A case of polyostotic fibrous dysplasia leading to multiple deformities
Articles > Article · 2014
Authors: Nilufer Ozdemir Kutbay, Banu Pınar ŞARER YÜREKLİ, Lütfiye Füsun SAYGILI
International Journal of Case Reports and Images, International, 2014, vol 5, pp 734-734
DOI: 10.5348/ijcri-201465-cl-10053
Summary
Fibrous dysplasia (FD) is a rare disease leading to increased bone resorption due to abnormal fibroblast proliferation and defective osteoblast differentiation. As it can be asymptomatic, FD can also cause bone pain, deformity and compression symptoms. Fibrous dysplasia can present itself in either a monostotic form, with involvement of one bone site, or a polyostotic form that includes multiple bone sites. Monostotic forms comprise 7080% of FD and rest of FD mostly consists of polyostotic forms. Fibrous dysplasia has a predilection for certain bones including the femur, tibia, ribs, pelvis, and craniofacial bones. Radiologically, FD lesions have a lytic appearance and are characterized by thinning of the cortex with a ground glass matrix and can be confirmed by a biopsy of the bone lesions. It is not uncommon for FD to be a silent disease, and some patients are asymptomatic; but when symptoms are present, they most often include bone pain, headache, bone deformities and asymmetry of the face, proptosis, diplopia, and pathological fractures, the latter especially during childhood. In our case, there were recurrent fractures of long bones in childhood period but not in later adolescent period. Involvement of craniofacial site occurs in 25% of FD patients. Facial involvement can cause both facial asymmetry and compression for the anatomic structures located near to the affected bone.