Adolescent Health Care: A Practical Guide

Chapter 15

Scoliosis and Kyphosis

Matthew J. Bueche

Introduction

Spinal deformity is a major concern in the adolescent population. Teens with poor posture are frequently brought to the physician by concerned parents, whereas those with more serious structural scoliosis and kyphosis may escape attention. Marked deformity can exist without symptoms. Although the efficacy of school screening programs is controversial, clinicians should routinely examine adolescents for spinal deformity, as treatment may improve the outcome.

Definition

Scoliosis is a lateral curvature of the spine, usually associated with rotational deformity of the spine and trunk. Scoliosis is defined as a curvature of 10 degrees or greater on a frontal radiograph (measured by the Cobb Method [Fig. 15.1]).

FIGURE 15.1 The Cobb method of radiographic measurement of scoliosis. A line is drawn parallel to the inferior end plate of the lower end vertebra (LEV) (the vertebra that is most tilted) (1), another line is drawn at the superior border of the upper end vertebra (UEV) (3), perpendicular lines are drawn from each of these previous lines (2 and 4), and the intersecting angle is then measured. Drawing the perpendiculars keeps the measurement from running off the edge of the film. A similar method is used on the lateral film to measure sagittal plane curves. (Adapted from O'Brien MF, Kuklo TR, Blanke KM, et al., eds. Radiographic measurement manual. Spinal Deformity Study Group. Memphis, Tennessee: Medtronic Sofamor Danek, 2004:110.)

Etiology

Scoliosis is a condition rather than a diagnosis. Most adolescents have adolescent idiopathic scoliosis (AIS), a condition with no apparent cause. Scoliosis may be associated with neuromuscular diseases such as spastic quadriplegic cerebral palsy and Duchenne muscular dystrophy. Congenital scoliosis is deformity secondary to vertebral malformations. Nonstructural or functional scoliosis may result from leg length discrepancy or from splinting due to back pain. Scoliosis may be part of a syndrome or disease such as Marfan syndrome, neurofibromatosis, or myelodysplasia.

Idiopathic Scoliosis

Types

There is no known cause of idiopathic scoliosis. It is usually categorized into three age-groups:

1. Infantilescoliosis is diagnosed between birth and 3 years, occurs more frequently in males during the first year of life, and may be related to postnatal supine positioning. Most curves resolve spontaneously, although brace treatment or surgery may be necessary. Infantile idiopathic scoliosis is quite rare.
2. Juvenilescoliosis manifests between 3 and 9 years of age. Boys and girls are affected equally. Like AIS, the risk

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of curve progression increases with curve severity and treatment may be initiated at similar curve magnitudes.

3. Adolescentscoliosis is the most frequent form and manifests between 10 years of age and the time of skeletal maturity. It occurs most commonly in girls.

TABLE 15.1 Risk of Progression of Idiopathic Scoliosis

Age at Presentation Initial Curve (degrees) Girls, 10–12 yr (%) Girls, 13–15 yr (%) Girls, >15 yr (%) Boys (%) From Nachemson A, Lonstein JE, Weinstein SL. Prevalence and natural history committee report. Read at the Annual Meeting Scoliosis Research Society. Denver, CO: Scoliosis Research Society, Sept. 25, 1982. <19 25 1 <1 3 20–29 60 40 10 6 30–59 90 70 30 — >60 100 90 — —

Prevalence

Approximately 2% to 3% of adolescents have a curve >10 degrees and 0.5% have a curve >20 degrees. Although minimal curves occur as often in boys as in girls, curves requiring treatment are found seven times more frequently in girls (Rogala et al., 1978).

Causes

The etiology of AIS is believed to be multifactorial (Miller, 1999):

1. There is an increased incidence of scoliosis in first-degree relatives (Wynne-Davis, 1968). Genomic testing studies to identify markers for AIS are underway (Miller et al., 2005;Bashiardes et al., 2004).
2. Subtle abnormalities in vestibular or other neurological function.
3. Abnormal connective tissue or muscle composition has been noted in subgroups of patients, but these abnormalities may be a result of the disorder rather than a cause.

Natural History

Long-term complications of continued curve progression including cosmetic effects, back pain, neurological compromise, and restrictive pulmonary disease leading to cor pulmonale and death have been described. Fifty-year follow-up of untreated AIS found a tendency toward continued progression of the deformity (Weinstein et al., 2003). Back pain was present more frequently in patients than in the age-matched control group, although most patients reported only mild or moderate pain. Shortness of breath was reported more frequently in the AIS group. In the same study, 3 of the 36 deaths were likely related to the scoliosis.

