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. 2008 Nov 24;1(1):347.
doi: 10.1186/1757-1626-1-347.

Outward bulging of the right parietal bone in connection with fibrous dysplasia in an infant: a case report

Ali Al Kaissi  1 , Klaus Klaushofer, Franz Grill
Affiliations

Outward bulging of the right parietal bone in connection with fibrous dysplasia in an infant: a case report

Ali Al Kaissi et al. Cases J. .

Abstract

Background: 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. 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 presentation: We 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.

Conclusion: Fibrous 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.

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Figures

Figure 1
Figure 1
Anteroposterior skull radiographs showed a multicystic lesion with slightly sclerotic margin in the right parietal bone, associated with significant outward bulging of the outer table without destruction of the inner table in tangential view of the right parietal bone.
Figure 2
Figure 2
Lateral radiograph of the skull showed large osteolytic/multicystic lesion associated with thick sclerotic rind in the right parietal bone.
Figure 3
Figure 3
T1 weighted image of the skull showed expansile lesion within the parietal bone. The lesion is larger than appreciated on the radiograph. There is mixed hyper and hypointenstities. It is significantly expanded with well-defined margins.
Figure 4
Figure 4
Sagittal T1-weighted MR image showed significant expansile lesion associated with a dense zone of calcification within the mass-arrow (fig 4).
See this image and copyright information in PMC

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