic scoliosis". Idiopathic refers to the fact that the curve is not associated with other known problems such as cerebral palsy, spina bifida, neurofibromatosis, or a number of other conditions. After age 18, the idiopathic scoliosis is termed "adult idiopathic scoliosis" It is the same curve present during the teen years, but the spine does not behave the same way as the teenage spine. As a person with scoliosis ages, the spine develops premature aging changes in the back joints such as bone spurs, degenerative discs, and thickened spinal ligaments. This leads to a condition known as "adult idiopathic scoliosis with degenerative changes". These degenerative changes superimposed on a curve that is already present can sometimes cause back pain, leg pain, spinal imbalance, and progression or worsening of the curve.
For adult curves greater than 50 degrees, our natural history studies suggest that they have a high likelihood of progressing about 1 degree per year. For curves in the lumbar spine or lower back, there is a high chance of progression if the curve is greater than about 40 degrees.
Degenerative Scoliosis - As arthritis begins to affect the spine, the discs lose their water content and consequently their ability to serve as the "shock absorber" of the spine. The facet joints in the back of the spine begin to wear out and lose their ability to maintain normal spinal alignment. The ver-tebrae begin to slip or abnormally move. This may lead to spinal instability, nerve compression, and pain. As both the disc and the facet joints lose their ability to maintain normal spinal motion, the spine can settle asymmetrically, leading to scoliosis.
When the lumbar spine was straight as an adult but develops a curve later in life (usually in the 60+ age group), it is termed De Novo Scoliosis, indicating spontaneous development of a scoliosis due to degenera-tion of the joints and discs in the spine. This can occur earlier in patients who have had spinal surgery for laminectomy. It never occurs without significant arthritis.
The most common sign of scoliosis is a prominence in the ribs on one side of the thoracic spine. In the lumbar spine, there is sometimes a prominence on one side, though often not. The prominence or "rib hump" is most apparent when bending forward. Sometimes there is an asymmetry in the waist, with one side being indented more than the other. Clothes begin to fit differently than they used to.
If the scoliosis is severe and unstable, spinal imbalance is common. Imbalance implies that patients lean to one side or forward when they try to stand straight upright. They may feel like they are tipping to one side, or always have the sense that they are falling forward. Most people with adult scoliosis notice that they are not as tall as they used to be.
Most young adults with scoliosis do not have significant back pain. The curve usually does not hurt unless or until it becomes degenerative. Sometime in life, however, because arthritis is age related and develops prematurely in this group, the adult with scoliosis may develop back pain. When it occurs, the pain is worse when upright and active, and better when the patient is resting.
Spinal instability occurs when the disc and facet joints are so worn out that they can no longer maintain normal spinal alignment. Pain comes from the arthritic joints as well as from the adjacent nerves, which are pinched and stretched as a result.
Buttock pain can occur due to referred pain from the arthritic spine, or it could be a manifestation of a more significant problem with nerve compression. Spinal nerve roots become pinched when arthritic bone spurs form around them and block their exit route from the spinal column. This condition is called Spinal Stenosis. In addition to buttock pain, other symptoms such as leg pain, numbness, tingling, and weakness are common. If any of these findings are present, advice from a physician should be sought without delay.
If spinal stenosis or nerve compression in the back is severe enough, control of bowel and bladder function will be lost. This is thankfully a rare event, but when it happens, it is a surgical emergency. If the pressure on the nerves is not relieved quickly, control of bladder and bowel may never be regained. Again, this is a very rare occur-rence and I only see two or three cases each year.
CHALLENGES
As the spine ages, it becomes stiffer. Flexibility is greatest in the teen years, and usually declines starting in the 40-50 age group. Stiffness of the spinal joints can become severe as bone spurs form and prohibit motion. In some cases, the bone spur formation is so severe that all motion is lost at one or more levels in the spine.
We all achieve our maximum bone density at about age 30-35. After age 35-40, there is a slow decline in the amount of bone present in the spine. After age 60, and particularly after menopause in women, the loss of bone becomes visible on x-rays. This is osteoporosis. If the bone loss becomes severe, spontaneous fractures can occur in the spine. These fractures can lead to scoliosis or kyphosis.
As we age, our general health can become more of a problem. Chronic disease processes such as high blood pressure, diabetes, and heart disease are prevalent among American seniors. When scoliosis becomes a problem in older patients, other health issues must be considered when treatment options are considered.
CONSERVATIVE TREATMENT
Nearly all patients with adult scoliosis will respond to conservative treatment and lead a normal, functional life. When pain is present, it is usually short term and manageable. Treatment for adult scoliosis should almost always begin with a non-invasive approach. I say, "Try the easy things first".
