骨纤维异常增殖症(fibrous dysplasia of bone,FD)是一种髓内良性的纤维性-骨性病变,可累及单骨或多骨.颅颌面骨为FD的高发病率部位.颅颌面骨纤维异常增殖症(craniofacial fibrous dysplasia,CFD)多表现为头面部无痛渐进的单个或多个非炎性肿块,不同颅面部组织器官受累可相应出现面部畸形、视力受损、鼻窦感染、听力障碍、牙齿移位缺失等,严重影响患者的容貌、生活质量和身心健康.特殊类型的CFD患者还可伴有皮肤和/或内分泌系统紊乱等骨骼外表现.由于CFD疾病谱复杂、临床表型多样,目前对于CFD的治疗策略仍处于探索阶段.现就如何平衡保守治疗与外科治疗,在适当的手术时机制定个性化手术方案,建立多学科合作团队等问题作一综述.
Comprehensive treatment strategy for craniomaxillofacial fibrous dysplasia
Craniofacial fibrous dysplasia(CFD)occurs in the craniofacial bones.CFD is characterized by painless and progressive single or multiple non-inflammatory masses on the head and face.The involvement of different craniofacial tissues and organs may result in facial deformities,impaired vision,sinus infections,hearing impairment,and displaced missing teeth,which seriously affect the patient's appearance,quality of life,and physical and mental health.Patients with specific types of CFD may also have extra-skeletal manifestations such as skin and/or endocrine system disorders.Due to the complex disease spectrum and diverse clinical phenotypes of CFD,the treatment strategy for CFD is still in the exploratory stage.We have discussed how to balance conservative and surgical treatment,develop individual-ized surgical plans at the appropriate time of surgery,and establish multidisciplinary teams for this disease.
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