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Diffuse pulmonary ossification.

Diffuse pulmonary ossification (DPO) is an uncommon condition that is characterized by metaplastic bone formation in the lung parenchyma. It is usually not diagnosed clinically and may be apparent radiographically only when extensive. However, it is occasionally encountered at autopsy or on pathologic evaluation of surgical specimens. This article will review the clinical, histologic, and radiographic manifestations of DPO, focusing primarily on the chest radiograph and CT findings, both of which may be underappreciated, for even experienced radiologists may confuse DPO with other entities such as metastatic calcification as seen in chronic renal failure or chronic granulomatous disease.

Calcinosis↗

Patterns of paravertebral ossification in the prehistoric saber-toothed cat.

In cases of paravertebral ossification in humans, the radiographic characteristics usually lead to a specific diagnosis. Similar manifestations are sometimes described in other species. We applied knowledge of the radiographic appearance of spinal alterations in modern humans to the evaluation of 48 fused thoracic and lumbar fossilized spine specimens from the prehistoric saber-toothed cat (Smilodon californicus), in which prominent paravertebral ossification is frequently found. Inspection, conventional radiography, and, in some cases, CT and fluoride analyses were performed. The spinal alterations in the saber-toothed cat, which previously were believed to be caused primarily by trauma, showed characteristics of three major pathologic processes: trauma, diffuse idiopathic skeletal hyperostosis, and inflammatory disease of a type similar to ankylosing spondylitis. The last two categories have rarely been diagnosed in species other than humans. The results suggest that diffuse idiopathic skeletal hyperostosis and inflammatory spondyloarthropathy are diseases that occurred in prehistoric times and are not confined to the human species.

Animals↗

Ossification in the sternum as a means of assessing skeletal age.

The centres of ossification in the sternum were investigated to determine whether there was any correlation between ossification in the sternum and chronological age. But this proved to be of no value in assessing skeletal age, although it may be of use in assessing skeletal maturity for pathological or forensic purposes.

Age Determination by Skeleton↗

Subungual exostosis of the foot.

Eleven subungual exostoses of the foot (10 on the hallux, 1 on the third toe) were studied. The initial symptom was subungual pain. When a subungual mass of fibrous tissue appeared, the nail was pushed up and in one case the mass became infected. X-rays exhibited a bone mass protruding from the terminal phalanx on the dorsomedial aspect of the toe in all cases. All patients underwent surgical excision of the lesions with partial onychectomy. Three layers were identified in five cases: a cap of fibrous tissue, a middle zone of hyaline cartilage with enchondral ossification, and a deep zone of cancellous bone. In three other cases, the histological pattern was pleomorphic and poorly characterized. The study shows that most subungual bone masses exhibited the pathological features of conventional osteochondromas. Nonetheless, a small number of lesions were pleomorphic and differed from osteochondromas, with abundant fibrous tissue merging irregularly into scattered islets of cartilage that was not organized in columns. Radical excision of the mass achieved complete relief of symptoms and recovery without recurrences in all cases.

Adolescent↗

CTGF/Hcs24 as a multifunctional growth factor for fibroblasts, chondrocytes and vascular endothelial cells.

Connective tissue growth factor/hypertrophic chondrocyte-specific gene product 24 (CTGF/Hcs24), a member of the CCN family, is a multifunctional growth factor for fibroblasts, chondrocytes and vascular endothelial cells. Depending on the type of cell with which it interacts, it promotes chemotaxis, migration, adhesion, proliferation, differentiation and/or extracellular matrix formation. Because gene expression of CTGF/Hcs24 is maximal in hypertrophic chondrocytes in the physiological state, a major physiological role for this factor should be the promotion of endochondral ossification. On the other hand, its expression is up-regulated during wound healing, indicating the involvement of this factor in this process as well. When overexpressed, CTGF/Hcs24 may cause pathological states such as fibrotic disorders and angiogenic diseases. This review focuses on the physiological and pathological significances of this novel type of growth factor.

Animals↗

[A case of angiographically occult arteriovenous malformation with metaplasia (so-called brain stone)].

