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Legg-CalveLegg-Calve-Perthes DiseaseDr.E.Kaizar Ennis
It is a self-limited non-infectious disease of the hip joint occuring in children characterised pathologicaly by avascular necrosis of the ossification center of capital femoral epiphysis which is alternately resolved and replaced resulting in variable degrees of deformity of the femoral head and restricted movements of the hip joints.
Described first by Waldenstrom in 1909 who mistakenly ascribed it to tuberculosis. In 1910 Arthur Legg , U. S. A Jacques Calve , France George Perthes ,Germany described and recognized it as non-infectious in origin. In 1922 Waldentrom gave the correct interpretation and described the stages.
1:1200 p 1:12000 4M:1F :1F 2- 12 years (mean 7 years) 5-20% bilateral (In bilateral cases changes appear in one hip at least one year earlier than the other)
British Isles: Catterall 1970(388 cases)
Arterial occlusion Mechanical obstruction Trauma
Venous congestion Thrombophilia & hypofibrinolysis Disorder epiphyseal cartilage The aetiology may be ultimately multifactorial Salter & Bell JBJS 1968 Glueck et al JBJS 1996 Ponseti JBJS 1983
BS of the infant femoral headMetaphyseal vessels Lateral Epiphyseal vessels Vessels of the ligamentum teres.
STAGES OF PATHOLOGICAL PROCESS1. Stage of synovitis 2. Stage of AVN 3. Stage of fragmentation 4. Stage of regeneration 5. Stage of healing.
Stage of AVNWaldenstrom sign crescent sign
Stage of regenerationA growth arrest line that outlines the ossific nucleus at the time of initial infarction is represented by ahead within a head SIGN
The greater Trochanter becomes hypertrophied and elevate proximally, elevation impairs the power of abductor muscles and leads positive trendelenburg. trendelenburg. The combination of a short femoral neck and a high greater trochanter is considered functional coxa vara vara
HEAD AT RISK SIGNSSIGNSCLINICAL
LOSS OF HIP MOTION IS PERSISTANT AND PROGRESSIVE INCREASED ADDUCTION CONTRACTURE OF HIP THE OBESE CHILD FEMALE CHILD AGE >7YRS
Mild intermittent pain in the hip,thigh or knee Onset of pain is acute or insidious The classical presentation has been described as painless limp
Because the presenting symptoms are usually mild, the parents do not seek medical attention long after the onset of symptoms. A small % of children have a history of trauma
Clinical ExaminationAntalgic gait Tenderness Muscle spasm Limitation of movement (abduction and internal rotation) Muscle wasting Leg length inequality
UNILATERAL CASES Septic arthritis Sponondyloepiphyseal dysplasia Rheumatoid arthritis Tuberculosis Juvenile rheumatoid arthritis
BILATERAL CASES Hypothyroidism Multiple epiphyseal dysplasia Mucopolysacharidosis Sickle cell disease Spondyloepiphyseal dysplasia
CBC ESR CRP Urine RE RADIOGRAPHY BONE SCINTIGRAPHY MRI USG ARTHROGRAM ANGIOGRAPHY
Catterall Salter and Thompson Herrings lateral pillar Stulberg
Group 1 anterior portion of the epiphysis Group 2-anterior and central 2 Group 3-Most of the epiphysis is 3sequestrated Group 4-All of the epiphysis is 4sequestrated
Salter Thompson classification
Type A Extent of subchondral fracture < 50% of superior dome of HOF Good prognosis
Type B Extent of subchondral fracture > 50 % of superior dome of HOF Fair or poor prognosis
Lateral Pillar Classification by Herring
Based on radiographic changes in the lateral portion of the femoral head when it enters the fragmentation stage as seen in AP view
Group A-Minimal density change in the Alateral pillar-good outcome pillarGroup B- height loss upto 50%- moderate B50%outcome Group C- height loss > 50%-worst C50%outcome
Group I Shape of HOF is normal Group II within 2 mm to a concentric circle Group III more than 2 mm (congruous incongruity) Group IV HOF flattened area > 1 cm in length(congruous incongruity) Group V Collapse of HOF (incongruous incongruity)
Determination of final outcome
MOSEMOSE-AP & LAT VIEWS with Mose templateFinal shape of the head may be compared to a perfect circle:When the head contour is within 1 mm of a given circle ,the result is deemed good. When the head contour is within 2 mm ,the result is deemed fair. When the head contour is greater than 2 mm ,the result is deemed poor.
