[Fibrous dysplasia]. / Fibröse Dysplasie.

ABSTRACT

Fibrous dysplasia is a sporadically occurring benign skeletal disease characterized by the replacement of normal bone tissue with excessively proliferating cellular fibrous tissue. It can occur in a monostotic or polyostotic form. Depending on the location, number and size of the lesions, the clinical picture can vary from an asymptomatic disease to a severe disability. Typical problems are bone pain, bone deformities and pathological fractures. In combination with endocrinopathies and/or skin manifestations (café au lait spots), it is referred to as the McCune-Albright syndrome. The diagnosis is mainly carried out radiologically and the bony lesions are characterized by a cloudy, frosted glass-like aspect. Causal treatment is not possible. Orthopedic treatment includes pain relief, bone stabilization, deformity correction and, if necessary, lesion cleansing as well as the prevention of progression by means of antiresorptive medication. Pathological fractures are preferably stabilized with intramedullary osteosynthesis procedures.