Bilateral osteonecrosis of the femoral head in an adult man affected by congenital estrogen deficiency

Osteonecrosis of femoral head is related to different predisposing factors. The pathogenesis is not completely understood, but an ischemic impairment seems to be one of the major determinants of bone necrosis. The association of bilateral necrosis of femoral heads and congenital aromatase deficiency is here reported. The absence of estrogen activity, as well as the persistence of unfused epiphyses for a long period of life, may be involved in the determinism of bilateral necrosis of bone femoral heads. The possibility of development of bone necrosis in patients affected by congenital estrogen deficiency needs to be considered and magnetic resonance imaging can be a useful method for an early detection of this disease.     

Computed tomography with multiplanar reformation and 3-dimensional image analysis in the preoperative evaluation of ischemic necrosis of the femoral head

Computed tomography (CT) with multiplanar reformation and 3-dimensional image reconstruction were utilized preoperatively to assess the extent of infarction and collapse of the femoral head in 15 patients with ischemic necrosis. Polyethylene models of the diseased femoral heads were constructed from 3-dimensional contouring of CT data, and compared with resected specimens in patients who underwent subsequent total hip arthroplasty. Information provided by this imaging strategy was valuable in both the selection and the planning of the most appropriate surgical procedure, and models were found to represent resected head configurations with reasonable accuracy. CT combined with multiplanar reformation and 3-dimensional analysis may be the optimal preoperative means for evaluating advanced ischemic necrosis involving the femoral head.     

Osteonecrosis.

Osteonecrosis (avascular necrosis, aseptic necrosis or subchondral avascular necrosis) is not a specific disease entity but the final common pathway of a number of conditions leading to bone death. The main predisposing factors (trauma, glucocorticosteroids, alcoholism and connective tissue disorders) should be carefully sought, but osteonecrosis can also be idiopathic in origin. Its most common localization is to the femoral head, followed by the humeral head, the knee and the small bones of the wrist and foot. New imaging techniques, especially magnetic resonance imaging, have improved the early diagnosis of osteonecrosis as radiographs may be normal in the initial stages. The outcome for the patient is influenced by many factors, the size and localization of the bone necrosis being of primary importance. Early intervention (both surgical and non-surgical) has definitively improved the outcome, but still nearly 50% of cases of femoral head osteonecrosis necessitate arthroplasty. In this chapter, we shall concentrate on patients at high risk of osteonecrosis, on evidence-based diagnosis, especially in the early stages, and on evidence-based treatment suited to the staging system of osteonecrosis.     

Evaluation of myocardial uptake of 99mtechnetium pyrophosphate in clinical exercise-induced ventricular ischemia.

Although acute infarction of the myocardium is known to accumulate 99mtechnetium pyrophosphate, it is not entirely clear that ischemia alone without necrosis does not result in abnormal uptake of 99mtechnetium pyrophosphate. The present study investigates whether transient myocardial ischemia is associated with localization of 99mtechnetium pyrophosphate by evaluating images obtained with the scintillation camera at rest and after exercise in 15 patients with unequivocal myocardial ischemia. All patients had angina pectoris, multivessel coronary artery stenoses by selective arteriographic studies, and electrocardiographic ischemic responses on treadmill exercise. Eleven of the 15 patients also underwent radionuclide imaging with 81rubidium at rest and after exercise; the results demonstrated scintigraphic ischemia. The scintiscans with 99mtechnetium pyrophosphate revealed no evidence of increased myocardial radioactivity after exercise compared to rest in 14 of the 15 patients. In contrast, myocardial activity was observed with 99mtechnetium pyrophosphate after treadmill exertion in the remaining patient, in whom a small subendocardial infarction appeared to have occurred with the exercise. It is concluded from these results that transient myocardial ischemia does not cause localization of 99mtechnetium pyrophosphate. These findings support the specificity of abnormal localization of 99mtechnetium pyrophosphate for acute myocardial infarction.     

