Familial vanishing limbs: four generations of idiopathic multicentric osteolysis

A family is described which has exhibited in four generations a bizarre form of arthritis mutilans and osteolysis, the features of which seem to fit most closely with a diagnosis of hereditary multicentric osteolysis, a subgroup of idiopathic multicentric osteolysis. The differential diagnosis of arthritis mutilans associated with osteolysis is discussed; this includes a wide variety of disorders ranging from rheumatoid arthritis to rare conditions such as the Winchester syndrome.     

Does osteolysis of the distal clavicle occur following spinal cord injury?

 Objective. To determine whether there is an association between spinal cord injury and ”atraumatic” osteolysis of the distal clavicle. Patients and design. Seventy-seven consecutive spinal cord injury patients without upper extremity injuries were studied. Of these, 39 each had a pair of chest radiographs – one at admission and one at least 1 month later – which included both acromioclavicular joints. Each radiograph was evaluated by two independent musculoskeletal radiologists, who were masked as to which radiograph was the baseline. Results. Ten of 76 acromioclavicular joints demonstrated osteolysis of the distal clavicle: three bilateral, three left side only, and one right side only. There was one case in which the admission radiograph was read as osteolysis but reverted to normal at 35 months follow-up. Conclusion. There is an apparent association between spinal cord injury and osteolysis of the distal clavicle.     

Radiographic texture analysis in the characterization of trabecular patterns in periprosthetic osteolysis

RATIONALE AND OBJECTIVES: Periprosthetic osteolysis is a disease attributed to the body's reaction to fine polyethylene wear debris shed from total hip replacements. The purpose of this preliminary study was to investigate the ability of radiographic texture analysis (RTA) to characterize the trabecular texture patterns on pelvic images for osteolysis and normal total hip arthroplasty (THA) cases. MATERIALS AND METHODS: Fourier-based and fractal-based texture features were calculated for a database of digitized radiographs from 202 THA cases, 70 of which developed osteolysis. The features were calculated from regions of interest selected at two time points: less than 1 month after surgery, and at the first clinical indication of osteolysis (or randomly selected follow-up time for normal cases). Receiver operating characteristic (ROC) analysis was used to compare feature performance at baseline and follow-up for osteolysis and normal cases. RESULTS: Separation between the RTA features for osteolysis and normal cases was negligible at baseline and increased substantially for the follow-up images. The directional Fourier-based feature provided the best separation with an A(z) value from ROC analysis of 0.75 for the follow-up images, in the task of distinguishing between normal and osteolytic cases. CONCLUSIONS: The results from this preliminary analysis indicate that qualitative changes in trabecular patterns from immediately after surgery to the eventual detection of osteolysis correspond to quantitative changes in RTA features. It therefore appears that RTA provides information that could potentially be useful to aid in the detection of this disease.     

Focal osteolysis in total hip replacement: CT findings

Objective To describe the CT findings of focal osteolysis following total hip replacement (THR).Design and patients CT imaging features of 30 THRs with focal osteolysis visualized on follow-up radiographs and undertaken revision surgery were reviewed. On CT scans, the shape and anatomic location of osteolytic lesions was recorded, as well as their size and number. The presence of cortical disruption or expansion, liner wear, metallosis, and heterotopic ossification was also noted. In each case, surgical and histologic findings were correlated with imaging features.Results Focal osteolysis was common in the superior part of acetabular and femoral components. CT features of focal osteolysis were multiple, expansile, oval, or round radiolucencies, which were conglomerated into multilobular shape. The cortex adjacent to the osteolytic lesions revealed irregular thinning and discontinuity (29/30, 97%) accompanied by a few tiny fragments. Liner wear was common (27/30, 90%), and metallosis was frequent (8/30, 26.7%). Pathologic results were foreign body reaction in 20 patients, chronic inflammation in eight, and fibrosis in two.Conclusion CT appearances of focal osteolysis following THR are multilobulated lucent areas with expansile periosteal reaction and cortical abnormalities, mimicking infection or tumor. We consider that CT is useful for the prediction and assessment of the nature and extent of focal osteolysis.     