The patient was a 69-year-old female. Right hemiparesis occurred on April 25, 1996, and then was relieved a day later. Because headache (dull pain in the left) persisted subsequently, she consulted our department on April 26. Head CT showed, without enhancement effect, osseous high density on the surface of the left frontal area. MRI showed high intensity on T1 and low intensity on T2 with flow-void like findings. Cerebral angiography showed a pooling of contrast medium in the same region. 123I-IMP-SPECT revealed reduced cerebral blood flow in the left frontal and parietal lobes just under the same region. On June 11, the patient underwent surgery during which a tumor with arachnoid hypertrophy was extracted en block. Histopathologically, there were abnormal blood vessels with elastic fibers, expanding to an ossified site, and AVM accompanying ossification was thus diagnosed. Postoperative 123I-IMP-SPECT showed improved cerebral blood flow in the left frontal and parietal lobes. The patient was discharged on June 22. The TIA pathologic condition, a symptom of its onset, was considered attributable to cerebral blood flow steal due to AVM.

Aged↗

Cartilage calcification: normal and aberrant.

Hydroxyapatite crystal deposition occurs physiologically in cartilage as a prelude to bone formation via endochondral ossification. Both extracellular molecules and organelles, and the chondrocytes themselves control the initial formation of hydroxyapatite, as well as the growth and orientation of the hydroxyapatite crystals. In this study pathologic calcium containing deposits from 48 patients (27 hydroxyapatite and 21 calcium pyrophosphate dihydrate) were subjected to crystallographic, histologic (light microscopy, scanning electron microscopy, transmission electron microscopy) and chemical analyses and compared to normal controls. It is suggested that both hydroxyapatite and calcium pyrophosphate dihydrate deposition involve elevations in ionic concentrations, exposure of mineral nucleators, and removal of mineral inhibitors. Peculiar to the matrices of pathologic deposits of hydroxyapatite are elevated concentrations of calcium acidic phospholipid phosphate complexes and lower concentrations of hexosamine while collagen (hydroxyprolin) and total lipid contents are not altered. Matrices of deposits of calcium pyrophosphate dihydrate were similar biochemically except that calcium acidic phospholipid phosphate complexes concentrations were not elevated.

Calcification, Physiologic↗

[Treatment of humeral shaft fracture with intramedullary procedures (Seidel nail, Marchetti-Vicenzi nail, Prevot pins)].

Humeral shaft fractures can be treated either conservatively or operatively. Plating of the humerus is the established operative method, but recently interest has also been focussed on intramedullary nailing. Fifty-nine cases of humeral fractures treated with intramedullary nailing (Seidel/Marchetti-Vicenzi/Prévot) from January 1991 to December 1995 (44 fractures after trauma, 11 pathological fractures, 3 pseudarthroses, 1 re-fracture). Closed reduction in 55/59 cases. One infection (soft tissue); 2/48 pseudarthrosis (indication for nailing: pseudarthrosis!). No iatrogenic palsy of the radial nerve. Functional postoperative treatment in all 44 cases of humeral fractures after adequate trauma. One poor functional result: periarticular ossification after retrograde nailing, possibly connected with long-term respiratory treatment after trauma. Treatment of humeral shaft fractures by intramedullary nailing is favoured in our clinic (low complication rates, excellent or good functional results, limited approaches, small scars). Proximal fractures should be treated by the Seidel nail (stable interlocking of the proximal fragment); very distal fractures need Prévot nailing (reaming of condylar canals). All other fractures of the humeral shaft can be treated by each of the implants used in our clinic. Pathological fractures are an excellent indication for intramedullary stabilization. These patients benefit from stable fixation without intense surgical trauma. Pseudarthrosis, according to our limited experience, seems to require plating plus bone grafting. Plating is also recommended if revision of the radial nerve becomes necessary.

Adolescent↗

Double child burial from Sunghir (Russia): pathology and inferences for upper paleolithic funerary practices.