Non-steroid Anti-inflammatory Drugs
(NSAID) have been the cornerstone of medical therapy for arthritic and inflammatory conditions. These medications can quiet the pain and relieve stiffness caused by degenerating discs and joints.
Physical Therapy is an excellent way to improve function, flexibility, endurance, and decrease pain. Usually the therapist will work with patients toward becoming less symptomatic, and maintaining the improvement with an active home exercise program. Working out in a supervised environment with the help of a physical therapist is the best way to achieve it. On average, therapy lasts 2-3 times per week for 4-8 weeks.
It is very important that adult patients with scoliosis get into the habit of doing a daily exercise routine. This will improve the strength of the trunk muscles and take some of the stress off the spine. Often when pain occurs, it is because the patient is not doing his or her exercises.
Sometimes a back brace is helpful in getting some relief from back pain in patients with degenerative scoliosis. A word of caution is in order however: the brace should not be used without faithful compliance with an active exercise program. Brace wear without exercise tends to lead to a weaker spine that becomes dependent on the brace. Daily exercises and occasional (when needed) brace wear lead to the best results, where bracing is concerned.
Medical management of osteoporosis and general health is important to maintaining an active lifestyle into old age, especially in patients with scoliosis. Solving small problems before they become big ones has always been good advice.
REASONS SURGERY MIGHT BE CONSIDERED
Few patients with adult scoliosis will ultimately require surgery. When necessary, the goals of surgery are to stop curve progression, stabilize the spine, establish correct spinal balance, decrease back and leg pain, and increase function with as little surgery and as few complications as possible. Patients who require surgery to straighten, stabilize and fuse their spinal curvature are patients with:
1. Increasing curvature over time (it will continue to get worse)
2. Unstable spine that hurts despite conservative care
3. Nerve compression causing pain, numbness, or weakness
4. Spinal imbalance which is painful or progressive
5. Large curve which will progress (better to do these earlier while health is good and before osteoporosis starts or worsens)
SURGICAL OPTIONS AND RESULTS
If the main problem is leg pain caused from a disc herniation, this can usually be taken care of with a small surgery to remove the disc fragment and decompress the nerve. A large procedure to correct the scoliosis and fuse the spine is not necessary.
Sometimes leg pain is caused by bone spurs that are compressing the spinal nerves. This is spinal stenosis. If stenosis is the problem, the solution usually will require removal of the offending bone spurs to get pain relief. If adequate bone is to be surgically removed to decompress the pinched nerves (laminectomy), the spine is often ren-dered unstable in the process. Back pain will increase, leg pain may return, and the spinal curvature will get bigger if the spine is not fused at the same time. In these cases, correction of the curve and fusion with bone graft and instrumentation is required to stabilize the spine and prevent what would be a certain need for future surgery.
When back pain, progressive deformity, or spinal imbalance are primary factors, the curve should be straightened and fused. The amount of correction obtained with surgery is limited compared to the corrections seen in the pediatric patients, due to increased spinal stiffness in adults.
SURGICAL TECHNIQUES TO CORRECT SCOLIOSIS
Once the decision for surgery has been made, the operative plan is formulated. Patients are routinely asked to donate blood before surgery to be stored and used during their surgery. The spinal cord is usually monitored throughout the surgery to make sure there is no compromise to spinal cord function.
Surgery to correct adult scoliosis is the most challenging surgery done in orthopedics, and is likely among the most complex and demanding surgeries of any kind being performed today. This type of surgery requires at least one assisting surgeon and often a surgical team, and can take from 4-14 hours to accomplish.
Anterior Surgery - If the spine must be fused anteriorly or from the front, a thoracic or general surgeon will be a part of the sur-gical team to safely mobilize the great blood vessels off the spine where the spine surgeon will work. The incision may be through the side of the chest. through the side of the abdomen, or through the front of the abdomen, depending on what is needed at the time of surgery. The purpose of anterior surgery is to remove the discs, and fill the space with bone graft. This serves to improve the correction which can be achieved and improve the reliability of the fusion. Sometimes the spine is "instrumented" from the front, meaning that screws are placed into the vertebra and attached to a rod that will correct the deformity and stabilize the spine.
More recently, the thoracoscope has been used in spine surgery. We can now remove discs from the thoracic spine and insert bone graft without making a large incision. All of the work is done through a few one-inch incisions on the side of the chest.
Posterior Surgery - Most of the correction of scoliosis is done from the back of the spine. If nerves are com-pressed by bone spurs or a disc herniation, the offending structures can be removed to allow more room for the nerves. The spine is then "instrumented" by placement of hooks or screws that attach to the vertebrae. These hooks and screws are then attached to rods that span the curve. The instrumentation is then distracted, compressed, or rotated in order to correct the spinal curvature. Without instrumentation, the curve cannot be corrected.