T1-weighted MR imaging for distinguishing large osteolysis of Paget's disease from sarcomatous degeneration

Objective. To report five symptomatic patients, four with unequivocal Paget’s disease and large areas of osteolysis and one patient with presumed osteolytic Paget’s disease, evaluated by MR imaging to confirm or exclude a sarcoma. Design and patients. Four men and one woman (median age 74 years) presented with new symptoms of pain. Four of these patients had unequivocal Paget’s disease with large areas of osteolysis; one patient presented with large focal osteolysis and no other finding. MR imaging was performed in each case to exclude malignancy in the area of osteolysis. Results. Two patients whose MR images showed a low signal abnormality on the T1-weighted sequence corresponding to osteolysis on the radiograph were found to have malignant degeneration. Three patients with osteolytic lesions on T1-weighted MR imaging showed preservation of fat signal in the areas of osteolysis, were not biopsied and have been free of malignant disease for from 12 months to 2¹/₂ years. One patient had one area of osteolysis in the iliac bone which showed malignancy and another area of osteolysis which showed preservation of fat signal on the T1-weighted sequence. Conclusions. The information obtained from T1-weighted MR imaging sequences performed on patients with Paget’s disease who have new symptoms and large areas of osteolysis could reliably be used in the clinical decision-making process between conservative follow-up and biopsy. Received: 26 December 2000 Revision requested: 16 January 2001 Revision received: 05 February 2001 Accepted: 07 February 2001     

Idiopathic multicentric osteolysis.

A 59 year old while female with striking carpal and tarsal osteolysis is reported. The process primarily involves the carpals and tarsals but includes other sites. The pertinent literature is reviewed and a simplified classification of idiopathic multicentric osteolysis is presented. Idiopathic multicentric osteolysis may be sporadic but can demonstrate either dominant or recessive inheritance. There may or may not be associated nephropathy. The underlying mechanism appears similar in all variations of this condition. Gorham's disease, the Winchester syndrome, and other osteolytic conditions which may predominantly affect the tarsal and carpal bones most likely are totally different processes, unrelated to idiopathic multicentric osteolysis.     

A Case of an Idiopathic Massive Osteolysis with Skip Lesions

A patient with a 2-year history of pain in the left arm, and decreased strengths unrelieved by non-steroidal anti-inflammatory therapy, was being referred for repeating radiography. The radiologic examinations have demonstrated a unique pattern of non-contiguous osteolysis in the left elbow, proximal and distal radius, ulna, wrist, carpal bones, proximal and distal metacarpals and phalanges. Multi-site biopsies were being performed and confirmed the diagnosis of massive osteolysis. To our knowledge, this is the first case in which multifocal, non-contiguous osteolysis with skip lesions without associated nephropathy and without a hereditary pattern is being described in one extremity.     

Idiopathic progressive osteolysis of craniofacial bones

 We present the case report of a diabetic 51-year-old male, who presented with painless mobilization of the superior incisor teeth. The X-ray study showed osteolysis, without any soft tissue mass, in craniofacial and orbital bones. In the control X-ray studies performed during the 2 years’ evolution, a progressive increase in the intensity and extension of the osteolysis was observed. The diagnosis of progressive idiopathic osteolysis is one of exclusion, based mainly on clinico-radiological findings, evolution and compatible histological findings.     

Magnetic resonance imaging of the postoperative hip

Magnetic resonance imaging (MRI) is ideally suited to imaging the patient with painful hip arthroplasty due to its superior soft tissue contrast, multiplanar capabilities, and lack of ionizing radiation. MRI is the most accurate imaging modality in the assessment of periprosthetic osteolysis and wear-induced synovitis, and can also assess regional tendons and neurovascular structures. This article discusses the technical aspects of MRI around metallic implants as well as the appearance of potential complications following hip arthroplasty, including osteolysis, wear-induced synovitis, infection, hemarthrosis, fracture, loosening, component displacement, heterotopic ossification, tendinopathy, and neurovascular impingement. The specific complication of metal hypersensitivity following metal-on-metal prostheses is reviewed.     

Post-traumatic and stress-induced osteolysis of the distal clavicle: MR imaging findings in 17 patients

OBJECTIVE: To describe the MR imaging findings in patients with osteolysis of the distal clavicle and to compare the MR imaging appearance of clavicular osteolysis following acute injury with that related to chronic stress. DESIGN AND PATIENTS: MR imaging examinations were reviewed in 17 patients (14 men, 3 women; ages 16-55 years) with the diagnosis of post-traumatic or stress-induced osteolysis of the clavicle. A history of a single direct injury was present in seven patients and a history of weight-lifting, participation in sports, or repetitive microtrauma was present in 10 patients. RESULTS: MR imaging showed edema in the distal clavicle in 17 patients and, of these, eight also had edema in the acromion. The edema was most evident in STIR and fat-suppressed T2-weighted pulse sequences. Other findings about the acromioclavicular (AC) joint were prominence of the joint capsule in 14, joint fluid in eight, cortical irregularity in 12, and bone fragmentation in six patients. No differences in the MR imaging features of post-traumatic and stress-induced osteolysis of the distal clavicle were observed. CONCLUSION: Post-traumatic and stress-induced osteolysis of the distal clavicle have similar appearances on MR imaging, the most common and conspicuous MR imaging feature being increased T2 signal intensity in the distal clavicle.     