The double child burial from Sunghir (Russia) is a spectacular Mid Upper Palaeolithic funerary example dated to about 24,000 BP. A boy (Sunghir 2) and a girl (Sunghir 3), about 12-13 and 9-10 years old, respectively, were buried at the same time, head to head, covered by red ocher and ornamented with extraordinarily rich grave goods. Examination of the two skeletons reveals that the Sunghir 3 femora are short and exhibit marked antero-posterior bowing. The two femora do not show any asymmetry in the degree of shortening and bowing. Bowing affects the whole diaphysis and shows a regularly incurved profile, with the highest point at midshaft. Pathology is confined to the femora, and no other part of this well-preserved specimen shows abnormality. The isolated nature of the Sunghir 3 anomalies points to cases reported in the medical literature under the label of "congenital bowing of long bones" (CBLB). These are a group of rare conditions exhibiting localized, sometimes bilateral, bowing and shortening which are nonspecific and may result from different causes, including abnormalities of the primary cartilaginous anlage (i.e., the aggregation of cells representing the first trace of an organ). Localized ossification disturbances, possibly linked to a diabetic maternal condition, might explain the shortening and the coincidence of maximum midshaft curvature with the position of the primary ossification center, as well as the lack of involvement of other skeletal parts. This scenario, rather than other possibilities (early bilateral midshaft fracture, acute plastic bowing deformities, or faulty fetal posture), provides the most likely explanation for the Sunghir 3 femoral deformities. The intriguing combination of a pathological condition apparent since birth with a spectacular burial of unusually positioned young individuals of different sexes recalls significant aspects of the triple burial from the contemporary site of Dolní Vestonice (Moravia), evoking a patterned relationship between physical abnormality and extraordinary Upper Paleolithic funerary behavior.

Adolescent↗

Clinical and radiological analysis of ossification of the anterior longitudinal ligament causing dysphagia and hoarseness.

OBJECTIVE: We retrospectively analyzed the clinical and radiological characteristics of ossification of the anterior longitudinal ligament (OALL). METHODS: Seventeen patients with OALL who underwent surgery between 1995 and 2003 were reviewed. Symptomatic OALL was found in four patients. In 13 asymptomatic OALL patients who experienced mild to severe myelopathy, no swallowing difficulty was noted. The OALL was classified into three types by sagittal computed tomographic (CT) scans as segmental, continuous, and mixed and three types on axial CT scans as flat, nodular, and globular type. The thickness, numbers of involved vertebral bodies, and type and shape for symptomatic OALL were analyzed and compared with those for asymptomatic OALL. RESULTS: The mean thickness of 13.5 mm for symptomatic OALL was significantly higher than that of 6.5 mm for asymptomatic OALL (P = 0.0009). A globular shape on axial CT was common for symptomatic OALL. There were no differences in the numbers of involved vertebral bodies and types of OALL on sagittal CT scans. Surgical excision of OALL was performed for all cases of symptomatic OALL. In 7 of 13 asymptomatic cases, OALL was simultaneously removed during anterior decompressive surgery for the associated pathology. CONCLUSION: The thickness on axial CT scans was an important contributing factor to dysphagia and hoarseness as was the shape of the OALL. The type of OALL on sagittal CT scans was similar to that of ossification of the posterior longitudinal ligament but did not influence the development of dysphagia. Good relief from symptoms was achieved for symptomatic OALL after removal of OALL.

Aged↗

Spontaneous osteochondrosis in rats.

143 Sprague Dawley rats, ranging from 19-63 weeks of age, were examined pathologically for the occurrence of spontaneous joint lesions. Macroscopically, a white round plaque was observed in the caudocentral portion of the medial femoral condyle or humeral head and sometimes it had a partially detached margin. Microscopic examination revealed a local thickening of the articular cartilage protruding into the subchondral bone, which suggested cessation of endochondral ossification and retention of cartilage. As the lesion advanced there was fissure formation in the thickened cartilage and proliferation of fibrous tissue beneath the fissure. Formation of a cartilage flap and extensive fibrosis with large cysts were also observed in the most advanced cases. Total incidence of the lesion in the distal femur was 22.2% in male and 2.8% in female rats. The change detected in the rats was identical with pathologic descriptions of osteochondrosis in domestic animals.

Animals↗

Case report: primary fibrosarcoma of the liver in a cow.

An unusual case of primary fibrosarcoma of the liver in a cow is described. The most prominent pathological findings were severe abdominal haemorrhage and neoplastic masses confined to the liver only. Histologically, spindeloid cells arranged in interlacing bundles running in various directions and numerous areas of necrosis and ossification were randomly scattered in the neoplasm.

Animals↗

GM1-gangliosidosis in Alaskan huskies: clinical and pathologic findings.

