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At least 1,225 records · Page 68Linked to original sources
PubMed1995

Centrifugal osteopetrosis: appendicular sclerosis with relative sparing of the vertebrae.

A previously unreported pattern of bone involvement in autosomal dominant osteopetrosis is described in three affected members of a Vietnamese kindred. The hands, feet, distal bones, and skull were markedly osteosclerotic on radiologic examination. The vertebrae displayed very minimal thickening of the endplates in two of the cases, and no visible involvement in the third. The vertebral bone density (assessed by dual-energy X-ray absorptiometry) was markedly elevated, suggesting that the spine was not completely spared. This pattern contrasts with the predominantly axial distribution of the recognized variants of osteopetrosis.

Adolescent↗
PubMed1995

MR findings of avulsive cortical irregularity of the distal femur.

Avulsive cortical irregularity, a benign condition occurring only among children and adolescents, has been known to simulate malignancy not only radiologically but also microscopically. Therefore, in addition to plain radiographs, further studies including by magnetic resonance (MR) imaging may occasionally be required. MR images of seven cases of avulsive cortical irregularity of the femur were reviewed. In all cases, the lesion appeared hypointense on T1-weighted images and hyperintense on T2-weighted images, with a dark rim on both sequences at or near the sites of the bony attachment of the medial head of the gastrocnemius muscle. In all cases, bilateral involvement was demonstrated by plain radiography, computed tomography, and/or MR imaging. The authors suggest that avulsive cortical irregularity involves both femora much more frequently than has been reported previously.

Adolescent↗
PubMedDate not supplied

A new motor-driven percutaneous bone biopsy system: technical note.

A new coaxial needle system was designed for percutaneous puncture of osteoclerotic lesions. Driven by a conventional motor unit, the instrument allows safe guidance even where difficult biopsy routes are used, and has been shown to penetrate radiopaque lesions. Preliminary clinical results from 29 patients are presented. The specimens obtained yielded a correct diagnosis in 28 cases. No major complications were observed.

Biopsy, Needle↗
PubMed2001

Expression of type I, type II, and type X collagen genes during altered endochondral ossification in the femoral epiphysis of osteosclerotic (oc/oc) mice.

The osteosclerotic (oc/oc) mouse, a genetically distinct murine mutation that has a functional defect in its osteoclasts, also has rickets and shows an altered endochondral ossification in the epiphyseal growth plate. The disorder is morphologically characterized by an abnormal extension of hypertrophic cartilage at 10 days after birth, which is later (21 days after birth) incorporated into the metaphyseal woven bone without breakdown of the cartilage matrix following vascular invasion of chondrocyte lacunae. In situ hybridization revealed that the extending hypertrophic chondrocytes expressed type I and type II collagen mRNA, as well as that of type X collagen and that the osteoblasts in the metaphysis expressed type II and type X collagen mRNA, in addition to type I collagen mRNA. The topographic distribution of the signals suggests a possible co-expression of each collagen gene in the individual cells. Immunohistochemically, an overlapping deposition of type I, type II, and type X collagen was observed in both the extending cartilage and metaphyseal bony trabeculae. Such aberrant gene expression and synthesis of collagen indicate that pathologic ossification takes place in the epiphyseal/metaphyseal junction of oc/oc mouse femur in different way than in normal endochondral ossification. This abnormality is probably not due to a developmental disorder in the epiphyseal plate but to the failure in conversion of cartilage into bone, since the epiphyseal plate otherwise appeared normal, showing orderly stratified zones with a proper expression of cartilage-specific genes.

Animals↗
PubMed2000

Erdheim Chester disease: a rare cause of knee and leg pain.

A case of Erdheim Chester disease in a 51-year-old Turkish patient is described. Erdheim Chester disease is a rare form of lipoid granulomatosis. Knee and leg pain are the most common symptoms, and physicians working in orthopaedics and traumatology are the first to be consulted. Our patient demonstrated a typical bilateral, symmetric sclerosis of the metaphyseal region of long bones of the lower extremity, histologic examination revealed foamy, lipid-loaded histiocytes. The patient also suffered from arterial hypertension, diabetes insipidus and exophthalmos of the left eye. The diagnosis was confirmed by a bone biopsy, and the patient was treated with non-steroidal anti-inflammatory drugs, corticosteroids and vincristine.

Granuloma↗
PubMed1998

[Nonbacterial osteomyelitis of the clavicle].

