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Bone remodeling in hypervitaminosis D3. Radiologic-microangiographic-pathologic correlations.

To investigate the mechanism of bone changes in hypervitaminosis D3, we compared contact radiographs, microangiograms by injection of Chinese ink, and corresponding histopathologic macrosections of 66 rabbits that received different doses of vitamin D3. In early stages, radiographs showed subperiosteal bone resorption and porotic cortical bone. The corresponding microangiograms showed proliferating vessels in the periosteum and cortical bone with associated dilatation of the Haversian and Volkmann's canals. When metastatic calcification fills these intracortical caverns or the intertrabecular spaces in the metaphysis and physis, the bone shows a diffuse increased density on radiographs. A dense band in the metaphysis reflects a thickening of calcified chondromatrix due to a hypotrophy of the distal metaphyseal capillaries. Six to 12 weeks after vitamin D3 withdrawal, radiographs showed further increased density of the cortical bone, due to newly formed bone and metastatic calcification. Alternating bands of increased and decreased density in the metaphysis and physis reflect the reinvasion of normal vasculature between growth cartilage and calcified chondromatrix, with normalization of endochondral ossification.

Angiography↗

Case report: computed tomography findings in lipoid proteinosis: report of two cases.

Lipoid proteinosis (Urbach-Wiethe disease (Urbach, E and Wiethe, C, Lipoidosis cutis mucosae, Virchows Arch. Patholog. Anat., 273, 285-319 (1929)) is a rare generalized disease with autosomal recessive inheritance. It most often involves the skin and mucosal membranes of the aerodigestive tract; but also involves the central nervous system, lung, lymph nodes and striated muscles. We present the computed tomography findings in the cranium and larynx of two siblings with lipoid proteinosis.

Adult↗

Ossifying malignant mixed epithelial and stromal tumor of the liver: a case report of a previously undescribed tumor.

BACKGROUND: Malignant mixed tumors of the liver in adults are rare. To the authors' knowledge, twenty-four cases of primary malignant mixed hepatic tumors have been reported in the literature since the first description by Walter in 1896. Many of the previously reported cases are now considered mixed hepatoblastoma, primary hepatocellular carcinomas with focal sarcomatous metaplasia, or epithelioid hemangioendothelioma. METHODS: Clinical, radiologic, histologic and immunohistochemic methods were used to characterize an unusual example of a mixed malignant tumor of the adult liver that the authors descriptively refer to as "ossifying malignant mixed epithelial and stromal tumor" of the liver. RESULTS: In contrast to previously reported cases, the tumor in question was morphologically distinct. It was composed of three distinct neoplastic phenotypes: malignant spindle cells, with adenocarcinomatous differentiation, and extensive osteoid formation. The tumor was treated by partial hepatectomy. The patient was alive 8 years postoperatively as of December 2001. The distinctive combination of morphologic features and prolonged survival are much different from previously reported cases of mixed malignant hepatic tumors, which were typically rapidly fatal. CONCLUSIONS: The authors believe that the clinical and pathologic features of this patient's tumor are sufficiently distinctive to exclude it from the well-established categories of hepatic neoplasms. Histologically, the authors suggest the descriptive name "ossifying malignant mixed epithelial and stromal tumor of the liver" as a preliminary description for this tumor until the origin and relationship to other hepatic neoplasms are further characterized.

Adenocarcinoma↗

Sonographic diagnosis of heterotopic bone formation in spinal injury patients.

A variety of stimuli including neurological insult may provoke primitive mesenchymal cells to differentiate into bone forming cells. Such a sequel following spinal cord injury can delay rehabilitation, enhance the spasticity and reduce the range of movement in the affected joint. It is characterised by an initial inflammatory phase followed by heterotopic bone formation. The early inflammatory lesion clinically mimics deep vein thrombosis, a developing pressure sore, infection, and tumour. An early diagnosis at a time of clinical uncertainty, before the plain radiographic features develop, has distinct advantages and therapeutic implications. The unique pathological evolution and maturation of the lesion is clearly demonstrated by sequential sonographic (ultrasonic) assessment with depiction of the 'zone phenomena' seen on histology. Sonographic scans in 7 spinal injury patients proved diagnostic, before there was radiographic evidence of bone formation, and confidently excluded HBF in a further 18 patients without any false negative results.

