Perthes disease,an elaborate lecture.pptx

Legg calves perthes disease………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………………………………….. ……………………………………………………………………………………………………

1. Dr SALIL IBRAHIM JR2. Dept of ORTHOPEDICS SBHGMC DHULE LEGG CALVE PERTHES DISEASE

2. Definition Legg calves perthes disease is a condition in which an avascular events affect the capital epiphysis of the femur

3. Incidence 1 in 10000 children B/L in 10-15% of cases Demographics Mc age of presentation:4-8 yrs Male to female ratio:5:1 High incidence in Lower Socioeconomic class

4. Etiology The Etiology of Legg calves perthes disease is probably multifactorial with the exact cause still uncertain Factors that may be Etiologic are 1)Trauma 2)Susceptible child 3)Hereditary factors 4)coagulopathy 5)Hyperactivity 6) Passive smoking

5. Pathology Pathological findings of LCPD(Waldenstorm classification) 1)Initial stage 2) Fragmentation stage 3) Revascularization stage/healing stage 4)Residual stage/healed stage

6. Pathogenesis of LCPD

7. Early stage Dead ,collapsed Trabecular bone Small,radiodense ossific nucleus->mild Flattening Thickened articular cartilage Physeal disruption Widening of medial joint space (waldenstorm sign) Subchondral fracture (crescent sign) Cartilage extending from the physis into the metaphysis,metaphyseal cyst

8. Fragmentation stage Invasion of vascular granulation tissue New bone forming on old trabeculae Woven new bone formations Further flattening of ossific nucleus Further lateralisation of the head Radiolucency of ossific nucleus

9. Healing stage Appearance of New bone in the periphery of the femoral head Return to the normal architecture Central and anterior aspect of the head are last to reossify Healed stage Normal radio density of the femoral head Shape of the femoral head may change until skeletal maturity

10. Clinical features Symptoms Limping Hip pain Knee pain History of trauma (?)

11. Signs Limp Decreased Hip Range of motion Spasm with log-roll

12. Clinical course of the disease Time from 1st radiographic evidence of disease to the start of fragmentation stage ranging from 1-14 months Fragmentation phase lasts ranging from 2-35 months Healing stage occupies 2-122 months

13. Association between clinical findings and radiographic stages of the disease

14. Natural history of Legg Calves Perthes Disease Disease severity - Varies from mild to severe, with most children experiencing moderate symptoms for 12-18 months,Followed by complete resolution of symptoms and a return to normal physical activities Patients age -Most consistent factor affecting course of the disease, usually those with Early onset (before 6 years of age)have mild disease Those with onset from 6-9 years have moderate symptoms and those with onset above 9 years of age have most severe course and worst outcome

15. Extent of radiographic changes Varies but poorest outcome seen in those hips with greatest degree of involvement Outcome affected by duration from onset of disease to complete resolution,shorter the duration better the final results

16. Radiographic finding Initial stage AP radiograph at the onset of disorder shows increased density in the femoral head and apparent widening of the joint space

17. Fragmentation stage AP radiograph obtained 9 months after the onset showing head entering into fragmentation stage.the central fragment remains dense and has collapsed relative to the lateral portion

18. Healing stage AP radiograph of 17 months after the onset showing early reossification of the femoral head

19. 22 months after the onset of disease .still widening of the joint space and acetabulum has a bicompartmental appearance

20. Healed stage Radiograph obtained 4years after the onset.femoral head is healed and in the residual state.still widening of joint space+,incongruity of the head with the acetabulum

21. AP radiograph 6 years after onset of disease,shows improved roundness of the femoral head and better joint congruity

22. Other radiographic findings Changes in metaphysis ‘Sagging rope sign’ is a radiodense line overlying the proximal femoral metaphysis It is a result of growth plate damage associated with significant metaphyseal response

23. Physis changes Patients of LCPD often experience Abnormal growth of the proximal femoral physis Premature physeal closure have been seen in 1/4th of the pts with LCPD Early closure is due to GT overgrowth,physeal shape changes,lateral extrusion of the capital nucleus and medial bowing of the femoral neck