Adults treated for AIS were found to recall transient body-image dissatisfaction and negative peer interactions during adolescence. Lower body-image scores persisted for several years in surgical patients (Noonan et al., 1997).

Risk Factors for Progression

A combination of the following factors can be helpful in predicting as many as 80% of progressive curves (Peterson and Nachemson, 1995).

1. Age at onset and gender (Table 15.1)
2. Magnitude of the curve: The amount of curvature is measured by the Cobb method (Fig. 15.1). In this method, a line is drawn parallel to the inferior end plate of the lower end vertebra (the vertebra that is most tilted), another line is drawn at the superior border of the upper end vertebra (that most tilted in the other direction), perpendicular lines are drawn from each of these previous lines, and the intersecting angle is then measured. Despite the apparent simplicity of the technique, there is considerable intraobserver and interobserver variability (Carman et al., 1990). Patients with a 20-degree curve have a 20% risk of progression, whereas those with a 50-degree curve have a 90% chance of progression (Lonstein and Carlson,1984).
3. Skeletal maturity: The Risser stage (0–5) gives a useful estimate of how much skeletal growth remains by grading the progress of ossification of the iliac apophysis. Risser stage is determined by the appearance of the secondary ossification center of the iliac crest. Risser grade zero signifies no ossification whereas Risser grade 5 signifies complete bony fusion of the apophysis (Fig. 15.2). Patients with Risser stage 0 show progression 36% to 68% of the time, whereas those who have a Risser stage 3 or 4 progress only 11% to 18% of the time.

Scoliosis Screening

The American Academy of Orthopedic Surgeons recommends screening girls at ages 10 and 12 (grades 5 and 7) and screening boys once at age 13 or 14 years (grades 8 or 9) (AAOS, 1992). The Scoliosis Research Society concurs with this recommendation. In addition, some state laws mandate school screening for scoliosis. In contrast, the U.S. Preventive Services Task Force's (USPSTF's) Guidelines for the Guide to Clinical Preventive Services states that “The

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USPSTF found fair evidence that treatment of adolescents with idiopathic scoliosis detected through screening leads to moderate harms, including unnecessary brace wear and unnecessary referral for specialty care. As a result, the USPSTF concluded that the harms of screening adolescents for idiopathic scoliosis exceed the potential benefits” (U.S. Preventive Services Task Force, 2004).

FIGURE 15.2 The Risser sign. The iliac apophysis appears first at the anterior superior iliac spine; ossification then progresses medially toward the posterior superior iliac spine. The crest is divided into four equal quadrants with stages 1 through 4 coinciding with extension of the ossification center into each quadrant and stage 5 being complete fusion of the physis (Risser, 1958). (From O'Brien MF, Kuklo TR, Blanke KM, et al., eds. Radiographic measurement manual. Spinal Deformity Study Group. Memphis, Tennessee: Medtronic Sofamor Danek, 2004:110.)

The sensitivity, specificity, and positive and negative predictive value of screening tests depend on the degree of curvature defined as abnormal, the training of the screener, and the prevalence in the population. Properly trained clinicians using an inclinometer (Fig. 15.3) can evaluate the need for scoliosis radiographs. “Schooliosis” or the over-referral of normal adolescents from school screening programs is a known phenomenon. Better communication and proper technique should allow screening to continue at efficient levels (Dvonch et al., 1990).

Clinical Evaluation

History

The patient history should include the following:

1. Age and menarchal status.
2. Presence of pain: Most scoliosis in adolescents is painless and diagnosed secondary to deformity. Significant pain requires a thorough evaluation for an identifiable cause.
3. Neurological symptoms.
4. Family history of scoliosis and spine disorders.

Physical Examination

Evaluation of suspected scoliosis should be directed at the deformity, its cause, and complications. The general examination should include the following:

1. Height: Serial measurements aid in determining risk of progression.
2. Neurological examination: Including reflexes, strength, sensation, and coordination/gait.
3. Skin: Including café-au-lait spots and hairy patches over the spine.
4. Pelvic obliquity: Leg length inequality.
5. Sexual maturity rating: To evaluate risk of progression.