Adult↗

The role of osteogenic index, octahedral shear stress and dilatational stress in the ossification of a fracture callus.

The exact mechanism by which mechanical stimulus regulates the healing process of a bone fracture is not understood. This has led to the development of several hypotheses that predict the pattern of differentiation of tissue during healing that may arise from characteristic fields of stress or strain at the fracture. These have so far remained unproved because data on stress fields in actual fracture tissue have been unavailable until recently. Thus the present study examines the predictive performance of the hypothesis proposed in J Orthop Res 6 (1988) 736, against measured and calculated data reported in J Biomech 33 (2000) 415, using a 2D FEM of a clinical fracture. The hypothesis was used to predict the influence of stress fields present in the Gardner et al. tissues at four temporal stages during healing. These predictions were then correlated with callus-size, rate of endochondral ossification and ossification pattern subsequently observed by Gardner et al. in the clinical fracture. Results corroborate the hypothesis that high octahedral shear stresses may increase the size of the callus during the initial phase of healing, and they also suggest that this may be true during the later stages of the fracture fixation period. However, compressive dilatational stresses were not found to inhibit endochondral ossification, as suggested by the hypothesis. Although high shear stresses were found in regions indicative of fibrous tissue as postulated by the hypothesis, this was not found to be the case for high tensile dilatational stresses. Also, contour diagrams of Osteogenic index (I) indicated only limited correlation with callus maturation and the pattern of healing. Therefore, the hypothesis was not wholly successful in predicting healing pattern.

Adult↗

The role of ultrasound in the early diagnosis and management of heterotopic bone formation.

Heterotopic bone formation (HBF) is one of the many causes of painful limb swelling and is well recognized as posing clinical, radiological, and pathological diagnostic difficulties. We have evaluated the use of sonography to determine its role in reaching a diagnosis at a time when symptoms are maximal and the radiological diagnostic features absent. Serial ultrasound examination identified unique 'zone phenomea' in a heterogeneous group of eight patients before the classic radiological features appeared. The zone transformation matches the evolution and maturation process described pathologically and forms the basis of an early definitive diagnosis in all cases. The sonographic features are specific for the condition, unlike the scintigraphic appearances. Although CT confirms the peripheral location of the calcification, the low cost, safety, and general availability of ultrasound scanning make it the optimum imaging method for diagnosing and monitoring heterotopic bone formation.

Adult↗

[Ectopic bone formation in neurological lesions (author's transl)].

The authors have operated on seventy instances of para-articular ectopic bone formation in fifty patients. Seventeen were paraplegic or tetraplegic, thirty were the result of trauma, two resulted from meningitis and one was a sequel of burns. The ectopic bone was located at the hip (thirty-three cases), the elbow (twenty-three cases), the knee (twelve cases) and the shoulder (two cases). The clinical, radiological and pathological features are reviewed. Several hypotheses on the mechanism of ectopic bone formation are put forward. The surgical procedures have included arthrolysis, arthrodesis, wide resection of the epiphysis, and total prostheses. There were ten recurrences. The authors conclude that the most extensive lesions occur in paraplegia and when the bone formation is situated at the hip.

Adolescent↗

[Uncommon form of idiopathic osteoporosis with hypercalciuria, growth retardation and mental retardation].

