Definition
The term scoliosis , first used by Galen (131-201 ad ), is derived from the Greek word meaning “crooked.” One of the most common deformities of the spine, scoliosis has been recognized since ancient times, with descriptions of normal and abnormal spinal curves found in the Corpus Hippocraticum . In 1741, André devised the crooked spine as his symbol for orthopaedics.
Currently, scoliosis is defined as lateral deviation of the normal vertical line of the spine, which when measured on a radiograph, is greater than 10 degrees ( Fig. 12-1 ). Because the lateral curvature of the spine is associated with rotation of the vertebrae within the curve, a three-dimensional deformity occurs. This complex deformity represents abnormal movement in three planes: (1) intervertebral extension in the sagittal plane leading to lordosis of the scoliotic segment, (2) lateral intervertebral tilting in the frontal plane, and (3) a rotatory component in the axial plane. This results in torsion of the spine, with the most significant abnormality located in the apical region. As the deformity worsens, structural changes develop in the vertebrae and rib cage. Relationships between intrathoracic and abdominal organs may be distorted as the deformity becomes severe, but rarely are the organs’ functions compromised.
Classification of Scoliotic Curves
A variety of terms are used to describe the different types of scoliotic curves. Box 12-1 provides definitions for the most common ones.
Adult scoliosis: Spinal curvature present after skeletal maturity as a result of any cause.
Cervicothoracic curve: Any spinal curvature in which the apex is at C7 or T1.
Compensatory curve: Secondary curve located above or below the structural component that develops to maintain normal body alignment.
Congenital scoliosis: Scoliosis caused by bony abnormalities of the spine that are present at birth. The anomalies are classified as failure of vertebral formation or failure of segmentation.
Double curve: Scoliosis in which two lateral curves are present in the same section of spine.
Double major curve: Scoliosis in which two structural curves, usually of similar size and rotation, are present.
Double thoracic curve: Scoliosis with a structural upper thoracic curve; a larger, more deforming lower thoracic curve; and a relatively nonstructural lumbar curve.
Hysterical scoliosis: Nonstructural deformity of the spine that is a manifestation of a psychological disorder.
Idiopathic scoliosis: Structural spinal curvature, the cause of which has not been definitely established.
Kyphoscoliosis: Seen as an increased round back on a lateral radiograph, this condition may represent a true kyphotic deformity (as occurs in some pathologic conditions), or it may represent such excessive rotation of the spine that a lateral radiograph is actually reflecting the scoliotic deformity. (In idiopathic scoliosis, true kyphotic deformity does not occur.)
Lordoscoliosis: Structural scoliosis associated with increased swayback or loss of normal kyphosis within the measured curve; it is nearly always present in idiopathic scoliosis.
Lumbar curve: Spinal curvature in which the apex is between L1 and L4.
Lumbosacral curve: Spinal curvature in which the apex is at L5 or below.
Neuromuscular scoliosis: Scoliosis caused by a neurologic disorder of the central nervous system or muscle.
Nonstructural (functional) curve: Curvature that does not have a fixed deformity and may be compensatory in nature. The curve may be a result of leg length discrepancy (in which case it disappears when the patient is supine), poor posture, muscle spasm, or some other cause.
Primary curve: The first or earliest curve present.
Structural curve: Segment of the spine that has a fixed lateral curvature.
Thoracic curve: Spinal curvature in which the apex is between T2 and T11.
Thoracolumbar curve: Spinal curvature in which the apex is at T12, L1, or the T12-L1 interspace.
Idiopathic Scoliosis
Idiopathic scoliosis, for which a definitive cause of the deformity has not been established, is the most common type and accounts for nearly 80% of patients with structural scoliosis. The diagnosis of idiopathic scoliosis can be made only after a thorough physical and radiographic examination has ruled out neurologic causes, syndromes, and congenital anomalies. Idiopathic scoliosis may have its onset at any age during growth, but three fairly well defined peak periods are accepted: (1) in the first year of life, (2) at 5 to 6 years of age, and (3) after 11 years of age to the end of skeletal growth.
The term adolescent idiopathic scoliosis (AIS) is used when the deformity is recognized after the child has reached 10 years of age but before skeletal maturity, although it is typically noted before the onset of puberty. Infantile idiopathic (younger than 3 years) and juvenile idiopathic scoliosis (3 to 10 years old) are now included within “early-onset” scoliosis, a group that includes any type of scoliosis diagnosed before the age of 10 years.
Scoliosis recognized after skeletal maturity is defined as adult scoliosis .
Adolescent Idiopathic Scoliosis
Prevalence
The prevalence of radiographic curves measuring at least 10 degrees ranges from 1.5% to 3.0%, that of curves exceeding 20 degrees is between 0.3% and 0.5%, and that of curves exceeding 30 degrees is between 0.2% and 0.3%.
A definite relationship between idiopathic scoliosis and sex has been noted, particularly as the magnitude of the curve increases. The ratio of affected females to males has been reported to be 1 : 1 for curves between 6 and 10 degrees, 1.4 : 1 for curves between 11 and 20 degrees, 5.4 : 1 for curves exceeding 21 degrees but not requiring treatment, and 7.2 : 1 for curves requiring orthopaedic intervention. This sex prevalence in idiopathic scoliosis—that is, an equal prevalence between the sexes for small curves (<10 degrees), with increasing female prevalence for larger and progressive curves—has been reported by several authors. The clinical significance of these observations is that curve progression is more common in girls.
Natural History
Few current natural history studies have examined curve progression in the untreated, skeletally immature scoliosis population, and consensus is lacking in the literature regarding the definition of curve progression. Measurable increases in curve size of 5, 6, and 10 degrees have all been reported as being representative of progression. * Most studies use increases of more than 5 or 6 degrees as indicative of definite progression.
* References .
Natural History Before Skeletal Maturity
Individuals with untreated curves of less than 20 degrees are at low risk for progression, particularly as they approach skeletal maturity. Because some patients, however, have curves that progress over the years and ultimately lead to health problems, it is important to recognize the factors associated with curve progression, including patient sex, remaining growth, curve magnitude, and curve pattern. Factors of no predictive value for curve progression before skeletal maturity include a family history of scoliosis, patient height-to-weight ratio, lumbosacral transitional anomalies, thoracic kyphosis, lumbar lordosis, and spinal balance.
Sex
The majority of patients whose curves progress and ultimately require treatment are female. Although the exact reason for this phenomenon remains unknown, hormonal influences have been proposed.
Remaining Growth
A young patient’s remaining growth is usually assessed by four maturity indices: the Risser sign (a skeletal marker of the pelvis), hand and wrist skeletal maturity, peak height velocity (PHV), and in females, menarchal status (a physiologic marker).
The Risser sign is a radiographic measurement based on ossification of the iliac apophysis, which is divided into four quadrants, beginning on the lateral aspect of the iliac apophysis and progressing medially ( Fig. 12-2 ). The Risser sign proceeds from grade 0, no ossification, to grade 4, in which all four quadrants of the apophysis show ossification (“capping”). When the ossified apophysis has fused completely to the ilium (Risser grade 5), the patient is fully skeletally mature. Patients with Risser grade 0 or 1 (and to a lesser extent, grade 2) are at greatest risk for curve progression because a significant amount of spinal growth remains. A modified Risser grading system has been created in which a new group, Risser 0 with closed triradiate cartilage, and Risser 1 were found to be the best predictors of the beginning of rapid curve progression. The triradiate cartilage cannot be used as an independent predictor of curve stability, but it may serve as an additional indicator of skeletal maturity.
Menarchal status is a clinical measurement applicable only to females. A premenarchal girl is still in the active growth period. After menarche, she enters the deceleration phase of growth, and the likelihood of curve progression lessens. The Tanner index of maturity, which is based on assessment of breast and genital development, is another clinical index that has been used to determine a child’s remaining growth and thus can indirectly predict the risk for curve progression.
PHV is a measurement of the maximal skeletal growth that occurs during the adolescent growth spurt ( Fig. 12-3 ). Calculated from changes in a patient’s height measurements over time, PHV is fairly consistent in the published literature and is reported to be about 8.0 cm/yr for girls and 9.5 cm/yr for boys. The reported average age at PHV in North American girls is approximately 11.5 years. Closure of the triradiate cartilage, a radiographic index of maturity, occurs after PHV and before Risser grade 1 and menarche. For PHV to be clinically useful, serial height measurements must be obtained. Six-month intervals are preferred because shorter intervals may result in significant measurement error. If height data are not available from the patient’s records, the information can often be obtained from the family, school, or pediatrician. Although PHV requires analysis of serial height measurements collected over time, it is the earliest and best index available to demonstrate that growth is slowing and the risk for curve progression is diminishing. In boys, use of PHV to predict the period of remaining growth is superior to the Risser sign and chronologic age, and closure of the triradiate cartilage approximates the time of PHV.
A simplified skeletal maturity scoring system for AIS that uses radiographs of the hand has also been developed. The Tanner-Whitehouse III score, which is based on the radiographic appearance of the epiphyses of the distal ends of the radius and ulna, the small bones of the hand, and the digital skeletal maturity scoring system (which is based on the metacarpals and phalanges), correlates highly with curve acceleration in girls with AIS. This system is reliable and correlates more strongly with the behavior of idiopathic scoliosis than does the Risser sign. It appears to be strongly prognostic of future scoliosis curve behavior.
Curve Magnitude
The size of the existing curve when scoliosis is recognized is helpful in predicting curve progression. The combination of this factor and assessment of remaining growth is used to predict the natural history in young patients with scoliosis. Immature patients (premenarchal, Risser grade 0) with curves greater than 20 degrees are at substantial risk for progression of spinal deformity ( Table 12-1 ). For immature patients with curves exceeding 25 to 30 degrees, the risk for curve progression is believed to be significant enough to recommend orthotic management at the time of initial evaluation.
Risser Grade | Percentage of Curves That Progress | |
---|---|---|
Curves 5-19 Degrees | Curves 20-29 Degrees | |
0 or 1 | 22 | 68 |
2, 3, or 4 | 1.6 | 23 |
Curve Pattern
The curve pattern is useful for predicting curve progression. Double curves and thoracic curves are most likely to progress, followed by thoracolumbar curves. Lumbar scoliosis is reportedly the least likely to worsen.
Prognostic Testing
A genetic screening test called the ScoliScore is available as an adjunct to clinical and radiographic information to determine the risk for progression of AIS. This test can be used in white (North American, European, Eastern European, Middle Eastern) patients between the ages of 9 and 13 years with a mild scoliotic curve (<25 degrees). The stated goal of the test is to determine the risk that the curve will increase to 40 degrees or greater. Further independent verification of the test is needed before its usefulness for this condition can be determined.
Natural History After Skeletal Maturity
In general, the rate of progression of scoliosis in adulthood is much slower than that in adolescence and depends on the size of the curve once skeletal maturity has been reached. Regardless of the curve pattern, curves of less than 30 degrees in a mature individual are unlikely to progress. Conversely, approximately two thirds of curves that exceed 50 degrees worsen, with thoracic curves progressing nearly 1 degree per year. Lumbar curves also tend to progress in adulthood and may do so at a magnitude smaller than 50 degrees if they are accompanied by a transitory shift between the lower vertebrae.
In terms of the long-term status of adults with untreated scoliosis, several Swedish studies have reported an overall mortality rate greater than that predicted by national mortality statistics. However, these studies included patients with nonidiopathic scoliosis and those with infantile deformities. When examined selectively, the mortality rate of patients with AIS appeared to be the same as that in the general population. A significant increase in the mortality rate was identified for patients with infantile and juvenile idiopathic scoliosis. Respiratory failure and cardiovascular disease accounted for most early deaths. Respiratory failure developed in adults with severe scoliosis (>110 degrees) as normal aging further reduced their ventilatory capacity. In patients who underwent surgery for scoliosis, respiratory failure tended to not develop, thus suggesting a preventive effect of corrective surgery.
Chronic back pain is common in adults with scoliosis, although it is not related to the size or location of the curvature. The pain does not usually interfere with the patient’s ability to work or perform daily activities. Lumbar osteoarthritis may also be seen in up to 83% of adults with scoliosis, but it is not necessarily associated with the duration or intensity of back pain. Despite outwardly apparent deformities because of long-standing untreated scoliosis, most individuals have no significant psychological difficulties when compared with persons without scoliosis (the sole exception being a slight dissatisfaction with body image).
In summary, thoracic scoliosis of greater than 50 to 60 degrees in adulthood may progressively worsen and potentially reduce pulmonary function. Lumbar curves, especially those greater than 50 degrees, are also likely to progress in adulthood and lead to osteoarthritis. Therefore, even when cosmetic factors are not taken into account, aggressive treatment of a child with a significant spinal deformity is justified.
Scoliosis Screening
School Screening Programs
A number of medical organizations support the general screening of children for scoliosis. In 2008, the Scoliosis Research Society (SRS), the American Academy of Orthopaedic Surgeons (AAOS), the Pediatric Orthopaedic Society of North America (POSNA), and the American Academy of Pediatrics (AAP) endorsed an information statement in support of screening for AIS. This 2008 statement was a response to a recommendation made by the U.S. Preventive Service Task Force. For years this task force stated that the evidence was insufficient to recommend either for or against routine screening of asymptomatic adolescents for idiopathic scoliosis. In 2004, based on a brief evidence update, the task force changed its position and recommended against routine screening. It reported that no good evidence could be found that screening of asymptomatic adolescents detects idiopathic scoliosis at an earlier stage. However, as the primary care providers for adolescents with idiopathic scoliosis, the SRS, AAOS, POSNA, and AAP do not support any recommendation against scoliosis screening given the available literature. If scoliosis screening is undertaken, the SRS, AAOS, POSNA, and AAP agree that girls should be screened twice, at ages 10 and 12 (grades 5 and 7), and boys once, at age 13 or 14 (grades 8 or 9).
The clinical logic behind school screening for idiopathic scoliosis assumes that screening is an accurate and reliable method of detecting curvatures, early detection results in improved health outcomes, and brace therapy is effective in altering the natural history of the deformity. The implications of these assumptions are that small curvatures detected through screening are likely to progress to curvatures of clinical significance, that scoliosis causes significant health problems, and that the benefits of early detection outweigh the potential adverse effects of screening and treatment. The many proponents of school screening believe that these assumptions are successfully addressed by school screening programs. The idea of general scoliosis screening is not universally accepted, however, and is becoming more controversial. Some authors argue that school screening programs have not reduced the prevalence or incidence of scoliosis requiring treatment, are not cost-effective, and result in children with no scoliosis or only a mild degree of curvature that does not require treatment being unnecessarily referred to orthopaedic surgeons or radiologists. However, a study involving more than 150,000 students reported that school screening is predictive and sensitive with a low referral rate. The authors recommended that screening be continued to facilitate early initiation of conservative treatment.
Screening Methods
Several clinical signs are indicative of possible scoliosis and are frequently used in screening programs, including shoulder asymmetry, unequal scapular prominence, appearance of an elevated or prominent hip, greater space between the arm and body on one side (with the arms hanging loosely at the side), head not centered over the pelvis, and a positive Adams forward-bending test. The Adams test is performed by having the child bend forward until the spine is horizontal and, while examining the patient from the rear, noting whether one side of the back appears higher than the other ( Fig. 12-4 ). This test is the most common noninvasive clinical method for evaluating scoliosis.
One of the constant features of structural scoliosis is axial rotation of the vertebrae affected by the curve. The spinous processes almost always rotate toward the concavity of the curvature. Rotation of the thoracic vertebrae is also impaired by rotation and deformity of the attached rib cage, with elevation on the side of the convexity and depression on the side of the concavity. This asymmetry is significantly accentuated when the patient bends forward. Examining patients in the forward-bent position is the standard method used to detect mild degrees of curvature in mass screening programs. In an effort to quantitatively assess the asymmetry and thus establish an appropriate degree of deformity that justifies referral for medical evaluation, Bunnell introduced the scoliometer in 1984 ( Fig. 12-5 ). This specially designed inclinometer (similar to a level used in a wood shop) measures the angle of vertebral rotation. When using the scoliometer, it is important that the screener stand behind the patient to view the back (as in the Adams forward-bending test). The screener’s eyes should be on a horizontal plane with the maximal deformity of the back. If the patient bends forward approximately 45 degrees, the outline of the trunk at the level of the thoracic spine is seen. With further bending, the outline of the trunk at the level of the thoracolumbar spine is seen, followed by the outline at the level of the lumbar spine.
If a rotational deformity of the back is noted at any level, the scoliometer is placed gently on the person’s back at the apex of the deformity, perpendicular to the long axis of the body, and the angle of inclination is read directly from the scale. Originally, the recommendation for orthopaedic referral was a 5-degree angle of trunk rotation at any level of the spine, which meant that the chance of missing a curve exceeding 20 degrees was small. However, because of excessive referrals, this recommendation has been modified to a 7-degree angle of trunk rotation. With this criterion the chance of missing a curve greater than 30 degrees (the curve magnitude at which bracing is usually initiated) is low. When this approach is used, the referral rate is approximately 3% of persons screened, with a 95% detection rate of curves requiring brace treatment.
