PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “FIBROUS DYSPLASIA OF BONE”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 775 records · Page 43Linked to original sources
PubMed1990

Surgical treatment of nasal obstruction secondary to craniofacial fibrous dysplasia.

Craniofacial fibrous dysplasia, a skeletal disorder most often affecting the mandible or maxilla, can cause a variety of complications. Nasal obstruction secondary to this unusual disorder presents a diagnostic and therapeutic challenge. The nasal obstruction in this case is due to expansion of fibrous lesions in adjacent bones as well as direct involvement of the turbinates with fibrous dysplasia. We describe the surgical correction of nasal obstruction in this unique setting and present a brief update of this intriguing disorder.

Adult↗
PubMed1988

[Surgical treatment of the polyostotic form of fibrous dysplasia in children and adolescents].

The work presents an experience with the treatment of 50 patients with polyossal form of fibrous osteodysplasia on whom 99 operations were performed. A method of plasty of bone defects was elaborated and used in order to eliminate severe deformations of lower extremity bones after removal of pathological tissue in combination with epiosseous nailing osteosynthesis. In patients with short bones the compression-distraction osteosynthesis with bone alloplasty was used. Long-term results of treatment of 48 patients make it possible to recommend the methods of bone alloplasty with extraosseous and intraosseous nailing osteosynthesis as well as compression-distraction devices for practical use.

Adolescent↗
PubMed2002

Fibrous dysplasia in combination with aneurysmal bone cyst of the occipital bone and the clivus: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Fibrous dysplasia of the cranium is a relatively uncommon disorder that affects primarily the anterior cranial region; its occurrence in the cranial base in combination with aneurysmal bone cyst (ABC) constitutes an extremely rare condition, only two cases of which have been reported previously in the literature. It is important to recognize and treat these cases properly because of the special location in the cranial base and the possibility of neural structure impingement. CLINICAL PRESENTATION: We report the case of a 19-year-old man with a slowly enlarging mass of the occiput, with computed tomographic and magnetic resonance imaging revealing involvement of petrous and basisphenoid bone and growing ABC. INTERVENTION: Open biopsy confirmed the diagnosis of fibrous dysplasia. Partial excision of the lesion and removal of the ABC were performed in a second stage after embolization. CONCLUSION: ABC associated with fibrous dysplasia of the cranial base may enlarge rapidly after puberty and require excision. This is facilitated by preoperative embolization.

Adult↗
PubMed1978

[Orthopedic problems in fibrous dysplasia in infant's bones. A report on 23 cases (author's transl)].

The authors report on 23 cases of fibrons in infants who had been treated surgically, and review the therapeutic problem which arise from this particular type of bone fragility. When located in the diaphysis they had been treated by screw fixation, isolated grafts being constantly unsuccessful. Various procedures had been used in 9 cases with localization in the proximal end of the femur, and they are reviewed critically.

Adolescent↗
PubMed2002

Juxta-articular myxoma and intramuscular myxoma are two distinct entities. Activating Gs alpha mutation at Arg 201 codon does not occur in juxta-articular myxoma.

Juxta-articular myxoma is a rare myxoid tumor of soft tissue that bears a close histologic resemblance to intramuscular myxoma but is distinguished from the latter by its clinical setting and behavior. Activating missense mutations at the Arg 201 codon of the Gs alpha gene ultimately leading to increased levels of cyclic adenosine monophosphate have been implicated in McCune-Albright syndrome and sporadic fibrous dysplasia of bone. Recently, we have demonstrated that the same Gs alpha mutations occur in intramuscular myxomas associated with fibrous dysplasia of bone (Mazabraud's syndrome) as well as in sporadic intramuscular myxoma. The overlapping histologic appearances of juxta-articular myxoma and intramuscular myxoma prompted us to investigate whether there is a relationship between the two entities. We studied this possibility by looking for Gs alpha mutations in juxta-articular myxoma using polymerase chain reaction (PCR) to amplify appropriate genomic DNA fragments extracted from formalin-fixed, paraffin-embedded specimens of five juxta-articular myxomas, followed by single-strand conformation polymorphism analysis. Using these techniques, no aberrant bands were detected in any of the five juxta-articular myxomas, indicating that they lack Gs alpha mutations. Moreover, DNA sequencing of the PCR products of two JAMs showed no abnormalities. We conclude that juxta-articular myxomas, in contrast to intramuscular myxomas, do not involve Arg 201 mutations of the Gs alpha gene, indicating that they represent distinct entities with different underlying molecular mechanisms.

Adult↗
PubMed2002

Transnasal endoscopic surgery for the treatment of fibrous dysplasia of maxillary sinus associated to aneurysmal bone cyst in a 5-year-old child.

