Exner G U, von Hochstetter A R
Klinik Balgrist, Orthopädische Universitätsklinik Zürich.
Orthopade. 1995 Feb;24(1):50-6.
Fibrous and osteofibrous dysplasia are benign lesions of the bone involving single bones as monostotic or multiple bones as polyostotic forms. When the patient is in the growth phase, the lesions, which are usually asymptomatic initially, may lead to deformities with typical bowing of the proximal femur ("shepherd's crook") and fractures. It is important to recognize osteofibrous dysplasia of the tibia and fibula, where any surgical measures, even a small biopsy, may activate the process and cause the lesion to grow, although it may spontaneously rest around puberty. Polyostotic fibrous dysplasia may rarely be associated with areas of cutaneous pigmentation and endocrine disturbances, especially precocious puberty (McCune-Albright syndrome).
纤维性发育异常和骨纤维性发育异常是骨骼的良性病变,可累及单骨(单骨型)或多骨(多骨型)。在患者生长阶段,这些病变最初通常无症状,但可能导致畸形,典型表现为股骨近端弯曲(“牧羊人之钩”)和骨折。认识到胫腓骨的骨纤维性发育异常很重要,因为在此处,任何手术措施,哪怕是小活检,都可能激活病变进程并导致其生长,尽管病变在青春期左右可能会自行静止。多骨型纤维性发育异常很少与皮肤色素沉着区域和内分泌紊乱相关,尤其是性早熟(McCune - Albright综合征)。