Fibrous dysplasia of bone is a rare, non-inherited skeletal disorder characterized by the abnormal development of fibrous tissues within the bones. It is a benign condition that primarily affects the long bones, ribs, skull, and facial bones. This disorder occurs due to a somatic mutation during embryonic development, resulting in the substitution of normal bone with abnormal fibrous tissue.
The affected bones in fibrous dysplasia become weak and brittle, leading to an increased susceptibility to fractures. Symptoms often include bone pain, deformities, and uneven growth. The involved bones may present with a characteristic appearance on imaging studies, showing areas of opacity mixed with areas of radiolucency, giving the appearance of a "ground-glass" appearance.
Fibrous dysplasia of bone can occur in isolated cases or be part of a syndrome, such as McCune-Albright syndrome. In McCune-Albright syndrome, patients may develop additional features including endocrine abnormalities (such as early puberty or hormonal imbalances) and patches of abnormal skin pigmentation.
Treatment for fibrous dysplasia is mainly focused on managing symptoms and preventing complications. This may involve pain management, physical therapy, and monitoring for fractures or deformities. In cases where the disorder significantly affects the patient's quality of life or causes severe complications, surgical intervention may be considered to stabilize or remove affected bones.
In conclusion, fibrous dysplasia of bone is a rare skeletal disorder characterized by the abnormal development of fibrous tissue within bones, resulting in bone weakness, deformities, and increased fracture risk.
