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Avascular Necrosis
A Case History and Literature Review
Curtis J. Wolfe, MD;
Kenneth L. Taylor-Butler, MD
Arch Fam Med. 2000;9:291-294.
ABSTRACT
We describe a patient with avascular necrosis in both shoulders. Confirmatory testing in making the diagnosis included plain radiography, bone scan, and magnetic resonance imaging. The pathogenesis and staging of the disease by radiography are presented in the article. Treatment options include a conservative regimen of shoulder range of motion exercises and nonsteroidal anti-inflammatory agents or surgery (arthroplasty or core decompression). The patient's risk factors include long-term corticosteroid use, smoking, and alcohol consumption. Other known risk factors include sickle cell disease, Gaucher disease, chemotherapy, lymphoma, dysbaric conditions, and trauma. This literature search shows that prevention and early diagnosis lend the best outcomes for the diagnosis of avascular necrosis.
INTRODUCTION
A 64-year-old African American woman initially presented with complaints of bilateral shoulder pain in May 1998. The patient stated that she had noticed the pain for almost 1 year. Initially, the chronic aching pain began in the right shoulder directly over the humeral head and then developed in the left shoulder 1 month later. Raising her arms above shoulder level exacerbated the pain. The patient also noticed progressive weakness with overhead movements. Pain and restricted range of motion prevented her from performing activities of daily living (eg, combing her hair). Findings from physical examination showed pertinent abnormalities of decreased active range of motion (secondary to pain) in shoulder abduction (85° bilaterally) while passive range of motion equaled 135° in the right shoulder and 120° in the left shoulder. Her forward flexion was also limited actively (85° bilaterally) and passively (135° bilaterally), and there was decreased symmetrical strength in abduction. During palpation of the greater tubercle of the humeral heads, the patient complained of mild tenderness bilaterally. Otherwise, the remainder of her strength, range of motion, and findings from neurovascular examinations were unremarkable.
The patient's medical history included the diagnosis of colon cancer in June 1996. She had surgery to remove the adenocarcinoma that had metastasized to the lung and liver. She finished 3 rounds of a 14-day regimen of continuous systemic or intrahepatic fluorouracil. Those treatments commenced at the time of diagnosis of colon adenocarcinoma and concluded in spring 1997. The patient also had a history of right lower extremity cellulitis that led to sepsis in March 1998, gastritis, normocytic anemia, cervical radiculopathy, and chorioretinitis. The chorioretinitis was diagnosed in 1992 and treated with oral corticosteroids. She used a cumulative corticosteroid total dose of 18 g throughout 6 years. Her initial list of medications at the time of presentation included aspirin, folic acid, famotidine, and nonsteroidal anti-inflammatory drugs. Notable findings from her social history included a 50 pack-year smoking habit and 1 to 2 alcoholic drinks per night for several years.
A plain x-ray film of the patient's shoulders showed notable sclerotic and cystic changes bilaterally with flattening of the left humeral head (Figure 1). These radiographic changes, along with the patient's history, led us to the diagnosis of stage III avascular necrosis (AVN) in the left shoulder and stage IV in the right shoulder. The consulted orthopedic surgeon presented 2 options: maximize her range of motion and strength with exercises and analgesic medication or surgery to replace her shoulder joints. Our patient declined to take the risk of surgery. She chose the conservative treatment option. This treatment included stretching exercises, avoiding repetitive strenuous overhead arm motions, and analgesic medications.
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Radiographs of the patient's shoulders.
