Avascular Necrosis (AVN)--Causes, symptoms, treatment options

AVN--also called osteonecrosis--is a progressive bone disorder that can require potentially significant surgical treatment. For anyone at risk of developing AVN, awareness is the best defense.

AVN is a well-named condition. "Avascular necrosis" literally means cell death resulting from the loss of blood supply. The disease is also referred to as "osteonecrosis", which literally means that bone tissue cells are dying ("osteo-" means bone).

As these names imply, AVN can develop following a reduction in blood supply to an area of bone. Bone is living tissue, and requires good blood circulation just like any other tissue. As the affected region of bone dies, it becomes brittle, and susceptible to breakage. Usually long bones (such as the thighbone) are affected, and the dying area is typically at the end of the bone, near the joint.

In its early stages, AVN sufferers may or may not experience joint pain. Joint pain can certainly have many causes, many of which are not serious. To diagnose AVN, an MRI (magnetic resonance imaging) scan is usually performed, because X-rays will only detect the late stages of AVN.

The time from the onset of symptoms until the function of the joint is irrevocably lost can be as short as several months, or well over a year, in the absence of interventions to slow the progression of AVN (see below). This time course is probably as short as it is in part because symptoms may not be present during the very early stages of AVN; in these cases, symptoms (i.e., joint pain) fails to appear until the bone loss has already progressed somewhat.

Late stage AVN can only be described as debilitating. In most cases, late stage AVN also involves arthritis in the affected areas.

Causes and risk factors

In the U.S., approximately 10,000 to 20,000 new cases of AVN are diagnosed each year, commonly in people between 30 and 50 years of age (more frequently in men than women). Given that approximately 84 million people in that age range live in the U.S., the overall prevalence in the general population can be estimated at no more than 0.02%.

Known risk factors for AVN

The following have been associated with an increased risk of developing AVN in the general population:

  • Bone fractures
  • Corticosteroid use
  • Alcohol use
  • Elevated cholesterol/triglycerides
  • Sickle cell anemia
  • Dysbaric conditions (the "bends", decompression disease)
  • Gaucher's disease
  • Pancreatitis
  • Lupus

Because AVN develops following the reduction of the blood supply to the bone, risk factors for AVN generally include conditions that reduce the blood supply. Alcohol dependency and the use of corticosteroids are thought to cause this by leading to the formation of fat deposits in the end region of arteries in the bone. Elevated cholesterol and triglycerides, as well as Gaucher's disease (a chemical defect that leads to the abnormal accumulation of fatty substances) probably also increase the risk of AVN in a similar fashion. AVN is actually the third most common complication of long bone fractures, because the fracture itself can lead to mechanical disruptions of the blood flow to bone. Sickle cell anemia is thought to predispose people to AVN because the blood becomes more viscous.

The "bends" have been implicated in the development of AVN in scuba divers, but the mechanism remains unclear.

Managing AVN

The best way of approaching AVN clinically is to prevent its occurrence in the first place--anyone with the above-mentioned risk factors should take measures to reduce them. AVN is a progressive disorder, and once it has developed to the latter stages, joint replacement surgery becomes necessary. This is a fairly significant procedure, so managing AVN before reaching late stages is best.

For patients with early stage AVN, several options are available. Mechanical approaches to reducing the stress on the affected bone can sometimes both provide pain relief and slow the progression of AVN.

Surgery (less radical than joint-replacement) during early-stage AVN can also stave off the progression of the disorder. Specifically, three types of procedures may be performed:

  • Core Decompression: As the name implies, this procedure aims to relieve pressure on the interior of the bone, which in turn encourages improved blood flow to affected areas. This is accomplished by drilling into the bone and removing the dead and dying bone.
  • Osteotomy: The affected bone is cut and realigned in such a way as to relieve stress to the bone.
  • Bone graft: Healthy bone is grafted onto a joint following core decompression surgery.

These procedures unfortunately do not cure AVN.

AVN risk factors and HIV

Before 1996, there were only 38 case reports of AVN in HIV+ people that were published in the medical literature. This does not mean that there were only 38 diagnosed cases, only that these were all the cases that were described in the literature. However, the fact that AVN had not received much attention from HIV clinicians is indicative of its rarity in this population.

A group at Georgetown University Medical Center reported at the 6th Conference on Retroviruses and Opportunistic Infections (Feb. 1999, Chicago, IL) that 8 of their HIV+ patients had AVN--since they had only about 600 patients, this represented a prevalence rate of over 1%! Of course, there is no way to know how representative this patient group is of the HIV-infected population at large. However, this rate is over 50-fold higher than that for the general population.

If the incidence of AVN is truly increasing among the HIV infected, what could be the culprit? Given the small population of HIV+ AVN sufferers discussed in published reports, it is very difficult to discern clearly what had predisposed these patients to AVN. One possibility is that AVN could be a direct or indirect consequence of recently-developed treatment options that are now routinely prescribed for HIV+ individuals. Alternatively, the reduction in HIV mortality that has resulted from the widespread use of cocktail therapies in this country could be unmasking AVN as a complication of long-term HIV infection.

Furthermore, some conventionally-accepted risk factors probably apply to a fair number of HIV-infected people, particularly those on treatment for HIV infection. In recent years, it has become clear that antiretroviral therapy can cause elevated triglycerides and cholesterol, and some specific medications (e.g., ddI) are associated with pancreatitis. Additionally, many HIV+ individuals receive steroid treatments in addition to their antiretroviral drugs. It remains to be determined whether the side effects of HIV therapies predispose patients to AVN.

A final word of caution

No one should consider stopping any of their medications on their own; anyone with concerns about the treatments they are receiving should discuss these concerns with their physician. Anyone who stops or reduces his/her medication on his/her own runs the serious risk of developing viral drug resistance and other health problems.

For more information on AVN, you may call the Arthritis Foundation at 800/283.7800. The National Institute of Arthritis and Musculoskeletal and Skin Diseases has an informative article on their website at www.nih.gov/niams/healthinfo/avnecqa.htm.

 

 

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