Osteomyelitis can be subdivided into a bacterial exogenic (post-traumatic/postoperative), bacterial endogenic (hematogenous) and an abacterial type, including the rare group containing primary chronic sclerosing osteomyelitis, which is typically localized in the clavicle. In a review of the literature, the criteria for the various types of chronic sclerosing osteomyelitis are analyzed. Out of a group of 17 patients with osteomyelitis of the clavicle treated between 1978 and 1996, three cases of primary chronic sclerosing osteomyelitis are demonstrated. In the differential diagnosis, primary chronic sclerosing osteomyelitis of the clavicle has to be taken into consideration despite its rareness. After establishing a diagnosis by biopsy, in contrast to the other forms, drug therapy will be the treatment of choice and not an operation.

Adolescent↗
PubMed2002

Osteosclerotic metaphyseal dysplasia: a skeletal dysplasia that may mimic lead poisoning in a child with hypotonia and seizures.

We report the case of a 23-month-old male with hypotonia, developmental delay, and complex seizures. Radiographs revealed profound sclerosis of the metaphyses and epiphyses of the long and short bones in the extremities, with a unique pattern of distribution. Sclerosis also involved the anterior ribs, iliac crests, talus, and calcaneus. The skull and vertebral bodies appeared unaffected. Blood lead levels were normal. We believe that this constellation of clinical and radiographic abnormalities closely resembles osteosclerotic metaphyseal dysplasia (OMD) due to an autosomal recessive defect. Characteristic skeletal findings were instrumental in determining the diagnosis. OMD is a very rare sclerosing bone disorder, first described in 1993. The syndrome is characterized clinically by developmental delay of a progressive nature, hypotonia, elevated alkaline phosphatase, and late-onset spastic paraplegia. We encountered a young child with these neurologic symptoms who displayed sclerotic metaphyseal changes on hand radiographs obtained to determine the bone age. Lead poisoning, a known cause of metaphyseal sclerosis, was initially suspected. Careful analysis of the metaphyseal bone changes helped to distinguish this bone dysplasia from lead poisoning and other causes of metaphyseal sclerosis.

Abnormalities, Multiple↗
PubMed2005

Cerebellar and cerebral atrophy in trichothiodystrophy.

Trichothiodystrophy is a rare neuroectodermal disorder of autosomal recessive inheritance that is characterized by brittle hair, nail dysplasia, ichthyosis, mental retardation, and gonadal failure. We describe a female patient whose cranial MRI revealed almost total lack of myelination in the supratentorial white matter, which is similar to the previously described cases. In addition, there was progressive cerebellar and cerebral atrophy, which has not been well documented in association with trichothiodystrophy.

Abnormalities, Multiple↗
PubMed2002

Osteosarcoma arising in a femur with melorheostosis and osteopathia striata.

Osteopathia striata is an asymptomatic autosomal dominant or sporadically inherited disorder that causes dense striations at sites of endochondral bone formation, with a predilection for the metaphyses of long bones. Melorheostosis is a mixed sclerosing dysplasia with disturbance of both endochondral and intramembranous ossification, in which disordered intramembranous ossification dominates. It presents typical radiological changes of cortical hyperostosis distributed along a sclerotome with variable associated cutaneous and clinical features. Overlap syndromes including one or more of these diseases are described. We report a 44-year-old man with both melorheostosis and osteopathia striata who presented with pain secondary to superimposed osteosarcoma. In reporting this case we discuss the relationship between sclerosing dysplasia and either coexisting or complicating sarcoma.

Adult↗
PubMed2004

Chondroblastoma of the hands and feet.

OBJECTIVE: To review the imaging findings, age and gender distribution of chondroblastoma of the hands and feet. DESIGN AND PATIENTS: Twenty-five cases of pathologically proven chondroblastoma of the hands and feet were reviewed. Available imaging modalities included radiographs (n=24), CT (n=3), MRI (n=5), and radionuclide bone scintigraphy (n=1). The following imaging features for each case were tabulated: location, presence of sclerotic margin, calcification, expansion, presence of fluid/fluid levels on cross-sectional imaging and surrounding edema on MRI. The images were evaluated for skeletal maturity using closure of the physeal plate in the region as a standard. RESULTS: The average age at time of diagnosis was 23 years (range 7-57 years). Eighty-four percent (n=21) of the patients were skeletally mature. Males (20 of 25) outnumbered females by a ratio of 5:1. The bones of the foot accounted for 22 cases: calcaneus (n=8), talus (n=8), metatarsals (n=3), and the cuboid (n=3). The bones of the hand accounted for three cases: phalanx (n=1), triquetrum (n=1), and a metacarpal (n=1). Radiographically all lesions were osteolytic with identifiable calcification in 54% (13 of 24). Fluid/fluid levels were seen in four of five cases on MRI. Edema on MR images was seen in 40% (2 of 5). The size of the lesions ranged from 2 to 41 cm(2). CONCLUSION: Chondroblastomas of the hands and feet share many of the radiographic characteristics seen in the long bones, but manifest in skeletally mature patients with a higher male to female ratio than in long bone chondroblastoma. Talar and calcaneal lesions were encountered only in males. Chondroblastoma of the wrist and hand appears to be exceptionally rare.