Bone graft is always used in scoliosis surgery. The spine must be fused in its new corrected and straightened position. The graft most commonly comes from the patient's own pelvis. Sometimes bone-bank bone is used when there is not sufficient bone available from the patient.
RESULTS FROM SURGERY
Adult patients who undergo major spinal surgery to correct their scoliosis generally do well. Pain is relieved in the majority. The fusion is successfully achieved and the correction maintained long term in 85-95% of people who have mild to moderate scoliosis corrected with or without nerve root decompression.
Complications can occur however, such as failure of the spine to solidly fuse, failure of the spine hardware (5%), infection (2-5%), ?nerve injury (>1%),medical complications, and others. The patients at greatest risk for complications are people taking steroids and those with severe osteoporosis or poor nutrition.
We recently presented to the Scoliosis Research Society and the North American Spine Society the results of three different studies of adult patients with stiff degenerative scoliosis. All patients required fusion with instrumentation of the spine to the sacrum. Several had severe spinal imbalance. A full 2 years after their surgery, all patients reported significant improvement in their pain. Narcotic medication decreased from 73% before surgery to 9% after surgery. The tech used were considered successful and promising.
THE FUTURE
On the horizon, there are several new technologies which are very exciting and will likely change the way we currently address spinal deformity. One of the most revolutionary concepts is the use morphogenic Protein to assist the spine in achieving a solid fusion. Look for BMP to be available to surgeons (outside of research programs) in about 3-5 years. (Editor's note: BMP is has been recently approved for use in surgery.)
Look for the spine surgery of the future to be done safer, with fewer complications and better results than anything we do now. Spine surgery has made light years of progress in just the past 15 years. The future looks just as bright, if not brighter.
From an article written for BackTalk by,
Michael G. Neuwirth, M.D.
Director, The Spine Institute of Beth Israel Medical Center
New York, New York
and
Haim D. Blecher, M.D.
Private Practice in New Jersey
The Spine Institute of Beth Israel Medical Center
New York, New York
ADULT SCOLIOSIS: A FUNCTIONAL PERSPECTIVE
Adult scoliosis presents one of the most challenging conditions to the spine care team. Over the past 15 years management of the adult patient with a spinal deformity has undergone a tremendous evolution. Both surgical and non-surgical management offer many options to patients presenting with an adult deformity.
There are about 500,000 adults in the United States with curves greater than 30 degrees. Adult scoliosis can be divided into two groups. Most commonly it represents untreated adolescent idiopathic scoliosis.4 It may, however, also represent adult onset degenerative deformity. Clinical presentation and surgical indications vary from patient to patient. Pain is the most common reason for seeking medical advice.2 Sources for the pain vary from osteoarthritis, muscle fatigue due to coronal and sagittal imbalance, instability and spinal stenosis resulting in neurogenic claudication.3 When compared to the surgical treatment of the adolescent idiopathic deformity, the surgical treatment of adult deformity is often more extensive and the rate of complications is higher.4 The natural history of scoliosis would suggest that curves greater than 50-degrees at skeletal maturity progress at an average of 1 degree a year. This allows us to have a general framework when deciding to intervene surgically. We also know that, although the incidence of back pain in the scoliosis population is similar to that of the general population, the intensity and persistence is greater in those patients with a deformity.4
In general, given the potential morbidity of surgical treatment, we exhaust all possible non-operative treatments for the pain whenever possible.4 The analogy to a train ride seems to describe it well; surgery is the last stop on the train and non-operative treatment represents all the stops on the way. Most of the time there are very few people that stay on the train until the last stop. This requires a team approach between the surgeon, physiatrist, physical therapist and the pain management team. Physical therapy programs improving muscle strength, flexibility, aerobic capacity and posture may show great benefits in the patient's function and pain reduction. Epidural steroid injections, selective nerve root blocks may also show great benefits to the patient suffering from secondary stenosis or foraminal impingement. Facet blocks, selective rhizotomies are also tools we frequently use to alleviate pain resulting from degenerative disease secondary to the deformity. Even though non-operative management does not halt curve progression, it certainly alleviates the pain and improves function in many patients so that they do not go on to have surgery. We find that patients that exhaust non-operative treatments and are still symptomatic enough to require surgery are better prepared mentally and physically for these extensive surgical reconstructions.
