Legg Calve Perthes Disease

Dr. Harun Ar Rashid, MD - Arthritis, Bones, Joints Pain, Trauma, and Internal Medicine Specialist Dr. Harun Ar Rashid, MD - Arthritis, Bones, Joints Pain, Trauma, and Internal Medicine Specialist
0 Views
Rx Musculoskeletal System (A - Z)

Legg-Calve-Perthes (LEG-kahl-VAY-PER-tuz) disease is a childhood condition that occurs when the blood supply to the ball part (femoral head) of the hip joint is temporarily interrupted and the bone begins to die. This weakened bone gradually breaks apart and can lose its round shape. This disease is caused by a poor blood supply to the upper growth plate of the thighbone near the hip joint. Typical symptoms include hip pain and trouble walking. The diagnosis is based on x-rays and sometimes magnetic resonance imaging. The most common upper limb abnormality was radial ray deficiency (138), followed by subgroups of undergrowth (91), upper limb defects due to constriction band syndrome (51), central ray deficiency (41), and ulnar ray deficiency

Legg-Calvé-Perthes disease (or Perthes disease) is a rare condition in children in which the ball-shaped head of the thigh bone, referred to as the femoral head, loses its blood supply. As a result, the femoral head collapses. Perthes disease is idiopathic, which means that there is no known cause for this condition. What is clear is that when blood supply to the femoral head is disrupted, the bone starts to break down. About one out of every 12,000 children develop Perthes disease.

The most common surgical procedure for treating Perthes disease is an osteotomy. In this type of procedure, the bone is cut and repositioned to keep the femoral head snug within the acetabulum. This alignment is kept in place with screws and plates, which will be removed after the healed stage of the disease.

Other Names

  • Coxa plana
  • Legg-Perthes
  • Legg Calve
  • Perthes disease
  • Idiopathic osteonecrosis of the capital femoral epiphysis of the femoral head
  • Idiopathic avascular necrosis of the capital femoral epiphysis of the femoral head
  • Osteochondrosis of the femoral head

Pathophysiology

  • General
  • Interruption of vascular supply theory
    • Unclear whether it is an arterial infarction or venous congestion
    • Systemic coagulopathy may play a role
  • Trauma
    • Acute Trauma to the immature hip may ben an accessory etiologic factor
    • Repetitive mechanical stress may also be implicated
  • Proposed four-phase model
    • 1) Necrosis
      • Disruption of the blood supply causing infarction, bone softening and death of the femoral capital epiphysis
    • 2) Fragmentation
      • Resorption of infarcted bone.
    • 3) Reossification
      • Osteoblastic activity and reforms the femoral epiphysis
    • 4) Remodeling
      • New femoral head reshapes during growth and response to conservative treatment will usually show healing in 2-4 years 
  • Coagulation disturbance
    • Increased coagulability including inherited coagulopathies (ie, Factor V Leiden), thrombophilias, and hypofibrinolysis
    • 2012 meta-analysis: found factor V leiden, Prothrombin II increased risk, but not MTHFR polymorphism
  • Inflammatory markers
    • Increased interleukin-6 (IL-6) polymorphism (G-174C/G-597A)
  • Genetics
    • Collagen type II gene COL2A1 mutation
    • Proapoptotic factor Bcl-2-associated X protein (Bax)
    • Twin-twin studies have identified a genetic relationship
  • HIV (Up to 5% of HIV patients have avascular necrosis of the hip)
  • Secondhand smoke exposure
    • Maternal smoking
  • Low socioeconomic status (SES)
    • Generally low SES
    • In rural areas
  • Microsomia
    • Birth weight < 2.5 kg in boys
  • Anthropometrics
    • Short stature
    • Obesity
  • Caucasian and Asian ethnicity 
  • Psychiatric
    • Attention Deficit Hyperactivity Disorder (ADHD)
  • Mechanical stress
  • Developmental
    • Delay in endochondral ossification in the proximal capital femoral epiphysis
  • Other
    • Increased Vascular endothelial growth factor (VEGF) and hypoxia-inducible factor (HIF-1) in rat models
    • Altered Insulin growth factor 1 (IGF-1) expression in rat models

