Abstract
BackgroundFibrous dysplasia (FD) is a developmental disease of bone in which there is replacement of normal spongiosa and filling of the medullary cavity of affected bones by an abnormal fibrous tissue that contains trabeculae of poorly calcified primitive bone formed by osseous metaplasia. Fibrous dysplasia is a common benign bone disease existing in monostotic and polyostotic forms. It is sometimes associated with aneurysmal bone cysts, and it is a component of McCune-Albright and Mazabraud syndromes.Case presentationWe describe here a 4-months old Austrian infant who presented with a hard bulging painless mass of (5 x 3 cm) of the right parietal bone. Radiographs showed a large irregular osteolytic lesion. T1-weighted MR image showed significant expansile lesion associated with a dense zone of calcification in the diploic space. To the best of our knowledge this is the first clinical report of an infant with early presentation of monostotic fibrous dysplasia of the right parietal bone.ConclusionFibrous dysplasia of the skull is a painless progressively expanding destructive bone swellings produce cosmetic deformities. The clinical course may be unpredictable, with sudden appearance of symptoms, some of which can be important and irreversible. In our present patient, the possibility that an early surgical correction might positively interfere with the natural history of the lesion has to be evaluated by taking into account the obvious difficulties that will be encountered in reconstructing the skull after a wide excision of the pathologic bone.
Highlights
Fibrous dysplasia (FD) is a developmental disease of bone in which there is replacement of normal spongiosa and filling of the medullary cavity of affected bones by an abnormal fibrous tissue that contains trabeculae of poorly calcified primitive bone formed by osseous metaplasia
Skeletal fibrous dysplasia is a localised disorder of bone characterised by abnormal proliferation of fibrous tissue interspersed with normal or immature bone, endocrine dysfunction, abnormal pigmentation, and precocious puberty in girls
Fibrous dysplasia, when it occurs in the craniofacial region, mostly involves the skull base and is rarely localized in the cranial vault
Summary
Most lesions of FD are monostotic, asymptomatic, and identified incidentally and can be treated with clinical observation and patient education. Knowledge of the various appearances, complications, and associations of fibrous dysplasia is important to ensure the accurate diagnosis and appropriate management of this disease. Surgery is indicated for confirmatory biopsy, correction of deformity, and prevention of pathologic fracture and or eradication of symptomatic lesions. It has been postulated, that surgical treatment is needed when the patient have significant clinical symptoms. FD: Fibrous dysplasia; GNAS 1: guanine nucleotide-binding protein, α-stimulating activity polypeptide 1
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