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Early Onset Scoliosis

The Scoliosis Research Society has published a consensus statement on early onset scoliosis
(Spine Deformity  3 (2015) p.107):

Early Onset Scoliosis refers to spine deformity that is present before 10 years of age.


Download the Early Onset Scoliosis Frequently Asked Questions Brochure (PDF -1.16 MB)

Different causes:

  • Idiopathic - Curves for which there is no apparent cause.
  • Congenital - Vertebrae develop incorrectly inutero. It is sometimes associated with cardiac and renal abnormalities. Evaluation may include studies of heart and kidneys.
  • Neuromuscular - 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

EOS, depending on the severity, may be associated with heart and lung problems in childhood which may become increasingly problematic in adult years. When untreated, severe EOS may be associated with an increased risk of early death due to heart and lung disease. Prognosis will also depend on any underlying disorders or comorbidities. Thoracic Insufficiency Syndrome (TIS) is commonly used to describe the potential combined spine and lung problems in EOS.

  • Likely to progress: Idiopathic EOS with curves greater than 30-35 degrees
  • Likely to resolve without treatment: Children younger than age 2 with infantile idiopathic curves less than 35 degrees

Evaluation

  • Plain X-rays are sufficient to make the diagnosis of EOS.
  • MRI may be indicated for certain patients with EOS.
  • 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 a young child.

Treatment Goals

  • Minimize spinal deformity over the life of the patient.
  • Maximize lung 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

Observation

The behavior of the curve may be monitored via repeated clinic visits and x-ray examinations at various times during development for worsening or progression of the scoliosis. Should the curve progress, or if the curve is larger, treatment may be appropriate.

Bracing / Casting

Bracing or casting programs may help by allowing growth while minimizing increases in the scoliosis. The need for surgery may be delayed and, in some instances, avoided. Bracing or casting congenital scoliosis is rarely effective, but some believe bracing or casting a compensatory curve may be helpful.

  • Bracing is prescribed depending on the flexibility of the curve, as determined by the bending or traction x-rays. If the curve is rigid and does not correct (get smaller) on the bending x-rays, a brace will do little good. Rarely does a brace permanently correct scoliosis, instead the goal of bracing is to allow the child to grow before a surgical procedure is done. The purpose of the brace is to slow the inevitable progression of the curve, not to correct the curve.
  • Anesthesia is usually required for casting children. Casting has been shown to correct curvature in some cases, especially in young children and those with smaller curvatures. Casting has been shown to delay the progression of curvature and need for more invasive surgery in many types of EOS.
  • Manipulation, physical therapy and/or exercise has not been shown to influence spinal deformity in EOS patients.

Patients treated with scoliosis casting. Holes are cut along the chest and abdomen to allow for normal breathing and eating. Shoulder straps are optional.

Surgery

Surgery is generally recommended if brace or cast treatment should fail to keep the scoliosis from progressing, or if the curve pattern does not appear amenable to brace or cast treatment.

The dilemma faced by the surgeon is how to stop the progression of a curve without adversely affecting future growth. Various growth-friendly surgeries are designed to follow the principles of EOS treatment outlined earlier, allowing the spine and lungs to grow while controlling spine and lung deformity. Generally, this type of surgery may be divided classified as Distraction-Based, Guided Growth, and Compression Based.

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