Scoliosis

1. Scoliosis

2. Scoliosis • What is it? • How do we screen for it? • When to refer? • How is it treated?

4. What is scoliosis? • Lateral curvature of the spine >10º accompanied by vertebral rotation • Idiopathic scoliosis - Multigene dominant condition with variable phenotypic expression & no clear cause • Multiple causes exist for secondary scoliosis

5. Secondary causes for scoliosis:Inherited connective tissue disorders - Ehler’s Danlos syndrome - Marfan syndrome - Homocystinuria

6. Tethered cord syndrome Syringomyelia Spinal tumor Neurofibromatosis Muscular dystrophy Cerebral palsy Polio Friedeich’s ataxia Familial dysautonomia Werdnig-Hoffman disease Secondary causes for scoliosis:Neurologic disorders

7. Secondary causes for scoliosis:Musculoskeletal disorders • Leg length discrepancy • Developmental hip dysplasia • Osteogenesis imperfecta • Klippel-Feil syndrome

8. Characteristics of idiopathic scoliosis: • Present in 2 - 4% of kids aged 10 – 16 years • Ratio of girls to boys with small curves (<10º) is equal, but for curves >30º the ratio is 10:1 • Scoliosis tends to progress more often in girls (so girls with scoliosis are more likely to require treatment)

9. Natural history of scoliosis • Of adolescents diagnosed with scoliosis, only 10% have curve progression requiring medical intervention • Three main determinants of curve progression are: (1) Patient gender (2) Future growth potential (3) Curve magnitude at time of diagnosis

10. Assessing future growth potential using Tanner staging: Tanner stages 2-3 (just after onset of pubertal growth) are the stages of maximal scoliosis progression Natural history of scoliosis

11. Natural history of scoliosis Assessing growth potential using Risser grading: - Measures progress of bony fusion of iliac apophysis - Ranges from zero (no ossification) to 5 (complete bony fusion of the apophysis) - The lower the grade, the higher the potential for progression

12. Curve (degree) Growth potential (Rissergrade) Risk* 10 to 19 Limited (2 to 4) Low 10 to 19 High (0 to 1) Moderate 20 to 29 Limited (2 to 4) Low/mod 20 to 29 High (0 to 1) High >29 Limited (2 to 4) High >29 High (0 to 1) Very high . Risk of Curve Progression *—Low risk = 5 to 15 percent; moderate risk = 15 to 40 percent; high risk = 40 to 70 percent; very high risk = 70 to 90 percent.

13. Natural history of scoliosis • Back pain not significantly higher in pts with scoliosis • Curves in untreated adolescents with curves < 30 º at time of bony maturity are unlikely to progress • Curves >50 º at maturity progress 1º per year • Up to 19% of females with curves >40 º have significant psychological illness • Life-threatening effects on pulmonary function do not occur until curve is >100 º (ie: Cor pulmonale)

14. Scoliosis Screening • In years past, widespread school-based screening led to many unnecessary referrals of adolescents with minimal curvatures • U.S. Preventive Services Task Force notes “insufficient evidence” to recommend for or against routine screening of asymptomatic adolescents for idiopathic scoliosis

15. Scoliosis Screening Recommendations • American Academy of Orthopedic Surgeons - Screen girls at ages 11 and 13 - Screen boys once at age 13 or 14 • American Academy of Pediatrics - Screen at 10, 12, 14 and 16 years

16. Adam’s forward bend test For this test, the patient is asked to lean forward with his or her feet together and bend 90 degrees at the waist. The examiner can then easily view from this angle any asymmetry of the trunk or any abnormal spinal curvatures.

17. Screening hints: • Shoulders are different heights – one shoulder blade is more prominent than the other • Head is not centered directly above the pelvis • Appearance of a raised, prominent hip • Rib cages are at different heights • Uneven waist • Changes in look or texture of skin overlying the spine (dimples, hairy patches, color changes) • Leaning of entire body to one side

18. Scoliometer An inclinometer (Scoliometer) measures distortions of the torso. • The patient bends over, arms dangling and palms pressed together, until a curve can be observed in the upper back (thoracic area). • The Scoliometer is placed on the back and measures the apex (the highest point) of the upper back curve. • The patient continues bending until the curve can be seen in the lower back (lumbar area). The apex of this curve is also measured.

