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Osteonecrosis

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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NORD is very grateful to John H. Klippel, MD, President and CEO, Arthritis Foundation, for assistance in the preparation of this report.

Synonyms of Osteonecrosis

Disorder Subdivisions

General Discussion

Osteonecrosis, also known as avascular necrosis, is a disease resulting from the temporary or permanent loss of the blood supply to the bones. Without blood, the bone tissue dies and causes the bone to collapse. If the process involves the bones near a joint, it often leads to collapse of the joint surface. This disease also is known, aseptic necrosis, and ischemic bone necrosis.

Although it can happen in any bone, osteonecrosis most commonly affects the ends (epiphysis) of long bones such as the femur, the bone extending from the knee joint to the hip joint. Other common sites include the upper arm bone, knees, shoulders, and ankles. The disease may affect just one bone, more than one bone at the same time, or more than one bone at different times. Orthopaedic doctors most often diagnose the disease.

The amount of disability that results from osteonecrosis depends on what part of the bone is affected, how large an area is involved, and how effectively the bone rebuilds itself. The process of bone rebuilding takes place after an injury as well as during normal growth. Normally, bone continuously breaks down and rebuilds--old bone is reabsorbed and replaced with new bone. The process keeps the skeleton strong and helps it to maintain a balance of minerals. In the course of osteonecrosis, however, the healing process is usually ineffective and the bone tissues break down faster than the body can repair them. If left untreated, the disease progresses, the bone collapses, and the joint surface breaks down, leading to pain and arthritis.

Symptoms

In the early stages of osteonecrosis, patients may not have any symptoms. As the disease progresses, however, most patients experience joint pain--at first, only when putting weight on the affected joint, and then even when resting. Pain usually develops gradually and may be mild or severe. If osteonecrosis progresses and the bone and surrounding joint surface collapse, pain may develop or increase dramatically. Pain may be severe enough to limit the patient's range of motion in the affected joint. In some cases, particularly those involving the hip, disabling osteoarthritis may develop. The period of time between the first symptoms and loss of joint function is different for each patient, ranging from several months to more than a year.

Causes

Osteonecrosis has several causes. Loss of blood supply to the bone can be caused by an injury (trauma-related osteonecrosis or joint dislocation) or by certain risk factors (nontraumatic osteonecrosis), such as some medications (steroids), blood coagulation disorders, or excessive alcohol use. Increased pressure within the bone also is associated with osteonecrosis. The pressure within the bone causes the blood vessels to narrow, making it hard for the vessels to deliver enough blood to the bone cells.

Injury
When a joint is injured, as in a fracture or dislocation, the blood vessels may be damaged. This can interfere with the blood circulation to the bone and lead to trauma-related osteonecrosis. Studies suggest that this type of osteonecrosis may develop in more than 20 percent of people who dislocate their hip joint.

Steroid Medications
Corticosteroids such as prednisone are commonly used to treat diseases in which there is inflammation, such as systemic lupus erythematosus, rheumatoid arthritis, inflammatory bowel disease, and vasculitis. Studies suggest that long-term, systemic (oral or intravenous) corticosteroid use is associated with 35 percent of all cases of nontraumatic osteonecrosis.

However, there is no known risk of osteonecrosis associated with the limited use of steroids. Patients should discuss concerns about steroid use with their doctor.

Doctors aren't sure exactly why the use of corticosteroids sometimes leads to osteonecrosis. They may interfere with the body's ability to break down fatty substances. These substances then build up in and clog the blood vessels, causing them to narrow. This reduces the amount of blood that gets to the bone.

Some studies suggest that corticosteroid-related osteonecrosis is more severe and more likely to affect both hips (when occurring in the hip) than osteonecrosis resulting from other causes.

Alcohol Use
Excessive alcohol use and corticosteroid use are two of the most common causes of nontraumatic osteonecrosis. In people who drink an excessive amount of alcohol, fatty substances may block blood vessels, causing a decreased blood supply to the bones that results in osteonecrosis.

Other Risk Factors
Other risk factors or conditions associated with nontraumatic osteonecrosis include Gaucher's disease, pancreatitis, radiation treatments and chemotherapy, decompression disease, and blood disorders such as sickle cell disease.

Affected Populations

Osteonecrosis usually affects people between 30 and 50 years of age; about 10,000 to 20,000 people develop osteonecrosis each year. Osteonecrosis affects both men and women and affects people of all ages. It is most common among people in their thirties and forties. Depending on a person's risk factors and whether the underlying cause is trauma, it also can affect younger or older people.