Ostéonécroses aseptiques,une revue générale des ostéonécroses de la hanche,et au-delà

Avascular necrosis is an ischemic or cytotoxic necrosis of epiphyseal bone, responsible for joint pain, altered life quality and frequently affecting young patients. Avascular necrosis can be unifocal or multifocal, underlining the possibility of a systemic origin. Avascular necrosis involves the femoral head in more than 75% of cases. Although avascular necrosis is irreversible, many risk factors must be sought, including corticosteroid treatment, hypercholesterolemia, sickle cell disease or alcohol abuse. MRI imaging is the main exploration for the diagnostic and staging of the disease, and should be performed in unexplained hip pain in young patients with normal X-rays. In the earlier stages of the disease (stage I and II of the Arlet and Ficat classification), joint surface is preserved, and conservative treatment is recommended. In the more advanced stages (III and IV of the Arlet and Ficat classification), the articular surface collapses and joint arthroplasty is the main treatment. However, there are some recent therapeutic advances, based on mesenchymal stem cells, which may contribute, in the future, to improve the bad functional prognosis of the disease.     

Bone marrow edema syndromes of the hip: MRI features in different hip disorders

The objectives of this study were to describe the essential magnetic resonance imaging (MRI) features of bone marrow edema syndromes affecting the hip joint. In addition, to evaluate the role of MRI in the assessment of hip joint involvement in different clinical settings that may share similar clinical findings. Thirty-four patients who complained of hip pain were studied consecutively. Of these, 21 were men (61.8%) and 13 were women (38.2%). After clinical assessment of possible hip disease, plain radiograph and MRI study of both hips were performed. The literature was searched using keywords: bone marrow edema, hip, and MRI. All patients had antalgic gait and limping. Initial clinical examination revealed painful limited internal and external rotation of the affected hip/hips suspect for hip disease. Unilateral hip involvement was identified in 31 patients (91.2%), and bilateral hip involvement was found in three patients (8.8%), with a total of 37 hips evaluated by MRI. The final diagnoses in our patients were: reactive arthritis (1), transient osteoporosis (7), avascular necrosis (10), osteoarthritis (2), tuberculous arthritis (4), septic arthritis (2), osteomyelitis (2), sickle cell anemia (2), lymphocytic leukemia (1), and femoral stress fracture (3). Bone marrow edema affecting the hip is neither a specific MR imaging finding nor a specific diagnosis and may be encountered in a variety of hip disorders due to different etiologies. MR imaging is the modality of choice when clinical examination is suspect for hip disease and plain radiographs are normal or equivocal. Early diagnosis and treatment is important in many of the disorders. The literature is reviewed regarding bone marrow edema of the hip.     

Study of magnetic resonance imaging in transient osteoporosis of the hip

To better define the magnetic resonance imaging (MRI) features during transient osteoporosis of the hip, we carried out sequential MRI of 8 patients with transient osteoporosis of the hip showing the typical radiographic pattern of diffuse osteoporosis of the femoral head. These cases displayed the same MRI changes in the femoral head and neck characterized by low signal intensity on the T1 weighted images and high signal intensity on the T2 weighted images. The MRI signal became normal within 11 months, but surprisingly, migration of the MRI abnormalities was observed during the course of the disease in 5 cases: abnormal MRI signals were first observed in the anterior area, then migrated to the posterior part, while a normal MRI signal reappeared in the anterior part. We conclude that MRI seems to show some particular features in transient osteoporosis of the hip, which normalize after a few months of evolution; this could represent a migration of MRI abnormalities. Such findings could help in the differential diagnosis of hip diseases such as aseptic bone necrosis.     

Ischemic necrosis of bone in systemic lupus erythematosus.