Etiology
The exact cause of idiopathic scoliosis remains unknown despite considerable investigation. Although growth has a significant influence on the deformity, it is not considered a causative factor. Since the 1990s, much of the research on the etiology of scoliosis has focused on central neurologic dysfunction, connective tissue abnormalities, and most recently, genetic factors. These influences have supplanted previous theories that idiopathic scoliosis was caused by a biochemical or nutritional deficiency, structural defects, or endocrine abnormality. The true cause is probably multifactorial and involves several of the aforementioned factors.
Neurologic Dysfunction
The literature in the past supported an underlying neurologic abnormality as the primary etiologic factor in idiopathic scoliosis. Dysfunction of the vestibular, ocular, or proprioceptive systems causes an interruption of equilibrium that is indicative of abnormalities involving the posterior column of the proximal portion of the spinal cord, brainstem, and cerebral cortex. † Somatosensory evoked potentials are useful parameters for evaluating neurologic function. Responses to vibratory stimuli are reportedly reduced significantly and asymmetric between the left and right sides in scoliotic patients when compared with controls. These findings support the concept that an aberration in function of the posterior column pathway of the spinal cord may play a role. Other investigators, however, have been unable to corroborate this opinion. Altered balance affecting foot posture and gait, particularly pes cavus, has been reported. In addition to abnormalities in the sensory pathways, motor dysfunction has been reported, thus suggesting that the organization of the entire brain is asymmetric in individuals with scoliosis. Regional differences in brain volume have also been reported in patients with AIS.
† References .
Another neurologically based theory regarding the cause of idiopathic scoliosis involved the role of melatonin in regulating normal spine growth. Secreted by the pineal gland, this neurohormone controls the circadian rhythm. Experiments on pinealectomized chickens revealed that melatonin deficiency contributes to the development of scoliosis in this model, probably by interfering with the normal symmetric growth of the proprioceptive system involving the paraspinal muscles and the spine. However, melatonin therapy after pinealectomy in chickens had no effect on the development or progression of scoliosis, thus raising doubts about its role. Significantly lower melatonin levels and impaired melatonin signaling have been reported in patients with scoliosis versus controls, but other investigators have refuted this finding. In addition, studies report no evidence to support mutations in the gene coding for human melatonin receptor.Connective Tissue Abnormalities
Another focus of research is on alterations in connective tissue involving the spine, paraspinal muscles, and platelets in patients with scoliosis. Differences in collagen have been found between normal individuals and those with AIS; however, this finding is not universal. These changes may be secondary to the mechanical effects of the spinal deformity rather than reflecting mutations in collagen itself. This theory has been substantiated by segregation analysis of genetic markers linked to the structural genes encoding types I and II collagen.
Other components of connective tissue may be abnormal as well. In histologic studies of the ligamentum flavum in scoliotic patients, the elastic fiber system was found to have disarranged fibers, a marked decrease in fiber density, and a nonuniform distribution of fibers throughout the ligament. These findings suggest that the elastic fiber system (which is predominantly fibrillin) may play a significant role in the pathogenesis of idiopathic scoliosis in some individuals. Bone mineral density has also been shown to be lower in young adolescents with scoliosis. It is uncertain, however, whether this finding is related to the primary cause of the disease or whether it is secondary to the asymmetric mechanical forces associated with the back deformities. Further investigation is needed to determine whether those with osteopenia have the same cause, pathogenesis, and risk for progression as those without osteopenia.
The paravertebral musculature in patients with scoliosis may exhibit abnormalities in the muscle spindle, in individual muscle fiber morphology, in histochemistry, and on electromyography. Some of these changes are more pronounced with severe curves, but they are believed to be secondary to muscle adaptation to the curve and not a primary cause of the deformity.
Abnormal platelet structure and function have been reported in patients with scoliosis. ‡ Calmodulin, a calcium-binding receptor protein found in platelets and skeletal muscle, regulates the contractile protein system (actin and myosin). If an underlying systemic contractile disorder is present, both platelets and skeletal muscle will be affected. Thus, measurable abnormalities of calmodulin in platelets are indicative of skeletal muscle abnormalities. Platelet calmodulin levels in adolescents with progressive scoliosis are significantly higher than those in normal individuals, in patients with stable curves, and in those whose progressive curves were stabilized by bracing or spinal fusion. Although this finding cannot be implicated as a direct cause of scoliosis, it may become a useful predictor of curve progression, but controversy remains.
‡ References .
Genetic Factors
Because idiopathic scoliosis can be seen in multiple members of the same family, attempts have been made to determine the genetic factors involved. Several extensive clinical studies of affected families conducted in the 1960s and 1970s revealed a high prevalence of familial scoliosis (6.9% to 11.1% of first-degree relatives). Both dominant and multifactorial inheritance patterns have been suggested to explain the genetic contributions to AIS ( Fig. 12-6 ). Not all studies suggested a genetic basis, however; some implicated older maternal age at the time of childbirth.
More recent literature has shown evidence of a strong genetic tendency in some families of patients with AIS. § In a meta-analysis of scoliosis in twins, monozygous twins had a significantly higher rate of concordance than did dizygous twins, and the curves in monozygous twins developed and progressed together.
§ References .
Studies are now under way to identify the genes that cause scoliosis and its progression. In this new investigative frontier, genomic DNA from families with apparent autosomal dominant inheritance of adolescent scoliosis is analyzed for linkage with disease. To date, findings have been reported that involve chromosomes 3p26.3 single nucleotide polymorphism, 6, 8, 9, 10, 16, 17, 18q, and 19, as well as the X chromosome. Familial analysis using this approach may enable investigators to track causative genes, such as the CHD7 gene associated with the CHARGE syndrome ( c oloboma of the eye, h eart anomaly, choanal a tresia, r etardation, and g enital and e ar anomalies) and later-onset idiopathic scoliosis, thereby providing more insight into the etiology of idiopathic scoliosis. It may also allow the identification of diagnostic markers.Pathophysiology
The extent of structural changes varies with the degree of scoliosis. These changes are greatest at the apex of the curve and diminish toward each end. In structural scoliosis, the vertebral body is rotated toward the convex side of the lateral curvature, so the spinous processes of the vertebrae are rotated toward the concavity of the curve. The asymmetric deformities found within the bodies of scoliotic vertebrae differ substantially from the vertebrae in normal spines. Forces during compression and distraction act on the growing spine and produce wedge-shaped changes in the vertebrae, which become higher on the convex side and lower on the concave side ( Fig. 12-7 ). The vertebral body becomes condensed on the concave side as a result of the greater pressure, and it is expanded and thinned on the convex side. The concave facets have a significantly thicker cortex than the convex facets do. In addition to changes in the frontal and axial planes, the scoliotic portion of the spine is lordotic in the sagittal plane. This three-dimensional deformity is appropriately termed torsion of the spine and is greatest at the apical region.
A scoliotic spine has associated changes in the neural canal and posterior arch. Abnormality in the neurocentral synchondrosis may contribute to the scoliotic deformity, as has been shown experimentally. With more severe deformities, the laminae on the convex side are broad and widely separated, whereas those on the concave side are narrow and close together ( Fig. 12-8 ). The pedicles are shorter and thinner (narrower endosteal transverse width) on the concave side. The transverse processes more closely approach the sagittal plane on the convex side and are more in the frontal plane on the concave side. The intraspinal canal becomes distorted because of the misshapen pedicles and articular processes ( Fig. 12-9 ).
As a result of pressure over time, the intervertebral disks on the concave side narrow and may show degenerative changes in adulthood. The adjoining portion of the vertebra becomes sclerotic, with marginal lipping.
In patients with right thoracic idiopathic scoliosis, the aorta is positioned more laterally and posteriorly relative to the vertebral body than its position in patients without spinal deformity. As demonstrated by magnetic resonance imaging (MRI) studies, this finding is even more striking with increasing curve severity and apical vertebral rotation.
The thoracic cage is also affected by the deformity. Because of rotation of the thoracic vertebrae, the ribs on the convex side are directed posteriorly, which produces a rib prominence that in severe cases may be referred to as a “razorback.” On the concave side the ribs are rotated forward, which can potentially produce prominence of the anterior chest wall. The sternum may be asymmetric and laterally displaced from the midline. The breasts are often mildly asymmetric as a result of the chest wall deformity. This breast asymmetry is often a major concern of patients.
Because of the spinal deformity, the thoracic cavity is no longer symmetric. Its capacity is diminished on the convex side and increased on the concave side. In severe cases with marked angulation of the ribs posteriorly, lung function may be altered.
In severe cases of scoliosis in which the shape of the intraspinal canal is distorted, the spinal cord may be stretched over the concave side, but rarely is any neurologic deficit present. Cord compression with neurologic deficit usually occurs only with extreme deformities that are accentuated by marked thoracic kyphosis.
Clinical Features
Initial Signs and Symptoms
Adolescents with scoliosis do not usually seek medical evaluation because of back discomfort but rather because of some physical aspect of their deformity, such as a high shoulder, one-sided prominence of a scapula or breast, elevated or protuberant iliac crest, and asymmetry in flank creases and the trunk. Except for being noticed personally by the adolescent, these findings are often first appreciated during school screening programs for scoliosis or during back-to-school examinations by the family physician.
Though uncommon, back pain is present in individuals with idiopathic scoliosis more often than was previously thought. Nearly 32% of adolescents with idiopathic scoliosis complain of back discomfort at some point (23% at initial evaluation and 9% during the period of observation). A significant association has been found between back pain and age older than 15 years, Risser grade 2 or greater skeletal maturity, postmenarchal status, and a history of injury. Back pain does not seem to be related to the sex of the patient, family history of scoliosis, limb length discrepancies, magnitude or type of curve, or spinal alignment. In patients with back pain, the source of discomfort can be identified only 10% of the time despite the use of appropriate imaging studies. The most common causes of discomfort are associated spondylolysis, spondylolisthesis, and Scheuermann kyphosis. Less likely causes include spinal cord syrinx, disk herniation, tethered spinal cord, and tumor. A painful left thoracic curve or an abnormal neurologic finding is most predictive of an underlying pathologic condition of the spinal cord.
When an adolescent with presumed idiopathic scoliosis has back pain, a careful history should be obtained, a thorough physical examination performed, and plain radiographs ordered. If findings on this initial evaluation are normal, a diagnosis of idiopathic scoliosis can be made, the scoliosis can be treated appropriately, and nonsurgical treatment of the back pain can be initiated. It is not necessary to perform extensive diagnostic studies in every adolescent with scoliosis and back pain. If the patient’s symptoms persist and significantly restrict normal activities and if the findings on neurologic examination are normal, a technetium bone scan may be useful. If the neurologic findings are abnormal, MRI of the spinal cord is indicated. Unlike backache in adults with lumbar scoliosis, backache in adolescents is not usually due to degenerative arthritis in the posterior articulations or to nerve root irritation.
Respiratory symptoms are uncommon in patients with AIS. Studies have shown that clinically significant cardiopulmonary compromise does not usually occur until the magnitude of the curve approaches 100 degrees, vital capacity becomes less than 45%, or thoracic lordosis significantly narrows the anteroposterior (AP) dimensions of the chest. Most curves are treated operatively before the spinal deformity becomes this severe. However, when the kinematics of the chest cage and spine during breathing is evaluated, individuals with AIS have decreased motion in comparison to healthy individuals. In patients with impaired pulmonary function, this stiffness plays a significant role.
Neurologic deficits are also rare in persons with AIS. Should an adolescent describe any suspicious symptoms (e.g., persistent neck pain, frequent headaches, ataxia, weakness), meticulous attention must be paid to the neurologic portion of the physical examination. If any neurologic deficits are found or if the convexity of the thoracic curve is to the left, appropriate imaging of the neural axis is undertaken. Normally, the convexity of thoracic curves in AIS is directed to the right. Abnormal left thoracic curves are more common in those with an underlying syrinx.
Physical Examination
Physical examination of an adolescent with idiopathic scoliosis should be performed with the patient properly draped. It is convenient if the patient wears a swimsuit. Alternatively, the patient may be dressed in underpants and an examination gown open at the back. The patient’s entire back, including the shoulders and iliac crests, must be visible.
The skin is inspected closely for abnormalities such as midline hemangiomas, hair tufts, and dimpling in the lumbosacral region. Any of these surface findings may indicate the presence of an underlying spinal cord abnormality such as a tethered cord or diastematomyelia. The spinous processes are palpated from the cervical region to the sacrum for any deficiencies or areas of discomfort. Occasionally, absence of a spinous process is noted, which usually corresponds to spina bifida occulta seen on a spinal radiograph ( Fig. 12-10 ).
With the patient standing, the examiner should determine whether the iliac crests are level. If they are not, a lower limb length discrepancy is likely, which can be quantified by placing measured blocks under the short extremity until the iliac crests are level. Leg length discrepancy can be responsible for the appearance of scoliosis, and the condition must not be overlooked. The back is then examined for asymmetry of the shoulders and flank creases, unequal scapular prominence, prominent iliac crest, and increased space between the arm and body on one side with respect to the other with the arms hanging loosely at the side ( Fig. 12-11 ).
Although these findings are consistent with scoliosis, the best noninvasive clinical test for evaluating spinal curvature is the Adams forward-bending test (see Fig. 12-4 ). With this test, the degree and direction of associated rotation of the vertebrae are clearly demonstrated. The examiner observes the adolescent from behind as the patient bends forward at the waist until the spine is horizontal. The patient’s knees should be straight, the feet together, the arms dependent, and the palms in opposition. Vertebral rotation causes one side of the back to appear higher. This is noted as rib prominence in the thoracic region or as paraspinal fullness in the lumbar region. Such asymmetry can be quantified with a scoliometer, which can provide measurements documenting change over time (see Fig. 12-5 ).
Frequently, if the patient is inspected from the front, asymmetry of the pectoral regions, breasts, or rib cage may be evident. Although these asymmetries are probably related to the spinal curvature, they may also occur in individuals without scoliosis. Occasionally, breast asymmetry is the primary concern of the patient and parents. Families should be informed that correcting the scoliosis may have little, if any, influence on this asymmetry.
Spinal balance is assessed by two different methods. The first method, known as coronal balance, is to determine the alignment of the head over the pelvis. The head is almost always positioned directly above the gluteal crease in patients with idiopathic scoliosis. To assess this balance, a plumb line is held from the base of the skull or from the spinous process of C7. Normally, the plumb line should not deviate from the center of the gluteal crease by more than 1 to 2 cm ( Fig. 12-12 ). If it does, this finding should be considered atypical, and a meticulous neurologic examination is necessary to rule out coexisting neurologic pathology. The second method, known as trunk balance, is to assess the position of the trunk over the pelvis. Unlike the position of the head over the pelvis, patients with idiopathic scoliosis may have significant imbalance of the trunk over the pelvis ( Fig. 12-13 ), particularly with single thoracic curve patterns.
Next, the examiner inspects the patient from the side and observes the sagittal contours of the spine. Normally, in individuals with idiopathic scoliosis the sagittal plane appears hypokyphotic throughout the scoliotic segment, with actual lordosis being present radiographically in the apex of the deformity. In more severe cases the entire sagittal plane may actually be lordotic and lead to a very narrow AP diameter of the thoracic cage. Rarely, as much as 90 degrees of rotation may be present in the apical vertebrae within the curve. In this instance the examiner is actually assessing the AP aspect of the spinal curvature when viewing the spine from the side. The resulting clinical appearance is an apparently increased kyphosis in the sagittal plane because in reality, the scoliotic deformity is being viewed from the side (see Fig. 12-7 ).
Sagittal plane deformity in an individual with apparent idiopathic scoliosis may also be an indicator of syringomyelia. If hypokyphosis is absent clinically and radiographically and if little or no rotation (rib prominence) is present, a diagnosis of idiopathic scoliosis should be made only after a syrinx has been ruled out.
Neurologic Examination
Because idiopathic scoliosis is basically a diagnosis of exclusion, a thorough evaluation is necessary to rule out a neurologic cause of the deformity. The neurologic examination begins by assessing the patient’s reflexes. Examination of the superficial abdominal reflexes is useful for determining which patients should undergo MRI to rule out syringomyelia ( Fig. 12-14 ).
The abdominal reflex examination is performed with the patient supine on an examination table and the arms relaxed along the side of the body. An area approximately 10 cm above and below the umbilicus and to each anterior axillary line is exposed. With the patient relaxed, the bluntly pointed handle of a reflex hammer is used to lightly stroke the skin in each quadrant over a distance of 10 cm ( Fig. 12-15 ). The stroke starts lateral to the umbilicus near the anterior axillary line and is directed diagonally toward the umbilicus in each quadrant. The umbilicus is observed for deviation toward the side on which the test is performed. If these reflexes are consistently present on one side and absent on the other side, further evaluation is warranted because this finding does not occur in neurologically normal patients with scoliosis. However, other variations might occur, such as absent reflexes in all quadrants.
