Legg-Calvé-Perthes Disease (LCPD)

Legg-Calvé-Perthes disease (LCPD) is characterized by idiopathic avascular necrosis of the femoral head. The disease presents as a limp with an insidious onset and associated hip pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain. Legg-Calvé-Perthes disease primarily affects school-age children and has a male predominance. The exact mechanism of this disease is unknown and the diagnosis is made by clinical findings and imaging. Management can be conservative or surgical, depending on severity and the age of the patient.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Overview

Definition

Legg-Calvé-Perthes disease (LCPD), also called coxa plana, is a disorder of the hip in which blood supply to the proximal femoral epiphysis Epiphysis The head of a long bone that is separated from the shaft by the epiphyseal plate until bone growth stops. At that time, the plate disappears and the head and shaft are united. Structure of Bones is temporarily interrupted, resulting in avascular necrosis and permanent deformity of the femoral head and acetabulum.

Epidemiology

  • Population statistics:
    • Affects 1 in 1,200 people in the United States
    • Male-to-female ratio: 4:1
    • Peak incidence between 4 and 8 years of age
    • 90% of cases are unilateral.
    • Highest incidence: White populations
    • Lowest incidence: East Asian populations
    • More prevalent in urban areas in patients with lower socioeconomic status
  • Risk factors: 
    • Genetics Genetics Genetics is the study of genes and their functions and behaviors. Basic Terms of Genetics (10% of cases are familial)
    • HIV (5% of patients have LCPD)
    • Clotting disorders
    • Trauma
    • Steroid use
    • Developmental hip dysplasia 
    • Low birth weight
    • Short stature
    • Exposure to tobacco

Etiology

Legg-Calvé-Perthes disease is a multifactorial disease that is likely caused by a combination of genetic and environmental factors. The exact etiology is unknown, but interruption of the blood supply is the inciting cause.

Proposed mechanisms for interruption of blood supply:

  • Trauma
  • Synovitis
  • Coagulopathies 
  • Steroid use

Pathophysiology

There are 4 stages of LCPD:

  1. Necrosis: Disruption of blood supply leads to necrosis of subchondral cortical bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Structure of Bones or infarction of femoral capital epiphysis Epiphysis The head of a long bone that is separated from the shaft by the epiphyseal plate until bone growth stops. At that time, the plate disappears and the head and shaft are united. Structure of Bones.
  2. Fragmentation: infarcted bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Structure of Bones reabsorbed
  3. Reossification: Osteoblastic activity becomes prominent.
  4. Remodeling: Femoral head reshapes during patient growth.

Complications

  • Deformities of femoral head with disease progression:
    • Coxa magna: widening of femoral head
    • Coxa plana: flattening of femoral head
  • Resultant hip incongruence can lead to:
    • Labral tears
    • Osteoarthritis Osteoarthritis Osteoarthritis (OA) is the most common form of arthritis, and is due to cartilage destruction and changes of the subchondral bone. The risk of developing this disorder increases with age, obesity, and repetitive joint use or trauma. Patients develop gradual joint pain, stiffness lasting < 30 minutes, and decreased range of motion. Osteoarthritis of hip with age
Morbus perthes x-ray

Deformation of the head of the femur Head of the femur The hemispheric articular surface at the upper extremity of the thigh bone. Hip Joint due to LCPD
Legg-Calvé-Perthes disease is a disorder of the hip in which blood supply to the proximal femoral epiphysis Epiphysis The head of a long bone that is separated from the shaft by the epiphyseal plate until bone growth stops. At that time, the plate disappears and the head and shaft are united. Structure of Bones is temporarily interrupted, resulting in avascular necrosis and permanent deformity of the femoral head and acetabulum.

Image: “Morbus Perthes” by Dr. J. Lengerke. License: Public Domain

Clinical Presentation

History

  • Chief complaint: limp or altered gait
  • Pain presents late in the disease course (after 3 months), usually worsens with activity: pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain usually mild (limp may be painless) and may be referred to anteromedial thigh Thigh The thigh is the region of the lower limb found between the hip and the knee joint. There is a single bone in the thigh called the femur, which is surrounded by large muscles grouped into 3 fascial compartments. Thigh or knee
  • May be associated with delayed bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Structure of Bones age and growth
  • Usually lacks systemic symptoms