Twenty-three patients with systemic lupus erythematosus (SLE) and ischemic bone necrosis are reported. All patients had received corticosteroids prior to the onset of ischemic necrosis, although one patient had received none for 13 years previously. Nineteen (83%) patients had multiple bone lesions including the femoral heads in 21 (91%) which were bilaterally involved in 15. In addition, humeral heads were affected in seven patients and the tibial plateaus, in three. The most striking feature of this group was the high incidence of Raynaud's phenomemon present in 14 (61%) of the 23 patients. Furthermore, central nervous system involvement was present in 10 (43%) patients, more prominent in those without Raynaud's (67%) than in those with vasospasm (29%). Thus, 20 of the 23 patients, or 87%, evidenced vascular abnormalities either in the form of Raynaud's phenomenon and/or systemic vasculitis.The pathogenesis of ischemic bone necrosis is discussed. In SLE, vasospasm or vasculitis, or both, augmented by corticosteroid therapy, could impede the microcirculation and result in the ischemic lesion.     

Long-term observation of avascular necrosis of the femoral head in systemic lupus erythematosus: an MRI study

OBJECTIVES: To assess long-term prognosis of clinically silent, early-stage avascular necrosis of the femoral head (ANFH) in patients with systemic lupus erythematosus (SLE). METHODS: Twenty-four hips that showed ANFH by magnetic resonance imaging (MRI) in 13 patients with SLE were studied. All hips were radiographically normal and clinically asymptomatic. The percentage volume of necrotic bone was calculated at each study by dividing the sum of the necrotic areas by the sum of the femoral head areas from all MRI slices. Hips were also classified into three categories by the relation of the necrotic area to the weight bearing portion according to the system of the Japanese Investigation Committee for avascular necrosis of the femoral head, with modifications: Type A (medial lesions): 8 hips, Type B (central lesions): 4 hips, and Type C (lateral lesions): 12 hips. Patients were followed up with MRI for 12-95 (mean 51) months. RESULTS: Fifteen hips improved (more than 15% reduction in the volume of necrosis), 5 did not change and 4 worsened during the observation period. All hips with a volume of necrotic area less than 25% showed improvement. All but one Type A hip and one Type B hip improved, while the mean volume of necrosis did not change in Type C. The volume of the necrotic area was smaller in Type A & B than in Type C hips (p < 0.001). CONCLUSIONS: Long-term prognosis of early-stage ANFH was favorable in patients with SLE when the necrotic area was small (less than 25%).     

Cushing's disease presenting with avascular necrosis of the hip: an orthopedic emergency.

Nontraumatic avascular necrosis (AVN) of the hip is commonly caused by exogenous glucocorticoid administration, whereas it has rarely been associated with endogenous hypercortisolism. We report a 30-yr-old woman with Cushing's disease whose presenting manifestation was early AVN of the hip. Although plain x-ray was negative, magnetic resonance imaging (MRI) of the hip showed stage 2 AVN. Her orthopedic disease was considered an emergency, and thus, it was treated with core decompression before the diagnosis of Cushing's syndrome (CS) was pursued further. The femur recovered fully, as demonstrated by her improved clinical picture and a subsequent MRI. AVN carries a poor prognosis, if not treated early. The diagnostic procedure of choice is MRI, because plain radiographs are falsely negative in early stages. This case illustrates that AVN can be the presenting manifestation of CS; to prevent irreversible effects on the femoral head, core decompression should not be delayed for the purpose of evaluation and treatment of CS.     

Relationship between magnetic resonance imaging and histologic findings by bone biopsy in nontraumatic osteonecrosis of the femoral head.

To attest the validity of magnetic resonance imaging (MRI) to evaluate the pathophysiology in nontraumatic osteonecrosis (ON) of the femoral head, we attempted to correlate the different MRI patterns with the histology in cases of early stages. We used not only the T1 and T2 pulse sequences, but also the T1 sequence after gadolinium-DTPA to demonstrate the presence of vascularization. Studying 24 core biopsies from 16 hips (9 patients), we explored the MRI presentations that corresponded to the main histologic findings of the different trabecular bone and bone marrow conditions. Histologic findings including trabecular bone necrosis and bone marrow necrosis represented by amorphous eosinophilic debris presented a low T1 signal intensity without enhancement after intravenous gadolinium injection and a low T2 signal intensity. Trabecular bone necrosis associated with mummified fat cells presented a normal T1 and T2 signal intensity. Trabecular bone necrosis with fibrosis filling the intertrabecular spaces had a low T1 signal intensity that was enhanced by gadolinium and had an intermediate T2 signal intensity. Bands of fibrosis without trabecular bone as seen in fracture zones showed a low T1 signal intensity that was enhanced by gadolinium with a high T2 signal intensity. Thickened trabecular bone with fibrosis as found in creeping substitution areas had also a low T1 signal enhanced by gadolinium, but the T2 signal intensity was low. Farther from the necrotic area, despite normal trabecular bone, we found some patchy necrosis of the bone marrow without any modification of the normal T1 and T2 MRI patterns.(ABSTRACT TRUNCATED AT 250 WORDS)     