Fibrous dysplasia (FD) and aneurysmal bone cyst involving the facial skeleton are rare but their synchronous association in other parts of the body is well-known. The most common treatment for these lesions has been based on surgical resection using an external approach. Only recently has the endonasal endoscopic approach been utilized for the partial or complete removal of these lesions. In this report, we attempt to outline the effectiveness of the endonasal endoscopic approach for the treatment of a 5-year-old child affected by a fibrous dysplasia associated with an aneurysmal bone cyst of the right maxillary.

Bone Cysts↗
PubMed2001

Comparative study of fibrous dysplasia and osteofibrous dysplasia: histopathological, immunohistochemical, argyrophilic nucleolar organizer region and DNA ploidy analysis.

Fibrous dysplasia and osteofibrous dysplasia are both benign fibro-osseous lesions of the bone. We retrospectively studied the clinicopathological findings in 90 cases of fibrous dysplasia and 17 cases of osteofibrous dysplasia. In these cases, the expression of proliferating cell nuclear antigen (PCNA) and the presence of argyrophilic nucleolar organizer regions (AgNOR), as well as DNA ploidy, were examined. The bones affected by fibrous dysplasia were the maxilla, femur and frontal bone. Osteofibrous dysplasia occurred exclusively in the tibia or fibula. The average age of patients with fibrous dysplasia (24.0 years) was higher than that of patients with osteofibrous dysplasia (12.9 years). Fibrous dysplasias were divided into four major histological subtypes: Pagetoid, Chinese alphabet, small bone and parallel bone. Bone lining cells, which are known as resting osteoblasts, were seen in some cases of fibrous dysplasia. Cartilage differentiation was not seen in osteofibrous dysplasia. PCNA expression was strongly positive in the nuclei of osteoblasts around the bone trabeculae in osteofibrous dysplasia, but negative in the nuclei of bone lining cells around the bone trabeculae in fibrous dysplasia. The number of AgNOR in osteofibrous dysplasia was slightly higher than that in fibrous dysplasia. Both fibrous dysplasia and osteofibrous dysplasia were diploid. These features suggest that fibrous dysplasia can be differentiated from osteofibrous dysplasia by anatomical site, patient age, histological appearance, cartilage differentiation and PCNA positivity. DNA content by image cytometry is not a useful tool for differentiating these two diseases.

Adolescent↗
PubMed2003

Dental characteristics of fibrous dysplasia and McCune-Albright syndrome.

OBJECTIVE: Fibrous dysplasia (FD) is a skeletal disorder often associated with McCune-Albright syndrome, a rare multisystem disorder caused by GNAS1 gene mutation. FD frequently affects the craniofacial bones, including the maxilla and the mandible; nevertheless, its effects on dental tissues and the implications for dental care remain unclear. The aim of this study was to characterize the dental features associated with FD and the reaction of affected bones to routine dental therapy. Study design Thirty-two patients with FD underwent dental evaluation and endocrine testing as part of the diagnosis of FD/McCune-Albright syndrome. Any dental anomalies were recorded, and the associations between endocrinopathies and dental anomalies were analyzed statistically by means of the paired t test. RESULTS: Eighty-four percent had FD in the maxilla and/or mandible; endocrine dysfunction; and/or renal phosphate wasting. The caries index scores were 2.9 (ages 4-17 years) and 9.6 (ages 18-50 years). Malocclusion (81%) and other prevalent dental anomalies (41%) included tooth rotation, oligodontia, and taurodontism. The expansion of the maxilla or mandible by FD did not distort the dental arch curvature, and routine dental therapies such as extractions, restorations, and orthodontic treatment did not exacerbate FD lesions. CONCLUSION: Maxillomandibular FD was associated with higher rates of caries and malocclusion than were present in healthy patients. Furthermore, patients with FD did not require special dental management and were able to undergo routine dental care without an exacerbation of FD lesions.

Adolescent↗
PubMed1980

Albright's hereditary osteodystrophy.

The authors observed different clinical forms of Albright's hereditary osteodystrophy in 4 members of a family (two sisters, their mother and the maternal grandfather). The sisters were affected by pseudohypoparathyroidism type I, the older manifested the hypocalcemic variety, the younger the normocalcemic variety; the mother and the grandfather presented only with short stature and subcutaneous calcifications. The variety of clinical and biochemical alterations observed in these 3 generations supports evidence that Albright's hereditary osteodystrophy has a broad spectrum and that distinctions between the various forms of pseudohypoparathyroidsim should not be rigidly considered.

Adult↗
PubMedDate not supplied

Value of bone scan in the McCune-Albright syndrome. Report of a case.

Bone scintigraphy proved valuable in diagnosing McCune-Albright's syndrome in a 6-year-old girl by detecting multiple, non-symptomatic foci in the skull, axial skeleton and in the extremities. Subsequent roentgen examination showed abnormal bone structure in the affected areas, consistent with fibrous dysplasia.

Bone and Bones↗