Clinical findings

  • Examination of gait:
    • Antalgic gait: short stance phase due to pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain when loading affected hip 
    • Trendelenburg gait: downward tilt of pelvis Pelvis The pelvis consists of the bony pelvic girdle, the muscular and ligamentous pelvic floor, and the pelvic cavity, which contains viscera, vessels, and multiple nerves and muscles. The pelvic girdle, composed of 2 "hip" bones and the sacrum, is a ring-like bony structure of the axial skeleton that links the vertebral column with the lower extremities. Pelvis contralateral to affected hip during swing phase of walking
    • Abductor lurch or intermittent limp, especially after exertion
  • Lower extremity exam:
    • Leg Leg The lower leg, or just "leg" in anatomical terms, is the part of the lower limb between the knee and the ankle joint. The bony structure is composed of the tibia and fibula bones, and the muscles of the leg are grouped into the anterior, lateral, and posterior compartments by extensions of fascia. Leg-length discrepancy (affected leg is shorter)
    • Atrophy of quadriceps/buttocks muscles from disuse
    • Reduced range of motion of hip joint Hip joint The hip joint is a ball-and-socket joint formed by the head of the femur and the acetabulum of the pelvis. The hip joint is the most stable joint in the body and is supported by a very strong capsule and several ligaments, allowing the joint to sustain forces that can be multiple times the total body weight. Hip Joint
Trendelenburg gait

Trendelenburg gait
Seen in patients with LCPD, this gait features a downward tilt of the pelvis Pelvis The pelvis consists of the bony pelvic girdle, the muscular and ligamentous pelvic floor, and the pelvic cavity, which contains viscera, vessels, and multiple nerves and muscles. The pelvic girdle, composed of 2 "hip" bones and the sacrum, is a ring-like bony structure of the axial skeleton that links the vertebral column with the lower extremities. Pelvis opposite to the affected hip during the swing phase of walking.

Image by Lecturio.

Diagnosis

The diagnosis of LCPD is clinical and confirmed through targeted imaging, so a high degree of suspicion is required in the approach to the limping child.

  • Labs may be used to exclude other diagnoses: complete blood count (CBC) and erythrocyte sedimentation rate (ESR) to rule out infectious causes of limp
  • X-rays X-rays X-rays are high-energy particles of electromagnetic radiation used in the medical field for the generation of anatomical images. X-rays are projected through the body of a patient and onto a film, and this technique is called conventional or projectional radiography. X-rays looking for pathological deformation of hip: anteroposterior (AP) and frog-leg lateral views
    • Early radiographs may appear normal.
    • Early findings: widening of joint space
    • Caffey’s sign or crescent sign: 
      • Subchondral radiolucent fracture Fracture A fracture is a disruption of the cortex of any bone and periosteum and is commonly due to mechanical stress after an injury or accident. Open fractures due to trauma can be a medical emergency. Fractures are frequently associated with automobile accidents, workplace injuries, and trauma. Overview of Bone Fractures line parallel to articular surface of femoral head
      • May be visible prior to fragmentation
    • Later findings:
      • Fragmentation of femoral ossification center
      • Lateral subluxation and flattening of femoral head
  • Magnetic resonance imaging (MRI) and bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Structure of Bones scans: may be part of evaluation if diagnosis is in question
    • MRI reveals marrow changes.
    • Bone scan reveals decreased perfusion to femoral head.

Management

Goals of medical therapy

  • Eliminating hip irritability and pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain
  • Maintaining adequate range of motion
  • Prevention of femoral epiphysis Epiphysis The head of a long bone that is separated from the shaft by the epiphyseal plate until bone growth stops. At that time, the plate disappears and the head and shaft are united. Structure of Bones collapse, containment of femoral head

Conservative management

  • Minimal weight bearing and protection of joint until ossification is complete:
    • Hip may be held in abduction by abduction casts or ambulatory braces. 
    • Goal is to promote containment of femoral head within acetabulum.
  • Physical therapy and stretching exercises to maintain range of motion and muscle tone Muscle tone The state of activity or tension of a muscle beyond that related to its physical properties, that is, its active resistance to stretch. In skeletal muscle, tonus is dependent upon efferent innervation. Skeletal Muscle Contraction
  • Nonsteroidal anti-inflammatory drugs (NSAIDs) if needed for pain management Pain Management Pain is defined as an unpleasant sensory and emotional experience associated with actual or potential tissue damage. Pain is a subjective experience. Acute pain lasts < 3 months and typically has a specific, identifiable cause. Pain Management
  • Children < 6–8 years old have more favorable outcomes to conservative therapy.