Case report of a 18 year old boy with short stature, microceophaly, mental retardation and multiple dysmorphic signs. At the age of 9 years a severe generalised osteoporosis was discovered. A pathological fracture of the greenwoor type healed without proper callus formation. The osteoporosis persists without signs of either deterioration or improvement. The serum phosphorus is slightly decreased, while serum calcium, alkaline phosphatase and renal functions are normal. The main biochemical finding is a constant hyperclaciuria of 6-13 mg/kg/24 h, which can be corrected by treatment with oral sodium phosphate. No other chronic disease could be found which would explain the bone disease. The complex disease of this boy does not fit into the known pictures of osteogenesis imperfecta, idiopathic juvenile osteoporosis or of idiopathic hypercalciuria, and might therefore be another type of demineralising bone disease. It is suggested, that the cause might be an impairment of the calcium fixation of collagen fibres during desmal ossification.

Abnormalities, Multiple↗

Normal fetal lumbar spine on postmortem MR imaging.

BACKGROUND AND PURPOSE: There is an increasing interest in use of postmortem MR imaging as an adjunct or alternative to autopsy. Before evaluating spinal pathology on postmortem MR imaging, it is important to have knowledge of the normal appearance of the fetal spine at different gestational ages. The aim of this study is to describe the MR imaging appearances of normal development of the fetal spine at different gestational ages. METHODS: Postmortem MR imaging was performed on 30 fetuses ranging from 14 to 41 gestational weeks. There was no structural abnormality of the spine in these fetuses on MR imaging or at autopsy. Fast spin-echo T2-weighted MR imaging of the lumbar spine was performed in the coronal plane in all cases and supplemented by sagittal and/or axial imaging. The following parameters were measured: height of the L1/2 disk and L2 vertebral body and area of ossification center in L2 vertebral body as well as area of vertebral body. The signal intensity of the disk space and the vertebral level of conus termination were also assessed. RESULTS: The height and area of the vertebral body increased linearly with gestational age (P <.01). The increase in disk space was proportionally greater than the increase in vertebral body height as gestational age increased (P <.01). The disk space appeared as a linear low-signal-intensity area in fetuses < or = 21 weeks gestation but increasingly developed high signal intensity in the disk after 21 weeks. The size of the ossification center increased with gestational age (P <.01), and the ratio of ossification center to the overall size of the vertebral body also increased with gestational age (P <.01). In fetuses less than 35 weeks of age, the conus lay between L2 and L5 level, whereas in fetuses more than 35 weeks of age, the conus lay between L1/2 and L2/3 level. CONCLUSION: Understanding the normal growth and signal-intensity characteristics of the fetal spine on postmortem MR imaging is essential before studying abnormal fetal spine.

Autopsy↗

Sudden deafness: histopathologic correlation in temporal bone.

The histopathological study of two cases of sudden deafness is presented. The temporal bones showed cochleosaccular abnormality. The most striking pathological changes were collapse of the organ of Corti, atrophy of the tectorial membrane, atrophy of the stria vascularis, decrease in the number of the cochlear nerves, collapse of the saccular membrane and partial absence of the sensory epithelial layer in the saccular macula. These changes are quite similar in type to those occurring in labyrinthitis of known viral etiology and to those in previously reported cases of sudden deafness which were assumed to be of viral origin. This evidence suggests that a viral infection was the most probable etiology of sudden deafness in these ears. In addition, unusual findings of endolymphatic hydrops limited to the extreme basal end of the cochlear duct were found in Case 1. A patent cochlear aqueduct and circumscribed perilymphatic labyrinthine ossification in the superior seimicircular canal were also observed. With these histopathological findings, the possibility of viral infection via the meninges as well as via the hematogenous route into the inner ear is proposed.

Cochlea↗

[MR imaging of primary bone and soft tissue tumors].