Related Disorders

Symptoms of the following disorder may be similar to those of Osteonecrosis. Comparisons may be useful for a differential diagnosis:

Osteopetrosis is a combination of several rare genetically caused symptoms grouped together as one disorder. It can be inherited as either a dominant or recessive trait and is marked by increased bone density, brittle bones, and in some cases skeletal abnormalities. Although symptoms may not initially be apparent to people with mild forms of this disorder, trivial injuries may cause bone fractures due to abnormalities of the bone. The dominantly transmitted form is milder than the recessive form and may not be diagnosed until adolescence or adulthood when symptoms first appear. More serious complications occur in the recessive form which may be diagnosed from examination of skeletal x-rays during infancy or childhood. (For more information on this disorder, choose "Osteopetrosis" as your search term in the Rare Disease Database.)

The following disorders may be associated with the development of Osteonecrosis. They are not necessary for a differential diagnosis:

Vasculitis is a common disorder characterized by an inflammation of the blood vessel walls. This inflammation causes a narrowing of the inside of the vessel and can obstruct the flow of blood to the tissues (ischemia). The lack of blood may cause damage to the tissues (necrosis), possible formation of blood clots (thrombosis), or a weakening or ballooning which can possibly cause a rupture of the vessel wall (aneurysm). Osteonecrosis can occur in people with vasculitis when blood flow has been obstructed to the bone or joint. (For more information on this disorder, choose "Vasculitis" as your search term in the Rare Disease Database.)

Rheumatoid Arthritis is an inflammatory autoimmune disease in which the body's natural defenses against foreign agents (antibodies & lymphocytes) attack healthy joints. This disorder is characterized by a lack of appetite (anorexia), fatigue, painful and deformed joints, early morning stiffness chiefly in the hands, knees, feet, jaw, and spine. People with Arthritis are at increased risk of also developing Osteonecrosis. (For more information on this disorder, choose "Arthritis" as your search term in the Rare Disease Database.)

Lupus (also known as SLE or Systemic Lupus Erythematosus) is an inflammatory connective tissue disease that can affect many parts of the body including the joints, skin and internal organs. Lupus is a disease of the body's immune system, most often striking young women between the ages of 15 and 35 years. People who have Lupus are also at increased risk of developing Osteonecrosis. (For more information on this disorder, choose "Lupus" as your search term in the Rare Disease Database.)

Legg-Calve-Perthes Syndrome is a rare disease affecting the hip joint. Abnormalities in bone growth early in life may result in permanent deformity of the hip joint several years later. Osteonecrosis of the femoral head is often seen in those people with Legg-Calve-Perthes Disease. (For more information on his disorder, choose "Legg-Calve-Perthes" as your search term in the Rare Disease Database.)

Gaucher's Disease is an inherited disease of lipid metabolism caused by the failure to produce the enzyme glucocerebrosidase. It is the most common of the 14 known lipid storage disorders which includes Tay-Sachs, Fabry's Disease, and Niemann-Pick Disease. There are three types of Gaucher's Disease Type I, II and III. All three are characterized by the presence of Gaucher (lipid-laden) cells in the bone marrow and other organs such as the spleen and liver. Bone deterioration (Osteonecrosis) is a major symptom of this disease and can affect any part of the body. (For more information on this disorder, choose "Gaucher" as your search term in the Rare Disease Database.)

Sickle Cell Disease is an inherited blood disease. Symptomatic of this disease are the "crisis periods" which often occur in conjunction with other infections. It is characterized by joint pain (arthralgia), fever, severe abdominal pain, vomiting, and damage to the head of the femur bone (osteonecrosis). (For more information on this disorder, choose "Sickle Cell" as your search term in the Rare Disease Database.)

Polycythemia Vera is a chronic proliferative disorder of the bone marrow. It is characterized by an increase in the number of red blood cells (erythrocytosis) and hemoglobin concentration in the blood. Osteonecrosis can occur in Polycythemia Vera. (For more information on this disorder, choose "Polycythemia Vera" as your search term in the Rare Disease Database.)

Caisson Disease, also called "Decompression Sickness" or the "Bends", is a disorder caused by the formation of nitrogen bubbles in the tissues and blood. This occurs from a very rapid reduction of air pressure after rising quickly from deep water with high atmospheric pressure, to normal air pressure. It is characterized by painful joints, bone deterioration (Osteonecrosis), chest tightness, giddiness, abdominal pain, vomiting and visual difficulties. In some with this disorder there may also be convulsions and paralysis.

Standard Therapies

Diagnosis
After performing a complete physical examination and asking about the patient's medical history (for example, what health problems the patient has had and for how long), the doctor may use one or more imaging techniques to diagnose osteonecrosis. As with many other diseases, early diagnosis increases the chances of treatment success.

It is likely that the doctor first will recommend a radiograph, commonly called an x ray. X rays can help identify many causes of joint pain, such as a fracture or arthritis. If the x ray is normal, the patient may need to have more tests.