Surgical therapy

  • Operative procedures include varus femoral osteotomy and Salter osteotomy (goal is containment of femoral head).
  • More commonly used in patient > 8 years of age
  • Lack of consensus on best treatment

Prognosis

  • Generally self-limited (2–3 years)
  • Patients left with propensity to degenerative changes: 
    • Most patients function well until their 40s.
    • Hip replacement may be indicated due to chronic osteoarthritis.

Clinical Relevance

Differential diagnosis

  • Sickle cell disease Sickle cell disease Sickle cell disease (SCD) is a group of genetic disorders in which an abnormal Hb molecule (HbS) transforms RBCs into sickle-shaped cells, resulting in chronic anemia, vasoocclusive episodes, pain, and organ damage. Sickle Cell Disease ( SCD SCD Sickle cell disease (SCD) is a group of genetic disorders in which an abnormal Hb molecule (HbS) transforms RBCs into sickle-shaped cells, resulting in chronic anemia, vasoocclusive episodes, pain, and organ damage. Sickle Cell Disease): hemoglobinopathy caused by point mutation Mutation Genetic mutations are errors in DNA that can cause protein misfolding and dysfunction. There are various types of mutations, including chromosomal, point, frameshift, and expansion mutations. Types of Mutations in 6th codon of β-globin gene on chromosome 11, which ultimately leads erythrocytes Erythrocytes Erythrocytes, or red blood cells (RBCs), are the most abundant cells in the blood. While erythrocytes in the fetus are initially produced in the yolk sac then the liver, the bone marrow eventually becomes the main site of production. Erythrocytes to adopt characteristic “sickle” appearance. Can impair blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure to femoral head, causing avascular necrosis of femoral head in young children with SCD SCD Sickle cell disease (SCD) is a group of genetic disorders in which an abnormal Hb molecule (HbS) transforms RBCs into sickle-shaped cells, resulting in chronic anemia, vasoocclusive episodes, pain, and organ damage. Sickle Cell Disease. May be referred to as osteonecrosis of femoral head (ONFH).
  • Developmental hip dysplasia (DHD): congenital disorder of hip joint Hip joint The hip joint is a ball-and-socket joint formed by the head of the femur and the acetabulum of the pelvis. The hip joint is the most stable joint in the body and is supported by a very strong capsule and several ligaments, allowing the joint to sustain forces that can be multiple times the total body weight. Hip Joint characterized by hip instability, displayed as subluxation or dislocation, frequently recognized in infants.

Within clinical spectrum of LCPD

Short stature: due to genetics, developmental disorders, and leg-length discrepancy, child may be shorter in stature.

Complication of LCPD

Osteoarthritis Osteoarthritis Osteoarthritis (OA) is the most common form of arthritis, and is due to cartilage destruction and changes of the subchondral bone. The risk of developing this disorder increases with age, obesity, and repetitive joint use or trauma. Patients develop gradual joint pain, stiffness lasting < 30 minutes, and decreased range of motion. Osteoarthritis (OA): most common form of arthritis, often referred to as “wear and tear” arthritis. Principally a disease of aging, characterized by hyaline articular cartilage Cartilage Cartilage is a type of connective tissue derived from embryonic mesenchyme that is responsible for structural support, resilience, and the smoothness of physical actions. Perichondrium (connective tissue membrane surrounding cartilage) compensates for the absence of vasculature in cartilage by providing nutrition and support. Cartilage loss, but also involving changes to subchondral bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Structure of Bones, synovium, and surrounding joint structures. Anatomical abnormalities produced by LCPD increase the risk for degenerative changes of the hip joint Hip joint The hip joint is a ball-and-socket joint formed by the head of the femur and the acetabulum of the pelvis. The hip joint is the most stable joint in the body and is supported by a very strong capsule and several ligaments, allowing the joint to sustain forces that can be multiple times the total body weight. Hip Joint and the need for surgical replacement of the hip.

References

  1. Deeney, V. F., & Arnold, J. (2018). Orthopedics. In B. J. Zitelli MD, S. C. McIntire MD & Nowalk, Andrew J., MD, Ph.D. (Eds.), Zitelli and Davis’ atlas of pediatric physical diagnosis (pp. 759-844). https://www.clinicalkey.es/#!/content/3-s2.0-B9780323393034000220
  2. Kim, H. K. W. (2020). Legg-calvé-Perthes disease. In D. J. Berry MD, & J. R. Lieberman MD (Eds.), Surgery of the hip (pp. 480-501). https://www.clinicalkey.es/#!/content/3-s2.0-B9780323554640000398

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