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PATHOPHYSIOLOGIC CHARACTERISTICS
Avascular necrosis represents a compromised circulation of blood to an area of bone.1 Eventually the involved area of bone dies and necrosis develops. The mechanisms for the impedance of flow include intraluminal obstruction (thromboembolism, sludging of blood cells, or stasis), vascular compression (vasospasm), and the physical disruption of the vessel. The femoral head and the humeral head are the joints most often affected by AVN. Complications of AVN include incomplete fracture and superimposed degenerative arthritis. True arthritis appears in nearly all the affected bones in patients with AVN stage V. This arthritis often occurs along the reactive interface at which extensive resorption and fibrous replacement has occurred. Sometimes a subchondral fracture occurs (pseudoarthritis) and leads to chondroid metaplasia.2-8
RISK FACTORS
Some common risk factors and associations for AVN can be elicited from the patient's medical history. One of the most common risk factors in the United States involves the use of oral corticosteroids. The risk increases more with the cumulative corticosteroid total dose rather than the daily dose.9 Other risk factors include alcohol use, systemic lupus, sickle cell disease, Gaucher disease, dysbaric conditions, trauma, cancer, and even some idiopathic cases.1, 7, 10
Hurel and Kendall-Taylor9 found that the risk of AVN increased significantly when the cumulative corticosteroid total dose is considered. The lowest reported total dose of corticosteroid use was 480 mg in 1 month.9 In one study by Gogas and Fennelly,11 the average cumulative corticosteroid total dose associated with the development of AVN was 4.32 g. Other factors considered were the high-dose corticosteroids used in chemotherapy regimens or intra-articular injections.9, 12
The period of time before symptoms arose varied. Gogas and Fennelly11 found that AVN could appear as early as 2 months or as late as 78 months after initiation of corticosteroid treatment. However, some patients studied by Hurel and Kendall-Taylor9 remained symptom free until 2 years after stopping corticosteroid use. Schlumpf and Vonwil13 describe an occurrence of AVN after the treatment of chorioretinitis with corticosteroids. Two young subjects were diagnosed with AVN. Each of the subjects had the disease in the femoral head after the use of oral and/or injectable (intrabulbar) corticosteroids.
Data suggest that corticosteroids are associated with a high serum lipid content, increased fat cell mass, and increased intraosseous pressure.1, 6-9,11-12,14-18 These circumstances are thought to cause microscopic fat emboli to lodge in the endarteries of bone.7 Corticosteroids then induce osteoporosis/osteomalacia with subsequent microfracture that ultimately lead to AVN. Some chemotherapy regimens contribute to AVN. The chemotherapy treatments for ovarian cancer, lymphoma, and acute leukemia often include the use of high-dose corticosteroids. Though these regimens do have a maintenance phase of corticosteroid treatment, some studies found patients developed symptoms 5 to 6 months after the 1-month induction phases of high-dose corticosteroids.11, 14-15,19
The consumption of alcohol is another factor to consider in the development of AVN. It has been documented that alcohol consumption causes fatty liver. Thus, one theory states that a fatty liver can lead to fat emboli forming and lodging in the endarteries supplying the bones.1, 7-8 This embolic event leads to increased intraosseous or intramedullary pressure and increased lipid content. The bone then develops ischemic necrosis.1-2,7 Some hereditary diseases have been associated with AVN. Two such diseases are sickle cell disease and Gaucher disease. Sickle cell disease is the most common cause of AVN throughout the world. In sickle cell disease, the development of AVN is thought to be due to the increased blood viscosity secondary to the sludging of the sickled red blood cells. An article by David et al20 describes a similar incidence of AVN in the head of the femur and humerus in patients with sickle cell disease. They also showed a 50% functional deficit in the shoulder, but only 26% of those patients showed evidence of disease on radiographic scan. Patients with sickle cell disease also tend to have more complications from surgical treatment.10
Gaucher disease also damages the blood cells. This hereditary disease causes an abnormal accumulation of glucocerebroside in the vessels, leading to increased intraosseous pressures. Damaged macrophages may cause an angiospasm of the vessels, leading to bone infarction. This infarction causes acute vascular crisis leading to fracture in vertebral bodies and long bones.10, 21 Trauma to the long bones is also correlated with the development of AVN. A simple fracture to the long bones can disrupt the blood supply to certain areas of the bone. Thus, AVN is the third most common complication of long bone fractures in the humeral head after stiffness and malunion.