The majority of patients who require surgical treatment need to have some type of long segment correction and fusion. Many patients will require combined anterior and posterior spinal fusion. Some patients will also require some type of decompression to alleviate neural foraminal encroachment or central stenosis. Surgical treatment of scoliosis in 2004 is quite different from that in 1980. The intraoperative techniques we have today have great implications on the postoperative course of the patients. Today, segmental fixation of the spine with pedicle screws has allowed for greater correction in both coronal and axial plane, better maintenance of sagittal balance, higher rates of fusion in certain cases and the virtual elimi-nation of post operative casting/bracing of the majority of patients.
The post operative rehabilitation period should be divided into three: immediate, intermediate and long term. In the immediate postoperative period, 1- 7 days post-op, several distinct goals should be achieved. Recovery from surgery usually entails several days in the ICU where hemodynamic parameters are guarded and stabilized, aggressive pain management is implemented, prophylaxis to prevent deep vein thrombosis is maintained and the critical post operative period is overcome. Out of bed activities with some basic ambulation with assistance is ample in the first few days post-op. Commode training and independence are important immediate post-op goals to allow for discontinuation of catheters, decreasing chances of urinary tract infections. As we transition the pain management to oral medications and patches, discontinuation of intravenous lines represents an "unleashing" of the patient which will hopefully allow for a smoother transition into the intermediate post operative period. During the intermediate post operative period (8 days - 6 weeks), we transition from the critical perioperative period in the beginning, to independence and return to basic daily activities. Most patients are able to go home after the immediate post operative period but some require a short term rehab facility. The rehabilitation team is met with a post-operative patient that has pain, stiffness and frequently has other medical problems that require attention. In the majority of cases, a long segment of the spine is rigidly fixed in order for it to fuse; many times the spine is fused to the pelvis. The majority of these patients will depend on other motion segments to accomplish their goals of daily living. Being able to independently sit from a supine position, stand from a sitting position and initiate ambulation from a stationary position form the basic building block from which the remainder of the rehab program stems. Self-hygiene is clearly an important goal necessary to accomplish during this period. Nutritional status is of utmost importance. This cannot be emphasized enough. The nutritional well being of the patient has a significant effect on their function, ability to heal soft tissues and fight off infections. The rehab team must also realize that their patients may be clinically depressed and careful attention to mood is important.
Once the patient is able to independently ambulate, take care of his/her self hygiene and nutrition and perform the ADLs comfortably, the goals of the intermediate post operative period are met. In the long-term post operative rehabilitation period, once the soft tissues have healed and the fusion is well on its way to heal, the patient should begin a more specific physical therapy and rehabilitation protocol. Patients left with a fused thoracic and lumbar spine to the pelvis need to develop and protect other joints and muscles that will help in their locomotion and flexibility. Range of motion maintenance of the hips and knees is of utmost importance. Hamstring and quadriceps strength and flexibility will allow the patient to flex and extend through the hip and knee joints with greater efficiency. Core muscle training and proprioception are also important parts of the rehabilitation process. By 4-6 months post operatively, the patient should accomplish most of the rehabilitation goals and be well educated as to the daily regimen that will help him/her maintain these goals.
We interviewed one of our physical therapists that has treated a number of our adult scoliosis patients. She stated that pre operative preparation and conditioning are extremely important. She finds that patients that are aerobically conditioned pre operatively and are familiar with the exercises that they will be required to do tend to fare better. She finds that the biggest difficulty post operatively is that the patients are afraid to move, afraid of causing harm to their back or the "rods". Pre and post operative reassurance by the physician and rehab team allow the patient to feel more secure with their back. She finds that other than post operative pain, stiffness remains a critical issue. Patients with a long segment fusion, mainly those fused to the pelvis, have difficulty bending forward to tie their shoes and put on socks. Hamstring stretching is a significant component of the post operative program. Fusion to the pelvis also eliminates the pelvic stabilization program and makes abdominal muscle strengthening more difficult. She creates lower extremity exercises that indirectly strengthen the abdominal musculature and hip flexors. Aerobics conditioning post op is a vital part of the program. Patients are able to maintain a more positive outlook, the more conditi ?oned and fit they are. She finds one or two meetings with the patient prior to the actual start of the physical therapy program beneficial as well.
But really, what should we expect of the postoperative adult scoliosis patient? The answer is quite a bit. Other than our personal extensive experience, which has yielded very good results and patient satisfaction, there have been several good studies looking at the functional outcomes. We can expect relief of pain in 70-85% of patients, improved ability to perform ADLs in 70-90% of patients, improved ability to sleep and ability to return to exercises and recreational activities in 60-80% of patients. In the majority of these studies, the combination of these improvements has yielded a high patient satisfaction, over 85% in some studies. Similar numbers of patients would have the surgery over again knowing what they know post op. Although a significant influence on patient satisfaction has been the coronal and sagittal balance as well as fusion rates, the postoperative rehabilitation and goal achievement have been indispensable. A patient with a regimented goal oriented rehabilitation program and a caring rehabilitation team has a significant head start.
