口腔颌面部溶骨症

Gorham-Stout syndrome in the maxillofacial region

溶骨症又称Gorham-Stout综合征,以自发性进行性骨吸收为主要特点,在临床上较罕见,其病因及发病机制尚不明确。病理以薄壁血管增生和纤维组织代替消失的骨组织为主要特征,临床表现多样,可发生于身体多处骨组织,甚或侵及临近的软组织。目前国内外关于颌面部溶骨症的报道较少,而且治疗方法的效果评价没有明确的标准。本文根据国内外文献,对该病的病因、临床表现、诊断与鉴别诊断及治疗进行简要综述。

Gorham-Stout syndrome, which is also called massive osteolysis, vanishing bone disease, phantom bone disease, is characterized by spontaneous progressie bone resorption, and is a rare disorder in the clinical, whereas the etiology is unknown. Gorham-Stout syndrome is characterized histologically by the proliferation of endothelial-lined vessels in bone and by the replacement of bone with fibrous tissue. Clinical manifestations are diverse. In most cases, only a single bone or several bones are affected. Associated infiltrative soft tissue abnormality is often found. Reports of maxillofacial bone resorption are few. According to the literature of domestic and overseas, the aim of this paper is to make a brief review about the etiology, clinical manifestations, diagnosis and differential diagnosis and therapy of Gorham-Stout syndrome.