MR imaging of 131 cases with pathologically confirmed primary bone and soft tissue tumors were studied. They included 44 bone tumors (25 benign tumors, 19 malignant tumors) and 87 soft tissue tumors (55 benign tumors, 32 malignant tumors). MR imaging was performed on 0.5T, superconductive magnet system. All tumors were evaluated with T1-weighted, T2-weighted and STIR images. In some cases, contrast enhanced MR imaging with Gd-DTPA was applied. MR imaging was proving to be a valuable technique in the evaluation of patients with primary bone and soft tissue tumors. MR imaging was superior to the other modalities in delineating the extent of the tumor and their relation to surrounding structures in all cases. However, plain radiography and CT were more useful for evaluation of calcification, ossification, cortical destruction and endosteal/periosteal reaction than MR imaging. Direct sagittal and coronal images from MR imaging added accurate assessment for the relation between the tumor and their adjacent structures. MR imaging was of limited value in distinguishing benign from malignant tumors with the demonstration of tumor structures only, especially soft tissue tumors. But in bone and soft tissue tumors which have specific morphologic features and intensity patterns, MR imaging was very useful for diagnosis.

Bone Neoplasms↗

[Radiological and histomorphological correlation of bone tumors and tumor-like diseases (author's transl)].

In the diagnosis of bone tumors and tumor-like lesions the radiographic examination plays a dominant role in the phase before performing a biopsy, with the support of this examination important facts are available for the further diagnostic procedure, for planning the biopsy and also for getting the preliminary guiding principles how the therapy has to be performed. In order to interpret a radiograph of a diseased bone optimally it is necessary to know the histomorphological background of an X-ray feature. This more particularly as the reaction of bone to a pathological process is limited to only 2 radiographic images: osteolysis or/and osteosclerosis. On the basis of the histological pattern and the dynamic micromorphological process the picture of osteolysis and osteosclerosis is analysed. The periosteal reaction is also considered in this manner. After the representation of these general principles a detailed tabulated synopsis is given about the correlation between the histological and roentgenological findings of all benign and malignant bone tumors and tumor-like conditions.

Biopsy↗

An unusual cause of otalgia--an ossified stylohyoid ligament.

Otalgia is often related to pathology in areas of the head and neck apart from the ear. This is due to the rich innervation of the ear via the fifth, seventh, ninth and tenth cranial nerves and the cervical plexus. It is estimated that 50% of ear pain is referred from non-otologic sites. A case is reported where otalgia was due to the presence of a bone in place of a ligament in the neck and was relieved by its removal.

Adult↗

Colchicine inhibits fracture union and reduces bone strength--in vivo study.

INTRODUCTION: Recent studies have demonstrated that Colchicine (CO) prevents heterotopic ossification (HO) after total hip replacement in patients suffering from familial Mediterranean fever (FMF). Other investigators have proved that CO is an in vitro inhibitor of proliferation of osteoblasts and osteosarcoma cells, and is a non-selective mitosis inhibitor and selective inhibitor of mineralization. METHODS: A double blind prospective study comprised four groups of adult rats. The left posterior tibia in each rat was fractured except in one of the control groups. The study groups were treated with CO 1 mg/kg/day 1 week before, or on the fracture day. The control groups did not receive CO treatment. Six weeks after fracture induction the groups were compared radiographically mechanically and histologically. RESULTS: Prolonged CO treatment had a significant negative influence on fracture healing according to radiological, clinical, mechanical (p<0.02), and pathological parameters (p<0.0001). CONCLUSIONS: We were able to demonstrate that prolonged CO treatment reduced bone healing.

Animals↗

Renal transplantation between HL-A identical siblings with partial nephrectomy and machine preservation for ossified renal cell carcinoma.

A case of renal transplantation between HL-A identical siblings is reported in which the donor kidney was found to have a calcified mass in the upper pole. Because an immediate pathologic diagnosis could not be made at the time of nephrectomy, the kidney was preserved with pulsatile perfusion for fifty-four hours after excision of the upper pole. At that time the diagnosis was still not available, and transplantation was performed only to have the report of ossified renal cell carcinoma established the following day.

Adenocarcinoma↗

Clinical and pathologic features of maxillonasal dysplasia (Binder's syndrome): significance of the prenasal fossa on etiology.

