Fibrous dysplasia of a bone
Contents:
Description:
Fibrous dysplasia — the disease which is characterized by disturbance of development (dysplasia) of a skeleton at which the normal bone is replaced with fibrous fabric with elements dysplasticly of the changed bone. Allocate monoossalny (about 85% of cases), monomelichesky (several next bones of one extremity, a shoulder or pelvic girdle are affected) and poliossalny (about 5% of cases) forms. The fibrous dysplasia as anomaly of development is sometimes combined with extra skeletal frustration: 1) the poliossalny form of defeat is combined with premature puberty and a nevus pigmentosus on skin in the form of coffee spots (Albright’s syndrome); 2) combination of a fibrous dysplasia to myxomas of soft tissues (Mazabraud’s syndrome). Meets at any age, it is the most frequent in the first 3 decades of life (70% of patients). The Monoossalny form meets at males, at a poliossalny form a ratio of persons men's and female 2 a little more often: 1. Any bone, the most frequent localization is surprised: proximal part of a femur, tibial and humeral bones, edges, bones of a skull, cervical department of a backbone. Damage of bones of a backbone only in 2,5% of cases.
Symptoms of the Fibrous dysplasia of a bone:
Pains of various intensity, curvature and deformation of bones, pathological changes.
Radiological — lytic defeat with accurate contours and the sclerosed edges; there can be bone swelling, a pathological change.
Patomorfologiya.
Macroscopically — dense fabric of white-gray color with small inclusions of bone density; pathological fabric is almost always located only in the marrowy channel.
Microscopy. A connective tissue stroma with bone trabeculas of a primitive structure, miksoidny changes, structures like cementomas, the centers of chondroid fabric, a cyst sometimes form. Bone trabeculas are only sometimes limited to osteoblasts. Cytologic atypia and mitoses are not characteristic. Seldom or never the atipizm of cells connected with degenerative or regressive changes can be observed.
Fibrous dysplasia of bones of a skull
Reasons of the Fibrous dysplasia of a bone:
The fibrous dysplasia is anomaly of development of bone system.
Treatment of the Fibrous dysplasia of a bone:
Regional resection of a bone with the subsequent plastics of defect. At the expressed deformations of bones, the regional resection has to be combined with corrective osteotomies with bone or nailing.
The outcome is favorable; the ozlokachestvleniye of a fibrous dysplasia is observed in 0,4% of cases, at Albright’s syndrome — in 4% of cases, more often at the age of 30–40 years. The fibrous dysplasia which is localized in bones of a facial skeleton (the lower and upper jaws) then a femur and pelvic bones ozlokachestvlyatsya generally.