Research studies have shown that magnetic resonance imaging, or MRI, is the most sensitive method for diagnosing osteonecrosis in the early stages. The tests described below may be used to determine the amount of bone affected and how far the disease has progressed.

X Ray
An x ray is a common tool that the doctor may use to help diagnose the cause of joint pain. It is a simple way to produce pictures of bones. The x ray of a person with early osteonecrosis is likely to be normal because x rays are not sensitive enough to detect the bone changes in the early stages of the disease. X rays can show bone damage in the later stages, and once the diagnosis is made, they are often used to monitor the course of the condition.

Magnetic Resonance Imaging (MRI)
MRI is quickly becoming a common method for diagnosing osteonecrosis. Unlike x rays, bone scans, and CT (computed/computerized tomography) scans, MRI detects chemical changes in the bone marrow and can show osteonecrosis in its earliest stages. MRI provides the doctor with a picture of the area affected and the bone rebuilding process. In addition, MRI may show diseased areas that are not yet causing any symptoms.

Bone Scan
Also known as bone scintigraphy, bone scans are used most commonly in patients who have normal x rays. A harmless radioactive dye is injected into the affected bone and a picture of the bone is taken with a special camera. The picture shows how the dye travels through the bone and where normal bone formation is occurring. A single bone scan finds all areas in the body that are affected, thus reducing the need to expose the patient to more radiation. Bone scans do not detect osteonecrosis at the earliest stages.

Computed/Computerized Tomography
A CT scan is an imaging technique that provides the doctor with a three-dimensional picture of the bone. It also shows "slices" of the bone, making the picture much clearer than x rays and bone scans. Some doctors disagree about the usefulness of this test to diagnose osteonecrosis. Although a diagnosis usually can be made without a CT scan, the technique may be useful in determining the extent of bone damage.

Biopsy
A biopsy is a surgical procedure in which tissue from the affected bone is removed and studied. Although a biopsy is a conclusive way to diagnose osteonecrosis, it is rarely used because it requires surger

Functional Evaluation of Bone
Tests to measure the pressure inside a bone may be used when the doctor strongly suspects that a patient has osteonecrosis, despite normal results of x rays, bone scans, and MRIs. These tests are very sensitive for detecting increased pressure within the bone, but they require surgery.

Treatment
Appropriate treatment for osteonecrosis is necessary to keep joints from breaking down. If untreated, most patients will experience severe pain and limitation in movement within 2 years.

Several treatments are available that can help prevent further bone and joint damage and reduce pain. To determine the most appropriate treatment, the doctor considers the following aspects of a patient's disease:

* The age of the patient
* The stage of the disease--early or late
* The location and amount of bone affected--a small or large area
* The underlying cause of osteonecrosis--with an ongoing cause such as corticosteroid or alcohol use, treatment may not work unless use of the substance is stopped.

The goal in treating osteonecrosis is to improve the patient's use of the affected joint, stop further damage to the bone, and ensure bone and joint survival. To reach these goals, the doctor may use one or more of the following treatments.

Conservative Treatment
Medicines--to reduce fatty substances (lipids) that increase with corticosteroid treatment or to reduce blood clotting in the presence of clotting disorders. Nonsteroidal anti-inflammatory drugs may also be prescribed to reduce pain.

Reduced weight bearing - If osteonecrosis is diagnosed early, the doctor may begin treatment by having the patient remove weight from the affected joint. The doctor may recommend limiting activities or using crutches. In some cases, reduced weight bearing can slow the damage caused by osteonecrosis and permit natural healing. When combined with medication to reduce pain, reduced weight bearing can be an effective way to avoid or delay surgery for some patients.

Range-of-motion exercises - may be prescribed to maintain or improve joint range of motion.

Electrical stimulation - to induce bone growth.

Conservative treatments have been used experimentally alone or in combination. However, these treatments rarely provide lasting improvement. Therefore, most patients will eventually need surgery to repair the joint permanently.

Surgical Treatment
Core decompression - This surgical procedure removes the inner layer of bone, which reduces pressure within the bone, increases blood flow to the bone, and allows more blood vessels to form. Core decompression works best in people who are in the earliest stages of osteonecrosis, often before the collapse of the joint. This procedure sometimes can reduce pain and slow the progression of bone and joint destruction in these patients.

Osteotomy - This surgical procedure reshapes the bone to reduce stress on the affected area. There is a lengthy recovery period, and the patient's activities are very limited for 3 to 12 months after an osteotomy. This procedure is most effective for patients with advanced osteonecrosis and those with a large area of affected bone.