Spinal Examination

Performed with the patient standing, wearing underwear and a gown open in the back

1. Side-to-side symmetry
2. Shoulder height
3. Iliac crest symmetry: They may have to be palpated to ascertain leg length equality.
4. Forward bending: The Adams forward bend test is performed with the adolescent bending forward at the waist until the spine becomes parallel to the horizontal plane, while holding palms together with arms extended and knees straight. The examiner looks along the horizontal plane of the spine from the back and side to detect an asymmetry in the contour of the back for a “rib hump” or paralumbar prominence. This examination may also reveal inflexibility of the spine or muscle spasm. Any rib hump or paralumbar hump should be measured with an inclinometer. The angle of trunk rotation (ATR) is measured by centering the device over the spinous process at the area of greatest asymmetry (Fig.15.3), with the patient flexed at the waist, such that this area is parallel to the floor. The test may not be specific for spinal deformity, because truncal asymmetry may exist in the absence of measurable scoliosis.
5. Lateral examination: Evaluation for sagittal plane deformity is performed with the examiner at the patient's side. Departures from the normal contours of thoracic kyphosis and lumbar lordosis are noted. The forward bend test is then repeated, noting the smoothness of the thoracic kyphosis (see Scheuermann kyphosis in the subsequent text).

Physical examination signs indicative of nonidiopathic scoliosis are listed in Table 15.2.

Imaging

Recommendations for Radiographs for Suspected Scoliosis

Radiographic studies are recommended for adolescents with an ATR >7 degrees, measured by an inclinometer. This threshold value balances the risks of false-positive and false-negative results (Bunnell, 1993).

Which Radiographs to Order?

The standard films for the evaluation of scoliosis are standing posteroanterior and lateral spinal radiographs using 36-in. films taken at 6-ft distance to allow measurement of the curve using the Cobb method and Risser staging of the iliac apophysis. Directing the beam from posterior to anterior measurably decreases the radiation dose to the breasts and thyroid (Levy et al., 1996).

Skeletally immature patients at risk for curve progression can be followed up with posteroanterior radiographs every 6 to 12 months.

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FIGURE 15.3 The inclinometer (scoliometer). The device is centered over the spinous process at the area of greatest asymmetry. The patient flexes at the waist sufficiently to bring the right thoracic area (A) and left lumbar area (B) parallel to the floor. (From Weinstein SL, ed. The pediatric spine. Principles and practice, 2nd ed. Philadelphia: Lippincott Williams & Wilkins, 2001 Figure 4, page 135.)

Radiographic indications of nonidiopathic scoliosis are listed in Table 15.3.

Magnetic Resonance Imaging

Magnetic resonance imaging (MRI) of the full spine is recommended for adolescents with the following findings:

1. Neurological findings (including café-au-lait spots) on physical examination.
2. Unusual pain.
3. Left major thoracic curve: These are associated (in 10% of cases otherwise thought to be idiopathic) with occult syrinx, Arnold-Chiari malformation, spinal cord tumor, and neuromuscular disorder. Only 2% of adolescent idiopathic thoracic curves are convex to the left (Davids et al., 2004).

Criteria for Referral

Consultation with an orthopedic surgeon (ideally, one who specializes in pediatric orthopedics or orthopedic spinal surgery) should be considered for

1. skeletally immature patients with Cobb angle >20 degrees;
2. skeletally mature patients with Cobb angle >40 degrees.

Treatment

Major treatment options include observation, bracing, and surgery (Table 15.4). Treatment choices in AIS often involve consideration of the teen's physiological (not chronological) maturity, curve magnitude and location, and the potential for progression.

TABLE 15.2 Signs of Nonidiopathic Scoliosis

Region Finding Possible Significance Skin Hairy patch over spine Congenital spinal anomaly Café-au-lait spots Neurofibromatosis Joints Hyperelasticity Marfan syndrome/Ehlers-Danlos syndrome Reflexes (including abdominal) Abnormal Neuromuscular disease Extremities Excessively long Marfan syndrome Excessively short Skeletal dysplasias Pelvis (standing) Obliquity Leg length discrepancy

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TABLE 15.3 Radiographic Signs of Nonidiopathic Scoliosis

Radiographic Finding Possible Significance Sharply angular curve Neurofibromatosis Fused ribs Congenital scoliosis Widened intrapedicular distance Syringomyelia Left thoracic curvature Neuromuscular etiologies

Brace Therapy

Brace therapy should be considered in the skeletally immature adolescent who has 30 to 40 degrees of curvature or who has a curve >25 degrees with a demonstrated increase of 5 degrees. The primary goal of brace management is to halt curve progression. This treatment modality should not be expected to permanently improve curvature. Although curve magnitude typically decreases during brace treatment, this improvement is often lost in the years following brace weaning.

