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Part II

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Part II

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  1. Part II Amy L. McIntosh, MD Pediatric Orthopedic Surgeon Mayo Clinic Rochester, Minnesota

  2. Hip • Similar cross sectional study performed at the NIH Clinical Center • 51 patients • Hip pain • Radiographic analysis Rose, PS: JPO 2001

  3. Abnormal Morphology • Present in 1/3rd of patients • Took many forms

  4. Protrusio Acetabuli (10%) • 10% of patients • Abnormal deepening of the hip socket

  5. Abnormal Neck-Shaft Alignment (23%) • Most commonly “valgus” alignment • Increased angle • Fewer patients with “varus” alignment • Decreased angle • Seen in 23% of patients

  6. Arthritis

  7. Hip Arthritis

  8. Hip Arthritis

  9. Hip Symptoms • Pain data gathered on all patients over age 5 • Chronic hip pain present in 63% of all patients • Present in 79% of adults • Statistically associated with • Increasing arthritis on x-rays • Increasing age

  10. Legg Calve Perthes (16%) • Loss of the blood supply to the immature femoral head • Occurs during youth or adolescence • Treat = surgical

  11. Implications of Hip Abnormalities • Assist in identifying and diagnosing Stickler Syndrome • Recognizes the burden of progressive arthritis in patients • Patients and doctors should be alert for Perthes

  12. Other Orthopedic Manifestations • Clubfeet • Genu valgum (knocked- knees)

  13. Clubfeet (3%)

  14. Intrauterine Ultrasound Diagnosis

  15. Ponsetti Treatment

  16. Genu Valgum (25%)

  17. Growth Modulation

  18. Orthopedic Treatment Options • Spine • General joint arthritis

  19. Treatment Options for Arthritis • A large percentage of patients with Stickler Syndrome will develop symptomatic arthritis • Relatively young age • Multiple joints involved

  20. Principles of Treatment Lifestyle Modifications Medical Treatments Reconstructive Surgery

  21. Lifestyle Modifications Prevalence of Obesity in the United States

  22. Obesity • Each extra pound adds 4 pounds across the knee joint and 3 across the hip in regular activities • Average person takes 2000-4000 steps per day • So… one pound extra puts an extra 8-16 tons of stress on the knees each day!

  23. Activity Choices

  24. But… • In patients with Stickler Syndrome, healthy weight and sensible decisions will mitigate but not eliminate joint symptoms