Differential Diagnosis

  • Fractures And Dislocations
    • Pelvic Fracture
    • Hip Fracture
    • Acetabular Fracture
    • Femoral Neck Stress Fracture
    • Pelvic Stress Fracture
    • Hip Dislocation
  • Arthropathies
    • Osteitis Pubis
    • Avascular Necrosis of the Hip
    • Hip Osteoarthritis
    • Femoroacetabular Impingement
    • Transient Osteoporosis of the Hip
  • Muscle and Tendon Injuries
    • Hip Flexor Tendonitis
    • Piriformis Syndrome
    • Hamstring Strain
    • Proximal Hamstring Tendinopathy
    • Adductor Strain
    • Greater Trochanteric Pain Syndrome
  • Bursopathies
    • Iliopsoas Bursitis
    • Ischial Bursitis
  • Ligament Injuries
    • Acetabular Labrum Tear
  • Neuropathies
    • Meralgia Paresthetica
  • Other
    • Snapping Hip Syndrome
    • Septic Arthritis
    • Gout
    • Leg Length Discrepancy
  • Pediatric Pathology
    • Transient Synovitis of the Hip
    • Developmental Dysplasia of the Hip (DDH)
    • Legg-Calve-Perthes Disease
    • Slipped Capital Femoral Epiphysis (SCFE)
    • Avulsion Fractures of the Ilium (Iliac Crest, ASIS, AIIS)
    • Ischial Tuberostiy Avulsion Fracture
    • Avulsion Fractures of the Trochanters (Greater, Lesser)
    • Apophysitis of the Ilium (Iliac Crest, ASIS, AIIS)

Diagnosis

  • History
    • Limp of acute or insidious onset from between 1 to 3 months
    • If present, pain localized to the hip with or without referred pain to the knee, thigh, or abdomen
      • May worsen with activity
    • No systemic symptoms (ie, fever, chills, weight loss, migratory joint pains) should be found
  • Physical Exam Physical Exam Hip
    • Decreased internal rotation and abduction of the hip
    • Pain on rotation referred to the anteromedial thigh and/or knee
    • Atrophy of thigh & buttock muscles
  • Special Tests
    • Trendelenburg Gait or antalgic gait may be present depending on the chronicity [24]
Legg-Calve-Perth of the left hip[25]

Radiographs

  • Standard Radiographs Hip
    • AP Pelvis, Frog leg views
  • Early Findings
    • Widening of joint space (epiphyseal cartilage hypertrophy)
    • Changes in the epiphysis (smaller, appears denser)
  • Late Findings
    • Flattening of the femoral head, fragmentation, healing (sclerosis)

MRI

  • Bone Scan and MRI may be used if X-ray are unequivocal
    • Bone scans show decreased perfusion to the femoral head
    • MRI may show marrow changes [26]

Classification

Lateral Pillar or Herring Classification

  • Clinical utility: Prognostication
    • Wiig et al found: 70% of lateral pillar A hips, 51% of lateral pillar B hips, and 30% of C hips had Stulberg I or II outcomes[27]
  • Group A: lateral pillar is at full height with no density changes
    • Consistently good prognosis.
  • Group B: lateral pillar maintains greater than 50% height
    • Poor outcome if the bone age is greater than 6.
  • Group C: Less than 50% of the lateral pillar height is maintained
    • All patients will experience a poor outcome. [28]

Stulberg classification

  • The gold standard for rating residual femoral head deformity and joint congruence on the radiograph
    • Recent studies show poor interobserver and intraobserver reliability
  • I: Normal, congruent Hip
    • Arthritis does not develop
  • II: Spherical head, concentric in acetabulum on AP, frog-leg lateral
    • Shortened femoral neck, abnormally steep acetabulum
  • III: Ovoid, mushroom or umbrella-shaped femoral head; not flat
    • Mild-to-moderate arthritis in adulthood
  • IV: Flathead and acetabulum, congruent joint
  • V: Flat femoral head, normal femoral neck, and acetabulum with incongruent joint
    • Severe arthritis before 50 years of age

