eMedicine Specialties > Rheumatology > Metabolic and Bone Disease

Avascular Necrosis

Author: Jeanne K Tofferi, MD, MPH, FACP, Assistant Chief, Department of Rheumatology, Walter Reed Army Medical Center
Coauthor(s): William Gilliland, MD, MPHE, FACP, FACR, Staff Rheumatologist, Walter Reed Army Medical Center; Professor of Medicine, Assistant Dean of Curriculum, Uniformed Services University of the Health Sciences
Contributor Information and Disclosures

Updated: Dec 17, 2009

Introduction

Background

Avascular necrosis (AVN) is defined as cellular death of bone components due to interruption of the blood supply; the bone structures then collapse, resulting in bone destruction, pain, and loss of joint function. AVN is associated with numerous conditions and usually involves the epiphysis of long bones, such as the femoral and humeral heads and the femoral condyles, but small bones can also be affected. In clinical practice, AVN is most commonly encountered in the hip. Recently, AVN of the jaw associated with bisphosphonate use has also been described.1

Early diagnosis and appropriate intervention can delay the need for joint replacement. However, most patients present late in the disease course. Without treatment, the process is almost always progressive, leading to joint destruction within 5 years. Patients taking corticosteroids and organ transplant recipients are particularly at risk of developing AVN. Most available data regarding the natural history, pathology, pathogenesis, and treatment of AVN pertains to femoral head necrosis.

Pathophysiology

Although the pathophysiology of AVN is not fully understood, the final common pathway is interruption of blood flow to the bone. AVN affects bones with a single terminal blood supply, such as the femoral head, carpals, talus, and humerus. These bones have limited collateral circulation. Interruption of the vascular supply and resultant necrosis of marrow, medullary bone, and cortex are theorized to be caused by the mechanisms listed below. However, individual patients usually have more than one risk factor; this indicates that the pathogenesis of AVN is likely multifactorial.

  • Vascular occlusion: This is characterized by the interruption of the extraosseous blood supply via factors such as direct trauma (eg, fracture, dislocation), nontraumatic stress, and stress fracture.
  • Altered lipid metabolism: Animal studies have led to the hypothesis that increased levels of serum lipids leads to lipid deposition in the femoral head, causing femoral hypertension and ischemia.2 Lipid-level–lowering drugs in animals reverse this process. Corticosteroid administration was associated with fat emboli in the femoral heads of rabbits.3
  • Intravascular coagulation: Disorders of the coagulation system have been implicated in the pathogenesis of AVN. Typically, it is a secondary event triggered by a familial thrombophilia, hypercholesterolemia, allograft organ rejection, other disorders (eg, infection, malignancy), or pregnancy.
  • Healing process: Necrotic bone triggers a process of repair that includes osteoclasts, osteoblasts, histiocytes, and vascular elements. Osteoblasts build new bone on top of the dead bone, leading to a thick scar that prevents revascularization of the necrotic bone, with resultant abnormal joint remodeling and joint dysfunction.
  • Primary cell death: Osteocyte death without other features of AVN has been seen in renal transplant patients, as well as in patients receiving steroids and those who consume significant amounts of alcohol.
  • Mechanical stress: Animal studies have shown an association between increased weight bearing and an increased incidence of AVN of the femoral head.

Frequency

United States

The frequency of AVN depends on the site involved. The most common site is the hip; other locations include the carpals, talus, femur, metatarsal, mandible, and humerus. In the United States, approximately 15,000 new cases of AVN are reported each year. AVN accounts for more than 10% of total hip replacement surgeries performed in the United States. Most recently, 380 cases of osteonecrosis of the jaw associated with bisphosphonate use have been reported. Most patients with osteonecrosis of the jaw also had an ongoing malignancy and/or had undergone a recent dental procedure.4,1

International

In most countries, the incidence and prevalence of AVN are unknown. A Japanese survey estimated that 2500-3300 cases of AVN of the hip occur each year; of these, 34.7% were due to corticosteroid use, 21.8% to alcohol abuse, and 37.1% to idiopathic mechanisms.5 A study from France reported AVN in 4.3% of allogenic bone marrow transplant recipients.6

Mortality/Morbidity

Data on mortality rates associated with AVN are not available. Most data involve AVN of the hip. Mortality rates are very low and vary based on the operative procedure used to treat AVN.

Morbidity rates are high and depend on the underlying cause. Morbidity rates associated with AVN of the hip are high; the prevalence of long-term disability is significant. Despite advances in orthopedic procedures, most patients with advanced AVN require more than one hemiarthroplasty or total hip replacement during their lifetime.