Adult scoliosis is not an uncommon entity. Despite the great challenge it poses, a team approach combining the surgeon and the rehabilitation team may yield very good results, helping these patients improve their quality of life.
Bibliography
1. Moe JH, Lonstein JE. Moe's Textbook of scoliosis and other deformities. 3rd ed. Philadelphia: WB Saunders, 1995;XIII:658.
2. Bradford DS, lay BK, Hu SS . Adult Scoliosis: Surgical Indications, Operative Management, Complications, and Outcomes.Spine1999;24:24 pp.2617-2629
3. Bridwell KH, Dewald RL. The Textbook of Spinal Surgery. 2nd ed. Philadelphia, PA: Lippincott-Raven, 1997;22: 1590-9.
4. Neuwirth MG, Osborn K. The Scoliosis Sourcebook; Everything You Needed to Know: Contemporary Books 2001.
From an article written for BackTalk by,
Andrew G.S. King, M.D.
Professor, Department of Orthopaedics
Co-Director of Spinal Unit, LSU Medical Center
Children's Hospital
New Orleans, Louisiana
CONGENITAL SCOLIOSIS
Introduction:
There are three main types of scoliosis. The most common is idiopathic scoliosis which has no known cause and is seen in otherwise healthy individuals. Neuromuscular Scoliosis is seen in people with known nerve or muscle problems and who are often confined to a wheelchair. Congenital Scoliosis is quite separate from these. It is caused by a birth defect in one or more of the 17 vertebrae which are stacked together to form the spinal column below the level of the neck.
There are three major types of birth defects:
1. HEMIVERTEBRA. Here the vertebra is wedged-shaped. It appears as if one half of the vertebra has not formed. This wedged nature of the vertebra causes the spine to tilt, causing scoliosis. This type of congenital scoliosis is called "failure of formation'.
2. CONGENITAL BAR. This is a solid block of bone confined to one side of the spine, halting any further growth on that side alone. However, the spine may continue to grow on the other side, and it grows with a progressively worsening tilt or scoliosis. This type of congenital scoliosis is termed "failure of segmentation," since in the normal spine there is a mobile disc space between each vertebrae or segment, which in this case, has been replaced by a solid block of bone.
3. A combination of 1 and 2. This is called "Hemivertebra with Contralateral Bar'.
Causes of Congenital Scoliosis
These birth defects appear to be caused by some unknown injury to the embryo during the first month of pregnancy. Therefore, this may occur even before the mother is aware she is pregnant. It is usually not hereditary and will usually not be passed on to future generations.
The kidneys are also being formed at this time. In up to 30% of cases of congenital scoliosis there will also be a congenital abnormality of the kidneys. Usually this will be an abnormality which will not affect health, but some will. Since the signs of kidney problems may be very subtle, each per-son diagnosed with congenital scoliosis should have his or her kidneys checked out. This can usually be simply done with an ultrasound examination.
A birth defect in the spinal cord may be present in up to 40% of cases. Usually, this will not show up on plain x-rays and an MRI scan is necessary to exclude this. The spinal cord may have one or more of the following three types of abnormality:
1. Tether - A thick band of fibers hold the spinal cord lower than normal in the spinal canal, causing the cord to be stretched as the child grows.
2. Syringomyelia (or syrinx) - An accumulation of fluid in the center of the spinal cord.
3. The spinal cord is split in two by a sliver of bone (diastematomyelia).
If these spinal cord birth defects are present, a consultation with a neurosurgeon will usually be necessary to determine if they have to be corrected.
Hemivertebrae
These are usually discovered in infancy or childhood, with a parent or relative noting an asymmetric prominence in the back. Usually the individual is otherwise quite normal and there are seldom complaints of pain. The diagnosis is confirmed by standard x-rays. Once discovered, scoliosis caused by the hemivertebra may take two courses. The first possibility is that there may be no or minimal worsening of the scoliosis with no health problems more throughout life. The second possibility may be progressive slow worsening of the scoliosis, leading to increasing deformity which may reach the point of being unacceptable. The worsening deformity may cause pain and fatigue, particularly in later life. Which course the scoliosis will take is termed the "natural history" and it is extremely important for the treating doctor to predict the natural history as accurately as possible when he first sees the patient, since this will form the basis of his recommended treatment. The doctor's prediction will be based partly on the configuration of the vertebra, its position in the spine, the size of the curve already present, and the amount of growth that the patient has left before maturity. Obviously, the worse the curvature when first seen and the more years of growth remaining, the greater the likelihood of the development of severe deformity and, therefore, the greater the need for surgical intervention.