There are a number of different bracing systems (e.g., Boston, Milwaukee) that have been used to prevent the need for surgery. The Milwaukee brace features a neck ring that may cause cosmetic concerns. Most other scoliosis braces are variants of the thoraco–lumbar–sacral orthosis (TLSO), an underarm brace. It is generally recommended that the braces be worn almost full-time, with removal for bathing and sports participation. Part-time (18 hour/day) programs may be more acceptable to the patient who refuses to wear the brace to school (Green, 1986).

Despite a high incidence of noncompliance, outcomes are better than would be expected from the natural history alone (Nachemson and Peterson, 1995). Less than full-time wear may decrease the psychosocial impact of brace treatment. However, a meta-analysis of brace studies indicated a trend toward decreasing efficacy with decreased time in brace (Rowe et al., 1997). Another study showed that decreasing the prescribed hours of daytime brace wear did not increase compliance (Takemitsu et al., 2004). Nighttime-only braces (e.g., Charleston, Providence) avoid some of the compliance problems associated with daytime braces. Such braces attempt to make up for decreased treatment time by hyper-correcting the curve into the opposite direction (difficult to accomplish in a brace designed for an upright patient, but more feasible when recumbent). These braces have shown promise in patients with specific curve patterns. Among those treated with the Charleston brace, 66% had <5 degrees progression and only 16% required subsequent surgery at long-term follow-up (Price et al., 1997).

TABLE 15.4 Management of Adolescent Idiopathic Scoliosis

Curve Size (degrees) Therapy 0–25 Serial observation if immature 25–30 (with progression of 5–10 degrees) Brace 30–40 Brace >40 Surgery if immature >50 Surgery (adult)

Skeletal maturation, defined as no further changes in height and a Risser stage IV, is usually considered the endpoint for brace use.

Surgery

Surgical management is recommended for skeletally immature patients who have a curve >40 to 45 degrees or in skeletally mature patients who have curves >50 degrees, particularly if curve progression has been documented. Surgery fuses the vertebrae in the curve, preventing further progression. Rods are implanted as internal fixation, both stabilizing the spine to allow for reliable fusion and allowing for considerable curve correction. Post-operative bracing is frequently unnecessary. Hospital stays are usually for less than a week and students miss approximately 1 month of school. Although mid- or low-lumbar fusions may cause long-term back stiffness, this does not commonly occur with thoracic fusions.

Other Treatments

Exercise programs have not been shown to alter the natural history of idiopathic scoliosis. Electrical stimulation has proved to be ineffective for scoliosis in multiple studies.

Increased Kyphosis

Normal sagittal plane posture includes rounded shoulders (thoracic kyphosis) and sway-back (lumbar lordosis). Increased kyphosis in adolescents is commonly caused by juvenile postural roundback and less commonly, by Scheuermann kyphosis.

Adolescent Roundback (Juvenile Postural Roundback)

Many adolescents manifest an excessively kyphotic posture. The spine is flexible enough that the adolescent can stand straighter if they desire, although they may complain of muscle pain and fatigue. On forward bending, the spine shows a smooth curvature when viewed from the side. Lateral radiographs may show the increased thoracic kyphosis, but the individual vertebrae and disc spaces will appear normal. It is more common among girls than among boys. It is usually painless.

An exercise program directed by a physical therapist may be helpful if the adolescent is in pain or unhappy with his or her appearance.

Scheuermann Kyphosis

Scheuermann kyphosis is a relatively rigid, abnormally increased kyphosis of the thoracic and thoracolumbar spine that does not correct with hyperextension of the spine. The kyphosis results from anterior vertebral wedging in the affected area of the spine. The matrix of the vertebral end plate has characteristic changes in the ratio of collagen to proteoglycans that leads to an altered ossification process. The exact etiology of Scheuermann disease is unknown but is thought to include excessive

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stress, genetic predisposition, and congenital malformation of the vertebral end plates. The prevalence is estimated to range from 0.4% to 8.0% in the adolescent population, with a peak age of onset at 12 to 13. There is equal incidence in boys and girls.

Definition and Classification

The radiographic diagnosis of Scheuermann kyphosis is made on a standing lateral 36-in. film. The criteria include the following:

1. More than 5 degrees of anterior wedging of at least three adjacent vertebral bodies
2. Narrowing of the intervertebral disc space
3. Irregularity of the vertebral endplates and Schmorl nodes—abnormal protrusions of intervertebral disc material into the vertebral bodies
4. More than 45 degrees of kyphosis (normal is 20 to 45 degrees), measured by the Cobb method

The classification of Scheuermann kyphosis is made by the location of the kyphosis. Thoracic curves are by far the most common (75%) and thoracolumbar curves are more likely to progress even after skeletal maturity. “Lumbar Scheuermann disease” is distinct from true Scheuermann kyphosis in that it does not have the anterior wedging of the vertebral bodies.

