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Fracture through the bone of traumatic myositis ossificans. A report of three cases.

We describe three cases of traumatic myositis ossificans in which fractures occurred through a mature, quiescent ossification mass. None of the fractures reactivated the original pathological process, no callus was formed and union did not occur. The nonunion became painless over a period of months. This unusual late complication of myositis ossificans seems to require only symptomatic treatment by temporary splintage and subsequent mobilisation. We could find no previous report of a similar case.

Adolescent↗

Congenital renal tubular dysplasia and skull ossification defects similar to teratogenic effects of angiotensin converting enzyme (ACE) inhibitors.

An apparently autosomal recessive syndrome of congenital renal tubular dysplasia and skull ossification defects is described in five infants from two separate, consanguineous, Pakistani Muslim kindreds. The clinical, pathological, and radiological features are similar to the phenotype associated with fetal exposure to angiotensin converting enzyme (ACE) inhibitors: intrauterine growth retardation, skull ossification defects, and fetal/ neonatal anuric renal failure associated with renal tubular dysplasia. There was no fetal exposure to ACE inhibitors in the affected infants. Phenotypic similarities between these familial cases and those associated with ACE inhibition suggest an abnormality of the "renin-angiotensin-aldosterone" system (RAS). It is postulated that the molecular pathology in this uncommon autosomal recessive proximal renal tubular dysgenesis could be related to mutations of the gene systems governing the RAS.

Abnormalities, Drug-Induced↗

Routine perinatal postmortem radiography in a peripheral pathology laboratory.

Routine postmortem radiography was done in 234 consecutive perinatal autopsies. Using ossification centre appearance and length of femoral shafts as variable it was a very useful and dependable method for estimating gestational age and intrauterine growth. In this way important conclusions can be drawn as to the reason for intrauterine growth deviations. Also many, sometimes diagnostic, abnormalities can be found.

Autopsy↗

Skeletal dysplasia in perinatal lethal osteogenesis imperfecta. A complex disorder of endochondral and intramembranous ossification.

Osteogenesis imperfecta (OI) Type II is a rare heritable disorder of bone matrix that results in catastrophic congenital skeletal dysplasia. Two cases of OI Type II had symmetric rhizomelic skeletal dysplasia apparent on ultrasound at 16 and 20 weeks' gestation. Histologic and histochemical studies performed on skeletal tissue from fetal autopsies showed the following: (1) abnormal growth plate tissue characterized by failure of formation of primary bony spongiosa; (2) persistence of calcified cartilage bars in the diaphysis; (3) metaphyseal microfractures; (4) abundant cartilaginous fracture callus; (5) absence of bony callus; (6) failure of formation of intramembranous cortical diaphyseal bone; (7) angulation of long bones in portions of the metadiaphyses bordered by fracture callus; and (8) mechanical failure of the perichondral ring of LaCroix with a normal fibrous ossification groove of Ranvier. These findings suggest that skeletal dysplasia in OI Type II results from the action of muscular forces on a skeleton weakened by a complex disorder of endochondral and intramembranous ossification. The paucity of primary metaphyseal trabeculae and subperiosteal cortical bone leads to pathologic fractures of the immature fiber bone and an imperfect attempt at fracture repair. Angulation and shortening of long bones occurs between numerous sites of focal endochondral fracture callus. Mechanical failure of the fibrous perichondral ring leads to further collapse and shortening without obvious functional impairment of the fibrous ossification groove. Perinatal lethal OI provides insight into how a molecular disorder predominantly of Type I collagen metabolism results in pathology of numerous tissues, leading to severe skeletal dysplasia without primarily affecting chondrogenesis.

Bone Diseases, Developmental↗

Selenium deficiency and fulvic acid supplementation induces fibrosis of cartilage and disturbs subchondral ossification in knee joints of mice: an animal model study of Kashin-Beck disease.

Kashin-Beck disease is an acquired, chronic and degenerative osteoarticular disorder. Selenium deficiency and fulvic acid in drinking water have been implicated in the cause of this disease. Pathologically, chondronecrosis of the growth plate and articular cartilage and subconsequent disturbance of ossification were observed in the joints. In this animal model study, mice were fed with a selenium deficient diet and fulvic acid supplemented drinking water for two generations. In undecalcified histological preparations of bone we carried out histological staining to detect mineralized and unmineralized bone and cartilage. The results revealed that selenium deficiency and fulvic acid supplementation induced degeneration of the articular cartilage in the knee joints of mice. Dynamic fluorescent labelling of ossification, enzyme histochemical detection of alkaline phosphatase activity in osteoblasts and a typical immunohistochemical localization of collagens type I and II indicated the development of fibrocartilage at the articular surface of knee joints, resembling the early stages of osteoarthrosis. This became obvious by disturbed development of the articular space and meniscus, markedly impaired formation of subchondral bone and early differentiation failure during enchondral ossification. This animal model provides an approach to study the molecular pathogenesis of Kashin-Beck disease.