The patellar and Achilles tendon reflexes should also be tested, with the expectation that they will be symmetric. Muscle testing and examination of the range of motion of all four extremities should always be conducted. The hands and feet should be examined for abnormal posture and for evidence of abnormal sensation (excessive callus formation or nail bed irregularities). Abnormal findings may be the only clinical evidence of underlying pathology of the neural axis, such as syringomyelia or tethered cord.
Patient Maturity
Sexual maturity can be assessed during the physical examination according to the Tanner system, which assesses breast and pubic hair development in girls and genital and pubic hair development in boys. Although the Tanner system may provide an indication of the patient’s physical maturity, more practical clinical emphasis is placed on the patient’s menarchal status and increase in height over time and on assessment of skeletal indicators of maturity (e.g., Risser sign, open or closed triradiate cartilage, and the Tanner-Whitehouse III score, which is based on the radiographic appearance of the epiphyses of the distal ends of the radius and ulna, the small bones of the hand, and the digital skeletal maturity scoring system).
Radiographic Findings
Plain Radiography
If older conventional radiology equipment is used, the initial examination of the spine should include posteroanterior (PA) and lateral radiographs on 36 × 14-inch film cassettes. With these long cassettes, nearly all the important radiographic features can be assessed on a single film. On the PA projection, such features include the curve pattern in its entirety, the type of scoliosis (congenital or idiopathic), the overall balance of the spine and trunk, skeletal maturity (as determined by the Risser sign, triradiate cartilage, or capital femoral physis), and the presence of a lower limb length discrepancy (pelvic tilt). The lateral projection is useful initially to evaluate the global sagittal contour of the thoracic and lumbar spine, determine the presence and severity of thoracic hypokyphosis, and screen for spondylolysis and spondylolisthesis. In very young children, 17 × 14-inch film cassettes may be large enough to provide all this information; however, these shorter cassettes are too small to be used for adolescent patients. With female patients 10 years or older, the radiology technologist should always inquire about the patient’s last menstrual period and the possibility of pregnancy; if pregnancy is suspected, the radiographic evaluation can be postponed.
Today, with the more common use of computed radiography and the picture archive and communication system (PACS), further technologic development in scoliosis imaging with less ionizing radiation has been achieved. Older conventional film images can be digitized into a PACS for monitor viewing. With computed radiography, an imaging plate inside a cassette is exposed and subsequently processed by a reader that converts the image into a digital format to be viewed and stored in a PACS. A series of up to four plates can be placed in a long-length cassette holder and exposed simultaneously to acquire a long scoliosis image in both the PA and lateral projections. Special software electronically stitches the images together for monitor display or, if needed, printing. Newer imaging opportunities are available with the use of a recently introduced low-dose radiation digital stereoradiography system known as the EOS system (EOS Imaging, Paris). At this point this system is found primarily in academic institutions, but its use will probably expand over time.
After the initial radiographic evaluation has been accomplished, effort is made to limit the number of follow-up films, thereby reducing the amount of radiation exposure. During the course of routine follow-up examinations, only the PA projection is needed. No set interval from one radiographic examination to the next has been determined for all patients. The period between evaluations depends on the maturity of the patient and the size of the spinal curvature. For example, a premenarchal, Risser grade 0, 11-year-old girl with a 25-degree thoracic curve should return for radiographic reevaluation after a 4-month interval, whereas a 2-year postmenarchal, Risser grade 4, 14-year-old girl with a 30-degree curve need not return for reevaluation before 1 year. In most cases the interval between radiographic evaluations ranges from 4 to 6 months.
During radiographic evaluation the patient should stand as erect as possible with the knees straight and the feet together ( Fig. 12-16 ). The patient should be barefoot so that if lower limb length inequality is suspected, the appropriate lift can be placed under the short limb. Unsupported sitting views are taken if the patient is unable to stand. Twisting of the trunk should be avoided. To ensure sufficient cephalic visualization, the upper limit of the cassette should extend to the external auditory meatus. In the upright lateral projection, the patient’s shoulders are flexed forward, the elbows are fully flexed, and the fists should rest on the clavicles. For the lateral radiograph, this position allows the best representation of the patient’s functional sagittal balance while still providing adequate lateral radiographic visualization of the spine. Although AP radiographs of the hand and wrist are used today to determine skeletal age, maturity continues to be more commonly assessed with the Risser sign of the iliac crest.
Bending radiographs (including the fulcrum bend test) and traction radiographs obtained with the patient supine are usually reserved for preoperative evaluation of spinal flexibility. The information gained from these AP radiographs can be helpful in determining appropriate fusion levels.
Measurement of Curve Magnitude
The Cobb method is considered the standard for measuring curve size. The measurement is started by determining the end vertebrae (top and bottom of the curve). The cephalic end vertebra’s superior surface and the caudal end vertebra’s inferior surface have the greatest amount of tilt into the curve ( Fig. 12-17 ). The intervertebral space on the concave side of the curve is generally wider above the cephalic (top) vertebra and narrower below it. The opposite applies to the inferior surface of the caudal (bottom) vertebra. Using a transparent plastic goniometer, the examiner draws lines perpendicular to the top vertebra’s superior surface and the bottom vertebra’s inferior surface. The angle formed by the intersection of these lines is the Cobb angle. If a second curve is present below the primary curve, the original curve’s bottom vertebra becomes the cephalic end vertebra for the second curve, and the same line along its inferior surface is used. With the use of PACSs in radiology departments today, electronic angular measurements simplify this process.
Although the Cobb method has good overall reliability, some variation among different observers’ measurements is always present. Such variability averages 7.2 degrees if the end vertebrae are not preselected but improves to 6.3 degrees when they are preselected. Another aspect of the accuracy of the Cobb method is that to achieve 95% statistical confidence that a true change in curve size has occurred, a measurement difference of 10 degrees between radiographs taken at different times would be needed. This finding is of particular interest because many studies use a criterion of a 5- to 6-degree change in curve size to determine the success or failure of brace treatment for scoliosis. This information reinforces the importance of meticulous line drawings and precise measurements.
Measurement of Vertebral Rotation
The Perdriolle method and the Nash-Moe method are the two most common means of assessing vertebral rotation on a plain frontal radiograph. The Perdriolle method uses a transparent torsionometer that is overlaid on the radiograph ( Fig. 12-18 ). The edges of the curve’s apical vertebra and its rotated pedicle constitute the landmarks. This method is accurate for measuring rotations that are less than 30 degrees. However, once the scoliotic spine has undergone instrumentation, the landmarks of the apical vertebra may become obstructed by the shadows of the rods or hooks, thus making accurate measurements difficult to obtain.
In the Nash-Moe method, the relationship of the pedicle to the center of the vertebral body is observed on AP radiographs, and the rotation is divided into five grades: grade 0 when both pedicles are symmetric, grade I when the convex pedicle has moved away from the side of the vertebral body, grade III when the convex pedicle is in the center of the vertebral body, grade II when the rotation is between grades I and III, and grade IV when the convex pedicle has moved past the midline. This method has only fair reliability.
Computed tomography (CT) can also be used to assess vertebral rotation. Although CT is more expensive, it is more accurate than the Nash-Moe method. For example, Nash-Moe grade 0 vertebrae have been found to have up to 11 degrees of rotation when measured with CT.
Measurement of Spinal Balance
Measurements of spinal balance are important to assess the amount of decompensation that exists preoperatively or that can occur postoperatively. Coronal balance represents the horizontal distance between the midpoint of C7 and the center of the pelvis ( Fig. 12-19 ). Coronal balance is considered poor, or decompensated, if this distance exceeds 2 cm. Trunk balance assesses the position of the thorax over the pelvis and is measured via lateral trunk shift. Lateral trunk shift is measured by drawing a horizontal line to the edges of the ribs of the trunk and a perpendicular line that bisects this horizontal line; the distance between this perpendicular line and the center of the pelvis represents lateral trunk shift ( Fig. 12-20 ). Another parameter used to indirectly assess trunk balance is thoracic apical vertebral translation, which is the distance measured between the midpoint of the apical thoracic vertebra and the C7 plumb line. Because the midpoint of C7 rarely corresponds to the exact center of the pelvis, thoracic apical vertebral translation must be used in combination with the coronal balance measurement to reflect trunk balance.
Measurement of Kyphosis and Lordosis on Lateral Radiographs
The end vertebrae are the last vertebrae that are maximally tilted into the concavity of the curve. In the thoracic area, the upper end vertebra is usually T3 or T4, and the lower end vertebra is T12. Perpendicular lines are drawn to the inferior and superior end-plates. The angle formed between the two perpendicular lines represents the degree of thoracic kyphosis. Normal thoracic kyphosis ranges from 20 to 45 degrees. No kyphosis or lordosis is present at the thoracolumbar junction (between T11 and L1-2). Lumbar lordosis usually begins at L1-2 and gradually increases caudally to the sacrum. To measure lumbar lordosis, the lower end vertebra for the thoracic curve becomes the upper end vertebra. The lower end vertebra for the measurement of lumbar lordosis is usually L5 or S1. Although attempts have been made to determine normal lumbar lordosis, no consensus has been achieved; reported normal values range from 50 to 65 degrees. Thoracic and lumbar regional alignment is very similar between adolescents and adults. Effort has been made to standardize levels of sagittal plane measurement to achieve more consistency between studies. Recommendations for sagittal plane measurements include T2-5, T5-12, T2-12, T10-L2, and T12-S1. All but the T2-5 measurement have shown interobserver reliability.
Surface Imaging
In an effort to decrease the amount of radiation exposure during the course of scoliosis management, techniques have been developed to assess changes in body surface in patients with scoliosis. The goals of surface imaging are to appropriately identify scoliosis, monitor curve progression, and provide information that can be used to make treatment decisions. However, natural history data and, in most cases, treatment decisions are based on Cobb angle measurements made from upright radiographs.
For surface-imaging systems to be useful, they must demonstrate some consistency with Cobb angle measurements. Moire topography, the Quantec spinal image system (raster-stereophotography), and the Integrated Shape Imaging System (ISIS) are three sophisticated techniques that use computer analysis of digitized topographic information ( Fig. 12-21 ). The presence, level, and side of the scoliosis curvature have been documented by these topographic techniques in patients with standard rotation; however, it is not possible to determine the magnitude of the scoliosis with sufficient accuracy to be clinically useful. ‖ These techniques continue to be investigated in an effort to determine their role in the management of scoliosis.
‖ References .
Magnetic Resonance Imaging
By providing a clear anatomic picture of abnormalities that occur within the spinal canal, MRI can be an extremely valuable tool in the assessment of scoliosis. Syringomyelia, Arnold-Chiari malformations, abnormalities in the brainstem, hydromyelia, spinal cord tumors, spinal cord tethering, and diastematomyelia have all been identified in individuals previously thought to have idiopathic scoliosis. ¶ However, because these abnormalities are rare, performing MRI as part of routine screening programs is impractical and cost prohibitive. MRI is usually reserved for patients with an atypical manifestation of idiopathic scoliosis. Although atypical manifestations have never been specifically defined, they generally include patients with neck pain and headache (particularly with exertion) and abnormal neurologic findings such as ataxia, weakness, and progressive foot deformities; patients with unusually rapid curve progression or excessive thoracic kyphosis; or patients requiring surgery who have left thoracic curves or asymmetric abdominal reflexes. Curves greater than 70 degrees do not increase the likelihood of finding a spinal cord anomaly. Routine preoperative MRI is not indicated for typical AIS if findings on the neurologic examination are normal.
¶ References .
Computed Tomography
Although CT may clearly demonstrate congenital abnormalities in the spine, it is rarely needed in the routine assessment of individuals with idiopathic scoliosis. However, with the emerging use of vertebral column resection (VCR) in those with extremely severe AIS, preoperative three-dimensional CT imaging is indicated to clarify the deformed spine’s anatomy. It also remains a useful tool postoperatively (particularly with three-dimensional reconstruction) for assessing bone fusion mass if pseudarthrosis is suspected, for evaluating changes in spinal rotation, and for verifying pedicle screw placement. # In addition, CT-myelography affords improved evaluation of the spinal cord when retained metal implants limit the effectiveness of MRI.
# References .
Treatment
Most adolescents with idiopathic scoliosis do not require treatment because of the low probability that their curves will progress. Treatment is warranted only for patients whose scoliotic curves are at substantial risk of worsening over time or for those with severe curves at initial evaluation. A clear understanding of the risk factors discussed earlier in the natural history section is useful in determining which patients need treatment, regardless of whether they are skeletally immature or mature.
In selecting treatment, the physician must consider the adolescent’s remaining growth potential, the severity of the curve at the time of detection, and the pattern and location of the scoliosis. The cosmetic appearance and social factors that may have an impact on treatment also enter into the decision-making process. The treatment choices available are observation, nonsurgical intervention, and surgical intervention, and it is imperative that physicians know which options are appropriate for each individual patient ( Table 12-2 provides general guidelines). Actively growing adolescents (Risser grade 2 or lower) with curves between 30 and 45 degrees should start brace therapy at the time of the initial visit. In very immature patients (Risser grade 0 and premenarchal if female) with curves exceeding 25 degrees, bracing should be started immediately. In most cases, growing adolescents with curves exceeding 45 to 50 degrees require operative stabilization because other forms of treatment are ineffective in controlling or correcting the scoliosis. Skeletally mature individuals with curves exceeding 50 to 55 degrees are also at risk for continued curve progression and should be considered for surgical treatment. Possible exceptions include patients with well-balanced double curves less than 60 degrees whose clinical appearance is acceptable to them. Continued observation would be necessary to document progression of the scoliosis, which would necessitate surgery.
Curve Magnitude (Degrees) | Risser Sign | ||
---|---|---|---|
Grade 0/Premenarchal | Grade 1 or 2 | Grade 3, 4, or 5 | |
<25 | Observation | Observation | Observation |
30-40 | Brace therapy (begin when the curve is >25 degrees) | Brace therapy | Observation |
>45 | Surgery | Surgery | Surgery (when the curve is >50 degrees) |
Observation
In general, no treatment is needed for curves less than 25 degrees, regardless of the patient’s maturity. Follow-up examinations are necessary, with the interval between visits depending on the patient’s maturity and the size of the curve. For example, a premenarchal Risser grade 0 adolescent with an initial curve measuring 24 degrees should undergo follow-up examinations every 3 to 4 months, and a brace may be needed if the curve progresses. For more skeletally mature patients (Risser grade 3 or higher), longer intervals between visits (e.g., 6 months) are appropriate because curve progression usually occurs at a slower rate, if at all. Clearly, predetermined guidelines do not apply to all cases, and follow-up must be individualized.
The magnitude of the patient’s curve at initial evaluation helps determine the frequency of follow-up visits. In general, for growing children with small curves (<20 degrees), the next follow-up evaluation should be approximately 6 months later. If the curve is between 20 and 30 degrees, radiographs should be obtained 3 to 4 months later because treatment may be necessary if the curve progresses 5 degrees or more. For patients whose curves do not progress, observation continues and the interval between visits gradually lengthens as maturity approaches.
What constitutes true curve progression is a matter of some debate. Traditionally, an increase in curve size of more than 5 to 6 degrees has been taken as representing progression; however, a 7- to 10-degree change in measurement is more accurate if a 95% confidence level is used to determine true progression. This should be taken into consideration when deciding whether the measured change in the patient’s scoliosis warrants either nonsurgical or surgical intervention. Nevertheless, throughout the literature a 5- to 6-degree measured change is considered indicative of curve progression. Not all progressive curves exceeding 30 degrees require treatment; the decision depends on the adolescent’s maturity and the size of the curve.
Nonsurgical Treatment
To be considered effective, nonsurgical treatment must prevent curve progression in those who are most at risk (curves of 25 to 45 degrees in patients with Risser grade 0 or 1), be of benefit in all curve patterns, result in an acceptable cosmetic appearance at the end of treatment, and reduce the need for surgery. In other words, nonsurgical treatment must improve the patient’s outcome with respect to the expected natural history. Over the years a great deal of experience has been gained with various forms of nonsurgical treatment, some of which orthopaedists consider effective (e.g., bracing) and others that have shown no beneficial effect (e.g., electrical stimulation). In Europe, the use of physical therapy exercises and biofeedback to enhance the effectiveness of bracing (Schroth technique and others) is common. Although its use in North America has not yet become established, this treatment method is being studied in various centers.
Orthotic (Brace) Treatment
Historically, Pare is credited with being the first to use metal braces, in the form of armor, to treat patients with scoliosis. Since then, various types of braces and casts have been advocated, such as the suspensory plaster cast of Sayre and the hinge or turnbuckle cast of Hibbs and Risser. In 1946 the Milwaukee brace was developed to replace postoperative plaster immobilization. Later, the brace was used as a nonoperative method of treatment when passive, active, and distraction forces were thought to be necessary to prevent curve progression. Subsequent studies have shown that the corrective forces of a brace are passive and that the predominant corrective component is transverse loading of the spine through the use of corrective pads. In the 1960s, thermoplastics were introduced into orthosis manufacturing, which led to the thoracolumbosacral orthoses (TLSOs) of today. In recent years, computer-assisted design and computer-assisted manufacturing have been used to fashion spinal orthoses.