Clinical and pathologic anatomic parameters were studied in 50 patients with maxillonasal dysplasia (Binder's syndrome). The skeletal deformity causing the flat and low-set nose was in typical patients a palpable depression in the anterior nasal floor (fossa prenasalis) and a localized maxillary hypoplasia in the alar base region. Class III malocclusion was found in 54 percent. In 6 percent of the patients a slope (sulcus prenasalis) was found instead of a fossa in the anterior nasal floor, and in one patient a rudimentary fossa was found. Concomitant malformations were noted in 18 percent, and a hereditary connection was seen in 16 percent. The etiology is discussed in relation to the development of the premaxilla and the appearance of a secondary external trabecular network of bone in the canine region. An inhibition of the latter ossification center would explain the localized hypoplasia in the floor and walls of the piriform aperture in maxillonasal dysplasia.

Abnormalities, Multiple↗

The development and application of pulsed electromagnetic fields (PEMFs) for ununited fractures and arthrodeses.

This article deals with the rational and practical use of surgically noninvasive pulsed electromagnetic fields (PEMFs) in treating ununited fractures, failed arthrodeses, and congenital pseudarthroses (infantile nonunions). The method is highly effective (more than 90 per cent success) in adult patients when used in conjunction with good management techniques that are founded on biomechanical principles. When union fails to occur with PEMFs alone after approximately four months, their proper use in conjunction with fresh bone grafts insures a maximum failure rate of 1 to 1.5 per cent. Union occurs because the weak electric currents induced in tissues by the time-varying fields effect calcification of the fibrocartilage in the fracture gap, thereby setting the stage for the final phases of fracture healing by endochondral ossification. The efficacy, safety, and simplicity of the method has prompted its use by the majority of orthopedic surgeons in this country. In patients with delayed union three to four months postfracture, PEMFs appear to be more successful and healing, generally, is more rapid than in patients managed by other conservative methods. For more challenging problems such as actively infected nonunions, multiple surgical failures, long-standing (for example, more than two years postfracture) atrophic lesions, failed knee arthrodeses after removal of infected prostheses, and congenital pseudarthroses, success can be expected in a large majority of patients in whom PEMFs are used. Finally, as laboratory studies have expanded knowledge of the mechanisms of PEMF action, it is clear that different pulses affect different biologic processes in different ways. Selection of the proper pulse for a given pathologic entity has begun to be governed by rational processes similar, in certain respects, to those applied to pharmacologic agents.

Arthrodesis↗

The development and application of pulsed electromagnetic fields (PEMFs) for ununited fractures and arthrodeses.

This article deals with the rational and practical use of surgically noninvasive pulsed electromagnetic fields (PEMFs) in treating ununited fractures, failed arthrodeses, and congenital pseudarthroses (infantile nonunions). The method is highly effective (more than 90 per cent success) in adult patients when used in conjunction with good management techniques that are founded on biomechanical principles. When union fails to occur with PEMFs alone after approximately four months, their proper use in conjunction with fresh bone grafts insures a maximum failure rate of 1 to 1.5 per cent. Union occurs because the weak electric currents induced in tissues by the time-varying fields effect calcification of the fibrocartilage in the fracture gap, thereby setting the stage for the final phases of fracture healing by endochondral ossification. The efficacy, safety, and simplicity of the method has prompted its use by the majority of orthopedic surgeons in this country. In patients with delayed union three to four months postfracture, PEMFs appear to be more successful and healing, generally, is more rapid than in patients managed by other conservative methods. For more challenging problems such as actively infected nonunions, multiple surgical failures, long-standing (for example, more than two years postfracture) atrophic lesions, failed knee arthrodeses after removal of infected prostheses, and congenital pseudarthroses, success can be expected in a large majority of patients in whom PEMFs are used. Finally, as laboratory studies have expanded knowledge of the mechanisms of PEMF action, it is clear that different pulses affect different biologic processes in different ways. Selection of the proper pulse for a given pathologic entity has begun to be governed by rational processes similar, in certain respects, to those applied to pharmacologic agents.

Adult↗