Early onset scoliosis (EOS)

What is it?

Early onset scoliosis refers to spinal deformities, characterized by a curvature of the spine in the frontal plane > 10°, deformity arise before the age of 10 years. The most recent classification of EOS is based on 4 aspect of the deformity: etiology, major curve magnitude, kyphosis and the annual rate of progression. All these parameters have to be considered in the choice of the correct treatment.

From an etiologic point of view, EOS are divided into idiopathic, congenital, neuromuscular and syndromic.

Idiopathic EOS is defined as scoliosis without known cause, no direct inheritance has been observed, but there is a familiar recurrence. Infantile idiopathic EOS is diagnosed before 3 years, while Juvenile idiopathic EOS is diagnosed between 4 and 10 years. Prevalence is almost equal between male and female despite the major female prevalence in adolescent idiopathic scoliosis.

Congenital EOS is due to abnormalities in vertebral formation or segmentation that occur within the first 6 weeks of embryogenesis. Therefore, are usually associated to multi-organ malformation: more often cardiovascular, urogenital, musculoskeletal and intraspinal anomalies are found. Risk factors have been identified in intrauterine hypoxia, hyperthermia and exposure to carbon monoxide, valproic acid and ethanol.

Neuromuscular EOS are related to alterations in muscular tone as in cerebral palsy, muscular dystrophies, myopathies and spinal cord injuries. Frequently patient are unable to walk and spine deformity is usually associated to pelvic imbalance.

Syndromic EOS include spine deformities associated to any other syndrome such as Marfan Syndrome, Neurofibromatosis and Prader-Willi Syndrome. Generally, children are healthier than the ones affected by neuromuscular EOS and each syndrome has unique considerations.

Which are the symptoms?

The main concern in children affected by EOS is pulmonary function. The spine grows most rapidly in the first 5 years and from 10 years to adulthood. The progressive spinal deformity in EOS occurs in a critical time for lungs development and threats for thoracic insufficiency syndrome. In major curves, sign and symptoms are due vicious positions: skin lesions, pain, difficulties in sit and stand positions. 

  • Deformity
  • Respiratory disfuncion
  • Skin lesions
  • Rib hump
  • Coronal imbalance
  • Sagittal imbalance

How is it diagnosed?

Diagnosis is made at birth for congenital EOS. In idiopathic EOS parents or physicians notice asymmetry in the trunk during growth. Children affected by neuromuscular disease and syndromes have to be strictly monitored for the high probability of developing spine deformity.

Radiological assessment is necessary for the consequent treatment. X-rays is the standard tool for radiological assessment. Posterior-anterior and lateral plain X-rays are taken of the whole spine with the patient standing (sitting if necessary). Pelvis and hips need to be included to assess pelvic parameters and skeletal maturity. In addition, hand X ray is useful to assess skeletal maturity. CT scan is always performed in congenital EOS to have a clear shape of the vertebral malformations, but in not strictly necessary in all other EOS. MRI is advised in all patients, especially if undergoing surgery, to highlight Arnold-Chiari malformations, syringomyelia, tethered chord or any other spinal dysraphism.

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How is it treated?

Treatment goals are minimizing spinal deformity while maximizing the thoracic volume and pulmonary function. It is well known that an early fusion of thoracic spine prevent the complete lung development leading to respiratory insufficiency and consequently reduced life expectation. Treatments for EOS include conservative treatments and surgical interventions.

Conservative treatments include casting and bracing. Serial casting is very effective in EOS. Idiopathic EOS respond better to casting, sometimes achieving complete resolution of the spine deformity. In congenital and neuromuscular EOS casting is used as bridge treatment to minimize the evolution of the deformity until the thoracic volume is big enough to perform surgery safely. Bracing is less effective and mainly used in idiopathic EOS and adolescent idiopathic scoliosis.

Surgical treatments should be taken into consideration if the conservative treatment fails to achieve a proper correction of the deformity or to stabilize it.

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