Apoptosis of osteocytes in glucocorticoid-induced osteonecrosis of the hip

An increase in osteoblast and osteocyte apoptosis has been demonstrated in mice and humans receiving glucocorticoids and may be involved in the pathogenesis of the associated osteonecrosis. To examine the spatial relationship between osteocyte apoptosis and glucocorticoid-induced osteonecrosis, we determined the prevalence of osteocyte apoptosis in whole femoral heads obtained from patients who underwent prosthetic hip replacement because of osteonecrosis due to chronic glucocorticoid treatment (n = 5), alcoholism (n = 3), and trauma (n = 1) as well as in femoral neck cores from patients with sickle cell disease (n = 5). Abundant apoptotic osteocytes and cells lining cancellous bone were found juxtaposed to the subchondral fracture crescent in femurs from the patients with glucocorticoid excess. In contrast, apoptotic bone cells were absent from the specimens taken from patients with trauma or sickle cell disease and were rare with alcohol abuse. These results indicate that glucocorticoid-induced osteonecrosis is a misnomer. The bone is not necrotic; instead, it shows prominent apoptosis of cancellous lining cells and osteocytes. Glucocorticoid-induced osteocyte apoptosis, a cumulative and irreparable defect, could uniquely disrupt the mechanosensory function of the osteocyte network and thus start the inexorable sequence of events leading to collapse of the femoral head.     

99Tc-亚甲基二膦酸盐治疗股骨头坏死动物研究及初步临床应用

目的 地塞米松诱导兔股骨头坏死后用^99Tc-亚甲基二膦酸盐治疗并观察疗效，以供初步临床应用于中、晚期股骨头无菌性坏死参考，了解^99Tc-亚甲基二膦酸盐的治疗价值。方法 新西兰白兔33只为动物模型制作材料，其中18只采用注射地塞米松针剂建立股骨头坏死模型，模型建立后分成3组，每组6只；正常对照组、股骨头坏死模型组和股骨头坏死模型^99Tc-亚甲基二膦酸盐治疗组。^99Tc-亚甲基二膦酸盐治疗14周后取实验兔股骨头作病理切片,分析^99Tc-亚甲基二膦酸盐治疗前、后股骨头X线、CT摄片、^99Tc-MDP骨显像检查结果。临床明确诊断为股骨头无菌性坏死患者67例，分为^99Tc-亚甲基二膦酸盐治疗组39例和传统保守治疗对照组28例。治疗40周后比较^99Tc-MDP骨显像、X线摄片、CT检查，同时结合患者的症状作出综合评价。结果采用肌肉注射地塞米松针剂4周的新西兰白兔病理证实股骨头骨质疏松伴坏死模型成立。在^99Tc-亚甲基二膦酸盐治疗前、后时间内取各组实验兔股骨头X线、CT摄片、^99Tc-MDP骨显像和病理分析比较：正常对照组无变化，股骨头坏死模型组对照组股骨头骨质疏松加重，模型^99Tc-亚甲基二膦酸盐治疗组的股骨头骨质疏松有较明显的改善。临床所采用^99Tc-亚甲基二膦酸盐治疗组40周随访结果：^99Tc-亚甲基二膦酸盐治疗组中6例改善，31例稳定，仅2例无效。常规保守治疗对照组6例稳定，19例无效，仅3例改善。结论 ^99Tc-亚甲基二膦酸盐是一种核素锝-99(^99Tc)标记二膦酸盐的化合物，动物试验结果显示对骨质疏松有较明显的稳定病情发展和镇痛作用，部分有修复作用。初步临床应用表明：^99Tc-亚甲基二膦酸盐治疗组与传统保守治疗对照组比较，前者治疗骨头无菌性坏死疗效较明显。     

