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Legg-Calve-Perthes Disease Guide
- 1. Legg-Calve-Perthes Disease Dr. Hafiz Zayn Zafar
- 2. INTRODUCTION It is a condition described by three different people independently in 1910 and their names were retained in the name of the disease. It is a painful disorder of childhood characterized by avascular necrosis of femoral head
- 3. EPIDEMIOLOGY It is a rare condition Incidence is 1 in 10,000. Higher in Japan and Central Europe Age group is 4-10 years old and boys are affected 4 times as often as girls
- 4. PATHOGENESIS Cardinal feature is ischemia of femoral head Upto the age of 4 months, femoral head is supplied by metaphyseal vessels which penetrate growth disc, lateral epiphyseal vessels running in retinacula and scanty vessels in ligamentum teres Metaphyseal supply disappers at age of 4 years but ligamentum teres vessels develop at age of 7 years Between the age of 4 and 7, sole supply is by lateral epiphyseal vessels which are susceptible to stretch and pressure, therefore causing rise in intraosseus pressure and consequent ischemia
- 6. PATHOLOGY Stage 1: Ischemia and bone death Stage 2: Revascularization and Repair Stage 3: Distortion and Remodeling
- 7. CLINICAL FEATURES Age group 4-8 years, commonly a boy Pain in groin and limping walk All hip movements may be painful but abduction in flexion and internal rotation nearly always limited May be associated with urogenital anomaly
- 8. RADIOLOGY Initially only widening of joint space may be seen Classical feature is increased density of ossific nucleus in epiphysis Abnormal healing may cause femoral head to be mushroom shaped with lateral displacement from acetabulum
- 11. PROGNOSIS Age < 6 years is good prognostic feature Boys have better prognosis than girls The greater the femoral head involved, worse the prognosis. <50% involvement is good Herring group A and B have better prognosis than Herring group C
- 12. HEAD AT RISK SIGNS Progressive uncovering of epiphysis Cartilage calcification lateral to ossific nucelus Radiolucent area at lateral edge of epiphysis (Gage) Severe metaphyseal resorption
- 13. GAGE SIGN
- 14. MANAGEMENT Education and counselling of parents Discourage wheelchair and crutch walking. Avoid sports and strenuous activity Encourage cycling and swimming Hip Strengthening and ROM Exercises Symptomatic treatment, containment of head in acetabulum and operative reconstruction
- 16. HERRING GUIDELINES < 6y Symptomatic Treatment 6-8 y Bone age < 6 Herring A and B Symptomatic Treatment Herring C Abduction Brace Bone age >6 Herring A and B Abduction Brace or Surgery Herring C Surgery 9 y and above Surgery
- 17. SURGERY Varus Osteotomy of Femur Innominate Osteotomy of Pelvis Both in some cases
- 18. DIFFERENTIALS Cretinism Epiphyseal Dysplasia Sickle Cell Disease Transient Synovitis