Clinical Review

Scoliosis Early Identification of Affected Patients

Clinician Reviews. 2012 August;22(8):16-22

Author(s):

Scoliosis encompasses a wide spectrum of orthopedic issues, from the simple (requiring watchful waiting) to the extreme (requiring multiple surgeries). Scoliosis represents a diagnostic challenge for the clinician, as it can be congenital, neuromuscular, syndrome-related, or idiopathic—as in 80% of cases. Adolescent idiopathic scoliosis, the most common form of this musculoskeletal diagnosis, is often painless and can be treated nonsurgically if it is identified early.

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References

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A majority of practitioners will at some time encounter scoliosis, the most common spinal deformity seen in pediatric practice.¹ This musculoskeletal condition can be found incidentally during routine exams, in the urgent care setting, in the emergency department (ED), or on chest x-rays or other radiographs. In primary care, scoliosis may be overlooked, particularly when the etiology is idiopathic or when well-child visits or routine back exams have been missed.¹

The focus of this review will be on early identification of affected patients (most commonly those with adolescent idiopathic scoliosis), with emphasis on evaluation through an excellent physical examination and ordering of appropriate radiographs. Watchful waiting and monitoring are the norm; however, implementing carefully selected treatment options, such as bracing, or making an appropriate referral may make it possible to slow or stop curve progression.^1-4

SCOLIOSIS: THE DEFINITION

Scoliosis is a coronal curvature (lateral deviation of the spine) of more than 10°, usually with a rotational component.^1,2 A healthy spine is straight in the frontal plane, but curved in the sagittal plane. Normal circumstances include thoracic kyphosis (outward curve of the upper back) and lumbar lordosis (inward curve of the lower back).⁵ The scoliotic spine deviates away from the midline, and maximal rotation is at the apex of the curve. The apex or point of the curve defines its center, that is, the most laterally deviated vertebra or disc. The first curve to develop is the primary curve; however, when a patient presents with two or three curves, it is difficult to determine which curve is primary.

The secondary curve is a compensatory curve, which develops as a means to balance the head and trunk over the pelvis. A similar compensation may occur in the sagittal plane, exacerbating the normal kyphosis or lordosis or possibly causing rib or scapular prominences.⁶ Cervical curves can also develop but are more commonly associated with conditions other than idiopathic scoliosis.³ Cervical scoliosis will not be reviewed here.

THE EPIDEMIOLOGY OF SCOLIOSIS TYPES

Eighty percent of all scoliosis patients are diagnosed with idiopathic scoliosis (which may be present in 2% to 3% of the population).^1,5 The remaining 20% are diagnosed with congenital, neuromuscular, or syndrome-related spinal deformities.¹ The overall prevalence of idiopathic scoliosis (with curves greater than 10°) is 1 to 3 per 100, with a comparable proportion of girls and boys. The prevalence of those with curves greater than 30° is 1 to 3 per 1,000, with a boy-girl ratio of 1:8.⁷ In the child who is skeletally mature, scoliosis curves measuring less than 30° usually do not progress. However, most curves greater than 50° tend to progress at about 1° per year.⁷

Idiopathic scoliosis is broken down into age ranges.⁸Infantile scoliosis is diagnosed from age 0 to 3 years and comprises 0.5% of cases; juvenile scoliosis, with an age range of 4 to 9 years, represents 10.5% of cases; and adolescent scoliosis, diagnosed any time after age 10, accounts for 89% of cases.⁷ The four main types of scoliosis will be discussed.

Congenital

Congenital scoliosis is defined as a malformed or segmented portion of the spine, a failure of formation of a portion of the spine, or a combination of the two.⁹ This results in block vertebrae or hemivertebrae, or possibly the fusing of two vertebrae with a bony bridge. Because the associated curves are often rigid, they can be resistant to correction. Worldwide, prevalence of congenital vertebral anomalies is 0.5 to 1.0 per 1,000 live births.¹⁰

Congenital scoliosis can be associated with many other congenital abnormalities, as embryonic development of the spine occurs at the same time as that of the heart, kidneys, bowel, and bladder.¹ Congenital scoliosis can occur with any of these defects or in the setting of a syndrome, such as VACTERL^1,9-11 (ie, vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal defects, and limb abnormalities¹; see Table 1^1,9,11,12). If even one such defect or abnormality is found, associated comorbid conditions should be actively sought.¹

In many instances, these abnormalities are noted when children are young; in children younger than 5 years, MRI is warranted, and these patients almost always need sedation during imaging. In the young child who also exhibits urologic issues, an MRI work-up for genitourinary defects can be performed at the same time.¹

Patients with abnormal cardiac findings (eg, heart murmur) should be referred for a cardiology work-up.^11,13 Chest wall deformities may also be present in children with congenital scoliosis. Multiple congenital rib fusions can cause chest wall constriction, possibly inhibiting growth and lung development. Untreated, this condition can ultimately shorten a child’s lifespan.¹⁴