Medullary impairment at early stage of non-traumatic osteonecrosis of the femoral head

Summary We found that a painful hip without radiological changes presented a modification of the magnetic resonance image of the femoral head and neck with diffuse low T1 weighted and high T2 weighted signal. Core biopsy showed that the bone marrow was replaced from the neck to the head of the femur by fibroblastic tissue and edema without detectable trabecular bone impairment. Classical radiographic, CT, and MRI abnormalities of osteonecrosis of the femoral head were present 14 months later. This report suggested that diffuse femoral head and neck medullary impairment with fat cell replacement by fibroblastic tissue without evidence of trabecular bone necrosis can be found at the early stages of idiopathic osteonecrosis.     

Detection of unstable angina by 99m technetium pyrophosphate myocardial scintigraphy.

99mTechnetium stannous pyrophosphate has been shown to accumulate in acutely infarcted myocardium. To determine if the isotope is also taken up by severely ischemic, but not necrotic myocardium, we performed myocardial scintigraphic studies in 17 patients with chest pains. Seven of the patients satisfied conventional clinical, electrographic, and laboratory criteria for the diagnosis of unstable angina and showed no electrocardiographic or enzymatic evidence of myocardial necrosis. Five of these seven patients with unstable angina demonstrated abnormal localized patterns, and one showed a borderline picture. Myocardial scintiscans were normal in all of a control group of ten patients with stable angina. Thus, scanning with 99mtechnetium stannous pyrophosphate is shown to be of value in the objective demonstration of myocardial abnormality in unstable angina.     

The effect of intertrabecular fluid on femoral head mechanics

The effect of intraosseous fluid on the mechanical characteristics of bone is unclear. To determine the potential effects of alteration in the fluid boundary conditions of the femoral head, a decompression procedure was performed. The stiffness of femoral heads from normal adult mongrel dogs was measured after the heads were subjected to a physiologic mechanical load. When the fluid boundary conditions were altered by drilling the femoral neck, femoral head stiffness was reduced by more than 30% (p = 0.045). Refilling of the femoral specimen with saline restored the stiffness to the baseline value. These observations demonstrate that in vitro changes of fluid boundary conditions can alter the mechanical integrity of the femoral head. Alteration in osseous fluid boundary conditions in vivo could affect joint integrity and be of pathogenetic significance in joint disease.     

Osteonecrosis of the Femoral Head: A Proposed New Treatment in Homozygous Sickle Cell Disease

Possibilities for bone reconstruction in osteonecrosis of the femoral head in sickle cell hemoglobinopathies before the end of growth have not been assessed. The aim of our study was to evaluate the morphological and functional results in 11 osteonecrosis of the femoral head because of homozygous sickle cell disease. Surgical treatment consisted of a triple acetabular osteotomy in seven cases, femoral varus osteotomy in two cases and a combination of both in two cases. The severity of the osteonecrosis was evaluated on radiographs and magnetic resonance imaging (MRI), pre- and postoperative, and function of the hip joint was assessed by Harris and Postel Merle d’Aubigné scores. The anesthetic part (pre- and postoperative) and eventual complications were collected and analyzed. The maximum follow-up was between 1 to 9.6 years. All patients were considered to be satisfactory at maximum follow-up. The severity of the initial damage was not associated with any morphological or functional outcome at maximum follow-up. All patients had an objective functional gain. We found no general complications. We proposed a decision tree for the patient’s management of avascular necrosis of the femoral head in sickle cell hemoglobinopathies before bone maturity, and with an Arlet and Ficat’s stage of 3 or 4. The results of this study confirmed the interest of conservative surgical treatment in children with homozygous sickle cell anemia in case of osteonecrosis of the femoral head. Level of evidence: IV (cases series of our department).     

