You are in: eMedicine Specialties > Orthopedic Surgery > HIP Legg-Calve-Perthes DiseaseArticle Last Updated: Jul 14, 2004AUTHOR AND EDITOR INFORMATIONSection 1 of 8
  Author: George D Harris, MD, MS, Professor of Medicine, University of Missouri-Kansas City School of Medicine and Truman Medical Center at Lakewood George D Harris is a member of the following medical societies: American Academy of Family Physicians Editors: B Sonny Bal, MD, Associate Professor, Department of Orthopedic Surgery, University of Missouri School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; James J McCarthy, MD, FAAOS, FAAP, Associate Professor, Consulting Orthopedic Surgeon, Department of Orthopedics and Rehabilitation, University of Wisconsin School of Medicine and Public Health;; Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital; Carlos J Lavernia, MD, FAAOS, Adjunct Clinical Professor, Department of Orthopedic Surgery, University of Miami School of Medicine; Medical Director, Orthopedic Institute at Mercy Hospital Author and Editor Disclosure Synonyms and related keywords: LCPD, osteochondrosis of the femoral head, avascular necrosis of the proximal femoral head, intermittent limp, abductor lurch, painless limp, persistent hip pain, Trendelenburg gait INTRODUCTIONSection 2 of 8
  Legg-Calvé-Perthes disease (LCPD) is avascular necrosis of the proximal femoral head resulting from compromise of the tenuous blood supply to this area. LCPD usually occurs in children aged 4-10 years. The disease has an insidious onset and may occur after an injury to the hip. In the vast majority of instances, the disorder is unilateral. Both hips are involved in less than 10% of cases, and the joints are involved successively, not simultaneously. FrequencyLCPD usually occurs in children aged 4-10 years, with a mean age of 7 years. It occurs more commonly in boys than in girls, with a male-to-female ratio of 4:1. The condition is rare, occurring in approximately 4 of 100,000 children. EtiologyThe cause is not known, but children with LCPD have delayed bone age, disproportionate growth, and a mildly shortened stature. LCPD condition may be idiopathic, or it may result from a slipped capital femoral epiphysis, trauma, steroid use, sickle-cell crisis, toxic synovitis, or congenital dislocation of the hip. PathophysiologyRapid growth occurs in relation to development of the blood supply of the secondary ossification centers in the epiphyses, creating an interruption of adequate blood flow and making these areas prone to avascular necrosis. Interruption of the blood supply to the bone results in necrosis, removal of the necrotic tissue, and its replacement with new bone. Bone replacement may be so complete and perfect that completely normal bone may result. The adequacy of bone replacement depends on the age of the patient, the presence of associated infection, congruity of the involved joint, and other mechanical and physiologic factors. Necrosis may occur after trauma or infection, but idiopathic lesions can develop during periods of rapid growth of the epiphyses. ClinicalThe earliest sign is an intermittent limp (abductor lurch), especially after exertion, with mild or intermittent pain in the anterior part of the thigh. LCPD is the most common cause of a limp in the 4- to 10-year-old age group, and the classic presentation has been described as a painless limp. The patient may present with limited range of motion of the affected extremity. The most common symptom is persistent pain. Hip pain may develop and is a result of necrosis of the involved bone. This pain may be referred to the medial aspect of the ipsilateral knee or to the lateral thigh. The quadriceps muscles and adjacent thigh soft tissues may atrophy, and the hip may develop adduction flexion contracture. The patient may have an antalgic gait with limited hip motion. Pain may be present with passive range of motion and limited hip movement, especially internal rotation and abduction. Children with LCPD can have a Trendelenburg gait resulting from pain in the gluteus medius muscle. Laboratory studies and radiography may supplement medical history taking and physical examination in the assessment of a child with a limp. Differential diagnoses include osteomyelitis, pyogenic arthritis, transient synovitis, abscess of the psoas muscle, juvenile rheumatoid arthritis, hemophilia, slipped capital femoral epiphysis, and neoplasm. WORKUPSection 3 of 8
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TREATMENTSection 4 of 8
Medical therapyConsultation with an orthopedist is recommended. Treatment goals include eliminating hip irritability, restoring and maintaining good range of motion in the hip, preventing femoral epiphyseal collapse, and attaining a spherical femoral head when the hip heals. Initial therapy includes minimal weight bearing and protection of the joint, which is accomplished by maintaining the femur abducted and internally rotated so that the femoral head is held well inside the rounded portion of the acetabulum. Abduction and rotation of the femur is accomplished either by the use of orthotic devices (bracing) or surgery (osteotomy). The Scottish Rite brace achieves containment by abduction while allowing free knee motion. Surgical therapyResults of surgical containment appear to be better than those of nonsurgical containment (orthosis). Surgical approaches include either femoral osteotomy to redirect the involved portion within the acetabulum or innominate osteotomy. Both procedures produce equal results, but femoral osteotomy may cause shortening of the limb, leading to a chronic limp. Surgery does not speed healing of the femoral head, but it does cause the head to reossify in a more spherical fashion. Follow-upFor excellent patient education resources, visit eMedicine's Foot, Ankle, Knee, and Hip Center and Sports Injury Center. Also, see eMedicine's patient education article Repetitive Motion Injuries. COMPLICATIONSSection 5 of 8
Because LCPD is a local self-healing disorder, treatment consists of protection of the joint by allowing new bone formation to occur and regain a spherical femoral head. The goal is to prevent (1) irregular contouring, flattening, or mushrooming of the head; (2) shortening and broadening of the neck; and (3) flattening of the vertical wall of the acetabulum. The development of any of these conditions can result in osteoarthritis at an early age. OUTCOME AND PROGNOSISSection 6 of 8
The prognosis can be good and depends on the completeness of involvement of the epiphyseal center. The functional result depends on the amount of deformity that develops when the structure is softened. The patient's short-term prognosis is related to femoral head deformity at the completion of the healing stage. Risk factors include a clinical onset at an older age, extensive femoral epiphyseal involvement, femoral head containment, reduced range of motion in the hip, and premature closure of the growth plate. The long-term prognosis is related to the potential for osteoarthritis of the hip as an adult. In patients with metaphyseal defects, in those in whom the disease develops late in childhood (age 10 y or older), and in those who have more complex involvement of the femoral head with residual deformity, the prognosis is worse, and degenerative arthritis occurs in nearly 100% of these patients. This rate is in comparison to those patients who are younger than 5 years when the problem develops. The incidence of degenerative arthritis is negligible in this younger population. MULTIMEDIASection 7 of 8
REFERENCESSection 8 of 8
Legg-Calve-Perthes Disease excerpt Article Last Updated: Jul 14, 2004 | |||||||||||||||||||||