Three Alaskan Huskies, two females and one male, were diagnosed with GM1-gangliosidosis. Clinically, diseased animals exhibited proportional dwarfism and developed progressive neurologic impairment with signs of cerebellar dysfunction at the age of 5-7 months. Skeletal lesions characterized by retarded enchondral ossification of vertebral epiphyses were revealed by radiographs of the male dog at 5.5 months of age. Histologic examination of the central nervous system (CNS) revealed that most neurons were enlarged with a foamy to granular cytoplasm due to tightly packed vacuoles that displaced the Nissl substance. Vacuoles in paraffin-embedded sections stained positively with Luxol fast blue and Grocott's method, and in frozen sections vacuoles were periodic acid-Schiff positive. Foamy vacuolation also occurred within neurons of the autonomic ganglia. Extracerebral cells such as macrophages and peripheral lymphocytes also displayed foamy cytoplasm and vacuolation. In the CNS of diseased animals, a mild demyelination and axonal degeneration was accompanied by a significant astrogliosis (P < 0.05) in the gray matter as compared with age- and sex-matched control dogs. There was also a significant loss (P < 0.05) of oligodendrocytes in the gray and white matter of affected animals as compared with controls. Ultrastructurally, the neuronal storage material consisted of numerous circular to concentric whorls of lamellated membranes or stacks of membranes in parallel arrays. GM1-gangliosidosis in Alaskan Huskies resembles beta-galactosidase deficiency in other canine breeds, and these CNS disorders may be a consequence of neuronal storage and disturbed myelin processing.

Animals↗

Methylprednisolone has a preventive effect on heterotopic bone formation and on knee thickening and stiffening following manipulation of immobilized rabbit legs.

The effect of methylprednisolone administered following manipulation of the immobilized knee of 35 adult rabbits was investigated. In the legs of 15 control rabbits which received no methylprednisolone, heterotopic cartilage and bone in the periarticular tissues and in the vastus intermedius muscle, and extensive knee stiffening and thickening regularly developed. In the other rabbits, methylprednisolone showed a marked preventive effect on the development of all these pathological signs.

Animals↗

Value of fetal skeletal radiographs in the diagnosis of fetal death.

The aim of this study was to assess the value of fetal skeletal radiographs in determining the etiology of fetal death. A total of 1193 post-mortem fetal skeletal radiographs were analysed. Fetuses were classified into one of three groups (group I: abnormality diagnosed during pregnancy; group II: maternal pathology; group III: spontaneous abortion of pregnancy, IIIa before 26 weeks of gestation (WG), IIIb after 26 weeks of gestation). Face, supine and lateral skeletal views were performed. Skeletal abnormalities were detected in 33.9% of the fetuses, including 22.7% with minor abnormalities (abnormal rib number, no nasal bone ossification, amesophalangia or P2 hypoplasia of the fifth digit) and 14.5% with major abnormalities (other skeletal abnormalities). Among the fetuses with major abnormalities, 98.8% came from group I, 2.9% came from group II, 2.3% came from group IIIa and none came from group IIIb. Fetal skeletal radiographs are not useful in fetuses arising from spontaneous abortion of pregnancy without abnormality on ultrasound screening, abnormality clinical examination or in fetuses with prenatal diagnosis of chromosomal abnormality. This practice is valuable only if there is a multidisciplinary team, with all the participants (pathologists, radiologists, geneticists) knowledgeable about fetal pathology. In the absence of this multidisciplinary approach, it is easier to X-ray all fetuses to avoid misdiagnosis and the important consequences for genetic counselling.

Bone Diseases↗

Progressive ankylosis (ank/ank) in mice: an animal model of spondyloarthropathy. II. Light and electron microscopic findings.

Murine progressive ankylosis was studied by light and electron microscopy (EM). By light microscopy, the earliest synovial lesion was inflammatory followed by synovial proliferation and cartilage erosions. Later changes included progressive joint ankylosis by fibrosis and ossification of articular and periarticular tissues and new bone proliferation. By EM, synovial cell proliferation and hypertrophy with collagen deposition was seen before the appearance of intracellular and extracellular hydroxyapatite crystals. These pathologic changes are the same as those found in the human spondyloarthropathies. Our EM findings demonstrate that hydroxyapatite crystals appear in the joints of ank/ank mice after the initial inflammatory phase.

Animals↗