摘要
目的 分析一个巨颌症家系中的 4例巨颌症患者的临床病理学特征、手术方式及随访结果 ,旨在对巨颌症与颌骨其他巨细胞病变的鉴别诊断和手术时机的选择有所帮助。方法 对一个巨颌症家系中的 4例患者的遗传学、临床、影像、血液学、组织学、生物学特征、治疗方式与随访的资料进行分析。结果 巨颌症在临床上表现为双侧颌骨的无痛性肿大 ,常发生在下颌 ;在影像学上表现为边界清楚的多房性透射影 ;在病理学上 ,骨组织被增生的纤维组织代替 ,纤维结缔组织内血管丰富 ,多核巨细胞沿血管排列 ,伴或不伴嗜伊红物质环绕成袖口状 ;1例未进行治疗的患者 ,病变自然终止 ;其余进行手术治疗的 3例患者术后未见复发。结论 巨颌症可根据其典型的临床、影像学特征 ,结合家族史及病理学特征作出诊断。虽然它的生物学特征表明 ,青春期后病变会逐渐消退 ,但病变期进行手术治疗不仅可以改善患者的生理和心理功能 。
Objective To investigate the clinicopathologic features of familial cherubism and its differentiation from other giant cell lesions in jaws and the results of surgical treatments with a long-term follow-up Methods Four cases of familial cherubism were reviewed and their clinical and radiographic features, histopathologic appearance, biochemical markers and surgical treatments analysed Results Clinically , cherubism was characterized by bilateral painless swelling of jaws, mandibular deformity was common Radiographs showed multilocular radiolucencies with sclerotic thickening border Histopathologically, numerous randomly distributed multinucleated giant cells and vascular spaces within a fibrous connective tissue stroma with or without eosinophilic collagen perivascular cuffing were shown The lesion regressed without treatment in 1 cases Curettage was performed in 3 cases with good results Conclusions Cherubism can be diagnosed according to its typical clinical and radiographical features with a positive family history It might regress without treatment But surgery intervention is suggested to improve physiological function and to solve the psychologic problem of the patients
出处
《中华口腔医学杂志》
CAS
CSCD
北大核心
2004年第6期475-477,共3页
Chinese Journal of Stomatology
基金
国家自然科学基金资助项目 (3 0 2 714 12 )