Indications for Brace Treatment.
Brace treatment is restricted to immature children in an attempt to prevent curve progression during further skeletal growth. In general, bracing is indicated in growing adolescents (Risser grade 0, 1, or 2) who on initial evaluation have curves in the range of 30 to 45 degrees or who have documented progression exceeding 5 degrees in curves that initially measured 20 to 30 degrees. Those who are Risser grade 0 should be considered candidates for bracing when their curves reach 25 degrees. Patients should consider their existing deformities cosmetically acceptable and must be willing to wear the brace the prescribed amount of time. Low-profile braces (TLSOs) are the most commonly used orthoses today, but their use is restricted to patients whose curve apex is at T7 or below. Fortunately, this is the case with most curve patterns in adolescents with idiopathic scoliosis. In 2005 the Bracing Committee of the SRS made recommendations concerning inclusion criteria for future brace studies involving AIS. Optimal inclusion criteria are age 10 years and older when the orthosis is prescribed, Risser grade 0 to 2, primary curve magnitude of 25 to 40 degrees, no previous treatment, and if female, either premenarchal or less than 1 year postmenarchal.
Contraindications to Brace Treatment.
Brace treatment has several contraindications. First, most studies concur that large curves (>45 degrees) in a growing adolescent cannot be effectively controlled by a brace and that these patients need surgical treatment. Even if progression could be controlled with a brace, the cosmetic appearance associated with these large curves is often unacceptable because of excessive trunk shift and rib prominence. An exception to this general rule involves very immature adolescents with large curves (approximately 50 degrees) who have not yet reached their PHV. These patients may benefit from bracing to delay curve progression until greater maturity is reached; this may avoid the need for additional anterior spinal fusion to prevent the crankshaft phenomenon.
In addition, bracing is not indicated for patients who find wearing an orthosis to be emotionally intolerable, although appropriate psychological counseling may result in eventual acceptance of a brace by an adolescent. Extreme thoracic hypokyphosis precludes the use of an orthosis. In these cases, normal positioning of the pads within the brace could exacerbate the rib deformity. If the hypokyphosis is 20 degrees or less, corrective pads should be lateralized to eliminate any anteriorly directed derotation forces. Finally, skeletally mature adolescents (Risser grade 4 or 5 and, if female, 2 years postmenarchal) should not be treated with braces.
Relative contraindications to bracing include a high thoracic or cervicothoracic curve, which ordinarily does not respond to orthotic treatment, and male sex. A relative lack of effectiveness of bracing in boys has been documented, in part because of extremely poor compliance.
Comparison of Orthoses.
Numerous reports in the literature attest to the effectiveness of brace treatment. * a In most of these studies, bracing was considered effective if the curve remained within 5 to 6 degrees of its original magnitude on completion of treatment. Some of these studies included low-risk patients (Risser grades 3 to 5, curves <20 degrees), patients still undergoing treatment, patients who had previously undergone treatment, and children younger than 10 years. In some studies, patients were eliminated from the study population because of noncompliance. All these factors make comparisons among studies difficult, particularly when one is trying to assess the effectiveness of bracing in patients most at risk (Risser grade 0 or 1, premenarchal girls, 25- to 45-degree curves). Some of the more recent literature has been more consistent in focusing on the population at greatest risk. †a These studies, along with a 1997 meta-analysis of the bracing literature, strongly reinforce the idea that bracing is effective in controlling curve progression. Perhaps the most compelling evidence for this at present comes from a study by Katz and colleagues. In this study, braced patients had compliance objectively verified through the use of a heat sensor in the orthosis. The total number of hours of brace wear correlated with curve progression. This effect was most significant in patients who were at Risser stage 0 or 1 at the beginning of treatment. Curves did not progress in 82% of patients who wore the brace more than 12 hr/day, as opposed to only 31% of those who wore the brace less than 7 hr/day.
References .
†a References .
The perception of brace effectiveness is not universal, however, in that some studies question whether orthotic management provides any benefit in those with scoliosis. ‡a‡a References .
Numerous orthoses are available. Most are named after their place of origin, such as the Milwaukee brace, the Boston brace, the Wilmington brace, the Charleston brace, and the Providence brace. All these braces are effective in preventing curve progression. Before deciding which brace to use, the orthopaedist should be familiar with the advantages and disadvantages of each.Milwaukee Brace.
The Milwaukee brace, introduced by Blount, Schmidt, and Bidwell in 1946, was the original modern design. The device consisted of three main components: a pelvic girdle, a suprastructure, and lateral pads. Over time, some design modifications have been made, and in the current model the pelvic girdle is made of thermoplastic material and is created from a positive mold of the patient’s pelvis. The suprastructure consists of one anterior and two widely separated posterior uprights, plus a cervical ring with a throat mold and occipital piece. In many cases a low-profile, over-the-shoulder structure may be used in place of the more standard neck-ring design. Today, because of the strong emphasis on self-image, use of the Milwaukee brace has decreased greatly, and it has largely been replaced by equally effective but lower-profile braces. Low-profile TLSOs, such as the Boston, Wilmington, and Miami braces, can often be hidden under loose shirts or sweaters, thus providing adolescents a more acceptable alternative.
Boston Brace.
The Boston brace was introduced in 1971 by Hall and associates. Its design consists of a prefabricated, symmetric thoracolumbar-pelvic module with built-in lumbar flexion and areas of relief opposite areas of pressure ( Fig. 12-22 ). Braces are individually constructed by an orthotist by using a blueprint created from the patient’s full-length radiograph. This brace is the most commonly used TLSO today and is effective in controlling curve progression, including larger curves measuring 35 to 45 degrees. §a It has been reported that the Boston brace exerts derotational force on the scoliosis; however, a long-term study found no lasting improvement in derotation of the spine. The brace is effective in treating either single- or double-curve patterns in which the apex of the most cephalic curve is located at T7 or below.
§a References .
Wilmington Brace.
The Wilmington brace was described in 1980. It is custom-made from a positive mold of the patient’s torso in which the scoliosis is maximally corrected in a Risser- or Cotrel-type cast. Indications for the Wilmington brace are the same as those for the Boston brace. This brace has not enjoyed the popularity of the Boston brace, although it continues to be used by several institutions.
Charleston Brace.
Development of the Charleston brace was based on the concept that part-time use may be effective. This brace holds the patient in maximal side-bending correction and is worn only at night for 8 to 10 hours. The side-bending force exerted by the brace does not allow its use in the upright position, thus making wear feasible only when the patient is recumbent. The main appeal of this brace is the limited number of hours of daily wear, all of which are accomplished during sleep.
Despite several studies in which the Charleston brace was found to be as effective as the Milwaukee and Boston braces, some skeptics doubt that such a limited amount of time in a brace can successfully control curve progression. A comparison of the Boston brace and the Charleston brace found that in patients with curves between 36 and 45 degrees, 83% of those treated with a Charleston brace experienced curve progression of more than 5 degrees as compared with 43% of those treated with a Boston brace. The authors concluded that the Charleston brace should be reserved for single lumbar or thoracolumbar curves less than 35 degrees.
Providence Brace.
The Providence brace, like the Charleston brace, is used only at night ( Fig. 12-23 ). Made with computer-assisted design and manufacturing technology, it is reported to be effective. We now use this brace in place of the Charleston brace for the treatment of thoracolumbar or lumbar curves.
Spine-Cor Brace.
This is a dynamic flexible brace. Several studies have reported its effectiveness for AIS. However, a failure rate significantly higher than that of a rigid spinal orthosis has been reported.
Brace Treatment Protocols.
The number of hours per day that the brace needs to be worn remains uncertain. Originally, 20 to 22 hr/day was advocated for the Milwaukee brace in immature adolescents with progressive curves; the same recommendation applied to the lower-profile TLSOs. Understandably, this caused adolescents some emotional distress, and poor compliance with brace wear was common. As a result, the idea of part-time brace use evolved, with the goal of approximately 16 hr/day of bracing. Although several studies have reported that part-time use of orthoses appears to be as effective as full-time wear in controlling curve progression, other reports emphasize that the outcome is better when more hours per day are spent in the brace. This was confirmed by Katz and colleagues in a 2010 study that objectively assessed brace compliance by using heat sensors in the orthosis.
In an effort to form a consensus from the literature on the effectiveness of bracing (including whether part-time bracing controls curve progression as effectively as full-time bracing does), the Prevalence and Natural History Committee of the SRS conducted a meta-analysis on more than 1900 patients from 20 studies. It concluded that bracing (with TLSOs or the Milwaukee brace) is effective in controlling curve progression in individuals with idiopathic scoliosis and that full-time bracing (23 hr/day) is more effective than part-time bracing (8 to 16 hr/day). The latter finding is supported by more recent studies.
When brace treatment is chosen for a patient, certain general guidelines should be followed. Once the brace has been constructed and fitted to the patient by the orthotist, the patient should work up to the prescribed number of hours of wear per day. After 2 to 4 weeks the adolescent should return to the orthopaedist’s office for an initial brace evaluation. At that time, any problems (e.g., intolerable pressure points) will have been identified by the patient and can be addressed by the orthotist. Equally important, an in-brace radiograph should be obtained to verify the amount of curve correction being achieved. With the Boston brace, a minimum of 40% to 50% curve correction should be obtained in the brace. With the Charleston and Providence braces, the amount of in-brace correction should approach 90% for flexible curves and 70% for rigid curves. Regardless of the type of brace used, insufficient in-brace curve correction leads to an unsatisfactory outcome that differs little from the expected natural history. If proper correction cannot be obtained with brace use, orthotic treatment should be discontinued.
During brace management, follow-up visits are scheduled at 4-month intervals for rapidly growing adolescents with large curves. The interval may be extended to 6 months for patients nearing maturity whose curves have shown no recent changes. During these visits a single standing PA thoracolumbar radiograph is obtained with the patient out of the brace. Curve progression, if it has occurred, is readily identifiable, and appropriate adjustments to the treatment program can be made. Some physicians obtain radiographs with the patient wearing the brace to show the brace’s effect on both the curve and spinal balance. However, curve progression may be missed if the patient undergoes imaging while wearing the brace.
With female patients, if the brace has been successful in controlling curve progression, plans can be made to discontinue treatment when the girl is approximately 18 to 24 months postmenarchal and Risser grade 4 and when no further increase in her height has occurred ( Fig. 12-24 ). Rather than tapering use of the brace, we discontinue it completely at that time. In male patients, curves exceeding 25 degrees have a tendency to progress even when Risser grade 4 maturity has been reached. Therefore, in boys, bracing may need to be continued until Risser grade 5 is achieved. Frequently, this does not occur until the later teenage years, which makes compliance with brace wear a challenge.
Electrical Stimulation
Electrical stimulation was used as an alternative to bracing in the early 1980s. Surface muscle stimulators were placed over the muscles on the convex side of the scoliotic curve and were activated for approximately 8 to 10 hours each night. In Canada, electrode stimulators were actually implanted in the paraspinal muscles. Although some preliminary success was reported with transcutaneous stimulation, most studies found that this form of treatment did nothing to favorably alter the natural history of scoliosis. Today, electrical stimulation is no longer considered a useful method in the management of idiopathic scoliosis.
Physical Therapy and Biofeedback
Consistent asymmetry in torso rotation strength has been documented in patients with AIS when parameters such as specific strength testing and myoelectric activity recording are used. Although muscle conditioning is beneficial to a patient’s overall well-being, only modest evidence supports the concept that exercises or physical therapy programs are helpful in controlling or improving scoliosis. Likewise, spinal manipulations and biofeedback have yet to be shown to alter the natural history of scoliosis.
Surgical Treatment
The primary goals of surgical intervention in the treatment of scoliosis are to reduce the magnitude of the deformity, to obtain fusion for prevention of future curve progression, and to do so safely. Operative treatment should result in a well-balanced spine in which the patient’s head, shoulders, and trunk are centered over the pelvis. Ideally, when this is accomplished with newer surgical implants, a significant amount of curve correction can be achieved in all three planes.
Improved correction of deformity has resulted from a combination of improved instrumentation systems that impart more corrective force on scoliotic spines and advances in surgical techniques that allow greater mobilization and derotation of the spine. All pedicle screw–systems provide greater opportunity to gain control of each vertebra, which allows greater correction of each segment either individually or through “en bloc” techniques. Today, with the introduction of pedicle screw fixation at nearly every level of the spinal segment requiring instrumentation, curve correction is even greater than that achieved with hook–rod segmental fixation systems. These new pedicle screw implant systems and the surgical techniques are complex and require a significant amount of training. The established segmental fixation systems—including Cotrel-Dubousset (CD), Texas Scottish Rite Hospital (TSRH), and Isola instrumentation—became popular in the mid-1980s and, with refinements that allow the use of more pedicle screw fixation points, remain so today. Each instrumentation system allows the surgeon to achieve increased curve correction, improved sagittal contouring, brace-free postoperative mobilization, and improved MRI compatibility (with the availability of titanium components).
The expansion of technology since the early 1990s has resulted in the availability of numerous other systems, with many manufacturers developing very similar concepts centered around multiple fixation points with pedicle screws that provide the opportunity to correct all three planes with especial focus on the use of axial plane correction strategies while attempting to maintain the sagittal plane, particularly thoracic kyphosis. Familiarity with one or more of these systems, including their limitations, is helpful when planning surgical treatment of the various curve patterns seen with idiopathic scoliosis.
Indications for Surgery
Although various considerations enter into surgical decision making, curve magnitude remains the primary factor. Curves less than 30 degrees at skeletal maturity are unlikely to progress, regardless of the curve pattern, and do not require surgery. Thoracic curves and double major curves that exceed 50 degrees at skeletal maturity have a significant probability of worsening over time and nearly always warrant operative intervention. Thoracolumbar and lumbar curves of less magnitude, when associated with marked apical rotation or translational shift, also have a propensity to worsen over time in mature patients. In these cases, surgery should be considered when the curves exceed 40 to 45 degrees.
In addition to curve magnitude, the patient’s appearance (as perceived by the patient, the family, and the surgeon) plays a role in surgical decision making. Patients and their families are usually most concerned about this aspect of the deformity. The patient’s spinal balance may be decompensated, with the thorax shifted noticeably away from the midline; rib prominence may be severe because of excessive rotation; and the shoulders and hips may appear uneven.
It is uncommon for back pain alone to serve as an indication for scoliosis surgery. Historically, 30% of patients with scoliosis describe associated back pain; however, more recently studies suggest that up to 75% of patients have back pain that may be improved with surgery. Less than 10% of these symptomatic patients have a definite cause found for the discomfort. In general, the treating orthopaedist should not assume that the patient’s discomfort will be remedied by spinal fusion.
Curve patterns in males appear to be more rigid than those in females with idiopathic scoliosis. When planning surgery in males, less curve correction should be expected, but short- and long-term functional outcomes and complication rates can be expected to be similar to those in female patients.
Preoperative Planning
Preoperative planning must take into consideration the patient’s curve pattern and spinal balance, curve flexibility, neurologic status, rib deformities, physical maturity and future growth potential, and other surgery-related needs (e.g., transfusion requirements, bone grafting, spinal cord monitoring, postoperative pain management). The surgeon’s selection of instrumentation depends on personal experience, availability of the various systems, and the choice of anterior or posterior instrumentation.
Curve Patterns
King Classification System.
In 1983, King and colleagues introduced a radiographic classification system for AIS in which five different thoracic curve types were described ( Fig. 12-25 ). This system provided useful recommendations for surgical intervention with Harrington implants; however, with the evolution of multiple hook or screw implant systems in the late 1980s, which allowed increased three-dimensional curve correction, several shortcomings in the descriptions and recommendations of the King system became evident. The one King curve pattern that is not better described in other systems is type IV, a thoracic curve in which the L4 vertebra is tilted into the curve. This curve pattern requires instrumentation to a more distal segment into the lumbar curve, generally the vertebra last touched by the center sacral vertical line (CSVL).
Lenke Classification System.
Lenke and co-workers developed a new classification system for AIS in 1997 that recognized the important contributions already made by King and colleagues. Specific goals for the new classification system were to allow more acceptable comparisons among the various types of operative treatment available, to support a treatment-based approach by determining which of a patient’s scoliotic curves should be included in the instrumentation and fusion, to encourage three-dimensional analysis of scoliosis, and to achieve greater intraobserver and interobserver reliability. The Lenke classification system is dependent on curve measurements in both the frontal and sagittal planes. It is comprehensive (42 different curve patterns can be derived) yet is fairly easy for surgeons to learn quickly. The three main variables requiring evaluation are curve type ( Table 12-3 ), lumbar spine modifiers, and thoracic sagittal modifiers ( Fig. 12-26 ).