Race

AVN has no racial predilection except for cases associated with sickle cell disease and hemoglobin S and SC disease, which predominantly occur in people of African and Mediterranean descent.

Sex

With the exception of AVN associated with systemic lupus erythematosus, AVN is more common in men, with an overall male-to-female ratio of 8:1.

Age

AVN is a disease of middle age that most often occurs during the fourth or fifth decade of life and is bilateral in 55% of cases.

Clinical

History

  • Avascular necrosis (AVN) may be asymptomatic and is occasionally discovered incidentally on radiographs. Symptoms depend on the affected joint. Medullary infarcts are usually silent, and infarcts of the small bones of the hands and feet are often symptomatic.
  • Pain in the affected joint is typically the presenting symptom of AVN, regardless of the location. Patients with AVN of the femoral head often report groin pain that is exacerbated by weight bearing. The pain may initially be mild but progressively worsens over time and with use. Eventually, the pain is present at rest and may be present at night.
  • Large infarcts, such as those due to Gaucher disease and hemoglobinopathies, are associated with very severe pain.

Physical

  • Initially, the physical examination findings of AVN may be unrevealing. Abnormal physical findings depend on the location and severity of disease. With progression of AVN of the hip, joint function deteriorates and the patient may walk with a limp. AVN of smaller, non–weight-bearing joints typically does not cause significant disability.
  • Patients with AVN may have tenderness around the affected bone.
  • Both active and passive joint movements may be restricted and painful.
  • A neurologic deficit may be present if a nerve is affected (compressed) because of necrosis and compression deformity of affected bones.
  • Advanced AVN can cause joint deformity and muscle wasting.

Causes

AVN is associated with several clinical conditions. The following etiological factors have been identified:

  • Primary or idiopathic
  • Secondary or associated with an underlying condition
    • Trauma
    • Systemic corticosteroid use or Cushing disease
    • Alcohol abuse
    • Systemic lupus erythematosus (with or without antiphospholipid syndrome), as well as other connective-tissue diseases
    • Hematologic (sickle cell disease, hemoglobinopathies)
    • Metabolic (hyperlipidemia, gout, renal failure)
    • Orthopedic disorders (slipped capital femoral epiphysis, congenital dysplasia of the hip, Legg-Calve-Perthes disease)
    • Infection (osteomyelitis, HIV infection [controversial])
    • Renal transplantation
    • Radiation therapy
    • Pancreatitis (uncommon)
    • Gaucher disease
    • Malignancy (marrow infiltration, malignant fibrous histiocytoma)
    • Caisson disease
    • Pregnancy
    • Bisphosphonate use

More on Avascular Necrosis

Overview: Avascular Necrosis
Differential Diagnoses & Workup: Avascular Necrosis
Treatment & Medication: Avascular Necrosis
Follow-up: Avascular Necrosis
Multimedia: Avascular Necrosis
References
Further Reading
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Keywords

avascular necrosis, AVN, osteonecrosis, avascular necrosis of the hip, AVN of the hip, avascular necrosis of the jaw, AVN of the jaw, corticosteroid-induced AVN, corticosteroid-induced avascular necrosis, aseptic necrosis, ischemic necrosis, femoral head necrosis, total hip arthroplasty, THA, core decompression, bone graft, bone grafting, osteotomy

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Contributor Information and Disclosures

Author

Jeanne K Tofferi, MD, MPH, FACP, Assistant Chief, Department of Rheumatology, Walter Reed Army Medical Center
Jeanne K Tofferi, MD, MPH, FACP is a member of the following medical societies: Alpha Omega Alpha and American College of Physicians
Disclosure: Nothing to disclose.

Coauthor(s)

William Gilliland, MD, MPHE, FACP, FACR, Staff Rheumatologist, Walter Reed Army Medical Center; Professor of Medicine, Assistant Dean of Curriculum, Uniformed Services University of the Health Sciences
Disclosure: Nothing to disclose.

Medical Editor

Bryan L Martin, DO, Chief, Allergy Immunology Department, Walter Reed Army Medical Center; Associate Professor of Medicine and Pediatrics, Uniformed Services University of the Health Sciences; United States Army Consultant in Allergy Immunology and Immunizations
Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, and American Osteopathic Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center
Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; UCB  Speaking and teaching; Omnicare Consulting fee Consulting; Centocor Consulting fee Consulting; Roche Grant/research funds Other

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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