24. Radiograph showing Trochanteric over growth and short femoral neck

25. Acetabular changes In most cases if there is change in femoral head ,accompanying changes are seen in acetabulum When the femoral head protrudes from the acetabulum ,the medial wall may form what looks like a second compartment for the head referred to as Bicompartmentalization Appearing as early as 3months from the onset of LCPD Bicompartmentalization usually resolves in the healing stage of LCPD

26. MRI imaging MRI is an accurate imaging modality for the early diagnosis of Legg calves perthes disease Varying modalities of MRI offers new insights into the pathophysiology of the disease MRI has also been reported to provide earlier and more reliable information about the true extent of femoral head necrosis than radiography or scintigraphy Sequential MRI studies of Legg-Calvé-Perthes disease have established a correlation with the Catterall classification system MRI can also provide better delineation of affected sites and the degree of involvement during the early phases of the disorder

27. MRI imaging of Legg Calves Perthes disease,Distorted shape of the head of femur is evident

28. MRI image of a 9-year-old boy with Legg-Calvé-Perthes disease showing extensive involvement of the left hip

29. Scintigraphy Technetium scanning is an effective means of diagnosing Legg-Calvé-Perthes disease in its early stages, before associated radiographic findings are apparent Scintigraphy has also been used to classify the severity of the disease, with grade I representing one-fourth epiphyseal involvement and grade IV representing complete involvement

30. Arthrography Arthrography shows well the configuration of the femoral head and its relationship to the acetabulum Arthrography can also provide reliable information regarding containment of the femoral head within the acetabulum The major advantage of arthrography is that the examiner can assess the congruity of the hip in many different positions Currently, arthrography is most often used in the early diagnosis of hinge abduction of the hip, in which the femoral head “hinges” out of the acetabulum when the hip is abducted

31. Ultrasonography and Computed tomography are also used as an imaging tool in LCPD

32. Classification systems based on radiographic findings Catteral Classification Herring Lateral Pillar Classification Modified Elizabethtown classification Salter and Thomson classification Modified herring classification Mose Classification Stulberg Classification

33. Catteral Classification Catterall used radiographic findings to identify four groups of patients in which treatment decisions could be individualized based on radiographic appearances In Catterall group I only the anterior portion of the epiphysis is affected In group II more of the anterior segment is involved and a central sequestrum is present groups I and II had benign prognoses and did not need medical intervention In group III most of the epiphysis is “sequestrated”, with the unaffected portions located medial and lateral to the central segment In group IV, all of the epiphysis is sequestrated. Patients in groups III and IV required treatment.

34. Group I

35. Group II at onset  (left)  and 4 months later  (right)

36. Group III AP  (left)  and frog-leg lateral  (right)  images

37. Group IV

38. Lateral Pillar Classification The lateral pillar classification system is based on radiographic changes in the lateral portion of the femoral head when it enters the fragmentation stage Group A No loss of height in the lateral pillar

39. Group B Partial collapse (<50%)in the lateral pillar

40. Group B/C Group B/C border—intermediate between B and C, approximately 50% collapse, thin pillar, irregular

41. Group C Group C—more than 50% collapse of the lateral pillar.

42. A strong correlation exists between the lateral pillar classification and subsequent outcome, with group A hips faring the best, group B having an intermediate outcome, and group C faring the worst Compared with the Catterall classification system, the lateral pillar classification system has been reported to have greater interobserver reliability and to be a better predictor of final outcome

43. Modified Elizebeth classification

44. Classification of End Results Mose classification The Mose classification system is based on fitting the contour of the healed femoral head to a template of concentric circles In good outcomes, the shape of the femoral head deviates no more than 1 mm from a given circle on both AP and frog-leg lateral radiographs. If the shape falls within 2 mm, it is considered a fair outcome. If the deviation is greater than 2 mm, it is a poor outcome

45. Stulberg classification Stulberg and colleagues classified the radiographic appearance of hips at skeletal maturity, separating the outcomes into five groups

46. Stulberg III Femoral head fully reossified and ovoid

47. Group V The femoral head is irregular, with hills and valleys, whereas the acetabulum has a smooth contour

48. Prognostic factors -poor Extent of uncovering of the femoral head, Catterall group III or IV, lateral calcification, lateral head displacement , widening of the femoral head before fragmentation the Saturn phenomenon (a sclerotic epiphysis surrounded by a ring of lucency), and widening of the femoral neck in the early stages of the disorder

