Summary
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Juvenile Idiopathic Scoliosis is a coronal plane spinal deformity which most commonly presents in children between ages 4 and 10.
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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°.
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Treatment can be observation, bracing, or surgical management depending on the skeletal maturity of the patient, magnitude of deformity, and curve progression.
Epidemology
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Incidence
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15% of all idiopathic scoliosis cases
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Demographics
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females > males
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Anatomic location
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most commonly appear as a right main thoracic curve
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Etiology
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Associated conditions
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high incidence of neural axis abnormalities (18-25%)
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syringomyelia
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cyst or tubular cavity within spinal cord
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can be seen in a scoliotic curve without rotation
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can manifest as an asymmetric umbilicus reflex
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Arnold-Chiari syndrome
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cerebellar tonsil are elongated and protruding through the opening of the base of the skull and blocking CSF flow)
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tethered cord
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dysraphism
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spinal cord tumor
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Classification
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Early onset scoliosis (EOS)
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early-onset scoliosis is a broader category including scoliosis in children <10 years old. It includes
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infantile idiopathic scoliosis
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juvenile idiopathic scoliosis
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congenital scoliosis
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neurogenic scoliosis
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syndromic scoliosis
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Marfan's
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Down's syndrome
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Presentation
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History
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important to determine when deformity was first noticed and any observed progression
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get perinatal history
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Presentation
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failure to develop bowel and bladder control by age ~ 3 or 4 may indicate neurologic involvement
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patients often referred from school screening where a 7° curve on scoliometer during Adams forward bending test is considered abnormal
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7° correlates with 20° coronal plane curve
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Physical exam
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general inspection
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cafe-au-lait spots (neurofibromatosis)
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leg length inequality
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shoulder height differences
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truncal shift
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waist asymmetry and pelvic tilt
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foot deformities (cavovarus)
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can suggest neural axis abnormalities and warrant a MRI
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spine inspection
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midline skin defects
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hairy patches
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dimples (signs of spinal dysraphism)
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nevi
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rib rotational deformity (rib prominence)
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Adams forward bending test
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axial plane deformity indicates structural curve
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forward bending sitting test
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can eliminate leg length inequality as cause of scoliosis
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neurologic
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motor
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upper and lower extremities exam
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reflexes
- abnormal abdominal reflexes
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associated with the presence of a syrinx
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gently stroking each abdominal quadrant should cause contraction of the abdominal muscles
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clonus
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Hoffman sign
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Babinski
- abnormal abdominal reflexes
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gait analysis
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Imaging
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Radiographs
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PA and lateral upright images are used to assess curve severity
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treatment based on Cobb angle
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Cobb angle
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> 10° defined as scoliosis
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intra-interobserver error of 3-5°
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bending radiographs can help determine which curves require fusion
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- MRI
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indicated in children <10 years old with a curve > 20°
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even in the absence of neurologic symptoms
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must rule out neural axis abnormalities (e.g., syringomyelia)
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presence of left-sided thoracic curve
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Treatment
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Nonoperative
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observation
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indications
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curves < 20°
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technique
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frequent radiographs to observe for curve progression
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bracing
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indications
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curves 20 - 50°
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designed to prevent curve progression, not correct the curve
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relative contraindication to bracing is thoracic hypokyphosis
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technique
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16-23h/day until skeletal growth completed or surgery indicated
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Operative
- non-fusion procedures (growing rods, VEPTR)
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indications
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curves > 50° in small children with significant growth remaining
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allows continued spinal growth over unfused segments
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definitive PSF + ASF performed when the child has grown and is closer skeletal maturity
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- traditional growing rods associated with greater curve correction and truncal height gain than VEPTR constructs
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anterior / posterior spinal fusion
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indications
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curves > 50° in younger patients
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required in order to prevent crankshaft phenomenon
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posterior spinal fusion
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indications
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curve > 50° in older patients near skeletal maturity
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remains gold standard for thoracic and double major curves (most cases)
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anterior spinal fusion
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indications
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curve > 50°
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best for thoracolumbar and lumbar cases with a normal sagittal profile
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- non-fusion procedures (growing rods, VEPTR)
Complications
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Crankshaft phenomenon
Prognosis
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High risk of progression
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70% require treatment (50% bracing, 50% surgery)
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Very few experience spontaneous resolution
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Can be fatal if not treated appropriately