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Juvenile Idiopathic Scoliosis

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

 

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