19. Red flags on PE: • Left-sided thoracic curvature • Pain • Significant stiffness • Abnormal neurologic findings • Stigmata of other clinical syndromes associated with curvature

20. Choose the most tilted verterbrae above & below apex of the curve. - Angle b/t intersecting lines drawn perpendicular to the top of the superior vertebrae and bottom of the inferior vertebrae is the Cobb angle. Measure spinal curvature using Cobb method:

21. Curve (degrees) Risser grade X-ray/refer Treatment 10 to 19 0 to 1 Every 6 months/no Observe 10 to 19 2 to 4 Every 6 months/no Observe 20 to 29 0 to 1 Every 6 months/yes Brace after 25 degrees 20 to 29 2 to 4 Every 6 months/yes Observe or brace* 29 to 40 0 to 1 Refer Brace 29 to 40 2 to 4 Refer Brace >40 0 to 4 Refer Surgery† Referral Guidelines & Treatment

22. Most common is Boston brace (aka Thoraco-lumbar-sacral orthosis) Braces have 74% success rate at halting curve progression (while worn) Bracing does not correct scoliosis, but may prevent serious progression Usually worn until patient reaches Risser grade 4 or 5 Brace Treatment for Scoliosis

23. Of patients with 20 º - 29 º curves, only 40% of those wearing braces ultimately required surgery, compared to 68% of those not wearing back braces Length of wearing time correlates with outcome (At least 16 hrs per day leads to best chance of preventing curve progression) Brace Treatment for Scoliosis

24. Surgical Treatment for Scoliosis • Curves in growing children greater than 40 º require a spinal fusion (Risser grade 0 to 1 in girls and Risser 2 or 3 in boys) • Skeletally mature patients can be observed until their curves reach 50 º • Posterior spinal fusion is best choice for thoracic curves • Anterior spinal fusion is best treatment for thoracolumbar and lumbar curves

25. Surgical Treatment for Scoliosis • Spinal surgery with instrumentation significantly corrects deformity & usually stops curve progression • Surgery is accompanied by spinal cord monitoring using somato- sensory & motor-evoked potentials (risk of neurologic injury is 1/7000)

26. Post-Op Treatment & Long Term Consequences of Spinal Fusion • If segmental instrumentation used, no post-op cast or brace required • Post-fusion back pain does occur and is more common in distal spinal fusions • Usually out of hospital in 4-5 days & back at school in 2 wks • OK to participate in athletics after 9 – 12 months (should avoid contact sports)

27. Case #1 MP is a 16-year-old male who presents to your office for his annual health assessment and sports physical. During the course of his examination, you note a mild convexity in the thoracic region of his spine with forward flexion at the hips. Based on your clinical examination, you estimate a lateral spinal curvature of about 5 degrees. You note these findings to the patient and then to his mother.

28. 1. 1. 1. Which one of the following procedures should be implemented next? Which one of the following procedures should be implemented next? • Which one of the following procedures should be • implemented next? • A. Recommend back-strengthening exercises. • B. Refuse to permit participation in contact sports. • Order a radiograph of the back to quantify the • curvature (e.g., Cobb angle). • D. Monitor the patient's condition. • E. Refer for orthopedic consultation. A. A. Recommend a back-strengthening program. Recommend a back-strengthening program. B. B. Refuse to permit participation in contact sports. Refuse to permit participation in contact sports. C. C. Order a radiograph of the back to quantify the curvature (e.g., Cobb angle). Order a radiograph of the back to quantify the curvature (e.g., Cobb angle). D. D. Monitor the patient's condition. Monitor the patient's condition. E. E. Refer for orthopedic consultation. Refer for orthopedic consultation. Question 1

29. Answer 1 The answer is D: monitor the patient's condition.

30. Question 2 Because you have recently agreed to serve as school physician in the district where your office is located, you wonder what scoliosis screening programs are in place and who has been examining these school children for scoliosis. Which one of the following procedures should you implement?

