Fibrous dysplasia of the craniofacial bones

Ramsey, Harold E.; Strong, Elliót W.; Frazell, Edgar L.

doi:10.1016/0002-9610(68)90390-5

Cited by 95 publications

(32 citation statements)

References 22 publications

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“…The treatment of fibrous dysplasia of the skull is, therefore, entirely surgical. Because of the paucity of information in the literature regarding the natural history, however, no clear consensus exists for the most appropriate surgical treatment strategy [5, 19, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37]. As surgical techniques have improved over the last three decades, there has been a trend towards more aggressive resections [18, 34, 38, 39].…”

Section: Discussionmentioning

confidence: 99%

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

et al. 2002

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Introduction: We evaluate the role of surgery in the treatment of fibrous dysplasia of the skull in children. Methods: We identified 48 consecutive cases of fibrous dysplasia of the skull that were surgically treated at a single institution over a 23-year interval. The 28 patients that initially presented during the first two decades of life were selected for further analysis. Presenting symptoms, signs, surgical treatment, surgical outcome and the state of the disease at the extended follow-up interval were recorded. Results: Fibrous dysplasia of the skull in children most often involved the frontal, sphenoid and ethmoid bones. Most patients presented with facial asymmetry or proptosis. A gross total resection was achieved in 7 patients, subtotal resection in 17 patients, and 4 patients underwent biopsy alone. Over the follow-up interval, 7 patients had symptomatic progression of subtotally resected fibrous dysplasia and 3 patients had asymptomatic progression. The extent of resection was correlated with recurrence risk. Conclusions: In order to prevent progression of disease, an attempt at gross total resection is indicated in cases of fibrous dysplasia of the skull in childhood where the risk of neurologic morbidity is low and cosmetic results will be acceptable.

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Section: Discussionmentioning

confidence: 99%

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

et al. 2002

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“…With this form of therapy the expectation is that the disease process will abate at skeletal maturity, obviating the need for the further recon struction: however, this is not always the case [8]. Accord ing to many authors, surgery is warranted only in the most severe cases because of concerns about the patient's appearance after massive resection [12,15,16].…”

Section: Discussionmentioning

confidence: 99%

Childhood Fibrous Dysplasia Presenting as Blindness: A Skull Base Approach for Resection and Immediate Reconstruction

Posnick

Wells²,

Drake³

et al. 1993

Pediatr Neurosurg

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Fibrous dysplasia is an abnormal fibroosseous process of bone of unknown cause. The Incidence of skull involvement varies, painless enlargement being the most common presenting symptom. Change in vision is a rare but recognized finding. We report a 3-year-old boy with extreme fibrous dysplasia involving the skull base, who presented with blindness. He underwent exposure osteotomies of the frontal bones and orbits to provide access for skull base tumor removal. The orbital roofs were reconstructed with microplate-fixed cranial grafts. One and one half years after tumor excision followed by immediate reconstruction, the boy retains facial symmetry, and his ocular function has not deteriorated.

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“…All three patients were young (11,12 and 15 years of age). Ramsey et al [7] have reported on 47 patients (age range, 3-37 years) with the disease. The majority of their patients (83%) were aged < 20 years.…”

Section: Discussionmentioning

confidence: 99%

“…Propor tions of the fibrous and osseous tissues vary, occasionally even in the zones of the same bone [2][3][4], Furthermore, recent data have pointed to a relationship between the preexisting primary bone lesion, such as fibrous dysplasia, and an aneurysmal bone cyst of the paranasal sinuses [5] ora mucocele [6], Various classifications of the disease have been pro posed. The one suggested by Ramsey et al [7] appears to be most convenient, describing three types of the disease: (a) monostotic, single or multiple lesions in the same bone, (b) polystotic, multiple lesions affecting more than one bone and (c) disseminated, multiple lesions, occasion ally accompanied by extraskeletal involvements.…”

Section: Introductionmentioning

confidence: 99%

Fibrous Dysplasia in Paranasal Cavities

Simović

Klapan²,

Bumber³

et al. 1996

ORL

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Presentation is made of 2 boys and a girl with fibrous dysplasia involving the frontal and ethmoidal sinuses and the maxilla. This tumor-like growth of the bone was not restricted to the sinus alone but expanded to the orbit, adjacent sinus, skull base and infratemporal or pterygoid fossa. In such cases, surgical therapy is the treatment of choice, with maximal preservation of healthy tissue and avoidance of major devastations that may result in undesired cosmetic defects. The etiology of the disease is still unknown. However, it should be emphasized that all 3 patients had suffered an injury to the adjacent bony structures at various time points preceding the disease manifestation. That is why we are inclined to believe that trauma might be a factor responsible for the onset of fibrous dysplasia.

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Fibrous dysplasia of the craniofacial bones

Cited by 95 publications

References 22 publications

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

Childhood Fibrous Dysplasia Presenting as Blindness: A Skull Base Approach for Resection and Immediate Reconstruction

Fibrous Dysplasia in Paranasal Cavities

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