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. Epidemiology 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