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5- Congenital Disorders of Lumbar/Thoracic Spine Scoliosis in General 3D deformity of the spine in coronal, sagittal and axial planes Diagnosis based on a coronal plane curvature of >10 degrees using Cobb method Thoracic rotation: chest wall deformity or rib hump Lumbar rotation: prominence of the flank In Infants, children, adolescents Congenital: failure of segmentation or formation of some vertebral elements and may be associated with fused ribs and spinal cord abnormalities (flat back of head) High incidence of genitourinary abnormalities Neuromuscular: occurs in children who have muscular dystrophy, congenital myopathies, spina bifida, spinal cord injuries, and other congenital or traumatic neuromuscular conditions More rapid progression of curvature than idiopathic Idiopathic: Curvature without an identifiable cause in an otherwise normal child (multifactoral) Most common, more common in girls, back pain is rare, genetics? 3 Types based on onset: Infantile: less than 3yo Juvenile: 3-10 yo Adolescent: 11yo and older (late onset) Evaluation: History: pain (back or leg), bowel/bladder dysfunction, other neurological abnormalities Medical/Surgical Hx: congenital heart disease, thoracotomy, radiation, laminectomy Family Hx: scoliosis, Marfan’s, Neurofibromatosis Diagnosis: screen annually during health supervision visit from age 6 until the end of puberty Primary through physical exam when viewing from behind (asymmetry, Adam’s forward bend) Stigmata signs: shoulder asymmetry, unilateral scapular prominence, waist asymmetry Findings are subtle if the child is overweight or obese Scoliometer PA spine radiograph to assess coronal curvature and a lateral radiograph to assess sagittal alignment while standing (Cobb angle) 5- Congenital Disorders of Lumbar/Thoracic Spine Idiopathic Scoliosis Typical idiopathic thoracic curve has an apex to the right, lumbar curves have left apex Direction of the curve (right or left) is defined by convexity Curve types in idiopathic scoliosis: Associated physical exam: height, skin (freckles, café au lait spots or vascular hypopigmented lesions), excessive skin or joint laxity, examine for signs of leg length discrepancy, full neuro exam, pubertal maturation, patch of hair overlying the spine Cobb angle: formed by the intersection of a line parallel to the superior end plate of the most cephalad vertebra in a curve with the line parallel to the inferior endplate of most caudad vertebrae of the curve Look at the spine, determine the vertebrae with the most extreme angles (from the top part of the angle going down and the bottom of the vertebrae pointing up) Upper and lower vertebrae are the most tilted toward the concavity of the curve Treatment: refer to orthopedist if: Pt trunk rotation or rib prominence exceeds 5-7 degrees by scoliometer measurement 20 degree Cobb angle on radiograph in a growing child or adolescent Primary treatment for adolescent: observation, bracing, surgery Observation: Patients with Cobb angles of less than 20-25 degrees may be observed every 6-8 months If Cobb angle has progressed by greater than 5 degrees, treatment may be needed and refer Young children should be followed more frequently than older children due to increased growth Bracing: Indicated only if skeletally immature: Goal is to prevent or minimize curve progression (correction uncommon): Best if use 23 hrs a day Curves >30 degrees or curves 20-25 degrees that progressed > 5 degrees Ineffective if >45 Milwaukee Brace: Infantile or Juvenile scoliosis Boston brace: adolescent scoliosis (softer for day use) Charleston brace: corrective (curves to opposite side: use at night) 5- Congenital Disorders of Lumbar/Thoracic Spine Surgery: Indicated if: skeletally immature (>45 thoracic curve); skeletally mature (>50-55 thoracic) Spinal fusion: uses rods attached to spine by laminar hooks/wires (harder for anterior approach for large curve corrections) Pedicle screw: implanted through the pedicle posteriorly and then attached to rod 1 wk hospital, return school 1 mo, slowly increase exercise until full return contact at 6 mo Prognosis/Natural History: Untreated patients: Curves < 30º rarely progress after skeletal maturity Curves > 45 or 50º progress at 1º per year through life Curves > 80º → restrictive lung disease and dyspnea Curves > 100-120º → reduce life expectancy (cardiopulmonary failure) Treated patients: increased incidence of back pain, may still have some curve progression Psychosocial concerns: higher incidence of suicidal ideation, decreased perception of health status, eben after treatment Thoracic Insufficiency Syndrome (early onset scoliosis): Consequence of untreated, severe, early onset scoliosis Impaired and restricted lung growth and function (due to deformed thoracic cage) May affect the total space available for lungs/restrict thoracic cage function (restrictive lung dx) Treated with Vertical Expandable Prosthetic Titanium Rib (VEPTR): surgically implanted device to attach ribs and spine and separate ribs so that the lungs can fill with air Adjust as patient grows Screening: 33 states provide school screening Females screened twice at 10 and 12 Boys once at 13 or 14 Kyphosis Curvature of the spine in the sagittal plane Normal thoracic spine= 25-50 degree kyphosis Postural Kyphosis: corrected voluntarily in the standing/prone position Due to poor posture, asymptomatic with normal vertebrae Treat via reassurance and exercise Scheuermann Disease: relative decrease in collagen leads to the alteration in endplate ossification and altered vertebral body growth (WEDGING) Most common cause of fixed kyphotic deformity Unknown cause (multifactoral); males>females; age 13-16 Hx poor posture, thoracic curve, hyperlordosis of lumbar spine, hamstring tightness 20% of affected persons have pain in the kyphotic regions Pathology: Wedging (>5 degrees) of 3 or more consecutive vertebral bodies Narrow disc spaces, irregular disc surfaces (endplates), Schmorl nodules (vertical disc herniation) Treat: No treatment if mild/no pain and skeletally mature Hyperextension Exercise Program: mild deformity (skeletally immature) Bracing (Milwaukee Brace): Curves 50-60 Surgery (Spinal fusion): Curves 70-80; back pain; cosmetic dissatisfaction Structural Kyphosis Congenital Kyphosis: Failure of formation, failure of segmentation, mixed 5- Congenital Disorders of Lumbar/Thoracic Spine Key Points for Boards! AIS (Adolescent Idiopathic Scoliosis) Stigmata Signs of Scoliosis Adams Forward Bend Test Scoliometer use Cobb Angle (Method) Criteria for referral to orthopedist Types/indications of Scoliosis treatments Prognosis/Natural History of Idiopathic Scoliosis Scheuermann Disease Classic X-ray findings