Familial expansile osteolysis—not exclusively an adult disorder

Familial expansile osteolysis (FEO, MIM174810) is a rare syndrome which was observed world-wide in only three kinships and in two unrelated American individuals. We report a patient with familial expansile osteolysis from the Czech Republic, not related to the previously reported cases. This patient’s extraordinary clinical course does not conform to the ordinary. Her radiographic bone involvement was unusually extensive, involving most of the peripheral skeleton and the skull. This case documents that familial expansile osteolysis is not only a disease of adults but does occur in childhood.     

Anatomo-radiological study of a case of post-radiotherapeutic osteolysis of the hip.

Anatomo-radiological study of a case of osteolysis of the femoral head and neck in a 56-year old male who had recieved, 22 years earlier, an intense radiotherapeutic treatment for a soft tissue tumour of the corresponding hip region. The treatment is considered as being the main etiological factor responsible for the bone lesions. The latter seems to have been favored by an indolent joint due to a thoracic cordotomy for post-irradiation pains; it may have been prepared by a secondary osteodystrophy which preceded the radiotherapy and seems due to two previous surgical excisions.     

创伤后骨质溶解症X线图像特征

目的 探讨创伤后骨质溶解症的X线表现,提高对该病认识.方法 回顾性分析经临床、病理证实的7例创伤后骨质溶解症X线表现.7例中女5例,男2例.年龄9～56岁,平均34岁.车祸3例,摔伤4例.受伤后距骨质溶解时间分别为18个月1例,10个月2例,6个月2例,3个月2例.结果 受伤时X线示坐、耻骨骨折2例,胫腓骨下段骨折1例,股骨颈骨折1例,肱骨外科颈粉碎性骨折1例,肩锁关节Ⅰ度脱位1例,腕部软组织肿胀1例.X线表现为块状骨质溶解3例,斑片状骨质溶解3例,囊状骨质溶解1例;溶解边缘骨质均无硬化,其中溶解区界限清楚5例,界限不清楚2例;溶解区无残留骨组织4例,残留部分骨组织3例;溶解区附近骨质均未见骨膜增生和骨皮质增厚;骨质修复2例,溶解骨质无修复5例.病理检查显示病变区见大量毛细血管和纤维组织增生,滑膜充血肿胀、增生,肉芽组织增生,骨坏死,破骨细胞活跃,少量炎性细胞,无肿瘤细胞.结论 创伤后骨质溶解症与外伤密切相关,X线表现为块状和斑片状骨质溶解或不规则骨质囊状破坏,早期诊断较难.     

Seventeen year follow-up and autopsy findings in a case of massive osteolysis

Clinical, radiologic, and pathologic findings in an unusual case of massive osteolysis are described. Peculiar clinical episodes of a foul body odor with fever and erythematous skin lesions, presence of radiographic findings such as acro-osteolysis and periarticular erosions, and the absence of angiomatous or lymphangiomatous dysplasias at autopsy, indicate that this is a new form of massive osteolysis rather than a variant of Gorham disease.     

Acro-osteolysis occurring in a patient with idiopathic multicentric osteolysis

The first report of acro-osteolysis occurring in a patient with idiopathic multicentric osteolysis is presented. In addition to lysis of the shafts and tufts of the distal phalanges, erosions developed in the interphalangeal, metacarpophalangeal, wrist, hip, knee, ankle, and metatarsophalangeal joints. Acroosteolysis can be part of the spectrum of bony erosions present with idiopathic multicentric osteolysis. A modification of a previous classification of idiopathic multicentric osteolysis is presented based upon a review of the literature.     

