Current Concepts in the Management of Early-onset Idiopathic Scoliosis

Ujjwal K Debnath

Pediatr Health. 2010;4(3):343-354.

Epidemiology

The incidence of EOS is approximately four-times higher in Europe compared with USA (1–2 per 10000 people). The Edinburgh survey by McMaster demonstrated an incidence of 50% of all patients with idiopathic scoliosis had infantile curves.^[7] By contrast, a survey from Boston with a population from a similar genetic stock showed an incidence of 0.5%.^[8] Scott and Morgan compared the incidence between England and USA and found the incidence of scoliosis before the age of 2 years was significantly higher in England.^[9] This difference led to speculations regarding the presence of a triggering factor in the environment that may be beneficial to babies in the USA.

The majority of the curves (75–90%) tend to be left-sided in contrast to the right-sided curve patterns in late-onset idiopathic curves. Males account for 60% of the cases of EOS; 10% of cases progress to a severe and disabling condition.^[10] The differences in gender and curve lateralization do not make any difference to the management.

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Executive Summary

Definition of early-onset scoliosis

Idiopathic scoliosis occurring before the age of 5 years without any associated congenital vertebral or rib anomalies.

Epidemiology

The incidence is approximately one or two per 10,000 people. The resolving type curves occur in 70–90% children.

Etiology

Little is known regarding etiology, particularly with regard to genetic defects. Further research in genetic engineering is necessary to understanding the factors for its development and progression.

Clinical manifestation

Left-sided thoracic curves are more common as opposed to adolescent idiopathic curves. Associated findings may include plagiocaphaly, hip dysplasia, inguinal hernia and congenital heart defects. Gradual progression occurs at 2–3° per year and malignant progression occurs at 10° per year. Curve resolution occurs in 90% children who are diagnosed before the age of 1 year.

Diagnosis

Diagnosis is confirmed by history, clinical and radiological examination. Plain radiography of whole spine (anterior–posterior and lateral) is followed by MRI scan. Mehta developed a method for differentiating resolving form of progressive curves in early-onset scoliosis (EOS) based on measurement of rib–vertebral angle difference, which denotes the difference of values of rib–vertebral angle on the concave and convex sides.

Treatment

Non-operative methods range from orthotics to casting to traction; however, in certain circumstances, these techniques cannot effectively prevent deformity progression or are not tolerated by the child and surgical methods are required. Current options for surgical management of spinal deformity in the growing child include definitive spinal fusion with or without instrumentation, selective fusion, growth modulation, spinal instrumentation without fusion, or more recently, dual growing rods and the use of the vertical expandable prosthetic titanium rib.

Conclusion

Progressive EOS in the growing child poses a unique challenge. The surgeon aims to attain maximal curve correction while maintaining spinal and thoracic growth, and preventing the morbidity and mortality from respiratory problems. Non-operative treatments include bracing and serial casting. The classic surgical treatment has been spine fusion with less than optimal results. This has resulted in the development of fusionless interventions for children with EOS. These include growing rods, intervertebral body stapling and the vertical expandable prosthetic titanium rib. Each of these offers unique advantages and disadvantages.

Future perspective

The etiology and treatment considerations are different for EOS. The future research towards the understanding of the disease process (genetic studies), natural history, spinal growth and pulmonary development, treatment (fusionless devices) and outcome measurement tools could serve these unfortunate children with a better quality of life.

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