1/44. Familial polyostotic fibrous dysplasia.A case of polyostotic fibrous dysplasia of the craniofacial type is presented, together with substantial evidence that this condition had a genetic basis in this patient. A review of the literature indicates that there is absolutely no previous evidence of a genetic basis to this condition. The possibility that the propositus suffered from polyostotic fibrous dysplasia of the Jaffe or the Albright type was excluded.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
2/44. Fibrous dysplasia of the ethmoid sinus.Although craniofacial bone is the second common site of fibrous dysplasia involvement, it is rarely found in the paranasal sinus. Among fibrous dysplasia of the head and neck, the maxilla and mandible are the most frequent sites to be involved. Fibrous dysplasia becomes dormant in adolescence and early adult life and is more common in female. It is one of the fibrous osseous lesions and should be differentiated from osteoma and ossifying fibroma. Radiographically, fibrous dysplasia showed "groundglass" bone appearance on CT scans with bone window. Histopathologically, it presents woven-type bone embedded in a cellular fibrous stroma without osteoblastic rimming. We presented a case of 25-year-old female with fibrous dysplasia in her right side ethmoid sinus. She visited to us with the chief complaint of right side headache since adolescence. The lesion was removed by endoscopic sinus surgery and pathology proved fibrous dysplasia. The patient was free of headache after operation. The advance of endoscopic sinus surgical technique, makes it an optimal method for the pathological diagnosis and treatment to avoid the cosmetic problems caused by external approach in limited paranasal sinus fibrous osseous lesions.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
3/44. Clinical and radiological evaluation of cherubism: a sporadic case report and review of the literature.Many cases have been published on cherubism since Jones described it first time in three children of the same family [Am. J. Cancer 17 (1933) 946]. cherubism is an autosomal-dominant disorder in which the normal bone is replaced by cellular fibrous tissue and immature bone. Extracranial skeletal involvement is rarely seen in hereditary and non-hereditary forms of the disorder. The mandible is the most severely affected craniofacial component, in which uncontrolled growth of the malady deteriorates the aesthetic balance of the face. Bilateral swelling of the cheeks, mandibular enlargement and maxillary spongious hypertrophy cause orbital manifestations and tendency of eyes looking up to the sky. Thus, the pathognomic clinical feature resembles the appearance of "raised to heaven" Renaissance cherubs. The sporadic case concerns a child affected by cherubism. Radiographic and clinical data of the patient are presented and brief review of the literature is included.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
4/44. Craniofacial fibrous dysplasia. conservative surgical management. review of literature and report of a case.The present study presents a case of craniofacial fibrous dysplasia attenuating the orbit and the nasal airway, which was treated by conservative surgical approaches. Nasal and orbital decompression, and facial recontouring were performed simultaneously. This was done via a transconjunctival and transcaruncular approaches with a lateral canthotomy, which provided access to the four walls of the orbit, midface and the lateral nasal wall. The reduction of the turbinates was done through a standard turbinectomy approach and the nasal decompression was done via intercartilaginous access and a full transfixion incision and nasal degloving. The bulk of the lesion was removed with chisels, gauges, and rotary instruments. The orbital debulking extended to the orbital apex, which was not involved by the fibrous dysplasia. Approximately 200 mg of dysplastic bone was removed. The functional and aesthetic results were good. The symptoms of chronic conjunctivitis and nasal obstruction have been alleviated and there is no deterioration of his condition. The symptoms of anosmia have also disappeared. Conservative surgical treatment of craniofacial dysplasia in adults with stable disease is recommended.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
5/44. 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.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
6/44. frontal sinus obliteration and craniofacial reconstruction with platelet rich plasma in a patient with fibrous dysplasia.frontal sinus obliteration is accomplished with many materials such as fat, bone, muscle and alloplasts. Fat obliteration is very common but not devoid of morbidity. An alternative method of treatment with PRP platelet rich plasma and human bank bone or autogenous cortical shavings is presented for obliteration and craniofacial repair. A patient with severe symptomatic fronto-orbital fibrous dysplasia underwent reconstruction with PRP. After an uneventful recovery there was complete remission of the symptoms and early bone formation; 18 months after surgery the patient remains asymptomatic and free from sequels. PRP associated to an allograft or to cortical bone shavings seems to be a safe and simple treatment with the advantage of the absence of donor site surgery and morbidity.- - - - - - - - - - ranking = 5keywords = craniofacial (Clic here for more details about this article) |
7/44. HMPAO scintigraphy, MRI, and CT of a vascular fibrous dysplasia of the craniofacial bones.The authors present the case of a child with a predominantly unilateral craniofacial fibrous dysplasia and a substantial redirection of internal carotid blood flow to the dysplastic bone. The case illustrates the advantages of using multiple imaging modalities in the evaluation of this disorder. CT showed the diagnostic findings of fibrous dysplasia and demonstrated the extent of bony involvement. MRI showed the extent and vascularity of the intradiploic fibrous mass and best demonstrated the distortion of underlying cerebral structures. Tc-99m HM-PAO brain scintigraphy demonstrated adequate ipsilateral cerebral perfusion, thereby excluding any significant cerebral "steal."- - - - - - - - - - ranking = 5keywords = craniofacial (Clic here for more details about this article) |
8/44. Localized craniofacial fibrous dysplasia in a young girl.We consider here the case of a young girl presenting a cystic bone mass in the left orbital roof. Although standard x-rays at first were not convincing, the findings on the CT examination and the histological aspect of the mass were typical of fibrous dysplasia. Although the exact diagnosis of fibrous dysplasia can only be made upon precise histological examination, radiological, and more specifically CT findings, may indicate this pathology.- - - - - - - - - - ranking = 4keywords = craniofacial (Clic here for more details about this article) |
9/44. Craniofacial fibrous dysplasia.Fibrous dysplasia is a benign localized bone disorder of unknown aetiology in which endocrinopathies, abnormal pigmentation of the skin and mucous membranes may form part of the disease. Five cases involving the facial and cranial bones are described to illustrate the various presentations and radiological appearances including computerized tomography and magnetic resonance imaging. Some of these cases demonstrate that serial radiology may be adequate for diagnosis and management without the necessity for histological confirmation. The place of limited surgical treatment for the craniofacial sites is debated.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
10/44. An unusual case of craniofacial fibrous dysplasia presenting in early infancy.Fibrous dysplasia (FD) of bone is one of the most frequently encountered anomalies of skeletal development. It may involve one or more bones and, particularly when polyostotic, is sometimes associated with abnormal skin pigmentation and endocrine abnormalities. FD occurs mainly in large limb bones, ribs, and craniofacial bones in older children and young adults. Usually craniofacial involvement is detected because of local swelling or asymmetry of the face or head. Neurological symptoms, primarily due to involvement of the foramina, have been reported but are not common. Infantile fibrous dysplasia of the craniofacial region has rarely been reported.- - - - - - - - - - ranking = 7keywords = craniofacial (Clic here for more details about this article) |
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