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Fibrous dysplasia of the femoral neck. Treatment by cortical bone-grafting.

Fibrous dysplasia of the femoral neck is difficult to treat. In a series of fifteen young patients, ten had a monostotic lesion and five, the polyostotic form of the disease. Twelve patients were first seen with a fatigue fracture. Grafts of cortical bone were used. The objectives of relief of pain, union of the fracture, and prevention of deformity were achieved in all fifteen patients. Two patients required a repeat procedure. None of the patients had important deformity of the femoral neck, and none needed an osteotomy.

Adolescent↗

[Monostotic fibrous dysplasia of the occipital bone].

A case of monostotic fibrous dysplasia of the occipital bone in a 18-year-old female is described. She was admitted to our hospital with a hard, painless mass (5 x 5 cm) in the occipital region. Neurological examination and laboratory data revealed no abnormalities. Skull x-rays showed a radiolucent lesion with a sclerotic margin in the occipital bone. CT scan revealed an intradiploic multilocular mass separated by bone trabeculae. RI bone scan showed an abnormal uptake in this lesion. T1 and T2 MR image showed an abnormal low-intensity lesion in the occipital region. Operation was performed to verify the nature of the lesion, which was histologically confirmed to be an inactive fibrous dysplasia. Postoperative course was uneventful. Although its occurrence in the occipital bone is uncommon, fibrous dysplasia should be considered in the differential diagnosis of skull tumors.

Adolescent↗

[Fibrous dysplasia of the facial bones. A CT study].

The authors report 7 cases of fibrous dysplasia of the facial bones which were evaluated with CT. The involvement of the facial bones by fibrous dysplasia is an uncommon event, which causes different syndromes according to the extent of bone invasion. In all the cases occurred to our observation CT allowed an exact spatial evaluation of the dysplasia, and therefore a correct surgical planning. Moreover, CT densitometric values and CT appearance of fibrous dysplasia often suggested the correct diagnosis.

Adolescent↗

[Fibrous dysplasia of facial bones. 43 cases (author's transl)].

Clinical material of 43 patients treated for fibrous dysplasia of facial bones at the Department of Maxillofacial Surgery, Medical Academy in Cracov in the years 1956--1978 has been investigated. The author's own observations concerning diagnostics and treatment of these patients are presented. Diveristy of clinical forms and variability of the disease process dynamics likewise the difficulties in treatment resulting from unacquaintance with etiology of the disease are emphasized. The results of treatment obtained on the basis of control examination during two to fifteen-year periods of observation are described.

Adolescent↗

Fibrous dysplasia of middle turbinate associated with Widal syndrome: endoscopic treatment of a rare case.

Fibrous dysplasia, a rare bony disease, is characterised by substitution of normal bone with immature tissue embedded in a fibrous stroma. It can be either monostotic or involve several bones. Fibrous dysplasia is usually asymptomatic but, in the advanced stage, pain due to neural compression or pathological fractures may occur. In the case of cranio-facial involvement, ocular, masticatory, respiratory or auditory functional alterations are possible. A case of fibrous dysplasia, limited to the middle turbinate and associated with Widal triad (sinus-nasal polyposis, asthma, acetyl salicylic acid intolerance), is described. Craniofacial computed tomography revealed enlargement of left middle turbinate with characteristic "ground-glass" appearance. The patient underwent anterior bilateral functional endoscopic sinus surgery with near-total resection of left middle turbinate. Histopathological examination confirmed the diagnosis of fibrous dysplasia. After 2 years the patient is still asymptomatic. Videorhinoscopy shows good sinus-nasal patency without disease recurrence. Even though exceptional, localization of fibrous dysplasia at middle turbinate has been described, therefore, it must be considered in the differential diagnosis of the craniofacial ossifying disorder. For localized and symptomatic lesions, endoscopic surgery is an effective option. Scrupulous life-long follow-up is necessary due to the high percentage of recurrence and possible malignant degeneration.

Adult↗

Osteogenic sarcoma and soft tissue myxoma in a patient with fibrous dysplasia and hemoglobins JBaltimore and S.

