Current Concepts in the Management of Early-Onset Idiopathic Scoliosis

Abstract and Introduction

Abstract

Early-onset scoliosis is a diagnosis when a child is presenting with scoliosis before the age of 5 years. This excludes other causes of scoliosis (e.g., congenital, neuromuscular or syndromic). Twin studies and observations of familial aggregation reveal significant genetic contributions to idiopathic scoliosis. Radiographic criteria help in distinguishing the progressive curves from those that will resolve spontaneously. One must do a complete clinical evaluation to exclude other organ involvement especially congenital heart disease, inguinal hernia and hip dysplasia. MRI scans of the neural axis are mandatory in curves greater than 20° at presentation to rule out any occult lesions in the CNS. Minor nonprogressive curves can be managed with observation until growth is completed. Some curves may be managed with casting and bracing. There is increased risk of morbidity and mortality due to respiratory failure in untreated children with early-onset scoliosis who have progressive curves. Therefore, progressive curves must be addressed surgically. Surgical procedures continue to evolve and are primarily directed at correcting and maintaining the curve correction while simultaneously preserving spinal and trunk growth. A definitive spinal fusion is indicated once the thoracic spinal growth is nearing completion.

Introduction

Nearly 80 years ago, Harrenstein first described early-onset idiopathic scoliosis.^[1] In 1951, James described at the first two types of curve patterns in structural idiopathic scoliosis.^[2] In 1954, James divided idiopathic scoliosis into three types according to the age of onset: infantile (birth to 3 years), juvenile (4–9 years) and adolescent (10 years to end of growth).^[3] These three periods correspond to the phases of increased growth velocity during childhood and adolescence, but it has been shown that growth velocity corresponds only to infancy and adolescence.^[4] During the traditional juvenile onset period, the spinal growth decelerates and therefore juvenile-onset scoliosis is rare. It was not until recently that Dickson described the term 'early onset', which develops before the age of 5 years.^[5] This is due to the fact that there is a real risk of cardiopulmonary compromise if there is a substantial thoracic deformity present before the age of 5 years.^[6] In this review, early-onset scoliosis (EOS) refers to all idiopathic scoliosis of more than 10˚ spinal curvature, which develop in children below the age of 5 years.

Cite this: Current Concepts in the Management of Early-onset Idiopathic Scoliosis - Medscape - Jun 01, 2010.

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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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Current Concepts in the Management of Early-onset Idiopathic Scoliosis

Abstract and Introduction

Abstract

Introduction

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Authors and Disclosures

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