Adolescent↗
PubMed2004

Dual-energy X-ray absorptiometry applied to the assessment of tibial subchondral bone mineral density in osteoarthritis of the knee.

OBJECTIVE: Plain X-ray is an imprecise tool for monitoring the subchondral bony changes associated with the development of knee osteoarthritis (OA). Our objective was to develop and validate a technique for assessing tibial subchondral bone density (BMD) in knee OA using dual energy X-ray absorptiometry (DXA). DESIGN: Patients with OA of at least one knee underwent DXA scanning of both knees. Regions of interest (ROI) were placed in the lateral and medial compartments of tibial subchondral bone. Weight-bearing plain X-rays and Te (99m) scintiscans of both knees were obtained and scored. RESULTS: One hundred and twelve patients (223 knees) underwent DXA and radiography. Intra-observer CV% was 2.4% and 1.0% for the medial and lateral ROI respectively. Definite OA (Kellgren and Lawrence Grade 2, 3 or 4) was correlated with age-related preservation of subchondral BMD compared to radiographically normal knees. Raised BMD was also associated with subchondral sclerosis, and positive scintigraphy. CONCLUSION: DXA may provide a safe, rapid and reliable means of assessing knee OA. Cross-sectional age-related subchondral tibial BMD loss is attenuated by knee OA.

Absorptiometry, Photon↗
PubMed2004

Three-dimensional trabecular bone architecture of the lumbar spine in bone metastasis from prostate cancer: comparison with degenerative sclerosis.

OBJECTIVE: Prostate cancer frequently metastasizes to bone, inducing osteosclerotic lesions. The objective of this study was to clarify the three-dimensional (3D) trabecular bone microstructure in bone metastasis from prostate cancer by comparison with normal and degenerative sclerotic bone lesions, using microcomputed tomography (micro-CT). DESIGN AND MATERIALS: A total of 32 cancellous bone samples were excised from the lumbar spine of six autopsy patients: 15 metastatic samples (one patient), eight degenerative sclerotic samples (four patients) and the rest from normal sites (three patients). The samples were serially scanned cross-sectionally by micro-CT with a pixel size of 23.20 microm, slice thickness of 18.56 microm, and image matrix of 512 x 512. Each image data set consisted of 250 consecutive slices. The volumes of interest (96 x 96 x 120 voxels) were defined in the original image sets and 3D indices of the trabecular microstructure were determined. RESULTS: The trabecular thickness (Tb.Th) in degenerative sclerotic lesions was significantly higher than that in normal sites, whereas no significant difference was observed in trabecular number (Tb.N). By contrast, in metastatic lesions, the Tb.N was significantly higher with increased bone volume fraction (BV/TV) than in normal sites, and no significant difference was found in Tb.Th. The characteristics of the trabecular surface in the metastatic samples showed concave structural elements with an increase in BV/TV, indicating osteolysis of the trabecular bone. In 3D reconstructed images, increased trabecular bone with an irregular surface was observed in samples from metastatic sites, which were uniformly sclerotic on soft X-ray radiographs. CONCLUSION: These results support, through 3D morphological features, the strong bone resorption effect in bone metastasis from prostate cancer.

Adult↗
PubMed1996

Computed tomography of the normal sternoclavicular joint.

OBJECTIVE: The objective of this work was to establish a reference population for the study of the sternoclavicular joint (SCJ). DESIGN: A prospective study of the SCJ was carried out by high-resolution computed tomography (CT). CT images were read by two radiologists and two rheumatologists using a pre-established grid (27 elementary signs). PATIENTS: Sixty healthy subjects, homogeneously distributed by sex and decade of life, from 20 to 80 years old, were studied. RESULTS AND CONCLUSIONS: The main results were the following: 98% of healthy subjects presented at least one sign; mean number of signs per subject was 2.4 +/- 1.9; mean number of signs was higher ( P < 0.005) in men (mean = 3.1 +/- 2.1) than in women (mean = 1.7 +/- 1.3); number of signs increased with age (P < 0.001). Some signs were very frequent: "ossification of the first costal cartilage" (88%), "Surrounded subchondral clavicular erosion" (27%). Some signs were significantly more frequent in the elderly: "sternal osteophyte" after the age of 70 (P < 0.01) and "meniscal calcification" (P < 0.01) and "sternal subchondral sclerosis" (P < 0.05) after the age of 60. The number of clavicular signs was greater than that of sternal signs (P < 0.001). These results can be used as references for the study of the SCJ.

Adult↗