Animals↗

Stromal cells of fibrodysplasia ossificans progressiva lesions express smooth muscle lineage markers and the osteogenic transcription factor Runx2/Cbfa-1: clues to a vascular origin of heterotopic ossification?

Fibrodysplasia ossificans progressiva (FOP) is a rare heritable genetic disorder, which is characterized pathologically by sporadic episodes of explosive growth of mesenchymal cells in skeletal muscle followed by cellular differentiation to heterotopic bone through an endochondral process. This study examined the histological origin and differentiation state of stromal cells in early FOP lesions and investigated the association between the phenotype of these FOP cells and bone formation. Interestingly, FOP lesional stromal cells were found to display characteristics of the smooth muscle (SM) cell lineage and are therefore potentially of vascular origin. These cells co-express multiple SM lineage markers along with multiple proteins associated with bone formation including the obligate osteogenic transcription factor Runx2/Cbfa-1. It is hypothesized that the stromal cells of early FOP lesions may be locally recruited vascular cells or cells of the bone marrow stroma and that these cells maintain the potential (given the correct environmental stimuli) to differentiate along an endochondral ossification pathway.

Biopsy↗

Ossification process and lumbar spine morphology in the prenatal period.

BACKGROUND: Developmental abnormalities are the causes of numerous pathologies affecting the lumbar segment of the vertebral column. The aim of our study was metrologic assessment and analysis of lumbar vertebral column angioarchitecture during fetal life with particular emphasis on the description of vasculature of ossification centers of vertebral arches and bodies. MATERIAL/METHODS: The study was carried out on 30 fetuses with crown-rump length range of 31-180 mm. Vasculature of the ossification centers was studied using Pickworth's method and computed image analysis techniques in Imtron and Scion for Windows 2001 systems. The analysis involved the use of filters, binarization, linear and non-linear transformations. The assessment of morphology of lumbar spine structural elements was based on detailed measurements and statistical analysis of results. RESULTS: The analysis of vascular system of the ossification centers revealed the presence of numerous sinuses of various morphology. Their long axes were oriented in accordance with the directions of growth. The vasculature of the vertebral arch and body centers was characterized by a laminar structure. Centrally located sinuses and arteriovenous plexuses were covered by disseminated complexes of small blood vessels forming a cortex. The ossification centers were usually supplied with blood from other sources than cartilaginous elements. The analysis of metrological data demonstrated a marked domination of the L2 vertebra. CONCLUSIONS: Ossification of the lumbar vertebral column occurs in one rostro-caudal direction, The vasculature of the centers adjusts its morphology to the growth directions and not tendencies characterizing the object. The ossification process is characterized by marked autonomy and specific regulation mechanisms.

Crown-Rump Length↗

[Periarticular ossification. Complication of total hip prosthesis].

The authors analyse peri-prosthetic ossification, a complication which can occur in the presence of certain risk factors. Grades I and II calcifications are frequent and do not interfere with the final post-operative result. On the other hand, grades II and IV result in a progressive stiffening of the coxo-femoral joint within one year after the operation. They may be bilateral and can sometimes be responsible for serious handicap. These cases should be re-operated in order to restore joint mobility, but surgical treatment should be completed by radiotherapy according to a defined protocol. Non-steroidal anti-inflammatory agents and diphosphonates also have a preventative action. Subjects at risk (ankylosing vertebral hyperostosis, spondyloarthritis, alcoholism, patients with a history of ossification of the first side) require effective prophylactic treatment. In the authors' own series, 49 cases of ossification were observed in a retrospective analysis of 100 patients, including 6 cases of grades III and IV ossification. They analyse their results in relation to the initial hip pathology treated by prosthesis, but their series is too small to allow an statistically valid conclusions. Complementary studies are required.

Anti-Inflammatory Agents↗

Correlation between operative outcomes of cervical compression myelopathy and mri of the spinal cord.