Clinical Manifestations

1. Back deformity: Presenting complaints usually concern the deformity, including the following:
2. Thoracic kyphosis with secondary lumbar hyperlordosis. On Adams forward bending test the kyphosis has a sharp apex, distinct from the smooth curve seen in adolescent roundback.
3. Protuberant abdomen.
4. Forward rounded (protracted) shoulders.
5. Forward protrusion of the head.

These deformities are not completely corrected with forward bending or prone hyperextension maneuvers.

2. Pain: Aching painthat is usually localized to the apex of the thoracic kyphosis after prolonged sitting or exercise may be present (20%–70%). The incidence of pain increases as the kyphosis progresses. Those with a thoracolumbar kyphosis are more likely to have pain as a presenting complaint.
3. Scoliosis: There is an increased incidence of scoliosis(20%–30%), spondylolysis and spondylolisthesis (32%), and thoracic disc herniation.

Differential Diagnosis

In addition to adolescent roundback, there are a number of other processes that must be considered before diagnosing Scheuermann kyphosis.

1. Infectious spondylitis must be considered especially in immunocompromised patients, intravenous drug abusers, and those at risk for tuberculosis. Computed tomography and MRI are helpful in diagnosing and determining the extent of the disease.
2. Compression fractures can be confused with Scheuermann kyphosis from a radiographic perspective, but there is usually a history of trauma or a metabolic bone disease.
3. In congenital kyphosis type II, there are anterior bony bridges between consecutive vertebrae.
4. Juvenile ankylosing spondylitis is seen more frequently in males and is characterized by the loss of lumbar flexibility and painful enthesitis (at points of ligament or tendon insertion) of the feet or knees, or both. In addition, rheumatoid factor (RF) and antinuclear antibody (ANA) are normal. Approximately 90% of those with juvenile ankylosing spondylitis test positive for HLA-B27.
5. Other diagnoses include osteodystrophies (Morquio and Hurler disease), tumors, and postsurgical deformity.

Natural History

Most patients with <75 degrees of kyphosis have a benign progression of their disease with some deformity, back pain, and fatigue. When patients with Scheuermann kyphosis were compared with healthy age- and sex-matched controls, they were found to have more intense back pain, jobs that tended to have lower requirements for activity, less range of motion of extension of the trunk and less strong extension of the trunk, and different localization of the pain. No significant differences were noted between self-esteem, social limitations, and level of recreational activities or preoccupation with physical appearance (Murray et al., 1993).

Treatment

Treatment is controversial and not all patients require intervention. The three main treatment modalities for patients with Scheuermann kyphosis are exercise, bracing, and surgery.

1. Exercise programs to strengthen the low back and improve hamstring and pectoral flexibility may be used for patients with smaller curves (<50 degrees). These programs have not been shown to improve vertebral wedging or the degree of kyphosis. Comfort, and to some extent appearance, may improve with exercise.
2. Bracing treatment has been used for patients with >50 degrees of kyphosis resulting in some short-term improvement. However, most of the improvement is lost during long-term follow-up out of the brace. The Milwaukee brace (which includes the cervical ring) has been recommended for thoracic kyphosis at or above T7. An underarm orthosis (TLSO) can be used for those with lower thoracic or thoracolumbar kyphosis.
3. Operative correction (spinal fusion) is rarely indicated. It is indicated for adolescents with curves >80 degrees that cannot be controlled with brace treatment or for those with chronic pain and curves >70 degrees.

Web Sites

For Teenagers and Parents

http://www.scoliosis-assoc.org/. Scoliosis Association, Inc., an international information and support organization.

http://www.scoliosis.org/. National Scoliosis Foundation. Patient advocacy and information.

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http://orthoinfo.aaos.org/fact/thr_report.cfm?Thread_ID=262&topcategory=Children and http://orthoinfo.aaos.org/brochure/thr_report.cfm?Thread_ID=14&topcategory=Spine. American Association of Orthopedic Surgeons brochure on Childhood Scoliosis.

http://www.niams.nih.gov/hi/topics/scoliosis/scochild.htm. National Institute of Arthritis and Musculoskeletal and Skin Diseases frequently asked questions about scoliosis.

http://www.mayoclinic.com/health/scoliosis/DS00194. Mayo Clinic information on Scoliosis.

For Health Professionals and Patients

http://www.srs.org. The Scoliosis Research Society.

http://www.nlm.nih.gov/medlineplus/scoliosis.html. Medline plus health information.

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