49. The Saturn phenomenon, a radiolucent ring occasionally seen in young children with Legg-Calvé-Perthes disease. When this pattern is present, the outcome is often poor

50. Differential Diagnosis

51. Treatment Symptomatic treatment for all children with onset on or before the eighth birthday Symptomatic treatment for children with onset after eighth birthday, lateral pillar groups A and C Surgical treatment for children with onset after eighth birthday, lateral pillar groups B and B/C border Surgical choices: femoral varus osteotomy, Salter innominate osteotomy, both osteotomies combined (onset after age 9 years) Late measures: femoral valgus osteotomy for established head and acetabular flattening, adducted hip, short leg gait Mechanical symptoms: hip arthroscopy with removal of osteochondrotic fragment, surgical dislocation for incongruity

52. Symptomatic treatment The two primary means of treating symptoms related to Legg-Calvé-Perthes disease are bed rest and traction. The use of nonsteroidal anti-inflammatory drugs for pain and discomfort and crutches to reduce weight bearing may also be of benefit The beneficial effects of bed rest are greatest around the time of development of the subchondral fracture Various means of traction include Simple longitudinal traction with the leg on the bed, balanced suspension and traction, and “slings and springs”

53. Longitudinal traction achieved with “slings and springs,” a simple and useful method of gradually abducting the affected leg and restoring range of motion to an irritable hip

54. Decrease in intraarticular pressure during traction is most when the hip was flexed 30 to 45 degrees and rotated slightly externally Partial weight bearing or non–weight bearing can help alleviate pain and increase range of motion

55. Nonsurgical Containment Using Orthotic Devices Number of different orthotic devices have been developed for patients with Legg-Calvé-Perthes disease to contain the femoral head nonsurgically All braces abduct the affected hip, most allow for hip flexion, and some control rotation of the limb Before starting containment therapy, however, it is important to restore normal range of motion to the “irritable” hip. Bed rest, traction, and reduced weight bearing are beneficial in this regard. Throughout containment treatment, it is vital that range of motion be preserved

56. Torrento brace The Toronto brace, with its universal joints, was designed to keep the hip abducted while allowing hip and knee flexion. Thus, the patient can both sit and stand

57. Birmingham brace It has a kneeling bar and a chain to keep the foot off the ground, while a specially altered crutch allows the abducted, internally rotated limb to clear the body when the patient walks

58. Trilateral socket hip abduction orthosis (the Tachdjian brace) It is a unilateral brace with an ischial seat

59. Newington brace It is a metal A-frame with a central support for the thighs

60. Atlanta Scottish Rite brace Consists of a metal pelvic band, hip hinges, thigh cuffs, and an extensile bar between the legs that permits abduction but restricts adduction With the hips abducted, the legs are usually flexed and externally rotated when the patient walks

61. Surgical Containment FEMORAL OSTEOTOMY Indication:onset of disease after 6 years of age and a hip with a poor prognosis based on radiographic findings The groups with better results after surgery were the lateral pillar B and B/C border hips in children older than 8 years of age at onset Before femoral osteotomy is performed, it is important that the patient regain a reasonable range of motion. Petrie casts can be used to achieve this goal INNOMINATE OSTEOTOMY indications for the procedure included onset of disease after 6 years of age, a moderately or severely affected head, and loss of containment

62. COMBINED FEMORAL AND INNOMINATE OSTEOTOMY VALGUS OSTEOTOMY SHELF ARTHROPLASTY HIP JOINT DISTRACTION CHIARI OSTEOTOMY

63. Long term prognosis The most important predictor of long-term outcome of Legg-Calvé-Perthes disease is the shape of the healed femoral head and its congruency with the acetabulum A number of patients who were not treated or who were treated for less than 6 months with bed rest had good to excellent results But, have noted an increased incidence of late degenerative arthritis in this patient population Factors associated with late degenerative disease include the patient's age at onset of the disease, lateral calcification, loss of sphericity of the femoral head,and the degree of steepness of the lateral edge of the acetabulum

64. Better outcomes were associated with diagnosis before 9 years of age, a congruous joint, and minimal involvement of the head and neck Final outcome is also significantly affected by the age of the patient at onset of the disease and by the duration of the condition. In general, the younger the patient is at onset, the milder the course of the disease In children older than 8 years of age at onset, surgical intervention should be considered

65. Thank you