The vertebrae in the spine are no different than other bones in the skeleton in that growth occurs from a plate of growing cells. In the spine this plate of growth cells is seen at the top and bottom of the vertebrae adjacent to the discs. Normal growth is moderated by body weight passing evenly across these plates, but with the vertebrae being wedged, the body is "thrown off" with more weight being passed on the inner side or concavity and less weight on the convexity. The consequence of increased weight or pressure across the growth plate is less growth, with the opposite on the convex side. It is, therefore, not hard to imagine the wedging or the vertebra increasing as the child grows. With this in mind, you can understand the options for treatment of congenital scoliosis caused by a hemivertebra.
1. Observation. With smaller curves which are causing no symptoms at the time they are discovered, the doctor may elect to carefully follow the curve with x-rays taken at periods of between four and twelve months, and only proceed to some type of surgical correction if the curve worsens. A particular time of concern is the pre-adolescent growth spurt. Some hemivertebra can be nicely balanced by another hemivertebra in another part of the spine which is wedged in the opposite direction. This configuration is less likely to need surgery.
2. Bracing. Bracing is not as helpful in congenital scoliosis as it is in idiopathic scoliosis and is rarely used. The curve is usually too sharp for the brace to affect the growth patterns
3. Prevent worsening of the scoliosis by fusing the spine to the normal vertebrae above and below the wedged vertebra. This is termed "in situ fusion" which means no correction of the existing deformity is hoped for; only that existing deformity will not get worse. Usually the fusion includes the front and back of the spine. The front of the spine may continue to grow even in the presence of a solid fusion in the back (posterior fusion), but it will grow in a corkscrew fashion increasing the size of the rib hump. Doctors call this the "crankshaft phenomenon".
4. Fuse only the convex side of the curve (convex growth arrest). If done early, this operation could allow some correction of the curve, particularly if there is still potential for growth on the concave side. Again, this operation must be done on both the front and back of the spine. For the thoracic spine, the front part has been successfully carried out using a minimally invasive approach through a scope.
5. Hemivertebrectomy. This operation seems simple conceptually. The wedge of bone which is tilting the spine is surgically removed, allowing the spine to be straightened. This operation is regaining popularity after earlier reports had suggested that there was an increased risk of nerve damage. With modern spinal techniques and spinal cord monitoring, the author believes this risk is now acceptably low, particularly in the lumbar spine. Hemivertebra in the lumbar spine, particularly in the lowermost part of the spine before it meets the pelvis, may be particularly troublesome since they disrupt the mechanics of the low back. In addition, spine surgery is safer in the lower lumbar spine since the spinal cord ends in the upper lumbar spine. Hemivertebrectomy may be carried out in patients as young as one year old in an effort to allow more normal development of the spine with growth.
Treatment of Congenital Scoliosis Caused by a Bar (Failure of Segmentation)
Here there are fewer options. The birth defect is almost always associated with a scoliosis that worsens, often up to 10 degrees per year. Therefore, observation is usually not an option and surgery should be carried out as soon as the bar is discovered. Most commonly this will take the form of an anterior and posterior fusion. There is no potential for growth on the concave side so a convex growth arrest makes no sense, unless it extends for a level above and below to try and get the spine to twist back to balance out the area of scoliosis. Excision of the bar seems tempting, but to my knowledge there have been very few reports of its being successfully carried out. The most likely outcome would be for the bone to form back over the area.
The use of metal rods in surgery for congenital scoliosis is not as common as it is for other types of scoliosis. They are often unnecessary in young children and infants who may be adequately treated in an old fashioned plaster or fiberglass cast. The purpose of the rods is mainly to hold the spine steady to allow the fusion to form, and less to attempt to straighten the spine.
Finally, the most difficult problem seen in the treatment of congenital scoliosis are the problems seen in adults who may not have had treatment as children and have been left with a severe curve, or whose previous surgery had been too little or unsuccessful. Both patients may feel they have an unacceptable cosmetic problem. They may have pain due to disruption of the low back mechanics, and there may be worsening scoliosis due to collapse of the spine where it curves into the area of the congenital defect either above it or, more often, below it. Surgical treatment here is much more complex than in the child, emphasizing the importance of having the spine corrected adequately in childhood. Much longer fusions of the spine may be necessary and, in addition, wedges of bone removed. These are called osteotomies, or partial or complete vertebrectomies. In contrast to the child, spinal instrumentation is nearly always required.