Malignant fibrous histiocytoma at the site of a total hip replacement: review of the literature and case report

 Tumors developing in association with metallic implants are being reported with increasing frequency in the orthopedic literature. The authors report the tenth case of malignant fibrous histiocytoma associated with a total hip replacement. The diagnosis was made during an evaluation for rapid radiographic osteolysis surrounding the femoral component detected less than 1 year after revision of a hemiarthroplasty to a total hip replacement. The short latency period and aggressiveness of this tumor mimicking rapid osteolysis prompted a review of the literature, etiology and significance of implant-associated malignant fibrous histiocytoma.     

Carpal-tarsal osteolysis with elbow involvement

Carpal-tarsal osteolysis is a rare condition that manifests as the progressive resorption of carpal and tarsal bones in young children. The diagnosis of this condition is often difficult and delayed as the initial clinical presentation is non-specific. Radiographic changes occur gradually, are often not seen at presentation and depend on recognising loss of bone in the ossification centres of the carpus and tarsus. MRI demonstrates morphological abnormalities in the cartilaginous, as well as the osseous components, of the developing carpal and tarsal bones and therefore may be helpful in predating the radiographic changes. Ultrasound appears to contribute little to the diagnosis and may be misleading. Exclusion of other conditions, particularly juvenile idiopathic arthritis, is important in making the diagnosis. MRI can be useful in excluding an inflammatory arthropathy, and suggesting the diagnosis of carpal-tarsal osteolysis.     

Insufficiency fracture of the sternum caused by osteopenia: plain film findings in seven patients

Sternal insufficiency fractures, whether nonbuckling (displaced or nondisplaced) or buckling, are rare and have been described in elderly osteopenic patients with accentuated kyphosis of the thoracic spine. We retrospectively analyzed the radiographs and clinical records in seven osteopenic patients with sternal insufficiency fractures and correlated the type of insufficiency fracture with the presence and degree of dorsal kyphosis, as well as the presence of chest pain, soft-tissue mass, and osteolysis. We found that displaced or nondisplaced nonbuckling fractures (five patients) may be associated with chest pain localized to the sternum (three patients), and may be present with (three patients) or without (two patients) associated thoracic kyphosis. This type of insufficiency fracture also may be accompanied by a soft-tissue mass and osteolysis (three patients), findings simulating pathologic fractures. All buckling insufficiency fractures (two patients) were associated with thoracic kyphosis and were asymptomatic. Our findings suggest that sternal insufficiency fractures may occur with or without exaggerated dorsal kyphosis. The fractures are an uncommon complication of osteopenia and may resemble pathologic fractures when osteolysis and soft-tissue swelling are present.     

Radiologic findings in Gorham-Stout syndrome

Gorham-Stout syndrome, also called "disappearing bone disease" or idiopathic massive osteolysis, is a rare disease of unknown etiology and pathogenesis. It is characterized by rapidly progressive localized massive osteolysis associated to a proliferation of vascular structures of benign origin in which the absence of new bone formation is representative. We present two cases (a six-year-old boy with affected right ulna, and a 15-year-old girl with right rib affection associated to chylothorax) of this disease with the characteristic clinical, histological, and imaging findings.     

Severe osteolysis and soft tissue mass around total hip arthroplasty: description of four cases and review of the literature with respect to clinico-radiographic and pathologic differential diagnosis

Periprosthetic osteolysis is a well known phenomenon caused by wear particle-induced bone resorption, particularly common and extensively reported in total hip arthroplasty. Its typical radiographic feature is a radiolucent area adjacent to an implant, sometimes associated with a soft tissue mass. Osteolytic changes may be caused by numerous other pathologic processes, including infection, metabolic disease, and neoplasia. Four cases of massive periprosthetic bone destruction associated with a large soft tissue mass around a failed total hip replacement are presented. In three cases, a diagnosis of periprosthetic osteolysis was correctly made and managed by revision surgery. However, in one case angiosarcoma of the ipsilateral hemipelvis went long unrecognized despite aggressive clinical course, requiring hind-quarter amputation and ultimately resulting in the patient's death. Periprosthetic malignancy in the form of either primary sarcoma or metastatic cancer is a very rare yet reported event in the setting of previous hip replacement, likely leading to catastrophic consequences when diagnosis is not established in a timely manner. The differential diagnosis of periprosthetic osteolysis should consider the entire spectrum of conditions that can present with radiolucent changes. Thorough review of patient's history and course of symptoms, along with careful evaluation of standard roentgenograms should be always performed and possibly integrated with imaging modalities such as CT, MRI, and bone scintigraphy in order to increase diagnostic accuracy. If uncertainty remains, biopsy should always be considered to rule out malignancy.