Curve Type | Description | Characteristic Curve Patterns | Structural Region | ||
---|---|---|---|---|---|
Proximal Thoracic | Main Thoracic | Thoracolumbar or Lumbar | |||
1 | Main thoracic | Nonstructural | Structural (major) Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T10 and L2 | Nonstructural | Main thoracic |
2 | Double thoracic | Structural Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T2 and T5 | Structural (major) Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T10 and L2 | Nonstructural | Proximal thoracic, main thoracic |
3 | Double major | Nonstructural | Structural (major) Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T10 and L2 | Structural Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T10 and L2 | Main thoracic, thoracolumbar, or lumbar |
4 | Triple major | Structural Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T2 and T5 | Structural (major) * Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T10 and L2 | Structural (major) * Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T10 and L2 | Proximal thoracic, main thoracic, thoracolumbar, or lumbar |
5 | Thoracolumbar or lumbar | Nonstructural | Nonstructural | Structural (major) Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T10 and L2 | Thoracolumbar or lumbar |
6 | Thoracolumbar or lumbar, main thoracic | Nonstructural | Structural Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T10 and L2 | Structural (major) Cobb angle: ≥25° on side-bending radiographs Kyphosis: +20° between T10 and L2 | Thoracolumbar or lumbar, main thoracic |
* Either the main thoracic curve or the thoracolumbar or lumbar curve can be the major curve.
Using standard-size projection-type slides of photographs of premeasured radiographs, the developers of the Lenke classification system found both interobserver and intraobserver reliability to be excellent. However, in clinical practice, physicians vary significantly both in their selection of end vertebrae and in their angular measurements of curves in individuals with idiopathic scoliosis. Because scoliosis surgeons rarely have access to premeasured radiographs when they evaluate patients and formulate surgical plans, the reliability of the Lenke classification system is less impressive when tested with unmarked radiographs. When all three of the main variables are combined, its overall reliability is fair. When each variable is reviewed separately, its reliability improves. Despite this limitation, the Lenke classification system offers a more comprehensive preoperative radiographic evaluation of patients with AIS than was available with previous systems and appears to correlate with surgical treatment of structural regions of the spine.
The first parameter to identify in the Lenke classification system is curve type (see Fig. 12-26 ), which is determined by first identifying the largest curve. The other curves are then deemed structural by magnitudes that are greater than 25 degrees on the supine best-bend radiograph or if junctional kyphosis (measured between T2 and T5 for the proximal and main thoracic curves and between T10 and L2 for the main thoracic and thoracolumbar/lumbar curves) is greater than 20 degrees. Once the type of curve is identified, the second main variable, the lumbar spine modifier, is assessed ( Fig. 12-27 ). The CSVL (vertical line drawn upward from the center of the sacrum) is drawn, and its position relative to the concave pedicle of the apical lumbar vertebra determines the lumbar modifier. The final main variable, the thoracic sagittal modifier (T5-12), is then assessed to gain a better understanding of the three-dimensional deformity. The sagittal modifier is hypokyphotic (<10 degrees), normal (10 to 40 degrees), or hyperkyphotic (>40 degrees). In addition to radiographic characterization, the clinical appearance is critical to determine curves requiring surgical treatment. A comparison of right and left shoulder height is important to determine when inclusion of the proximal thoracic curve is necessary, waistline asymmetry helps determine when inclusion of the lumbar curve is necessary, and the axial plane rotational deformities are critical when deciding which curves are structural.
Construct Selection.
Three major trends with respect to surgical treatment of scoliosis have occurred: first, the posterior approach has had greater use than the anterior approach for nearly all curves, including Lenke 5 and 6 thoracolumbar and lumbar curves; second, pedicle screws have been used in all regions, including the thoracic spine, in place of hooks and hybrids ; and third, because of greater use of posterior releases and more powerful posterior implants, use of an anterior release has declined.
Proponents of screws suggest they provide stronger fixation than hooks do, are safe when inserted by experienced surgeons, avoid placing metal within the canal, provide a stronger corrective force for three-dimensional curve correction, may obviate the need for anterior fusion to avoid the crankshaft phenomenon because of the strength of the construct, and may avoid the need to perform thoracoplasty. Those who question the routine use of pedicle screw placement for thoracic scoliosis cite the significant increase in cost with no documentation of measurable improvement in outcome studies, early reports of loss of thoracic kyphosis with screws, and the potential risk for injury to the spinal cord by surgeons inexperienced in the technique.
Fusion selection recommendations are based on correct identification of curve patterns and the clinical deformity in each patient. Some generalities on selection of the level can be taken into consideration when developing plans for fusion. The upper instrumented vertebra (UIV) is usually the proximal end vertebra for thoracic and thoracolumbar/lumbar curves and, most often, T2 when fusing a structural proximal thoracic curve. The lowest instrumented vertebra (LIV) for lumbar/thoracolumbar curves is usually the distal end vertebra when performing posterior instrumentation. When fusing vertebrae for idiopathic scoliosis via the anterior approach, they should generally be the UIV and LIV. In addition to these guidelines, each curve pattern has some specific rules to follow.
Lenke type 1A and 1B curves have single thoracic structural curves with nonstructural lumbar curves that do not cross the midline (CSVL). Clinically, trunk imbalance is more pronounced to the right in these patients than in those with double-curve patterns and responds nicely to surgical treatment. For posterior constructs the UIV is generally the proximal end vertebra or one proximal to it when it is significantly off midline. The rules for selection of the LIV are more varied, with three general rules: first, fusion to one or two levels proximal to the stable vertebra ; second, fusion to the vertebra that last touches the CSVL; and third, as reported by Suk and colleagues, rules based on the relative position of the neutral vertebra (the most proximal vertebra that has neutral axial plane rotation based on the position of the pedicles) and the position of the distal end vertebra. Preoperative planning for posterior instrumentation using only screws is shown in Figure 12-28 . Frequently, the implant is a hybrid, with screws used as an anchor at the bottom of the constructs and some combination of hooks, sublaminar wires, or tape and screws used above. For the King-Moe type IV curve (a Lenke 1A curve when L4 is tilted into the curve), the end vertebra may be chosen as the LIV, especially when the stable vertebra is L4, and posterior instrumentation can be stopped at L2 (two levels cephalic to the stable vertebra). Anterior instrumentation has shown that even more inferior motion segments can be preserved because the implant extends down to only the lowest vertebra included in the measured curve.
Patients with Lenke type 1C curves have single thoracic structural curves with nonstructural lumbar curves, and the apical lumbar vertebra completely crosses the midline (i.e., is not in contact with the CSVL). In the late 1980s and 1990, use of “derotation” instrumentation for selective thoracic fusions often led to spinal imbalance manifested as a shift in the patient’s trunk or head (or both) to the left of midline—so-called decompensation. ‖a
‖a References .
Despite many purported reasons, including improper choice of fusion levels, overcorrection of the thoracic curve, incorrect identification of curve patterns, lumbar curve stiffness, and progression, the cause of decompensation was most likely multifactorial, and improvement occurred over time as the uninstrumented lumbar curve adapted ( Fig. 12-29 ).When using this method of instrumentation for selective fusions in individuals with Lenke type 1C curves, the surgeon should keep in mind some principles that can minimize the chance of postoperative imbalance. First, the instrumentation should not extend beyond the stable vertebra; second, if using the rod rotation maneuver, incomplete rotation should occur to avoid overcorrection or excessive straightening of the thoracic curve; and third, other correction strategies such as apical translation with fixed proximal and distal anchors or a cantilever can be performed. Preoperative planning involving pedicle screw anchors for selective thoracic instrumentation and fusion of a Lenke type 1C curve is shown in Figure 12-30 .
Failure to properly distinguish Lenke type 1C or King type II curves from true double major curves (Lenke type 3C) may be responsible for some cases of postoperative imbalance following selective thoracic fusions. Useful guidelines have been developed to help differentiate these curve patterns. Relative ratios between the thoracic and lumbar curves with regard to their size, rotation, and deviation from midline can be assessed preoperatively on a standing radiograph. If thoracic curve parameter–lumbar curve parameter ratios are less than 1.0, both curves will require fusion. If the ratios are greater than 1.2 for curve size and deviation and greater than 1.0 for rotation, selective thoracic fusion can be performed safely. With true double major curves, both curves must be included in the posterior fusion to achieve a balanced spine with segmental fixation systems.
Selective anterior fusion on the convexity of the thoracic component in Lenke type 1C curves (using screws and either a threaded or a smooth rod) has been advocated. Reported advantages over posterior instrumentation include improved balance, correction of a hypokyphotic thoracic spine, and preservation of more inferior motion segments.
Preliminary studies have reported breakage of the threaded rod in 31% of patients, but this complication can be remedied by the use of a larger rod and thorough diskectomies and bone grafting. Thoracoscopically placed anterior instrumentation has become used less often but overall demonstrates comparable results to posterior fusion with inclusion of fewer fusion levels.
Patients with Lenke type 2 curves have double thoracic structural curves with nonstructural lumbar curves. The first thoracic vertebra is tilted into the upper curve, with junctional kyphosis between the proximal and main thoracic curves. The patient’s shoulder on the side of the convexity of the upper curve is nearly always elevated. The upper curve and the shoulder elevation may worsen if only the lower main thoracic component is instrumented. Therefore, most Lenke type 2 curves require posterior instrumentation of both thoracic curves. Selective main thoracic fusion using anterior instrumentation has been successful when the proximal thoracic curve is sufficiently flexible. The posterior instrumentation should extend up to the second thoracic vertebra if T1 is tilted into the upper curve and the shoulder is elevated on the convex side of the upper curve, if the upper curve is greater than 30 degrees with limited flexibility, or if the transitional vertebra between the curves is located at T6 or below. Lenke type 3 curves represent double major structural curve patterns in which both the thoracic and lumbar components require instrumentation. Preoperative planning for instrumentation of double major curves is shown in Figure 12-31 , with LIV selection principally to the distal end vertebra. Curve characteristics that allow stopping short of the end vertebra include thoracolumbar or lumbar curves smaller than 55 degrees, flexible curves, and apex of the thoracolumbar or lumbar curves that is two or more levels proximal to the intended LIV. Lenke type 4 curves are triple major curve patterns in which both thoracic curves, in addition to the lumbar curve, are structural. All three curves require posterior instrumentation.
In Lenke type 5 curves, only the thoracolumbar or lumbar curve is structural. The anterior approach has traditionally been used for these curves with excellent three-dimensional correction. However, the use of posterior instrumentation with aggressive release techniques provides a viable alternative to the anterior approach. Proponents of the posterior approach suggest that the same number of levels are fused (proximal end vertebra to distal end vertebra), coronal plane correction is the same, surgical time is shorter, no chest tube is necessary, and intensive care unit and hospital stay is shorter. Advocates of the anterior approach suggest that not all curve patterns will allow similar fusion levels and the axial plane correction is less because the disk and soft tissues are still intact with the posterior approach. Anterior spine surgery for these curves has always had challenges in achieving fusion; however, constructs using one or two solid rods were developed and resulted in a decreased incidence of pseudarthrosis, better maintenance of restored sagittal lordosis, and elimination of postoperative brace immobilization. Preoperative planning for anterior or posterior instrumentation of a thoracolumbar curve is shown in Figure 12-32 .
Lenke type 6 curves represent double curve patterns, with the primary thoracolumbar or lumbar curve accompanied by a smaller but structural main thoracic curve. Selective fusion of the thoracolumbar or lumbar curve will not yield similar results as selective thoracic fusion because the thoracic curve will not respond to maintain balance. Posterior fusion and instrumentation are necessary in this circumstance.
Preoperative Curve Flexibility.
Preoperative curve flexibility can be assessed by a number of different techniques, including supine best-effort, side-bending radiographs; the fulcrum bend test; or a supine resting radiograph. We use supine best-bend radiographs because they realistically reflect the amount of curve correction that can be achieved posteriorly and determine flexibility of the spine with the newer generation of instrumentation systems. These radiographs are used to determine the flexibility of the remaining curves to provide a Lenke classification. They can also be used to help determine or confirm the LIV for isolated thoracolumbar or lumbar curves. For left thoracolumbar or lumbar curves, a right-bend film should demonstrate that the proposed LIV is centered over the sacrum and, on the left-bend film, should demonstrate “reversal” of the disk above the intended LIV. Large curves (>75 degrees) are best analyzed with traction films because the mechanics of the bend films is minimized for these large and stiff curves. The push-prone radiograph was shown to be the best preoperative indicator of flexibility for predicting the final lumbar curve measurement in patients undergoing selective thoracic fusion for Lenke type 1B and 1C curves. Significant study using the fulcrum bend test has demonstrated that for the thoracic curve, this test best predicts the status of the thoracic curve following posterior spinal fusion and instrumentation.
Neurologic Status.
If a subtle neurologic abnormality (e.g., asymmetric abdominal reflexes) is detected in an otherwise normal individual, MRI of the entire spinal canal should be considered to rule out syrinx, cord tethering, or diastematomyelia. Preoperative MRI should also be performed in patients with left thoracic curves and in those in whom the typical apical lordotic sagittal deformity is absent because of the association with intracanal abnormalities. The MRI study can be ordered when surgery is scheduled. Studies have demonstrated that excessive rotation or kyphosis in the thoracic spine is an indication to perform MRI.
Rib Deformities.
The rotational deformity inherent in AIS is often seen most by families and is generally mild to moderate with rare occurrence of the razorback deformity (it is more common in nonidiopathic conditions such as neurofibromatosis). Suggested indications for thoracoplasty include a preoperative rib prominence exceeding 10 degrees (measured from a tangential radiograph with the patient bent forward 90 degrees), preoperative curves greater than 60 degrees, and flexibility less than 20%. Surgeon preference and experience play a large role in determining the indications for thoracoplasty; however, the general trend has been less use of this procedure because of improved methods for direct vertebral rotation (DVR). Nonetheless, Professor Suk, the developer of the DVR technique, continues to perform thoracoplasty in all patients with AIS. In addition to improving the patient’s cosmetic appearance, partial resection of three to five apical ribs provides bone graft in amounts sufficient to obviate an iliac crest graft. A percutaneous thoracoplasty technique has resulted in excellent correction but some loss of coronal plane correction. Because some studies report a decline in pulmonary function following thoracoplasty, this technique is contraindicated in patients with compromised preoperative pulmonary or cardiac status. More recent studies have demonstrated an early decline in pulmonary function test results without long-lasting effects when compared with a cohort that did not undergo thoracoplasty.
Future Growth Potential.
Correction of scoliosis by posterior spinal instrumentation and fusion is usually maintained over time and is not adversely affected by any remaining anterior spinal growth; however, the crankshaft phenomenon (resumption of the curve secondary to anterior growth in patients with posterior fusion) can still occur. Dubousset coined the term when he observed that the entire spine and trunk gradually rotated and deformed as the anterior portion of the spine continued to grow and twist around the axis of the fusion mass (in a manner similar to an automobile crankshaft) ( Fig. 12-33 ). Methods to prevent this phenomenon include careful assessment of the growth remaining, the use of anterior fusion when appropriate, and greater use of and correction with pedicle screw fixation. Although its severity is difficult to quantify, the crankshaft phenomenon can best be appreciated by examining serial clinical photographs that demonstrate progressive changes in rib deformities, narrowing of the chest, and imbalance in the thoracic and lumbar spine. Radiographs can also demonstrate progressive changes over time, such as alterations in curve size, rotation, and rib–vertebral angle difference; translation of the apical vertebra toward the chest wall on the convexity; and changes in the vertical inclination of the instrumentation. Radiographic changes of more than 10 degrees in curve size, apical vertebral rotation, and the rib–vertebral angle difference are all thought to reflect progression of the deformity secondary to the crankshaft phenomenon. However, during the first 6 to 12 months after surgery, it is important to not automatically assume that changes in radiographic measurements are a result of the crankshaft phenomenon; these changes are often due to stress relaxation of the spine, gradual maturation of the fusion mass, and realignment of the curve.
For female adolescents in need of surgery who have not yet reached their PHV, who are premenarchal, and whose triradiate cartilage remains open, strong consideration should be given to combining anterior and posterior fusion to prevent the crankshaft phenomenon. For anterior spinal fusion, a conventional open thoracotomy approach has been compared with the newer, less invasive video-assisted thoracoscopic surgery (VATS). Advantages of VATS include muscle sparing, improved cosmetic results (less scarring), greater access to the entire length of the thoracic spine, and less effect on pulmonary function than with open thoracotomy. Instruments are used through multiple intercostal portals to resect disk material, perform anterior release, and insert bone graft. However, surgeons require extensive training in the VATS technique.
Some reports suggest that stiff posterior constructs, particularly when screws are used at nearly every level in the segment fused, may be strong enough to prevent the crankshaft phenomenon in immature patients, thus avoiding the need for anterior fusion. More research is needed to prove the effectiveness of this approach.
Transfusion Requirements.
Several procedures are available to reduce the need for homologous blood transfusions in patients undergoing posterior spinal instrumentation for scoliosis, including controlled hypotensive anesthesia, autologous blood predonation of 1 or 2 units, acute normovolemic hemodilution, intraoperative and postoperative salvage of lost blood, intraoperative use of antifibrinolytics, and transfusion decisions based on clinical judgment rather than on a predetermined hemoglobin value.