Summary
Congenital scoliosis is caused by a birth defect in one or more vertebrae. It may take the form of a wedged vertebra or a bar of bone. The patient often appears normal, apart from a prominence on the back. Kidneys may also be affected and should be checked out. The treating doctor will try to determine the natural history of the defect and this, in turn, will determine his recommendation for surgery or observation. Surgical options include removal of the hemivertebra to straighten the spine, or altering growth of the spine with a fusion. The best results are seen when treatment starts early and severe deformity is prevented.
Bibliography
McMaster, M.J., Ohtsuka, K.
The Natural History of
Congenital Scoliosis:
A study of 251 patients
Journal of Bone and Joint Surgery
641A: 1128- 1147 1982
Winter, R.B., M.D.
Congenital Spine Deformity
Moe's Textbook of Scoliosis and other
Spinal deformities
M.B. Saunders Co. 1987
CONGENITAL SCOLIOSIS
From an article written for BackTalk by,
Robert B. Winter, M.D.
Clinical Professor of Orthopedic Surgery
University of Minnesota, and Research
Consultant to the Twin Cities Spine Center
Minneapolis, Minnesota
By definition, congenital scoliosis refers specifically to scoliosis (a lateral curvature of the spine) caused by congenital anomalies of vertebral development. The deformity may be present at birth or may develop later. Congenital scoliosis is much more common than congenital lordosis or congenital kyphosis.
As in many other congenital problems, patients with congenital scoliosis frequently have other congenital anomalies. The most frequent are abnormalities of the spinal cord (40%), genitourinary system (25%), and heart (15%). For this reason, a thorough total evaluation of the child is very worthwhile.
Unlike idiopathic scoliosis which is strongly hereditary, congenital scoliosis is seldom hereditary. In over 2000 patients seen at our center over the past 40 years, only 20 (1%) have had a parent, child, or sibling with congenital scoliosis. Even in identical twins, usually one will have the problem and the other not.
The natural history of congenital scoliosis has been studied in detail at several centers around the world and the results have been quite consistent. About 75% of children show progressive deformity, and only 25% are not progressive. Of those which are progressive, two-thirds have severe progression and only one-third mild progression.
Progression is related to growth, and therefore the two periods of most rapid progression are from birth to age three years and during the pubertal growth spurt. Congenital scoliosis can, therefore, produce severe deformity and even early death. One patient I saw died of lung failure at age eight years.
Since a high percentage of patients show progression of their curvatures, and since these progressive curvatures cause bad problems, what can be done to prevent bad outcomes. In idiopathic scoliosis we know that braces can be highly effective in preventing curve progression. Unfortunately, in congenital scoliosis the opposite is true, i.e. very few curves respond to bracing. Even those that do respond during the quiet growth years (4-10) will then progress, despite the brace during the pubertal growth spurt.
Therefore, since most curves are progressive and very few of these respond to bracing, many patients require surgery. Surgery is, therefore, the "backbone" of management, and should never be considered as a "last ditch" method, but rather as the standard-of-care, usual and customary treatment. The issues then revolve around two basic questions: what form of surgery, and when should it be done?
One of the most fundamental concepts in dealing with congenital scoliosis is that surgery is a preventive treatment. In the ideal situation the problem is recognized early, the potential or actual progression documented, and the definitive surgery done before major deformity occurs.
There are certain well known anatomic situations, easily diagnosed on routine x-rays, where the surgeon has 100% certainty that severe curve progression will take place. In these specific situations it is best to do the surgery promptly and not procrastinate. Parents have often thought me "knife-happy" when I told them that their one year old child needed an immediate fusion, but this can be precisely the best treatment.
Many congenital curvatures cannot be so precisely predicted. This is particularly true of hemivertebra problems. In such cases, very careful periodic monitoring is necessary in order to establish whether or not the curve is progressing. If it is, then that is the time to do the surgery without further procrastination.
Thus, the proper time for surgery depends on the individual situation and cannot be generalized. One fundamental rule is that surgery should never be delayed until the end of growth. By that time irreparable harm will have been done. The second fundamental rule is to never allow progression to go on and on. Once demonstrated, it must be stopped.
Many parents worry that such early surgery will "stunt the child's trunk growth'. Nothing is farther from the truth. Because of the anomalies, normal vertical growth cannot happen, only deformed growth. The child will have a longer torso with early fusion than if the surgery is procrastinated.
In my career the two most common mistakes I have seen are: (1) failure to recognize that progression is occurring, and (2) failure to do something when progression is occurring.