STUDY DESIGN: Magnetic resonance images of cervical compression myelopathy were retrospectively analyzed in comparison with surgical outcomes. OBJECTIVES: To investigate which magnetic resonance findings in patients with cervical compression myelopathy reflect the clinical symptoms and prognosis, and to determine the radiographic and clinical factors that correlate with the prognosis. SUMMARY OF BACKGROUND DATA: Signal intensity changes of the spinal cord on magnetic resonance imaging in chronic cervical myelopathy are thought to be indicative of the prognosis. However, the prognostic significance of signal intensity change remains controversial. METHODS: The participants in this study were 73 patients who underwent cervical expansive laminoplasty for cervical compression myelopathy. Their mean age was 64 years, and the mean postoperative follow-up period was 3.4 years. The pathologic conditions were cervical spondylotic myelopathy in 42 patients and ossification of the posterior longitudinal ligament in 31 patients. Magnetic resonance imaging (spin-echo sequence) was performed in all the patients. The transverse area of the spinal cord at the site of maximal compression was computed, and spinal cord signal intensity changes were evaluated before and after surgery. Three patterns of spinal cord signal intensity changes on T1-weighted sequences/T2-weighted sequences were detected as follows: normal/normal, normal/high-signal intensity changes, and low-signal/high-signal intensity changes. Surgical outcomes were compared among these three groups. The most useful combination of parameters for predicting prognosis was determined using a stepwise regression analysis. RESULTS: The findings showed 2 patients with normal/normal, 67 patients with normal/high-signal, and 4 patients with low-signal/high-signal change patterns before surgery. Regarding postoperative recovery, the preoperative low-signal/high-signal group was significantly inferior to the preoperative normal/high-signal group. There was no significant difference between the transverse area of the spinal cord at the site of maximal compression in the normal/high-signal group and the low-signal/high-signal group. A stepwise regression analysis showed that the best combination of surgical outcome predictors included age (correlation coefficient R = -0.348), preoperative signal pattern, and duration of symptoms (correlation coefficient R = -0.231). CONCLUSIONS: The low-signal intensity changes on T1-weighted sequences indicated a poor prognosis. The authors speculate that high-signal intensity changes on T2 weighted images include a broad spectrum of compressive myelomalacic pathologies and reflect a broad spectrum of spinal cord recuperative potentials. Predictors of surgical outcomes are preoperative signal intensity change pattern of the spinal cord on radiologic evaluations, age at the time of surgery, and chronicity of the disease.

Adult↗

Progressive arthropathy in mice with a targeted disruption of the Mop3/Bmal-1 locus.

Disruption of the murine Mop3 (also known as Bmal1 or Arntl) locus results in a loss of behavioral and molecular circadian rhythms. Although Mop3 null mice do not display anomalies in early development, they do display reduced activity as they age. In an effort to explain this decreased activity, we characterized the physiological and anatomical changes that occurred with age. We observed that Mop3 null mice display an increased mortality after 26 weeks of age and a phenotype best described as a progressive noninflammatory arthropathy. Although little pathology is observed prior to 11 weeks of age, by 35 weeks of age essentially all Mop3 null animals develop joint ankylosis due to flowing ossification of ligaments and tendons and almost complete immobilization of weight-bearing and nonweight-bearing joints. This pathology appears to explain the decreased activity of Mop3 null mice and suggests that MOP3 is an inhibitor of ligament and tendon ossification.

ARNTL Transcription Factors↗

Bone remodeling in pathologic conditions. A scanning electron microscopic study.

Bone remodeling in pathologic conditions was studied with the scanning electron microscope (SEM). Benign and malignant ossification were examined in cases of myositis ossificans, ossifying fibroma, osteoid osteoma, and osteosarcoma, Resorption of bone due to invasion by non-ossifying tumors was found in cases of squamous cell carcinoma, adenocarcinoma, ameloblastoma, and multiple myeloma. Bone formation due to excessive production of growth hormone was studied in a case of acromegaly. Resorption of bone due to pathologic processes resembled the pattern found in surfaces which were undergoing resorption by osteoclasts. Lamelar-cortical bone formation in acromegally was similar in nature to normal bone. The deformities were rleated to the excessive continuous osteogenesis that occurs in these instances. Neoplastic ossification was characterized by calcifying globules, the diameters of which ranged from 1 to 3 micron. The surfaces of these globules were constructed of minute calcospherites with diameters ranging from 0.1 to 0.3 micron. It is suggested that the pattern of globular calcification is similar to the type that was found with the SEM in fetal bone and cartilage, during healing of fractured bone, and also with the TEM in normal and pathologic calcification.

Acromegaly↗

Case reports: ossified mass of the rotator cuff tendon in the subacromial bursa.

Unlike calcification, ossification is infrequent in the rotator cuff. We describe the clinical, radiographic, and pathologic findings in 64-year-old man with an ossified mass arising from a calcified portion of the rotator cuff tendon within the subacromial bursa. Mechanical stress and ischemic events are possible causes of cartilage formation followed by endochondral ossification, producing a mass causing outlet impingement.

Acromion↗

Solid variant of aneurysmal bone cyst: a case report with bilateral involvement of the distal femoral metaphyses.