31. Question 2 (cont.) • Arrange scoliosis screening for all students between 10 and 16 years of age. • B. Arrange scoliosis screening for all students 10, 12 , 14 and 16 years of age. • C. Contact the school nurse and review skills for scoliosis screening procedures. • Visually inspect for severe curves only when the back is examined for other reasons. • Screen girls for scoliosis at 11 and 13 years of age and boys at 13 and 15 years of age.

32. Answer 2 • According to AAP the answer is B: screen at 10, 12, 14 & • 16 years • According to U.S. Prev Services Task Force, the answer is D: • visually inspect for severe curves only when the back is • examined for other reasons.

33. Question 3 Which of the following statement(s) about treatment for adolescent scoliosis is/are correct? • Exercise therapy has been shown to be an effective treatment for preventing progression of scoliosis. • B. Spinal surgery for scoliosis is not supported by studies showing improvements in clinical outcomes, such as decreased back pain and increased functional status. • C. Lateral electrical surface stimulation for eight hours nightly can limit progression of spinal curvature • D. Back bracing (e.g., orthoses) reduces symptoms of low back pain.

34. Answer 3 The answer is B: Although surgery for scoliosis is generally not recommended without marked curvature, well-conducted outcomes studies with patients who have had surgery have not been completed. Symptoms of back pain do not appear to correlate with magnitude of surgical correction.

35. Conclusions • Screening for scoliosis remains controversial & has led to many unnecessary referrals • Adolescent scoliosis can be followed by family docs if the curve has a low risk of progression & underlying causes have been excluded • Curves demonstrating significant progression with continued growth remaining or those at high risk of progression should be referred for orthopedic evaluation • Always refer when red flags are present on PE or X-ray

36. Conclusions • 90% of kids with scoliosis will not require medical intervention • Girls are much more likely than boys to need intervention for scoliosis • Bracing can slow progression of many curves and significantly decrease need for surgery • Spinal fusion surgery is recommended for curves greater than 45 – 50 degrees

37. Torticollis

38. What is it? • Also known as Wryneck • Head and chin are tilted at opposite angles, causing head to twist • Asymmetrical Appearance • Effected muscle:sternocleidomastoid

40. What is it? • Can exist before or at birth • Congenital Muscular Torticollis • Can occur during childhood up through adult age • Acquired/Noncongenital Muscular Torticollis • Both cause asymmetrical appearance and function in the neck and head of those afflicted

42. Prevalence • Less than .4% of newborns • Torticollis does not prefer one side of head or the other • In CMT, ratio of boys to girls is 3:2 • Increased head size in male babies

43. Prevalence • In adults, noncongenital muscular torticollis has an average onset of 40 years old • Females twice as likely afflicted than males • Usually equal distribution between right and left side of body afflicted • Slightly more right torticollis in older female populations

44. Causes? • Not well understood • Almost 80 entities have been reported to cause torticollis • Common causes: • Developmental disorders affecting sternocleidomastoid muscle • Imbalance in function of cervical muscles • Other abnormalities in skull/cervical area

45. Other Causes • Genetic defect • Infants position during pregnancy or delivery • Tumors in head or neck • Arthritis of neck • Pseudotumors in infants • Certain medications • Genes • More likely to be afflicted if family member had torticollis or similar disorder

46. Symptoms • Adults and Children: • Abnormal contraction of the neck • Limited range of motion • Stiff neck muscles • Possible swelling and pain • Can often be mistaken for more serious condition • See medical professional immediately

47. Symptoms • Infants: • Tilting of chin • Small mass (pseudotumor) in neck • Small neck spasms • Diagnosed before 1 month old = shorter physical therapy

48. Prognosis • Most helpful diagnosis is made early • Not life threatening • May self correct itself • May be chronic and reoccurring • Any complications may result from compressed nerve roots

49. Treatments • Stretching and lengthening affected neck muscles • Applying heat, massage, analgesics • Can be combined with TENS • Transcutaneous Electrical Nerve Stimulation • Medical treatment—Bacolfen or Botox • Injection every three months

50. Treatments • Surgery in severe cases • Patients whose pathology does not resolve after 12 months of physical therapy or who develops facial asymmetry • Risk of injury to spinal nerves