Treatment

  • Goal: maintain the sphericity of the femoral head and the congruency of the femur-acetabulum relationship
    • Prevent the development of secondary Hip Osteoarthritis

Prognosis

  • Wiig et al found younger patients have better outcomes[27]
    • 57% of patients <6 years were found to be Stulberg I or II
    • Only 38% of patients >6 years were found to be Stulberg I or II
  • Lateral Pillar Classification (degree of femoral head involvement: A [least] to C [most])
    • >8 years old and patients in lateral pillar group B or B/C have better outcomes with surgery
    • <8 years old and patients in group B do well regardless of treatment choice
    • Patients in lateral pillar C group do poorly regardless of age or treatment method
    • Group C patients experience poor outcomes regardless of treatment choice
  • Recovery
    • 50% of patients almost fully recover with no long-term sequelae (need citation)
  • Pain and Disability
    • 50% of patients develop pain, disability, degenerative joint disease and in their 40-50s leading to hip replacement in 60s-70s.
  • Gender
    • Female patients have worse outcomes if onset occurs >8 years of age [26]

Nonoperative

  • Indications
    • Children with bone age less than 6 or lateral pillar A involvement
  • Activity restriction and protective weight-bearing until ossification is complete
  • NSAIDs can be prescribed for comfort
  • Treatment Options: Bracing, No treatment, Range of Motion
    • Herring et al found no difference between these three treatment choices [29]

Operative

  • Femoral or Pelvic Osteotomy
    • Indications: children older than 8 years
    • Lateral pillars B and B/C have improved outcomes with surgery compared to A and C
    • Studies suggest early surgery before femoral head deformity develops
  • Valgus or Shelf Osteotomies
    • Indications: children with hinge abduction
    • Improves abductor mechanism
  • Hip Arthroscopy
    • It May be used for treating mechanical symptoms and impingement

You Might Also Like This :

