Summary

• Juvenile Idiopathic Scoliosis is a coronal plane spinal deformity which most commonly presents in children between ages 4 and 10.

• Diagnosis is made with full-length standing PA and lateral spine radiographs. MRI studies are indicated in children <10 years old with a curve > 20°.

• Treatment can be observation, bracing, or surgical management depending on the skeletal maturity of the patient, magnitude of deformity, and curve progression.

Epidemology

• Incidence

  • 15% of all idiopathic scoliosis cases

• Demographics

  • females > males

• Anatomic location

  • most commonly appear as a right main thoracic curve

Etiology

• Associated conditions

  • high incidence of neural axis abnormalities (18-25%)

    • syringomyelia

      • cyst or tubular cavity within spinal cord 

      • can be seen in a scoliotic curve without rotation

      • can manifest as an asymmetric umbilicus reflex

    • Arnold-Chiari syndrome

      • cerebellar tonsil are elongated and protruding through the opening of the base of the skull and blocking CSF flow) 

    • tethered cord

    • dysraphism

    • spinal cord tumor

Classification

• • • • Early onset scoliosis (EOS)

        • early-onset scoliosis is a broader category including scoliosis in children <10 years old. It includes

          • infantile idiopathic scoliosis 

          • juvenile idiopathic scoliosis 

          • congenital scoliosis 

          • neurogenic scoliosis 

          • syndromic scoliosis

            • Marfan's 

            • Down's syndrome 

Presentation

• History

  • important to determine when deformity was first noticed and any observed progression

  • get perinatal history

• Presentation

  • failure to develop bowel and bladder control by age ~ 3 or 4 may indicate neurologic involvement

  • patients often referred from school screening where a 7° curve on scoliometer during Adams forward bending test is considered abnormal

    • 7° correlates with 20° coronal plane curve

• Physical exam

  • general inspection

    • cafe-au-lait spots (neurofibromatosis)

    • leg length inequality 

    • shoulder height differences

    • truncal shift

    • waist asymmetry and pelvic tilt

    • foot deformities (cavovarus)

      • can suggest neural axis abnormalities and warrant a MRI

  • spine inspection

    • midline skin defects

      • hairy patches

      • dimples (signs of spinal dysraphism)

      • nevi

    • rib rotational deformity (rib prominence)

    • Adams forward bending test 

      • axial plane deformity indicates structural curve

    • forward bending sitting test

      • can eliminate leg length inequality as cause of scoliosis

  • neurologic

    • motor

      • upper and lower extremities exam

    • reflexes

      • abnormal abdominal reflexes 

         

        • associated with the presence of a syrinx

        • gently stroking each abdominal quadrant should cause contraction of the abdominal muscles

      • clonus

      • Hoffman sign

      • Babinski

  • gait analysis

Imaging

• Radiographs

  • PA and lateral upright images are used to assess curve severity

    • treatment based on Cobb angle

  • Cobb angle

    • > 10° defined as scoliosis

    • intra-interobserver error of 3-5°

    • bending radiographs can help determine which curves require fusion

• MRI 

   

  • indicated in children <10 years old with a curve > 20°

    • even in the absence of neurologic symptoms

    • must rule out neural axis abnormalities (e.g., syringomyelia)

    • presence of left-sided thoracic curve 

Treatment

• Nonoperative

  • observation

    • indications

      • curves < 20°

    • technique

      • frequent radiographs to observe for curve progression

  • bracing

    • indications

      • curves 20 - 50°

      • designed to prevent curve progression, not correct the curve

      • relative contraindication to bracing is thoracic hypokyphosis

    • technique

      • 16-23h/day until skeletal growth completed or surgery indicated

• Operative

  • non-fusion procedures (growing rods, VEPTR) 

     

    • indications

      • curves > 50° in small children with significant growth remaining

        • allows continued spinal growth over unfused segments

        • definitive PSF + ASF performed when the child has grown and is closer skeletal maturity

    • traditional growing rods associated with greater curve correction and truncal height gain than VEPTR constructs  

       

  • anterior / posterior spinal fusion

    • indications

      • curves > 50° in younger patients

      • required in order to prevent crankshaft phenomenon

  • posterior spinal fusion

    • indications

      • curve > 50° in older patients near skeletal maturity

      • remains gold standard for thoracic and double major curves (most cases)

  • anterior spinal fusion

    • indications

      • curve > 50°

      • best for thoracolumbar and lumbar cases with a normal sagittal profile

Complications

• Crankshaft phenomenon

Prognosis

• High risk of progression

  • 70% require treatment (50% bracing, 50% surgery)

• Very few experience spontaneous resolution

• Can be fatal if not treated appropriately