Legg-Calve-Perthes Assoc. Prof. Melih Güven Yeditepe University Hospital Department of Orthopaedics and Traumatology

Learning Objectives Should be able to explain the etiopathogenesis of osteonecrosis of the femoral head in children. Should be able to list the stages and clinical, radiographic features of the stages. Should be able to define the risk factors of Perthes’ disease. Should be able to list the radiographic features of the femoral head under risk. Should be able to make differential diagnosis of Perthes’ disease. Should be able to explain the clinical findings of Perthes’ disease. Should be able to list conservative and surgical treatment methods of Perthes’ disease.

Perthes disease Idiopathic juvenile avascular necrosis of the femoral head Waldenström disease 1/1200 E/K = 7-8/1 Most commonly is seen in children aged 4-8 (2-12) years, with an average age of 7 years %10-15 bilateral

Etiology ??? Synovitis Joint effusions (e.g. infection) Trauma Cartilage hypertrophy Congenital vascular hypoplasia Steroids Coagulation defects Systemic disorders Hereditary influence, environmental influence, hyperactivity

Etiology Disruption of blood flow to capital femoral epiphysis (CFE)‏

Pathology The blood supply to the capital femoral epiphysis is interrupted (arteries and veins) Bone infarction occurs, especially in the subchondral cortical bone, while articular cartilage continues to grow. (Articular cartilage grows because its nutrients come from the synovial fluid)‏ Revascularization occurs, and new bone ossification starts Changes to the epiphyseal growth plate occur secondary to the subchondral fracture

Clinical symptoms Hip and groin pain, which may be referred to the thigh ± knee pain !!! Limp – with or without pain Especially Trendelenburg test (+) Limitation in abduction and internal rotation (decreased range of motion) Atrophy of thigh muscles Flexion contracture

Radiological examination Conventional radiographs Pelvis AP and frogleg USG Bone scan (scintigraphy) MRI

Radiographic presentation (Stages) Synovitis ( 4 – 6 weeks ) Avascular necrosis ( 6 months ) Fragmentation ( 1 – 2 years ) Reossification Residual stage ( 3 – 4 years )

Synovitis Effusion in joint capsule, edema and thickening of the capsule Increased medial joint space Cresent sign Demineralization in metaphysis

Avascular necrosis Lateralization of the femoral head; sclerosis Decreased size of ossification center Decreased height of epiphysis Collapse of the epiphysis Physeal irregularity Metaphyseal cysts Superolateral Gage sign

Fragmentation - resorption Resorption of avascular bone Fragmented epiphysis More irregular acetabular contour Reossification – regeneration (revascularation)

Residual stage Healing of the metaphyseal cysts Reossified femoral head Remodeling of the femoral head and acetabulum Sometimes disrupted relationship between femoral head and acetabulum Coxa magna, plana, breva

Differential diagnosis Synovitis Toxic synovitis Septic arthritis Acute rheumatic fever Juvenile arthritis Tbc arthritis Osteoid osteoma

Differential diagnosis Avascular necrosis Sickle cell anemia Gaucher disease Traumatic avascular necrosis

Differential diagnosis Fragmentation Tbc arthritis Multiple epiphyseal dysplasia Hypothyroid

Prognostic factors Age Gender Time to diagnose and treatment Amount of femoral head involvement “Head at risk” signs

Classifications After occurance of subchondral cresent sign Salter - Thompson At the fragmentation stage Catterall Herring-Lateral pillar After skeletal maturity Stulberg

Salter – Thompson Group A Group B

Catterall C-1 C-2 C-3 C-4

Herring-Lateral pillar Group A Lateral pillar intact Group B Head involvement < 50% Group C Head involvement > %50 Detrmine treatment and prognosis

“Head at risk” signs Lateralization of the femoral head from the acetabulum Calcifications in the lateral portion of the epiphysis Diffuse metaphyseal cysts Horizontally aligned epiphyseal plate Gage sign (large metaphyseal cyst)

Goal of treatment Preservation of the roundness of the femoral head and prevention of deformity while the condition runs its course

Treatment algorithm < 7 years of age, C-1 and C-2 or Herring lateral pillar group A – No treatment > 7 years of age, C-3 and C-4, Herring lateral pillar group B and C – Treatment

Treatment algorithm

Phases of treatment Initial period Containment provider modalities Reconstructive procedures (salvage methods)

Phases of treatment Initial period Observation Bed rest Manual or skin traction NSAI

Phases of treatment Containment provider modalities Containment of the femoral epiphysis within the confines of the acetabulum Orthoses

Phases of treatment Containment provider modalities Containment of the femoral epiphysis within the confines of the acetabulum Femoral varization ± derotation osteotomy Iliac osteotomy

Phases of treatment Reconstructive procedures (salvage methods) Shelf acetabuloplasty Double or triple iliaca osteotomies ± femoral osteotomies

Thanks !