1/15. Clinical presentation of fibrodysplasia ossificans progressiva: pitfalls in diagnosis.A patient presented with a rapidly growing mass in the cervicothoracic paraspinous region. Associated clinical and radiographic skeletal abnormalities and histopathologic findings showing mild to moderate proliferation of spindle cells in a myxoid stroma led to the diagnosis of fibrodysplasia ossificans progressiva. Recognizing the clinical features of this disease is important and should avoid the need for obtaining tissue to make the diagnosis, because performing a biopsy may be particularly morbid in these patients.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/15. A brachial plexopathy due to myositis ossificans. Case report and review of the literature.myositis ossificans (MO) is a disorder characterized by the intramuscular proliferation of fibroblasts and osteoblasts, with subsequent deposition of bone and cartilage. A typical clinical presentation involves traumatic injury to a young adult, usually localized to the thigh, buttock, or upper arm, with resultant MO and mildly restricted range of motion in adjacent joints. Rarely, MO is associated with peripheral neuropathies involving the radial, median, sciatic, and sural nerves. The authors present an unusual case of MO causing a brachial plexopathy. To their knowledge, this is the first description of such a presentation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/15. Fibrodysplasia ossificans progressiva.Fibrodysplasia ossificans progressiva, a rare genetic disabling disease characterized by heterotopic bone formation, is of special interest for general medicine since the bone morphogenetic proteins (especially BMP-4) involved in its pathogenesis are known to play a role in skeletal morphogenesis, and the gene antagonist to BMP-4 noggin might be useful in preventing lamellar bone formation. We present two cases with characteristic musculo-skeletal abnormalities and histopathological features (inflammatory infiltrates which destroyed muscle tissue replacing it by proliferating fibroblasts). In one patient due to high activity of fibroblasts, the histopathologic pattern appeared to be atypical and the lesion was recognized by a general pathologist as sarcoma. The other patient, due to the symmetrical induration of sternocleidomastoid muscles, was primarily recognized as scleredema. We stress the diagnostic importance of skeletal abnormalities (hallux valgus and others), and discuss differentiation from progressive osseous heteroplasia (POH) and congenital or acquired localized cutaneous and muscle ossifications which have a much better prognosis, as well as Albright's hereditary osteodystrophy, which differs by the presence of various systemic abnormalities. A study of FOP might provide an important clue to the genetic molecular mechanism of bone formation, development of heterotopic bone and a possible prevention by molecular manipulation with the gene responsible for bone morphogenetic proteins and their antagonists.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/15. The subcutaneous fascial analogue of myositis proliferans: electron microscopic examination of two cases and comparison with myositis ossificans localisata.Two cases of the so-called fascial analogue of myositis proliferans were investigated by histological and electron microscopic methods. It was found that the structure of the fascial variant corresponds almost completely to the true myositis proliferans localized within the musculature. The electron microscopic observations show a preponderantly histiocytic differentiation of the cells and strongly activated proliferating capillaries, and exclude a myogenic origin of the characteristic ganglion-like giant cells. Ultrastructurally a traumatic genesis appears possibly, the cells of the lesion could derive from multipotent cells of the microvasculature. The relations to myositis ossificans and fascitis nodularis are discussed.- - - - - - - - - - ranking = 7keywords = life (Clic here for more details about this article) |
5/15. Reactive mesenchymal proliferation.One hundred and thirteen cases in the files of the netherlands Committee on Bone Tumors were diagnosed as heterotopic soft tissue ossification. myositis ossificans was diagnosed in 62, ossifying hematoma in 21, and pseudomalignant osseous tumor of soft tissues in 30 cases. Antecedent trauma was present in 37%, 46% and 7%, respectively. myositis ossificans arose in the large muscle groups of the thighs and upper arms; when closely related to the shaft of a bone, periosteal reactions were more outspoken. Pseudomalignant osseous tumor of soft tissues was located in the hands, feet, and pelvis, some cases were not in muscle groups and some were almost periosteal. Ossifying hematoma was located in the upper and lower legs and usually in close relation to bone. The three entities belong to the same kind of reactive mesenchymal proliferative process. The radiologic and histologic patterns are reflections of whether the lesions are closely localized to bone shafts or in soft tissues, e.g. in muscles and therefore the term "reactive mesenchymal proliferation" is preferred to myositis ossificans, pseudomalignant osseous tumor of soft tissues and ossifying hematoma. Diagnostic problems are encountered in early phases when cellularity, mitotic activity, and infiltrative spread suggest malignancy. Recognition of these reactions in early phases is important to avoid mutilating surgery.- - - - - - - - - - ranking = 6keywords = life (Clic here for more details about this article) |