There are four different types of surgery available: (1) posterior spine fusion, (2) anterior and posterior spine fusion, (3) hemivertebra excision and spine fusion, and (4) convex growth arrest and hemifusion. Time does not permit a detailed discussion of these alternatives, but each have their proper place.
Instrumentation is sometimes used in congenital scoliosis, but often not, due to the young age of the child, small bones, abnormal bones, and abnormal spinal cords. Thus, casts or braces are commonly used after surgery.
In summary, congenital scoliosis is very different from the more common idiopathic and neuromuscular scoliosis. Surgery is very common, braces are rarely useful, and surgery at a young age is the rule rather than the exception.
From an article written for "BackTalk" by
Merv Letts, M.D., FRCSC
Head, Division of Pediatric Orthopaedics
Children's Hospital of Eastern Ontario
Ottawa, Ontario
Neuromuscular Scoliosis in The 90s
(editors comment: although written in the 90s, this is still valid today)
Over the past 15 years there have been a number of improvements in the care and management of children with neuromuscular scoliosis. Neuromuscular scoliosis includes those children with spinal curvature secondary to conditions such as cerebral palsy, muscular dystrophy and myelomeningocele. Scoliosis in such children is frequently progressive and can interfere significantly with good sitting posture due to secondary pelvic obliquity. Since many children with neuromuscular scoliosis depend on a wheelchair for their mobility, sitting stability is an extremely important objective in the treatment of the spinal curvature.
Neuromuscular scoliosis frequently occurs in children who are not ambulatory and depend on a wheelchair existence. The scoliosis usually results in poor sitting balance and the children must use their hands to keep themselves upright . Discomfort is also a problem, especially when the scoliosis reaches such a magnitude that the rib cage rests on the pelvic brim. This results in decreased sitting tolerance and the child spends more time recumbent, increasing the risk of developing skin ulcers and skin breakdown. By straightening the spine and making the pelvis more level, balance is restored, the hands are freed up to do other things and the chronic discomfort of the rib cage on the pelvis is eliminated. The result is usually a much happier child who is much easier to care for due to improved sitting balance, increased volume of the abdominal cavity, and the potential beneficial effects on respiratory, gastrointestinal and genitourinary infections.
With the introduction of segmental instrumentation by wiring of Luque rods directly to the lamina of the vertebra, a new era in the control of long "C" curves typical of neuromuscular scoliosis dawned. This removed the reliance on only two hooks of the Harrington system and allowed each vertebra to be corrected in an incremental manner, resulting in powerful correction and utilizing the Galveston technique of placing the L portion of the rod directly into the pelvis. Thus, pelvic obliquity could also be controlled in a much more efficient manner. Dr. Cohn Moseley's modification of the Luque rod system to a unit rod by joining the two Luque rods at the top added even more stability to the construct, and a powerful system for the correction of neuromuscular scoliosis evolved which is now in common usage and providing excellent results . In neuromuscular curvatures over 60 degrees, many surgeons will elect to perform an anterior release either at the same time as the posterior fusion or preceding the posterior fusion by about a week This loosens up the curvature and allows better correction at the time of the posterior instrumentation. Combined with the anterior release is removal of the discs and the performance of an inter body fusion. This results in a circumferential fusion and a very stable spine. Some surgeons may elect to supplement the anterior release and fusion with anterior instrumentation. Other types of posterior instrumentation can also be utilized such as the Cotrel-Dubousset or one of its many variations. Because the combination of pelvic obliquity and scoliosis in neuromuscular compromised children may result in a devastating seating dilemma, it is essential that both components be addressed and instrumentation to the pelvis allows this to be accomplished. Since the pelvis virtually becomes part of the spine curvature, any corrective techniques usually must involve the pelvis. Absolute anatomical correction of the spine however, is not necessary to achieve considerable improvement in the sitting balance and a redistribution of weight bearing over both the thighs and buttocks. The key word is "balance" and if this is achieved, the sitting tolerance of the child can be much improved, as well as the potential for learning and socialization.
Medical advances in other areas of the health care team have also improved the management of children with severe neuromuscular scoliosis. This has included advances in anesthetic technique, improved intensive care management and a better understanding of the preoperative requirements of children with neuromuscular disorders. This includes augmented preoperative nutrition as well as the correction of gastro esophageal reflux, especially in children with cerebral palsy. As a result of these improvements, children with neuromuscular scoliosis can now be operated on at an early age, are in better preoperative condition and can tolerate longer and more involved procedures, such as the performance of an anterior and posterior fusion during the same operative procedure, saving the child the morbidity of two major procedures.
The correction of the neuromuscular scoliosis is usually quite gratifying and appreciated by, not only the child, but by parents and care givers alike.