1/12. The aetiology of congenital angulation of tubular bones with constriction of the medullary canal, and its relationship to congenital pseudarthrosis.It is suggested that there is a group of cases of congenital angulation of tubular bones in which the lesion is a defect of ossification of the primary cartilaginous anlage and in which neurofibromatosis is not implicated. It appears that in this group the prognosis with regard to the resolution of deformity and the prevention of pseudarthrosis with conservative treatment or relatively simple surgical procedures is better than that in the neurofibromatous type.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
2/12. A case report on aggressive fibromatosis with bone involvement.Aggressive fibromatosis is a locally infiltrative fibroblastic tumour that arises from fascial planes of soft tissue but does not metastasize. It is known to invade muscle, subcutaneous tissue and neurovascular structures. However, bone involvement is very rare and there has been few reports of bone involvement. We present a case of a young man with aggressive fibromatosis of the right lower leg with fibula involvement.- - - - - - - - - - ranking = 6keywords = fibromatosis (Clic here for more details about this article) |
3/12. hamartoma involving the pseudarthrosis site in patients with neurofibromatosis type 1.Congenital pseudarthrosis is a rare disease with variable clinical effects. The disease remains 1 of the most controversial pediatric entities in terms of etiopathogenesis, therapy, and prognosis. Between 0.5% and 2.2% of patients with neurofibromatosis demonstrate pseudarthrosis in any of the long bones. The exact origin of the lesion is even unclear; although several attempts have been made to determine the type of tissue involving the pseudarthrosis site, only fibrous tissue has been documented in different reports. We present 2 unrelated Mexican patients (male and female) with familial neurofibromatosis and congenital pseudarthrosis of the tibia and fibula. Histochemical and immunostain studies after surgical resection of the affected ends from the pseudarthrosis site of both patients showed a picture compatible with hamartoma. This is the first time when histologic evidence of hamartomatous tissue involving the pseudarthrosis site is presented.- - - - - - - - - - ranking = 6keywords = fibromatosis (Clic here for more details about this article) |
4/12. Recognizing an index case of type 1 neurofibromatosis.Neurofibromatosis is a relatively common autosomal dominant disorder with variable penetrance. The disorder usually presents in childhood. Hallmarks of type 1 neurofibromatosis are cafe-au-lait macules and neurofibromas. Neurologic complications include mental retardation, learning disabilities and seizures. Tumors of the eighth cranial nerve, as well as other intracranial and spinal neoplasms, are the typical lesions in type 2 neurofibromatosis. Both forms of neurofibromatosis have a highly variable course and may result in progressive neurologic deterioration, disfigurement and impingement syndromes. In the 50 percent of cases that represent new mutations, diagnosis may be delayed if the physician is not familiar with the salient features of the disorder. Thorough initial evaluation, genetic counseling and close follow-up are important aspects of management.- - - - - - - - - - ranking = 8keywords = fibromatosis (Clic here for more details about this article) |
5/12. Recurrent massive subperiosteal hemorrhage in a patient with neurofibromatosis: a case report.The authors report a rare complication of neurofibromatosis, recurrent rapidly expanding massive subperiosteal hematomas, in the thigh of a 22-year-old woman. The diagnosis was confirmed by plain radiography, ultrasonography and computed tomography. The first two hematomas were drained through a longitudinal lateral incision, tightly closing the periosteum over drains and applying a compression dressing postoperatively. The third hematoma was treated expectantly. Recurrent subperiosteal hematomas may lead to the formation of huge bone cysts and grotesque limb deformity, for which amputation may be the only solution.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
6/12. Subperiosteal hematoma. Another scintigraphic "doughnut".The "doughnut" sign has been described frequently in brain and skeletal scintigraphy, but not previously in a subperiosteal hematoma associated with neurofibromatosis and focal gigantism. The primary cause and evolution of this skeletal "doughnut" sign is discussed; a brief review of other causes of this scintigraphic finding also is given.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
7/12. Congenital multiple fibromatosis (infantile myofibromatosis).A female newborn had a rare case of congenital multiple fibromatosis, consisting of multiple fibrous lesions that histologically resemble myofibromas. Bony and soft-tissue lesions usually undergo spontaneous resolution, but excision may be required if vital structures are compromised as was the case in this patient. When the lesions involve the viscera in a generalized form of the disease, the result is often fatal.- - - - - - - - - - ranking = 9keywords = fibromatosis (Clic here for more details about this article) |
8/12. Massive subperiosteal hemorrhage in neurofibromatosis.Neurofibromatosis is seen in association with elephantiasis neuromatosa and overgrowth of abnormal bones, but rarely with subperiosteal hemorrhage.This is a secondary finding after severe or minor trauma to the periosteum, which is abnormally loose from mesodermal dysplasia. The clinical, plain radiographic, and angiographic findings of 2 cases of massive subperiosteal hemorrhage are presented, and the literature reviewed.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
9/12. Neurofibromatosis with central neurofibroma of the mandible: review of the literature and report of case.The clinical, radiographic, and etiological factors of neurofibromatosis have been discussed. A rare case of a large neurofibroma of the mandible in association with systemic neurofibromatosis and a characteristic mandibular dysplasia has been presented. The unique coexistence of mandibular dysplasia associated with sphenoidal and orbital dysplasia should be considered in the diagnosis of multiple neurofibromatosis.- - - - - - - - - - ranking = 7keywords = fibromatosis (Clic here for more details about this article) |
10/12. Subperiosteal hemorrhage and cyst formation in neurofibromatosis: a case report.A 7-year-old girl was disabled by neurofibromatosis complicated by leg hypertrophy, recurrent massive hemorrhage, and cyst formation in her right tibia. A grotesque deformity resulted in the right lower leg over a three-year period despite elevation, compression, and aspiration for various bleeding episodes. Massive areas of bone had to be resected on two separate occasions. The extreme vascularity of the region caused postoperative recurrences, but there was always some improvement. The pathologic findings consisted of true schwannomas in the periosteum. The basic problem of neurofibromatosis was the endoneural cell proliferation that was responsible for the hypertrophy, hemorrhages, and cyst formation. While there are various hemorrhagic problems in neurofibromatosis, this case demonstrates the characteristic massive hemorrhage and rapid new bone formation that follow minimal trauma, and illustrated the difficulties that are encountered in both nonoperative and operative management.- - - - - - - - - - ranking = 7keywords = fibromatosis (Clic here for more details about this article) |
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