6/15. Radiologic demonstration of temporal development of bizarre parosteal osteochondromatous proliferation.We report a case of bizarre parosteal osteochondromatous proliferation (BPOP) in which radiologic examinations showed temporal development of BPOP following a traumatic event. This evidence supports the theory that BPOP is caused by trauma.- - - - - - - - - - ranking = 5keywords = life (Clic here for more details about this article) |
7/15. myositis ossificans in children: description of a clinical case with a rare localization.myositis ossificans (MO) also defined as myosteosis or hematoma ossificans, is a benign condition presenting as an heterotopic, well- defined neoformation in muscles and soft tissues. It was first described by Guy Patyn in 1692 and defined in its histopathological aspects by Von Dusch in 1868. It most frequently has a post-traumatic onset (60-75% of cases), usually following small repeated traumas or a single bruising episode. MO is rare in subjects under 10 years of age, whereas it is more frequent in teen-age athletes, and over 50% of cases are diagnosed in the third decade of life. Its etiopathogenesis in unknown, although it is associated with a traumatic event in 75% of cases. MO most common localizations are arms, legs, shoulders and hands, rarely chest. The lesion presents with different degrees of maturation and diagnostic tools are echotomography (ECT) as a primary investigation, and NMR for a better diagnostic assessment. Because of the self-limiting nature of the lesion and its spontaneous resolution, a conservative treatment is advised along with radiological follow-up which is most indicated in the presence of either typical MO features or highly suggestive ECT o NMR findings. In case of uncertain diagnosis, relevant muscular function impairment, considerable lesion dimension or severe pain, exeresis and histological examinations are suggested. The present paper describes and discusses a clinical case of MO in a child, with a rare localization.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/15. Anaesthetic management of a child with fibrodysplasia ossificans progressiva.Fibrodysplasia Ossificans Progressiva (FOP) is a rare inherited disease in which progressive ossification of striated muscles leads to severe disability and respiratory impairment early in life and there are associated characteristic congenital skeletal malformations. Although this condition may prove demanding for the anaesthetist, few reports are available regarding anaesthesia in paediatric patients. We review the clinical features of the disease relevant to anaesthesia and describe the perioperative care of an 18-month-old boy with FOP who underwent endoscopic third-ventriculostomy for hydrocephalus associated with a brainstem mass. We emphasize the importance of an appropriate anaesthetic management of these patients, as inadequate care may lead to exacerbation of the disease with permanent sequelae.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/15. anesthesia in 'stone man': myositis ossificans progressiva.myositis ossificans progressiva is a rare disease leading to complete ossification of the muscular system. Very little information about this rare disease and its anesthetic implications has appeared in anesthetic literature. This disease is felt to have an autosomal dominant pattern of inheritance and is usually associated with anomalies of the hands and feet. Afflicted patients are frequently misdiagnosed in childhood as having a rheumatologic disorder. Later in life (as true bone is formed in striated muscle, ligaments, and fascia), the correct diagnosis becomes obvious. Although muscles of the heart, diaphragm, larynx, and sphincters are spared, those of the chest wall are not, and pulmonary function progressively deteriorates. death frequently occurs as a result of a pulmonary infection. Specific anesthetic considerations include positioning to avoid injury, potential need for fiberoptic intubation or tracheostomy, decreased thoracic compliance with the need for increased ventilating pressures, and the ultimate in disuse atrophy contraindicating the use of succinylcholine. Myositis ossificans progressiva can present the anesthesiologist with interesting challenges. Anesthetic management will need to be individualized according to the severity of the disease.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/15. Peripartum pseudomalignant myositis ossificans of the finger.Two cases of pseudomalignant peripartum myositis ossificans of the finger are reported. The benign diagnosis is confirmed by the histologically characteristic zone phenomenon, consisting of a transition from an inner zone of proliferating spindle cells to a middle zone of well-oriented osteoid and finally to an outer zone of mature bone. In Case No. 1, because of the question of malignancy, a ray amputation was performed. In Case No. 2, the patient was observed during the peripartum period to show diminution in the size of the tumor. The lesion was eventually treated by local excision.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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