Various combinations of these methods have been shown to significantly reduce exposure of patients to homologous blood products during scoliosis surgery. ¶a The combination of predonated autologous blood, hypotensive anesthesia, and intraoperative salvage of lost blood is probably the one used most frequently for healthy individuals with idiopathic scoliosis. Intraoperative salvage of lost blood, the most expensive of the available techniques, is most effective when blood loss is expected to exceed 1000 mL. Acute normovolemic hemodilution appears to be a satisfactory alternative to the use of predonated autologous blood.
¶a References .
The antifibrinolytic agent ε-aminocaproic acid (Amicar) is reportedly a safe, effective, and inexpensive method of reducing perioperative blood loss in patients with scoliosis. We generally prefer tranexamic acid (TXA), which has also been shown to reduce intraoperative and postoperative blood loss during posterior spinal fusion and instrumentation for AIS in a matched cohort. The response to TXA depends on the dose administered, with a higher loading dose of 20 mg/kg followed by a 10-mg/kg/hr infusion appearing to have a greater effect.Bone Grafting.
The primary goal of scoliosis surgery is to achieve a solid arthrodesis, which is enhanced by meticulous cleaning of soft tissue from the spine, facetectomies, decortication, and adequate bone grafting. Although autogenous iliac crest bone grafting has previously been the standard, significant postoperative pain at the donor site persists and remains the greatest problem with the use of autogenous grafting. Because of these postoperative symptoms, alternative bone graft substitutes have been sought. Numerous studies of successful fusions using allografts of frozen, bank-stored bone as a substitute for autogenous bone have been reported without an increase in pseudarthrosis rates.
To minimize the risk of transmitting human immunodeficiency virus, hepatitis virus, and any other potential viral pathogens, the donor blood and tissue are tested at the site of recovery, and testing is usually continued throughout the harvesting process. Freeze-dried cancellous bone is usually exposed to low-dose gamma radiation to sterilize all nonsystemic bacterial and fungal contaminants. Bone morphogenetic protein has become popular for use in single-level lumbar spinal fusions but has yet to become cost-effective for routine use in multisegment scoliosis fusions. The authors’ preferred technique is to perform thorough stripping of the spine, followed by aggressive facetectomies, decortications of the spine, and the use of allograft bone, without any pseudarthrosis in individuals with AIS being documented in the past 8 years.
Spinal Cord Monitoring.
Spinal cord monitoring using both spinal somatosensory evoked potentials (SSEPs) and motor evoked potentials (MEPs) is the standard of care during scoliosis surgery and is critical to the safety of any spine deformity surgery. SSEPs record the sensory function of the spinal cord and provide continuous monitoring throughout the procedure. #a This test may, however, be adversely affected by changes in anesthetic level and perfusion, and critical changes tend to lag behind MEPs. With impending neurologic deficit, MEPs are used to monitor the anterior spinal cord motor tracts and are ideally performed by applying a stimulus to the motor cortex of the brain (transcranial MEPs [tcMEPs]). This provides direct stimulation of the motor cortex, which then travels through the anterior column tracts with responses noted in the upper and lower extremity musculature ( Fig. 12-34 ). When MEPs are used in conjunction with SSEPs, the chance of unrecognized injury to the spinal cord is minimized. A large multicenter study of 1121 patients demonstrated that 38 (3.4%) had a critical change on monitoring when tcMEPs and SSEPs were used. Hypotension was responsible for nine changes (corrected by elevating blood pressure), whereas three were due to segmental vessel ligation. The tcMEP/SSEP combination did not miss any patient with a transient motor or sensory deficit. Similar studies have demonstrated excellent results with combined multimodal intraoperative neuromonitoring during AIS surgery. Although total intravenous anesthesia with propofol is necessary to obtain good tcMEP data, other techniques to assist in obtaining good monitoring data have been evaluated. The current increase in the use of thoracic pedicle screws for fixation points has led some surgeons to use triggered electromyographic (EMG) monitoring; however, this has been relatively unreliable in the thoracic region without clearly identified thresholds to indicate when medial screw penetration is seen.
#a References .
The wake-up test, a gross evaluation of motor function, is no longer used routinely if spinal cord monitoring is available and results are normal throughout surgery. The wake-up test can be performed if changes in SSEPs or MEPs are noted during correction of the spine because spinal cord injury may exist even when monitored variables return to baseline. The authors generally do not perform this test during AIS surgery because first, a “normal” wake-up test does not provide a detailed examination and strength testing is not possible and, second, even if normal, tcMEP/SSEP monitoring can indicate some stress or subclinical deficit in the spinal cord that may become a permanent deficit with continued surgery. For this test the anesthesiologist allows the patient to regain partial consciousness and motor function during the surgical procedure. Intraoperative neuromonitoring is especially useful when intraoperative traction is used because critical changes develop in a third of patients during surgery and are related to having a thoracic curve, a larger Cobb angle, and a rigid curve. A stepwise response to these changes, including removal of the traction, resulted in overall good results, and the presence of MEP recordings at the completion of the surgery was associated with normal neurologic function.Postoperative Pain Management.
Patient-controlled analgesia (PCA) and epidural analgesia are the two methods used regularly for the management of postoperative pain. PCA provides safe and effective analgesia in children as young as 5 years. It allows the patient to self-administer small, preprogrammed doses of opioids via a pump connected to the patient’s intravenous tubing. This enables the patient to titrate an opioid blood level in direct response to the changing intensity of pain. The built-in safety mechanism of PCA systems prevents oversedation. In addition, PCA devices can deliver a continuous infusion so that therapeutic levels of analgesia are maintained during sleep.
The use of epidural analgesia for scoliosis surgery has become increasingly popular because it provides excellent pain relief; however, meticulous attention to detail is required. * b At the end of the surgical procedure but before closure, the surgeon inserts an epidural catheter. The catheter is tunneled lateral to the incision and is usually left in place for 48 to 72 hours. Low-dose opioids are infused to provide effective analgesia, usually under the direction of pain management teams experienced in this technique. Close monitoring of the patient’s respiratory status and the use of pulse oximetry are necessary for 24 hours after the infusion has been discontinued. Postoperative pulmonary toileting is optimized with this technique.
References .
Ketorolac, an injectable nonsteroidal antiinflammatory drug, is effective for the short-term management of moderate to severe postoperative pain. It is often used in conjunction with opioids because the combination provides more effective analgesia than either drug alone does. Although its use has been associated with pseudarthrosis after adult low back surgery, this problem has not been demonstrated in large series of patients with AIS undergoing surgical correction.Antibiotic Prophylaxis for Dental Procedures.
Antibiotic prophylaxis for dental procedures in patients with spinal instrumentation is a controversial issue. Currently, no scientific evidence supports the position that antibiotics should be given during routine dental care. Streptococcus viridans , the predominant bacterium in normal human oral flora and the most common organism isolated from blood after dental procedures, has not been reported in delayed deep wound infections following spinal instrumentation. In those in whom early postoperative wound infections develop, Staphylococcus aureus is the predominant organism. Yet S. aureus accounts for only 0.005% of the normal oral flora and is rarely isolated after dental procedures.
Guidelines similar to those provided in the advisory statement issued by the AAOS regarding antibiotic prophylaxis for dental patients with total joint replacement should be used for those who have undergone spinal instrumentation. If antibiotic prophylaxis is given, the following regimen is recommended: patients who are not allergic to penicillin can be treated with cephalexin, cephradine, or amoxicillin, 2 g orally 1 hour before the dental procedure; patients who are allergic to penicillin should receive clindamycin, 600 mg orally 1 hour before the dental procedure.
Posterior Spinal Instrumentation
Exposure of the spine for posterior instrumentation must be meticulous and thorough, regardless of the implant system selected (see Plate 12-1 on page 292 ).
Harrington Instrumentation.
Harrington developed his technique in the late 1950s and first reported it in 1962. In this system, hooks apply distraction forces to the concave side of the spinal curve via a ratchet mechanism. Compression force is applied to the convex side of the thoracic curve at the base of the transverse processes, with the amount of force adjusted by tightening nuts on a threaded rod.
Long-term follow-up studies have reported that approximately 30% to 40% of curve correction is maintained through the years with Harrington instrumentation. †b However, minimal, three-dimensional correction of the spine was achieved because distraction forces flattened the spine and the implants provided insufficient stability to allow brace-free postoperative mobilization. The technique is detailed in the second edition of Tachdjian’s Pediatric Orthopaedics .
†b References .
Multiple-Hook Segmental Instrumentation.
The CD instrumentation system was developed in France by Cotrel and Dubousset and was introduced in the United States in the mid-1980s ( Fig. 12-35 ). The system revolutionized posterior instrumentation for idiopathic scoliosis by enhancing the surgeon’s ability to improve the three-dimensional orientation of the spine. This was accomplished through the “derotation maneuver” popularized by Dubousset, whereby the contoured rod is secured to the spine with various hooks and rotated 90 degrees to bring the concave spine posterior and medial for correction. This maneuver, which continues to be used today, improves the sagittal contour, achieves significant curve correction, and improves the rotation or translation of the spine. The second rod increases the construct’s strength and torsional stability, particularly when rigidly united to the first rod via a rod-connecting device.
Numerous reports have documenteded significant improvement in the correction of idiopathic scoliosis with CD instrumentation. Rib deformities were reduced, curve correction in the range of 48% to 69% was achieved and maintained, and nearly normal sagittal alignment was restored. ‡b The ability to preserve lumbar lordosis in curves requiring long fusion to L3 or L4 avoided the long-term “flat back” problems that occurred with Harrington distraction instrumentation.
‡b References .
The TSRH instrumentation system was introduced in 1988 and, like the CD system, uses multiple hooks and screws to attach smooth, precontoured rods to the spine. Once the system is assembled, selective compression, distraction, and rotation maneuvers can be performed to correct the spinal deformity. These maneuvers follow the principles introduced by Cotrel and Dubousset and have resulted in outcomes similar to those with CD instrumentation. The technique of a multiple-hook system is illustrated in Plate 12-2 on page 294 on the accompanying website.Pedicle Screws.
The use of pedicle screws has dramatically changed the operative treatment of all spinal deformity, including AIS. The use of pedicle screw fixation for correction of deformity was first described in the lumbar spine in the mid-1990s. These studies demonstrated that pedicle screws provide greater ability to obtain and maintain coronal plane correction of the thoracolumbar or lumbar curves in individuals with double major idiopathic scoliosis. When compared with hooks, initial correction was 72% versus 60% with hooks, and less loss of correction occurred at follow-up (5% versus 13%). Significant improvement in LIV tilt (82% versus 50%) and translation (50% versus 23%) was seen when compared with hooks.
Pedicle screws placed in the lumbar spine for pediatric spinal deformity have a very good track record, with few complications. The improved correction of deformity and maintenance of the correction achieved with lumbar pedicle screws led to their use in the thoracic spine ( Fig. 12-36 ). Suk and coauthors first reported the routine use of pedicle screws in the thoracic spine for spinal deformity surgery in 1995 and achieved improved coronal plane correction in the screw group (72%) versus hooks (55%) and hybrids (66%). The initial reports of their safety demonstrated overall good results, with a pedicle screw breech rate of between 1.5% and 15% and few neurologic complications. The learning curve is steep, and greater surgeon experience leads to improved accuracy. Reports of improved correction of spinal deformity have led to fairly enthusiastic adoption of the technique by surgeons, with overall improved radiographic correction when compared with more traditional hook constructs. §b The improved coronal plane correction achieved with pedicle screw fixation can be attributed to several factors. First, surgeons generally place pedicle screws at more levels than when hooks or other anchors are used; second, the three-column fixation of the spine provides better “grip” of the vertebrae, so correction maneuvers yield greater improvement in the spinal deformity; third, the use of procedures to mobilize the spine has expanded and included greater use of Ponte-style or Smith-Petersen osteotomies, concave and convex rib osteotomies, and for very severe curves, use of the VCR procedure; and finally, with improved fixation, the surgeon can use a number of correction strategies, including the traditional rod rotation maneuver, segmental distraction or compression, and segmental in situ bending. Fixation of each vertebra in the instrumented segment may be an important factor when coronal plane correction with thoracic pedicle screws is analyzed. However, the appropriate screw “density” (the number of pedicles filled with pedicle screws relative to the total number that are available) is not well known, and early studies demonstrated conflicting results, with some showing a positive effect with improved coronal correction and others demonstrating no difference between high and low screw density. In North America, longer-term follow-up has demonstrated maintenance of correction with nearly 70% coronal plane correction.
§b References .
Perhaps the greatest advantage of thoracic pedicle screws is improved axial plane correction. Lee and associates described a DVR maneuver in which the concave and convex screws in the juxtaapical vertebrae were rotated opposite the direction of the rod rotation maneuver. With CT they demonstrated better apical rotation in the group that underwent DVR than in those who did not (42.5% versus 2.4%). The technique of apical derotation can be performed via a number of methods, and all have demonstrated some success in improving the rotational deformity in a variety of AIS curves. Use of this technique may obviate the need for thoracoplasty and the associated detrimental effect on pulmonary function (see Plate 12-3 on page 297 ). However, care in maintaining thoracic kyphosis when performing these DVRs is necessary to avoid creation of lordosis as one pushes anteriorly on the spine. This loss of thoracic kyphosis is accompanied by a loss of lumbar lordosis. Large-diameter rods with an accentuated thoracic kyphosis contour help maintain the thoracic kyphosis with the posterior approach, whereas the anterior approach maintains kyphosis because correction of the coronal plane is achieved with shortening of the anterior column. It is important to preserve the tension band of the soft tissues proximal to the planned instrumented levels to prevent junctional kyphosis, which has been reported with the use of pedicle screws.Surgical treatment of spinal deformity has three main aspects. The first is to grab onto the spine with anchors strategically placed on the spine, typically with pedicle screws today. The second is to mobilize the spine when necessary through a variety of techniques, including intraoperative traction, multiple Ponte-style osteotomies, or even resection procedures. Finally, once the anchor points are placed and the spine is flexible (either inherently so or following mobilization techniques), a variety of surgical techniques can be used to improve the spinal deformity.
Thoracic pedicle screws can be placed in several ways, but generally two main methods are used: freehand, in which the screws are placed without the use of fluoroscopic guidance, and image guided, in which the screws are inserted under the guidance of some radiographic imaging modality.
Freehand Technique.
The freehand technique relies on a thorough understanding of pedicle anatomy in the thoracic spine, including the anatomic landmarks for the starting point and the trajectory and general guidelines with regard to the width, height, and depth of the pedicles in the various regions of the thoracic spine. ‖b In addition, an understanding of the surrounding anatomic structures laterally and medially is important to avoid injury. In general, the width of the thoracic pedicle is smaller in the proximal part of the thoracic spine, on the concavity of the upper and main thoracic curves, and with greater curve magnitude. The spinal cord is positioned adjacent to the concave pedicles with less than 1 mm of epidural space, as compared with 3 to 5 mm on the convex side. At the apex of a right thoracic scoliosis, the aorta is positioned more lateral and posterior to the vertebral body than in a normal, straight spine. The combination of narrow pedicles, dural sac proximity medially, and aorta proximity laterally makes safe screw placement challenging on the concavity of these thoracic curves. The most challenging pedicles are those in the proximal part of the thoracic spine, especially on the concave aspect. The freehand technique entails identifying the starting point for screw insertion, decorticating that level with a burr, entering the cancellous channel with a pedicle finder, and traveling down the pedicle via manual pressure. The channel is then probed to ensure that all five walls (anterior, medial, lateral, superior, and inferior) of the pedicle are intact, followed by tapping the pedicle, reprobing to ensure maintenance of the pedicle walls, and placing the screw. Fluoroscopy or plain radiography is then used to check the position of the screws. Kim and co-workers reported on 3204 screws placed in the thoracic spine for spinal deformity and randomly analyzed 577 screws with CT imaging. They demonstrated that 6.2% of the screws had moderate cortical perforation, including 1.7% with medial wall violation; however, no neurologic or vascular complications occurred. Pedicle screws have been placed successfully in patients with severe deformities and scoliosis curves greater than 90 degrees, with a thoracic pedicle screw accuracy rate of 96.3% and no neurologic complications.
‖b References .
Image-Guided Techniques.
A variety of image guidance techniques have been described, from plain radiography following guidewire placement to continuous stepwise fluoroscopic evaluation to true surgical navigation using preoperative or intraoperative CT scanning and intraoperative computer navigation. ¶b Suk and colleagues described a method in which anatomic landmarks are used to identify the starting point and guidewires are drilled into the center of the pedicle, which are then visualized on a plain PA radiograph. Based on these radiographic images, adjustments are made in the position of the guidewires before placing the screws. A review of their experience in placing 4604 thoracic pedicle screws in 462 patients demonstrated that 1.5% of the screws were malpositioned in 10.4% of the patients, with only 4 screws (0.09%) placed medially. One patient experienced transient paraparesis, and three had dural tears.
¶b References .