Head at Risk Factors-Radiological Factors1. 2.
Lateral subluxation of femoral head Gages sign a radioluscent V in the lateral aspect of the epiphysis Calicification lateral to the epiphysis (*Cage sign) A horizontal physeal line Diffuse metaphyseal reaction (Metaphyseal cyst)
Small osteoporotic segment forming a translucent VVshaped trough in the lateral part of the epiphysis
Calcification of the lateral epiphysis.
Crescent sign/Salter sign/Caffreys
Trabeculae in dead bone may fracture in subchondral region
Sagging Rope Sign
Goals of Treatment1.
Elimination of hip irritability Restoration & maintenance of hip motion Prevention of extrusion and collapse. Attainment of a spherical HOF
Full mobility Containment of the femoral head Resume wt. bearing & activity as soon as possible.
Phases of Treatment
Phase 1 Initial phase Phase 2 Containment and maintenance of HOF within acetabulum and restoration of full ROM Phase 3 Reconstructive surgery
Restore full ROM Traction at home B/L counterpoised split Russels traction with a medial rotation stop on the thigh Investigation Analgesics Observation
Phase 2(Containment by orthoses/surgery)
Containment using Orthoses All braces abduct the affected hip, allows for hip flexion and control rotation of the limb Before starting containment, restore normal ROM byBed rest Traction Analgesics Decrease weight bearing by crutches
PREREQUISITESFULL RANGE OF MOTION WITH RELIEF OF MUSCLE SPASM ENTIRE FEMORAL HEAD SHOULD BE CONCENTRIC AND FULLY CONTAINED WITH IN THE ACETABULUM MOTOR STRENGTH AND BALANCE TO USE THE ORTHOSES REASSESS EVERY 4-8 WEEKS FOR 4
GAIT RANGE OF MOTION XRAY
TYPES OF ORTHOSIS
NON AMBULATORY BROOMSTICK PLASTIC CAST BIVALVED HIP SPICA CAST
AMBULATORY STATIC HARRISON HIP CONTAINTMENT
BILATERAL NEWINGTON HIP CONTAINMENT SCOTTISH RITE
UNILATERAL TRILATERAL SOCKET HIP ABDUCTION
Adujustable broomstick plaster
NEWINGTON HIP CONTAINMENT
Atlanta Scottish Rite Brace
Containment by orthosis:Signs of healingAppearance of irregular ossification in the capital femoral epiphysis No new radio opaque areas Medial segment of femoral head should increase in height There should be an intact lateral column
PERSISTENT OR RECURRENT LOSS OF HIP MOTION PROGRESSIVE COLLAPSE AND SUPEROLATERAL EXTRUSION OF FEMORAL HEAD
ADVANTAGES NO END POINT OF TREATMENT IS REQUIRED RAPID RESUMPTION OF ACTIVITY
DIS ADVANTAGES SECOND OPERATION TO REMOVE IMPLANT COMPLICATION OF SURGERY
ABSENCE OF IRRITABILITY OR RESTRICTION OF HIP MOTION ABSENCE OR MINIMAL DEFORMITY OF FEMORAL HEAD CONCENTRIC CONTAINMENT BY ABD,MEDIAL ROTATION AND FLEXION
CONTAINMENT PROCEDURESInnominate Osteotomy (Salter) Femoral Osteotomy
Lateral opening wedge osteotomy Varus derotation osteotomy
Combined Femoral & Innominate osteotomy LATERAL SHELF PROCEDURE
Phase 3 Reconstructive SurgeriesLOWER LIMB INEQUALITY GREATER TROCHANTER OVERGROWTH AND COXA BREVA HINGED ABDUCTION COXA MAGNA INCONGROUS HIP OSTEOCHONDRITIS DISSECANS
Phase 3 Reconstructive SurgeriesValgus extension osteotomy Valgus flexion and internal rotation osteotomy Cheilectomy Shelf procedure by Staheli
Garceaus cheilectomy Chiaris osteotomy Trochanteric epiphyseodesis Trochanteric advancement Valgus osteotomy Hip replacement
AGE EXTENT PROTRUSION OF FEMORAL HEAD GROWTH DISTURBANCE OF PHYSIS METAPHYSEAL CHANGES STAGE IN THE NATURAL COURSE OF THE DISEASE PERSISTENT LOSS OF HIP MOTION OBESE CHILD