An extremely rare case of bilateral, symmetrical involvement of distal femoral metaphyses by the solid variant of aneurysmal bone cyst (ABC) in a boy aged 13 years is described. Although there is no difference between the conventional ABC and the solid variant in terms of clinical and radiological presentation, the lesion is solid, composed of fibrohistiocytic cells with abundant giant cells and/or areas of florid, heterotopic ossification, while aneurysmal channels are sparse or absent. The lesion needs to be differentiated from giant cell tumour of bone, when the osteoclastic component predominates, while fibrous dysplasia, osteoblastoma and even osteosarcoma need to be excluded any time ossification is prominent. Careful evaluation of the clinical, radiological and pathological findings is necessary.

Adolescent↗

A biomechanical model of Peyronie's disease.

Peyronie's disease is a pathological condition of the penis which is characterized by localized ossification of the tunica albuginea. A common symptom of the chronic stage is penile deformity during erection, which is frequently associated with pain and erectile dysfunction. A two-dimensional biomechanical model of the penis was applied to study the development of Peyronie's disease by simulating the mechanical stress distribution which would result from the interaction of the ossified tunical tissue with other penile soft tissues. The model was solved by using commercial finite element software for a characteristic erectile pressure. The results demonstrate that Peyronie's plaques may induce intensified stresses around the penile nerves and blood vessels, up to double those in the normal penis. These elevated stresses may cause a painful sensation of neural origin or ischemia in regions of compressed vascular tissue. Severe penile deformities have been shown to develop if Peyronie's plaques develop only around one of the corpora cavernosa due to the non-homogeneous resistance of the tunica to expansion during erection. The present model can be clinically applied as an aid in the planning process of reconstructive surgery or insertion of a prosthesis.

Biomechanical Phenomena↗

Heterotopic mesenteric ossification ('intraabdominal myositis ossificans'): report of five cases.

Intraabdominal heterotopic ossification is a very uncommon disorder. We report five new cases, review the previous literature, and discuss the clinical and pathologic features of these lesions. The clinical features of the current cases and of those previously reported are remarkably similar. All patients were middle-aged to elderly men (range, 43-80 years; mean, 61 years) who had small bowel obstruction associated with heterotopic bone formation in the small bowel mesentery, often after one or more abdominal operations. In one case, an initial diagnosis of extraosseous osteosarcoma was considered. This unusual reactive process shares many of the clinical and pathologic features of myositis ossificans, as classically described in somatic soft tissues. We propose to designate this condition heterotopic mesenteric ossification.

Adult↗

Temporal bone findings in a case of sudden deafness and relapsing polychondritis.

Light microscopic examination was made of the right temporal bone from a 57-year-old female who developed sudden total hearing loss in both ears 1 year before death. The patient had suffered from relapsing polychondritis for 1 1/2 years prior to death. Pathological changes were compatible with known viral endolymphatic labyrinthritis. Slight ossification and fibrous tissue proliferation in the perilymphatic space seem to have been caused by a spread of infection from the middle ear. Susceptibility to viral infection in the labyrinth in patients with a long-devastating illness is discussed.

Acoustic Maculae↗

[Ossification of a protruded lumbar vertebral disc. A report of 17 cases].

From 1973 to 86, 308 patients were operated upon for protruded lumbar vertebral disc. 17 of them had 22 discs protruded and 19 of the 22 discs had been ossified. Operative findings and pathological characteristics of the specimens suggested that the ossified mass could be the result of ossification of the superficial tissue of the protruded disc, a sequence of turnover of fibrocartilage into osseous matter. We are of the confidence that operation is the choice of treatment of such lesion; and it should be performed early following diagnosis, as the ossified mass could never be absorbed, but progress on. The symptoms, lumbago and sciatica, of the 17 patients went immediately off after operation. Follow-up of 16 of them for 1.5 to 7.5 years (averaging 2.5 yrs.) showed excellent and good results in 87.8%.

Adult↗

Parosteal lipomas: a new perspective.

Parosteal lipomas, benign adipose tissue tumors situated directly on bone cortex, are unusual neoplasms that appear to emerge from multidirectional mesenchymal "modulation" within the periosteum. These tumors have been described as "periosteal lipomas", "chondrolipomas of soft tissue" and "lipomas of nerves" but they are most commonly believed to originate from the periosteum. Although over 100 of such tumors have been described in the literature, they have not been the subject of a comprehensive review, nor their potential for chondroid modulation and enchondral ossification emphasized. A review of 14 parosteal lipomas from the Bone Tumor Registry, Armed Forces Institute of Pathology, indicates these tumors are frequently associated with chondroid and/or osseous modulation, which permits subclassification into 4 distinct variants. Each of the 4 subtypes (I: No Ossification; II: Pedunculated Exostosis; III: Sessile Exostosis; IV: Patchy Chondro-Osseous Modulation) is illustrated to demonstrate the morphologic basis for radiologic/pathologic correlation and subclassification. A brief overview of the literature and pathogenesis of this unusual lesion is presented and discussed.

Aged↗