Hyperbaric exposure secondary to trauma is another cause of AVN to the humeral head. In professional divers and caisson workers, dysbaric AVN is an occupational hazard. However, even in amateur divers there seems to be an increasing number of cases of AVN. The incidence of dysbaric AVN increases with the depth, pressure, and number of dives. The pressure of the dive is related to the dive location. There is no known pathogenesis to this type of AVN. One theory considers the increase in helium in the gas mixtures as a possible culprit.22
DIAGNOSIS AND STAGING
Staging of AVN can be decided by several methods (Table 1).7, 23 Findings from plain radiography can identify the latter stages of the disease. Magnetic resonance imaging (MRI) is the most sensitive and acceptable standard noninvasive diagnostic method at any stage. Magnetic resonance imaging used early in testing can be diagnostic or confirmatory.1, 3, 5-7,9, 11, 14-15,21, 23 The bone normally should have a homogenous intensity on MRI. Avascular necrosis shows a classic "double-line sign" on MRI. This double-line sign represents a high-signal intensity line within 2 parallel rims of decreased signal intensity on a T2-weighted MRI image. This difference in intensities is thought to represent the reparative interface between necrotic and living bone.24 Findings from a bone scan can help confirm the diagnosis.1-3,5, 7-8,10-11,23, 25-26 "Cold lesions" seen on bone scan are representative of a decreased uptake of the radioactive anion that has been distributed.
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Staging of Avascular Necrosis by Plain Radiography and Bone Scan
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TREATMENT
Currently there is no cure for AVN. Two treatment options are apparent: conservative treatment (range of motion exercises and anti-inflammatory medicines) or surgery. Conservative treatment is effective for patients across the range of severity of disease. One small study of patients using the conservative treatment option showed that 50% were satisfied with the outcome.23 Of those who were satisfied with conservative treatment, 40% had AVN stage I/II, and 60% had AVN stage III/IV.8, 23
Some patients with AVN of the humeral head may choose a more invasive treatment regimen. One controversial technique involves the core decompression of the humeral head, which is drilling into the humeral head and removing the necrotic and osteopenic areas. One study showed this technique had excellent results as defined by decreased pain, increased strength, and increased range of motion for patients in AVN stage I/II.23 In one report,25 nearly 73% of patients with AVN stage III reported good and/or excellent reports after 5 years. For patients with AVN stage IV, core decompression delayed the need for arthroplasty for up to 5 years. However, it is still unknown whether there is any long-term benefit of core decompression in halting the natural course of the disease.4, 8, 23, 25
Another surgical treatment option, arthroplasty, replaces the humeral head and/or the glenoid fossa with an artificial joint. Between 91% and 100% of patients with AVN stage III or higher who underwent shoulder arthroplasty reported good pain relief.1, 5, 8, 21 However, those patients with AVN stage III/IV who also have Gaucher disease or sickle cell disease seem to have less favorable results after undergoing arthroplasty.1, 20-21
The best outcome for patients with AVN would entail early diagnosis and cessation of exacerbating factors, if possible. Otherwise, this disease shows progressive damage radiographically and clinically. Overall, there is no cure for this disease; however, with treatment, symptoms can be minimized.
AUTHOR INFORMATION
Accepted for publication October 28, 1999.
Dr Wolfe would like to acknowledge his residency academic advisor, Anne K. Sly, MD; the Trinity Lutheran Family Practice Residency faculty for their general support, led by Scott Thompson, MD, and Tom Maddox, MD; C. Craig Satterlee, MD, Greg Reuter, MD, Mike Parsa, MD, and Desi Bravo, medical librarian at Trinity Lutheran Hospital, for their technical help; and his wife, Annie Wolfe, BS, for her contributions and support.
Corresponding author: Curtis J. Wolfe, MD, 1704 Commercial Cir, Wamego, KS 66547 (e-mail: cwolfe{at}stormontrail.org).
From the Trinity Lutheran Family Medicine Center, Kansas City, Mo.
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