Fibrous Dysplasia of the Skull in Children

Rocco, Concezio Di; Marchese, Enrico; Velardi, F.

doi:10.1159/000120649

Cited by 17 publications

(7 citation statements)

References 15 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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“…49 Consideration of aggressive surgical approaches to control disease-related complications must be weighed against the known difficulties associated with reconstructing large bone defects and patients' unpredictable clinical courses. 27 Advocates for early surgery believe that conservative treatment during the "active" phase in children is unacceptable 16 because there is no indication that surgical procedures inhibit the growth rate of residual normal tissues 49 and progression of disease can continue into adulthood (Fig. 3).…”

Section: Fibrous Dysplasiamentioning

confidence: 99%

Tumors of the skull base in children: review of tumor types and management strategies

Tsai¹,

Santoreneos²,

Rutka³

2002

FOC

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Although many treatment strategies for skull base tumors in adults have been reported, relatively little has been reported regarding such therapies in the pediatric population. Skull base tumors in children present a therapeutic challenge because of their unique pathological composition, the constraints of the maturing skull and brain, and the small size of the patients. In this review, the authors examine the pediatric skull base lesions that occur in the anterior, middle, and posterior cranial base, focusing on unique pediatric tumors such as encepahalocele, fibrous dysplasia, esthesioneuroblastoma, craniopharyngioma, juvenile nasopharyngeal angiofibroma, cholesteatoma, chordoma, chondrosarcoma, and Ewing sarcoma. They review management strategies that include radio- and chemotherapy, as well as surgical approaches with emphasis on the modifications and complications associated with the procedures as they apply in children. Evidence for the advantages and limitations of radiotherapy, chemotherapy, and surgery as it pertains to the pediatric population will be examined. With a working knowledge of skull base anatomy and special considerations of the developing craniofacial skeleton, neurosurgeons can treat skull base lesions in children with acceptable morbidity and mortality rates. Outcomes in this population may be better than those in adults, in part because of the benign histopathology that frequently affects the pediatric skull base, as well as the plasticity of the maturing nervous system.

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“…Some authors have stated that FD of the skull respects bony sutures, this is not always the case as evidenced by our patient [8]. Thus differentiation between monostotic and polyostotic FD in craniofacial cases is sometimes very difficult [9]. Craniofacial FD usually presents as a painless mass [9].…”

Section: Discussionmentioning

confidence: 73%

“…Thus differentiation between monostotic and polyostotic FD in craniofacial cases is sometimes very difficult [9]. Craniofacial FD usually presents as a painless mass [9]. Symptoms are secondary to mass effect (e.g.…”

Section: Discussionmentioning

confidence: 99%

Concomitant Fibrous Dysplasia and Aneurysmal Bone Cyst of the Skull Base

et al. 1998

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The combination of fibrous dysplasia and aneurysmal bone cyst of the skull base is extremely rare yet it should be recognized, lest the rapid growth of the lesion and the radiological picture lead to the wrong diagnosis of a malignant process. We report on a 6½-year-old patient who suffered from concomitant fibrous dysplasia and aneurysmal bone cyst of the skull base. He is still disease-free 4 years postoperatively. The treatment of choice remains early surgical removal of the maximal amount of involved bone that can safely be achieved. There is no role for radiotherapy in the treatment of these lesions. A review of the literature is included.

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Fibrous Dysplasia of the Skull in Children

Cited by 17 publications

References 15 publications

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

Tumors of the skull base in children: review of tumor types and management strategies

Concomitant Fibrous Dysplasia and Aneurysmal Bone Cyst of the Skull Base

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