Early Onset Scoliosis Consensus Statement

SRS Growing Spine Committee, June 2014

Early Onset Scoliosis (EOS) refers to spine deformity which is present before 8 – 10 years of age. EOS is further organized to reflect etiology as applicable:

Diagnostic Categories of EOS:

Idiopathic:

• Curves for which there is no apparent cause or related underlying etiology
  • Infantile idiopathic- a subgroup of idiopathic curves which develop in infants and children less than 3

Congenital:

• A particular type of EOS in which the vertebrae develop incorrectly in utero.
  • Congenital scoliosis is sometimes associated with cardiac and renal abnormalities. Evaluation may include studies of heart and kidneys.

Thoracogenic:

• A particular type of EOS that may be encountered in the following settings:
  • Multiple congenital rib fusions as seen in spondylocostal or spondylothoracic dysostosis, which may have congenital vertebral anomalies as well and may also be considered congenital scoliosis.
  • Changes in the chest wall following thoracic surgery which may function as a tether which promotes change in the shape of the spine.

Neuromuscular:

• Scoliosis which may develop in children with neuromuscular disorders including spinal muscular atrophy, cerebral palsy, spina bifida and brain or spinal cord injury.

Syndromic:

• Certain syndromes, such as Marfans, Ehlers-Danlos and other connective tissue disorders, as well as neurofibromatosis, Prader-Willi, and many bone dysplasias may be associated with EOS.

Prognosis of EOS:

• EOS, depending on the severity, may be associated with compromised pulmonary function in childhood which may also become increasingly problematic in adult years.
• When untreated, severe EOS may be associated with an increased risk of early death due to pulmonary compromise.
• The term Thoracic Insufficiency Syndrome (TIS) is commonly used to describe the potential combined spine and pulmonary problems in EOS. TIS is defined as "the inability of the thorax to support normal respiration or lung growth".
• Idiopathic EOS with a Cobb angle of >35 degrees is likely to progress.
• In many children <2 years old with infantile idiopathic curves <35 degrees, scoliosis may resolve without treatment.
• Prognosis will also depend on any underlying disorders or comorbidities.

Evaluation of EOS:

• Plain X-rays are sufficient to make the diagnosis of EOS.
• MRI may be indicated for curves that are larger than 20 degrees or progressive, or if there are signs or symptoms of neurologic problems and in persistent or progressive infantile idiopathic scoliosis. Intra-spinal abnormalities are commonly associated with EOS, particularly congenital scoliosis.
• CT best helps visualize bone anatomy in congenital scoliosis, and is often useful for surgical planning, but must be weighed against risk of radiation to young child.

Treatment Goals in EOS:

• Minimize spinal deformity over the life of the patient
• Maximize thoracic volume and function over the life of the patient
• Minimize the extent of any final spinal fusion, maximize motion of chest and spine
• Minimize complications, procedures, hospitalizations and burden for the family.
• Consider overall development of the child .

Treatment options in EOS may include:

Observation:

• The behavior of the curve may be monitored via repeated exam and radiographic evaluations at various time-points during development to look for worsening or progression of the scoliosis. Should the curve progress, or if the curve is larger, treatment may be appropriate:

Bracing or casting:

• Bracing or casting programs may help by allowing growth while minimizing curve progression. The need for surgery may be delayed and, in some instances such as infantile idiopathic scoliosis, surgery may be avoided. Anesthesia is usually required for casting children. Bracing or casting congenital scoliosis is rarely effective, but some believe bracing or casting a compensatory curve may be helpful.

Surgery:

• Various growth-friendly surgeries are designed to follow the principles of EOS treatment outlined above, allowing the spine and chest to grow while controlling spine and chest deformity. Surgery is generally recommended if brace or cast treatment should fail to control progression, or if curve pattern does not appear amenable to brace or cast treatment.
• Extensive thoracic spinal fusion in the very young child is associated with pulmonary compromise, and is rarely the best option.

Exercise:

• Manipulation, physical therapy and/or exercise has not been shown to influence spinal deformity in EOS