Long term effectiveness of low dose mithramycin for Paget's disease of bone.

Nineteen patients with Paget's disease of bone were studied 7 months to 5 years after therapy with mithramycin in a dose averaging 11.5 microgram/kg body weight daily for 10 days. Thirteen patients, including 3 with the longest followup intervals, remained free of pain. Objective measures of disease activity (serum alkaline phosphatase level and 99mtechnetium pyrophosphate bone scan) were less favorable. There was no evidence of long term toxicity.     

The value of dipyridamole-technetium-99m carbomethoxyisopropylisonitrile (CPI) myocardial tomography imaging in diagnosing coronary artery disease

Fifty cases of clinically proven or suspected coronary artery disease (CAD) underwent dipyridamole-technetium-99m CPI (DP-99mTc. CPI) myocardial tomography imaging and coronary angiography. All cases with angiographically proven CAD had positive DP-99 mTc. CPI. The sensitivity was 100%. Six of 13 cases with normal coronary arteriogram showed false positive results (3 cases of hypertrophic cardiomyopathy and 3 cases of chest pain with unknown causes). Therefore, the specificity was 53.8% (7/13). The positive predictive accuracy of DP-99m Tc. CPI myocardial imaging was 88.0%. However, We do not consider it justified to apply this statistics to general population as our patients were highly selected. One hundred and twelve myocardial segments of left ventricle were shown to be infarcted or ischemic by either radionuclear imaging or ECG. In 108 segments with abnormal images, only 10 in patients with hypertrophic cardiomyopathy had normal coronary artery supply. Therefore, the reliability of DP-99 mTc. CPI myocardial imaging to display infarcted or ischemic segments was 91.0% (102/112). The ability for 99mTc. CPI imaging to differentiate between infarcted and ischemic lesions was somewhat indefinite, especially in case of localized infarction. The reason of this shortcoming was discussed. Ninety segments were shown to be infarcted or ischemic by ECG, 4 of which had no corresponding coronary artery stenosis. However, in 12 of the 22 segments with normal ECG pattern the corresponding coronary arteries were either occluded or stenosed, resulting in 54.5% (12/22) false negativity. Most of these false negatives were found in posterior and septal walls. Angina pectoris after dipyridamole infusion occurred in 4 of our 50 patients.(ABSTRACT TRUNCATED AT 250 WORDS)     

IMAGING STUDY ON THE MODE OF DEVELOPMENT AND CHANGES IN AVASCULAR NECROSIS OF THE FEMORAL HEAD IN SYSTEMIC LUPUS ERYTHEMATOSUS: LONG-TERM OBSERVATIONS

To find out when and how avascular necrosis of the femoral head(ANFH) develops in patients with SLE who are receiving corticosteroids,we carried outa 3-yr prospective study using imaging methods.Twenty-three patients with SLE who were free of hip pain andradiographically negative were enrolled in the study. Initially,abnormal findings characteristic of ANFH were detected in eight(35%) and six (26%) patients on MRI and radionuclide uptakebone scanning, respectively. During a 3-yr observation periodabnormalities on MRI, bone scanning and radiography were foundin four, fiveand four patients, respectively and three patientsdeveloped hip pain. Analyses of imaging changes demonstratedthat only a small proportion of patients (2/8, 25%) who initiallyshowed abnormal MRI progressed to beradiographically positivefor ANFH over the 3 years. High doses of corticosteroids overthe 3-yr period were found to be responsible for the emergenceof change in MRI abnormalities. It is also suggested that abnormalMRI findings tend to develop either within a relatively shortinterval after the start of corticosteroid treatment or areassociated with the exacerbation of SLE. KEY WORDS: Magnetic resonance imaging, Radionuclide uptake scanning, Radiography, Prospective study, Corticosteroids, Exacerbation of systemic lupus erythematosus