  1. Slipped Capital Femoral Epiphysis: Causes, Symptoms And Treatment Slipped Capital Femoral Epiphysis or SCFE is a hip condition that mostly occurs in teenagers. The hip is a ball and socket joint in which the head of the thigh bone fits into the socket of the large pelvic bone called acetabulum. The thigh bone grows along two growth plates (physis) that are found at […]...
  2. Slipped Capital Femoral Epiphysis: Causes, Symptoms And Treatment Slipped Capital Femoral Epiphysis or SCFE is a hip condition that mostly occurs in teenagers. The hip is a ball and socket joint in which the head of the thigh bone fits into the socket of the large pelvic bone called acetabulum. The thigh bone grows along two growth plates (physis) that are found at […]...
  3. Slipped Capital Femoral Epiphysis: Causes, Symptoms And Treatment Slipped Capital Femoral Epiphysis or SCFE is a hip condition that mostly occurs in teenagers. The hip is a ball and socket joint in which the head of the thigh bone fits into the socket of the large pelvic bone called acetabulum. The thigh bone grows along two growth plates (physis) that are found at […]...
  4. Slipped Capital Femoral Epiphysis: Causes, Symptoms Treatment Slipped Capital Femoral Epiphysis or SCFE is a hip condition that mostly occurs in teenagers. The hip is a ball and socket joint in which the head of the thigh bone fits into the socket of the large pelvic bone called acetabulum. The thigh bone grows along two growth plates (physis) that are found at […]...
  5. Slipped Capital Femoral Epiphysis: Causes, Symptoms, Treatment Slipped Capital Femoral Epiphysis or SCFE is a hip condition that mostly occurs in teenagers. The hip is a ball and socket joint in which the head of the thigh bone fits into the socket of the large pelvic bone called acetabulum. The thigh bone grows along two growth plates (physis) that are found at […]...
  6. Slipped Capital Femoral Epiphysis: Causes, Symptoms And Treatment Slipped Capital Femoral Epiphysis or SCFE is a hip condition that mostly occurs in teenagers. The hip is a ball and socket joint in which the head of the thigh bone fits into the socket of the large pelvic bone called acetabulum. The thigh bone grows along two growth plates (physis) that are found at […]...
  7. Slipped Capital Femoral Epiphysis: Causes, Symptoms And Treatment Slipped Capital Femoral Epiphysis or SCFE is a hip condition that mostly occurs in teenagers. The hip is a ball and socket joint in which the head of the thigh bone fits into the socket of the large pelvic bone called acetabulum. The thigh bone grows along two growth plates (physis) that are found at […]...
  8. Femoroacetabular Impingement Femoroacetabular impingement (FAI), also called hip impingement, is a condition where the hip joint is not shaped normally. This causes the bones to painfully rub together. This condition can be treated with corticosteroids, nonsteroidal anti-inflammatory drugs (NSAIDs), physical therapy, rest, and surgery. FAI is caused by deformities in the femur, hip socket, or a combination of both. […]...
  9. Panners Disease Panner disease is often caused by excessive throwing due to valgus stress. The disease causes pain and stiffness in the affected elbow and may limit extension; the affected elbow is usually on the dominant arm the child uses. The disease may be associated with pitching and athletic activity. Panner’s disease is caused by stress on the capitellum […]...
  10. Kienböcks Disease; Causes, Symptoms, Treatment Kienböcks Disease, also known as Avascular Necrosis Of The Lunate, is a medical condition in which the blood supply to the lunate bone in the wrist joint is disrupted. The condition may also lead to the death of the bone. Lunate is an important bone that provides support and assists in the movement of the […]...
  11. Orthopedic Treatment For Knee Osteonecrosis Death of a bone due to loss of blood supply is referred to as Osteonecrosis. The condition mostly affects the hip joint although the knees may also get damaged. It is one of the most common causes of knee pain in women above the age of 60 years. The knee joint gets its blood supply […]...
  12. What Is Knee Joint Ligaments? – Types, Disease Condition What Is Knee Joint Ligaments?/Ligaments are bands of tough, elastic connective tissue that surround a joint to give support and limit the joint’s movement? Knee ligaments are thick strands of tissue made of collagenous fibers that connect the upper leg bones to the lower ones. There are four main knee joint ligaments: the lateral collateral […]...
  13. Movements of the Hip – Functions, Nerve Supply Movements of the Hip/The hip joint is a ball and socket joint that is the point of articulation between the head of the femur and the acetabulum of the pelvis. The joint is a diarthrodial joint with its inherent stability dictated primarily by its osseous components/articulations.  The primary function of the hip joint is to […]...
  14. Scheuermann’s Disease Scheuermann’s disease is a self-limiting skeletal disorder of childhood. Scheuermann’s disease describes a condition where the vertebrae grow unevenly with respect to the sagittal plane; that is, the posterior angle is often greater than the anterior. Symptoms of Scheuermann’s Disease include hump appearance in the back, muscle cramps or spasms, pain or stiffness in the back after […]...
  15. Quadratus Femoris – Origin, Nerve Supply, Function The Quadratus Femoris is a flat, quadrilateral skeletal muscle. Located on the posterior side of the hip joint, it is a strong external rotator and adductor of the thigh,[rx] but also acts to stabilize the femoral head in the acetabulum. Quadratus femoris use in Meyer’s muscle pedicle grafting to prevent avascular necrosis of femur head. Origin Quadratus femoris tear is an uncommon injury that usually causes […]...

To Get Daily Health Newsletter

We don’t spam! Read our privacy policy for more info.

Terms and Conditions of Use
Privacy Policy
Cookie Policy
Editorial Policy
Advertising Policy
EU User Consent Policy
Correction Policy
Citation Policy
Ethics and Logical Policies
About Us
Contact Us
Newsletter
Career
Sitemap
Advertise with us
Editorial Board Members
Review Board Member
Team RxHarun
Web Developers Team
Guest Posts and Sponsored Posts
Request for Board Member
Women Writers
Download Mobile Apps
Follow us on Social Media
© 2012 - 2025; All rights reserved by authors. Powered by Mediarx International LTD, a subsidiary company of Rx Foundation.
RxHarun
Logo