True image guidance systems rely on either a preoperative or an intraoperative CT scan to define the spinal anatomy (initial data acquisition), followed intraoperatively by image-to-patient registration. Surgical navigation is then performed during placement of the pedicle screws. Early results demonstrated similar or improved screw accuracy when compared with more conventional imaging. One study reported that a potentially unsafe screw was 3.8 times less likely to be inserted with navigation and that the chance of a significant medial breach was 7.6 times higher without navigation. Alternatively, CT imaging may be used to confirm accurate screw placement, although the systems currently available provide less detail than a typical CT scanner found in radiology suites does. All these image guidance techniques expose the patient and surgeon to increased radiation, which may have long-term effects.Other Methods of Assessing Screw Placement.
Other methods to determine safe and accurate screw placement include EMG stimulation, which has produced reliable results and clear guidelines when lumbar pedicle screws are within the pedicle. The premise of this technique is that when the electrical current is passed through a completely intraosseous pedicle screw, it will not result in a triggered EMG peripheral response. With greater stimulus intensity, however, even a well-placed screw will trigger a peripheral response, so guidelines have been established based on the level of stimulation required to elicit a response: greater than 8 mA defines a screw completely within the pedicle; between 4 and 8 mA is intermediate, which means that the screw should be removed and the medial wall probed; and less than 4 mA indicates a strong likelihood of a pedicle wall defect. Although good success has been achieved in the lumbar spine, studies involving the thoracic spine have not been able to clearly define threshold values for determining a safe screw. The technique of EMG stimulation for ascertaining thoracic pedicle screw placement is technician dependent and takes some experience to perform. It should serve only as an adjunct to thorough understanding of the pedicle anatomy, meticulous surgical technique, and good imaging.
The improved radiographic correction seen with the use of thoracic pedicle screws has not been directly associated with improved clinical outcomes. Comparisons of thoracic pedicle screws and hook constructs or a hybrid construct (hooks proximally and screws distally) have demonstrated no difference in 2-year postoperative scores and little or no correlation between coronal plane correction in AIS and the SRS outcome instrument.
The advantages of improved radiographic correction when using thoracic pedicle screws for the treatment of spinal deformity must be weighed against the risk and cost of these implants. In most series the incidence of the most feared complications—neurologic injury and major injury to soft tissue structures, including the great vessels—is very low (<1%). #b A study of more than 19,000 patients who underwent surgery for pediatric spinal deformity demonstrated fewer neurologic deficits with pedicle screws (0.7%) than with wires (1.7%). This may be due to the significant experience gained by the initial users of the technique, as well as the relatively easy transition from the lumbar spine to the thoracic spine. Neurologic injuries directly related to the use of thoracic pedicle screws have been reported, however. The final consideration is the cost of the implants, which is higher than that of hook or hybrid constructs because of the higher cost per implant, as well as the increased number of screws used in each case.
#b References .
Posterior Mobilization Techniques.
Removal of the inferior facet at all instrumented levels has been the standard technique to achieve fusion when performing surgery to correct spinal deformity. The opportunity for greater three-dimensional spine correction with the use of pedicle screws has been accentuated by a trend toward performing more aggressive spine mobilization procedures, thereby providing an opportunity to gain greater correction of deformity. Beyond standard facetectomies, the gradual resection of more posterior structures provides greater spine mobility in the following order: complete facetectomies/ligamentum release (Ponte or Smith-Petersen osteotomies), concave or convex rib resections (or both), vertebral body decancellation with wedge resection of the vertebra, pedicle subtraction osteotomy, and finally, complete VCR.
A Ponte-style osteotomy generally refers to complete removal of both the superior and inferior facets at all levels, typically in the setting of Scheuermann kyphosis. However, this nomenclature has been applied to the scoliotic spine deformity as well, especially when the anterior column is unfused. The Smith-Petersen osteotomy, in contrast, typically refers to performance of these complete facetectomies in the setting of a fully or partially arthrodesed anterior column. The ability to achieve correction with Ponte or Smith-Petersen osteotomies for AIS was characterized well for thoracolumbar and lumbar curves when it was first done on the convex spine, with overall 80% correction of these curves. Correction of thoracic curves with these osteotomies is most likely less than that seen in the lumbar spine because the ribs and chest wall limit correction and blood loss and operative time can be higher. However, bilateral complete facetectomies allow one to lengthen the posterior column to restore thoracic kyphosis while also “untethering” the concave spine to allow shortening and correction of the coronal plane deformity. Following complete removal of the inferior facet, the ligamentum flavum is removed with a large Leksell rongeur in the midline until epidural fat is seen, and completion of the flavectomy is then performed with a Kerrison rongeur ( Fig. 12-37 ). The superior facet on the convex side can be largely removed with a small Leksell rongeur because the dural sac is positioned closer to the concave spine. Care is taken to limit the depth of penetration of the rongeurs on the convex spine because the epidural veins are more prominent on this side. The concave superior facet is more safely removed with Kerrison rongeurs because the dural sac is in close proximity and care must be taken to remain directly on the bone when resection is performed. Subtle SSEP changes may be seen following these osteotomies.
The next level of posterior release would be rib resections on the concave side of the spine to allow lateral and posterior translation of the spine. Three to five ribs are resected by first performing subperiosteal dissection, typically beginning a couple of centimeters lateral to the transverse process and continuing for 3 to 5 cm. Because the ribs are close to the anterior aspect of the chest, small tears in the parietal pleura tend to occur and should be recognized at the time of surgery; placement of a chest tube may be required to prevent pneumothorax. The authors do not generally perform these resections because experience has shown limited appreciable correction and the risk for postoperative pulmonary issues and pneumothorax is high.
The VCR procedure is the most aggressive technique for achieving correction of the spine because it removes one or more vertebral segments at the apex of the deformity. It was initially described for severe spine deformity and trunk shift and used an anterior and posterior approach to the spine, with overall outstanding radiographic and clinical results. Bradford and Tribus reported on 24 patients with severe curves in which greater than 80% correction of the coronal and sagittal plane translation was achieved along with 50% correction of the curves. Despite complications in 14 of the patients, the overall satisfaction rate in patients was extremely high. Suk and co-workers described the posterior-only approach for the VCR procedure and reported 62% correction in the coronal plane and 45 degrees of correction in the sagittal plane in 70 patients with severe deformity from kyphoscoliosis, postinfectious kyphosis, and adult scoliosis. Similar to previous reports, the complication rate was relatively high, with 24 patients having complications, including 2 with postoperative spinal cord deficits who had neurologic deficits preoperatively. Lenke and coauthors reported their experience in performing posterior VCR for pediatric spinal deformity in 35 consecutive patients. The patients were divided into five diagnostic categories, including severe scoliosis, global kyphosis, angular kyphosis, kyphoscoliosis, and congenital scoliosis, with reported correction of 51%, 55%, 58%, 54%, and 60%, respectively. No spinal cord–related complications were reported; however, two patients lost intraoperative neuromonitoring data during correction with return of baseline monitoring, which resulted in prompt surgical intervention. The authors’ experience is similar in 32 consecutive VCR procedures: 4 were done via an anterior/posterior approach and the remainder via a posterior-only approach, with coronal plane correction from 122 degrees to 53 degrees and an average increase in T1 to T12 height of 6.1 cm associated with improvement in pulmonary function. The incidence of intraoperative changes in neuromonitoring parameters during surgery was 35%, with two patients waking with a decline in neurologic function, which returned to baseline.
The indications for posterior VCR are not fully defined for AIS; however, given its significant neurologic risk and long operative times with associated potential for significant blood loss and pulmonary complications, this procedure should be chosen as the technique of last resort. The authors generally use this procedure in individuals with very severe deformity—angular scoliosis or kyphosis—especially when previous fusion has been performed. This procedure should be performed only by the most skilled and experienced surgeons who have worked their way up the learning curve by performing less arduous spine mobilization procedures. A multicenter study of 147 pediatric VCR procedures performed by senior surgeons reported complications in 59% of patients, including 27% who had an intraoperative neurologic event without paraplegia. The posterior approach to the VCR procedure begins with rigid fixation of the spine with pedicle screws proximal and distal to the planned resection levels ( Fig. 12-38, A ). Typically, long-tabbed reduction screws are placed distal to the planned resection levels. The ribs associated with the resected levels are removed entirely, including the rib heads. A laminectomy is next performed to identify the neural elements. The authors prefer to ligate the nerve roots on the convex side first and to remove the pedicle or pedicles to the level of the floor of the spinal canal ( Fig. 12-38, B ). A provisional rod is then placed on the convex side and the concave osseous elements are removed, including the pedicle and the concave side of the vertebral body after the concave nerve roots have been ligated and transected. The concave pedicle and floor of the canal are very difficult to remove because the dural sac and spinal cord are tightly adherent to these structures and the cancellous channel to the concave pedicle is limited. Following complete resection of the concave bone, a second provisional rod is secured so that two provisional rods (one on the concave and one on the convex side) are in place ( Fig. 12-38, C ). The remainder of the convex bony anatomy can be removed with both rods in place because the convex bone is anterior to the convex rod. Following complete resection, exchange of the provisional rods for the final rods is accomplished. The authors typically remove the convex provisional rod and replace it with an undercontoured final rod that is seated in the proximal screws and secured firmly and then partially seated in the tips of the first two reduction screws distal to the resected area ( Fig. 12-38, D ). The final concave rod is placed similarly. The distal aspects of the two final rods are then pushed anteriorly to unhinge them from the set screws on the reduction screws while still providing some guidance to the spine segments. The spine can then be manually compressed without jeopardizing the screw–bone interface. Before this manipulation an anterior cage is placed in the resected area to maintain the length of the spine and to avoid kinking of the dura and potential neurologic deficits ( Fig. 12-38, D ). Sequential reduction of the distal aspect of the rod to the remaining screws is performed, compression is subsequently applied to achieve the final correction, and the rib grafts initially harvested at the time of resection are placed over the exposed dura ( Fig. 12-38, E ).
The choice of spine mobilization procedure is dependent on several factors related to the spine deformity, including its severity, flexibility, amount of previous fusion, and experience of the surgeon. The major risk is neurologic deficit, which occurs more commonly with more aggressive release procedures. The most recent review of the SRS database demonstrated an overall 0.9% incidence of neurologic deficits for all AIS surgery; however, the incidence increased when a Smith-Petersen osteotomy was performed (1.1%) and was greatest when VCR was performed (7.3%).
Anterior Mobilization Techniques.
Anterior release including discectomy, and possibly rib head resection when necessary, can be performed through an open thoracotomy or through a thoracoscopic approach. The thoracoscopic approach has some advantage over the open thoracotomy because smaller incisions are used with less chest wall disruption and less detrimental impact on pulmonary function, especially when it is performed in the prone position with double lung ventilation using a regular single-lumen endotracheal tube ( Video 12-1 ). The combined thoracoscopic release in the prone position and posterior instrumented fusion has resulted in overall excellent radiographic and clinical results.
Correction Techniques
With introduction of the CD implant system, the 90-degree concave rod rotation maneuver was used in an attempt to improve the three-dimensional deformity. The rod was shaped to the coronal plane deformity, and for the typical right thoracic curve, the left rod was rotated counterclockwise so that the scoliotic bend in the rod became the kyphotic bend in the rod to maintain or restore kyphosis.
Excellent coronal and sagittal correction could be achieved with hook constructs; however, the axial plane correction was somewhat limited. The introduction of thoracic pedicle screws and the use of rods with various characteristics of stiffness and strength, together with spine mobilization techniques and more advanced instrumentation, provide an opportunity to better achieve axial plane correction.
Rod rotation continues to be used as the initial step in correction and is maximized with segmental fixation and larger-diameter rods. Alternatively, the apical reduction screws can be used to translate the apex of the spine posteriorly and laterally with an overcontoured rod that is fixed in its final position proximally and distally. This requires significant releases for stiff spines to allow this translation to occur. A stiff cobalt-chrome 6.25-mm rod is preferred in general by the authors, especially for severely hypokyphotic spines.
Following initial placement of the left rod, in situ coronal bending can be performed together with compression on the convex side and distraction on the concave side. At this point, axial plane correction at the apex can be performed with a variety of techniques to correct this component of the deformity via DVR, which allows one to directly rotate each spinal segment individually, or, with the use of connectors for the DVR instruments, en bloc rotation can be performed. A counterforce proximal and distal to the apex should be imparted to most effectively improve the axial plane deformity. The set screws of the apical segments must be loosened on the concave rod to allow maximal axial plane correction. The DVR technique is most effective when only a single rod is in place to allow maximal correction. The axial plane correction maneuver can be performed segmentally, with each screw head being manipulated individually to correct the axial plane ( Fig. 12-39, A and B ), or the screws can be linked together and rotated en bloc ( Fig. 12-39, C and D ). Suk and colleagues reported greater coronal plane correction and rib hump height correction when the DVR technique was used together with thoracoplasty than when thoracoplasty was used alone.
Complications of Posterior Instrumentation and Fusion for Adolescent Idiopathic Scoliosis.
The primary complications associated with posterior surgery and instrumentation include infection, pseudarthrosis, neurologic deficit, and implant-related problems such as prominence, discomfort, and implant failure associated with pseudarthrosis. The overall incidence of reoperation after posterior instrumentation and fusion varies but is below 10% in more recent series. A 1% to 10% incidence of delayed wound infection has been reported, presumably related either to the increased amount of hardware or to the multiple hook–rod connections. * c Some of these episodes of delayed drainage were culture negative and attributed to micromotion at the hook–rod interface. Micromotion causes metallic debris, which leads to a foreign body reaction, formation of a false membrane and fluid, and finally loosening of the implant. Rather than representing an aseptic process, it is more likely that these delayed infections result from low-virulence organisms that are seeded at the time of surgery and remain quiescent over an extended period.
References .
Risk factors for the development of a delayed infection are the presence of a significant past medical history, receiving a blood transfusion, and not having a deep drain placed. The incidence of pseudarthrosis is very low with modern double-rod systems, segmental instrumentation, and the use of allograft bone.The most feared complication in spine deformity surgery is neurologic deficit, whose incidence has remained steady through the years and is still below 1%. Reames and coauthors reported the most recent analysis of the SRS database, which found a 0.8% incidence of neurologic deficit at the time of surgery for AIS. The likelihood of complete or partial recovery of neurologic deficits is high in all series. The incidence of neurologic deficit is generally regarded to be higher with combined anterior/posterior surgery and when osteotomies are performed and is generally thought to be of vascular origin.
Anterior Spinal Instrumentation
Anterior Approach to Thoracolumbar and Lumbar Curves.
The anterior approach to thoracolumbar and lumbar curves has been successful in correcting deformity and preventing curve progression. However, its use for these curves has diminished with greater use of pedicle screws and spine mobilization procedures. Shufflebarger and associates reported 80% correction of these curves with a posterior approach that involved the use of segmental pedicle screws at each level with wide Ponte-style osteotomies and convex compression. Comparison studies between the posterior and anterior approaches have demonstrated similar levels of instrumentation with similar correction of deformity; however, the posterior approach is associated with a shorter operative time and hospital stay without the need for a chest tube or a general surgeon to perform the surgical approach. In general, we treat all thoracolumbar and lumbar curves via a posterior approach except when the planned LIV is significantly translated from the CSVL, in which case the anterior approach with complete diskectomies and powerful derotation of the spine may allow better correction of the three-dimensional deformity of the spine.
Solid-Rod Anterior Instrumentation.
TSRH instrumentation extended the concepts of Zielke by using a stiff, smooth, solid rod as the longitudinal connection between vertebral screws ( Fig. 12-40 ). The resulting stiffer, fatigue-resistant construct enhances the maintenance of correction and the likelihood of arthrodesis without postoperative external immobilization in most cases. Deformity is corrected by rotation of a 6.4-mm rod that is precontoured for lordosis (similar to the CD instrumentation principles for thoracic curves posteriorly, but in reverse). Because correction of deformity is achieved by gradual rod rotation, corrective forces are evenly distributed all along the construct simultaneously rather than applied acutely or gradually at a single segment.
The early results of anterior single-rod treatment of thoracolumbar and lumbar curves included a high incidence of pseudarthrosis, which improved with rib strut grafts. The addition of anterior interbody structural support in the form of a titanium cage or femoral ring allograft significantly increased the flexion–extension stiffness of the construct when using single-rod implants, and it appears to increase the likelihood of fusion. It is important to place the structural interbody support in the most anterior aspect of the intervertebral disk space to obtain optimal load sharing and in the concavity of the curve to aid in coronal plane correction. The concept of dual-rod constructs for anterior scoliosis surgery was popularized by Kaneda as a method of increasing the stiffness of the single-rod construct while maintaining coronal and sagittal plane correction and preventing pseudarthrosis. The stiffness of dual-rod systems is better than that of single-rod systems in flexion, extension, and torsion, with no increase in lateral-bending stiffness. The addition of anterior structural support to a dual-rod system increases overall construct stiffness in flexion but not in other loading conditions.