A 41-year-old man with recognized polyostotic fibrous dysplasia since late childhood developed fibroblastic osteogenic sarcoma in the left tibia. Four months after the initial diagnosis, an intramuscular myxoma was discovered in the left thigh. Twenty years previously he had been found to be heterozygous for hemoglobins JBaltimore and S. Malignant transformation in fibrous dysplasia is unusual and may be associated in some individuals with prior irradiation. Soft tissue myxomas associated with fibrous dysplasia are even rarer. To the best of the authors' knowledge the occurrence of both of these lesions in a patient with fibrous dysplasia has been reported only once before. Patients with both fibrous dysplasia and myxomas may be at greater risk for malignant transformation than are individuals with only one of these lesions. There is no well-recognized association between hemoglobinopathies and either fibrous dysplasia or bone tumors. It is therefore probable that the rare constellation of findings is in this patient a stochastic event.

Adult↗

Fibrous dysplasia of mandibular bone in a dog.

A nine-year-old, male German shepherd dog was presented with a firm, discrete mass lingual to the interdental space of the mandibular left third and fourth premolars. The lesion was excised and diagnosed as fibrous dysplasia of bone based on histopathologic examination. Fibrous dysplasia of bone is a rare, non-aggressive, radiolucent, non-neoplastic lesion considered developmental in origin. Usually observed as a solitary lesion, it has been reported in animals and man in the polyostotic form. The disease process causes deformity of bone which may lead to loss of bone strength. Clinical signs are secondary to site specific problems associated with expansile bone lesions. Oral examination performed 18-months postoperatively indicated no signs of recurrence.

Animals↗

In vivo effects of surgical sympathectomy on intramembranous bone resorption.

Bone modeling and remodeling are highly regulated processes in the mammalian skeleton. The exact mechanism by which bone can be modeled at a local site with little or no effect at adjacent anatomic sites is unknown. Disruption of the control of modeling within the temporal bone may lead to various bone disease such as otosclerosis, osteogenesis imperfecta, Paget's disease of bone, fibrous dysplasia, or the erosion of bone associated with chronic otitis media. One possible mechanism for such delicate control may be related to the ubiquitous and rich sympathetic innervation of all periosteal surfaces. Previous studies have indicated that regional sympathectomy leads to qualitative alterations in localized bone modeling and remodeling. In this study, unilateral cervical sympathectomy resulted in significant increases in osteoclast surface and osteoclast number within the ipsilateral bulla of experimental animals. The mechanisms by which sympathectomy leads to increased local bone loss is unknown. Potential mechanisms include disinhibition of resorption, secondary to the elimination of periosteal sympathetics, as well as indirect vascular effects.

Animals↗

[Rare diseases of the ear: fibrous dysplasia of the tympanic bone, extensive papillomatosis, ceruminoma, aspergilloma, spontaneous évidement of the tympanic bone].

The authors report six cases of rare ear diseases: fibrous dysplasia of the tympanum mimicking an partially obstructive osteoma of the external auditory canal. The authors draw a parallel between the rarity of these monostotic forms of the temporal bone which are strictly localised to a single region (tympanum-mastoid-atrium) and the diffuse forms which involve several regions at the same time; extensive papillomatosis of the external and middle ear, a rare condition with serious repercussions; two ceruminomas with very different clinical presentations and outcomes; an atrial pseudo-tumoral aspergilloma which was easily excised ans repaired with a myringoplasty with a good long term result; hypoplasia of the tympanum, which constituted a spontaneous évidement, discovered at operation for an attic cholesteatoma. This finding was sufficiently unusual to warrant reporting.

Adenocarcinoma↗

McCune-Albright syndrome and acromegaly: clinical studies and responses to treatment in five cases.

We report here five new patients with McCune-Albright syndrome and acromegaly. In the five patients studied (three males and two females aged 18-42 years), acromegaly began before the age of 20 years and was recognized after the diagnosis of fibrous dysplasia, which was polyostotic in three cases and monostotic in two. Bone fibrous dysplasia always involved the base of the skull and in four patients prevented surgical removal of the pituitary adenoma, which was visualized easily by magnetic resonance imaging. Serum growth hormone (GH) levels ranged between 20 and 48 micrograms/l and were not suppressed by an oral glucose load. Thyrotropin-releasing hormone administration produced a paradoxical increase in serum GH levels in all the patients. Four of the five patients had hyperprolactinemia (43-670 micrograms/l). In the sole patient who could be operated on, a typical adenoma with positive immunostaining for GH was incompletely removed and postoperative radiation therapy failed to cure the acromegaly. In two patients, medical therapy with bromocriptine and/or octreotide was partially or totally ineffective whatever the dose (up to 1.5 mg per day) and duration (2-4 years) of octreotide treatment.

Acromegaly↗