Bone graft - A bone graft may be used to support a joint after core decompression. Bone grafting is surgery that transplants healthy bone from one part of the patient, such as the leg, to the diseased area. Commonly, grafts (called vascular grafts) that include an artery and veins are used to increase the blood supply to the affected area. There is a lengthy recovery period after a bone graft, usually from 6 to 12 months. This procedure is complex and its effectiveness is not yet proven. Clinical studies are under way to determine its effectiveness.

Arthroplasty/total joint replacement - Total joint replacement is the treatment of choice in late-stage osteonecrosis and when the joint is destroyed. In this surgery, the diseased joint is replaced with artificial parts. It may be recommended for people who are not good candidates for other treatments, such as patients who do not do well with repeated attempts to preserve the joint. Various types of replacements are available, and people should discuss specific needs with their doctor.

For most people with osteonecrosis, treatment is an ongoing process. Doctors may first recommend the least complex and invasive procedure, such as protecting the joint by limiting movement, and watch the effect on the patient's condition.

Other treatments then may be used to prevent further bone destruction and reduce pain. It is important that patients carefully follow instructions about activity limitations and work closely with their doctor to ensure that appropriate treatments are used.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Osteonecrosis Resources

Organizations:

References

TEXTBOOKS
Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:453-54.

Bennett JC, Plum F, eds. Cecil Textbook of Medicine. 20th ed. W.B. Saunders Co., Philadelphia, PA; 1996:1387.

REVIEW ARTICLES
Lieberman JR, Berry DJ, Mont MA, et al. Osteonecrosis of the hip: management in the 21st century. Instr Course Lect. 2003;52:337-55.

Ferlic RJ, Lee DH, Lopez-Ben RR. Pediatric Kienbock's disease: case report and review of the literature. Clin Orthop. 2003;408:237-44.

Assouline-Dayan Y, Chang C, Greenspan A, et al. Pathogenesis and natural history of osteonecrosis. Semin Arhritis Rheum. 2002;32:94-124.

Schmidt AH, Swiontkowski MF. Femoral neck fractures. Orthop Clin North Am. 2002;33:97-111.

Low K, Mont MA, Hungerford DS. Steroid-associated osteonecrosis of the knee: a comprehensive review. Instr Course Lect. 2001;50:489-93.

JOURNAL ARTICLES
Kocher MS, Mandiga R, Murphy JM, et al. A clinical practice guideline for treatment of septic arthritis in children: efficacy in improving process of care and effect on outcome of septic arthritis of the hip. J Bone Joint Surg Am. 2002;85-A:994-99.

Wang Y, Li Y, Mao K, et al. Alcohol-induced adipogenesis in bone and marrow: a possible mechanism for osteonecrosis. Clin Orthop. 2003;410:213-24.

Tauchmanova L, De Rosa G, Serio B, et al. Avascular necrosis in long-term survivors after allogenic or autologous stem cell transplantation: a single center experience and review. Cancer. 2003;97:2453-61.

Stulberg BN. Osteonecrosis: What to do, what to do! J Arthroplasty. 2003;18 (3 Suppl 1):74-79.

Brotons A, Penarrocha M. Neurogenic pain and maxillofacial ischemic osteonecrosis. A review. Med Oral. 2003;8:157-65.

Pogrel MA, Miller CE. A case of maxillary necrosis. J Oral Maxillofac Surg. 2003;61:489-93.

Johns LC, Mont MA, Le TB, et al. Procoagulants and osteonecrosis. J Rheumatol. 2003;30:783.

FROM THE INTERNET
Osteonecrosis. MEDLINEplus. Last Updated: 04 June 2003. 2pp.
www.nlm.nih.gov/medlineplus/osteonecrosis.html

Questions and Answers about Avascular Necrosis. National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). January, 2001. 7pp.
www.niams.nih.gov/hi/topics/avasculat_necrosis/index.htm

Center for Osteonecrosis Research and Education (CORE).
www.osteonecrosis.org

Osteonecrosis. NOF and CORE. nd. 9pp.
www.ostonecrosis.org/nofbrochure/nonf-brochure.htm

Legg-Calve-Perthes Disease. National Osteonecrosis Foundation (NOF). nd. 5pp.
www.nonf.org/perthesbrochure/perthes_brochure.htm

Osteonecrosis (Avascular Necrosis). The Arthritis Foundation. nd. 2pp.
www.arthriti.org/conditions/DiseaseCenter/osteonecrosis.asp

Osteonecrosis Symptoms. Arthritis-Symptom.com. nd. 3pp.
www.arthritis-symptom.com/osteonecrosis-symptoms/

Your Orthopedic Connection.
Osteonecrosis of the hip. October 2000. 2pp.
www.Orthoinfo.aaos.org/fact/thr_report.cfm?thread_id=237&topcategory=hip

Report last updated: 2009/06/11 00:00:00 GMT+0