The surgical technique for dual-rod systems is to initially place two screws in each vertebra, with the posterior screw traversing along the posterior cortex of the vertebral body and the anterior screw directed in an anterior-to-posterior direction to provide increased pullout and plow resistance (see Plate 12-4 , I-M on page 306 .) A staple is similarly used to capture both screws. A contoured rod with the desired lordosis is placed into the posterior screws, and a rod rotation maneuver is performed in a similar manner as a single-rod technique to correct the scoliosis and restore or maintain lordosis. Anterior structural support in the form of a titanium mesh cage filled with the autologous rib graft harvested during the surgical approach is next placed to help maintain lordosis and assist in achieving correction of scoliosis. The second rod is then placed and slight compression is applied to lock in the anterior structural support. Kaneda and associates reported their results with Kaneda’s dual-rod system without anterior structural support in 25 patients (20 with idiopathic scoliosis and 5 others) with thoracolumbar or lumbar curves; they demonstrated average coronal correction of 83%, restoration of 9 degrees of lordosis (7 degrees of preoperative kyphosis), and no cases of pseudarthrosis. Others have demonstrated excellent correction and maintenance of correction with a dual-rod system. The Halm-Zielke system is composed of a lid plate fixed to the lateral aspect of the vertebral body with two screws—a screw sunk anteriorly and a second ventral derotation spondylodesis screw posteriorly. This low-profile system has yielded excellent correction with few complications. The dual-rod systems emphasize the importance of construct stiffness and the stability provided by the second rod.
Fusion levels for thoracolumbar and lumbar scoliosis are generally from the proximal end vertebra to the distal end vertebra, as assessed on the preoperative PA radiograph. Deciding on the distal fusion level is challenging because of the relatively common occurrence of a postoperative oblique disk (which opens into the convexity of the curve) below the fusion levels. This problem is more likely to occur when the disk below the distal end vertebra is parallel and the LIV is closer to the apex of the curve on the preoperative radiograph. Despite these challenges, relatively good success has been achieved with the so-called short-segment anterior fusion for mild to moderate curves that are relatively flexible. The fusion levels are based on the level of the apex of the deformity: when a disk is the apex, fusion should be two levels above and two levels below the disk; when the apex is a vertebra, fusion is one level above and one below the apex. The success of this surgical strategy is dependent on achieving overcorrection of the instrumented segments to translate the spine and balance the patient.
Anterior Instrumentation for Thoracic Deformity.
Dwyer and colleagues proposed this technique in the 1960s but found that correction was unsatisfactory with the cable systems. Since then, use of anterior instrumentation for thoracic deformity has reemerged. In 1988, Harms began repopularizing the idea after conjecturing that anterior correction without a posterior derotation maneuver of the thoracic curve in King type II deformities would prevent the lumbar curve from decompensating, as had been described following selective posterior instrumentation.
The technique of anterior spinal fusion and instrumentation (ASFI) of thoracic curves achieves excellent coronal curve correction and restoration of thoracic kyphosis, and the lumbar curve responds nicely in selected thoracic fusion situations. Advantages of the anterior approach over the posterior approach include the ability to save motion segments, no stripping of the paraspinal muscles and thus avoidance of scarring and perhaps subsequent pain, limited development of late operative site pain (seen with posterior implants), and a lower incidence of infection. Betz and colleagues, in a comparison of anterior and posterior approaches for thoracic scoliosis, found 58% correction of the coronal curve and an average of 2.5 levels that were saved with the use of anterior surgery; however, a high incidence (31%) of rod fracture occurred when a small-diameter rod (3.2 mm) was used. More recent studies using rods 4.0, 5.0, or 5.5 mm in diameter showed 47% curve correction, but pseudarthrosis developed in 7% of patients. A lower incidence of pseudarthrosis is seen with dual rods, but the challenge of placing two screws in the thoracic spine may prevent widespread use of this technique.
When placing anterior instrumentation in the thoracic spine it is necessary to understand the anatomic landmarks, including the relationship between the rib head and the vertebral body. The correct starting point for the screw must be identified to avoid the spinal canal. On the contralateral side of the chest, the aorta is positioned more laterally and posteriorly than is the case with a straight, normal spine. This exposes the aorta to a higher risk for injury when placing anterior screws. Transvertebral anterior screws should penetrate the far cortex only slightly to avoid injuring these important soft tissue structures.
The traditional surgical approach for ASFI of the thoracic spine was through an open thoracotomy incision ( Fig. 12-41 ). Thoracoscopic ASFI was first introduced in the mid-1990s following experience with thoracoscopy for anterior release and fusion in conjunction with posterior spinal fusion and instrumentation. Advantages of the thoracoscopic technique include smaller incisions, which results in improved cosmesis, and less chest wall dissection, which results in both less postoperative pain and improved pulmonary function. The disadvantage of the technique is that it is technically demanding, which results in a fairly steep learning curve, long operative times, and a relatively high pseudarthrosis rate.
Ideal patients for thoracoscopic ASFI include those with good pulmonary function; active patients with high bone mineral density for good screw purchase; tall, thin patients to allow easy portal placement and manipulation; and those with thoracic curves that have a coronal magnitude of less than 70 degrees and a flexibility index greater than 50%. Follow-up has demonstrated that the results of thoracoscopic AFSI are similar to those of open ASFI, with 55% coronal plane correction and a pseudarthrosis rate of 6%. When compared directly with a posterior approach, thoracoscopic ASFI is associated with similar coronal plane correction and less intraoperative blood loss but longer surgical times ( Fig. 12-42 ).
Juvenile Idiopathic Scoliosis
The prevalence of juvenile idiopathic scoliosis (3 to 10 years of age) is 8% to 12% in Europe and 13% to 16% in the United States. The deformity is usually recognized clinically by the age of 6 to 7 years. The female-to-male ratio ranges from 1.6 : 1 to 4.4 : 1, with the difference increasing with age. Convex right thoracic curve patterns are most common, and relatively few patients have thoracolumbar or lumbar curves.
Predicting Curve Progression
Juvenile scoliosis is more likely to progress, less likely to respond to bracing, and more likely to require surgical treatment than AIS is. Unlike infantile scoliosis, use of the rib–vertebral angle difference (RVAD) does not predict curve progression in juvenile scoliosis. Patients with progressive curves have a steady increase in the RVAD, whereas those whose curves will resolve usually show a decrease in the RVAD. If the RVAD does not improve following bracing of a progressive curve, spinal fusion will probably be required as definitive treatment. The level of the most rotated vertebra at the apex of the primary curve appears to be the most useful factor in determining the prognosis of patients with juvenile idiopathic scoliosis. Those with a curve apex at T8, T9, or T10 have an 80% chance of requiring spinal arthrodesis by 15 years of age. The predictive value of two other factors once thought to be associated with a poor prognosis—thoracic kyphosis of less than 20 degrees and left-sided curves in boys—is uncertain.
Neural Axis Abnormalities
MRI studies have provided greater insight into juvenile idiopathic scoliosis. The incidence of neural axis abnormalities in these patients is 18% to 26%. Most of these children are asymptomatic and have no physical signs (other than scoliosis) of an underlying neural axis abnormality. MRI abnormalities include Chiari type I malformations with cervical syrinx, thoracic syrinx, brainstem tumor, dural ectasia, and low-lying conus. Many of these abnormalities may benefit from neurosurgical treatment. As a result, some authors recommend MRI during the initial evaluation of patients presumed to have juvenile idiopathic scoliosis. If scoliosis surgery is planned, it is imperative that preoperative MRI evaluation be undertaken. Neurologic deficits following spinal surgery have been reported in patients with neural axis abnormalities that were not recognized preoperatively.
Treatment options for children with juvenile scoliosis are outlined in the section “Early-Onset Scoliosis.” As the child approaches 8 to 10 years of age, a definitive surgical procedure should be considered ( Fig. 12-43 ).
Congenital Spinal Deformities
Congenital deformities of the spine are caused by anomalies in the growing vertebrae. These anomalies may be subtle and found incidentally on radiographs obtained for some other reason, or they may be complex and lead to severe spinal deformity with accompanying neurologic deficits. Congenital scoliosis, congenital kyphosis, and a combination of the two are the deformities encountered. They are much less common than idiopathic scoliosis.
Etiology
The cause of congenital vertebral anomalies remains unknown. During embryologic development, these abnormalities develop in the spine between the fifth and eighth weeks of gestation, but it is very uncommon to identify any traumatic or teratologic type of maternal insult during this stage of pregnancy.
Research has found that carbon monoxide exposure and the resulting hypoxia can lead to reproducible congenital spinal deformities in mice offspring. Such deformities include wedged vertebrae, hemivertebrae, fused vertebrae, and missing vertebrae, as well as fused ribs. The severity of the deformities is related to both the dose of carbon monoxide and the time during gestation that exposure occurred. Correlating with this basic science study, the same institution reported clinical data indicating a potential increase in exposure to fumes (chemical fumes and carbon monoxide) in the mothers of children with congenital spinal deformities.
Investigation of genetic causes has provided modest insight. A positive family history can be found in approximately 1% of patients with congenital spinal deformities. Idiopathic scoliosis has been reported in 17% of families of children with congenital scoliosis. An isolated anomaly, such as a hemivertebra, usually occurs as a sporadic event and carries no risk for a similar abnormality in other offspring. Studies of identical twins, only one of whom was affected, showed no genetic pattern, but other reports of twins with similar congenital deformities suggested the possibility of genetic causes. Scientists have identified the human gene HuP48 , a member of the Pax family of developmental control genes, as having a role in establishing the segmented pattern of the vertebral column. As yet, no mutations in this gene have been found in those with vertebral segmentation defects. A chromosomal aberration, deletion of 17p11.2, has been reported in congenital scoliosis but needs further verification. Analysis of the candidate gene DLL3 has raised the possibility of its involvement in congenital scoliosis. However, no definitive cause of anomalous vertebral development has yet been established.
Associated Abnormalities
The neural axis, vertebral column, and other organ systems develop at a similar stage in utero. A nonspecific insult during this embryonic period has been suggested to destabilize the developmental control systems and may result in congenital malformation of any organ undergoing concurrent epigenesis. The most common associated finding is intraspinal anomaly, a general category that includes numerous abnormalities such as tethered cord, diastematomyelia, syringomyelia, diplomyelia, Arnold-Chiari malformations, and intraspinal tumors. †c The incidence of one of these associated neural axis abnormalities developing ranges from 21% to 37%. All these abnormalities are best identified with MRI.
†c References .
Once an intraspinal abnormality (such as a diastematomyelia spur) has been identified, it should be addressed neurosurgically if a progressive neurologic deficit has developed or if surgical correction of the scoliotic deformity is needed ( Fig. 12-44 ). To some physicians the mere presence of a potentially tethering intraspinal lesion may be sufficient reason for prophylactic surgical treatment. The rationale for this early aggressive approach is to address the lesion before the development of any neural dysfunction. Any of these neural axis lesions may be associated with a more visible clinical abnormality such as a hairy patch, a nevus, or a distinct neurologic deficit. Subtle deficits can also be present, thus making a careful neurologic examination imperative for any patient with a congenital spinal abnormality. In view of the relatively high incidence of intraspinal anomalies and the fact that clinical manifestations may not be detectable initially, MRI has been recommended as part of the initial evaluation in all patients with congenital spinal deformities, even in the absence of clinical findings.In addition to neural axis abnormalities, approximately 60% of patients have associated abnormalities affecting other systems. Approximately 20% of patients have an anomaly of the genitourinary system, and cardiac anomalies are seen in approximately 12% to 26% of patients. Other abnormalities include cranial nerve palsy, radial hypoplasia, clubfoot, dislocated hip, Sprengel deformity, imperforate anus, and hemifacial microsomia.
Congenital Scoliosis
Congenital scoliosis may not become evident until later childhood, even though the vertebral anomalies are present at birth. In a child younger than 3 years, differentiation between infantile idiopathic scoliosis and congenital scoliosis can be difficult. Close examination of radiographs usually reveals the vertebral abnormalities present in children with congenital scoliosis.
The variety of vertebral anomalies that can exist in those with congenital scoliosis leads to an unpredictable natural history. The deformity may remain mild, or it may progress dramatically over time and ultimately result in severe spinal deformity and pulmonary compromise. Understanding which vertebral anomalies place the scoliotic spine at greatest risk for progressive deformity allows the treating physician to intervene at the appropriate time.
Classification
Two basic types of abnormalities lead to congenital scoliosis: defects of vertebral formation and defects of vertebral segmentation ( Fig. 12-45 ). Hemivertebrae and wedged vertebrae are examples of defects of formation. Defects of segmentation include block vertebrae, unilateral bars, and unilateral bars accompanied by hemivertebrae. Approximately 80% of the vertebral anomalies associated with congenital scoliosis can easily be classified into one of the two types; the remaining 20% cannot be precisely classified. Many patients have a combination of deformities in which one type predominates.
A newer classification system introduced in 2009 uses three-dimensional CT images of congenital spinal deformities. Four types of congenital vertebral abnormalities were introduced: type 1, solitary simple; type 2, multiple simple; type 3, complex; and type 4, segmentation failure ( Table 12-4 ). This improved three-dimensional understanding of the variations in congenital scoliosis may be helpful in preoperative planning for correction of these complex deformities.
Type 1 | Solitary simple (unison) type |
Hemivertebra | |
Wedged vertebra | |
Butterfly vertebra | |
Defect | |
Others | |
Type 2 | Multiple simple (unison) type |
Combination of hemivertebra, wedge vertebra, or butterfly vertebra | |
Discrete, adjacent, or others | |
Type 3 | Complex (discordant) type |
Mismatched complex type | |
Mixed complex type | |
Type 4 | No abnormal formation type |
Pure segmentation failure |
Defects of Formation
Defects of formation may be partial or complete. Partial unilateral failure of formation produces a wedged or trapezoid-shaped vertebra that contains two pedicles, although one of them may be hypoplastic. The associated scoliosis worsens slowly and may not require treatment.
True hemivertebrae are caused by complete failure of formation on one side and result in laterally based wedges consisting of half the vertebral body, a single pedicle, and a hemilamina. Occasionally, the lamina associated with the hemivertebra may be incorporated into that of the adjacent normal-appearing vertebra. When this occurs, differentiating between the anterior vertebral abnormality and the corresponding posterior abnormality becomes difficult. Hemivertebrae in the thoracic spine are usually accompanied by an extra rib. Hemivertebrae may be fully segmented (most common), semisegmented, nonsegmented, or incarcerated (least common) ( Fig. 12-46 ). Distinguishing among these various types is important because the associated differences in growth potential have a profound effect on the eventual severity of the spinal deformity.
A fully segmented hemivertebra has the highest likelihood of progressive deformity because it is separated from the adjacent vertebrae by intact end-plates and intervertebral disks. The hemivertebra is nearly always located at the apex of the scoliosis. Lower thoracic and thoracolumbar curves tend to worsen more rapidly than do curves at other levels. When two or more hemivertebrae are present on the same side of the spine, the deformity progresses at a faster rate. Conversely, the spinal deformity may be balanced and nonprogressive if two hemivertebrae are situated opposite each other. A fully segmented hemivertebra at the lumbosacral junction creates significant obliquity between the spine and pelvis and is usually accompanied by a long compensatory scoliosis in the lumbar or thoracolumbar region. This readily apparent deformity is best treated surgically (usually by hemivertebrectomy) at an early age, before the compensatory curve becomes fixed ( Fig. 12-47 ).
A semisegmented hemivertebra is separated from one adjacent vertebra (superior or inferior) by a normal vertebral growth plate and disk but is fused to the other adjacent vertebra. Although growth of the spine should remain balanced, the hemivertebra can induce a slowly progressive scoliosis. Treatment is necessary only if the deformity is progressive (see Fig. 12-46 ).
A nonsegmented hemivertebra is fused to both adjacent vertebrae (above and below) and therefore has no vertebral end-plates or adjacent disks. In the absence of any asymmetric growth, a nonsegmented hemivertebra does not cause progressive spinal deformity. An incarcerated hemivertebra is more ovoid and smaller than a fully segmented (nonincarcerated) hemivertebra. The vertebrae above and below compensate for this hemivertebra, and as a result, minimal if any scoliosis is present.
Defects of Segmentation
Defects of segmentation result in a bony bar or bridge between two or more vertebrae, either unilaterally or involving the entire segment. Circumferential, symmetric failure of segmentation leads to a block vertebra ( Fig. 12-48 ). This does not cause any angular or rotational spinal deformity but does lead to some loss of longitudinal growth. Klippel-Feil syndrome in the cervical spine represents a severe form of this failure of segmentation.
Unilateral failure of segmentation of two or more vertebrae (unilateral bar) is the most common cause of congenital scoliosis. Usually, a bar of bone fuses the disk spaces, pedicles, and facet joints on one side of the spine, thus precluding growth on the side of the concavity ( Fig. 12-49 ). Growth usually proceeds on the convexity and leads to worsening of the deformity. Rib fusions or other rib abnormalities on the concavity of the scoliosis are often seen adjacent to the bony bar bridging the vertebrae.